Taylors.10 Minute - Diagnosis.manual - Symptoms.and - Signs.in - The.time Limited - Encounter.

Table of Contents
Front
................................................................................................................................................................................................................
Cover ....................................................................................................................................................................................................... 1
Editors and Authors ........................................................................................................................................................................... 2
Dedication ........................................................................................................................................................................................... 24
Preface to the Second Edition ................................................................................................................................................... 25
Preface to the First Edition ......................................................................................................................................................... 26
Acknowledgments ........................................................................................................................................................................... 27
Contents ................................................................................................................................................................................................... 28
1 - Principles of the 10-Minute Diagnosis .................................................................................................................... 33
2 - Undifferentiated Problems ................................................................................................................................................. 38
2.1 - Anorexia .................................................................................................................................................................................... 38
2.2 - Dizziness .................................................................................................................................................................................. 44
2.3 - Edema ....................................................................................................................................................................................... 48
2.4 - Falls ............................................................................................................................................................................................ 51
2.5 - Fatigue ....................................................................................................................................................................................... 57
2.6 - Fever .......................................................................................................................................................................................... 61
2.7 - Headaches ............................................................................................................................................................................... 66
2.8 - Hypersomnia ........................................................................................................................................................................... 70
2.9 - Insomnia ................................................................................................................................................................................... 75
2.10 - Nausea and Vomiting ....................................................................................................................................................... 79
2.11 - Night Sweats ......................................................................................................................................................................... 83
2.12 - Syncope .................................................................................................................................................................................. 86
2.13 - Weight Loss .......................................................................................................................................................................... 89
3 - Mental Health Problems ...................................................................................................................................................... 93
3.1 - Anxiety ....................................................................................................................................................................................... 93
3.2 - Depression ............................................................................................................................................................................... 97
3.3 - Suicide Risk ......................................................................................................................................................................... 102
4 - Problems Related to the Nervous System ........................................................................................................ 106
4.1 - Ataxia ...................................................................................................................................................................................... 106
4.2 - Coma ....................................................................................................................................................................................... 109
4.3 - Delirium .................................................................................................................................................................................. 112
4.4 - Dementia ............................................................................................................................................................................... 116
4.5 - Memory Impairment ......................................................................................................................................................... 120
4.6 - Paresthesia and Dysesthesia ....................................................................................................................................... 124
4.7 - Seizures ................................................................................................................................................................................. 128
4.8 - Stroke ...................................................................................................................................................................................... 132
4.9 - Tremors .................................................................................................................................................................................. 135
5 - Eye Problems ............................................................................................................................................................................ 138
5.1 - Blurred Vision ...................................................................................................................................................................... 138
5.2 - Corneal Foreign Body ...................................................................................................................................................... 142
5.3 - Diplopia .................................................................................................................................................................................. 145
5.4 - Nystagmus ............................................................................................................................................................................ 149
5.5 - Papilledema ......................................................................................................................................................................... 154
5.6 - Pupillary Inequality ............................................................................................................................................................ 158
5.7 - Red Eye ................................................................................................................................................................................. 162
5.8 - Scotoma ................................................................................................................................................................................. 167
6 - Ear, Nose, and Throat ....................................................................................................................................................... 173
6.1 - Halitosis ................................................................................................................................................................................. 173
6.2 - Hearing Loss ........................................................................................................................................................................ 177
6.3 - Hoarseness ........................................................................................................................................................................... 181
6.4 - Nosebleed ............................................................................................................................................................................. 185

6.5 - Pharyngitis ............................................................................................................................................................................ 189
6.6 - Rhinitis .................................................................................................................................................................................... 194
6.7 - Stomatitis .............................................................................................................................................................................. 198
6.8 - Tinnitus ................................................................................................................................................................................... 202
6.9 - Vertigo .................................................................................................................................................................................... 207
7 - Cardiovascular Problems
................................................................................................................................................
212
7.1 - Atypical Chest Pain ........................................................................................................................................................... 212

7.2 - Chest Pain ............................................................................................................................................................................ 216
7.3 - Bradycardia .......................................................................................................................................................................... 220
7.4 - Cardiomegaly ...................................................................................................................................................................... 224
7.5 - Congestive Heart Failure ............................................................................................................................................... 228
7.6 - Heart Murmurs, Diastolic ............................................................................................................................................... 233
7.7 - Heart Murmurs, Systolic ................................................................................................................................................. 238
7.8 - Hypertension ........................................................................................................................................................................ 243
7.9 - Palpitations ........................................................................................................................................................................... 251
7.10 - Pericardial Friction Rub ................................................................................................................................................ 255
7.11 - Raynaud's Disease ......................................................................................................................................................... 258
7.12 - Tachycardia ........................................................................................................................................................................ 261
8 - Respiratory Problems
9-
......................................................................................................................................................... 264
8.1 - Cough ..................................................................................................................................................................................... 264
8.2 - Cyanosis ................................................................................................................................................................................ 268
8.3 - Hemoptysis ........................................................................................................................................................................... 272
8.4 - Pleural Effusion .................................................................................................................................................................. 276
8.5 - Pleuritic Pain ....................................................................................................................................................................... 280
8.6 - Pneumothorax ..................................................................................................................................................................... 285
8.7 - Shortness of Breath .......................................................................................................................................................... 289
8.8 - Stridor ..................................................................................................................................................................................... 293
8.9 - Wheezing .............................................................................................................................................................................. 297
Gastrointestinal Problems .............................................................................................................................................. 301
9.1 - Abdominal Pain .................................................................................................................................................................. 301
9.2 - Ascites .................................................................................................................................................................................... 306
9.3 - Constipation .......................................................................................................................................................................... 311
9.4 - Diarrhea ................................................................................................................................................................................. 317
9.5 - Dysphagia ............................................................................................................................................................................. 323

9.6 - Epigastric Distress ............................................................................................................................................................. 330
9.7 - Upper Gastrointestinal Bleeding ................................................................................................................................. 336
9.8 - Hepatitis ................................................................................................................................................................................. 340
9.9 - Hepatomegaly ..................................................................................................................................................................... 344
9.10 - Jaundice .............................................................................................................................................................................. 348
9.11 - Rectal Bleeding ................................................................................................................................................................ 353
9.12 - Steatorrhea ........................................................................................................................................................................ 356
10 - Renal and Urologic Problems
..................................................................................................................................
361
10.1 - Dysuria ................................................................................................................................................................................. 361

10.2 - Hematuria ........................................................................................................................................................................... 364
10.3 - Impotence ........................................................................................................................................................................... 369
10.4 - Urinary Incontinence in Adults ................................................................................................................................... 372
10.5 - Nocturia ............................................................................................................................................................................... 377
10.6 - Oliguria and Anuria ......................................................................................................................................................... 381
10.7 - Priapism .............................................................................................................................................................................. 385
10.8 - Scrotal Mass ...................................................................................................................................................................... 388
10.9 - Scrotal Pain ....................................................................................................................................................................... 392
10.10 - Urethral Discharge ....................................................................................................................................................... 397
11 - Problems Related to the Female Reproductive System .................................................................... 402
402
406
411
414
417
422
426
435
439
12 - Musculoskeletal Problems .......................................................................................................................................... 444
12.1 - Arthralgia ............................................................................................................................................................................. 444
12.2 - Calf Pain ............................................................................................................................................................................. 448
12.3 - Hip Pain ............................................................................................................................................................................... 452
12.4 - Knee Pain ........................................................................................................................................................................... 456
12.5 - Low Back Pain .................................................................................................................................................................. 462
12.6 - Monoarticular Joint Pain .............................................................................................................................................. 468
12.7 - Neck Pain ........................................................................................................................................................................... 474
12.8 - Polymyalgia ....................................................................................................................................................................... 481
12.9 - Shoulder Pain ................................................................................................................................................................... 485
13 - Dermatologic Problems ................................................................................................................................................. 491
13.1 - Alopecia ............................................................................................................................................................................... 491
13.2 - Erythema Multiforme ..................................................................................................................................................... 495
13.3 - Maculopapular Rash ...................................................................................................................................................... 498
13.4 - Pigmentation Disorders ................................................................................................................................................ 502
13.5 - Pruritus ................................................................................................................................................................................ 507
13.6 - Rash Accompanied by Fever ..................................................................................................................................... 511
13.7 - Urticaria ............................................................................................................................................................................... 515
13.8 - Vesicular and Bullous Eruptions ............................................................................................................................... 518
14 - Endocrine and Metabolic Problems .................................................................................................................... 522
14.1 - Diabetes Mellitus ............................................................................................................................................................. 522
14.2 - Gynecomastia ................................................................................................................................................................... 526
14.3 - Hirsutism ............................................................................................................................................................................. 530
14.4 - Hypothyroidism ................................................................................................................................................................ 535
14.5 - Polydipsia ........................................................................................................................................................................... 539
14.6 - Thyroid Enlargement, Goiter ...................................................................................................................................... 543
14.7 - Thyroid Nodule ................................................................................................................................................................. 547
14.8 - Hyperthyroidism, Thyrotoxicosis .............................................................................................................................. 551
15 - Vascular and Lymphatic System Problems ................................................................................................... 556
15.1 - Lymphadenopathy, Generalized ............................................................................................................................... 556
15.2 - Lymphadenopathy, Localized .................................................................................................................................... 560
15.3 - Petechiae and Purpura ................................................................................................................................................. 563
15.4 - Splenomegaly ................................................................................................................................................................... 567
16 - Laboratory Abnormalities - Hematology and Urine Determinations ......................................... 571
16.1 - Anemia ................................................................................................................................................................................. 571
16.2 - Eosinophilia ....................................................................................................................................................................... 576
16.3 - Neutropenia ....................................................................................................................................................................... 579
16.4 - Polycythemia ..................................................................................................................................................................... 582
16.5 - Thrombocytopenia .......................................................................................................................................................... 585
16.6 - Erythrocyte Sedimentation Rateand C-Reactive Protein .............................................................................. 588
16.7 - Proteinuria .......................................................................................................................................................................... 592
17 - Laboratory Abnormalities - Blood Chemistry and Immunology .................................................... 595
17.1 - Alkaline Phosphatase, Elevated ............................................................................................................................... 595
17.2 - Aminotransferase Levels, Elevated ........................................................................................................................ 600
17.3 - Antinuclear Antibody Titer, Elevated ...................................................................................................................... 605
11.1 - Amenorrhea .......................................................................................................................................................................
11.2 - Breast Mass .......................................................................................................................................................................
11.3 - Chronic Pelvic Pain ........................................................................................................................................................
11.4 - Dysmenorrhea ..................................................................................................................................................................
11.5 - Menorrhagia .......................................................................................................................................................................
11.6 - Nipple Discharge in the Nonpregnant Female ...................................................................................................
11.7 - Pap Smear Abnormality ...............................................................................................................................................
11.8 - Postmenopausal Bleeding ...........................................................................................................................................
11.9 - Vaginal Discharge ...........................................................................................................................................................
17.4 - Brain Natriuretic Peptide .............................................................................................................................................. 611

17.5 - D-Dimer ............................................................................................................................................................................... 616
17.6 - Hypercalcemia .................................................................................................................................................................. 621
17.7 - Hyperkalemia .................................................................................................................................................................... 626
17.8 - Hypokalemia ..................................................................................................................................................................... 632
18 - Diagnostic Imaging Abnormalities

18.1 18.2 18.3 18.4 -
........................................................................................................................
Bone Cyst ...........................................................................................................................................................................
Mediastinal Mass .............................................................................................................................................................
Osteopenia .........................................................................................................................................................................
Solitary Pulmonary Nodule .........................................................................................................................................
636
636
639
644
649
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Cover
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Editors and Authors
Editors:Paulm an, Paul M.; Paulm an, Audre y A.; Harrison, Je ffre y D.
Title : Taylor's 10- M inute D iagnosis M anual: S ymptoms and S igns in the
T i m e - L i m i t e d E n c o u n t e r, 2 n d E d i t i o n
C o p y r i g h t 2 0 0 7 L i p p i n c o t t W i l l i a ms & W i l k i n s
> Front of Book > Editors
E dit ors
Paul M. Paulm an M D
Professor/Predoctoral Director
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of N ebr as k a M edi c al C ent er, O maha,
N ebr as k a
Audre y A. Paulm an M D
C linical Associate P rofessor
N ebr as k a
Je ffre y D. Harrison M D
Program Director
R ur al R es i denc y P r ogr am, D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of
N ebr as k a M edi c al C ent er, O maha, N ebr as k a
Secondary Editors
Sony a Se igafuse
Acquisitions E ditor
N a n c y Wi n t e r
Managing Editor
Martha Cushm an
D e v e l o p me n t a l E d i t o r
Alicia Jackson
Project Manager
Kathle e n Brow n
Manufacturing Manager
Kim be rly Schonbe rge r
Marketing Manager
Larry Didona
Cover Designer
Te r r y M a l l o n
Design Coordinator
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Editors and Authors
Lase rw ords Priv ate Lim ite d, Che nnai, I ndia

P r oduc t i on S er v i c es
Donne lle y Craw fordsv ille
P r i nt er
Section Editors
Kathry n M. Andolse k M D, M P H
Clinical Professor
D epar t ment of C ommuni t y and F ami l y M edi c i ne, D uk e U ni v er s i t y S c hool of
M edi c i ne; A s s oc i at e D i r ec t or, G r aduat e M edi c al E duc at i on, D uk e U ni v er s i t y
H os pi t al , D ur ham, N or t h C ar ol i na
11 . P r o b l e ms R e l a t e d t o t h e F e ma l e R e p r o d u c t i v e S y s t e m
Gre gory J. Babbe M D
Assistant P rofessor
N ebr as k a
1 5 . Va s c u l a r a n d L y mp h a t i c S y s t e m P r o b l e ms
Richard D. Blonde ll M D
Associate P rofessor
D epar t ment of F ami l y M edi c i ne, S t at e U ni v er s i t y of N ew Yor k at B uf f al o;
A t t endi ng P hy s i c i an, D epar t ment of C hemi c al D ependenc y, E r i e C ount y M edi c al
C ent er, B uf f al o, N ew Yor k
1 4 . E n d o c r i n e a n d M e t a b o l i c P r o b l e ms
Frank S. Ce le stino M D
D epar t ment of F ami l y and C ommuni t y M edi c i ne, Wak e F or es t U ni v er s i t y, S c hool
of M edi c i ne; A t t endi ng P hy s i c i an, D epar t ment of F ami l y and C ommuni t y
M edi c i ne, N or t h C ar ol i na B apt i s t H os pi t al , Wi ns t on- S al em, N or t h C ar ol i na
6 . 9 E a r, N o s e , a n d T h r o a t P r o b l e ms
R . W h i t n e y C u r r y J r. M D
P r o f e s s o r a n d C h a i r ma n
D epar t ment of C ommuni t y H eal t h and F ami l y M edi c i ne, U ni v er s i t y of F l or i da,
G ai nes v i l l e, F l or i da
1 0 . R e n a l a n d U r o l o g i c P r o b l e ms
Enrique S. Fe rnande z M D, M S Ed
D epar t ment of C l i ni c al F ami l y M edi c i ne and C ommuni t y H eal t h, U i v er s i t y of
M i ami S c hool of M edi c i ne, M i ami , F l or i da, D i agnos t i c I magi ng A bnor mal i t i es
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Editors and Authors
1 8 . D i a g n o s t i c I ma g i n g A b n o r ma l i t i e s
Judith A. Fishe r M D
D epar t ment of F ami l y P r ac t i c e and C ommuni t y M edi c i ne, U ni v er s i t y of
P enns y l v ani a S c hool of M edi c i ne, P hi l adel phi a, P enns y l v ani a
1 7 . L a b a r o t o r y A b n o r ma l i t i e s : B l o o d C h e mi s t r y a n d I mmu n o l o g y
Richard H. Hurd M D
Director
D epar t ment of C l ar k s on F ami l y M edi c i ne; P hy s i c i an, D epar t ment of F ami l y
M edi c i ne, N ebr as k a M edi c al C ent er, O maha, N ebr as k a
2 . U n d i f f e r e n t i a t e d P r o b l e ms
Carol A. LaCroix M D
C linical Assistant P rofessor
N ebr as k a
1 6 . L a b o r a t o r y A b n o r ma l i t i e s : H e ma t o l o g y a n d U r i n e D e t e r mi n a t i o n s
Shou Ling Le ong M D
Professor
D epar t ment of F ami l y and C ommuni t y M edi c i ne, P enn S t at e C ol l ege of M edi c i ne;
P r of es s or, F ami l y and C ommuni t y M edi c i ne, M i l t on S . H er s hey M edi c al C ent er,
H er s hey, P hi l adel phi a
5 . E y e P r o b l e ms
Jim Me dde r M D, M P H
N ebr as k a
3 . M e n t a l H e a l t h P r o b l e ms
Michae l A. My e rs M D
Program Director
L i nc ol n F ami l y M edi c i ne P r ogr am, L i nc ol n M edi c al E duc at i on P ar t ner s hi p,
L i nc ol n, N ebr as k a
4 . P r o b l e ms R e l a t e d t o t h e N e r v o u s S y s t e m
Lae th Nasir M B B S
Professor
N ebr as k a
1 2 . M u s c u l o s k e l e t a l P r o b l e ms
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Editors and Authors
Michae l L. O'De ll M D, M S H A
Chair and Program Director
D epar t ment of F ami l y M edi c i ne, N or t h M i s s i s s i ppi M edi c al C ent er F ami l y
M edi c i ne R es i denc y ; C hai r, D epar t ment of F ami l y M edi c i ne, N or t h M i s s i s s i ppi
M edi c al C ent er, Tupel o, M i s s i s s i ppi
1 3 . D e r ma t o l o g i c P r o b l e ms
Jose ph E. Sche rge r M D
Professor
D epar t ment of F ami l y and P r ev ent i v e M edi c i ne, U ni v er s i t y of C al i f or ni a, S an
D i ego, C al i f or ni a
8 . R e s p i r a t o r y P r o b l e ms
Joann E. Schae fe r M D
D epar t ment of F ami l y M edi c i ne, C r ei ght on U ni v er s i t y M edi c al C ent er, O maha,
N ebr as k a
7 . C a r d i o v a s c u l a r P r o b l e ms
Michae l R. Spie ke r M D
Associate C linical P rofessor
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of Was hi ngt on, S eat t l e, Was hi ngt on;
S t af f P hy s i c i an, D epar t ment of F ami l y M edi c i ne, N av al H os pi t al B r emer t on,
B r emer t on, Was hi ngt on
9 . G a s t r o i n t e s t i n a l P r o b l e ms
R o b e r t B . Ta y l o r M D
Professor
D epar t ment of F ami l y M edi c i ne, O r egon H eal t h S c i enc es U ni v er s i t y, S c hool of
M edi c i ne, P or t l and, O r egon
1. Principles of the 10-Minute Diagnosis
C ont ribut ors

Sam ue l B. Adkins I I I M D
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of Tex as M edi c al B r anc h, G al v es t on,
Tex as ; D i r ec t or, F ami l y M edi c i ne R es i denc y, A us t i n M edi c al E duc at i on P r ogr am,
A us t i n, Tex as
12.3 Hip Pain
P a u l V . A i t k e n J r. M D , M P H
D epar t ment of F ami l y and C ommuni t y M edi c i ne, P enn S t at e U ni v er s i t y C ol l ege of
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Editors and Authors
M edi c i ne, H er s hey, P hi l adel phi a; P r ogr am D i r ec t or, R es i denc y i n F ami l y and
C ommuni t y M edi c i ne, P enn S t at e U ni v er s i t y, G ood S amar i t an H os pi t al , L ebanon,
P hi l adel phi a
11 . 3 C h r o n i c P e l v i c P a i n
11 . 4 D y s me n o r r h e a
I m an S. Al-Jabi M D
F a c u l t y M e mb e r a n d L e c t u r e r
D epar t ment of C ommuni t y and F ami l y M edi c i ne, U ni v er s i t y of J or dan, A mman,
J or dan
12.1 Arthralgia
Karthry n M. Andolse k M D, M P H
Clinical Professor
M edi c i ne; A s s oc i at e D i r ec t or, D epar t ment of G r aduat e, M edi c al E duc at i on, D uk e
U ni v er s i t y H os pi t al , D ur ham, N or t h C ar ol i na
11 . 5 M e n o r r h a g i a
Mark D. Andre w s M D
D epar t ment of F ami l y M edi c i ne, Wak e F or es t U ni v er s i t y S c hool of M edi c i ne,
Wi ns t on- S al em, N or t h C ar ol i na
6.1 Halitosis
Kam le sh G. Ansingkar M D, M S H I
F i n a l Ye a r R e s i d e n t
N ebr as k a
7.10 Pericardial Friction Rub
Elisabe th L. Backe r M D
N ebr as k a
1 6 . 3 E r y t h r o c y t e S e d i me n t a t i o n R a t e A n d C - R e a c t i v e P r o t e i n
Jame s R. Barre tt M D
Professor
D epar t ment of F ami l y and P r ev ent i v e M edi c i ne, U ni v er s i t y of O k l ahoma, H eal t h
S c i enc es C ent er, O k l ahoma c i t y, O k l ahoma
12.7 Neck Pain
Sandra B. Baum be rge r M D
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Editors and Authors
Chief Resident
D epar t ment of F ami l y M edi c i ne, C r ei ght on U ni v er s i t y M edi c al S c hool , C r ei ght on
U ni v er s i t y M edi c al C ent er, O maha, N ebr as k a
7 . 11 R a y n a u d ' s D i s e a s e
Thom as C. Be nt M D
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of C al i f or ni a, I r v i ne, O r ange,
C al i f or ni a; M edi c al D i r ec t or, C hi ef O per at i ng O f f i c er, L aguna B eac h C ommuni t y
C l i ni c , L aguna B eac h, C al i f or ni a
8 . 9 W h e e zi n g
Charle s S. Blackadar M D
S t af f F ac ul t y P hy s i c i an, P uget S ound F ami l y M edi c i ne R es i denc y, B r emer t on
N av al H os pi t al , B r emer t on, Was hi ngt on
9.7 Upper Gastrointestinal Bleeding
Douglas G. Brow ning M D
of M edi c i ne; A s s i s t ant P r of es s or, D epar t ment of F ami l y and C ommuni t y M edi c i ne,
N or t h C ar ol i na B apt i s t H os pi t al , Wi ns t on- S al em, N or t h C ar ol i na
6.4 Nosebleed
C h a r l e s L . B r y n e r J r. M D , P h D
D epar t ment of F ami l y M edi c i ne, O r ange P ar k M edi c al C ent er, O r ange P ar k ,
F l or i da
9.12 Steatorrhea
Je nnife r J. Bue sche r M D, M S P H
Faculty
M edi c i ne, T he N ebr as k a M edi c al C ent er, O maha, N ebr as k a
2 . 8 H y p e r s o mn i a
2 . 9 I n s o mn i a
2 . 1 0 N a u s e a a n d Vo mi t i n g
Ke ndall M. Cam pbe ll M D
D epar t ment of C ommuni t y H eal t h and F ami l y M edi c i ne, U ni v er s i t y of F l or i da
C ol l ege of M edi c i ne, G ai nes v i l l e, F l or i da
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Editors and Authors
10.8 Scrotal Mass

Sandra M. Carr-Johnson M D
Chief
C l i ni c al E duc at i on and S er v i c es ; P r ogr am D i r ec t or, F ami l y M edi c i ne R es i dent ,
S out her n R egi onal A r ea H eal t h C ent er, F ay et t ev i l l e, N or t h C ar ol i na
11 . 9 Va g i n a l D i s c h a r g e
Katrina Carte r M D
D epar t ment of F ami l y M edi c i ne, N ebr as k a M edi c al C ent er, O maha, N ebr as k a
1 6 . 1 A n e mi a
1 6 . 5 P o l y c y t h e mi a
Frank S. Ce le stino M D
of M edi c i ne; A t t endi ng P hy s i c i an, D epar t ment of F ami l y and C ommuni t y
6 . 9 Ve r t i g o
Ku-Lang Chang M B, B C H
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of F l or i da C ol l ege of M edi c i ne,
1 0 . 2 H e ma t u r i a
Brian Cole m an M D
S c i enc es C ent er, O k l ahoma C i t y, O k l ahoma
12.7 Neck Pain
Dav id R. Congdon M D
Assistant P rogram D irector
P uget S ound F ami l y M edi c i ne R es i denc y, B r emer t on N av al H os pi t al , B r emer t on,
Was hi ngt on
9 . 1 A b d o mi n a l P a i n
Joy ce A. Cope land M D
D epar t ment of F ami l y M edi c i ne, D uk e U ni v er s i t y S c hool of M edi c i ne, D ur ham,
N or t h C ar ol i na
11 . 2 B r e a s t M a s s
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11 . 6 N i p p l e D i s c h a r g e I n T h e N o n p r e g n a n t F e ma l e
Ronnie Coutinho M D
D epar t ment of A dv anc ed I nt r oduc t i on t o C l i ni c al M edi c i ne, R os s U ni v er s i t y S c hool
of M edi c i ne, E di s on, N ew J er s ey
18.2 Mediastinal Mass
R o n a l d D . C r a i g M D , F A A F P, D i p l o m a t e A B F P
Faculty Physician
L i nc ol n M edi c al E duc at i on P ar t ner s hi p, B uf f al o, N ew Yor k
4.8 Stroke
4 . 9 T r e mo r s
J. Ste v e n Crame r M D, M S
D epar t ment of F ami l y M edi c i ne, S t at e U ni v er s i t y of N ew Yor k at B uf f al o, B uf f al o,
N ew Yor k
14.1 Diabetes Mellitus
P e t e r F. C r o n h o l m M D , M S C E
Physician
D epar t ment of F ami l y M edi c i ne and C ommuni t y H eal t h, P enn P r es by t er i an
M edi c al C ent er ; A s s i s t ant P r of es s or, D epar t ment of F ami l y M edi c i ne and
C ommuni t y H eal t h, U ni v er s i t y of P enns y l v ani a H eal t h S y s t em, P hi l adel phi a
17.1 Alkaline P hosphatase, E levated
1 7 . 2 A mi n o t r a n s f e r a s e L e v e l s , E l e v a t e d
1 7 . 3 A n t i n u c l e a r A n t i b o d y T i t e r, E l e v a t e d
1 7 . 6 H y p e r c a l c e mi a
L. Gail Curtis M D
D epar t ment of F ami l y and C ommuni t y M edi c i ne, Wak e F or es t U ni v er s i t y M edi c al
C ent er, Wi ns t on- S al em, N or t h C ar ol i na
6.3 Hoarseness
Ta n i k a L . D a y M D
C linical Associate
D epar t ment of C ommuni t y and F ami l y M edi c i ne, D uk e U ni v er s i t y M edi c al C ent er,
D ur ham, N or t h C ar ol i na
11 . 1 A me n o r r h e a
R o n a l d F. D o m m e r m u t h M D
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Editors and Authors

D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of Was hi ngt on; P r ogr am D i r ec t or,
P uget S ound F ami l y M edi c i ne R es i denc y, N av al H os pi t al B r emer t on; S t af f
P hy s i c i an, D epar t ment of F ami l y M edi c i ne, N av al H os pi t al B r emer t on, B r emer t on,
Was hi ngt on
9.10 Jaundice
Ale x andra Duke D O
Assistant C linical P rofessor
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of C al i f or ni a, I r v i ne C ol l ege of
M edi c i ne, I r v i ne, C al i f or ni a
8.8 Stridor
Mike Duke low M D
C r ei ght on F ami l y H eal t hc ar e, O maha, N ebr as k a
7.12 Tachycardia
Kristy D. Edw ards M D, C W S
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of N ebr as k a, N ebr as k a M edi c al
C ent er ; O maha, N ebr as k a
1 5 . 1 L y mp h a d e n o p a t h y, G e n e r a l i ze d
1 5 . 2 L y mp h a d e n o p a t h y, L o c a l i ze d
Chad Ezze ll M D
Sports Medicine Fellow
of M edi c i ne; S por t s M edi c i ne F el l ow, D epar t ment of F ami l y and C ommuni t y
6.4 Nosebleed
Dav id B. Fe lle r M D
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of F l or i da C ol l ege, of M edi c i ne,
10.7 Priapism
Tina M . F lore s M D
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of N ebr as k a, M edi c al C ent er, O maha,
N ebr as k a
16.2 Eosinophilia
Norm an Be njam in Fre drick M D
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D epar t ment of F ami l y and C ommuni t y M edi c i ne, P enns y l v ani a S t at e U ni v er s i t y,
C ol l ege of M edi c i ne; F ac ul t y, M i l t on S . H er s hey M edi c al C ent er, H er s hey,
P enns y l v ani a
5 . 1 B l u r r e d Vi s i o n
5.3 Diplopia
M a r c i a W. F u n d e r b u r k M D
H eal t h S c i enc e C ent er / J ac k s onv i l l e; A s s oc i at e P r of es s or, D epar t ment of
C ommuni t y H eal t h and F ami l y M edi c i ne, S hands at J ac k s onv i l l e, J ac k s onv i l l e,
F l or i da
10.6 O liguria and Anuria
M a r k F. G i g l i o M D
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of C al i f or ni a, I r v i ne C ol l ege of
8.4 Pleural Effusion
Mark D. Goodm an M D
D epar t ment of F ami l y M edi c i ne, C r ei ght on U ni v er s i t y ; C r ei ght on U ni v er s i t y
M edi c al C ent er, O maha, N ebr as k a
He ath A. Grame s PhD
D epar t ment of C hi l d and F ami l y S t udi es , U ni v er s i t y of S out her n M i s s i s s i ppi ,
H at t i es bur g, M i s s i s s i ppi
3.3 Suicide Risk
Sara Gray bill M D
N ebr as k a
16.4 Neutropenia
Christian Ky le Hae fe le M D, FA A F P
Faculty Physician
4 . 4 D e me n t i a
4 . 5 M e mo r y I mp a i r me n t
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Thom as J. Hanse n M D
P r o g r a m D i r e c t o r, A s s i s t a n t P r o f e s s o r
D epar t ment of F ami l y M edi c i ne, C r ei ght on U ni v er s i t y, M edi c al C ent er, O maha,
N ebr as k a
7.1 Atypical C hest P ain
Ste phe n J. Hartsock M D
Advanced O ccupational M edicine S pecialists
C hi c ago, I l l i noi s
12.2 Calf Pain
Robe rt L. Hatch M D, M P H
C ol l ege, of M edi c i ne, G ai nes v i l l e, F l or i da
10.8 Scrotal Mass
Ray m ond D. He lle r M D
D epar t ment of F ami l y M edi c i ne, C r ei ght on U ni v er s i t y, O maha, N ebr as k a
7.8 Hypertension
Dav id C. Holub M D
D epar t ment of F ami l y and C ommuni t y M edi c i ne, P enns y l v ani a S t at e U ni v er s i t y,
C ol l ege of M edi c i ne, H er s hey, P enns y l v ani a; A s s oc i at e P r ogr am D i r ec t or, F ami l y
and C ommuni t y M edi c i ne R es i denc y P r ogr am, G ood S amar i t an H os pi t al , L ebanon,
P enns y l v ani a
5.6 P upillary Inequality
5.7 T he Red Eye
5 . 8 S c o t o ma
Kare n Hughe s M D
Attending F aculty
D epar t ment of F ami l y M edi c i ne, N or t h M i s s i s s i ppi M edi c al C ent er F ami l y
M edi c i ne R es i denc y ; A t t endi ng P hy s i c i an, D epar t ment of F ami l y M edi c i ne, N or t h
M i s s i s s i ppi M edi c al C ent er, Tupel o, M i s s i s s i ppi
13.1 Alopecia
1 3 . 4 P i g me n t a t i o n D i s o r d e r s
Richard H. Hurd M D
Director
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2 . 2 D i zzi n e s s
2 . 11 N i g h t S w e a t s
Scott I ppolito M D
Associate D ean of C linical S ciences
R os s U ni v er s i t y S c hool of M edi c i ne, D omi ni c a, Wes t I ndi es
18.3 Osteopenia
Kim be rly J. Jarzy nka M D
N ebr as k a
1 5 . 4 S p l e n o me g a l y
Am y K. Je spe rse n M D
Director
2.5 Fatigue
2 . 1 3 We i g h t L o s s
Andre w D. Jone s M D
Faculty Physician
D epar t ment of F ami l y M edi c i ne, E x empl a S ai nt J os eph H os pi t al F ami l y M edi c i ne
R es i denc y P r ogr am; E x empl a S ai nt J os eph H os pi t al , D env er, C ol or ado
12.9 Shoulder Pain
Victoria S. Kaprie lian M D
Clinical Professor
M edi c i ne; A t t endi ng S t af f , D uk e U ni v er s i t y H os pi t al , D ur ham, N or t h C ar ol i na
11 . 8 P o s t me n o p a u s a l B l e e d i n g
M a r t i n a K e l l y M D , M R C G P, M I C G P
Lecturer
D epar t ment of G ener al P r ac t i c e, U ni v er s i t y C ol l ege C or k , C or k , I r el and
1 2 . 8 P o l y my a l g i a
Mary D. Knudtson D N Sc, N P
P r o f e s s o r, D i r e c t o r o f F N P P r o g r a m
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of C al i f or ni a, I r v i ne, C ol l ege of
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8 . 3 H e mo p t y s i s
Charle s M. Kodne r M D
D epar t ment of F ami l y and G er i at r i c M edi c i ne, U ni v er s i t y of L oui s v i l l e S c hool , of
M edi c i ne, L oui s v i l l e, K ent uc k y
1 4 . 2 G y n e c o ma s t i a
Dav id C. Krulak M D, M P H
9.4 Diarrhea
Louis Kuritzky M D
1 0 . 3 I mp o t e n c e
Carol A. Lacroix M D
N ebr as k a
16.6 Proteinuria
Kathry n M. Larse n M D
Clinical Professor
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of C al i f or ni a, I r v i ne, C ol l ege of
M edi c i ne, I r v i ne, C al i f or ni a; C hai r, U ni v er s i t y of C al i f or ni a, I r v i ne, M edi c al C ent e
O r ange, C al i f or ni a
8 . 3 H e mo p t y s i s
J o s e p h T. L a V a n M D
Faculty
D epar t ment of F ami l y M edi c i ne, P uget S ound F ami l y M edi c i ne R es i denc y,
B r emer t on, Was hi ngt on; F ac ul t y, D epar t ment of F ami l y M edi c i ne, M adi gan A r my
M edi c al C ent er, Tac oma, Was hi ngt on
9.3 Constipation
Peter R. Lewis MD
D epar t ment of F ami l y and C ommuni t y M edi c i ne, P enn S t at e U ni v er s i t y C ol l ege of
M edi c i ne; P hy s i c i an, D epar t ment of F ami l y and C ommuni t y M edi c i ne, M i l t on S .
H er s hey M edi c al C ent er, H er s hey, P enns y l v ani a
5.2 Corneal Foreign Body
5 . 4 N y s t a g mu s
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5 . 5 P a p i l l e d e ma
Dsire A. Lie M D, M S Ed
Clinical Professor
C al i f or ni a
8.1 Cough
R i c h a r d W. L o r d M D
6.5 Pharyngitis
J e l y n W. L u M D
Resident
C r ei ght on M edi c al S c hool , O maha, N ebr as k a
7 . 6 D i a s t o l i c H e a r t M u r mu r s
Wi l l i a m M . L u c a s M D
Attending S taff
D epar t ment of F ami l y M edi c i ne, P uget S ound F ami l y M edi c i ne, R es i denc y,
B r emer t or N av al H os pi t al , B r emer t or, Was hi ngt on
9.2 Ascites
Jam e s R. Lundy M D
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of M i s s i s s i ppi M edi c al C ent er,
J ac k s on, M i s s i s s i ppi ; F ac ul t y of F ami l y M edi c i ne R es i denc y P r ogr am, N or t h
M i s s i s s i ppi M edi c al C ent er, Tupel o, M i s s i s s i ppi
13.3 Maculopapular Rash
13.5 Pruritis
Chris Madde n M D
L ongs P eak S por t s and F ami l y M edi c i ne; A s s i s t ant C l i ni c al F ac ul t y, D epar t ment
of F ami l y M edi c i ne, U ni v er s i t y of C ol or ado H eal t h S c i enc es , L ongmont , C ol or ado
12.4 Knee Pain
Barbara A. Maje roni M D
D epar t ment of F ami l y M edi c i ne, S t at e U ni v er s i t y of N ew Yor k at B uf f al o;
A t t endi ng P hy s i c i an, E r i e C ount y M edi c al C ent er, B uf f al o, N ew Yor k
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1 4 . 8 H y p e r t h y r o i d i s m/ T h y r o t o xi c o s i s
Andre a Many on M D
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of B uf f al o, B uf f al o, N ew Yor k
14.1 Diabetes Mellitus
G a i l S . M a r i o n PA - C , P h D
6.6 Rhinitis
Roge r Massie M D
Faculty
2 . 3 E d e ma
2.12 Syncope
Ta h a n y M a u r i c e - H a b a s h y M D
Private practice
O r ange C ount y, C al i f or ni a
8.8 Stridor
Ronald McCoy M B B S
General Practitioner and Medical Educator
R oy al A us t r al i an C ol l ege of G ener al P r ac t i t i oner s , S out h M el bour ne, Vi c t or i a,
A us t r al i a
12.5 Low Back Pain
Lou Ann McStay M D
D epar t ment of F ami l y M edi c i ne, C r ei ght on U ni v er s i t y M edi c al S c hool ; A c t i v e
S t af f , D epar t ment of F ami l y M edi c i ne, C r ei ght on U ni v er s i t y M edi c al C ent er,
O maha, N ebr as k a
7 . 4 C a r d i o me g a l y
Albe rt A. Me y e r M D
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of N or t h C ar ol i na, S c hool of M edi c i ne,
C hapel H i l l , N or t h C ar ol i na; F ami l y M edi c i ne F ac ul t y, R es i denc y i n F ami l y
M edi c i ne, N ew H anov er R egi onal M edi c al C ent er, Wi l mi ngt on, N or t h C ar ol i na
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11 . 3 C h r o n i c P e l v i c P a i n
11 . 4 D y s me n o r r h e a
J o s e p h A . M o r a n M B , B C h , B A O , M C L S C , M R C G P, M I C G P
Lecturer
D epar t ment of G ener al P r ac t i c e, U ni v er s i t y C ol l ege C or k , C or k , I r el and
1 2 . 8 P o l y my a l g i a
S o r a y a P. N a s r a t y M D
Medical Director
D epar t ment of F ami l y and G er i at r i c M edi c i ne, U ni v er s i t y of L oui s v i l l e; A c t i v e
S t af f , D epar t ment of F ami l y M edi c i ne, J ewi s h H os pi t al , N or t on H os pi t al , and
U ni v er s i t y of L ouv i l l e H os pi t al , L oui s v i l l e, K ent uc k y
14.5 Polydipsia
Sara Ne al M D
Assistant D irector of R esidency T raining
D epar t ment of F ami l y and C ommuni t y M edi c i ne, Wak e F or es t U ni v er s i t y S c hool
of M edi c i ne, Wi ns t on- S al em, N or t h C ar ol i na
6.8 Tinnitus
Mark R. Ne e dham M D
S ai nt J ohns H os pi t al , S ant a M oni c a, C al i f or ni a
18.1 Bone Cyst
1 8 . 4 S o l i t a r y P u l mo n a r y N o d u l e
J a n i s F. N e u m a n M D
K ai s er P er manent e M edi c al G r oup, R i v er s i de, C al i f or ni a
8.2 Cyanosis
V i n c e n t H . O b e r J r. B S , M D
C ol l ege of M edi c i ne, J ac k s onv i l l e, F l or i da
10.5 Nocturia
Michae l L. O'De ll M D, M S H A
Chair and Program Director
D epar t ment of F ami l y M edi c i ne, N M M C F ami l y M edi c i ne R es i denc y ; C hai r,
F ami l y M edi c i ne, D epar t ment of F ami l y M edi c i ne, N or t h M i s s i s s i ppi M edi c al
C ent er, Tupel o, M i s s i s s i ppi
1 3 . 6 R a s h A c c o mp a n i e d b y F e v e r
1 3 . 8 Ve s i c u l a r a n d B u l l o u s E r u p t i o n s
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Nicole J. Otto M D
Staff Physician
S t udent H eal t h S er v i c es , U ni v er s i t y of P enns y l v ani a; C l i ni c al A s s oc i at e P r of es s or
D epar t ment of F ami l y M edi c i ne, H os pi t al of t he U ni v er s i t y of P enns y l v ani a,
P enns y l v ani a, P hi l adel phi a
1 7 . 4 B r a i n N a t r i u r e t i c P e p t i d e , 0 7 . 5 D - D i me r
Trish P alm e r M D
S por t s M edi c i ne, D epar t ment of F ami l y M edi c i ne at R us h, M i dwes t O r t hopedi c s at
R us h, C hi c ago, I l l i noi s
12.6 Monoarticular Joint Pain
Pam e la L. Pe ntin M D
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of Was hi ngt on, S c hool of M edi c i ne,
S eat t l e, Was hi ngt on; F ac ul t y P hy s i c i an, D epar t ment of F ami l y M edi c i ne, N av al
H os pi t al B r emer t on, B r emer t on, Was hi ngt on
9.6 Epigastric Distress
T. R a y P e r r i n e M S , M D F A A F P
F aculty Attending
F ami l y M edi c i ne R es i denc y C ent er, N or t h M i s s i s s i ppi M edi c al C ent er ; A c t i v e
S t af f , D epar t ment of F ami l y M edi c i ne, N or t h M i s s i s s i ppi M edi c al C ent er, Tupel o,
M i s s i s s i ppi
1 3 . 2 E r y t h e ma M u l t i f o r me
13.7 Urticaria
Ke nne th D. Pe te rs M D
Faculty
2.4 Falls
2.7 Headaches
C a r l o s A . P r e n d e s J r. M D
D epar t ment of F ami l y M edi c i ne, C r ei ght on U ni v er s i t y ; S t af f , C r ei ght on U ni v er s i t y
M edi c al C ent er, O maha, N ebr as k a
7 . 7 S y s t e mi c H e a r t M u r mu r s
Lay ne A. Pre st B A, M A, PhD
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D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of N ebr as k a M edi c al C ent er ; U N M C
P hy s i c i ans , D epar t ment of F ami l y M edi c i ne, N ebr as k a H eal t h S y s t em, O maha,
N ebr as k a
3 . 1 A n xi e t y
Dav id M. Quille n M D
10.1 Dysuria
Kaly anakrishnan Ram akrishnan M D
S c i enc es C ent er, O k l ahoma C i t y, O k l ahoma
12.9 Shoulder Pain
Richard Rathe M D
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of F l or i da C ol l ege of M edi c i ne,
10.4 U rinary Incontinence in Adults
Robe rt R. Raune r M D, FA A F P
Faculty Physician
4 . 1 A t a xi a
4 . 2 C o ma
4.3 Paresthesia and Dyesthesia
Je ri R. Re id MD
D epar t ment of F ami l y and G er i at r i c M edi c i ne, U ni v er s i t y of L oui s v i l l e, L oui s v i l l e,
K ent uc k y
1 4 . 6 T h y r o i d E n l a r g e me n t / G o i t e r
14.7 T hyroid Nodule
W. D a v i d R o b i n s o n P h D
N ebr as k a
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3.2 Depression
Christine Rom ascan D O
9 . 11 R e c t a l B l e e d i n g
Ge orge P N Sam raj M D, M R C O G
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of F l or i da, G ai nes v i l l e, F l or i da
10.10 Urethral Discharge
He m ant K. Satpathy M D
D epar t ment of F ami l y P r ac t i c e, C r ei ght on U ni v er s i t y M edi c al S c hool , O maha,
N ebr as k a
7.5 Congestive Heart Failure
Chhabi Satpathy M D
Lecturer
D epar t ment of C ar di ol ogy, S C B M edi c al C ol l ege, C ut t ac k , O r i s s a, I ndi a
7.5 Congestive Heart Failure
S i e g f r i e d O . F. S c h m i d t M D , P h D , F A A F P
Associate P rofessor and M edical D irector
H ampt on O ak s M edi c al , P l aza F ami l y M edi c i ne; S t af f P hy s i c i an, D epar t ment of
M edi c i ne, S hands at A G H and S hands at U F, G ai nes v i l l e, F l or i da
1 0 . 2 H e ma t u r i a
Michae l D. Schooff M D
Associate D irector
C l ar k s on F ami l y M edi c i ne, R es i denc y P r ogr am, O maha, N ebr as k a
2 . 1 A n o r e xi a
2.6 Fever
Ke ndall G. Scott M D
D i r e c t o r o f F a mi l y M e d i c i n e
F onat ana, C al i f or ni a
P e r r y W. S e x t o n M D
Staff Physician
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S t udent H eal t h S er v i c es , U ni v er s i t y of P enns y l v ani a; S t af f P hy s i c i an, D epar t ment

of F ami l y M edi c i ne, P enn, P r es by t er i an H os pi t al , P enns y l v ani a, P hi l adel phi a
17.4 Brain Natriuretic Peptide
1 7 . 5 D - D i me r
Sanje e v Sharm a M D
N ebr as k a
7.2 Chest Pain
Hal S. Shim azu M D

Clinical Professor
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of C al i f or ni a, I r v i ne, Tus t i n, C al i f or ni a
John L. Sm ith M D
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of N ebr as k a, C ol l ege of M edi c i ne,
O maha, N ebr as k a
15.3 Petechiae and Purpura
Brian A. Sm ole y M D
F a c u l t y D e v e l o p me n t F e l l o w
D epar t ment of F ami l y M edi c i ne, M adi gan A r my M edi c al C ent er, Tac oma,
Was hi ngt on
9.8 Hepatitis
John G. Spangle r M D
D epar t ment of F ami l y and C ommuni t y M edi c i ne, Wak e F or es t U ni v er s i t y S c hool
of M edi c i ne, Wi ns t on- S al em, N or t h C ar ol i na
6 . 7 S t o ma t i t i s
Re be cca L. Spaulding M D
Clinical Instructor
D epar t ment of F ami l y M edi c i ne U ni v er s i t y of N or t h C ar ol i na, S c hool of M edi c i ne,
C hapel H i l l ; F ac ul t y, D epar t ment of F ami l y M edi c i ne and S por t s M edi c i ne, M os es
C one H eal t h S y s t em, G r eens bor o, N or t h C ar ol i na
12.2 Calf Pain
Michae l R. Spie ke r M D
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Editors and Authors

9.5 Dysphagia
Jose ph B. Straton M D, M S C E
D epar t ment of F ami l y M edi c i ne and C ommuni t y H eal t h, U ni v er s i t y of
P enns y l v ani a, P enns y l v ani a, P hi l adel phi a
17.1 Alkaline P hosphatase, E levated
1 7 . 2 A mi n o t r a n s f e r a s e L e v e l s , E l e v a t e d
1 7 . 3 A n t i n u c l e a r A n t i b o d y T i t e r, E l e v a t e d
1 7 . 6 H y p e r c a l c e mi a
Te r e s a S t u m p D O
1 6 . 7 T h r o mb o c y t o p e n i a
Robe rt M. The al M D
Assistant C hief of S ervice
D epar t ment of F ami l y M edi c i ne, K ai s er F oundat i on H os pi t al , F ont ana, C al i f or ni a
8.7 Shortness of Breath
Fide l A. Vale a M D
A s s o c i a t e P r o f e s s o r, R e s i d e n c y P r o g r a m D i r e c t o r
D epar t ment of O bs t et r i c s and G y nec ol ogy, D uk e U ni v er s i t y M edi c al C ent er ; D uk e
U ni v er s i t y M edi c al C ent er, D ur ham, N or t h C ar ol i na
11 . 7 PA P S me a r A b n o r ma l i t y
Charle s Ve ga M D
C al i f or ni a
8 . 6 P n e u mo t h o r a x
Le s Ve skrna M D
Faculty Physician
4.3 Delirium
4 . 7 S e i zu r e s
D a v i d We b n e r M D
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of P enns y l v ani a; A s s i s t ant P r of es s or,
D epar t ment of F ami l y M edi c i ne, U ni v er s i t y of P enns y l v ani a H eal t h S y s t em,
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Editors and Authors
P enns y l v ani a, P hi l adel phi a

1 7 . 7 H y p e r k a l e mi a
1 7 . 8 H y p o k a l e mi a
S t e p h e n F. W h e e l e r M C h e , M D
D epar t ment of F ami l y and G er i at r i c M edi c i ne, U ni v er s i t y of L oui s v i l l e S c hool of
M edi c i ne, L oui s v i l l e, K ent uc k y
14.4 Hypothyroidism
1 4 . 6 T h y r o i d E n l a r g e me n t / G o i t e r
14.7 T hyroid Nodule
1 4 . 8 H y p e r t h y r o i d i s m/ T h y r o t o xi c o s i s
Darry l G. White M D
D epar t ment of F ami l y M edi c i ne, U T H eal t h S c i enc e C ent er S an A nt oni o; S t af f
P hy s i c i an, C hi ef , D epar t ment of F ami l y M edi c i ne, Val l ey B apt i s t M edi c al C ent er,
H ar l i ngen, Tex as
9 . 9 H e p a t o me g a l y
G e o r g e R . Wi l s o n M D
Associate P rofessor and Associate C hair
C ommuni t y H eal t h and F ami l y M edi c i ne, U ni v er s i t y of F l or i da C ol l ege of
M edi c i ne; C hi ef , D epar t ment of F ami l y M edi c i ne and O c c upat i onal M edi c i ne,
S hands at J ac k s onv i l l e M edi c al C ent er, J ac k s onv i l l e, F l or i da
10.9 Scrotal Pain
J o h n Wi n t e r s M D
Resident
C r ei ght on M edi c al S c hool , O maha, N ebr as k a
7.9 Palpitations
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Dedication
> Front of Book > Dedication
Dedication
T h i s b o o k i s d e d i c a t e d t o o u r c h i l d r e n , R o g e r, K a t e , C r a i g , a n d A d a m , w h o
inspire us
24 / 652
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Preface to the Second Edition
> Front of Book > Preface to the Second Edition
Preface to the Second Edition

P r i ma r y c a r e p h y s i c i a n s a n d o t h e r h e a l t h c a r e p r o v i d e r s , r e s i d e n t s , a n d s t u d e n t s
often face the challenge of diagnosing conditions for patients on the basis of
u n d i f f e r e n t i a t e d p r e s e n t i n g c o mp l a i n t s o r c o n c e r n s . P r e s s u r e s f r o m p a y e r s o f
me d i c a l c a r e t o i n c r e a s e c l i n i c a l e f f i c i e n c y w h i l e ma i n t a i n i n g h i g h - q u a l i t y c a r e h a s
ma d e t h e e f f e c t i v e u s e o f t i me i n t h e c l i n i c v e r y i mp o r t a n t t o p r i ma r y c a r e
practitioners.
Tay l or ' s 10- M i nut e D i agnos i s M, anual

2nd ed., has been specifically designed to
s u p p o r t t h e b u s y p r a c t i t i o n e r i n t h e p r o c e s s o f d i a g n o s i n g p a t i e n t p r o b l e ms i n t h i s
e n v i r o n me n t .
T h e M a n u a l i s o r g a n i ze d a r o u n d c o mmo n p r e s e n t i n g s y mp t o ms , s i g n s , a n d
l a b o r a t o r y a n d i ma g i n g f i n d i n g s , a n d e a c h c h a p t e r s e r v e s a s a s t a n d - a l o n e , c o n c i s e
c l e a r, a n d e a s i l y r e a d i n f o r ma t i o n s o u r c e f o r t h e a r e a c o v e r e d . T h e M a n u a l w o r k s
well at the point of care and fits inside the lab coat pocket.
T h e e d i t o r s a r e p l e a s e d t o i n c l u d e i n t h i s n e w e d i t i o n t h e l a t e s t i n f o r ma t i o n a n d
clinical evidence in addition to changes in clinical practice since the first edition w
p u b l i s h e d . W h i l e a d d i n g t h i s n e w c o n t e n t , t h e e d i t o r s o f t h e 2 n d e d i t i o n h a v e ma d e
e v e r y e f f o r t t o ma i n t a i n t h e e xc e l l e n t r e a d a b i l i t y a n d u t i l i t y t h a t D r. T a y l o r a n d t h e
authors and editors of the 1st edition were able to achieve.
A l l t h e a u t h o r s a n d e d i t o r s o f t h e 2 n d e d i t i o n Tay
h o pleort'hsa10t M i nut e D i agnos i s
M anual i s u s e f u l t o y o u w h i l e y o u c a r e f o r p a t i e n t s .
For the authors and editors,
P a u l M . P a u l ma n M D
O ma h a , N e b r a s k a , L e a d E d i t o r
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Preface to the First Edition
> Front of Book > Preface to the First Edition
Preface to the First Edition
T he 10- M i nut e D i agnos i s M anual

is intended to be a quick-reference source for the
p r i ma r y c a r e c l i n i c i a n f a c e d w i t h a d i a g n o s t i c p r o b l e m s u c h a s h e a d a c h e , f a t i g u e ,
a n e mi a , o r j a u n d i c e . T h e b o o k i s s t r u c t u r e d i n t h e s a me w a y i n w h i c h w e a r r i v e a t a
d i a g n o s i s , s t a r t i n g n o t w i t h a d i s e a s e l a b e l , b u t w i t h a c h i e f c o mp l a i n t o r, p e r h a p s ,
a n u n e xp e c t e d c l i n i c a l f i n d i n g . C h a p t e r t o p i c s w e r e s e l e c t e d b e c a u s e t h e y o c c u r
c o mmo n l y i n t h e p r i ma r y c a r e s e t t i n g o r b e c a u s e t h e y a r e l i k e l y t o b e f i r s t
e n c o u n t e r e d b y t h e p r i ma r y c a r e c l i n i c i a n . T h e s e t o p i c s i n c l u d e s y mp t o ms ( e . g . ,
d i zzi n e s s ) , p h y s i c a l a b n o r ma l i t i e s ( e . g . , s p l e n o me g a l y ) , a n d l a b o r a t o r y
d e t e r mi n a t i o n s ( e . g . , p r o t e i n u r i a ) . T h e c h a p t e r s i n t h e b o o k a r e a b o u t d i a g n o s i s , a n
t h e r a p y i s me n t i o n e d o n l y i f i t mi g h t b e r e l e v a n t t o d i a g n o s i s , s u c h a s t h e r e s p o n s e
o f a n i n f l a me d j o i n t t o c o l c h i c i n e .
T h e 1 0 - mi n u t e p r e mi s e o f t h e b o o k i s b a s e d o n s t u d i e s s h o w i n g t h a t 1 0 - mi n u t e
o f f i c e v i s i t s a r e t h e n o r m i n p r i ma r y c a1r eEtvoedna y.
with longer duration visits, the
t i me d e v o t e d t o d i a g n o s i s a n d n o t t o t h e r a p y, p r o c e d u r e s , p a t i e n t e d u c a t i o n , a n d s
f o r t h i s l i k e l y t o b e a b o u t t e n mi n u t e s . A l s o , t o t h e d e g r e e p o s s i b l e i n a q u i c k r e f e r e n c e g u i d e , a u t h o r s h a v e p r e s e n t e d i n f o r ma t i o n u s i n g a n e v i d e n c e - b a s e d
a p p r o a c h2. F o r mo r e o n t h e b o o k ' s p r e mi s e a n d a p p r o aCchha,pst e re O n. e
Chapters are of a uniform length that is convenient for rapid reading during a patie
c a r e s e s s i o n , a n d e a c h c h a p t e r i s o r g a n i ze d a c c o r d i n g t o s i x ma j o r h e a d i n g s :
A p p r o a c h , H i s t o r y, P h y s i c a l E xa mi n a t i o n , T e s t i n g , D i a g n o s t i c A s s e s s me n t , a n d
References.
I am grateful to the 18 section editors and to the 139 contributing authors. I also
t h a n k t h e f o l l o w i n g i n d i v i d u a l s w h o a s s i s t e d w i t h t h e d e v e l o p me n t a n d p r o d u c t i o n o
t h i s b o o k : C o e l l e d a O ' N e i l a n d Vi c t o r i a B r o w n o f t h e D e p a r t me n t o f F a mi l y M e d i c i n e
o f t h e O r e g o n H e a l t h S c i e n c e s U n i v e r s i t y, E xe c u t i v e E d i t o r R i c h a r d W i n t e r s a n d
D e v e l o p me n t a l E d i t o r M i c h e l l e L a P l a n t e o f L i p p i n c o t t W i l l i a ms & W i l k i n s , a n d
P r o d u c t i o n E d i t o r E mi l y L e r ma n .
I h o p e t h a t y o u w i l l f i n d t h i s b o o k a u s e f u l g u i d e t o c o mmo n l y e n c o u n t e r e d s y mp t o m
and signs, and that you will reach for this book first when you need help with
d i a g n o s i s d u r i n g a b u s y p r a c t i c e d a y.
R obert B . Taylor M D
Portland, Oregon
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Acknowledgments
> F r o n t o f B o o k > A c k n o w l e d g me n t s
Acknowledgments
T h e e d i t o r s oTay
f l or ' s 10- M i nut e D i agnos i s M, anual
2nd edition, would like to
acknowledge the work and contributions of the section editors and chapter authors
w h o p r o v i d e d e xc e l l e n t ma n u s c r i p t s . We a l s o t h a n k t h e e d i t i n g a n d p r o d u c t i o n s t a f f
a t L i p p i n c o t t W i l l i a ms & W i l k i n s : i t w a s t r u l y a p l e a s u r e t o w o r k w i t h y o u o n t h i s
b o o k . T h e e d i t o r s a r e g r a t e f u l t o D r. R o b e r t T a y l o r f o r e d i t i n g t h e f i r s t e d i t i o n : i t
p r o v i d e d a n e xc e l l e n t t e mp l a t e f r o m w h i c h t o w o r k .
S p e c i a l t h a n k s t o S a r a h B r y a n w i t h o u t w h o s e h e l p , o r g a n i za t i o n a l t a l e n t , a n d h a r d
work this book would never have been published.
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TABLE OF CONTENTS
[+] 1 - Principles of the 10-Minute Diagnosis
Contents
[-] 2 - Undifferentiated Problems

[+] 2.1 - Anorexia
[+] 2.2 - Dizziness
[+] 2.3 - Edema
[+] 2.4 - Falls
[+] 2.5 - Fatigue
[+] 2.6 - Fever
[+] 2.7 - Headaches
[+] 2.8 - Hypersomnia
[+] 2.9 - Insomnia
[+] 2.10 - Nausea and Vomiting
[+] 2.11 - Night Sweats
[+] 2.12 - Syncope
[+] 2.13 - Weight Loss
[-] 3 - Mental Health Problems
[+] 3.1 - Anxiety
[+] 3.2 - Depression
[+] 3.3 - Suicide Risk
[-] 4 - Problems Related to the Nervous System
[+] 4.1 - Ataxia
[+] 4.2 - Coma
[+] 4.3 - Delirium
[+] 4.4 - Dementia
[+] 4.5 - Memory Impairment
[+] 4.6 - Paresthesia and Dysesthesia
[+] 4.7 - Seizures
[+] 4.8 - Stroke
[+] 4.9 - Tremors
[-] 5 - Eye Problems
[+] 5.1 - Blurred Vision
[+] 5.2 - Corneal Foreign Body
[+] 5.3 - Diplopia
[+] 5.4 - Nystagmus
[+] 5.5 - Papilledema
[+] 5.6 - Pupillary Inequality
[+] 5.7 - Red Eye
[+] 5.8 - Scotoma
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Contents
[-] 6 - Ear, Nose, and Throat Problems

[+] 6.1 - Halitosis
[+] 6.2 - Hearing Loss
[+] 6.3 - Hoarseness
[+] 6.4 - Nosebleed
[+] 6.5 - Pharyngitis
[+] 6.6 - Rhinitis
[+] 6.7 - Stomatitis
[+] 6.8 - Tinnitus
[+] 6.9 - Vertigo
[-] 7 - Cardiovascular Problems
[+] 7.1 - Atypical Chest Pain
[+] 7.2 - Chest Pain
[+] 7.3 - Bradycardia
[+] 7.4 - Cardiomegaly
[+] 7.5 - Congestive Heart Failure
[+] 7.6 - Heart Murmurs, Diastolic
[+] 7.7 - Heart Murmurs, Systolic
[+] 7.8 - Hypertension
[+] 7.9 - Palpitations
[+] 7.10 - Pericardial Friction Rub
[+] 7.11 - Raynaud's Disease
[+] 7.12 - Tachycardia
[-] 8 - Respiratory Problems
[+] 8.1 - Cough
[+] 8.2 - Cyanosis
[+] 8.3 - Hemoptysis
[+] 8.4 - Pleural Effusion
[+] 8.5 - Pleuritic Pain
[+] 8.6 - Pneumothorax
[+] 8.7 - Shortness of Breath
[+] 8.8 - Stridor
[+] 8.9 - Wheezing
[-] 9 - Gastrointestinal Problems
[+] 9.1 - Abdominal Pain
[+] 9.2 - Ascites
[+] 9.3 - Constipation
[+] 9.4 - Diarrhea
[+] 9.5 - Dysphagia
[+] 9.6 - Epigastric Distress
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Contents
[+] 9.7 - Upper Gastrointestinal Bleeding

[+] 9.8 - Hepatitis
[+] 9.9 - Hepatomegaly
[+] 9.10 - Jaundice
[+] 9.11 - Rectal Bleeding
[+] 9.12 - Steatorrhea
[-] 10 - Renal and Urologic Problems
[+] 10.1 - Dysuria
[+] 10.2 - Hematuria
[+] 10.3 - Impotence
[+] 10.4 - Urinary Incontinence in Adults
[+] 10.5 - Nocturia
[+] 10.6 - Oliguria and Anuria
[+] 10.7 - Priapism
[+] 10.8 - Scrotal Mass
[+] 10.9 - Scrotal Pain
[+] 10.10 - Urethral Discharge
[-] 11 - Problems Related to the Female Reproductive System
[+] 11.1 - Amenorrhea
[+] 11.2 - Breast Mass
[+] 11.3 - Chronic Pelvic Pain
[+] 11.4 - Dysmenorrhea
[+] 11.5 - Menorrhagia
[+] 11.6 - Nipple Discharge in the Nonpregnant Female
[+] 11.7 - Pap Smear Abnormality
[+] 11.8 - Postmenopausal Bleeding
[+] 11.9 - Vaginal Discharge
[-] 12 - Musculoskeletal Problems
[+] 12.1 - Arthralgia
[+] 12.2 - Calf Pain
[+] 12.3 - Hip Pain
[+] 12.4 - Knee Pain
[+] 12.5 - Low Back Pain
[+] 12.6 - Monoarticular Joint Pain
[+] 12.7 - Neck Pain
[+] 12.8 - Polymyalgia
[+] 12.9 - Shoulder Pain
[-] 13 - Dermatologic Problems
[+] 13.1 - Alopecia
[+] 13.2 - Erythema Multiforme
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Contents
[+] 13.3 - Maculopapular Rash

[+] 13.4 - Pigmentation Disorders
[+] 13.5 - Pruritus
[+] 13.6 - Rash Accompanied by Fever
[+] 13.7 - Urticaria
[+] 13.8 - Vesicular and Bullous Eruptions
[-] 14 - Endocrine and Metabolic Problems
[+] 14.1 - Diabetes Mellitus
[+] 14.2 - Gynecomastia
[+] 14.3 - Hirsutism
[+] 14.4 - Hypothyroidism
[+] 14.5 - Polydipsia
[+] 14.6 - Thyroid Enlargement/Goiter
[+] 14.7 - Thyroid Nodule
[+] 14.8 - Hyperthyroidism/Thyrotoxicosis
[-] 15 - Vascular and Lymphatic System Problems
[+] 15.1 - Lymphadenopathy, Generalized
[+] 15.2 - Lymphadenopathy, Localized
[+] 15.3 - Petechiae and Purpura
[+] 15.4 - Splenomegaly
[-] 16 - Laboratory Abnormalities: Hematology and Urine Determinations
[+] 16.1 - Anemia
[+] 16.2 - Eosinophilia
[+] 16.3 - Neutropenia
[+] 16.4 - Polycythemia
[+] 16.5 - Thrombocytopenia
[+] 16.6 - Erythrocyte Sedimentation Rateand C-Reactive Protein
[+] 16.7 - Proteinuria
[-] 17 - Laboratory Abnormalities: Blood Chemistry and Immunology
[+] 17.1 - Alkaline Phosphatase, Elevated
[+] 17.2 - Aminotransferase Levels, Elevated
[+] 17.3 - Antinuclear Antibody Titer, Elevated
[+] 17.4 - Brain Natriuretic Peptide
[+] 17.5 - D-Dimer
[+] 17.6 - Hypercalcemia
[+] 17.7 - Hyperkalemia
[+] 17.8 - Hypokalemia
[-] 18 - Diagnostic Imaging Abnormalities
[+] 18.1 - Bone Cyst
[+] 18.2 - Mediastinal Mass
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Contents
[+] 18.3 - Osteopenia

[+] 18.4 - Solitary Pulmonary Nodule
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1 - Principles of the 10-Minute Diagnosis
> Table of C ontents > 1 - P rinciples of the 10- M inute D iagnosis
1
Principles of the 10-M inute Diagnosis
R o b e r t B . Ta y l o r
T e n mi n u t e s f o r d i a g n o s i s ? R e a l l y ?
Ye s , r e a l l y !
I f o n l y w e h a d 9 0 mi n u t e s t o p e r f o r m a d i a g n o s t i c e v a l u a t i o n , a s w e d i d a s t h i r d - y e a
me d i c a l s t u d e n t s o n h o s p i t a l r o t a t i o n s . O r, i f w e h a d e v e n 3 0 mi n u t e s f o r d i a g n o s i s ,
as I recall from internship. B ut those days are gone. Todayas clinicians practicing
i n t h e a g e o f e v i d e n c e - b a s e d , c o s t - e f f e c t i v e h e a l t h c a r e o f f i c e v i s i t s a r e o f mu c h
s h o r t e r d u r a t i o n t h a n i n y e a r s p a s t . F o r e xa mp l e , i n a r e c e n t s t u d y o f 4 , 4 5 4 p a t i e n t
s e e i n g 1 3 8 p h y s i c i a n s i n 8 4 p r a c t i c e s , t h e me a n v i s i t d u r a t i o n w a1)
s .1 0 mi n u t e s (
A n o t h e r s t u d y o f 1 9 , 1 9 2 v i s i t s t o 6 8 6 p r i ma r y c a r e p h y s i c i a n s e s t i ma t e d t h e v i s i t
d u r a t i o n t o b e 1 6 . 3 mi n u2)t e. sE(v e n w h e n t h e t o t a l v i s i t d u r a t i o n e xc e e d s 1 0
mi n u t e s , t h e t i me a c t u a l l y d e v o t e d t o d i a g n o s i s a n d n o t t o g r e e t i n g t h e p a t i e n t ,
e xp l a i n i n g t r e a t me n t , d o i n g ma n a g e d c a r e p a p e r w o r k , o r e v e n t h e p a t i e n t ' s d r e s s i n g
a n d u n d r e s s i n g i s s e l d o m mo r e t h a n 1 0 mi n u t e s .
S o , i f y o u a n d I g e n e r a l l y h a v e o n l y 1 0 mi n u t e s p e r o f f i c e v i s i t f o r d i a g n o s i s , w e
n e e d t o b e f o c u s e d , w h i l e r e ma i n i n g me d i c a l l y t h o r o u g h a n d p r u d e n t . A c t u a l l y, s u c h
a n a p p r o a c h i s p o s s i b l e a n d i s h o w e xp e r i e n c e d c l i n i c i a n s t e n d t o p r a c t i c e . T h e
f o l l o w i n g a r e s o me p r a c t i c e g u i d e l i n e s t o t h e 1 0 - mi n u t eDdxi10
a )g.n A
o ns ids, (t o
i l l u s t r a t e , l e t u s c o n s i d e r a p aJ oan
t i e n tS
: . , a 49- y ear - ol d mar r i ed woman, i n y our
of f i c e f or a f i r s t v i s i t , whos e c hi ef c ompl ai nt i s s ev er e, one- s i ded headac hes t hat
hav e bec ome wor s e ov er t he pas. t (yFear
o r a mo r e c o mp l e t e a p p r o a c h t o t h e
d i a g n o s i s o f h e a d a c h e , Csheaep t e r 2 . )7
Search for Diagnostic Cues T hroughout the

Clinical Encounter
Note how the patient relates to the staff, takes off a jacket, and sits in the
e xa mi n a t i o n r o o m. H o w d o e s t h e p a t i e n t b e g i n t o d e s c r i b e t h e p r o b l e m a n d w h a t
d o e s h e o r s h e s e e m t o w a n t f r o m t h e v i s i t ? W h o a c c o mp a n i e s t h e p a t i e n t t o t h e
o f f i c e a n d w h o s e e ms t o d o t h e t a l k i n g ?
B e s u r e t o u s e t e l l me a b o u t o p e n - e n d e d q u e s t i o n s . T h e i n e xp e r i e n c e d c l i n i c i a n
mo v e s e a r l y t o c l o s e d - e n d e d Ye s o r N o q u e s t i o n s , b u t Dt hx e10vcel it ne ircaina n
h a s l e a r n e d t h a t u s i n g n a r r o w q u e s t i o n s t o o e a r l y c a n l e a d t o mi s l e a d i n g
c o n c l u s i o n s , w h i c h a r e i n t h e l o n g r u n , a t t h e l e a s t , w a s t e f u l o f t i me a n d , a t w o r s t ,
d a n g e r o u s . A n e xa mp l e w o u l d b e a t t r i b u t i n g c h e s t p a i n i n a p p r o p r i a t e l y t o
33 / 652
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gastroesophageal reflux disease because the patient has a past history of

e s o p h a g e a l r e f l u x a n d r e s p o n d s a f f i r ma t i v e l y t o q u e s t i o n s a b o u t c u r r e n t h e a r t b u r n
and intolerance to spicy foods.
Wa t c h t h e f a c i a l r e a c t i o n t o i s s u e s d i s c u s s e d . T u n e i n t o h e s i t a t i o n a n d e v a s i v e
a n s w e r s a n d b e w i l l i n g t o f o l l o w t h e s e d i a g n o s t i c p a t h s , w h i c h ma y l e a d t o o t h e r w i s
h i d d e n p r o b l e ms s u c h a s d r u g a b u s e o r d o me s t i c I vni ot he
l e n c as
e . e of J oan S . ,
does s he ans wer ques t i ons r eadi l y or does s he s eem ev as i v e when addr es s i ng
s ome t opi c s , s uc h as f ami l y c onc er ns or her home l i f e?
T hink Most Com m on First
I r e mi n d me d i c a l s t u d e n t s o f t h e t i me - h o n o r e d a p h o r i s m t h a t t h e mo s t c o mmo n
p r o b l e ms o c c u r mo s t c o mmo n l y. W h e n w o r k i n g w i t h a p a t i e n t , t h e p h y s i c i a n
d e v e l o p s d i a g n o s t i c h y p o t h e s e s e a r l y i n t h e e n c o u n t e r. W h e n f a c e d w i t h a p a t i e n t
with headache, we should
P. 4
i n i t i a l l y c o n s i d e r t e n s i o n h e a d a c h e a n d mi g r a i n e r a t h e r t h a n t e mp o r a l a r t e r i t i s . O f
c o u r s e , t hD
e x 10 c l i n i c i a n t h i n k s o f s p e c i a l c o n c e r n s , s u c h a s t h e p o s s i b i l i t y t h a t t h e
p a t i e n t w i t h h e a d a c h e mi g h t p o s s i b l y h a v e a b r a i n t u mo r. T h e i n i t i a l h i s t o r y s e e k s
t h e c h a r a c t e r i s t i c s a n d c h r o n o l o g y o f t h e s y mp t o ms . T h e n t h e c l i n i c i a n u s e s s e l e c t
q u e s t i o n s t h a t h e l p r u l e i n o r o u t t h e d i a g n o s t i c h y p o t h e s e s : W h a t s e e ms t o p r e c e d
the headache pain? Has the nature or the severity of your pain changed in any
w a y ? T h e c l i n i c i a n a l s o s e e k s i mp o r t a n t p a s t me d i c a l , s o c i a l , a n d f a mi l y h i s t o r y :
W h a t s t r e s s a r e y o u e xp e r i e n c i n g t h a t ma y b e i n f l u e n c i n g y o u r s y mp t o ms ? D o e s
a n y o n e e l s e i n y o u r f a mi l y h a v e a h e a d a c h e p r o b l e m?
T h e p h y s i c a l e xa mi n a t i o n s h o u l d b e l i mi t e d t o t h e b o d y a r e a s l i k e l y t o c o n t r i b u t e t o
t h e d i a g n o s i s , a n d a f u l l p h y s i c a l e xa mi n a t i o n i s a c t u a l l y s e l d o m n e e d e d .
T her ef or e, f or our pat i ent wi t h r ec ur r ent headac hes , J oan
D x1 S
0ex
. , ami
t he nat i on
i s l i k el y t o be l i mi t ed t o t he v i t al s i gns , head, and nec k , wi t h a s c r eeni ng of
c oor di nat i on, deep t endon r ef l ex es , and c r ani al ner v e f unc t i on. E x ami nat i on of t he
c hes t , hear t , and abdomen i s unl i k el y t o c ont r i but e t o t he
. di agnos i s
T e s t s s h o u l d b e l i mi t e d t o t h o s e t h a t w i l l h e l p c o n f i r m o r r u l e o u t a d i a g n o s t i c
h y p o t h e s i s o r, l a t e r, t h o s e t h a t w o u l d h e l p ma k e a t h e r a p e u t i c d e c i s i o n . F o r mo s t
p a t i e n t s w i t h h e a d a c h e a s a p r e s e n t i n g c o mp l a i n t , n o l a b o r a t o r y t e s t o r d i a g n o s t i c
i ma g i n g i s n e e d e d .
O f c o u r s e , t h e u n c o mmo n p r o b l e m os comet
c u r si mes
. O c c a s i o n a l l y, y o u w i l l
e n c o u n t e r t h e u n e xp e c t e d f i n d i n g : t h e p a t i e n t w i t h h e a d a c h e h a v i n g u n a n t i c i p a t e d
unilateral deafness or the fatigued individual with an enlarged spleen. Stop and thi
w h e n y o u n o t e a c l u s t e r o f s i mi l a r u n e xp e c t e d f i n d i n g s ; s u c h a l e r t n e s s h e l p e d
clinicians identify the Muerto Canyon virus as the cause of the 1993 outbreak of the
h a n t a v i r u s p u l mo n a r y s y n d r o me i n t h e s o u t h w e s t e r n U n i t e d S t a t e s a n d a l s o t h e
o c c u r r e n c e o f p r i ma r y p u l mo n a r y h y p e r t e n s i o n i n p a t i e n t s u s i n g d e xf e n f l u r a mi n e f o r
w e i g h t c o n t r o l . A f e w t i me s i n y o u r c a r e e r y o u w i l l h a v e t h e o p p o r t u n i t y t o e xp e r i e n c
a d i a g n o s t i c e p i p h a n y ;Dtxh10
e c l i n i c i a n w i l l s e i ze t h i s o p p o r t u n i t y b y s t a y i n g a l e r t
f o r t h e u n e xp e c t e d d i a g n o s t i c c l u e .
34 / 652
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Use all Available Assistance
I n a d d i t i o n t o y o u r p r o f e s s i o n a l k n o w l e d g e , e xp e r i e n c e , a n d t i me , y o u r d i a g n o s t i c
r e s o u r c e s i n c l u d e y o u r s t a f f , t h e p a t i e n t a n d h i s o r h e r f a mi l y, a n d t h e v a s t a r r a y o f
me d i c a l r e f e r e n c e s o u r c e s a v a i l a b l e .
Yo u r o f f i c e a n d h o s p i t a l s t a f f c a n b e v a l u a b l e a l l i e s i n d e t e r mi n i n g t h e d i a g n o s i s .
I mp o r t a n t c l u e s ma y b e o f f e r e d w h e n t h e p a t i e n t c a l l s f o r a n a p p o i n t me n t o r w h e n
b e i n g e s c o r t e d t o t h e e xa mi n a t i o n r o o m. I f a p a t i e n t r e ma r k s t o t h e r e c e p t i o n i s t o r
n u r s e t h a t h i s c h e s t p a i n i s j u s t l i k e my f a t h e r h a d b e f o r e h i s h e a r t a t t a c k o r i f
another wonders if her heartburn could be related to her 15-year-old daughter's
mi s b e h a v i o r, t h e s t a f f me mb e r s h o u l d a s k t h e p a t i e n t ' s p e r mi s s i o n a n d t h e n s h a r e
t h e i n f o r ma t i o n w i t h t h e p h y s i c i a n .
T h e p a t i e n t a n d t h e f a mi l y g e n e r a l l y h a v e s o me i n s i g h t i n t o t h e c a u s e o f s y mp t o ms
such as fatigue, diarrhea, or loss of appetite. In a study of patient's differential
d i a g n o s i s o f c o u g h , B e r g h f o u n d t h a t w h i l e p h y s i c i a n s c o n s i d e r e d a me a n o f 7 . 6
d i a g n o s t i c p o s s i b i l i t i e s , p a t i e n t s r e p o r t e d a me a n o f 6 . 5 p o s s i b i l i t i e s , w i t h o n l y 2 . 8
p o s s i b i l i t i e s c o mmo n t o b3)o.t hJ oan
(
S . and per haps her f ami l y, may of f er
di agnos t i c s ugges t i ons t hat y ou hav e not s t r ongl y c ons i der ed; al s o, t hes e ot her
hy pot hes es r epr es ent c onc er ns t hat s houl d ev ent ual l y be addr es s ed t o pr ov i de
r eas s ur anc e. F or ex ampl e, mi ght J oan be i n t he of f i c e t oday c hi ef l y bec aus e an ol d
f r i end has r ec ent l y been di agnos ed wi t h br ai n c anc er and s he has bec ome
c onc er ned about t he s i gni f i c anc e of her own headac hes ?
Consider the Psychosocial Aspects of the Problem

T o c o n t i n u e t h e c a s e o f t h e p a t i e n t w i t h h e a d a c h e , a mi g r a i n e d i a g n o s i s i s
i n c o mp l e t e i f i t f a i l s t o i n c l u d e t h e c o n t r i b u t i o n o f ma r i t a l o r j o b s t r e s s t o t h e
s y mp t o ms o f f a mi l y e v e n t c a n c e l l a t i o n s , t r i p s t o t h e e me r g e n c y r o o m, a n d l a r g e
p h a r ma c y b i l l s f o r s u ma t r i p t a n i n j e c t i o n s , a s w e l l a s t h e i mp a c t o n o t h e r s . N o
d i a g n o s i s o f c a n c e r o r d i a b e t e s i s c o mp l e t e w i t h o u t c o n s i d e r i n g t h e i mp a c t o n t h e
p a t i e n t ' s l i f e a n d t h e l i v e s o f f a mi l y me
4)mb
. ers (
P. 5
T h e D x 10 c l i n i c i a n w i l l b e e s p e c i a l l y w a r yI nt
o fert hnat
e i onal C l as s i f i c at i on of
D i s eas es , N i nt h E di,t i(on
IC D-9) diagnostic categories, which facilitate statistical
a n a l y s i s a n d ma n a g e d c a r e p a y me n t s , b u t w h i c h l a c k t h e r i c h n e s s o f n a r r a t i v e a n d
a l s o t h e p e r s o n a l a n d f a mi l y c o n t e xt . F o r e xa mp l e , c o mp a r e d i a b e t e s me l l i t u s ,
u n c o mp l i c a t e d , I C D - 9 c o d e 2 5 0 . 0 0 w i t h t y p e 2 d i a b e t e s me l l i t u s i n a n e l d e r l y
p a t i e n t w i t h p o o r d i e t , ma r g i n a l r e t i r e me n t i n c o me , a n d i s o l a t i o n f r o m t h e f a mi l y.
F a i l u r e t o c o n s i d e r t h e p s y c h o s o c i a l a s p e c t s o f d i s e a s e i n v i t e s a n i n c o mp l e t e l y
u n d e r s t o o d o r e v e n a mi s s e d d i a g n o s i s : h o w ma n y i n s t a n c e s o f c h i l d a b u s e h a v e
b e e n o v e r l o o k e d a s b u s y e me r g e n c y r o o m p h y s i c i a n s c a r e f o r c h i l d h o o d f r a c t u r e s
w i t h o u t a l s o e xp l o r i n g t h e c a u s e o f t h e i n j u r y a n d t h e h o me e n v i r o n me n t ?
W hen el i c i t i ng a medi c al hi s t or y f r om J oan S . , i t wi l l be i mpor t ant t o l ear n t he

c ur r ent s t r es s es at wor k and at home, and how s he t hi nk s her l i f e woul d be
di f f er ent i f t he headac hes wer e. gone
Seek Help When Needed

35 / 652
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T o d a y, h e a l t h c a r e , i n c l u d i n g d i a g n o s i s , mu s t b e e v i d e n c e b a s e d a n d n o t g r o u n d e
i n a n e c d o t e o r e v e n i n y o u r y e a r s o f c l i n i c a l e xp e r i e n c e . T h e e v i d e n c e i s , o f
c o u r s e , t h e v a s t b o d y o f me d i c a l k n o w l e d g e , i n c l u d i n g r e s e a r c h r e p o r t s a n d me t a a n a l y s e s f o u n d i n c l i n i c a l j o u5)
r n,aol sn (t h e Wo r l d W i d e We
6) ,b a(n d i n r e f e r e n c e
b o o k s s u c h aTshe 10- M i nut e D i agnos i s M anual . W hen t hi nk i ng about J oan S . , y ou
mi ght s ear c h t he l i t er at ur e f or r ec ent ar t i c l es on t he appr oac h t o mi gr ai ne
headac hes
.
Help is also available from colleagues. Consider a consultation when you have a
d i a g n o s i s t h a t i s s o me h o w n o t s a t i s f y i n g . A p e r s o n a l p h y s i c i a n i n a l o n g - t e r m
r e l a t i o n s h i p w i t h a p a t i e n t c a n d e v e l o p a b l i n d s p o t , a n d t h e d i a g n o s i s ma y b e
a p p a r e n t o n l y t o s o me o n e t a k i n g a f r e s h l o o k . W h a t ma y b e n e e d e d a t s u c h a t i me i
a r e t h i n k i n g o f t h e p r o b l e m a l mo s t t h e a n t i t h e s i s o f c o n t i n u i t y.
H e l p c a n b e a v a i l a b l e f r o m t h e s a me - s p e c i a l t y c o l l e a g u e d o w n t h e h a l l o r f r o m a
subspecialist.
T hink in Term s of a Continually Evolving

Diagnosis
Yo u d o n o t a l w a y s n e e d t o ma k e t h e d e f i n i t i v e d i a g n o s i s o n t h e f i r s t v i s i t ; i n f a c t ,
s u c h a n a p p r o a c h t e n d s t o f o s t e r p r o l o n g e d v i s i t s , e xc e s s i v e t e s t i n g , o v e r l y
b i o me d i c a l d i a g n o s e s , a n d h i g h - c o s t me d i c i n e w i t h o u t a d d i n g q u a l i t y. W h e n f a c e d
w i t h a n e l u s i v e d i a g n o s i s , t h e b e s t t e s t i s o f t e n t h e p a s s a g e o f t i me a n d a f o l l o w - u p
v i s i t . F o r e xa mp l e , w e a l l k n o w t h a t h e a d a c h e s a r e o f t e n i n f l u e n c e d b y s t r e s s f u l l i f e
e v e n t s . Ye t , a n e w p a t i e n t ma y n o t b e r e a d y t o s h a r e h i s o r h e r p e r s o n a l , o f t e n
e mb a r r a s s i n g , b u r d e n s , a n d i t i s o n l y w h e n a t r u s t f u l r e l a t i o n s h i p h a s b e e n
established that the clinician learns about the abusive spouse, the pregnant
t e e n a g e r, o r t h e i mp e n d i n g f i n a n c i a l d i s a s t e r.
It is often useful to use the descriptive, categorical diagnosis and seek the definitiv
d i a g n o s i s o v e r t i me . E xa mp l e s i n c l u d e t h e t e e n a g e g i r l w i t h c h r o n i c p e l v i c p a i n , t h e
y o u n g a d u l t w i t h c o u g h f o r 3 mo n t h s , t h e mi d d l e - a g e d p e r s o n w i t h l o s s o f a p p e t i t e ,
a n d t h e o l d e r p e r s o n w i t h f a t i g u e o r i n s o mn i a . S o me t i me s , o n a n i n i t i a l v i s i t , t h i s
approach is the only reasonable option.
T h e D x 10 c l i n i c i a n w i l l b e c a r e f u l n o t t o f a l l i n l o v e w i t h t h e i n i t i a l d i a g n o s i s a n d
r e a l i ze t h a t t h e d e p r e s s e d p a t i e n t l o s i n g w e i g h t mi g h t a l s o h a v e c a n c e r a n d t h a t i t i
t o o e a s y t o a t t r i b u t e a l l n e w s y mp t o ms t o a k n o w n d i a g n o s i s o f me n o p a u s e o r
d i a b e t e s me l l i t uI fs .J oan S . ' s headac hes f ai l t o r es pond as ex pec t ed ov er t i me,
y ou may wi s h t o r ec ons i der y our or i gi nal di agnos i s and per haps s eek f ur t her
t es t i ng t hat woul d hav e s eemed ex c es s i v e on t he i ni t i al v i s i t . F or ex ampl e, mi ght
t he 1- y ear dur at i on of i nc r eas ed s ev er i t y mer i t i magi ng i f a f av or abl e r es pons e t o
i ni t i al t her apy does not oc c ur ?
I n y o u r d a i l y p r a c t i c e , u s e t h e t i me s a v e d i n t h e s t e p s d e s c r i b e d h e r e t o c o n s i d e r a
r e c o n s i d e r y o u r d i a g n o s e s a s y o u r e v i e w c h a r t n o t e s , r e a d me d i c a l j o u r n a l s , s e a r c
me d i c a l w e b s i t e s , a n d s e e t h e p a t i e n t i n f o l l o w - u p Dv ixs10
i t sc.l iTn ihcei a n w i l l
r e ma i n o p e n t o r e t h i n k i n g t h e p a t i e n t ' s d i a g n o s t i c p r o b l e m l i s t . I n t h e e n d , p a t i e n c e
and
P. 6
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p e r s e v e r a n c e o f t e n me a s u r e d i n 1 0 - mi n u t e a l i q u o t s o v e r t i me w i l l y i e l d a n
insightful, biopsychosocially inclusive, and clinically useful diagnosis.
References
1 . S t a n g e K C , Z y za n s k i S J , J a e n C R . I l l u mi n a t i n g t h e b l a c k b o x: a d e s c r i p t i o n o f
4 4 5 4 p a t i e n t v i s i t s t o 1 3 8 f a mi l y p h y sJ i cFi am
a n sP
. r ac1t 9 9 8 ; 4 6 : 3 7 7 3 8 9 .
2 . B l u me n t h a l D , C a u s i n o N , C h a n g Y C . T h e d u r a t i o n o f a mb u l a t o r y v i s i t s t o
p h y s i c i a n sJ. F am P r ac1t 9 9 9 ; 4 8 : 2 6 4 2 7 1 .
3. B ergh K D . T he patient's differential diagnosis: unpredictable concerns in visits
f o r c o u g hJ. F am P r ac1t 9 9 8 ; 4 6 : 1 5 3 1 5 8 .
4 . T a y l o r R B . F a mi l y p r a c t i c e a n d t h e a d v a n c e me n t o f me d i c a l u n d e r s t a n d i n g : t h e
f i r s t 5 0 y e a rJs .F am P r ac1t 9 9 9 ; 4 8 : 5 3 5 7 .
5 . R i c h a r d s o n W S , W i l s o n M C , G u y a t t G H , e t a l . U s e r ' s g u i d e t o t h e me d i c a l
literature: how to use an article about disease probability for differential
d i a g n o s i sJ. A M A 1 9 9 9 ; 2 8 1 : 1 2 1 4 1 2 1 9 .
6 . H e r s h W. A w o r l d o f k n o w l e d g e a t y o u r f i n g e r t i p s : t h e p r o mi s e , r e a l i t y, a n d
f u t u r e d i r e c t i o n s o f o n - l i n e i n f o r ma t i o n rAect rad
i e vM
a led
. 1999;74:240243.
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> T a b l e o f C o n t e n t s > 2 - U n d i f f e r e n t i a t e d P r o b l e ms > 2 . 1 - A n o r e xi a
2.1
Anorexia
Michae l D. Schooff
I . Background
A n o r e xi a i s p r o l o n g e d d i mi n i s h e d a p p e t i t e o r a p p e t i t e l o s s . T h i s c o mmo n s y mp t o m i
t o b e d i f f e r e n t i a t e d f r o m t h e e a t i n g d i s o r d e r a n o r e xi a n e r v o s a , w h i c h i s o f t e n s i mp l y
c a l l e danor ex i .a A n o r e xi a ma y o r ma y n o t b e a s s o c i a t e d w i t h w e i g h t l o s s . I t i s a l mo s t
a l w a y s a r e s u l t o f o n e o r mo r e u n d e r l y i n g c a u s e s .
I I . Pathophysiology
A. General mechanisms
A l t h o u g h t h e e xa c t me c h a n i s ms b y w h i c h t h e b o d y r e g u l a t e s a p p e t i t e a n d b o d y
w e i g h t h a v e y e t t o b e f u l l y d e t e r mi n e d , mo d e r n r e s e a r c h i s p r o v i d i n g mo r e a n d mo r e
i n f o r ma t i o n o n t h e s u b j e c t . T h e h y p o t h a l a mu s i s b e l i e v e d t o r e g u l a t e b o t h s a t i e t y
a n d h u n g e r, l e a d i n g t o h o me o s t a s i s o f b o d y w e i g h t i n i d e a l s i t u a t i o n s . T h e
h y p o t h a l a mu s i n t e r p r e t s a n d i n t e g r a t e s a n u mb e r o f n e u r a l a n d h u mo r a l i n p u t s t o
c o o r d i n a t e f e e d i n g a n d e n e r g y e xp e n d i t u r e i n r e s p o n s e t o c o n d i t i o n s o f a l t e r e d
e n e r g y b a l a n c e . L o n g - t e r m s i g n a l s c o mmu n i c a t i n g i n f o r ma t i o n a b o u t t h e b o d y ' s
e n e r g y s t o r e s , e n d o c r i n e s t a t u s , a n d g e n e r a l h e a l t h a r e p r e d o mi n a n t l y h u mo r a l .
S h o r t - t e r m s i g n a l s , i n c l u d i n g g u t h o r mo n e s a n d n e u r a l s i g n a l s f r o m h i g h e r b r a i n
c e n t e r s a n d t h e g u t , r e g u l a t e me a l i n i t i a t i o n a n d t e r mi n a t i o n . H o r mo n e s i n v o l v e d i n
t h i s p r o c e s s i n c l u d e l e p t i n , i n s u l i n , c h o l e c y s t o k i n i n , g h r e l i n , p o l y p e p t i d e Y Y,
p a n c r e a t i c p o l y p e p t i d e , g l u c a g o n l i k e p e p t i d e - 1 , a n d o xy 1)
n t .o mo
A l t de ur al itni o(n s i n
a n y o f t h e s e h u mo r a l o r n e u r o n a l p r o c e s s e s c a n l e a d t o a n o r e xi a .
B. Etiology
T a b l e 2 . 1 . l1i s t s s o me c a u s e s o f a n o r e xi a . T h e s e c a u s e s c a n b e d i v i d e d i n t o f o u r
c a t e g o r i e s : p a t h o l o g i c , p h a r ma c o l o g i c , p s y c h i a t r i c , a n d s o c i a l .
1. P a t h o l o g i c a u s e s ma y b e a c u t e , s u c h a s a p p e n d i c i t i s o r o t h e r s u r g i c a l
e me r g e n c i e s , o r c h r o n i c , s u c h a s h e a r t o r r e n a l f a i l u r e o r ma l i g n a n c i e s .
P a t h o l o g i c c a u s e s r a r e l y p r e s e n t w i t h o u t o t h e r s i g n s o r s y mp t o ms i n a d d i t i o n t o
a n o r e xi a .
2. P h a r m a c o l o g icca u s e s i n c l u d e s u b s t a n c e s t a k e n a n d t h o s e r e c e n t l y
discontinued. Substances of abuse, such as alcohol, tobacco, narcotics,
ma r i j u a n a , a n d s t i mu l a n t s , c a n a f f e c t a p p e t i t e . P r e s c r i p t i o n a n d o v e r - t h e - c o u n t e
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me d i c a t i o n s , a s w e l l a s d i e t a r y s u p p l e me n t s , c a n l e a d t o a n o r e xi a .
3. P s y c h i a t r i icl l n e s s e s a r e s o me t i me s mo r e d i f f i c u l t t o f i n d t h a n t h e o t h e r
c a t e g o r i e s , r e q u i r i n g t i me a n d a h i g h i n d e x o f s u s p i c i o n . A n o r e xi a ma y b e t h e
r e s u l t o f a p r i ma r y e a t i n g d i s o r d e r, s u c h a s a n o r e xi a n e r v o s a , o r o t h e r i l l n e s s e s
s u c h a s d e p r e s s i o n , p e r s o n a l i t y d i s o r d e r s , s c h i zo p h r e n i a , a n d b i p o l a r d i s o r d e r s
(2) .
4. S o c i a lf a c t o r s o f t e n a f f e c t a p p e t i t e . B e r e a v e me n t , s t r e s s , a n d l o n e l i n e s s ma y
c a u s e a n o r e xi a . M o v i n g f r o m o n e ' s h o me , l o s s o f a b i l i t y t o s h o p f o r f o o d o r
p r e p a r e me a l s , a n d l a c k o f f i n a n c e s ma y a l s o r e s u l t i n a p p e t3)
i t e. c h a n g e s (
I I I . Evaluation
A. History
A c a r e f u l h i s t o r y i s k e y t o d e t e r mi n i n g t h e c a u s e o f a n o r e xi a i n mo s t p a t i e n t s .
1. H i s t o r y o f p r e s e n t i l l nTe hs es f i r s t s t e p i s t o u n d e r s t a n d t h e e xa c t n a t u r e o f
t h e a n o r e xi a . I s t h e p r o b l e m a l o s s o f d e s i r e t o e a t o r a l o s s o f a p p e t i t e w i t h
ma i n t a i n e d d e s i r e ? I s i t t r u l y a s s o c i a t e d w i t h a p p e t i t e , o r i s i t r e l a t e d t o e a r l y
s a t i e t y, d i f f i c u l t o r p a i n f u l s w a l l o w i n g , a b d o mi n a l s y mp t o ms t h a t f o l l o w e a t i n g ,
l o s s o f p l e a s u r e o r s a t i s f a c t i o n w i t h e a t i n g , o r l o s s o f a b i l i t y t o p r e p a r e a me a l ?
W h a t d o e s t h e p a t i e n t t h i n k t h e u n d e r l y i n g p r o b l e m i s ? A r e t h e s y mp t o ms
c o n s t a n t o r d o t h e y f l u c t u a t e ? A r e t h e r e a n y c o e xi s t i n g e mo t i o n a l p r o b l e ms ? H a
t h e p a t i e n t l o s t w e i g h t , a n d i f s o , h o w mu c h ?
P. 1 0
P. 11
P. 1 2
TAB L E 2.1.1 Etiology of Anorexia
Pathologic
Pharm acologic
taking or
discontinuing
Psy chiatric
Social
Ne oplasm s
Tobacco
Depression
B e r e a v e me n t
L y mp h o ma
Alcohol
A n xi e t y
Stress
Colon cancer
Caffeine
Personality
disorders
Loneliness
Lung cancer
A mp h e t a mi n e s
S c h i zo p h r e n i a C h a n g e o f
location
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S t o ma c h
cancer
Marijuana
Eating
disorders
Inability to
shop for food
Endocrine
disorde rs
A n t i h i s t a mi n e s
Obsessivec o mp u l s i v e
disorder
Inability to
prepare
me a l s
Diabetes
me l l i t u s
Anticonvulsants
Bipolar
disorder
Insufficient
finances
Addison's
disease
Antidepressants
D e me n t i a
H y p o n a t r e mi a
Ephedrine
Delirium
H y p e r c a l c e mi a C h r o mi u m
Psychosis
H ypothyroidism Xanthenes
Z inc deficiency Antibiotics
I nfe ctious
dise ase s
Digitalis
Vi r a l h e p a t i t i s M o r p h i n e
HIV
Codeine
Tuberculosis
Meperidine
Intestinal
p r o t o zo a
Aspirin
Chronic
dise ase s
N S AIDs
A n e mi a
Clonidine
COPD
C h e mo t h e r a p e u t i c s
Renal failure
Sedatives
Heart failure
P arkinson's disease
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A l zh e i me r ' s d e me n t i a
Multiple sclerosis
Cirrhosis
I nflam m atory disorde rs
Pancreatitis
I n f l a mma t o r y b o w e l d i s e a s e
Peptic/duodenal ulcer
Cholelithiasis
Appendicitis
G E R D/esophagitis
L u p u s e r y t h e ma t o s u s
Othe rs
Pain
Fever
I mp a i r e d s e n s e o f s me l l
I mp a i r e d s e n s e o f t a s t e
Poor dentition
Dysphagia/odynophagia
I r r i t a b l e b o w e l s y n d r o me
Pregnancy
H I V, h u ma n i mmu n o d e f i c i e n c y v i r u s ; N S A I D s , n o n s t e r o i d a l a n t i i n f l a mma t o r y d r u g s ; C O P D , c h r o n i c o b s t r u c t i v e p u l mo n a r y d i s e a s e ;
G E R D, gastroesophageal reflux disease.
2. P a s t m e d i c a l h i s t oI sr yt h e r e a n y h i s t o r y o f p r e v i o u s e a t i n g d i s o r d e r s ,
p s y c h i a t r i c c o n d i t i o n s , o r c h r o n i c me d i c a l c o n d i t i o n s ?
3. M e d i c a t i o n s a n d h a bW
i t hsa t me d i c a t i o n s i s t h e p a t i e n t t a k i n g ? W h a t
me d i c a t i o n s h a s t h e p a t i e n t r e c e n t l y d i s c o n t i n u e d ? D o e s t h e p a t i e n t t a k e a n y
o v e r - t h e - c o u n t e r me d i c a t i o n s , d i e t a r y s u p p l e me n t s , o r h e r b a l p r o d u c t s ? D o e s
the patient use alcohol, tobacco, or illicit drugs?
4. S o c i a l h i s t o rEya t i n g i s a v e r y s o c i a l f u n c t i o n i n mo s t c u l t u r e s . S t r e s s ,
b e r e a v e me n t , t r o u b l e s w i t h r e l a t i o n s h i p s , l o n e l i n e s s , a n d g u i l t c a n a l l l e a d t o
a n o r e xi a . W h o d o e s t h e p a t i e n t l i v e w i t h ? I s f o o d a v a i l a b l e i n t h e h o me ? I s t h e
p a t i e n t c a p a b l e o f s h o p p i n g a n d p r e p a r i n g me a l s ( e . g . , mo b i l i t y, v i s i o n , a n d
cognitive capacity)? Are there financial concerns?
5. R e v i e w o f s y s t e mAsg e n e r a l r e v i e w o f s y s t e ms s h o u l d b e p e r f o r me d , w i t h
f o c u s o n g a s t r o i n t e s t i n a l ( e . g . , d i f f i c u l t o r p a i n f u l s w a l l o w i n g , n a u s e a , a b d o mi n a
pain or bloating, diarrhea or constipation, and rectal bleeding), psychiatric (e.g
d e p r e s s i o n a n d a n xi e t y ) , a n d n e u r o l o g i c ( e . g . , me n t a l s t a t u s a n d r e c e n t h e a d
i n j u r y ) s y s t e ms . A d i e t h i s t o r y, e i t h e r r e t r o s p e c t i v e o r p r o s p e c t i v e , t h r o u g h t h e
u s e o f a f o o d d i a r y, i s o f t e n h e l p f u l .
B. Physical Examination
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1. G e n e r a l a p p e a r a n D
c eo e s t h e p a t i e n t l o o k h e a l t h y o r i l l ? I s t h e r e f e v e r o r
t a c h y c a r d i a , s u g g e s t i v e o f a s y s t e mi c i l l n e s s ? C a r e f u l l y me a s u r e t h e p a t i e n t ' s
w e i g h t a n d c o mp a r e t o p r e v i o u s r e c o r d i n g s .
2. H e a d , e y e s , e a r s , n o s e , a n d t h r o a t ( HLEoEoNk Tc)a r e f u l l y f o r p o o r d e n t i t i o n ,
o r a l l e s i o n s , d i f f i c u l t s w a l l o w i n g , l y mp h a d e n o p a t h y, a n d t h y r o i d a b n o r ma l i t i e s .
3. C a r d i o r e s p i r a t o r y s y s tEexa
m mi n e f o r a r r h y t h mi a s , c h r o n i c o b s t r u c t i v e
p u l mo n a r y d i s e a s e , a n d s i g n s o f h e a r t f a i l u r e , s u c h a s j u g u l a r v e n o u s d i s t e n s i o n
or rales.
4. G a s t r o i n t e s t i n a l s y s tLeim
s t e n f o r a b n o r ma l b o w e l s o u n d s . E xa mi n e f o r
t e n d e r n e s s , r i g i d i t y, a s c i t e s , a n d h e p a t o me g a l y. R e c t a l e xa mi n a t i o n , i n c l u d i n g
g u a i a c t e s t i n g , s h o u l d b e p e r f o r me d .
5. S k i n J a u n d i c e , s k i n t r a c k s , c y a n o s i s , l a n u g o , h y p e r p i g me n t a t i o n , a n d t u r g o r
should be noted.
6. N e u r o l o g i c a n d p s y c h o l o g i c a l s y sEt xa
e mmis n e t h e f u n c t i o n s o f c r a n i a l
n e r v e s , i n c l u d i n g s me l l a n d t a s t e . L o o k f o r f o c a l o r g e n e r a l i ze d w e a k n e s s , g a i t
o r b a l a n c e d i s t u r b a n c e s , o r mo v e me n t d i s o r d e r s . A s s e s s t h e p a t i e n t ' s f u n c t i o n a l
c a p a c i t y a n d me n t a l s t a t u s . A s s e s s f o r a n xi e t y, d e p r e s s i o n , d e me n t i a , d e l i r i u m,
and psychosis.
C. Testing
A s i n a l l a r e a s o f me d i c i n e , d i a g n o s t i c s t u d i e s s h o u l d b e g u i d e d b y t h e h i s t o r y a n d
p h y s i c a l e xa mi n a t i o n . T e s t s t o c o n s i d e r i n a n o r e xi a i n c l u d e a c o mp l e t e b l o o d c o u n t ,
e l e c t r o l y t e s p a n e l , h e p a t i c p a n e l , a n d a l b u mi n . W h e n a s s e s s i n g n u t r i t i o n a l s t a t u s ,
me a s u r i n g p r e a l b u mi n l e v e l ma y b e p r e f e r r e d o v e r a l b u mi n l e v e l i n a c u t e c a s e s o f
a n o r e xi a b e c a u s e p r e a l b u mi n i s t h e e a r l i e s t ma r k e r o f c h a n g e s i n n u t r i t i o n a l s t a t u s
(4,5) . C h e s t x- r a y a n d t u b e r c u l o s i s t e s t i n g c a n b e h e l p f u l i n s o me c a s e s , a s mi g h t
e s o p h a g o g a s t r o d u o d e n o s c o p y, c o l o n o s c o p y, a n d a b d o mi n a l c o mp u t e d t o mo g r a p h y
o r u l t r a s o n o g r a p h y. L e s s c o mmo n l y o r d e r e d t e s t s i n c l u d e h u ma n i mmu n o d e f i c i e n c y
v i r u s , t h y r o i d - s t i mu l a t i n g h o r mo n e a n d t h y r o i d h o r mo n e , v i r a l h e p a t i t i s p a n e l , a n d
u r i n e p r o t e i n , a n d t e s t i n g f o r t o xi c o l o g y a n d d r u g s o f a b u s e .
I V. Diagnosis
A l t h o u g h t h e c a u s e s o f a n o r e xi a a r e n u me r o u s a n d s p a n t h e b i o p s y c h o s o c i a l
s p e c t r u m, a t h o u g h t f u l e v a l u a t i o n w i l l g e n e r a l l y r e v e a l t h e u n d e r l y i n g c a u s e ( s ) o f t h
loss of appetite, and specific interventions can then be instituted.
References
1 . M u r p h y K G , B l o o m S R . G u t h o r mo n e s i n t h e c o n t r o l oEf xapp pPehy
t i tsei .ol
2004;89:507516.
2 . G a r f i n k e l P E , G a r n e r D M , K a p l a n A S , e t a l . D i f f e r e n t i a l d i a g n o s i s o f e mo t i o n a l
d i s o r d e r s t h a t c a u s e w e i g h t Cl oan
s sM
. ed A s s oc 1J9 8 3 ; 1 2 9 ( 9 ) : 9 3 9 9 4 5 .
P. 1 3
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3 . M o r l e y J E . A n o r e xi a i n o l d e r p e r s o n s : e p i d e mi o l o g y a n d o p t i ma l t r e a t me n t .
D r ugs A gi ng1 9 9 6 ; 8 ( 2 ) : 1 3 4 1 3 5 .
4 . B e c k F K , R o s e n t h a l T C . P r e a l b u mi n : a ma r k e r f o r n u t r i t i o n a A
l m
evaluation.
F am P hy s i c i an
2002;65:15751578.
5 . L a b M a r k e r s o f M a l n u t r i t i o n . A c c e sFs ami
e d al yt P r ac t i c e N ot ebook . c om
( h t t p : / / w w w. f p n o t e b o o k . c o m/ P H A 4 8 . h t m) o n 1 9 M a y 2 0 0 5 .
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2.2 - Dizziness
> T a b l e o f C o n t e n t s > 2 - U n d i f f e r e n t i a t e d P r o b l e ms > 2 . 2 - D i zzi n e s s
2.2
Dizziness
Richard H. Hurd
I . Background
D i zzi n e s s i s a r a t h e r i mp r e c i s e t e r m o f t e n u s e d b y p a t i e n t s t o d e s c r i b e a n y o f a
n u mb e r o f p e c u l i a r s u b j e c t i v e s y mp t o ms . T h e s e s y mp t o ms ma y i n c l u d e f a i n t n e s s ,
giddiness, light-headedness, or unsteadiness. True vertigo, a sensation of irregular
o r w h i r l i n g mo t i o n , i s a l s o i n c l u d e d i n a p a t i e n t ' s c o mp l a i n t o f d i zzi n e s s . D i zzi n e s s
represents a disturbance in a patient's subjective sensation of relationship to space
(1) .
A. Etiology
T h e c a u s e s o f d i zzi n e s s a r e n u me r o u s . I t i s h e l p f u l f o r t h e d i a g n o s t i c i a n t o t h i n k i n
g e n e r a l c a t e g o r i e s o f c a u s e s w h e n s e a r c h i n g f o r a n e t Ti oal bolgey 2(.s2e). e1.
B. Epidemiology
D i zzi n e s s i s t h e c o mp l a i n t i n a n e s t i ma t e d 7 mi l l i o n c l i n i c v i s i t s i n t h e U n i t e d S t a t e s
e a c h y e a r2,3)
( . I t i s o n e o f t h e mo s t f r e q u e n t r e a s o n s f o r r e f e r r a l t o n e u r o l o g i s t s
and otolaryngologists. T he reasons for frequent referral of this usually benign
c o n d i t i o n a r e ma n y. R u l i n g o u t p o t e n t i a l l y s e r i o u s c a u s e s , i n c l u d i n g t h o s e o f c a r d i a
and neurologic origin, can be difficult. In addition, the fact that there is no
P. 1 4
s p e c i f i c t r e a t me n t f o r ma n y o f t h e c a u s e s o f d i zzi n e s s l e a d s t o f r u s t r a t i o n f o r b o t h
the patient and the physician.
TAB L E 2.2.1 Com m on Causes of Dizziness

Pe riphe ral
v e s t i b u l aar
Benign
positional
vertigo
Ce ntral
v e s t i b u l abr
P s y c h i a t r ci c
N o n v e s t i b u l a r,
n o n p s y c h i a t rdi c
Cerebrovascular HyperventilationPresyncope
d i s e a s ee
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2.2 - Dizziness
Labrynthitis
T u mo r fs
Disequilibrium
Meniere's
disease
Cerebellar
atrophy
Medications
O t h e gr
Migraine
Metabolic
disturbances
Multiple sclerosis
Infection
Epilepsy
T r a u ma
Unknown
causeh
s
aE n c o mp a s s e s 4 4 % o f p a t i e n t s .
bA c c o u n t s f o r 11 % o f d i zzi n e s s c a s e s .
cC a u s e s ma k e u p 1 6 % o f t h e d i a g n o s i s .
dA c c o u n t s f o r 3 7 % o f t h e d i a g n o s i s .
eS t r o k e o r t r a n s i e n t i s c h e mi c a t t a c k a n d d e h y d r a t i o n c o mp r i s e t h e l a r g e s t
part of this group.
f U s u a l l y a c o u s t i c n e u r o ma .
g I n c l u d e s d r u g - i n d u c e d , o t o t o xi c i t y, a n d n o n s p e c i f i c v e s t i b u l o p a t h y.
hA s i g n i f i c a n t p a r t o f t h i s s u b s e t a n d a s i g n i f i c a n t p a r t o f a l l c a s e s o f
d i zzi n e s s .
I I I . Evaluation
A. History
I t i s e xt r e me l y i mp o r t a n t , a n d c a n b e v e r y d i f f i c u l t , t o g e t t h e p a t i e n t t o d e s c r i b e
e xa c t l y w h a t t h e y me a n w h e n c o mp l a i n i n g o f d i zzi n e s s . A d e s c r i p t i o n o f t h e a t t a c k ,
c o n t e xt , l e n g t h , d u r a t i o n , a n d f r e q u e n c y i s i mp o r t a n t . A n y p r e c i p i t a t i n g f a c t o r s s h o u
b e e xp l o r e d . C o n c u r r e n t s y mp t o ms s u c h a s n a u s e a , h e a d a c h e , c h e s t f l u t t e r i n g , o r
t i n n i t u s c a n h e l p t o d i r e c t t h e c l i n i c i a n t o a c a u s e . A n y n e w o r me d i c a t i o n c h a n g e s
should be inquired about.
B. Physical examination
T h e p h y s i c a l e xa mi n a t i o n , a l t h o u g h t h o r o u g h , i s o f t e n f o c u s e d o n a s p e c i f i c s y s t e m
b a s e d o n t h e h i s t o r y. I t i s s e l d o m d i a g n o s t i c i n i t s e l f , b u t i s mo r e o f t e n c o n f i r ma t o r y
1. Vi t a l s i g n s i n c l u d i n g o r t h o s t a t i c b l o o d p r e s s u r e s b e g i n t h e e xa mi n a t i o n .
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2.2 - Dizziness
2. A n e u r o l o g i c e xa mi n a t i o n mu s t b e c o mp l e t e d .
3. A c a r d i o v a s c u l a r e xa mi n a t i o n i n c l u d i n g t h e h e a r t f o r mu r mu r o r a r r h y t h mi a a n d
c a r o t i d a r t e r i a l a u s c u l t a t i o n s h o u l d b e c o mp l e t e d .
4. A n o t o s c o p i c e xa mi n a t i o n t o a s s e s s i n f e c t i o n a n d n y s t a g mu s e xa mi n a t i o n
i n c l u d i n g g a ze , D i x- H a l l p i k e ' s ma n e u v e r s , a n d h e a d s h a k i n g a r e i mp o r t a n t .
5. A n o b s e r v a t i o n o f g a i t t o a s s e s s c e r e b e l l a r f u n c t i o n i s a l s o a p a r t o f t h e
e xa mi n a t i o n .
C. Testing
I t i s o b v i o u s t h a t t h e r e i s n o l a b o r a t o r y o r i ma g i n g s t u d y d i r e c t l y r e l a t e d t o d i zzi n e s
Instead, these types of studies are dictated by the etiology that the clinician feels i
mo s t l i k e l y. T h e y a r e mo r e t o c o n f i r m a d i a g n o s i s t h a n t o a c t u a l l y ma k e i t .
1. T e s t s mi g h t i n c l u d e c o mp l e t e b l o o d c o u n t , e l e c t r o l y t e s , a p p r o p r i a t e d r u g l e v e l s ,
and thyroid levels.
2. I ma g i n g s t u d i e s s u c h a s ma g n e t i c r e s o n a n c e i ma g i n g mi g h t b e i n d i c a t e d i f t h e
c o n c e r n o f t u mo r i s h i g h .
3. A h e a r i n g t e s t a s w e l l a s ma n e u v e r s c a r r i e d o u t i n a t i l t - c h a i r t o t e s t l a b y r i n t h
f u n c t i o n ma y b e o f v a l u e .
D. Genetics
T h e r e d o e s n o t a p p e a r t o b e a n y g e n e t i c p r e d i s p o s i t i o n t o d i zzi n e s s .
I V. Diagnosis
A. Differential diagnosis
1. T h e d i f f e r e n t i a l d i a g n o s i s o f d i zzi n e s s i n c l u d e s a l l o f t h o s e c o n d i t i o n s me n t i o n e
i n t h e p r e c e d i n g t e xt t h a t c a u s e t r u e d i Tzzi
a bnlees 2s . 2( ). .1 I t a l s o i n c l u d e s
ma n y o t h e r c o n d i t i o n s t h a t c a u s e p a t i e n t s t o f e e l a b n o r ma l i n s o me v a g u e w a y,
c a u s i n g t h e m t o c o mp l a i n o f d i zzi n e s s . P s y c h o l o g i c c o n d i t i o n s s u c h a s a n xi e t y,
d e p r e s s i o n , p a n i c d i s o r d e r, o r s o ma t i za t i o n ma y a l l c a u s e a p a t i e n t t o c o mp l a i n
o f d i zzi n e s s . C a r d i a c a r r h y t h mi a s , i s c h e mi c o r v a l v u l a r h e a r t d i s e a s e ,
v a s o v a g a l , a n e mi a , o r p o s t u r a l h y p o t e n s i o n a r e s o me o f t h e c o n d i t i o n s l e a d i n g
to cerebral hypoperfusion, and therefore, Presyncope.
2. D e g e n e r a t i v e c h a n g e s i n t h e e l d e r l y ma y a f f e c t t h e v e s t i b u l a r a p p a r a t u s , v i s i o n
o r p r o p r i o c e p t i o n , a l l o f w h i c h ma y b e i n t e r p r e t e d a s d i zzi n e s s . F i n a l l y,
p e r i p h e r a l n e u r o p a t h y o r c e r e b e l l a r d i s e a s e ma y a l s o b e c o n f u s e d w i t h
d i zzi n e s s .
B. Clinical manifestations
T h e c l i n i c a l ma n i f e s t a t i o n s o f d i zzi n e s s a r e a s v a r i e d a s t h o s e e n t i t i e s i n c l u d e d i n
b o t h t h e e t i o l o g i c a n d t h e d i f f e r e n t i a l d i a g n o s i s s e c t i o n s . T h e f a c t t h a t d i zzi n e s s i s
46 / 652
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2.2 - Dizziness
mo r e o f t e n a s y mp t o m o f s o me o t h e r c o n d i t i o n t h a n a s e p a r a t e d i a g n o s i s l e a d s t o a
w i d e v a r i e t y o f ma n i f e s t a t i o n s t h a t t h e c l i n i c i a n mu s t d e c i p h e r.
References
1 . M . B a j o r e k . N i g h t s w e a t s . I n : T a y l o r TRhe
. , e10d . mi nut e di agnos i s manual
.
P h i l a d e l p h i a , PA : L i p p i n c o t t W i l l i a ms & W i l k i n s , 2 0 0 0 : 3 1 3 3 .
2 . K r o e n k e K , H o f f ma n R , E i n s t a d t e r D . H o w c o mmo n a r e v a r i o u s c a u s e s o f
D i zzi n e s s ? A c r i t i c a l r e vSi out
e w. h M ed J2 0 0 0 ; 9 3 ( 2 ) : 1 6 0 1 6 8 , T a b l e 2 , P 7 .
3 . S l o a n e P D . D i zzi n e s s i n p r i ma r y c a r e : r e s u l t s f r o m t h e N a t i o n a l A mb u l a t o r y
M e d i c a l C a r e S u r v Je y.F am P r ac1t 9 8 9 ; 2 9 : 3 3 3 8 .
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2.3 - Edema
> T a b l e o f C o n t e n t s > 2 - U n d i f f e r e n t i a t e d P r o b l e ms > 2 . 3 - E d e ma
2.3
Edema
Roge r Massie
I . Background
E d e ma i s d e f i n e d a s a c l i n i c a l l y a p p a r e n t i n c r e a s e i n t h e i n t e r s t i t i a l f l u i d v o l u me t h
ma y e xp a n d b y s e v e r a l l i t e r s b e f o r e t h e a b n o r ma l i t y 1)
i s. e v i d e n t (
A. Etiology
T h e e t i o l o g y i s mu l t i f a c t o r i a l , r e v o l v i n g a r o u n d t h e i n t r i c a t e b a l a n c e o f c a p i l l a r y
b l o o d a n d o n c o t i c p r e s s u r e s , t i s s u e p r e s s u r e s , c a p i l l a r y p e r me a b i l i t y, a n d l y mp h a t i c
f l o w. A c h a n g e i n a n y o f t h e s e f a c t o r s c a n o f f s e t t h e e xt r a v a s c u l a r f l u i d b a l a n c e a n d
r e s u l t i n e d e ma f o r ma t2)
i o.n (
B. Epidemiology
T h e e p i d e mi o l o g y o f e d e ma i n t h e U n i t e d S t a t e s i s u n k n o w n .
I I I . Evaluation
A. History
T h e f o l l o w i n g f a c t o r s a r e p e r t i n e n t t o t h e e s t a b l i s h me n t o f t h e e t i o l o g y o f e d e ma .
1. O n s e t : g r a d u a l o r s u d d e n
2. S i t e o f t h e e d e ma
3. H i s t o r y o f r e c u r r e n c e o r c h r o n i c i t y
4. C o l o r, w a r mt h , i n d u r a t i o n , s e n s i t i v i t y, a n d / o r p a i n
5. A s s o c i a t e d d y s p n e a o r o r t h o p n e a
6. A s s o c i a t e d f e v e r o r c h i l l s
7. M e d i c a t i o n s s u c h a s n o n s t e r o i d a l a n t i i n f l a mma t o r i e s , c a l c i u m c h a n n e l b l o c k e r s ,
-blockers and -blockers, corticosteroids
8. E n d o c r i n e d i s e a s e s : h y p o t h y r o i d i s m, C u s h i n g ' s d i s e a s e
9. P r o l o n g e d d e p e n d e n t p o s i t i o n
1 0. P r e g n a n c y
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11. I n c r e a s e d s o d i u m c h l o r i d e i n t a k e
1 2. T r a u ma : e c c h y mo s i s , a b r a s i o n s
A c o mp l e t e o r f o c u s e d e xa mi n a t i o n s h o u l d b e p e r f o r me d , d e p e n d i n g o n i n f o r ma t i o n
o b t a i n e d f r o m t h e h i s t o r y. S p e c i a l a t t e n t i o n s h o u l d b e p a i d t o w h e t h e r t h e e d e ma i s
g e n e r a l i ze d o r l o c a l i ze d T( as eb el e 2 . 3 ). .1 Vi t a l s i g n s s h o u l d b e n o t e d w i t h s p e c i a l
a t t e n t i o n t o a n e l e v a t e d t e mp e r a t u r e , d e c r e a s e d o xy g e n s a t u r a t i o n , t a c h y p n e a ,
and/or tachycardia. Mental status changes reported by the patient or the patient's
f a mi l y s h o u l d b e n o t e d . N e c k v e i n d i s t e n s i o n s h o u l d b e e v a l u a t e d . I t i s n e c e s s a r y t
l i s t e n c a r e f u l l y f o r a g a l l o p i n t h e h e a r t r h y t h m. C r a c k l e s i n t h e l u n g s s h o u l d a l s o b
n o t e d . A s c i t e s a n d h e p a t o s p l e n o me g a l y s h o u l d
P. 1 6
b e e v a l u a t e d . I t s h o u l d b e n o t e d w h e t h e r t h e e d e ma i s g e n e r a l i ze d o r l o c a l i ze d ,
whether it is pitting or nonpitting, and whether there is coloration if a painful
s e n s a t i o n i s p r e s e n t . T h e f i n d i n g s o n p h y s i c a l e xa mi n a t i o n s h o u l d b e v e r y h e l p f u l i n
d e t e r mi n i n g t h e e t i o l o g y o f t h e e d e ma .
TAB L E 2.3.1 Causes of Localized Edem a

Injury
Local allergic reactions
A r t h r i t i s / j o i n t i n f l a mma t i o n s
I n s e c t s t i n g s / v e n o ma t i o n s
Ve n o u s t h r o mb o s i s o r o c c l u s i o n
S u r g i c a l i n t e r r u p t i o n o f v e i n s o r l y mp h i t i s
A n g i o e d e ma
Idiopathic
P h y s i o l o g i c ( e . g . , d e p e n d e n t p e d a l e d e ma )
Infections
TAB L E 2.3.2 Drugs Associated with Edem a

N o n s t e r o i d a l a n t i - i n f l a mma t o r y d r u g s
Antihypertensive agents
Direct arterial/arteriolar vasodilators
M i n o xi d i l
H y d r a l a zi n e
Clonidine
Methyldopa
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2.3 - Edema
Guanethidine
Calcium channel antagonists
Adrenergic antagonists
S t e r o i d h o r mo n e s
Glucocorticoids
Anabolic steroids
Estrogens
Progestins
Cyclosporine
G r o w t h h o r mo n e
I mmu n o t h e r a p i e s
Interleukin-2
O K T 3 mo n o c l o n a l a n t i b o d y
F r o m B r a u n w a l d E , e d . E d e maH.arI nr i: s on' s pr i nc i pl es of i nt er nal
medi c i ne
, 1 5 t h e d . N e w Yo r k , N Y : M c G r a w H i l l ; 2 0 0 1 : 2 1 7 2 2 2 .
C. Testing
T he following tests should be entertained and/or ordered depending on the history

a n d p h y s i c a l e xa mi n a t i o n : c o mp l e t e b l o o d c o u n t , u r i n a l y s i s , t h y r o i d - s t i mu l a t i n g
h o r mo n e , c o mp r e h e n s i v e me t a b o l i c p r o f i l e i n c l u d i n g a l b u mi n a n d l i v e r f u n c t i o n t e s t s
c o n g e s t i v e h e a r t f a i l u r e p e p t i d e , c h e s t x- r a y, e l e c t r o c a r d i o g r a m, c o mp u t e d
t o mo g r a p h y, ma g n e t i c r e s o n a n c e i ma g i n g , v e n o u s D o p p l e r s t u d y, a n d v e n o g r a ms .
I V. Diagnosis
F o r t h e d i f f e r e n t i a l d i a g n o s i s o f d r u g - i n d u c e d e dTeama
b l e, 2s .e3e. 2
References
1 . B r a u n w a l d E , e d . E d e maH
. ar
I n r: i s on' s pr i nc i pl es of i nt er nal medi
, 1 5ctihne
e d . N e w Yo r k , N Y : M c G r a w - H i l l , 2 0 0 1 : 2 1 7 2 2 2 .
2 . T e r r y, M . , O ' B r i e n , S . , K e r s t e i n , M D . L o w e r - e xt r e mi t y e d e ma : e v a l u a t i o n a n d
d i a g n o s i sW
. ounds1 0 ( 4 ) : 11 1 2 4 : 1 9 9 8 .
50 / 652
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2.4 - Falls
> T a b l e o f C o n t e n t s > 2 - U n d i f f e r e n t i a t e d P r o b l e ms > 2 . 4 - F a l l s
2.4
Falls
Ke nne th D. Pe te rs
I . Background
F a l l s a r e mo s t c o mmo n a t a g e e xt r e me s . I n c h i l d r e n o l d e r t h a n 1 y e a r o f a g e , i n j u r i e
a r e t h e n u mb e r o n e c a u s e o f d e a t h . F a l l s a c c o u n t f o r 2 5 % o f t h e s e d e a t h s . B i k e
i n j u r i e s a c c o u n t f o r 6 8 % o f f a l l s i n c h i l d r e n f r o m 5 t o 1 4 y1)
e a. rIsn opfa at igeen t(s
older than 65 years of age, the incidence of falls is 30%; in those older than 80
years of age, it is > 50%. Accidents are the fifth leading cause of death in patients 6
y e a r s o f a g e a n d o l d e r, a n d f a l l s a c c o u n t f o r t w o t h i r d s o f t h e s e d e a t h s . O f e l d e r l y
p a t i e n t s h o s p i t a l i ze d f o r f a l l s , o n l y 5 0 % a r e a l i v e l 2)y .e a r l a t e r (
Factors that contribute to falls need to be identified and evaluated for preventive
me a s u r e s t o b e t a k e n . C h i l d r e n f a l l f r o m h e i g h t s ; e l d e r l y f a l l f r o m l e v e l s u r f a c e s .
A. Children and adolescents
Falls from heights over 3 feet and falls of infants younger than 1 year of age result
i n c r e a s e d r i s k o f s k u l l f r a c t u r e a n d i n t r a c r a n i a l b l e e d i n g . E me r g e n t e v a l u a t i o n i s
n e e d e d i n c a s e s o f l o s s o f c o n s c i o u s n e s s , b e h a v i o r a l c h a n g e s , s e i zu r e s , o r o n g o i n g
v o mi t i n g .
B. Falls in the elderly
One half of the falls are secondary to accidents, including factors affecting stability
T h e o t h e r h a l f o f t h e f a l l s a r e s e c o n d a r y t o me d i c a l d i sToar db el er s2 .( 4s ).e1.e I f
s y n c o p e o c c u r r e d w i t h a f a l l , i t mu s t b e d e t e r mi n e d w h e t h e r t h e c a u s e i s c a r d i a c o r
n o n c a r d i a c ( s eTea b l e 2 . 4 ). 2(C h a p t e r 2 . 1) .2 C a r d i a c mo r t a l i t y i n f a l l s r e l a t e d t o
s y n c o p e a t 1 y e a r i s 2 0 % t o 3 0 % , w h e r e a s n o n c a r d i a c mo r t 3)
a l .i t yT hi se r<e5i%
s(
a s t r o n g a s s o c i a t i o n b e t w e e n f a l l s a n d n u r s i n g h o me p l a c e me n t i n t h e e l d e r l y ;
f u r t h e r mo r e , s p e c i f i c i n d i v i d u a l i ze d i n t e r v e n t i o n s h e l p p4)
r e.v eT nhte f railsl sk (o f
h i p f r a c t u r e i n t h e f r a i l e l d e r l y c a n b e r e d u c e d w i t h t h e u s e o f a n a n a t o mi c a l l y
d e s i g n e d e xt e r n a l h i p p r o t e5)c t. o r (
I I I . Evaluation
A. History
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2.4 - Falls
1. H i s t o r y o f t h e f A
a lnl i n t e r v i e w o f a w i t n e s s t o t h e f a l l i s e s s e n t i a l . T h i s ma y
i d e n t i f y a n y s e i zu r e a c t i v i t y, l o s s o f c o n s c i o u s n e s s , a n d me t h o d o f f a l l . A s k w h a
the patient was doing prior to the fall, including occurrence with positional
changes or after voiding, eating, or constipation. Are there associated
p a l p i t a t i o n s i mp l y i n g a r r h y t h mi a ? D i d t h e p a t i e n t h a v e a f a l l o r s y n c o p e d u r i n g
e xe r c i s e , w h i c h ma y i n d i c a t e a c a r d i a c c a u s e ? I s t h e r e a n y c o n f u s i o n t h a t i s
n e w o r c h a n g e d f r o m t h e p a s t t h a t s u g g e s t s c e n t r a l n e r v o u s s y s t e m t r a u ma o r
s e i zu r e ? Wa s u r i n e o r b o w e l i n c o n t i n e n c e p r e s e n t ? Q u e s t i o n s c o n c e r n i n g h o me
a n d r i s k f a c t o r s s h o u l d b e r aTi as be lde ( 2 . 4 ). .1
2. P a s t h i s t o r E
y xp l o r e c o e xi s t i n g i l l n e s s t h a t ma y h a v e c o n t r i b u t e d t o t h e f a l l
(T a b l e 2 . 4 ). .1 A f a mi l y h i s t o r y o f s u d d e n d e a t h c a n i mp l y a r r h y t h mi a s .
F u r t h e r mo r e , i n q u i r e a b o u t a n y h i s t o r y o f p r i o r f a l l s .
T his should include:
1. A s s e s s me n t o f v i t a l s i g n s , i n c l u d i n g h e a r t r a t e a n d r h y t h m, o r t h o s t a t i c b l o o d
p r e s s u r e c h a n g e s , t e mp e r a t u r e , a n d r e s p i r a t o r y r a t e .
2. A g e n e r a l b o d y s u r v e y f o r a n y e v i d e n c e o f t r a u ma .
3. E xa mi n a t i o n o f t h e e y e ( f u n d u s c o p i c , v i s u a l a c u i t y, a n d f i e l d s ) , mo u t h ( t o n g u e
lacerations), neck (bruits), lung (congestive heart failure or infection), and
c a r d i o v a s c u l a r ( mu r mu r s a n d r h y t h m) .
4. A n e u r o l o g i c e xa mi n a t i o n t h a t i n c l u d e s me n t a l s t a t u s , e v a l u a t i o n o f b a l a n c e ,
g a i t , mo b i l i t y, a n d t e s t s f o r p e r i p h e r a l n e u r o p a t h y.
5. T h e g e t u p a n d g o t e s t ( r i s e f r o m a c h a i r, w a l k 1 0 f e e t , r e t u r n , a n d s i t d o w n ) ,
w h i c h i s a s i mp l e r a p i d w a y o f a s s e s s i n g g e n e r a l c o n d i t i o n a n d mu s c u l o s k e l e t a l
a n d n e u r o l o g i c s t a t6)
u s. (
P. 1 8
TAB L E 2.4.1 Factors Affecting Falls
Factors affe cting stability
Me dical proble m s
contributing to
falls
D e c r e a s e d mu s c l e t o n e / s t r e n g t h
Arthritis
Changes in gait
Previous stroke
Changes in postural control
Hip fracture
Decreased depth perception
D e me n t i a
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Decreased hearing
Osteoporosis
Decreased proprioception
Parkinsonism
Decreased vision
Foot disorders
S l o w e r r e f l e xe s
Peripheral
neuropathies
H a za r d o u s l i v i n g a r r a n g e me n t s ( e . g . , p o o r l i g h t iHn ygp, e r t h y r o i d i s m
slick floors, loose rugs, stairways, unstable furniture)
Alcoholism
Medications
Hypertension
Hypotension
Myocardial
infarction
A r r h y t h mi a s
Congestive heart
failure
Acute stroke
Internal bleeding
Infections
Va l v u l a r h e a r t
disease
S e i zu r e s
C. Testing
1. C l i n i c a l l a b o r a t o r y t eMs ot ss t b l o o d t e s t s a r e o f l o w y i e l d a n d s h o u l d b e d o n e
to confirm clinical suspicion. An electrocardiogram is useful in the elderly to rul
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o u t a r r h y t h mi a , a t r i o v e n t r i c u l a r b l o c k , p r o l o n g e d Q T s y n d r o me , o r i s c h e mi a . A
diagnosis of the cause of the fall can be obtained in 50% to 60% of cases based
o n h i s t o r y, p h y s i c a l , a n d e l e c t r o c a r d i o g r a p h7)
i c. s t u d y (
2. D i a g n o s t i c i m a g i n
S gk u l l x- r a y ( f r a c t u r e ) a n d c o mp u t e d t o mo g r a p h y s t u d i e s t o
d e t e c t i n t r a c r a n i a l b l e e d i n g a r e r e c o mme n d e d i n a l l i n f a n t s y o u n g e r t h a n 1 y e a r
o f a g e o r i f t h e f a l l w a s f r o m > 3 f e e t . A l s o c o n s i d e r i ma g i n g w i t h a n y l o s s o f
c o n s c i o u s n e s s , e v i d e n c e o f h e a d t r a u ma , b e h a v i o r a l c h a n g e s , s e i zu r e d i s o r d e r,
o n g o i n g v o mi t i n g , o r f o c a l n e u r o l o g i c d e f i c i t s .
3. O t h e r t e s t i n g t o c o n s i d e r i n c l u d e s e c h o c a r d i o g r a m ( v a l v u l a r h e a r t d i s e a s e ) ,
e l e c t r o e n c e p h a l o g r a m ( s e i zu r e ) , c a r o t i d u l t r a s o u n d ( b r u i t s ) , c a r o t i d s i n u s
ma s s a g e ( i f s u g g e s t e d b y h i s t o r y ) , a n d t i l t t a b l e t e s t i n g ( i f a v a s o v a g a l c a u s e o
f a l l i s c o n s i d e r e d ) . A mb u l a t o r y c a r d i a c mo n i t o r f o r s u d d e n i n f r e q u e n t f a l l s .
I V. Diagnosis
A f a l l b y a n e l d e r l y i n d i v i d u a l f r e q u e n t l y r e q u i r e s a h o me v i s i t t o e v a l u a t e f a c t o r s
c o n t r i b u t i n g t o f a l l s a n d t o c o r r e c t u n s a f e c oTnadbi ltei o2n.s4 ).( 1. S y mp t o ms o f
c a r d i a c d i s e a s e c a n o c c u r w i t h e xe r t i o n o r s t r a i n i n g . C a r d i a c a r r h y t h mi a s t e n d t o b e
s u d d e n w i t h o u t w a r n i n g , a l t h o u g h o n c e i n a w h i l e , p a t i e n t s c a n c o mp l a i n o f
palpitations. Noncardiac causes include the vasovagal reaction where the patient
g e n e r a l l y c o mp l a i n s o f d i zzi n e s s o r l i g h t h e a d e d n e s s p r i o r t o a f a l l , o f t e n w i t h
changes in position or when upright. T hese can be associated with sweating and
nausea.
P. 1 9
O r t h o s t a t i c n o n c a r d i a c c a u s e s h a v e g r a d u a l o n s e t a n d r e s o l u t i o n . T h e s e a r e mo s t
o f t e n a s s o c i a t e d w i t h me d i c a t i o n s , i n c l u d i n g a n t i h y p e r t e n s i v e s , s e d a t i v e s ,
a n xi o l y t i c s , a n t i d e p r e s s a n t s , h y p o g l y c e mi c s , p s y c h o t r o p i c2s b, lhoicskt ae mi
r s ,n e
a l c o h o l , o v e r - t h e - c o u n t e r c o l d me d i c i n e s , a n d me d i c a t i o n s w i t h e xt e n d e d h a l f - l i v e s .
Neurologic noncardiac events can usually be diagnosed by history and physical
e xa mi n a t i o n . A p s y c h i a t r i c c a u s e f o r f a l l s i s l e s s l i k e l y, b u t o n e s h o u l d b e s u s p i c i o u
i n c a s e s o f f r e q u e n t s y mp t o ms w i t h n o i n j u r y.
TAB L E 2.4.2 Causes of Syncope

Cardiac
Noncardiac
Obstructiv e
Vasov agal
Va l v u l a r d i s e a s e
Pain
H y p e r t r o p h i c c a r d i o my o p a t hVo
y iding
P u l mo n a r y h y p e r t e n s i o n
Increased stress
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P u l mo n a r y e mb o l i
Cough
M y xo ma s
S i mp l e f a i n t
Arrhy thm ia
Carotid sinus disease
S i c k s i n u s s y n d r o me
Orthostatic
Atrial fibrillation
Medication
Vo l u me d e p l e t i o n
Diabetes
Parkinsonism
Ne urologic
Stroke
S e i zu r e
Migraine
F o r t h e d i f f e r e n t i a l d i a g n o s i s , r Te af ebrl et so 2 . 4 .a1n d2 . 4 . 2.
References
1 . G r u s k i n K D , S c h u t zma n S A . H e a d t r a u ma i n c h i l d r e n y o u n g e r t h a n 2 y e a r s :
a r e t h e r e p r e d i c t o r s f o r c o mp l i c aAt irocnhs P
? edi at r A dol es c M1ed
999;153:15
20.
2 . S t e i n w e g K K . T h e c h a n g i n g a p p r o a c h t o f a l l s i n t hAeme Fl dam
e r l y.
P hy s i c i an1 9 9 7 ; 5 6 : 1 8 1 5 1 8 2 4 .
3 . W i l e y T M . A d i a g n o s t i c a p p r o a c h t o s yRnes
c oipde .S t af f P hy s i c i an
1998;44:2947.
4 . T i n e t t i M E , W i l l i a ms C S . F a l l s , i n j u r i e s d u e t o f a l l s , a n d t h e r i s k o f a d mi s s i o n
t o a n u r s i n g h o me
N ew
. E ngl J M ed
1997;337(18):12791284.
5 . K a n n u s P, P a r k k a r i J , N i e mi S . P r e v e n t i o n o f h i p f r a c t u r e i s e l d e r l y p e o p l e
w i t h u s e o f a h i p p r o t eN
c tew
o r. E ngl J M ed
2000:343(21):15061513.
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6 . A l b e r t S , D a v i d R , A l i s o n M . C o mp r e h e n s i v e g e r i a t r i c a s s e s s me n t . I n : W i l l i a m
H , E d w i n B , J o h n B , e t a l . Perdi nc
s . i pal s of ger i at,r i3crsd e d . N e w Yo r k , N Y :
McGraw-Hill, 1994: Chapter 17:206.
7 . H u p e r t N , K a p o o r W N . S y n c o p e : a s y s t e mi c a p p r o a c h f o r Pt at
h ei ent
cause.
C ar e 1 9 9 7 ; 3 1 : 1 3 6 1 4 7 .
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> T a b l e o f C o n t e n t s > 2 - U n d i f f e r e n t i a t e d P r o b l e ms > 2 . 5 - F a t i g u e
2.5
Fatigue
Am y K. Je spe rse n
I . Background
A. General considerations
F a t i g u e i s a v e r y c o mmo n c o mp l a i n t i n t h e p r i ma r y c a r e o f f i c e . I t ma y b e t h e p r i ma r y
r e a s o n a p a t i e n t s e e k s c a r e o r a s e c o n d a r y c o mp l a i n t . We a r e a l l b o t h e r e d b y f a t i g u
a t s o me p o i n t i n t i me . H o w e v e r, f o r mi l l i o n s o f p a t i e n t s e a c h y e a r, i t b e c o me s
b o t h e r s o me e n o u g h t o s e e k me d i c a l a t t e n t i o n . T r u e f a t i g u e n e e d s t o b e
d i s t i n g u i s h e d f r o m w e a k n e s s a n d f r o m e xc e s s i v e s o mn o l e n c e s e c o n d a r y t o s l e e p
d i s t u r b a n c e s . F a t i g u e l a s t i n g l e s s t h a n a mo n t h i s c o n s i d e r e d a c u t e . I f s y mp t o ms l a
mo r e t h a n a mo n t h , f a t i g u e i s c o n s i d e r e d p r o l o n g e d .
B. Definitions
1. C h r o n i c f a t i g u e i s d i a g n o s e d w h e n s y mp t o ms l a s t > 6 mo n t h s . T h e C e n t e r f o r
D i s e a s e C o n t r o l a n d P r e v e n t i o n h a s d e f i n e d c h r o n i c f a t i g u e s y n d r o me ( C F S ) a s
p r o f o u n d f a t i g u e o f 6 mo n t h s d u r a t i o n t h a t p r e s e n t s w i t h f o u r o f t h e f o l l o w i n g
e i g h t s y mp t o ms :
a. I mp a i r me n t i n s h o r t - t e r m me mo r y o r c o n c e n t r a t i o n
b. S o r e t h r o a t
c. T e n d e r l y mp h a d e n o p a t h y
d. M y a l g i a s
e. M u l t i j o i n t p a i n
f. H e a d a c h e s o f a n e w t y p e , p a t t e r n , o r s e v e r i t y
g. U n r e f r e s h i n g s l e e p
h. P o s t e xe r t i o n a l ma l a i s e l a s t i n g > 2 4 1)h o u r s (
2. I d i o p a t h i c c h r o n i c f a t i g u e i s d i a g n o s e d i f a p a t i e n t h a s b e e n f a t i g u e d f o r o x> 6
mo n t h s , b u t d o e s n o t me e t t h e o t h e r c r i t e r i a f o r C F S .
A. Etiology
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2.5 - Fatigue
S o me o f t h e c o mmo n c a u s e s o f C F S a r e lTi satbelde i2n. 5 . 1F a t i g u e ma y b e d u e t o

me d i c a l d i s o r d e r s , p s y c h i a t r i c d i s e a s e , o r l i f e s t y l e f a c t o r s . I n s o me c a s e s , a c a u s e
n e v e r d e t e r mi n e d . F a t i g u e t h a t p e r s i s t s f o r s e v e r a l mo n t h s o r y e a r s i s mo r e l i k e l y t o
h a v e a p s y c h i a t r i c e t i o l o g y, w h e r e a s a s h o r t e r d u r a t i o n o f f a t i g u e i s mo r e l i k e l y t o
h a v e a me d i c a l e xp l a n a t2)
i o. nI f( a me d i c a l c a u s e o f f a t i g u e i s p r e s e n t , i t i s u s u a l l y
i d e n t i f i a b l e o n t h e i n i t i a l h i s t o r y, p h y s i c a l a n d l a b o r a t3)o .r y t e s t i n g (
B. Epidemiology
T h e t r u e i n c i d e n c e o f p r o f o u n d f a t i g u e i s u n k n o w n . I t h a s b e e n e s t i ma t e d t h a t o v e r
mi l l i o n o f f i c e v i s i t s p e r y e a r a r e f o r c o mp l a i n t s 3)
o f. fTa ht ieg ut reu (e g e n d e r
p r e d i l e c t i o n i s a l s o u n k n o w n , h o w e v e r, w o me n p r e s e n t t o t h e p h y s i c i a n ' s o f f i c e t w i c
a s o f t e n a s me n . P a t i e n t s y o u n g e r t h a n 4 5 y e a r s o f a g e a r e mo r e l i k e l y t o p r e s e n t f
f a t i g u e t h a n p a t i e n t s o l d e r t h a n 4 5 y e a r s2)o. f a g e (
I I I . Evaluation
A. History
1. A d e t a i l e d h i s t o r y a n d r e v i e w o f s y s t e ms s h o u l d b e p e r f o r me d . T h e o n s e t ,
d u r a t i o n , a n d d e g r e e o f f a t i g u e s h o u l d b e e xp l o r e d , a l o n g w i t h a n y p o s s i b l e
precipitating events. Specific attention should be given to sleep patterns,
d a y t i me s o mn o l e n c e , o r s l e e p a p n e a s y mp t o ms .
2. T h e p a t i e n t ' s e xe r c i s e h a b i t s , c a f f e i n e i n t a k e , a n d d r u g o r a l c o h o l u s e s h o u l d b
e xp l o r e d , a n d me d i c a t i o n s s h o u l d b e r e v i e w e d .
3. A p s y c h i a t r i c h i s t o r y t o e v a l u a t e s y mp t o ms o f d e p r e s s i o n o r a n xi e t y s h o u l d b e
o b t a i n e d . L i f e s t y l e i s s u e s s u c h a s s t r e s s a t h o me o r i n t h e w o r k p l a c e , c h i l d c a r e
responsibilities, shift work, or changing work schedules should be addressed.
A t h o r o u g h p h y s i c a l e xa mi n a t i o n s h o u l d b e p e r f o r me d . Vi t a l s i g n s s h o u l d b e c a r e f u l
n o t e d . A t t e n t i o n s h o u l d b e g i v e n t o t h e p r e s e n c e o f p a l l o r, mu s c l e w e a k n e s s , g o i t e r
l y mp h a d e n o p a t h y, a n d b o d y h a b i t u s . A p s y c h i a t r i c
P. 2 1
e v a l u a t i o n f o r s i g n s o f d e p r e s s i o n , a n xi e t y, o r o t h e r me n t a l i l l n e s s s h o u l d b e
p e r f o r me d . I n o l d e r a d u l t s , a me n t a l s t a t u s e xa m t o e v a l u a t e c o g n i t i v e f u n c t i o n ma y
be appropriate.
TAB L E 2.5.1 Com m on Causes of Fatigue

Me dical conditions
Hypothyroidism
A n e mi a
C a r d i o my o p a t h y
P s y c h i a t r i c d i s o r d e r sL i f e s t y l e f a c t o r s
Major depressive
disorder
Bipolar disorder
Sleep deprivation
Marital discord
Job stress
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COPD
Morbid obesity
Sleep disorders
Medication side effects
- B lockers
C entrally acting Blockers
Antidepressants
Subacute infections
Vi r a l i n f e c t i o n s ( C M V,
E B V, H I V )
Bacterial endocarditis
Malignancy
F i b r o my a l g i a
A l c o h o l i s m/ s u b s t a n c e C a r i n g f o r y o u n g
abuse
children
A n xi e t y
Altering work
S o ma t o f o r m d i s o r d e r ss c h e d u l e
C O P D , c h r o n i c o b s t r u c t i v e p u l mo n a r y d i s e a s e ; C M V, c y t o me g a l o v i r u s ; E B V,
E p s t e i n - B a r r v i r u s ; H I V, h u ma n i mmu n o d e f i c i e n c y v i r u s .
C. Testing
1. I n i t i a l l a b o r a t o r y t e s t i n g s h o u l d b e l i mi t e d t o :
a. C o mp l e t e b l o o d c o u n t
b. C o mp r e h e n s i v e me t a b o l i c p r o f i l e
c. T h y r o i d - s t i mu l a t i n g h o r mo n e
d. E r y t h r o c y t e s e d i me n t a t i o n r a t e
e. U r i n e a n a l y s i s
2. O t h e r t e s t s ma y b e i n d i c a t e d b y t h e h i s t o r y o r p h y s i c a l e xa mi n a t i o n :
a. A n t i n u c l e a r a n t i b o d y
b. R h e u ma t o i d f a c t o r
c. M o n o s p o t
d. C h e s t x- r a y
e. C o l o n o s c o p y
f. S l e e p s t u d y
3. S c r e e n i n g t e s t s a p p r o p r i a t e f o r a g e a n d g e n d e r s h o u l d b e p e r f o r me d .
I V. Diagnosis
F a t i g u e i s a v e r y c o mmo n l y e n c o u n t e r e d c o mp l a i n t . I n mo s t c a s e s , a t h o r o u g h
h i s t o r y, p h y s i c a l a n d a l i mi t e d n u mb e r o f a n c i l l a r y t e s t s r e v e a l a mo r e p r e c i s e
d i a g n o s i s . F a t i g u e i s r a r e l y t h e o n l y p r e s e n t i n g s y mp t o m i n c a s e s o f ma l i g n a n c y o r
c o n n e c t i v e t i s s u e d i s e a s e . S t u d i e s h a v e s h o w n t h a t a mo n g p a t i e n t s w i t h f a t i g u e ,
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c o n n e c t i v e t i s s u e d i s e a s e . S t u d i e s h a v e s h o w n t h a t a mo n g p a t i e n t s w i t h f a t i g u e ,
a p p r o xi ma t e l y 4 0 % h a v e a n u n d e r l y i n g me d i c a l d i a g n o s i s , a p p r o xi ma t e l y 4 0 % h a v e a
p s y c h i a t r i c d i a g n o s i s , a n d 1 2 % h a v e b o t h me d i c a l a n d p s y c h i a t r i c e xp l a n a t i o n s f o r
t h e i r f a t i g u e . A p p r o xi ma t e l y 8 % o f p a t i e n t s h a v e n o d i s c e r n i b2)
l e. dI if a g n o s i s (
u n d i a g n o s e d f a t i g u e p e r s i s t s f o r > 6 mo n t h s a n d me e t s t h e o t h e r c r i t e r i a f o r C F S ,
t h a t d i a g n o s i s i s a p p l i e d . I f t h e o t h e r c r i t e r i a f o r C F S a r e n o t me t , t h e t e r m i d i o p a t h
c h r o n i c f a t i g u e i s u s e d . F a t i g u e t h a t c a n n o t b e a t t r i b u t e d t o a me d i c a l o r p s y c h i a t r i
diagnosis is often thought to be due to lifestyle factors.
P. 2 2
References
1 . C e n t e r s f o r D i s e a s e C o n t r o l a n d P r e vCe hr
n t oni
i o n .c f at i gue s y ndr,ome
accessed at National Center for Infectious Diseases
( w w w. c d c . g o v / n c i d o d / d i s e a s e s / c f s / i n d e x. h t m) o n 1 3 M a y 2 0 0 5 .
2 . M o r r i s o n J D . F a t i g u e a s a p r e s e n t i n g c o mp l a iJn tF am
i n FPP.r ac t
1980;10:795801.
3. Epstein K R. T he chronically fatigued M
p aed
t i eC
n tl .i n N or t h A1m9 9 5 ; 7 9 : 3 1 5
327.
4 . C r a i g T , K a k u ma n u S . C h r o n i c f a t i g u e s y n d r o me : e v a l u a t i o n a n d t r e a t me n t .
A m F am P hy s i c i an
2002;65:10831089.
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2.6 - Fever
> T a b l e o f C o n t e n t s > 2 - U n d i f f e r e n t i a t e d P r o b l e ms > 2 . 6 - F e v e r
2.6
Fever
Michae l D. Schooff
I . Background
F e v e r i s a n e l e v a t i o n i n t h e c o r e b o d y t e mp e r a t u r e a b o v e t h e i n d i v i d u a l ' s n o r ma l
r a n g e t h a t o c c u r s i n c o n j u n c t i o n w i t h a n i n c r e a s e i n t h e h y p o t h a l a mi c t e mp e r a t u r e
s e t p o i n t . F e v e r i s d e f i n e d a s a c o r e b o d y t e mp e r a t u r e o f 3 8 C ( 1 0 0 . 4 F ) .
H y p e r t h e r mi a i s a n e l e v a t e d b o d y t e mp e r a t u r e w i t h o u t a c h a n g e i n t h e h y p o t h a l a mi c
s e t t i n g . H y p e r p y r e xi a , a me d i c a l e me r g e n c y, i s d e f i n e d a s a t e mp e r a t u r e o v e r 4 1 . 1 C
(106.0F).
A. M echanisms of temperature control
B o d y t e mp e r a t u r e i s c o n t r o l l e d b y t h e h y p o t h a l a mu s , w h i c h r e c e i v e s i n p u t s f r o m b o t h
t h e p e r i p h e r a l n e r v e s a n d f r o m t h e t e mp e r a t u r e o f t h e b l o o d s u p p l y i n g t h e a r e a .
N o r ma l b o d y t e mp e r a t u r e i s ma i n t a i n e d a c r o s s e n v i r o n me n t a l v a r i a t i o n s t h r o u g h t h e
r e g u l a t i o n o f h e a t p r o d u c t i o n f r o m me t a b o l i c a c t i v i t y ( mo s t l y o f t h e mu s c l e s a n d l i v e r )
a n d h e a t d i s s i p a t i o n f r o m t h e s k i n a n d l u n g s . I t i s w i d e l y h e l d t h a t n o r ma l b o d y
t e mp e r a t u r e i s 3 7 . 0 C ( 9 8 . 6 F ) , b u t s e v e r a l s t u d i e s h a v e s h o w n t h a t a v e r a g e
t e mp e r a t u r e s i n h e a l t h y a d u l t s r a n g e f r o m 3 0 . 0 C t o 3 7 . 2 C ( 8 6 . 0 F 9 9 . 0 F ) w i t h
averages 36.4C to 36.8C (97.5F98.2F) and 99th percentile 37.5C to 37.7C
(99.5F99.9F
1,2)
)( .
B. Temperature measurement
H o w t h e t e mp e r a t u r e i s t a k e n c a n a f f e c t t h e r e s u l t . R e c t a l t e mp e r a t u r e i s c o n s i d e r e d
t h e c l o s e s t a p p r o xi ma t i o n t o c o r e t e mp e r a t u r e . S u b l i n g u a l t e mp e r a t u r e s a r e f e l t t o b e
f a i r l y r e l i a b l e , a n d g e n e r a l l y me a s u r e 0 . 6 C ( 1 . 0 F ) l o w e r t h a n r e c t a l t e mp e r a t u r e s .
A xi l l a r y a n d t y mp a n i c me a s u r e me n t s a r e l e s s r e l i a b l e , w i t h a xi l l a r y t e mp e r a t u r e s
r a n g i n g f r o m 0 . 2 5 C t o 0 . 8 5 C ( 0 . 4 F 1 . 5 F ) l o w e r t h a n r e c t a l me a s u r e me n t s , a n d
t y mp a n i c me a s u r e me n t s r a n g i n g f r o m 1 . 3 C ( 2 . 3 F ) l o w e r t h a n r e c t a l t o 0 . 7 C
( 1 . 3 F ) h i g h e3,4)
r (.
C. Temperature variation
N o r ma l b o d y t e mp e r a t u r e v a r i e s b y a n a v e r a g e o f 0 . 5 C ( 0 . 9 F ) t h r o u g h o u t t h e d a y,
w i t h t h e l o w e s t t e mp e r a t u r e e a r l y i n t h e mo r n i n g a n d p e a k i n t h e mi d a f t e r n o o n .
O t h e r f a c t o r s t h a t i n f l u e n c e n o r ma l b o d y t e mp e r a t u r e i n c l u d e a g e , r a c e , p h y s i c a l
a c t i v i t y, p o s t p r a n d i a l s t a t e , p r e g n a n c y o r o v u l a t i o n , e n d o c r i n e d i s o r d e r s , c l o t h i n g ,
a n d a mb i e n t t e mp e r a t u r e a n d h u mi d i t y.
I I I . Evaluation
A. History
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A d e t a i l e d h i s t o r y i s e s s e n t i a l t o e s t a b l i s h i n g t h e c a u s e o f f e v e r. T h e h i s t o r y s h o u l d
i n c l u d e t h e f o l l o w i n g c o mp o n e n t s :
1. C o mp l e t e r e v i e w o f s y s t e ms a s w e l l a s p a s t me d i c a l p r o b l e ms
2. P r e v i o u s s u r g e r i e s , w i t h a t t e n t i o n t o a n y i mp l a n t e d ma t e r i a l s o r d e v i c e s
3. M e d i c a t i o n s , s u p p l e me n t s , a n d o t h e r d r u g s u s e d
4. R e c e n t a n d r e mo t e t r a v e l
5. E xp o s u r e t o i l l i n d i v i d u a l s
6. E xp o s u r e t o a n i ma l s o r i n s e c t s
7. O c c u p a t i o n
8. I n g e s t i o n o f a n y q u e s t i o n a b l e f o o d s o r s u b s t a n c e s
9. F a mi l y h i s t o r y o f u n u s u a l i l l n e s s e s
P. 2 3
C a r e f u l p h y s i c a l e xa mi n a t i o n s h o u l d b e p e r f o r me d .
1. T e mp e r a t u r e a n d o t h e r v i t a l s i g n s s h o u l d b e me a s u r e d a c c u r a t e l y. H e a r t r a t e ,
b l o o d p r e s s u r e , a n d r e s p i r a t o r y r a t e n o r ma l l y i n c r e a s e i n t h e f a c e o f f e v e r.
B r a d y c a r d i a ma y b e a s i g n o f a t y p i c a l i n f e c t i o n s . H y p o t e n s i o n ma y b e a s i g n o f
s y s t e mi c s e p s i s .
2. A n e xa mi n a t i o n o f a l l o r g a n s y s t e ms a n d b o d y a r e a s s h o u l d b e p e r f o r me d , w i t h
e mp h a s i s g i v e n t o t h e s k i n , l y mp h a t i c s , h e a r t , l u n g s , a n d n e r v o u s s y s t e m. I n
a d d i t i o n , g e n i t a l a n d r e c t a l e xa mi n a t i o n s s h o u l d b e p e r f o r me d r e g a r d l e s s o f
g e n d e r.
C. Testing
D i a g n o s t i c t e s t i n g s h o u l d b e g u i d e d b y t h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n .
1. W h e n t h e s o u r c e o f t h e f e v e r i s u n c l e a r, h e l p f u l l a b o r a t o r y t e s t s i n c l u d e w h i t e
b l o o d c e l l c o u n t w i t h d i f f e r e n t i a l , u r i n a l y s i s w i t h mi c r o s c o p i c e xa mi n a t i o n a n d
c u l t u r e i f r e s u l t s a r e a b n o r ma l , a n d a e r o b i c a n d a n a e r o b i c b l o o d c u l t u r e s .
P. 2 4
TAB L E 2.6.1 Potential Causes of Fever of Unknown Origin

I nfe ctious
dise ase s
Collage n
v ascular
dise ase s
Malignancie s
Me dications
Othe r
Tuberculosis
S t i l l ' s d i s e a s e L y mp h o ma
C a r b a ma ze p i n e G r a n u l o ma t o u s
diseases
Occult
abscess
T e mp o r a l
arteritis
L e u k e mi a
Phenytoin
O s t e o my e l i t i s
Polyarteritis
H y p e r n e p h r o ma A n t i h i s t a mi n e s Ve n o u s
P u l mo n a r y
e mb o l u s
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2.6 - Fever
nodosa
Endocarditis
R h e u ma t i c
fever
Sinusitis
t h r o mb o s i s
Other solid
t u mo r s
Methyldopa
Endocrine
disorders
S y s t e mi c
A t r i a l my xo ma
lupus
e r y t h e ma t o s u s
Allopurinol
Factitious fever
H IV (late
stage)
R h e u ma t o i d
arthritis
Colon cancer
S u l f o n a mi d e s
Cerebrovascular
accident
Q fever
P o l y my a l g i a
r h e u ma t i c a
H e p a t o ma
C ephalosporins Alcoholic
hepatitis
Tropheri
B r uc el l a
M y c opl as ma
C hl amy di a
H i s t o p l a s mo s i s
L egi onel l a
B ar t onel l a
H AC E K
A me b i c
hepatitis
Medical device
infections
I s o n i a zi d
Cirrhosis
H A C E K ,H aemophi l us s pec i(es

H . par ai nf l uenzae
, H . aphr ophi l ,usa n dH . par aphr ophi )l us
,
A c t i nobac i l l us ac t i nomy c et emc omi
, Ctar
ans
di obac t er i um homi, E
nii sk enel l a c or r odens
,
a n d K i ngel l as p e c i e s .
2. A c h e s t r a d i o g r a p h a n d a b d o mi n a l i ma g i n g ( c o mp u t e d t o mo g r a p h y o r u l t r a s o u n d )
ma y b e h e l p f u l i f t h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n s u g g e s t a p u l mo n a r y o r
a b d o mi n a l i n f e c t i o n .
3. I f t h e s o u r c e o f t h e f e v e r r e ma i n s u n d e t e r mi n e d a f t e r t h e s e c o mmo n t e s t s h a v e
b e e n p e r f o r me d , o t h e r t e s t s t o c o n s i d e r i n c l u d e t h e h u ma n i mmu n o d e f i c i e n c y
v i r u s s e r o l o g y, r a p i d p l a s ma r e a g i n , r h e u ma t o i d f a c t o r, a n t i n u c l e a r a n t i b o d y,
s e d i me n t a t i o n r a t e o r C - r e a c t i v e p r o t e i n , s e r u m c h e mi s t r i e s a n d e n zy me s ,
t u b e r c u l o s i s s k i n t e s t i n g , e xa mi n a t i o n o f t h e s p i n a l f l u i d , a n d t e c h n e t i u m- l a b e l e d
bone scan.
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2.6 - Fever
I V. Diagnosis
A. Acute febrile illness
I n t h e o u t p a t i e n t s e t t i n g , mo s t f e v e r s a r e a s s o c i a t e d w i t h a n a c u t e i l l n e s s a n d a r e
c a u s e d b y s e l f - l i mi t e d v i r a l i n f e c t i o n s , s u c h a s u p p e r r e s p i r a t o r y t r a c t i n f e c t i o n s o r
acute gastroenteritis. T hese infections usually resolve in 7 to 10 days and require
o n l y s u p p o r t i v e a n d s y mp t o ma t i c t h e r a p y. C o mmo n b a c t e r i a l i n f e c t i o n s r e q u i r i n g
antibiotic therapy include streptococcal pharyngitis, cellulitis,
P. 2 5
u r i n a r y t r a c t i n f e c t i o n s , p n e u mo n i a , a c u t e e xa c e r b a t i o n o f c h r o n i c b r o n c h i t i s , a n d
b a c t e r i a l s i n u s i t i s . A c a r e f u l h i s t o r y a n d p h y s i c a l e xa mi n a t i o n , s u p p o r t e d b y s e l e c t e d
d i a g n o s t i c t e s t s s h o u l d l e a d t o a s p e c i f i c d i a g n o s i s i n a l mo s t a l l o f t h e s e c a s e s .
F i g u r e 2 . 6.. 1A p p r o a c h t o t h e e v a l u a t i o n o f f e v e r. F U O , f e v e r o f u n k n o w n o r i g i n .
B. Fever in the elderly

I n f e c t i o n s i n t h e e l d e r l y o f t e n d o n o t r e s u l t i n t h e s a me s i g n s a n d s y mp t o ms a s i n
y o u n g e r p a t i e n t s . W h i l e s o me e l d e r l y p a t i e n t s ma y f a i l t o mo u n t a f e b r i l e r e s p o n s e t o
s o me i n f e c t i o n s , f e b r i l e e l d e r s a r e mo r e l i k e l y t o h a v e a b a c t e r i a l i l l n e s s t h a n a r e
their younger counterparts. A careful search for an infection should be undertaken
f o r o l d e r i n d i v i d u a l s w i t h f e v e r, s i g n s o r s y mp t o ms o f a n i n f e c t i o n ( e . g . , c o u g h ,
urinary frequency), or a change in their appetite, behaviors, physical abilities, or
me n t a l s t a t u s . C o mmo n s i t e s o f i n f e c t i o n s i n t h e e l d e r l y i n c l u d e t h e s k i n , l u n g s , a n d
t h e u r i n a r y s y s t e m.
C. Postoperative fever
C o mmo n c a u s e s o f f e v e r i n t h e p o s t o p e r a t i v e p e r i o d c a n b e c a t e g o r i ze d b y h o w
soon after surgery the fever develops. Fever in the first 2 days after surgery is often
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2.6 - Fever
c a u s e d b y a t e l e c t a s i s o f t h e l u n g s . U r i n a r y t r a c t i n f e c t i o n s , p n e u mo n i a , a n d
i n f e c t i o n s o f i n t r a v a s c u l a r a c c e s s s i t e s c o mmo n l y p r e s e n t o n p o s t o p e r a t i v e d a y s 3
through 5. Fevers beyond the fifth day after surgery should lead one to consider
wound infections or abscesses.
D. Fever of unknown origin (FU O)

N u me r o u s d e f i n i t i o n s o f F U O e xi s t , b u t mo s t i n c l u d e a f e v e r d o c u me n t e d o n s e v e r a l
different days over 2 or 3 weeks, with no diagnosis found following repeated physical
e xa mi n a t i o n s a n d r o u t i n e d i a g n o s t i c t e s t s . D i s e a s e s t h a t ma y c a u s e F U O a r e l i s t e d
i n T a b l e 2 . 6 . 1O n e a p p r o a c h t o e v a l u a t i n g t h e p a t i e n t w i t h F U O i F
s i sghuor ew n i n
2 . 6 . 1. T h e u n d e r l y i n g e t i o l o g y i s e v e n t u a l l y f o u n d i n o v e r 9 0 % o f F U O c a s e s .
H i s t o r i c a l l y, t h e mo s t c o mmo n c a u s e o f F U O w a s i n f e c t i o n , f o l l o w e d b y
ma l i g n a n c i e s , a n d t h e n r h e u ma t o l o g i c d i s e a s e s . I n mo r e r e c e n t s t u d i e s ,
r h e u ma t o l o g i c c a u s e s h a v e s u r p a s s e d ma l i g n a n c i e s a s t h e s e c o n d mo s t c o mmo n
c a u s e o f F U O . F U O i s mu c h mo r e l i k e l y t o b e c a u s e d b y a n u n u s u a l p r e s e n t a t i o n o f
a c o mmo n d i s e a s e t h a n a c o mmo n p r e s e n t a t i o n o f a n u n u s u5)
a l. d i s e a s e (
References
1 . M a c k o w i a k PA , Wa s s e r ma n S S , L e v i n e M M . A c r i t i c a l a p p r a i s a l o f 9 8 . 6
d e g r e e s F, t h e u p p e r l i mi t o f t h e n o r ma l b o d y t e mp e r a t u r e , a n d o t h e r l e g a c i e s o f
C a r l R e i n h o l d A u g u s t Wu n d e Jr lAi cMh A
. 1992;268:15781580.
2 . L e c k i e TN. or mal t emper at ur e i n t he el. der
L a sl yt mo d i f i e d 2 2 J u n e 2 0 0 4 .
A c c e s s e d a t B e s t B E T s : B e s t E v i d e n c e T o p i c s ( w w w. b e s t b e t s . o r g / c g i - b i n / b e t s . p l ?
record=00774) on 15 May 2005.
3 . R i d e l l A , E p p i c h SW.
houl d t y mpani c t emper at ur e meas ur ement be t r us t ed?
L a s t mo d i f i e d 1 9 F e b r u a r y 2 0 0 3 . A c c e s s e d a t B e s t B E T s : B e s t E v i d e n c e T o p i c s
( w w w. b e s t b e t s . o r g / c g i - b i n / b e t s . p l ? r e c o r d = 0 0 3 4 0 ) o n 1 5 M a y 2 0 0 5 .
4 . C r a i g J V, L a n c a s t e r G A , W i l l i a ms o n P R , e t a l . T e mp e r a t u r e me a s u r e d a t t h e
a xi l l a c o mp a r e d w i t h r e c t u m i n c h i l d r e n a n d y o u n g p e o p l e : s y s tBeM
miJc r e v i e w.
2 0 0 0 ; 3 2 0 : 11 7 4 11 7 8 .
5 . M o u r a d O , P a l d a V, D e t s k y A S . A c o mp r e h e n s i v e e v i d e n c e - b a s e d a p p r o a c h t o
fever of unknown oriA
g irnc.h I nt er n M ed
2003;163:545551.
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2.7 - Headaches
> T a b l e o f C o n t e n t s > 2 - U n d i f f e r e n t i a t e d P r o b l e ms > 2 . 7 - H e a d a c h e s
2.7
Headaches
Ke nne th D. Pe te rs
I . Background
H e a d a c h e i s o n e o f t h e 2 0 mo s t f r e q u e n t r e a s o n s t h a t c a u s e p a t i e n t s t o v i s i t p r i ma r
c a r e p r o v i d e r s i n t h e U n i t e d S t a t e s . I n a s t u d y o f 2 0 , 4 6 8 p a t i e n t s , mi g r a i n e
h e a d a c h e , o n e o f t h e c o mmo n c a u s e s o f r e c u r r e n t h e a d a c h e , o c c u r r e d o n e o r mo r e
t i me s y e a r l y i n 1 7 . 6 % o f w o me n a n d i n 5 . 7 %1)o. f me n (
I n t h e e v a l u a t i o n o f a r e c u r r e n t h e a d a c h e , t h e i mp o r t a n t t a s k s a r e t o c a t e g o r i ze t h e
h e a d a c h e t y p e w i t h a s mu c h p r e c i s i o n a s p o s s i b l e a n d t o e l i mi n a t e p o t e n t i a l l y
serious causes.
A. Headache types
B a s i c a l l y, t w o t y p e s o f r e c u r r e n t h e a d a c h e s a r e s e e n : p r i ma r y h e a d a c h e s a n d
h e a d a c h e s c a u s e d b y o t h e r i l l n e s s eTsa b( sl e e2 . 7 ). .1
B. Special concerns
T h e s e i n c l u d e a b r a i n t u mo r, i n t r a c r a n i a l b l e e d , me n i n g i t i s , o r o t h e r s e r i o u s c a u s e s
I n p r i ma r y c a r e p a t i e n t s w i t h h e a d a c h e a s a p r e s e n t i n g s y mp t o m, t h e r i s k o f
s e r i o u s i n t r a c r a n i a l p a t h o l o g y i 2)
s .< 1G%
e n( e r a l l y, s u c h p a t i e n t s h a v e a h i s t o r y o f a
n e w - o n s e t o r w o r s e n i n g h e a d a c h e p a t t e r n o r a n a b n o r ma l n e u r o l o g i c f i n d i n g , w h i c h
mi g h t i n c l u d e a s e i zu r e .
I I I . Evaluation
A. History
1. C h a r a c t e r i s t i c s o f t h e h e a d aWchhaet i s t h e t y p e o f p a i n , i t s l o c a t i o n , i t s
d u r a t i o n , a n d i t s i n t e n s i t y ? W h a t s y mp t o ms p r e c e d e o r a c c o mp a n y t h e p a i n ?
D o e s a n y t h i n g t r i g g e r t h e h e a d a c h e o r ma k e t h e p a i n b e t t e r o r w o r s e ? I n f o r m
the patient about a typical headache from beginning to end.
a. F o o d s t h a t t r i g g e r mi g r a i n e i n c l u d e a l c o h o l , a g e d c h e e s e , c h o c o l a t e , a n d
a s p a r t a me .
b. A p p r o xi ma t e l y 2 0 % t o 3 0 % o f mi g r a i n e u r s r e p o r t a n a u r a , t y p i c a l l y v i s u a l i n
66 / 652
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2.7 - Headaches
nature.
c. P a t i e n t s w i t h c l u s t e r h e a d a c h e r e p o r t u n i l a t e r a l t e mp o r a l h e a d a c h e ,
o c c u r r i n g g e n e r a l l y o n c e d a i l y, u s u a l l y i n t h e e v e n i n g a n d a s s o c i a t e d w i t h
ipsilateral nasal stuffiness and conjunctival injection.
d. P a t i e n t s w i t h c h r o n i c d a i l y h e a d a c h e ( C D H ) e xp e r i e n c e i t a t l e a s t 1 0 t o 1 5
d a y s / mo n t h a n d u s u a l l y r e p o r t h e a v y u s e o f r e l i e f d r u g s .
e. R e d f l a g s t h a t mi g h t s u g g e s t i n t r a c r a n i a l p a t h o l o g y i n c l u d e a l o s s o f
c o n s c i o u s n e s s , p e r s i s t e n t v i s u a l l o s s , s e i zu r e s , s t a g g e r i n g , o r h e a r i n g l o s s .
2. C h r o n o l o g y o f t h e h e a d a M
c hoes t p r i ma r y h e a d a c h e s r e c u r p e r i o d i c a l l y f o r
y e a r s , w i t h o n l y s u b t l e c h a n g e s o v e r t i me . I f t h e h e a d a c h e i s b e c o mi n g w o r s e ,
t h e c a u s e ma y b e p s y c h o s o c i a l s t r e s s o r s , me d i c a t i o n o v e r u s e , o r e v o l v i n g
i n t r a c r a n i a l p a t h o l oTgayb (l e 2 . 7 ). .1 A s k w o me n w h e t h e r t h e h e a d a c h e s e e ms
related to
P. 2 7
me n s e s . P a s t a n d c u r r e n t me d i c a t i o n u s e a n d h o w t h e y a f f e c t t h e h e a d a c h e c a n
b e i mp o r t a n t c l u e s t o h e a d a c h e s e v e r i t y a n d h o w t h e p a t i e n t ma y r e s p o n d t o t h e
t r e a t me n t .
TAB L E 2.7.1 Types of Recurrent Headache With

Selected Exam ples
P r i m a r y h e a d a c h etse:n s i o n - t y p e , mi g r a i n e , c l u s t e r, o t h e r
S e c o n d a r y h e a d a c h(eas
s s oc i at ed wi) t: hb r a i n t u mo r, v a s c u l a r
a b n o r ma l i t y, h y p e r t e n s i o n , i n f e c t i o n , c h r o n i ca,dsaui lbys H
t aAn c e
w i t h d r a w a l , t e mp o r a l a r t e r i t i s , n e u r a l g i a , s i n u s H A , me t a b o l i c d i s o r d e r s ,
other
aC h r o n i c d a i l y H A i s c l a s s i f i e d a s a s e c o n d a r y H A b e c a u s e i t t y p i c a l l y
p r e s e n t s a s a r e b o u n d h e a d a c h e i n a mi g r a i n e u r w h o o v e r u s e s
analgesics.
H A, headache.
3. F a m i l y h i s t o rMy i g r a i n e h e a d a c h e s o f t e n e xh i b i t a f a mi l i a l p a t t e r n ; t h e c a u s e s
of secondary headaches generally do not. Tension headache can represent a
f a mi l y p a t t e r n o f r e a c t i n g t o s t r e s s .
4. P s y c h o s o c i a l a s p e c t s o f t h e h e a dWa hc ahte d o e s t h e p a t i e n t b e l i e v e i s t h e
c a u s e o f t h e h e a d a c h e ? W h a t l i f e e v e n t s mi g h t b e p l a y i n g a r o l e ? H o w d o e s t h e
p a t i e n t ' s f a mi l y r e a c t t o t h e h e a d a c h e ? A s k : I f y o u d i d n o t h a v e t h e h e a d a c h e ,
h o w w o u l d y o u r l i f e b e d i f f e r e n t ? T h e k e y t o t h e ma n a g e me n t o f r e c u r r e n t
p r i ma r y h e a d a c h e s o f t e n l i e s i n t h e r e s p o n s e s t o t h e s e q u e s t i o n s , w h i c h c a n
r e v e a l u n a n t i c i p a t e d s t r e s s o r s , s e c o n d a r y g a i n , o r f a mi l y d i s c o r d .
5. O t h e r i n f o r m a t i oI mp
n ortant data includes the use of tobacco, alcohol, or
67 / 652
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2.7 - Headaches
c a f f e i n e ; r e s p o n s e t o e xe r c i s e , a h i s t o r y o f h e a d t r a u ma ; e xp o s u r e t o t o xi c f u me
o r c h e mi c a l s . H a v e t h e r e b e e n s y mp t o ms o f f e v e r, o r f a t i g u e ? A s k a b o u t
d e p r e s s i o n , w h i c h i s o f t e n s e e n i n mi g r a i n e u r s . G e n e r a l l y, s u p r a t e n t o r i a l s p a c e
o c c u p y i n g l e s i o n s c a u s e n e u r o l o g i c s e q u e l a e a n d s e i zu r e s , w h e r e a s
i n f r a t e n t o r i a l l e s i o n s g e n e r a l l y c a u s e h e a d a c h e , ma l a i s e , n a u s e a , a n d s t i f f n e c k
A l l o d y n i a i s a c o mmo n f i n d i n g i n c h r o n i c mi3)g .r a i n e s (
1. A f o c u s e d p h y s i c a l e x a m i n aTt ihoi sn s h o u l d i n c l u d e v i t a l s i g n s ( n o t a b l y b l o o d
p r e s s u r e ) a n d a n e xa mi n a t i o n o f t h e s c a l p ; e y e s , i n c l u d i n g f u n d u s c o p i c
e xa mi n a t i o n ; e a r s ; n o s e ; p a r a n a s a l s i n u s e s ; t h r o a t ; a n d n e c k . A s c r e e n i n g
n e u r o l o g i c e xa mi n a t i o n , i n c l u d i n g c r a n i a l n e r v e s , c o o r d i n a t i o n ( f i n g e r - t o - n o s e
t e s t ) , a n d d e e p t e n d o n r e f l e xe s , i s s u f f i c i e n t i n mo s t i n s t a n c e s . I n t h e
mi g r a i n e u r, t h e e xa mi n a t i o n f i n d i n g s s h o u l d b e a l l n o r ma l i n t h e a b s e n c e o f a
current headache; a positive finding warrants further testing.
2. O t h e r p h y s i c a l e x a m i n a t i o n m a n e uT vhe rsse a r e a p p r o p r i a t e i f t h e me d i c a l
history suggests specific secondary headache causes: palpation of the
s u p e r f i c i a l t e mp o r a l a r t e r i e s ( t e mp o r a l a r t e r i t i s ) , a u d i o me t r y ( a c o u s t i c n e u r o ma )
t r a n s i l l u mi n a t i o n o f t h e p a r a n a s a l s i n u s e s ( s i n u s h e a d a c h e ) , o r c h e c k i n g f o r
n u c h a l r i g i d i t y p l u s K e r n i n g ' s a n d B r u d zi n s k i ' s s i g n s ( me n i n g e a l i r r i t a t i o n ) .
C. Testing
1. C l i n i c a l l a b o r a t o r y t eFsot rs mo s t p a t i e n t s w i t h r e c u r r e n t h e a d a c h e , n o b l o o d ,
u r i n e , o r o t h e r c l i n i c a l l a b o r a t o r y t e s t s a r e n e e d e d . L a b o r a t o r y t e s t s t h a t mi g h t
b e s u g g e s t e d b y t h e c l i n i c a l h i s t o r y a n d t h e p h y s i c a l e xa mi n a t i o n i n c l u d e
e r y t h r o c y t e s e d i me n t a t i o n r a t e ( t e mp o r a l a r t e r i t i s ) , h e ma t o c r i t o r t h y r o i d s t u d i e s
( f a t i g u e ) , c e r e b r o s p i n a l f l u i d e xa mi n a t i o n ( me n i n g e a l i r r i t a t i o n ) , a n d w h i t e b l o o d
c o u n t w i t h d i f f e r e n t i a l ( s y s t e mi c i n f e c t i o n ) .
I ng mo s t i n s t a n c e s , d i a g n o s t i c i ma g i n g i s n o t n e e d e d . I n
o n e s t u d y, 3 5 0 p a t i e n t s w i t h a c h i e f c o mp l a i n t o f h e a d a c h e , r e g a r d l e s s o f t h e
c o mp l a i n t h e a d a c h e , o f t h e p r e s e n c e o r a b s e n c e o f n e u r o l o g i c s i g n s , w e r e
r e f e r r e d f o r c o mp u t e d t o mo g r a p h y ( C T ) s c a n . O n l y 2 % h a d c l i n i c a l l y s i g n i f i c a n t
C T f i n d i n g s , a n d a l l p a t i e n t s w i t h s i g n i f i c a n t C T f i n d i n g s h a d a b n o r ma l p h y s i c a l
e xa mi n a t i o n f i n d i n g s o r u n u s u a l c l i n i c a l s y4)
mp
. t o ms (
a. D i a g n o s t i c i ma g i n g ma y b e i n d i c a t e d i n p a t i e n t s w i t h a t y p i c a l h e a d a c h e
p a t t e r n s , a h i s t o r y o f s e i zu r e s , o r f o c a l n e u r o l o g i c s i g n s o5)
r .s y mp t o ms (
N e w - o n s e t a n d w o r s t e v e r h e a d a c h e s a r e s i g n i f i c a n t c o mp l a i n t s ( i . e . ,
atypical headache patterns).
b. D e s p i t e t h e g r e a t e r c o s t , ma g n e t i c r e s o n a n c e i ma g i n g p r o v i d e s t h e b e s t
i ma g i n g f o r t h e d e t e c t i o n o f b r a i n t u mo r s a n d mo s t o t h e r c h r o n i c p a t h o l o g i c
c a u s e s o f h e a d a c h e t h a t c a n b e d e t e c t e d b y i ma g i n g .
c. M o r e r e c e n t d e v e l o p me n t s i n i ma g i n g t e c h n o l o g y c a n h e l p d i f f e r e n t i a t e
b e n i g n a n d ma l i g n a n t l e s i o n s a n d t o mo r e p r e c i s e l y d e f i n e t h e a n a t o my
68 / 652
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2.7 - Headaches
( e . g . , s i n g l e - p r o t o n e mi s s i o n C T s c a n a n d ma g n e t i c r e s o n a n c e
a n g i o g r a p h y3)) (.
I V. Diagnosis
T h e k e y t o t h e d i a g n o s i s o f h e a d a c h e i s t h e c l i n i c a l h i s t o r y. A h i s t o r y o f a n a c h i n g ,
b i t e mp o r a l h e a d a c h e t h a t i s a s s o c i a t e d w i t h s t r e s s a n d t h a t w a xe s a n d
P. 2 8
wanes is a typical tension headache. Migraine is characteristically a one-sided
h e a d a c h e , t h r o b b i n g i n n a t u r e , o f t e n a s s o c i a t e d w i t h n a u s e a a n d v o mi t i n g , f r e q u e n t
a c c o mp a n i e d b y p h o t o p h o b i a a n d s o n o p h o b i a , a n d l a s t i n g 4 t o 1 2 h o u r s , p e r h a p s
l o n g e r. I t ma y b e w i t h a u r a ( c o mmo n mi g r a i n e ) o r w i t h o u t a u r a ( c o mmo n
mi g r a i n e ) , w i t h t h e l a t t e r s e e n i n 7 0 % t o 8 0 % o f mi g r a i n e u r s . C l u s t e r h e a d a c h e i s a
s t r i c t l y o n e - s i d e d , r e c u r r i n g h e a d a c h e t h a t c h i e f l y a f f e c t s me n , a n d t h a t o c c u r s i n
c l u s t e r s o f 1 t o 2 mo n t h s o f e p i s o d e s . A n i n c r e a s i n g n u mb e r o f p a t i e n t s h a v e C D H
o f t e n w i t h v i r t u a l l y c o n s t a n t d i s c o mf o r t ; ma n y C D H s a r e t h e r e s u l t o f t r a n s f o r me d
mi g r a i n e f o l l o w i n g d a i l y a n a l g e s i c u s e , e s p e c i a l l y c o d e i n e6)d.e r i v a t i v e s (
Because recurrent headache is caused, at least in part, by life stresses and becaus
i t a l s o c a u s e s p e r s o n a l a n d f a mi l y s t r e s s , t h e d i a g n o s t i c a s s e s s me n t i s i n c o mp l e t e
u n t i l t h i s c o mp l e x r e l a t i o n s h i p h a s b e e n a d e q u a t e l y e xp l o r e d o v e r a s e r i e s o f v i s i t s .
References
1 . S t e w a r t W F, L i p t o n R B , C e l e n t a n o D D , e t a l . P r e v a l e n c e o f mi g r a i n e
h e a d a c h e i n t h e U n i t e d S t aJ tAeM
s .A 1 9 9 2 ; 2 6 7 : 6 4 6 9 .
2 . B e c k e r L , I v e r s o n D C , R e e d F M , e t a l . P a t i e n t s w i t h n e w h e a d a c h e i n p r i ma r y
c a r e : a r e p o r t f r o m A SJP N
F am
.
P r ac1t 9 8 8 ; 2 7 : 4 1 4 7 .
3 . S i l b e r s t e i n S D , H e a d a c h e d i a g n o s i s a n d ma n a56t
g e hmeAnnnual
t.
M eet ,i ng
S a n F r a n c i s c o , C A : A me r i c a n A c a d e my o f N e u r o l o g y, A p r i l 2 4 , 2 0 0 4 M a y 1 ,
2004.
4 . M i t c h e l l C S , O s b o r n R E , G r o s s k r e u t z S R . C o mp u t e d t o mo g r a p h y i n t h e
h e a d a c h e p a t i e n t : i s r o u t i n e e v a l u a t i o n , r e a l l y nH
e ceadac
e s s a rhe
y ?1 9 9 3 ; 3 3 : 8 3
86.
5 . T h e u t i l i t y o f n e u r o i ma g i n g i n t h e e v a l u a t i o n o f h e a d a c h e i n p a t i e n t s w i t h
n o r ma l n e u r o l o g i c e xa mi n a t i o n s : s u mma r y s t a t e me n t . R e p o r t o f t h e Q u a l i t y
S t a n d a r d s S u b c o mmi t t e e o f t h e A me r i c a n A c a d e my o f N eur
e u r ol
o l ogy
ogy
1994;44:13531354.
6. Silberstein S D, L ipton R B, Sliwinski M. Classification of daily and near daily
h e a d a c h e s : f i e l d t r i a l s o f r e v i s e d I H S Nc reur
i t e ol
r i aogy
. 1997;49:638639.
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2.8 - Hypersomnia
> T a b l e o f C o n t e n t s > 2 - U n d i f f e r e n t i a t e d P r o b l e ms > 2 . 8 - H y p e r s o mn i a
2.8
Hypersomnia
Je nnife r J. Bue sche r
I . Background
H y p e r s o mn i a , a l s o k n o w n a s e xc e s s i v e d a y t i me s l e e p i n e s s ( E D S ) , i s d e f i n e d b y a
n e e d t o s l e e p d u r i n g d a y t i me h o u r s a n d t h e a b i l i t y t o f a l l a s l e e p i n s i t u a t i o n s d u r i n g
w h i c h a l e r t n e s s i s d e s i1)
r e. dE(D S s h o u l d b e d i f f e r e n t i a t e d f r o m g e n e r a l i ze d f a t i g u e
a n d n o n s p e c i f i c t i r e d n e s s , a s p a t i e n t s o f t e n u s e t h e t e r ms i n t e r c h a n g e a b l y.
G e n e r a l i ze d f a t i g u e i s a p r o b l e m o f d e c r e a s e d p h y s i c a l e n e r g y, mu s c l e e xh a u s t i o n ,
a n d p o s s i b l y p o o r c o n c e n t r a t i o n a n d me mo r y, w h e r e a s h y p e r s o mn i a i s t h e p r o b l e m o
f a l l i n g a s l e e p a t i n a p p r o p r i a t e o r u n d e s i r e d t i me s .
A. Etiology
H y p e r s o mn i a c a n b e a p r i ma r y s l e e p d i s o r d e r, b u t i s mo r e c o mmo n l y s e c o n d a r y t o
d i s o r d e r s t h a t d i s r u p t n o r ma l s l e e p p a t t e r n s . P o o r q u a l i t y s l e e p ma y b e c a u s e d b y
b e h a v i o r a l r o u t i n e s , e n v i r o n me n t a l d i s t u r b a n c e s , me d i c a l a n d p s y c h o l o g i c a l
d i s o r d e r s , o r i n s o mn i a .
B. Epidemiology
E D S i s a c o mmo n p r o b l e m, a f f e c t i n g b e t w e e n 5 % a n d 1 5 % o f t h e g e n e r a l a d u l t
p o p u l a t i o n2)(, a n d a s ma n y a s 5 8 % o f p s y c h i a t r i c p3)
a t.i eTnht se (p r e v a l e n c e o f
n a r c o l e p s y i s t h o u g h t t o b e a p p r o xi ma t e l y 0 . 0 5 % , a l t h o u g h e p i d e mi o l o g i c s t u d i e s
vary greatly depending on the country of origin and the definitions of the disease.
I I I . Evaluation
T h e e v a l u a t i o n o f h y p e r s o mn i a s h o u l d i n c l u d e a n i n v e s t i g a t i o n f o r i n t r i n s i c , e xt r i n s i
a n d c i r c a d i a n r h y t h m s l e e p d i s o r d e r s , a s w e l l a s me d i c a l a n d
P. 2 9
psychiatric diseases that are associated with sleepiness. Intrinsic causes of daytim
s l e e p i n e s s , i n c l u d i n g p r i ma r y s l e e p d i s o r d e r s , a r e r e l a t i v e l y u n c o mmo n a s c o mp a r e d
t o e xt r i n s i c c a u s e s a n d c i r c a d i a n r h y t h m d i s o r d e r s ( mi s a l i g n me n t o f s l e e p p a t t e r n s
w i t h l o c a l t i me
1)).(
A. History and review of systems

G e n e r a l i ze d f a t i g u e s h o u l d b e d i f f e r e n t i a t e d f r o m d a y t i me s l e e p i n e s s i n w h i c h o n e
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2.8 - Hypersomnia
e xp e r i e n c e s h e a d s a g g i n g , e y e l i d d r o o p i n g , a n d s h o r t p e r i o d s o f s l e e p a t
i n a p p r o p r i a t e t i me1)s. (A h i s t o r y o f s l e e p a t t a c k s ( s u d d e n l y f a l l i n g a s l e e p i n
d a n g e r o u s s i t u a t i o n s ) o r e p i s o d e s o f c a t a p l e xy ( s u d d e n a n d t r a n s i e n t l o s s o f mu s c l e
strength) can be particularly dangerous and highly suggestive of a diagnosis of
n a r c o l e p s y1,3)
( . A f u l l r e v i e w o f s y s t e ms o f t e n h i g h l i g h t s o t h e r me d i c a l a n d
p s y c h i a t r i c d i s o r d e r s t h a t ma y b e c a u s i n g d a y t i me s l e e p i n e s s . A b r u p t s y mp t o m o n s e
s h o u l d h e i g h t e n c o n c e r n f o r c e n t r a l n e r v o u s s y s t e m t u mo r s a n d i s c h e mi c s t r o k e .
1. S l e e p p a t t e r nAs t h o r o u g h d i s c u s s i o n o f t h e p a t i e n t ' s s l e e p p a t t e r n s h o u l d
i n c l u d e d e t a i l s a b o u t b e d t i me a n d w a k e t i me , n i g h t t i me a w a k e n i n g s , e a t i n g
habits prior to sleep, work patterns, and the sleep behavior of bed partners and
o t h e r me mb e r s o f t h e h o u s e h o l d .
2. M e d i c a t i o n h i s t oM
r ye d i c a t i o n s t h a t i n d u c e s o mn o l e n c e o r i n t e r r u p t t h e n o r ma l
s l e e p c y c l e a r e l i s t eTdaibnl e 2 . 8 . 1
T h e p h y s i c a l e xa mi n a t i o n i s mo s t u s e f u l i n t h e e v a l u a t i o n o f me d i c a l a n d
p s y c h o l o g i c a l d i s o r d e r s t h a t ma y b e c a u s i n g s l e e p i n e s s . P a t i e n t s w i t h i n t r i n s i c ,
e xt r i n s i c , a n d c i r c a d i a n r h y t h m s l e e p d i s o r d e r s a r e l i k e l y t o h a v e a n o r ma l
P. 3 0
p h y s i c a l e xa mi n a t i o n .
TAB L E 2.8.1 Substances T hat Can Cause

Som nolence or Disordered Sleep
Pharm acologic age nts
He rbal age nts
Tricyclic antidepressants
C h a mo mi l e
Lithium
R o s e ma r y
-1 and 2 antagonists
Kava kava
Anticholinergics
Marjoram
Anticonvulsants
Poppy seeds
A n t i e me t i c s
Va l e r i a n
Melatonin
Antiparkinsonian agents
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Antipsychotics
Othe r
Barbiturates
Alcohol
B e n zo d i a ze p i n e s
A mp h e t a mi n e s
- B lockers
Nicotine
Clofibrate
Illicit drugs
Decongestants
G e mf i b r o zi l
M u s c l e r e l a xa n t s
Opiates
Selective serotonin reuptake inhibitors
Spironolactone
A d a p t e d f r o m Q u r e s h i A , L e e - C h i o n g T J r. M e d i c a t i o n s a n d t h e i r e f f e c t s o n
s l e e p .M ed C l i n N or t h A2m0 0 4 ; 8 8 ( 3 ) : 7 5 1 7 6 6 .
TAB L E 2.8.2 Differential Diagnosis of Hypersom nia

I n t r i n s i c d i s o r d e 1,2)
rs (
E x t r i n s i c d i s o r d e 2,4)
rs (
N a r c o l e p s y w i t h o r w i t h o u t c a t a p l e xy
P o s t t r a u ma t i c h y p e r s o mn i a
Obstructive sleep apnea
R e s t l e s s l e g s s y n d r o me
P e r i o d i c l i mb mo v e me n t d i s o r d e r
I d i o p a t h i c h y p e r s o mn i a
Alcohol (decreases sleep

latency and causes rebound
a r o u s a l i n t h e mi d d l e o f t h e
sleep cycle)
E n v i r o n me n t a l f a c t o r s c a u s i n g
poor sleep (e.g., snoring
spouse, loud noises)
Sleep deprivation
C i r c a d i a n r h y t h m s l e e p d i s o r 3)
de rs ( Me dical and psy chiatric
d i a g n o s e s2)(
Jet lag type
Alcoholism and substance

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D e l a y e d s l e e p p h a s e t y p e ( mo r e c o mmoanb ui ns e
adolescents and young adults, usually A
v el zh
r y e i me r ' s d i s e a s e a n d o t h e r
l a t e b e d t i me s a n d l a t e o r mi d d a y w a k ed e me n t i a s
t i me s )
A n xi e t y d i s o r d e r s
A d v a n c e d s l e e p p h a s e t y p e ( mo r e c o mmo
C hnr o n i c o b s t r u c t i v e
i n o l d e r a d u l t s , e a r l y b e d t i me a n d e a r l py u l mo n a r y d i s e a s e
w a k e t i me )
Depressive disorders
Shift work type
Gastroesophageal reflux
Hypothyroidism
M e n o p a u s a l s y mp t o ms , h o t
flashes
Obstructive sleep apnea
Panic disorders
P a r o xy s ma l n o c t u r n a l
dyspnea
Central nervous system
a b n o r ma l i t i e s ( r a r e )
C. Testing
I n t r i n s i c s l e e p d i s o r d e r s a r e b e s t e v a l u a t e d u s i n g a mu l t i p l e s l e e p l a t e n c y t e s t
( M S LT ) a n d / o r a n o v e r n i g h t p o l y s o mn o1,3)
g r a. pAh s( l e e p l a t e n c y o f < 5 mi n u t e s i s
c o n s i d e r e d p a t h o l o g i c s l e e p i 1,3)
n e s .s T( h e p o l y s o mn o g r a m c a n d e t e c t e xc e s s i v e
l i mb mo v e me n t , s l e e p d i s o r d e r e d b r e a t h i n g , a p n e a , a n1)
d . h y p o xi a (
I V. Diagnosis
T h e d i f f e r e n t i a l d i a g n o s i s f o r h y p e r s o mn i a i sT al ibs ltee d2 .i 8n. 2
H y p e r s o mn i a ma y p r e s e n t w i t h s i g n i f i c a n t f a t i g u e , t r o u b l e w i t h w o r k o r s c h o o l
p e r f o r ma n c e , ma r i t a l o r r e l a t i o n s h i p p r o b l e ms , a c c i d e n t s o r p e r s o n a l i n j u r y
( p a r t i c u l a r l y w i t h n a r c o l e p s y ) , a n d c a n b e t h e f i r s t s i g n o f d e me n t i a o r o t h e r
neurologic disorders.
References
1 . S i l b e r M , K r a h n L , M o r g e n t h aSl el reep
T . medi c i ne i n c l i ni c al pr.ac t i c e
L ondon: Taylor & F rancis, 2004.
2 . R o t h T , K r y g e r M H , D e me n t PWr iCnc
. i pl es and pr ac t i c e of s l eep medi
, c i ne
3 r d e d . P h i l a d e l p h i a , PA : W B S a u n d e r s , 2 0 0 0 .
3 . D o g h r a mj i K . A s s e s s me n t o f e xc e s s i v e s l e e p i n e s s a n d i n s o mn i a a s t h e y r e l a t e
t o c i r c a d i a n r h y t h m s l e e p d i s o Jr dC
e rl isn. P s y c hi at2r y0 0 4 ; 6 5 ( S u p p l 1 6 ) : 1 7 2 2 .
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4 . Q u r e s h i A , L e e - C h i o n g T J r. M e d i c a t i o n s a n d t h e i r e f f e c tM
s ed
o n Cs l iene p .
N or t h A m2 0 0 4 ; 8 8 ( 3 ) : 7 5 1 7 6 6 .
74 / 652
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2.9 - Insomnia
> T a b l e o f C o n t e n t s > 2 - U n d i f f e r e n t i a t e d P r o b l e ms > 2 . 9 - I n s o mn i a
2.9
Insomnia
I . Background
I n s o mn i a i s d e f i n e d aper
s as i s t ent di f f i c ul t y i ni t i at i ng and/ or mai nt ai ni(1)
ng. s l eep
I n s o mn i a c a n b e a p r i ma r y s y n d r o me ; h o w e v e r, i n s o mn i a i s mo r e c o mmo n l y a
s e c o n d a r y s y mp t o m t o u n d e r l y i n g e n v i r o n me n t a l , me d i c a l , o r p s y c h i a t r i c d i s o r d e r s .
A. Etiology
I n s o mn i a i s c a u s e d b y i n t r i n s i c d i s o r d e r s o f t h e s l e e p - w a k e c y c l e a n d e xt r i n s i c
f a c t o r s a f f e c t i n g t h e q u a l i t y o r t h e t i mi n g o f s l e e p . D e p r e s s i o n , a n xi e t y, a n d t r a u ma
c a n t r i g g e r i n s o mn i a , a n d o v e r t h e y e a r s a p e r p e t u a l c y c l e o f p o o r s l e e p c a n
c o n t i n u e e v e n a f t e r t h e t r i g g e r i s t r e a t e d o r r e mo v e d ( p s y c h o p h y s i o l o g i c a l
i n s o mn i a2)
) ( . P o o r s l e e p h y g i e n e , a d i s r u p t i v e s l e e p e n v i r o n me n t , a l c o h o l d e p e n d e n c e , a n d c h r o n i c u s e o f h y p n o t i c s c a n a l s o c a u s e2) .i nCs oi rmn
c a di ai a(n
r h y t h m d i s t u r b a n c e s ( mi s a l i g n me n t o f s l e e p p a t t e r n s w i t h1)l occaanl pt irme
e s )e(n t
w i t h t h e p r i ma r y s y mp t o m o f i n s o mn i a . F i n a l l y, ma n y me d i c a l a n d p s y c h i a t r i c
d i s o r d e r s c a n c a u s e o r b e a s s o c i a t e d w i t h i n s o mn i a .
B. Epidemiology
A s ma n y a s 3 3 % o f t h e g e n e r a l a d u l t p o p u l a t i o n r e p o r t s y mp t o ms o f i n s o mn i a , a n d
b e t w e e n 9 % a n d 2 1 % r e p o r t i n s o mn i a w i t h s e r i o u s d a y t i me c o n3)s .e q u e n c e s (
I n s o mn i a i s mo r e p r e v a l e n t i n w o me n a n d i n c r e a s e s i n p r e v a l e n c e w i t h i n c r e a s i n g
a g e 4( ) .
I I I . Evaluation
A. History
A t h o r o u g h h i s t o r y s h o u l d f o c u s o n d e t e r mi n i n g t h e c a u s e a n d d u r a t i o n o f i n s o mn i a .
P r e c i p i t a t i n g e v e n t s s u c h a s e mo t i o n a l t r a u ma , i l l n e s s , s t r e s s , a n d p r e s c r i p t i o n o r
o t h e r d r u g u s e s h o u l d b e e xp l o r e d w i t h t h e p a t i e n t a n d h i s o r h e r b e d p a r t n e r.
1. S l e e p p a t t e r nAs t h o r o u g h d i s c u s s i o n o f t h e p a t i e n t ' s s l e e p p a t t e r n a n d t h e
s l e e p p a t t e r n s o f o t h e r me mb e r s o f t h e i r h o u s e h o l d s h o u l d i n c l u d e t h e t i mi n g
a n d t h e c o n t e n t o f e v e n i n g me a l s , b e d r o o m e n v i r o n me n t ( t e mp e r a t u r e , n o i s e ,
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c o mf o r t o f b e d ) , w o r k s c h e d u l e s , a n d s l e e p s c h e d u l e s ( i n c l u d i n g d a y t i me
napping).
2. R e v i e w o f s y s t e mTsh e r e v i e w o f s y s t e ms s h o u l d p a y s p e c i a l a t t e n t i o n t o
c o mmo n me d i c a l a n d p s y c h o l o g i c p r o b l e ms t h a t a r e a s s o c i a t e d w i t h i n s o mn i a .
3. M e d i c a t i o nTsa b l e 2 . 9 . l1i s t s me d i c a t i o n s t h a t c a n c a u s e i n s o mn i a .
T h e p h y s i c a l e xa mi n a t i o n s h o u l d f o c u s o n a s s o c i a t e d me d i c a l c o n d i t i o n s a s s o c i a t e d
w i t h i n s o mn i a . I n p r i ma r y i n s o mn i a , t h e p h y s i c a l e xa mi n a t i o n i s l i k e l y t o b e n o r ma l .
C. Testing
L a b o r a t o r y a n d d i a g n o s t i c t e s t i n g i s i n f r e q u e n t l y u s e f u l i n t h e d i a g n o s i s o f p r i ma r y
i n s o mn i a . A s k i n g t h e p a t i e n t t o k e e p a 7 t o 1 4 d a y s l e e p d i a r y ma y h e l p i n
d e t e r mi n i n g e xt r i n s i c f a c t o r s c a u s i n g i n4,5)
s o mn
. Pi ao l (y s o mn o g r a p h y c a n a s s i s t i n
t h e d i a g n o s i s o f s o me s l e e p d i s o r d e r s , i n c l u d i n g s l e e p a p n e a , r e s t l e s s l e g s y n d r o m
a n d p e r i o d i c l i mb mo v e me n t d i s o r d e r.
D. Genetics
F a t a l f a mi l i a l i n s o mn i a i s a r a r e p r i o n d i s e a s e t h a t t e n d s t o r u n i n f a mi l i e s . I n
g e n e r a l , i n s o mn i a i s n o t a s s u me d t o b e g e n e t i c a l l y2)i n. h e r i t e d (
I V. Diagnosis
(see Table 2.9.2)
T h e d i f f e r e n t i a l d i a g n o s i s o f i n s o mn i a i n c l u d e s p r i ma r y s l e e p d i s o r d e r s a s w e l l a s
me d i c a l , p s y c h i a t r i c , a n d e n v i r o n me n t a l d i s t u r b a n c e s .
I n s o mn i a c a n c a u s e s i g n i f i c a n t f u n c t i o n a l i mp a i r me n t , d i f f i c u l t y i n w o r k o r s c h o o l , a
ma r i t a l o r r e l a t i o n s h i p p r o b l e ms . C h r o n i c s l e e p l o s s f r o m u n t r e a t e d i n s o mn i a c a n
cause fatigue-related accidents at work or while driving, job loss, decreased
p r o d u c t i v i t y, ma j o r d e p r e s s i o n , a n d s u i c i d a l
P. 3 2
P. 3 3
i d e a t i o n3)( . I n d i v i d u a l s w i t h i n s o mn i a ma y p r e s e n t w i t h g e n e r a l i ze d f a t i g u e o r
d a y t i me s l e e p i n e s s , a n d a c a r e f u l h i s t o r y c a n h e l p e l u c i d a t e p o s s i b l e u n d e r l y i n g
p r o b l e ms o f i n s o mn i a .
TAB L E 2.9.1 Drugs that Cause I nsom nia

Pre scription drugs
Nonpre scription drugs
Methylphenidate
Caffeine
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T heophylline
S t i mu l a n t d i e t p i l l s
Albuterol
Nicotine
Quinidine
Illicit drugs (e.g., cocaine,

a mp h e t a mi n e s )
P e mo l i n e
D e xt r o a mp h e t a mi n e
Pseudoephedrine
Phenylephrine
P h e n y l p r o p a n o l a mi n e
Selective serotonin reuptake

inhibitors
M o d i f i e d f r o m E d d y M , Wa l b r o e h l G S . I n A
s ommn
F iam
a . P hy s i c i an
1 9 9 9 ; 5 9 ( 7 ) : 1 9 11 1 9 1 6 , 1 9 1 8 .
TAB L E 2.9.2 Differential Diagnosis of I nsom nia

Prim ary insom nia
Ex trinsic sle e p
P s y c h o p h y s i o l o g i c i n s o mn i a
disorde rs
Sle e p re late d disorde re d bre athing
E n v i r o n me n t a l s l e e p
Sleep apnea
disorder (adverse
Loud snoring
s l e e p e n v i r o n me n t )
Hypnosia
Alcohol-dependent
A s t h ma
i n s o mn i a
Circadian rhy thm sle e p disorde rs
Hypnotic-dependent
Jet lag type
i n s o mn i a
D e l a y e d s l e e p p h a s e t y p e ( mo r e c o mmo n i n I l l i c i t d r u g u s e
a d o l e s c e n t s a n d y o u n g a d u l t s , u s u a l l y v e r y l a tAesbseodc i a t e d m e d i c a l
t i me s a n d l a t e o r mi d d a y w a k e t i me s )
and psy chiatric
A d v a n c e d s l e e p p h a s e t y p e ( mo r e c o mmo n i n oclodne dr i t i o n s
a d u l t s , e a r l y b e d t i me a n d e a r l y w a k e t i me )
A l zh e i me r ' s a n d o t h e r
Shift work type
d e me n t i a
Env ironm e ntal disruptions
A n xi e t y d i s o r d e r s
Loud noises
A s t h ma
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Bed partner with sleep disorder

Chronic obstructive
U n c o mf o r t a b l e o r u n s a f e s l e e p i n g c o n d i t i o n s p u l mo n a r y d i s e a s e
Depressive disorders
Gastroesophageal
reflux
Hyperthyroidism
Mania
Nocturia
Restless leg
s y n d r o me
Pain, acute, or
chronic
P e r i o d i c l i mb
mo v e me n t d i s o r d e r
M o d i f i e d f r o m E d d y M , Wa l b r o e h l G S . I n A
s ommn
F iam
a . P hy s i c i an
1 9 9 9 ; 5 9 ( 7 ) : 1 9 11 1 9 1 6 , 1 9 1 8 a n d S i l b e r M , K r a h n L , M o r gSe lneep
thaler T.
medi c i ne i n c l i ni c al pr acLtoi cned o n : T a y l o r & F r a n c i s ; 2 0 0 4 .
References
1 . E s p i e C A , M o r i n CIM
ns. omni a a c l i ni c al gui de t o as s es s ment and t. r eat ment
2003.
2 . S i l b e r M , K r a h n L , M o r g e n t h aSl el reep
T . medi c i ne i n c l i ni c al pr ac t i c e
L ondon: Taylor & F rancis, 2004.
3 . S c h e n c k C H , M a h o w a l d M W, S a c k R L . A s s e s s me n t a n d ma n a g e me n t o f
i n s o mn i aJ. A M A 2 0 0 3 ; 2 8 9 ( 1 9 ) : 2 4 7 5 2 4 7 9 .
4 . N a t i o n a l H e a r t , L u n g , a n d B l o o d I n s t i t u t e Wo r k i n g G r o u p o n I n s o mn i a .
I n s o mn i a : a s s e s s me n t a n d ma n a g e me n t i n p r i ma
A rmy F
c aam
r e . P hy s i c i an
1 9 9 9 ; 5 9 ( 11 ) : 3 0 2 9 3 0 3 8 .
5 . E d d y M , Wa l b r o e h l G S . I n s o mn
A mi aF. am P hy s i c i an
1 9 9 9 ; 5 9 ( 7 ) : 1 9 11
1916,1918.
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2.10 - Nausea and Vomiting
> T a b l e o f C o n t e n t s > 2 - U n d i f f e r e n t i a t e d P r o b l e ms > 2 . 1 0 - N a u s e a a n d Vo mi t i n g
2.10
Nausea and Vomiting
I . Background
Nausea is a vague, intensely disagreeable sensation of sickness or queasiness
t h a t ma y o r ma y n o t b e f o l l o w e d b y v o mi t i n g a n d i s d i s t i n g u i s h e d f r1)
om
. a n o r e xi a (
Vo mi t i n g i s t h e a c t i v e a n d f o r c e f u l e xp u l s i o n o f g a s t r i c c o n t e n t s .
A. Etiology
N a u s e a i s a s u b j e c t i v e s y mp t o m e xp e r i e n c e d i n ma n y o f t h e d i s o r d e r s t h a t a l s o
c a u s e v o mi t i n g . Vo mi t i n g c a n r e s u l t f r o m t h e s t i mu l a t i o n o f o n e o f f o u r n e u r o l o g i c
p r o c e s s e s : ( a ) v a g a l a n d s p l a n c h n i c f i b e r s i n t h e v i s c e r a , s t i mu l a t e d b y d i s t e n t i o n ,
i n f l a mma t o r y i r r i t a t i o n , o r i n f e c t i o n , ( b ) v e s t i b u l a r s y s t e m f i b e r s me d i a t e d t h r o u g h
mu s c a r i n i c c h o l i n e r g i c a n d h i s t a 1mirneec eHp t o r s , ( c ) h i g h e r c e n t r a l n e r v o u s s y s t e m
( C N S ) c e n t e r s w h e r e s i g h t s , s o u n d s , o r e mo t i o n s c a n t r i g g e r v o mi t i n g , a n d ( d ) a
c h e mo r e c e p t o r t r i g g e r zo n e w i t h i n t h e b r a i n t h a t i s r i c h i n o p i o i d , s e r o t o n e r g i c , a n d
d o p a mi n e r e c e p t o r s t r i g g e r e d b y t o xi n s , h y p o xi a , a c i d o s i s , r a d i a t i o n t h e r a p y, u r e mi a
a n d c h e mo t h e r a p1)y . (
B. Epidemiology
N a u s e a a n d v o mi t i n g a r e c o mmo n s y mp t o ms a n d f r e q u e n t l y s e e n i n o u t p a t i e n t ,
i n p a t i e n t , a n d e me r g e n c y s e t t i n g s .
I I I . Evaluation
A. History
A t h o r o u g h h i s t o r y s h o u l d d i s c u s s s l e e p h a b i t s , t h e o n s e t a n d f r e q u e n c y o f v o mi t i n g
t h e s y mp t o ms o f o t h e r f a mi l y me mb e r s , a n d t h e r e l a t i o n s h i p o f n a u s e a a n d v o mi t i n g
t o me a l s o r t y p e s o f f o o d .
1. A r e v i e w o f s y s t e ms s h o u l d s p e c i f i c a l l y a d d r e s s a s s o c i a t e d a n o r e xi a , w e i g h t
l o s s , a b d o mi n a l p a i n , g a s t r o i n t e s t i n a l ( G I ) s y mp t o ms , a n d n e u r o l o g i c s y mp t o ms .
Vo mi t i n g p r i o r t o b r e a k f a s t i s mo r e c o mmo n i n p r e g n a n c y, a l c o h o l o v e r u s e ,
u r e mi a , a n d i n c r e a s e d i n t r a c r a n i a l p r 1)
e s. s Vo
u r emi(t i n g o f u n d i g e s t e d f o o d o n e
o r mo r e h o u r s a f t e r me a l s s h o u l d r a i s e c o n c e r n f o r g a s t r i c o u t l e t o b s t r u c t i o n o r
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g a s t r o p a r e s i s , w h e r e a s v o mi t i n g i mme d i a t e l y a f t e r a me a l i s mo r e c o mmo n w i t h
p s y c h o g e n i c v o mi t i n g a n d b u 1)
l i mi
. a (
2. M a n y me d i c a t i o n s c a n i n d u c e n a u s e a a n d / o r v o mi t i n g , i n c l u d i n g n o n s t e r o i d a l
a n t i - i n f l a mma t o r y d r u g s , o p i a t e s , a n t i b i o t i c s , c a l c i u m c h a n n e l b l o c k e r s ,
a n t i c o n v u l s a n t s , a n t i p a r k i n s o n i a n a g e n t s , n i c o t i n e , - b l o c k e r s , d i g o xi n ,
a n t i a r r h y t h mi c s , a l c o h o l , a n d c h e mo t h e r a p e u t i 1,2)
c a g. e n t s (
T h e p h y s i c a l e xa mi n a t i o n s h o u l d e v a l u a t e f o r a c u t e d e h y d r a t i o n a n d s i g n s o f
i n f e c t i o n . T h e a b d o mi n a l e xa m s h o u l d a t t e mp t t o l o c a l i ze p a i n
P. 3 4
and evaluate for peptic ulcer disease, gallbladder disease, liver disease, or an acu
a b d o me n .
C. Testing
I n s e v e r e a c u t e o r p e r s i s t e n t v o mi t i n g , a f l a t a n d u p r i g h t a b d o mi n a l r a d i o g r a p h c a n
h e l p r u l e o u t G I o b s t r u c t i o n o r a p e r f o r a t e d1)v .i sBc ao rui su m
( r a d i o g r a p h y, a n
u p p e r G I s e r i e s , o r a b d o mi n a l c o mp u t e d t o mo g r a p h y ma y b e h e l p f u l i n t h e d i a g n o s i s
o f g a s t r i c o u t l e t o b s t r u c t i o n o r g a s t r o 1)
p a. r eCsoi ns c(e r n s f o r i n t r a c r a n i a l l e s i o n s
s h o u l d p r o mp t c o mp u t e d t o mo g r a p h y o r ma g n e t i c r e s o n a n c e i ma g i n g o f t h e b r a i n .
B a s e d o n t h e c l i n i c a l a s s e s s me n t , b l o o d t e s t s , l i v e r f u n c t i o n , a my l a s e , p r e g n a n c y
t e s t i n g , h e p a t i t i s t e s t i n g , o r a me t a b o l i c p r o f i l e ma y b e a p p r o p r i a t e . F o r a c u t e
g a s t r o e n t e r i t i s t h a t i s n o t c o mp l i c a t e d b y d e h y d r a t i o n , l a b o r a t o r y t e s t s a r e n o t
n e c e s s a r y.
I V. Diagnosis
T h e d i a g n o s i s o f a c u t e n a u s e a a n d v o mi t i n g c a n o f t e n b e ma d e o n c l i n i c a l h i s t o r y
a l o n e 3)
( . C h r o n i c s y mp t o ms ma y b e mo r e d i f f i c u l t t o d i a g n o s e a n d ma y r e q u i r e
l a b o r a t o r y a n d d i a g n o s t i c t eT
s tai bn lge. 2 . 1 0 .l1i s t s t h e d i f f e r e n t i a l d i a g n o s i s o f
n a u s e a a n d v o mi t i n g .
M o s t p a t i e n t s w i t h n a u s e a a n d v o mi t i n g d o n o t s e e k me d i c a l c o n s u l t a t i o n a n d t h e i r
d i s e a s e i s s e l f - l i mi t 2)
i n.g P( e r s i s t e n t o r s e v e r e n a u s e a a n d v o mi t i n g c a n l e a d t o
s e v e r e w e i g h t l o s s , h y p o k a l e mi a a n d o t h e r e l e c t r o l y t e d i s t u r b a n c e s , d e h y d r a t i o n ,
a n d me t a b o l i c a l k a l o1,3)
s i s .(
TAB L E 2.10.1 Differential Diagnosis of Nausea and

Vom iting
I nfe ctions
Vi r a l : s e l f - l i mi t i n g
Dy sm otility
Gastroparesis:
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B a c t e r i a lS: t aphy l oc oc c us aur,eus

E s c her i c hi a c ol i , d i a b e t i c , p o s t v i r a l ,
B ac i l l us c er eus
, C l os t r i di um per f r i ngens , and
p o s t v a g o t o my
C ampy l obac t er, H el i c obac t er, S hi gel l a, S al monel
S mal a,
ll intestine:
Vi br i oo r g a n i s ms
s c l e r o d e r ma ,
Hepatitis A or B
a my l o i d o s i s
Meningitis, encephalitis
Ve stibular and
Peritonitis: spontaneous bacterial peritonitis, ce ntral ne rv ous
appendicitis, perforated viscus
sy ste m disorde rs
Pyelonephritis
Labyrinthitis
Gastrointe stinal irritation
M n i r e ' s s y n d r o me
Migraine headache
Alcohol
Motion sickness
Gastroesophageal reflux disease
T u mo r s : c a u s i n g
Esophagitis
increased IC P
Obstruction
Intracranial
G a s t r i c o u t l e t o b s t r u c t i o n : ma l i g n a n c y, g a s t r i c h e mo r r h a g e
v o l v u l u s , p e p t i c u l c e r d i s e a s e , p y l o r i c s t e n o s i s( i n c r e a s e d I C P )
(infants)
Othe r
S ma l l i n t e s t i n e o b s t r u c t i o n : a d h e s i o n s , v o l v u l uAs c, u t e p a n c r e a t i t i s
Crohn disease, hernia
Adrenocortical crisis
C o l o n o b s t r u c t i o n : t u mo r s , c o n s t i p a t i o n , o b s t i pBa ut iloi mi
n a
Cholecystitis,
choledocholelithiasis
Diabetic
ketoacidosis
Nephrolithiasis
Pancreatitis
Psychogenic
v o mi t i n g
Pregnancy
U r e mi a
Medications
C h e mo t h e r a p y,
radiation therapy
Postoperative
n a u s e a a n d v o mi t i n g
I C P, i n t r a c r a n i a l p r e s s u r e .
M o d i f i e d f r o m M c Q u a i d K . A l i me n t a r y t r a c t . I n : T i e r n e y L J , M c P h e e S ,
P a p a d a k i s M , e dCsur
. r ent medi c al di agnos i s & t r eat ment
. N2005
e w Yo r k ,
N Y: M c G raw- H ill; 2005.
P. 3 5
References
1 . M c Q u a i d K . A l i me n t a r y T r a c t . I n : T i e r n e y L J , M c P h e e S , P a p a d a k i s M , e d s .
81 / 652
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C ur r ent medi c al di agnos i s & t r eat ment

. N2005
e w Yo r k , N Y : M c G r a w - H i l l , 2 0 0 5 .
2 . S p i l l e r R C . A B C o f t h e u p p e r g a s t r o i n t e s t i n a l t r a c t : a n o r e xi a , n a u s e a ,
v o mi t i n g , a n d p aBi nr . M ed J2 0 0 1 ; 3 2 3 ( 7 3 2 5 ) : 1 3 5 4 1 3 5 7 .
3 . A me r i c a n G a s t r o e n t e r o l o g i c a l A s s o c i a t i o n . M e d i c a l p o s i t i o n s t a t e me n t : n a u s e a
a n d v o mi t i n gG. as t r oent er ol ogy
2001;120(1):261263.
82 / 652
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2.11 - Night Sweats
> T a b l e o f C o n t e n t s > 2 - U n d i f f e r e n t i a t e d P r o b l e ms > 2 . 11 - N i g h t S w e a t s
2.11
Night Sweats
Richard H. Hurd
I . Background
N i g h t s w e a t s a r e d r e n c h i n g s w e a t s t h a t r e q u i r e a c h a n g e 1)
o f. b e d d i n g (
A. Etiology
Night sweats that are caused by fever represent a separate entity and are discussed
i n C h a p t e r 2 . 6N i g h t s w e a t s a r e l i k e l y t o b e a n a u t o n o mi c r e s p o n s e t o s o me
p h y s i c a l o r e mo t i o n a l c o n d i t i o n , r e p r e s e n t i n g a r a t h e r n o n s p e c i f i c s y mp t o m t h a t
s h o u l d p r o mp t t h e c l i n i c i a n t o s e e k a s p e c i f i c c a u s e .
B. Epidemiology
O n e s t u d y o f a p p r o xi ma t e l y 8 0 0 p a t i e n t s i n t h e p r a c t i c e s o f s e v e r a l p r i ma r y c a r e
p h y s i c i a n s i n d i c a t e d t h a t a b o u t 1 0 % w e r e b o t h e r e d t o s o me d e g r e e b y n i g h t s w e a t s .
S e v e n t y p e r c e n t o f p a t i e n t s a f f e c t e d r e p o r t e d s o me t r o u b l e , 2 0 % a f a i r a mo u n t o f
t r o u b l e , a n d 1 0 % a g r e a t d e a l o f t r2)o .u b l e (
I I I . Evaluation
A. History
1. I t i s h e l p f u l t o c h a r a c t e r i ze n i g h t s w e a t s b y d e t e r mi n i n g t h e o n s e t , f r e q u e n c y,
e xa c e r b a t i o n s , a n d r e mi s s i o n s o f s y mp
3) t. oTms
h e ( c l i n i c i a n mu s t t h o r o u g h l y
e xp l o r e a n y r i s k o f e xp o s u r e t o i n f e c t i o u s c a u s e s o f n i g h t s w e a t s . T h i s h i s t o r y
mu s t i n c l u d e q u e s t i o n s a b o u t b e h a v i o r s t h a t p u t t h e p a t i e n t a t r i s k o f h u ma n
i mmu n o d e f i c i e n c y v i r u s ( H I V ) - r e l a t e d i n f e c t i o n s , s e xu a l l y t r a n s mi t t e d d i s e a s e s ,
h e p a t i t i s , a n d t u b e r c u l o s i s . E xp l o r a t i o n s o f t r a v e l a n d o c c u p a t i o n a l e xp o s u r e s
should be carried out.
2. T h e s t a t e o f c o n c u r r e n t me d i c a l c o n d i t i o n s mu s t b e d e t e r mi n e d . C h a n g e s i n
t r e a t me n t mo d a l i t i e s t h a t a r e b o t h p h y s i c i a n d r i v e n a s w e l l a s t h o s e i n i t i a t e d b y
t h e p a t i e n t ma y b e a c a u s a t i v e f a c t o r f o r n i g h t s w e a t s . A l l t h e p a t i e n t ' s c u r r e n t
a n d r e c e n t l y d i s c o n t i n u e d me d i c a t i o n s mu s t b e d e t e r mi n e d .
3. A t h o r o u g h r e v i e w o f t h e s y s t e ms ma y p o i n t t h e c l i n i c i a n t o w a r d a p r i ma r y
d i s e a s e t h a t i n c l u d e s n i g h t s w e a t s a s a s y mp t o m. P s y c h o l o g i c f a c t o r s t h a t mi g h t
precipitate night sweats in healthy individuals should be discussed.
4. I n t e r v i e w i n g t h e s l e e p i n g p a r t n e r i s a n i mp o r t a n t s o u r c e o f i n f o r ma t i o n t h a t mu s t
n o t b e n e g l e c t e d . A h i s t o r y o f s n o r i n g o r a p n e i c e p i s o d e s ma y p o i n t t o s l e e p
a p n e a t h a t h a s b e e n r e p o r t e d t o b e a c o mmo n c a u s e o f n i 4)
g h. t s w e a t s (
T he fact that night sweats in the absence of any other identifiable cause constitute a
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2.11 - Night Sweats
p h y s i c a l l y b e n i g n c o n d i t i o n ma k e s i t i mp e r a t i v e t h a t a c o mp l e t e p h y s i c a l e xa mi n a t i o n
b e c a r r i e d o u t i n a n e f f o r t t o d e t e r mi n e t h e p r e s e n c e o f a n y c o n c u r r e n t c o n d i t i o n .
T h e h i s t o r y ma y d i r e c t t h e p h y s i c i a n t o w a r d a mo r e d e t a i l e d e xa mi n a t i o n o f a
s p e c i f i c o r g a n s y s t e m; h o w e v e r, t h e k n o w l e d g e t h a t t h e c a u s e ma y b e mu l t i f a c t o r i a l
mu s t b e r e me mb e r e d , a n d n o p a r t o f t h e p h y s i c a l e xa mi n a t i o n s h o u l d b e n e g l e c t e d .
C. Testing
T h e c h o i c e o f l a b o r a t o r y t e s t s i s g u i d e d b y t h e h i s t o r y. F o r t h o s e p a t i e n t s w i t h
k n o w n me d i c a l c o n d i t i o n s , a p p r o p r i a t e t e s t s f o r e xa c e r b a t i o n s s h o u l d b e
P. 3 6
c a r r i e d o u t . T h e t e s t s mi g h t i n c l u d e a c o mp l e t e b l o o d c o u n t t o a s s e s s t h e s t a t u s o f
t h e i n f e c t i o n , e r y t h r o c y t e s e d i me n t a t i o n r aI ct,ea, nHdb aAC - r e a c t i v e p r o t e i n . A
h i s t o r y o f e xp o s u r e mi g h t s u g g e s t a n H I V t e s t , a h e p a t i t i s p a n e l , o r a p u r i f i e d p r o t e i n
derivative skin test for tuberculosis. T hyroid function testing in individuals identified
a s a t r i s k s h o u l d a l s o b e c a r r i e d o u t . S p e c i a l t e s t i n g ma y b e r e q u i r e d i n i n d i v i d u a l s
w i t h t r a v e l - r e l a t e d e xp o s u r e .
TAB L E 2.11.1 Causes of Night Sweats

Malignancy
I nfe ctious
Endocrine
R h e u m a t o l o g i cD r u g s
Othe r
L y mp h o ma
H u ma n
H y p e r t h y r o i d i s m R h e u ma t o i d
i mmu n o d e f i c i e n c y
arthritis
virus
Sleep apnea
L e u k e mi a
Tuberculosis
Ovarian failure Lupus
Gastroesophageal
reflux disease
Diabetes
me l l i t u s
Juvenile
r h e u ma t o i d
arthritis
Angina
Endocrine
t u mo r s
T e mp o r a l
arteritis
A n xi e t y
Pregnancy
Overbundling
E xt r e me h e a t
Other
Endocarditis
ma l i g n a n c y
Lung infections
Other infection
D. Genetics
T h e r e i s n o t a r e p o r t e d f a mi l i a l c a u s e o f p r i ma r y n i g h t s w e a t s .
I V. Diagnosis
T h e d i a g n o s i s o f n i g h t s w e a t s i s l a r g e l y h i s t o rTi ac ba l e. S2e. 11
e .. 1
N i g h t s w e a t s a r e mo s t f r e q u e n t l y a ma n i f e s t a t i o n o f a n u n d e r l y i n g i l l n e s s . A s s u c h ,
t h e ma n i f e s t a t i o n i s mo s t o f t e n a p a r t o f a mu c h l a r g e r s y mp t o m c o mp l e x. T h e
d i a g n o s i s c e n t e r s o n d i s c o v e r i n g t h e u n d e r l y i n g d i s e a s e e n t i t y. W h e n n o c a u s e i s
e v i d e n t , w a t c h f u l w a i t i n g i s u s e f u l i n a n a t t e mp t t o l e t t h e c a u s e b e c o me mo r e
84 / 652
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2.11 - Night Sweats
e v i d e n t 5)( .
References
1 . S me t a n a G W. D i a g n o s i s o f n i g h t s w
JA
e aMt sA.1 9 9 3 ; 7 0 : 2 5 0 2 2 5 0 3 .
2 . M o l d J W, R o b e r t s M , A b o s h a d y H . P r e v a l e n c e a n d p r e d i c t o r s o f n i g h t s w e a t s ,
d a y s w e a t s , a n d h o t f l a s h e s i n o l d e r p r i ma r y c a r eA m
p aF
t i am
e n t sM. ed
2004;2(5):391397.
3 . B a j o r e k M a r k . N i g h t s w e a t s , I n : R o b e r t T aTy he
l o r,10e dmi
. nut e di agnos i s
manual. P h i l a d e l p h i a , PA : L i p p i n c o t t W i l l i a ms & W i l k i n s , 2 0 0 0 : 3 1 3 3 .
4 . D u h o n D R . N i g h t s w e a t s : t w o o t h e r cJaAu M
s eAs1. 9 9 4 ; 2 7 1 : 1 5 7 7 .
5 . C h a mb l i s s M L . F r e q u e n t l y a s k e d q u e s t i o n s f r o m c l i n i c a l p r a c t i c e . W h a t i s t h e
a p p r o p r i a t e d i a g n o s t i c a p p r o a c h f o r p a t i e n t s w h o c o mp l a i n o f nAi gr chht s w e a t s ?
F am M ed1 9 9 9 ; 2 : 1 6 8 1 6 9 .
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2.12 - Syncope
> T a b l e o f C o n t e n t s > 2 - U n d i f f e r e n t i a t e d P r o b l e ms > 2 . 1 2 - S y n c o p e
2.12
Syncope
Roge r Massie
I . Background
S y n c o p e i s d e f i n e d at sr ans
a i ent l os s of c ons c i ous w
nes
i t hs a n i n a b i l i t y t o ma i n t a i n
a p o s t u r a l t o n e t h a t i s f o l l o w e d b y s p o n t a n e o u s r e c o v e r y. T h e t e r m s y n c o p e
e xc l u d e s s e i zu r e s , c o ma , s h o c k , o r o t h e r s t a t e s o f a l t e r e d c o n1)s.c i o u s n e s s (
A. Etiology
C a r d i a c c a u s e s i n c l u d e v a s c u l a r d i s e a s e , c a r d i o my o p a t h y, a r r h y t h mi a s , o r v a l v u l a r
dysfunction. Noncardiac causes include vasovagal response to pain, dehydration
with orthostasis, situational syncope, dysfunction, although neurovascular causes
a r e r a r e . A l t e r n a t i v e l y, t h e e t i o l o g y ma y b e u n k n o w n .
B. Epidemiology
S y n c o p e i s a p r e v a l e n t d i s o r d e r, a c c o u n t i n g f o r 1 % t o 3 % o f e me r g e n c y d e p a r t me n t
v i s i t s a n d u p t o 6 % o f h o s p i t a l a d mi s s i o n s e a c h y e a r i n t h e U 1)
n i .t eIdn S
t ht ea t e s (
U n i t e d S t a t e s , t h e d a t a f r o m t h e F r a mi n g h a m s t u d y d e mo n s t r a t e s a f i r s t o c c u r r e n c e
r a t e o f 6 . 2 c a s e s / 1 , 0 0 0 p a t i e n t / y e a r. T h r e e p e r c e n t h a v e r e c u r r e n c e s , a n d
a p p r o xi ma t e l y 1 0 % h a v e a c a r d i a c e t1)
i o.l o g y (
I I I . Evaluation
A t h o r o u g h h i s t o r y a n d p h y s i c a l e xa mi n a t i o n h a v e b e e n s h o w n t o e s t a b l i s h t h e c a u s
in up to 45% of patients. A 12-lead electrocardiogram (E C G) provides another 5% to
1 0 % y i e l d . H o w e v e r, a f t e r t h i s i n i t i a l e xa mi n a t i o n , s y n c o p e r e ma i n s u n e xp l a i n a b l e i n
3 4 % t o 4 7 % o f p a t i e n2)t s. (
A. History
1. A d e t a i l e d a c c o u n t o f t h e e p i s o d e b y t h e p a t i e n t a n d f r o m a n y p e r s o n w i t n e s s i n g
t h e e p i s o d e i s v e r y i mp o r t a n t . S p e c i f i c a l l y, i n f o r ma t i o n a b o u t a c t i v i t y p r i o r t o t h
syncopal episode, including position or change in position, precipitating factors
s u c h a s f a t i g u e , a l c o h o l c o n s u mp t i o n , s t r o n g e mo t i o n s , h u n g e r, a n d a w a r m
e n v i r o n me n t s h o u l d b e e l i c i t e d . A l s o , q u e s t i o n s c o n c e r n i n g p r i o r d i zzi n e s s ,
nausea, diaphoresis, chest pain, dyspnea, visual changes, headache, and focal
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n e u r o l o g i c c h a n g e s s h o u l d b e a s k e d . F i n a l l y, t h e p a t i e n t a n d / o r w i t n e s s s h o u l d
b e a s k e d t o e s t i ma t e t h e t o t a l t i me o f t h e s y n c o p a l e p i s o d e .
2. P a s t me d i c a l h i s t o r y s h o u l d i n c l u d e a c o mp l e t e l i s t o f me d i c a t i o n s , w h e t h e r t h e y
a r e p r e s c r i p t i o n d r u g s , o v e r - t h e - c o u n t e r me d i c a t i o n s , s t r e e t d r u g s , v i t a mi n s ,
a n d / o r h e a l t h s u p p l e me n t s . I n a d d i t i o n , t h e u s u a l i n q u i r y r e l a t i n g t o d i s e a s e
s t a t e s s u c h a s h y p e r t e n s i o n , c o r o n a r y a r t e r y d i s e a s e , d i a b e t e s me l l i t u s , p r i o r
s t r o k e , d e e p v e i n t h r o mb o s i s , a n d a n e mi a s h o u l d b e ma d e . A l s o , i f t h e p a t i e n t i s
a w o ma n o f c h i l d b e a r i n g a g e , t h e p o s s i b i l i t y o f p r e g n a n c y s h o u l d b e d e t e r mi n e d
3. I t i s i mp o r t a n t t o s p e c i f i c a l l y i n q u i r e a b o u t a f a mi l y h i s t o r y o f s u d d e n d e a t h ,
h e a r t d i s e a s e , a n d d i a b e t e s me l l i t u s , e s p e c i a l l y i n f i r s t - d e g r e e r e l a t i v e s .
A c o mp l e t e p h y s i c a l e xa mi n a t i o n i s a l w a y s n e c e s s a r y w h e n a p a t i e n t p r e s e n t s w i t h
s y n c o p e . Vi t a l s i g n s i n c l u d i n g me n t a l s t a t u s s h o u l d b e o b t a i n e d . S y n c o p e a s a
p r e s e n t i n g c o mp l a i n t a l w a y s n e c e s s i t a t e s a h e a d t o t o e e xa mi n a t i o n ; s p e c i f i c a l l y
l o o k i n g f o r p r e v i o u s o r p r e s e n t t r a u ma , c a r d i a c , p u l mo n a r y, a b d o mi n a l , a n d / o r
n e u r o l o g i c a b n o r ma l i t i e s . A l s o , a r e c t a l e xa mi n a t i o n s h o u l d b e p e r f o r me d a l o n g w i t h
a test for occult blood.
C. Testing
I f a s y n c o p a l p a t i e n t p r e s e n t s t o t h e e me r g e n c y d e p a r t me n t , a n i mme d i a t e f i n g e r
stick blood sugar and/or E C G should be obtained, even as vital signs are taken.
T h e n a c o mp l e t e b l o o d c o u n t , c o mp r e h e n s i v e me t a b o l i c p r o f i l e , c a r d i a c e n zy me s ,
a n d a c h e s t x- r a y s h o u l d b e o b t a i n e d . L a t e r t e s t s o r c o n d i t i o n s t o c o n s i d e r i f
i n d i c a t e d b y t h e h i s t o r y a n d t h e p h y s i c a l e xa mi n a t i o n a r e :
1. A t e s t f o r p u l mo n a r y e mb o l u s o r a b d o mi n a l a o r t i c a n e u r y s m.
2. A h e a d - u p t i l t - t a b l e t e s t w h i c h i s u s e f u l f o r c o n f i r mi n g a u t o n o mi c d y s f u n c t i o n
safety and can generally be arranged to be done as an outpatient.
3. A n e l e c t r o e n c e p h a l o g r a m t o b e o b t a i n e d i f a s e i zu r e i s s u s p e c t e d .
P. 3 8
I V. Diagnosis
T h e d i f f e r e n t i a l d i a g n o s i s o f s y n c o p e i s p r e sTeanbtl e d2i.n1 2.. 1
TAB L E 2.12.1 Differential Diagnosis of Syncope

Cardiac
B r a d y d y s r h y t h mi a
C a r d i a c my xo ma
Cardiac outflow obstruction
D y s r h y t h mi a s
Noncardiac
H y p o g l y c e mi a
Orthostasis
D r u g t o xi c i t i e s s t i mu l a n t s ( a mp h e t a mi n e s ,
cocaine)
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2.12 - Syncope
Hypertrophic subaortic
Antidepressants
stenosis
- B lockers
P a r o xy s ma l s u p r a v e n t r i c u l a r C a l c i u m c h a n n e l b l o c k e r s
tachycardia
A n t i d y s r h y t h mi c s
P a r o xy s ma l v e n t r i c u l a r
Adrenal insufficiency and crisis
tachycardia
Va s o mo t o r
P r o l o n g e d Q T s y n d r o me
Dehydration
H y p o v o l e mi a w h e t h e r d u e t o h e mo r r h a g e
Aortic dissections
or other factors
Atrial fibrillation
Carotid sinus syncope
Heart blocks
Cough (post-tussive) syncope
Myocardial infarction
Defecation syncope
P u l mo n a r y e mb o l i s m
Micturition syncope
Hyperventilation
Migraine headache
Narcolepsy
Panic attacks
S e i zu r e d i s o r d e r s
References
1 . H o n g o R H , G o l d s c h l a g eE
r vNal. uat i ng pat i ent s wi t h unex pl ai ned s y nc ope
M E D S C A P E , 11 / 2 0 0 4 .
2 . M o r a g R emedi
.
c i ne. c omS y nc ope ex cJer
u npte 2 0 0 5 .
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2.13 - Weight Loss
> T a b l e o f C o n t e n t s > 2 - U n d i f f e r e n t i a t e d P r o b l e ms > 2 . 1 3 - We i g h t L o s s
2.13
Weight Loss
Am y K. Je spe rse n
I . Background
Unintentional weight loss is generally considered to be significant when greater tha

5 % o f b o d y w e i g h t i s l o s t o v e r a p e r i o d o f 6 mo n t h s o r l e s s . I t i s o f t e n a s s o c i a t e d
w i t h i n c r e a s e d mo r b i d i t y a n d mo r t a l i t y e s p e c i a l l y a mo n g t h e e l d e r l y. P e r c e i v e d
weight loss should be verified before initiating a workup, because 50% of patients
w i t h p e r c e i v e d w e i g h t l o s s d o n o t h a v e t r u e w e i g h t l o s s . O f p a t i e n t s w i t h c o n f i r me d
w e i g h t l o s s , a n e xp l a n a t i o n i s g e n e r a l l y f o u n d i n 7 5 % o f c a s e s . I n 2 5 % o f p a t i e n t s a
e xp l a n a t i o n i s n e v e r f o u n d . I f a p h y s i c a l c a u s e i s p r e s e n t , i t i s u s u a l l y d i s c o v e r e d
w i t h i n 6 mo n t h1,2,3)
s ( .
T he various conditions that cause unintentional weight loss do so through one or
mo r e o f t h e f o l l o w i n g me c h a n i s ms : i n a d e q u a t e c a l o r i c i n t a k e ,
P. 3 9
e xc e s s i v e me t a b o l i c d e ma n d s , o r l o s s o f n u t r i e n t s t h r o u g h u r i n e o r s t o o l . O t h e r
conditions that cause weight loss include:
A. M alignant conditions
C a n c e r i s o f t e n t h e p a t i e n t ' s o r p h y s i c i a n ' s g r e a t e s t f e a r. M a l i g n a n c y i s t h e c a u s e f
u n i n t e n t i o n a l w e i g h t l o s s i n 1 6 % t o 3 6 % o1,2,3)
f c a s.e A
s l t( h o u g h a n y c a n c e r c a n
c a u s e w e i g h t l o s s , t h e mo r e c o mmo n ma l i g n a n c i e s t o c o n s i d e r a r e g a s t r o i n t e s t i n a l
( G I ) , l e u k e mi a o r l y mp h o ma , l u n g , o v a r i a n , a n d p r o s t a t e c a n c e r s .
B. Benign medical conditions
M a n y c h r o n i c me d i c a l c o n d i t i o n s c a n c a u s e a n o r e xi a , n a u s e a , d i a r r h e a , o r
p o s t p r a n d i a l s y mp t o ms w h i c h d i s c o u r a g e t h e p a t i e n t f r o m e a t i n g . M e d i c a l c o n d i t i o n s
ma y a l s o n e c e s s i t a t e l i mi t i n g s a l t , f a t , o r s u g a r i n t h e d i e t , l e a v i n g t h e p a t i e n t l e s s
inclined to eat.
1. G I d i s o r d e r s a c c o u n t f o r t h e mo s t c o mmo n p h y s i c a l c a u s e o f w e i g h t l o s s
a f f e c t i n g a p p r o xi ma t e l y 1 7 % o f p a4)
t i e. nTt hs e(s e i n c l u d e :
a. P e p t i c u l c e r d i s e a s e / g a s t r o e s o p h a g e a l r e f l u x d i s e a s e
b. I n f l a mma t o r y b o w e l d i s e a s e
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2.13 - Weight Loss
c. H e p a t i t i s , c h o l e s t a s i s
d. P a n c r e a t i t i s
e. A t r o p h i c g a s t r i t i s
f. C o n s t i p a t i o n .
2. C a r d i a c d i s e a s e s , e s p e c i a l l y c o n g e s t i v e h e a r t f a i l u r e .
3. R e s p i r a t o r y d i s e a s e s , s u c h a s c h r o n i c o b s t r u c t i v e p u l mo n a r y d i s e a s e .
4. R e n a l d i s e a s e .
5. N e u r o mu s c u l a r d i s o r d e r s ma y a f f e c t t h e a b i l i t y t o s w a l l o w. T h e s e i n c l u d e :
a. S c l e r o d e r ma
b. P o l y my o s i t i s
c. S y s t e mi c l u p u s e r y t h e ma t o s u s .
6. E n d o c r i n e d i s o r d e r s c a n i n c r e a s e me t a b o l i c r a t e o r c a u s e n u t r i e n t l o s s . T h e s e
include:
a. H y p e r t h y r o i d i s m
b. D i a b e t e s me l l i t u s
c. O t h e r c a u s e s , s u c h a s p h e o c h r o mo c y t o ma , p a n h y p o p i t u i t a r i s m, a d r e n a l
i n s u f f i c i e n c y, a n d h y p e r t h y r o i d i s m.
7. I n f e c t i o n , e s p e c i a l l y t u b e r c u l o s i s , f u n g a l d i s e a s e , s u b a c u t e b a c t e r i a l
endocarditis, and any prolonged febrile illness can decrease appetite and
i n c r e a s e me t a b o l i c d e ma n d . H u ma n i mmu n o d e f i c i e n c y v i r u s i n f e c t i o n i s a s p e c i a
c o n s i d e r a t i o n w i t h p a t i e n t s h a v i n g mu l t i p l e c a u s e s f o r w e i g h t l o s s .
8. N e u r o l o g i c c o n d i t i o n s ( e . g . , d e me n t i a , P a r k i n s o n ' s d i s e a s e , s t r o k e ) c a n c a u s e
w e i g h t l o s s s e c o n d a r y t o a p a t h y, d e c r e a s e d a p p e t i t e , o r d i f f i c u l t y s w a l l o w i n g .
9. M e d i c a t i o n s c a n c a u s e a n o r e xi a , n a u s e a , a b d o mi n a l p a i n , o r d i a r r h e a , o r i n h i b i t
g a s t r i c e mp t y i n g .
C. Psychiatric causes
T h e s e a r e r e s p o n s i b l e f o r w e i g h t l o s s i n 1 0 % t o 2 0 % o1,2,3)
f p a t.i e n t s (
1. D e p r e s s i o n i s t h e mo s t c o mmo n p s y c h i a t r i c c a u s e .
2. S u b s t a n c e a b u s e , e s p e c i a l l y a l c o h o l i s m, a n d b e r e a v e me n t a r e o t h e r c a u s e s .
D. Social and age related causes

T hese include the following:
1. F i n a n c i a l h a r d s h i p .
2. D i mi n i s h e d s e n s e o f t a s t e a n d s me l l .
3. F u n c t i o n a l i n a b i l i t y t o s h o p o r p r e p a r e f o o d .
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2.13 - Weight Loss
4. P o o r d e n t i t i o n .
I I I . Evaluation
A. History
A detailed history should be obtained from the patient and caregivers if applicable.
S p e c i a l a t t e n t i o n s h o u l d b e g i v e n t o t h e t y p e s a n d q u a n t i t y o f f o o d c o n s u me d ;
a l c o h o l u s e ; h i s t o r y o f c i g a r e t t e s mo k i n g ( c u r r e n t a n d r e mo t e ) ; e xe r c i s e p a t t e r n s ;
me d i c a t i o n s ; p r e s e n c e o f n a u s e a , v o mi t i n g , d i a r r h e a , e a r l y s a t i e t y, d i f f i c u l t y
s w a l l o w i n g ; h i s t o r y o f G I i l l n e s s e s o r p r e v i o u s a b d o mi n a l s u r g e r y ; c a r d i a c h i s t o r y ;
r e s p i r a t o r y h i s t o r y ; h i s t o r y o f k i d n e y d i s e a s e ; d e p r e s s i v e s y mp t o ms ; s o c i a l s i t u a t i o n
including financial resources; and functional ability to shop for groceries and prepa
me a l s .
P. 4 0
1. D o c u me n t w e i g h t a n d c o mp a r e i t t o p r e v i o u s w e i g h t s .
2. P e r f o r m a t h o r o u g h p h y s i c a l e xa mi n a t i o n , p a y i n g s p e c i a l a t t e n t i o n t o a n o r a l
e xa mi n a t i o n , e s p e c i a l l y d e n t i t i o n ; a r e s p i r a t o r y e xa mi n a t i o n ; a c a r d i a c
e xa mi n a t i o n ; a G I e xa mi n a t i o n ; a p s y c h o l o g i c e xa mi n a t i o n ; a n d a n e v a l u a t i o n o f
c o g n i t i v e f u n c t i o n , e s p e c i a l l y i f t h e p a t i e n t i s e l d e r l y.
C. Testing
E xt e n s i v e u n d i r e c t e d l a b o r a t o r y t e s t i n g i s n o t i n d i c a t e d a n d i s r a r e l y h e l p f u l .
1. I n i t i a l l a b o r a t o r y t e s t s s h o u l d i n c l u d e :
a. C o mp l e t e b l o o d c o u n t
b. C o mp r e h e n s i v e me t a b o l i c p r o f i l e
c. T h y r o i d - s t i mu l a t i n g h o r mo n e
d. U r i n a l y s i s
e. F e c a l o c c u l t b l o o d t e s t i n g .
2. O t h e r l a b o r a t o r y t e s t s a s i n d i c a t e d b y h i s t o r y o r p h y s i c a l e xa mi n a t i o n ma y
include:
a. C h e s t x- r a y, w h i c h ma y b e h e l p f u l , e s p e c i a l l y w i t h a h i s t o r y o f c i g a r e t t e
s mo k i n g , o r a n e w o r d i f f e r e n t c o u g h o r d y s p n e a .
b. A p p r o p r i a t e a g e a n d g e n d e r b a s e d s c r e e n i n g ( e . g . , ma mmo g r a p h y,
colonoscopy).
c. O t h e r t e s t s a s i n d i c a t e d b y h i s t o r y ( e . g . , u p p e r G I e n d o s c o p y ) .
I V. Diagnosis
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D i a g n o s i s o f w e i g h t l o s s i s ma d e b y v e r i f y i n g t h a t a l o s s o f mo r e t h a n 5 % o f b o d y
w e i g h t h a s o c c u r r e d . A t h o r o u g h h i s t o r y a n d p h y s i c a l e xa mi n a t i o n a n d d i r e c t e d
l a b o r a t o r y a n d a n c i l l a r y t e s t s r e s u l t i n a n e xp l a n a t i o n a p p r o xi ma t e l y 7 5 % o f t h e t i me
Malignancy is the cause of weight loss in 16% to 36% of cases. Psychiatric causes,
usually depression, is the cause in 10% to 20% of the cases. An organic cause othe
t h a n ma l i g n a n c y i s p r e s e n t i n 3 0 % t o 5 0 % o f c a s e s . T w e n t y - f i v e p e r c e n t o f t h e t i me
no identifiable cause is found. If a physical cause is responsible, but not identified
o n i n i t i a l w o r k u p , i t u s u a l l y b e c o me s e v i d e n t w i t h i n4)6. mo n t h s (
References
1 . M a r t o n K E , S o x H C J r, K r u p p J R . I n v o l u n t a r y w e i g h t l o s s : d i a g n o s t i c a n d
p r o g n o s t i c s i g n i f i c a nAcnn
e . I nt er n M ed
1981;95:568574.
2. Rabinovitz M, Pitlik S D, L eifer M, et al. Unintentional weight loss. A
r e t r o s p e c t i v e a n a l y s i s o f 1 5 4 cAarscehs .I nt er n M ed
1986;146:186187.
3 . T h o mp s o n M P, M o r r i s L K . U n e xp l a i n e d w e i g h t l o s s i n t h e a mb u l aJ t o r y e l d e r l y.
A m G er i at r S oc
1991;39:497500.
4 . H u f f ma n G B . E v a l u a t i n g a n d t r e a t i n g u n i n t e n t i o n a l w e i g h t l o s s i n t h e e l d e r l y.
2002;65:640650.
5. Wise G R, Craig D. Evaluation of involuntary weight loss. Where do you start?
P os t gr ad M ed 1J9 9 4 ; 9 5 : 1 4 3 1 4 6 , 1 4 9 1 5 0 .
6 . R e i f e C M . I n v o l u n t a r y w e i g h t Ml oed
s s .C l i n N or t h A1m9 9 5 ; 7 9 ( 2 ) : 2 9 9 3 1 3 .
7 . B o u r a s E P, L a n g e S M , S c o l a p i o J S . R a t i o n a l a p p r o a c h t o p a t i e n t s w i t h
uni ntenti onal wei ght lM
o say
s . o C l i n P r oc
2001;76:923929.
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3.1 - Anxiety
> T a b l e o f C o n t e n t s > 3 - M e n t a l H e a l t h P r o b l e ms > 3 . 1 - A n xi e t y
3.1
Anxiety
Lay ne A. Pre st
I . Background
T h e e xp e r i e n c e o f a n xi e t y i s u b i q u i t o u s i n s o c i e t y. A n xi e t y c a n b e p a r t o f a n a d a p t i v
o r p r o t e c t i v e r e s p o n s e t o t h r e a t ( e . g . , t h e f i g h t - f r e e ze - f l i g h t r e s p o n s e ) o r a n a t u r a l
r e a c t i o n t o p h y s i c a l a n d e mo t i o n a l s t r e s s , b u t i t c a n a l s o b e d e b i l i t a t i n g a n d a
s e r i o u s h e a l t h c o n c e r n . A t i t s c o r e , a n xi e t y i s a c o mp l e x b i o - p s y c h o s o c i a l - s p i r i t u a l
e xp e r i e n c e t h a t r e q u i r e s c o mp r e h e n s i v e a s s e s s me n t a n d t r e a t me n t . U n d i a g n o s e d
a n xi e t y d i s o r d e r s c o n t r i b u t e t o i n a p p r o p r i a t e o r o v e r u t i l i za t i o n o f h e a l t h c a r e
r e s o u r c e s , b u t a s ma n y a s 8 0 % o f t h e i n d i v i d u a l s w i t h a n xi e t y d i s o r d e r s c a n b e
s i g n i f i c a n t l y h e l p e d t h r o u g h a p p r o p r i a t e t r e a t me n t .
A. Etiology
M a n y f a c t o r s c o n t r i b u t e t o b o t h t h e d e v e l o p me n t a n d e xp e r i e n c e o f a n xi e t y. T h e s e
i n c l u d e g e n e t i c / n e u r o l o g i c p r e d i s p o s i t i o n , f a mi l y h i s t o r y, a c u t e a n d c h r o n i c s t r e s s o r
r e s o u r c e s f o r c o p i n g , c o mo r b i d c o n d i t i o n s , a n d o v e r a l l p h y s i c a l h e a l t h . E xt r e me
a n xi e t y r e s p o n s e s , k n o wanx
n a si et y di s or der
, asr e o f t e n c o mo r b i d w i t h mo o d
disorders or other chronic health conditions (e.g., coronary artery disease, cancer)
T h e s e d i s o r d e r s u s u a l l y i n c l u d e d e b i l i t a t i n g p h y s i c a l a n d e mo t i o n a l s y mp t o ms a n d
ma y b e d u e , a t l e a s t i n p a r t , t o p r i ma r y me d i c a l p r o b l e ms s u c h a s h y p e r t h y r o i d i s m o
h y p o xi a . C o n s e q u e n t l y, a n xi o u s p a t i e n t s p r e s e n t t o t h e e me r g e n c y r o o m o r p r i ma r y
c a r e s e t t i n g w i t h c o mp l a i n t s t h a t c a n b e d i f f i c u l t t o a s s e s s a n d d i a g n o s e .
B. Epidemiology
A c c o r d i n g t o t h e N a t i o n a l I n s t i t u t e o f M e n t a l H e a l t h ( N I M H ) , 1 9 mi l l i o n A me r i c a n s
e xp e r i e n c e a n a n xi e t y d i s o r d e r a t a n y o n e t i me . E s t i ma t e s o f t h e p r e v a l e n c e o f t h e
v a r i o u s a n xi e t y d i s o r d e r s v a r y f r o m s t u d y t o s t u d y b u t g e n e r a l l y a r e a s f o l l o w s :
g e n e r a l i ze d a n xi e t y d i s o r d e r ( G A D ) 4 mi l l i o n , 2 . 8 % ( w o me n t w i c e a s l i k e l y a s me n )
o b s e s s i v e c o mp u l s i v e d i s o r d e r 3 . 3 mi l l i o n , 2 . 3 % ( e q u a l l y c o mmo n a mo n g me n a n d
w o me n ) ; p a n i c d i s o r d e r 2 . 4 mi l l i o n , 1 . 7 % ( w o me n t w i c e a s l i k e l y ) ; p o s t t r a u ma t i c
s t r e s s d i s o r d e r 5 . 2 mi l l i o n , 3 . 6 % ( w o me n mo r e l i k e l y t h a n me n ) ; s o c i a l a n xi e t y
d i s o r d e r 5 . 3 mi l l i o n , 3 . 7 % ( e q u a l l y c o mmo n a mo n g me n a n d w o me n ) ; a n d s p e c i f i c
p h o b i a 6 . 3 mi l l i o n , 4 . 4 % ( w o me n t w i c e a s l i k e l y a s me n ) . T h e v a r i o u s a n xi e t y
d i s o r d e r s a f f e c t a p p r o xi ma t e l y 1 0 % o f p r i ma r y c a r e1) p. a t i e n t s (
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I I I . Evaluation
A. History
1. P a t i e n t s w i t h a n xi e t y d i s o r d e r s f r e q u e n t l y d e s c r i b e e xp e r i e n c i n g p h y s i c a l
s y mp t o ms s u c h a s c h e s t p a i n , d i zzi n e s s , p a l p i t a t i o n s , f a t i g u e , s h o r t n e s s o f
b r e a t h , s w e a t i n g , mu s c l e a c h e s o r t e n s i o n , o r a v a r i e t y o f g a s t r o i n t e s t i n a l
c o mp l a i n t s . C o mmo n p s y c h o l o g i c s y mp t o ms c a n i n c l u d e s h a k i n e s s ,
n e r v o u s n e s s , f e a r o f d y i n g o r g o i n g c r a zy, o r a s e n s e o f u n r e a l i t y o r d e t a c h me n
from oneself.
2. S o me p a t i e n t s a t t r i b u t e t h e i r a n xi e t y t o t h e i r p h y s i c a l s y mp t o ms ( O f c o u r s e , I
w a s a n xi o u s . I t h o u g h t I w a s h a v i n g a h e a r t a t t a c k ) . C o n s e q u e n t l y, t h e
a s s e s s me n t o f a n xi e t y d i s o r d e r s s h o u l d i n c l u d e t h e n a t u r e , f r e q u e n c y, a n d
d u r a t i o n o f t h e p r e c e d i n g s y mp t o ms a n d t h e e xt e n t t o w h i c h t h e s y mp t o ms h a v e
i mp a c t e d t h e i n d i v i d u a l ' s l i f e a n d a c t i v i t y.
3. T h e p a t i e n t s h o u l d a l s o b e a s k e d a b o u t p r e c i p i t a n t s o f t h e s y mp t o ms , i n c l u d i n g
s t r e s s o r s , me d i c a t i o n s ( e . g . , s t i mu l a n t s ) , a n d o t h e r d r u g u s e ( e . g . , c a f f e i n e ,
cocaine).
4. Q u e s t i o n s a b o u t t h e p a t i e n t ' s g e n e r a l me d i c a l c o n d i t i o n a r e a l s o a p p r o p r i a t e .
As with all patients, those whose clinical picture is suspected of including a
s i g n i f i c a n t c o mp o n e n t o f a n xi e t y s h o u l d b e e xa mi n e d c a r e f u l l y.
P. 4 4
T h e e xt e n t o f t h e p h y s i c a l e xa mi n a t i o n s h o u l d b e d i c t a t e d b y t h e p a t i e n t ' s p e r s o n a l
h e a l t h a n d me d i c a l h i s t o r y.
1. T h e e xa mi n a t i o n ma y i n c l u d e t h e f o l l o w i n g : b l o o d p r e s s u r e ( h y p e r t e n s i o n ,
h y p o v o l e mi a ) , c a r d i o v a s c u l a r ( a n g i n a , a r r h y t h mi a , c o n g e s t i v e h e a r t f a i l u r e ,
v a l v u l a r h e a r t d i s e a s e ) , r e s p i r a t o r y ( c h r o n i c o b s t r u c t i v e l u n g d i s e a s e , p u l mo n a r
e mb o l i s m, p n e u mo n i a ) , a n d n e u r o l o g i c ( t u mo r, e n c e p h a l o p a t h y, v e r t i g o ) .
2. P a t i e n t s f r e q u e n t l y p r e s e n t w i t h n e r v o u s a g i t a t i o n , i n t e r mi t t e n t e y e c o n t a c t ,
s o me w h a t p r e s s u r e d s p e e c h , a n d , i n t h e p r i ma r y c a r e c o n t e xt , a w o r r i e d f o c u s
o n t h e s o ma t i c c o n c e r n s d e s c r i b e d i n t h e p r e c e d i n g t e xt .
C. Testing
U s e f u l l a b o r a t o r y t e s t s i n c l u d e s e r u m c a l c i u m ( h y p o c a l c e mi a ) , h e ma t o c r i t ( a n e mi a ) ,
a n d t h y r o i d - s t i mu l a t i n g h o r mo n e ( h y p e r t h y r o i d i s m/ h y p o t h y r o i d i s m) . D e p e n d i n g o n t h
c l i n i c a l s c e n a r i o , a n e xe r c i s e s t r e s s t e s t t o e v a l u a t e c h e s t p a i n o r o t h e r t e s t s t o r u l
o u t o r g a n i c c a u s e s ( s u c h a s d r u g s c r e e n , o xi me t r y ( h y p o xi a ) , g l u c o s e
( h y p o g l y c e mi a ) , a n d e l e c t r o l y t e s ) ma y b e u s e f u l a s w e l l .
I V. Diagnosis
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3.1 - Anxiety
1. G A D i s c h a r a c t e r i ze d b y p e r s i s t e n t a n d e xc e s s i v e w o r r y a b o u t a n u mb e r o f
i s s u e s o n mo s t d a y s f o r a p e r i o d o f a t l e a s t s i x mo n t h s . G A D u s u a l l y b e g i n s b y
e a r l y a d u l t h o o d , i s e xa c e r b a t e d b y s i t u a t i o n a l s t r e s s o r s , a n d u s u a l l y i n v o l v e s a
c o mb i n a t i o n o f p s y c h o l o g i c a n d p h y s i c a l s y mp t o ms .
2. P a n i c d i s o r d e r, w i t h o r w i t h o u t a g o r a p h o b i a , p r e s e n t s w i t h r e c u r r e n t p a n i c
a t t a c k s d i s c r e t e e p i s o d e s o f a n xi e t y i n v o l v i n g s h o r t n e s s o f b r e a t h , f e a r o f
d y i n g , i mp e n d i n g d o o m o r l o s i n g c o n t r o l , p o u n d i n g h e a r t , s w e a t i n g , c h e s t p a i n ,
p a r e s t h e s i a s , t r e mb l i n g , a n d n a u s e a . T h e p a n i c a t t a c k s ma y b e p r o v o k e d b y
i d e n t i f i a b l e s t r e s s o r s o r s i t u a t i o n s b u t o f t e n s e e m t o c o me o u t o f t h e b l u e .
I n d i v i d u a l s w i t h p a n i c d i s o r d e r ma y b e s o f e a r f u l o f b e i n g i n a s i t u a t i o n i n w h i c h
they have another panic attack and are unable to escape that they develop
agoraphobia (an intense fear of being in open or crowded places, which often
contributes to the individuals being reluctant to leave the perceived safety of
t h e i r h o me ) .
3. A c u t e s t r e s s d i s o r d e r ( A S D ) a n d P o s t t r a u ma t i c s t r e s s d i s o r d e r ( P T S D ) a r e
c h a r a c t e r i ze d b y r e e xp e r i e n c i n g ( t h r o u g h r e c o l l e c t i o n s , f l a s h b a c k s , n i g h t ma r e s )
a n e xt r e me l y t r a u ma t i c a n d p o s s i b l y l i f e - t h r e a t e n i n g e xp e r i e n c e ( e . g . , r a p e ,
mu r d e r, mo t o r - v e h i c l e a c c i d e n t , w a r ) , f o l l o w e d b y h y p e r - a r o u s a l , p a n i c ,
d e p r e s s e d mo o d , s l e e p d i s t u r b a n c e , a n d h y p e r - v i g i l a n c e . T h e i n d i v i d u a l u s u a l l y
a t t e mp t s t o a v o i d t h e s e me mo r i e s o r t h e c h a n c e o f b e i n g i n d a n g e r a g a i n
t h r o u g h n u mb i n g , d i s s o c i a t i o n , r e p r e s s i o n , a n d b e h a v i o r a l c h a n g e s . T h e ma i n
d i s t i n c t i o n b e t w e e n A S D a n d P T S D i s t h e d u r a t i o n o f s y mp t o ms ( i . e . , i n P T S D
s y mp t o ms l a s t l o n g e r t h a n o n e mo n t h ) .
4. S p e c i f i c p h o b i a i s e xc e s s i v e a n xi e t y p r o v o k e d b y e xp o s u r e t o a s p e c i f i c f e a r e d
o b j e c t o r s i t u a t i o n . C o mmo n p h o b i a s i n c l u d e f e a r o f a n i ma l s o r i n s e c t s , n a t u r a l
e n v i r o n me n t ( e . g . , h e i g h t s , s t o r ms , w a t e r ) , b l o o d - i n j e c t i o n - i n j u r y, o r s i t u a t i o n s
(e.g., tunnels, bridges, elevators, flying, driving).
5. S o c i a l p h o b i a i s a n e xc e s s i v e a n xi e t y p r o v o k e d b y e xp o s u r e t o s o c i a l o r
p e r f o r ma n c e s i t u a t i o n s a n d u n f a mi l i a r i n d i v i d u a l s o r s u r r o u n d i n g s . A s a r e s u l t ,
individuals with social phobia avoid these types of situations.
6. O b s e s s i v e - c o mp u l s i v e d i s o r d e r i s c h a r a c t e r i ze d b y o b s e s s i o n s t h a t c a u s e
a n xi e t y ( e . g . , g e r ms o n h a n d s ) a n d c o mp u l s i o n s ( b e h a v i o r s a i me d a t r e d u c i n g
t h e a n xi e t y s u c h a s h a n d - w a s h i n g ) . T h e o b s e s s i o n s u s u a l l y f a l l i n t o o n e o r mo r e
o f t h e f o l l o w i n g c a t e g o r i e s : i n f e c t i o n / c o n t a g i o n , s a f e t y, r e l i g i o s i t y, s e xu a l i t y,
death/dying, orderliness.
7. A d j u s t me n t r e a c t i o n w i t h a n xi o u s f e a t u r e s i s a c o n d i t i o n i n w h i c h a p a t i e n t
e xp e r i e n c e s s i g n i f i c a n t a n xi e t y i n r e a c t i o n t o a s p e c i f i c s t r e s s o r s u c h a s a ma j o
life event or interpersonal conflict. To qualify for this diagnosis, the level of
a n xi e t y s h o u l d b e a s s e s s e d a s b e i n g mo r e t h a n e xp e c t e d u n d e r t h e
c i r c u ms t a n c e s . I n a d d i t i o n t o t h e s e c o n d i t i o n s , t h e c l i n i c i a n s h o u l d i n v e s t i g a t e
t h e p o s s i b i l i t y o f mo o d , s u b s t a n c e a b u s e , a n d o t h e r p s y c h i a t r2)
i c. d i s o r d e r s (
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3.1 - Anxiety
M o s t a n xi o u s p a t i e n t s p r e s e n t i n t h e p r i ma r y c a r e s e t t i n g w i t h a p r i ma r y f o c u s o n
t h e i r b o d i e s a n d s o ma t i c s y mp t o ms r a t h e r t h a n t h e i r
P. 4 5
mi n d s . B u t i n e v i t a b l y t h e r e i s a s i g n i f i c a n t c o mp o n e n t o f w o r r y, f e a r, a p p r e h e n s i o n
a n d s o o n i n t h e b a c k g r o u n d . B e c a u s e a n e xc l u s i v e f o c u s o n p h y s i c a l c o mp l a i n t s
( e . g . , c h e s t p a i n , d i zzi n e s s ) c a n o b s c u r e t h e d i a g n o s i s , i t i s i mp o r t a n t t o a s k p a t i e n
about their psychologic state, living situation, and current stressors, as well as
e v a l u a t e t h e m f o r u n d e r l y i n g me d i c a l i s s u e s .
References
1 . A me r i c a n A n xi e t y D i s o r d e r s A s s o c i a t i o n . S i l v e r S p r i n g , M D . Av a i l a b l e a t :
w w w. a d a a . o r g , a c c e s s e d o n J u l y 2 0 , 2 0 0 5 .
2 . A me r i c a n P s y c h i a t r i c A s s o c i aDt i agnos
on.
t i c and s t at i s t i c al manual of ment al
di s or der, s4 t h e d . Wa s h i n g t o n , D C : A me r i c a n P s y c h i a t r i c A s s o c i a t i o n , 1 9 9 4 .
96 / 652
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3.2 - Depression
> T a b l e o f C o n t e n t s > 3 - M e n t a l H e a l t h P r o b l e ms > 3 . 2 - D e p r e s s i o n
3.2
Depression
W. D a v i d R o b i n s o n
I . Background
A. Definition
D e p r e s s i o n i s a n i l l n e s s t h a t a f f e c t s t h e mi n d , b o d y, mo o d , t h o u g h t s , a n d
r e l a t i o n s h i p s . I t i s n o t j u s t u n h a p p i n e s s b u t a n o v e r w h e l mi n g s e n s e o f s a d n e s s a n d
physical decline that has potential far-reaching deleterious effects.
B. Cost
Depression costs the United States billions of dollars annually in lost productivity
a n d d i r e c t me d i c a l c o s t s . H e a l t h s e r v i c e c o s t s a r e 5 0 % t o 1 0 0 % g r e a t e r f o r
d e p r e s s e d p a t i e n t s c o mp a r e d t o p a t i e n t s w i t h o u t d e p r e s s i o n . T h e s e i n c r e a s e d c o s t s
a r e d u e t o h i g h e r me d i c a l u t i l i za t i o n a n d n o t d u e t o s p e c i a l i t y me n t 1)
a l. h e a l t h c a r e (
D e p r e s s i o n a l s o c o n t r i b u t e s t o i mp a i r e d c o n c e n t r a t i o n , f a i l u r e t o a d v a n c e i n
e d u c a t i o n a n d v o c a t i o n a l e n d e a v o r s , i n c r e a s e d s u b s t a n c e a b u s e , i mp a i r e d o r l o s t
r e l a t i o n s h i p s , a n d i n c r e a s e d r i s k o f s2)u.i c i d e (
A. Etiology
T h e b i o p s y c h o s o c i a l mo d e l i s a n e f f e c t i v e w a y t o c o n c e p t u a l i ze t h e e t i o l o g y o f
a n xi e t y a n d d e p r e s s i o n b e c a u s e t h e f a c t o r s t h a t c r e a t e a n xi e t y a n d d e p r e s s i o n a r e
varied. T he interplay between the biologic, psychological, and social aspects of the
p a r t i c u l a r p a t i e n t s h o u l d b e a s s e s s e d t o d e t e r mi n e t h e e t i o l o g y o f t h e d i s e a s e .
1. B i o l o g i cT h e r e i s a mp l e i n f o r ma t i o n s u g g e s t i n g t h a t g e n e t i c s p l a y s a r o l e i n t h e
d e v e l o p me n t o f mo o d d i s o r d e r s . S t u d i e s r e l a t e d t o t w i n s h a v e s h o w n t h a t t h e
r a t e o f mo o d d i s o r d e r s i n i d e n t i c a l t w i n s i s 6 7 % t o 7 6 % b u t o n l y 1 9 % i n f r a t e r n a
t w i n s 3)
( . Wo me n a r e a t l e a s t t w i c e a s l i k e l y t o s u f f e r f r o m d e p r e s s i o n a s me n .
I n d i v i d u a l s w i t h a f a mi l y h i s t o r y o f mo o d d i s o r d e r s a r e a t a h i g h e r r i s k o f
d e v e l o p i n g a d i s o r d e r t h e ms e l v e s . O t h e r i mp o r t a n t b i o l o g i c a l f a c t o r s i n c l u d e
c o mo r b i d i t i e s o r d e p r e s s i o n a s a r e s u l t o f me d i c a l p r o b l e ms a n d a b u s e o f
s u b s t a n c e s , w h i c h ma y b e e i t h e r t h e c a u s e o r t h e s y mp t o m o f t h e d e p r e s s i o n .
2. P s y c h o l o g i c aI nl d i v i d u a l s w h o a r e c o n t i n u a l l y u n d e r a g r e a t d e a l o f s t r e s s ,
h a v e a n e g a t i v e o u t l o o k o n l i f e , o r a p a s s i v e t e mp e r a me n t a r e mo r e l i k e l y t o
97 / 652
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3.2 - Depression
s u f f e r f r o m a mo o d d i s o r d e r. T h e s e i n d i v i d u a l s o f t e n e n g a g e i n c o g n i t i v e
d i s t o r t i o n s , i n c l u d i n g u n r e a l i s t i c e xp e c t a t i o n s , o v e r g e n e r a l i zi n g a d v e r s e e v e n t s ,
p e r s o n a l i zi n g n e g a t i v e o r d i f f i c u l t e v e n t s , a n d o v e r r e a c t i n g t o s t r e s s o r s .
B e h a v i o r a l l y, i n d i v i d u a l s w h o a r e c o n t i n u a l l y u n d e r s t r e s s o f t e n b e l i e v e t h a t a n y
action on their part would be futile, and therefore they continue to repeat selfd e f e a t i n g o r p r o b l e ma t i c b e h a v i o r s o r d o n o t h i n g a t a l l ( l e a r n e d h e l p l e s s n e s s ) .
3. S o c i a lT h e r e a r e ma n y s o c i a l i n f l u e n c e s t h a t a r e r e l a t e d t o mo o d d i s o r d e r s .
T h e s e i n c l u d e d i f f i c u l t ma r r i a g e s , d i v o r c e , p r o b l e ms w i t h c h i l d r e n , f a mi l y a n d
c o mmu n i t y v i o l e n c e , a n d e c o n o mi c d i f f i c u l t i e s . M a n y i n d i v i d u a l s d o n o t h a v e
P. 4 6
t h e s o c i a l r e s o u r c e s ( e . g . , f r i e n d s h i p s , f a mi l y, c o mmu n i t y ) o r b u f f e r s t h a t a i d i n
coping (e.g., spirituality).
B. Epidemiology
D e p r e s s i o n i s o n e o f t h e mo s t c o mmo n c o n d i t i o n s s e e n i n p r i ma r y c a r e . R e l i a b l e
e s t i ma t e s s u g g e s t t h a t d e p r e s s i v e s y mp t o ms a r e p r e s e n t i n a p p r o xi ma t e l y 7 0 % o f
p a t i e n t s w h o v i s i t p r i ma r y c a r e p r o v i d e r s w i t h a p p r o xi ma t e l y 1 5 % t o 2 0 % o f t h e s e
p a t i e n t s s u f f e r i n g f r o m ma j o r d e p r e4)s .s iTohne( p r e v a l e n c e o f ma j o r d e p r e s s i o n i s
t w o t o t h r e e t i me s h i g h e r i n g e n e r a l me d i c a l p r a c t i c e t h a n i n t h e o v e r a l l p o p u l a t i o n .
H o w e v e r, p h y s i c i a n s o f t e n u n d e r d i a g n o s e p a t i e n t s w i t h d e p r e s s i o n . E v e n a mo n g
p a t i e n t s c o r r e c t l y d i a g n o s e d , mo s t p a t i e n t s w i t h d e p r e s s i o n s t i l l d o n o t r e c e i v e
t r e a t me n t c o n c o r d a n t w i t h r e c o mme n d e d g u i d e l i n e s . F u r t h e r, p a t i e n t a d h e r e n c e t o
t h e r e c o mme n d e d t r e a t me n t p l a n i5)
s .l o w (
I I I . Evaluation
A. History
T o b e d i a g n o s e d w i t h ma j o r d e p r e s s i o n , a n i n d i v i d u a l mu s t h a v e e xp e r i e n c e d o v e r
t h e s a me t w o - w e e k p e r i o d f i v e o r mo r e s y mp t o ms a n d mu s t h a v e d e p r e s s e d mo o d
a n d / o r a n h e d o n i a . T h e mn e mo n i c S I G E C A P S h i g h l i g h t s t h e s y mp t o ms o f a ma j o r
depressive episode:
1. Sl e e p d i s t u r b a n c e e a r l y mo r n i n g a w a k e n i n g s o r r e s t l e s s s l e e p
2. In t e r e s t l i t t l e i n t e r e s t i n a c t i v i t i e s t h e y u s e d t o e n j o y ( a n h e d o n i a )
3. Gu i l t f e e l i n g g u i l t y o r w o r t h l e s s
4. En e r g y f e e l i n g t i r e d o r f a t i g u e d
5. Co n c e n t r a t i o n i mp a i r e d c o n c e n t r a t i o n a n d / o r i n d e c i s i v e n e s s
6. Ap p e t i t e w e i g h t c h a n g e a n d / o r c h a n g e s i n t h e i r n o r ma l e a t i n g p a t t e r n s ( e a t i n g
l e s s o r mo r e t h a n u s u a l )
7. Ps y c h o mo t o r d i s t u r b a n c e a n y p s y c h o mo t o r a g i t a t i o n o r r e t a r d a t i o n
8. Su i c i d a l t h o u g h t s r e c u r r e n t t h o u g h t s o f d e a t h , s u i c i d a l i d e a t i o n , a n d s u i c i d e
a t t e mp t .
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3.2 - Depression
A n y p a t i e n t w i t h d e p r e s s i o n s e v e r e e n o u g h t o w a r r a n t t r e a t me n t s h o u l d h a v e b o t h a
g e n e r a l s c r e e n i n g p h y s i c a l e xa mi n a t i o n ( p a y i n g p a r t i c u l a r a t t e n t i o n t o s i g n s o f
a n e mi a a n d e n d o c r i n o p a t h i e s [ e . g . , h y p o t h y r o i d i s m] ) a n d a c a r e f u l s c r e e n i n g
n e u r o l o g i c e xa mi n a t i o n . D e p r e s s i o n i s a l s o o f t e n a s y mp t o m o f ma n y me d i c a l
conditions that are related to depressive disorders (e.g., cardiovascular disease,
mu l t i p l e s c l e r o s i s , c a n c e r, t h y r o i d d i s o r d e r s , a c q u i r e d i mmu n o d e f i c i e n c y s y n d r o me ,
endocrine changes).
C. Testing
L a b o r a t o r y t e s t s s h o u l d b e o r d e r e d o n t h e b a s i s o f t h e h i s t o r y a n d e xa mi n a t i o n
f i n d i n g s ( e . g . , c o mp l e t e b l o o d c o u n t f o r a n e mi a , t h y r o i d - s t i mu l a t i n g h o r mo n e f o r
thyroid disorders).
1. M u l t i p l e s c r e e n i n g i n s t r u me n t s c a n b e u s e d t o d e t e c t d e p r e s s i o n ( i . e . , Z u n g ,
Beck depression inventory). A relatively new screening tool that has been found
t o b e e f f i c i e n t a n d e f f e c t i v e i n d e t e r mi n i n g d e p r e s s i o n s e v e r i t y i s t h e P a t i e n t
H e a l t h Q u e s t i o n n a i r e - 9 ( P H6)
Q .- 9T) h( e P H Q - 9 i s a s c r e e n i n g q u e s t i o n n a i r e
c h o s e n b e c a u s e o f t h e e a s e o f a d mi n i s t r a t i o n , s c o r i n g , a n d i t s h i g h
s e n s i t i v i t y / s p e c i f i c i t y. T h e P H Q - 9 i s a 9 - q u e s t i o n f o r m t h a t a d d r e s s e s a l l t h e
s y mp t o ms o f ma j o r d e p r e s s6)i o. nQ(u e s t i o n s a r e a n s w e r e d u s i n g a 4 - p o i n t Likert scale (0 = not at all and 3 = nearly everyday). Scores of 5 to 9 are
a s s o c i a t e d w i t h mi l d d e p r e s s i o n , a n d s c o r e s o f 1 0 o r a b o v e a r e a s s o c i a t e d w i t h
mo d e r a t e o r s e v e r e d e p r e s 7)
s i o. n (
2. T h e U . S . P r e v e n t i v e S e r v i c e s T a s k 8)
F orrecceo mme
(
nds screening adults for
depression in clinical practice. A quick two question screen for depression can
be used to identify individuals at risk: over the past two weeks have you (a) felt
down, depressed, or hopeless? and (b) felt little interest or pleasure in doing
things? If an individual answers yes to one or both of these, he/she should be
further evaluated for depression.
I V. Diagnosis
1. I n a d d i t i o n t o t h e me d i c a l c o n d i t i o n s r e l a t e d t o d e p r e s s i o n d i s c u s s e d e a r l i e r,
e a c h i n d i v i d u a l s u s p e c t e d o f d e p r e s s i o n s h o u l d a l s o b e s c r e e n e d f o r a n xi e t y
d i s o r d e r s , a l c o h o l a n d d r u g a b u s e , s u i c i d a l i t y, h o mi c i d a l i t y, a n d d o me s t i c
violence or
P. 4 7
perpetration of abuse.
2. T h e r e a r e ma n y t y p e s o f d e p r e s s i v e d i s o r d e r s . I t i s i mp o r t a n t t o d i s t i n g u i s h t h e
s p e c i f i c t y p e o f d i s o r d e r s o a s t o mo s t e f f e c t i v e l y r e c o mme n d t r e a t me n t o p t i o n s .
a. M a j o r D e p r e s s i v e D i s o r d e r i s p r e s e n t w h e n t h e i n d i v i d u a l h a s t w o o r mo r e
ma j o r d e p r e s s i v e e p i s o d e s .
99 / 652
tmdmss
3.2 - Depression
b. D y s t h y mi c D i s o r d e r i s c h a r a c t e r i ze d b y a t l e a s t t w o y e a r s o f l o w - g r a d e
depression
( d o e s n o t me e t t h e c r i t e r i a f o r a ma j o r d e p r e s s i v e e p i s o d e ) .
c. D e p r e s s i v e D i s o r d e r N o t O t h e r w i s e S p e c i f i e d i s u s e d w h e n t h e i n d i v i d u a l
d o e s n o t me e t c r i t e r i a f o r o t h e r d e p r e s s i v e c o n d i t i o n s , b u t d e p r e s s i v e
f e a t u r e s e xi s t .
d. B i p o l a r I D i s o r d e r i s c h a r a c t e r i ze d b y o n e o r mo r e ma n i c e p i s o d e s a n d i s
u s u a l l y a c c o mp a n i e d b y ma j o r d e p r e s s i v e e p i s o d e s . I t i s i mp o r t a n t t o r u l e
o u t ma n i a i n i n d i v i d u a l s w h o a r e d e p r e s s e d b e c a u s e p s y c h o p h a r ma l o g i c a l
t r e a t me n t o f d e p r e s s i o n c a n ma k e i n d i v i d u a l s w i t h B i p o l a r d i s o r d e r b e c o me
ma n i c . M a n i c e p i s o d e s mu s t i n c l u d e a t l e a s t t h r e e o f t h e f o l l o w i n g
s y mp t o ms l a s t i n g f o r a p e r i o d o f a t l e a s t s e v e n d a y s :
i. i n f l a t e d s e l f - e s t e e m o r g r a n d i o s i t y
i i. d e c r e a s e d n e e d f o r s l e e p
i i i. mo r e t a l k a t i v e t h a n u s u a l o r p r e s s u r e t o k e e p t a l k i n g
i v. f l i g h t o f i d e a s o r s u b j e c t i v e e xp e r i e n c e o f t h o u g h t s r a c i n g
v. d i s t r a c t i b i l i t y
v i. i n c r e a s e i n g o a l - d i r e c t e d a c t i v i t y ( s o c i a l l y, a t w o r k , o r s e xu a l l y ) o r
p s y c h o mo t o r a g i t a t i o n
v i i. e xc e s s i v e i n v o l v e me n t i n p l e a s u r a b l e a c t i v i t i e s t h a t h a v e a h i g h
p o t e n t i a l f o r p a i n f u l c o n s e q u e n c e s ( e . g . , s p e n d i n g , g a mb l i n g , s e xu a l
indiscretions).
e. B i p o l a r I I D i s o r d e r i s c h a r a c t e r i ze d b y o n e o r mo r e ma j o r d e p r e s s i v e
e p i s o d e s a n d a t l e a s t o n e h y p o ma n i c e p i s o d e ( l a s t i n g a t l e a s t f o u r d a y s ) .
H y p o ma n i c e p i s o d e s u s e t h e s a me c r i t e r i a a s ma n i c e p i s o d e s , b u t
h y p o ma n i a d o e s n o t c a u s e ma r k e d i mp a i r me n t i n s o c i a l o r o c c u p a t i o n a l
f u n c t i o n i n g o r r e q u i r e h o s p i t a l i za t i o n .
f. C y c l o t h y mi c D i s o r d e r i s c h a r a c t e r i ze d b y a t l e a s t t w o y e a r s o f n u me r o u s
p e r i o d s o f l o w - g r a d e d e p r e s s i o n a n d h y p o ma n i c s y mp t o ms .
g. M o o d D i s o r d e r D u e t o a G e n e r a l M e d i c a l C o n d i t i o n a n d S u b s t a n c e - I n d u c e d
M o o d D i s o r d e r a r e c h a r a c t e r i ze d b y a mo o d d i s t u r b a n c e c a u s e d b y t h e
d i r e c t p h y s i o l o g i c a l c o n s e q u e n c e o f e i t h e r a g e n e r a l me d i c a l c o n d i t i o n o r
s u b s t a n c e u s e , r e s p e c t i v e l y.
h. S e a s o n a l A f f e c t i v e D i s o r d e r, G r i e f R e a c t i o n , a n d A d j u s t me n t D i s o r d e r w i t h
D e p r e s s e d M o o d a r e o t h e r d i s o r d e r s t h a t a r e c a u s e d b y t h e t i me o f t h e
y e a r, r e s p o n s e t o l o s s , a n d r e s p o n s e t o a s i g n i f i c a n t c h a n g e ( e . g . , d i v o r c e ) ,
r e s p e c t i v e l y.
1. D e p r e s s i o n d o e s n o t o f t e n p r e s e n t i n a p r i ma r y c a r e s e t t i n g b y t h e p a t i e n t
c o mp l a i n i n g o f d e p r e s s e d mo o d . M o r e l i k e l y t h e p a t i e n t w i l l d i s c u s s t h e
100 / 652
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3.2 - Depression
s y mp t o ms o f d e p r e s s i o n ( e . g . , f a t i g u e , i n s o mn i a , g a s t r o i n t e s t i n a l u p s e t ) . I t i s
t h e r e f o r e i mp o r t a n t f o r t h e p h y s i c i a n t o n o t o n l y a t t e n d t o t h e p h y s i c a l
c o mp l a i n t s b u t a l s o p r o b e i n t o t h e e mo t i o n a l s y mp t o ms ( e . g . , d y s p h o r i a ,
anhedonia).
2. T h e t r e a t me n t o f d e p r e s s i v e d i s o r d e r s i s i mp o r t a n t . O n t h e b a s i s o f t h e
b i o p s y c h o s o c i a l mo d e l , t r e a t me n t mo d a l i t i e s t h a t a d d r e s s t h e b i o l o g i c ,
psychological, and social aspects of the individual difficulties have been found
t o b e t h e mo s t e f f e c t i v e . C r e a t i n g a t r e a t me n t p l a n t a i l o r e d t o t h e i n d i v i d u a l s '
needs, including addressing the use of psychotherapy (cognitive-behavioral or
i n t e r p e r s o n a l ) , ma r i t a l t h e r a p y, me d i c a t i o n , a n d e xe r c i s e , h a v e b e e n f o u n d t o b e
mo s t e f f e c t i v e .
References
1 . H e n k H J , K a t ze l n i c k D J , K o b a k K A , e t a l . M e d i c a l c o s t s a t t r i b u t e d t o
d e p r e s s i o n a mo n g p a t i e n t s w i t h a h i s t o r y o f h i g h me d i c a l e xp e n s e s i n a h e a l t h
ma i n t e n a n c e o r g a n i za tAi ornc .h G en P s y c hi at1r9y9 6 ; 5 3 ( 1 0 ) : 8 9 9 9 0 4 .
P. 4 8
2 . P i n c u s H A , P e t t i t A R . T h e s o c i e t a l c o s t s o f c h r o n i c ma j o r Jd eCpl ri ens s i o n .
P s y c hi at r y2 0 0 1 ; 6 2 ( S 6 ) : 5 9 .
3 . P a p o l o s D , P a p o l o sOJv.er c omi ng depr es s, i on
3 r d e d . N e w Yo r k :
HarperCollins, 1997.
4. Montano C B, Montano M
A Bnew
.
par adi gm f or t r eat i ng depr es s i on i n t he
pr i mar y c ar e s et t i ng. M edi c al E duc at i on C ol l,abor
a t : at i v e
h t t p : / / w w w. me d s c a p e . c o m, a c c e s s e d o n O c t o b e r 1 0 , 2 0 0 4 .
5 . Yo u n g A S , K l a p R , S h e r b o u r n e C D , e t a l . T h e q u a l i t y o f c a r e f o r d e p r e s s i v e
a n d a n xi e t y d i s o r d e r s i n t h e U n i t e d A
S rt caht e G
s .en P s y c hi at2r0y0 1 ; 5 8 ( 1 ) : 5 5
61.
6 . K r o e n k e K , S p i t ze r R L , W i l l i a ms J B W. Va l i d i t y o f a b r i e f d e p r e s s i o n s e v e r i t y
me a s u r e J. G en I nt er n M ed
2001;16(9):606613.
7 . T h e M a c A r t h u r I n i t i a t i v e o n D e p r e s s i o n a n d P r i ma r y C a r e . T o o l K i t , a t :
h t t p : / / w w w. d e p r e s s i o n - p r i ma r y c a r e . o r g , a c c e s s e d o n J u l y 2 2 , 2 0 0 5 .
8. U . S . P reventive S ervices Task F orce. S creening for depression:
r e c o mme n d a t i o n s a n d r a t i o nAann
l e .I nt er n M ed
2002;136(10):760764.
101 / 652
tmdmss
3.3 - Suicide Risk
> T a b l e o f C o n t e n t s > 3 - M e n t a l H e a l t h P r o b l e ms > 3 . 3 - S u i c i d e R i s k
3.3
Suicide Risk
He ath A. Grame s
I . Background
S u i c i d e i s r a n k e d a s t h e 11 t h l e a d i n g c a u s e o f d e a t h i n t h e g e n e r a l U S p o p u l a t i o n
and the 3rd leading cause of death for adolescents and young adults from age 15 to
2 4 y e a r s1)( . A l t h o u g h t h e r i s k o f s u i c i d e c a n b e d i f f i c u l t t o a s s e s s a n d p r e d i c t
b e c a u s e o f t h e n u mb e r o f f a c t o r s t h a t c o n t r i b u t e t o s u c h a d e c i s i o n , a n
u n d e r s t a n d i n g o f t h e r i s k f a c t o r s a n d a s s e s s me n t q u e s t i o n s ma y p r e v e n t t h i s t r a g i c
e v e n t . M a n y p a t i e n t s v i s i t t h e i r p r i ma r y c a r e p r o v i d e r ( P C P ) s o me mo n t h s b e f o r e
a t t e mp t i n g s u i c i d2)e. (I f t h e r e d f l a g s o f s u i c i d a l i d e a t i o n a r e p r e s e n t a n d
r e c o g n i ze d b y t h e P C P, a n o p p o r t u n i t y i s c r e a t e d f o r p o s i t i v e i n t e r v e n t i o n t o p r e v e n
s u i c i d e a t t e mp t s .
A. Etiology
S u i c i d e i s a t o p i c t h a t r e c e i v e s mu c h a t t e n t i o n t h r o u g h me d i a a n d o t h e r p l a t f o r ms o
d i s c u s s i o n p e r h a p s b e c a u s e s u i c i d e i s o f t e n v i e w e d a s a mo r a l d e c i s i o n t h a t i s
c o n t r a r y t o ma n y r e l i g i o u s a n d s o c i e t a l v a l u e s . H o w e v e r, f o r ma n y i n d i v i d u a l s w h o
a t t e mp t o r c o mmi t s u i c i d e , t h e i r q u a l i t y o f l i f e ( p h y s i c a l , e mo t i o n a l , a n d / o r s p i r i t u a l )
h a s b e c o me s o d e p l e t e d t h a t t h e y ma y s e e n o o t h e r o p t i o n s . M a n y f a c t o r s c o n t r i b u t
to a low level of quality of life and, therefore, the decision to take one's own life.
B o t h p h y s i c a l a n d p s y c h i a t r i c d i s o r d e r s a r e r e c o g n i ze d a s b e i n g a mo n g t h e s e
factors. Physical contributors include chronic illness and changes in
n e u r o t r a n s mi t t e r s ( i . e . , s e r o 1)
t o .n iPns) (y c h i a t r i c d i s o r d e r s i n c l u d e ma j o r d e p r e s s i o n ,
s u b s t a n c e a b u s e , s c h i zo p h r e n i a , p a n i c d i s o r d e r, a n d b o r d e r l i n e p e r s o n a l i t y d i s o r d e
(3) . O t h e r f a c t o r s i n c l u d e a h i s t o r y o f s u i c i d e a t t e mp t s b y t h e i n d i v i d u a l o r c l o s e
r e l a t i v e s a n d f r i e n d s , v i o l e n c e a t h o me , h i s t o r y o f p h y s i c a l o r s e xu a l a b u s e ,
o w n e r s h i p o f a f i r e a r m, h i s t o r y o f f a mi l y me n t a l i l l n e s s , a r e c e n t c r i s i s ( i . e . , l o s s o f
i n c o me , d i v o r c e ) , i l l n e s s , a n d o l d a g e . I n a d d i t i o n , h o p e l e s s n e s s , h o s t i l i t y, n e g a t i v e
s e l f - e s t e e m, a n d i s o l a t i o n h a v e b e e n i d e n t i f i e d a s s u i c i d e r i s k f a c t o r s i n a d o l e s c e n t
(4) .
B. Epidemiology
T h e mo s t c u r r e n t d a t a o n s u i c i d e r a t e s p o s t e d b y t h e N a t i o n a l I n s t i t u t e o f M e n t a l
Health (N IM H) from 2001 show that 30,622 individuals died by suicide in the United
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3.3 - Suicide Risk
S t a t e s t h a t y e a r. T o p u t t h i s i n p e r s p e c t i v e , t h i s i s h i g h e r t h a n t h e n u mb e r o f
i n d i v i d u a l s w h o d i e d b y h o mi c i d e b y a r a t i o o f t h r e e t o t w o a n d t w i c e a s ma n y a s
t h o s e w h o d i e d f r o m c o mp l i c a t i o n s f r o m h u ma n i mmu n o d e f i c i e n c y v i r u s / a c q u i r e d
i mmu n o d e f i c i e n c y s y n d r o me . T h e h i g h e s t r i s k p o p u l a t i o n f o r c o mmi t t i n g s u i c i d e i s
w h i t e me n a g e 8 5 a n d o v e r ( 5 4 d e a t h s b y s u i c i d e p e r
P. 4 9
1 0 0 , 0 0 0 ) , w h i c h i s f i v e t i me s g r e a t e r t h a n t h e g e n e r a l p o p u l a t i o n ( 1 0 . 7 / 1 0 0 , 0 0 0 ) .
A mo n g c h i l d r e n , a d o l e s c e n t s , a n d y o u n g a d u l t s , t h e l a t t e r a r e a t s l i g h t l y g r e a t e r r i s
o f c o mmi t t i n g s u i c i d e ( 1 2 / 1 0 0 , 0 0 0 ) t h a n t h e n a t i o n a l a v e r a g e . A mo n g a l l a g e - g r o u p s
me n a r e mo r e l i k e l y t o c o mmi t s u i c i d e t h a n a r e w o me n ( 4 : 1 ) , b u t w o me n a r e r e p o r t e
t o h a v e ma d e mo r e s u i c i d e a t t e mp t s . W h i t e me n a r e mo s t l i k e l y t o c o mmi t s u i c i d e ,
a c c o u n t i n g f o r 7 3 % o f a l l d e a t h s b y s u i c i d e . F i r e a r ms a r e t h e mo s t c o mmo n mo d e o f
s u i c i d e b y b o t h me n a n d w o1)
me. n (
I I I . Evaluation
O n e o f t h e b e s t p r e c a u t i o n s a P C P c a n t a k e t o a s s e s s f o r s u i c i d a l r i s k i s c o mp l e t i n
a b r i e f h i s t o r y w i t h p a t i e n t s , a s k i n g a b o u t t h e p r e v i o u s l y me n t i o n e d r i s k f a c t o r s .
A l t h o u g h i t i s n o t n e c e s s a r y t o e xh a u s t i v e l y i n t e r v i e w a l l p a t i e n t s , e v e r y o n e w h o h a
any suicide risk factors should be queried about these issues.
A. History
P a t i e n t s r a r e l y t a l k t o t h e i r P C P s a b o u t s u i c i d a l t h o u g h t s o r p a s t a t t e mp t s . I n f a c t ,
p a t i e n t s r a r e l y b r i n g u p i s s u e s t h a t ma y r e v e a l r i s k f a c t o r s f o r s u i c i d e ( e . g . ,
d e p r e s s i o n ) w i t h o u t b e i n g a s k e d d i r e c t l y. I n s t e a d , t h e y o f t e n d i s c u s s t h e p h y s i c a l
ma n i f e s t a t i o n s s u c h a s h e a d a c h e s , mu s c l e p a i n , a n d i n s o mn i a . T o a s s e s s f o r
s u i c i d a l i d e a t i o n , t h e P C P mu s t a s k p a t i e n t s s p e c i f i c q u e s t i o n s t o u n c o v e r t h e i r
i n t e n t i o n s . P a t i e n t s a t g r e a t e r r i s k f o r s u i c i d a l i d e a t i o n o r a t t e mp t s s h o u l d b e
a s s e s s e d f o r c u r r e n t t h o u g h t o f h a r mi n g t h e ms e l v e s . T h i s c a n b e d o n e e a s i l y a s p a
o f a n a s s e s s me n t f o r d e p r e s s i o n s u c h a s t h e P a t i e n t H e a l t h Q u e s t i o n n a i r e - 9 ( P H Q 9 ) (5) o r b y s i mp l y a s k i n g t h e p a t i e n t s i f t h e y a r e e xp e r i e n c i n g t h o u g h t s o f h a r mi n g
t h e ms e l v e s . S o me P C P s ma y f e e l h e s i t a n t t o a s k s u c h a d i r e c t q u e s t i o n p e r t a i n i n g
t o s u i c i d e f o r f e a r t h a t t h e y ma y b e p l a n t i n g a n i d e a i n a p a t i e n t w h o i s a l r e a d y
s t r u g g l i n g . H o w e v e r, n o r e s e a r c h h a s s u g g e s t e d t h a t a p a t i e n t ma y k i l l h i ms e l f o r
h e r s e l f s i mp l y b e c a u s e o f b e i n g a s k e d a b o u t t h o u g h t s o f s u i c i d e . O n t h e c o n t r a r y,
a t - r i s k p a t i e n t s mu s t b e a s k e d a b o u t s u i c i d a l t h o u g h t s a n d b e h a v i o r s o t h a t
a p p r o p r i a t e me a s u r e s c a n b e t a k e n .
B. Assessment
1. I f p a t i e n t s h a v e t h o u g h t s o f s u i c i d e a n d a p l a n f o r t h e s a me , t h e P C P s h o u l d
d e t e r mi n e h o w s e r i o u s t h e i n d i v i d u a l i s a b o u t c a r r y i n g o u t t h e p l a n s o f s u i c i d e
a n d t h e t i me f r a me f o r t h e s u i c i d e aLt tev
e mp
el tof
. s er i ous nes
i ns c a r r y i n g o u t
suicide plans can be assessed by asking patients if they have told other
i n d i v i d u a l s o f t h e p l a n t o c o mmi t s u i c i d e a n d t h e d e t a i l s o f t h e p l a n a n d b y
a s k i n g p a t i e n t s d i r e c t l y h o w s e r i o u s t h e y a r e a b o u t a c t u a l l y h a r mi n g t h e ms e l v e s
Also, the P C P should distinguish between a realistic and unrealistic plan to
c o mmi t s u i c i d e .r eal
A i s t i cp l a n r e f e r s t o a p l a n w h e r e t h e i n d i v i d u a l h a s t h e
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3.3 - Suicide Risk
a c c e s s a n d me a n s t o c o mp l e t e a s u i c i d e s u c h a s a p a t i e n t w h o t h r e a t e n s t o
o v e r d o s e o n a me d i c a t i o n t h a t ma y h a v e b e e n p r e s unr
c r i beal
e d .i sAt inpc l a n
r e f e r s t o a p l a n t h a t i s b a s e d o n u n l i k e l y o r i mp o s s i b l e me a n s o f f u l f i l l i n g
s u i c i d a l d e s i r e s . F o r e xa mp l e , a p a t i e n t ma y s t a t e t h a t h e w a n t s t o s h o o t h i ms e l
b u t d o e s n o t o w n a g u n , d o e s n o t k n o w s o me o n e w h o o w n s a g u n , c a n n o t
p u r c h a s e a g u n , a n d d o e s n o t c o n s i d e r o t h e r mo r e r e a l i s t i c me a n s o f s u i c i d e t o
w h i c h h e h a s a c c e s s . A l t h o u g h t h i s p a t i e n t me r i t s c o n c e r n a n d i n n e e d o f a
s u i c i d e ma n a g e me n t p l a n , h e ma y b e i n l e s s i mme d i a t e d a n g e r t h a n t h e p a t i e n t
w h o h a s d e v e l o p e d a r e a l i s t i c a n d a c c e s s i b l e me a n s t o e n d h i s l i f e . D e t e r mi n i n g
if the plan is realistic or not gives insight into how likely the individual is to
c o mmi t s u i c i d e .
2. T o d e t e r mi n e t sh ui
e c i de at t empt t i me f r, ame
patients should be asked when
t h e y p l a n t o a t t e mp t s u i c i d e a n d e s t i ma t e h o w l i k e l y t h e y a r e t o a t t e mp t s u i c i d e
b e f o r e t h e n e xt v i s i t t o t h e p h y s i c i a n . T h e a n s w e r t o t h e s e q u e s t i o n s s h o u l d
c a r r y w e i g h t i n d e t e r mi n i n g t h e l e v e l o f ma n a g e me n t . F o r e xa mp l e , a p a t i e n t ma
be very depressed, state a realistic plan of suicide, and offer little reason to liv
b u t t h e n s a y t h a t h e w i l l n o t a t t e mp t u n t i l h i s t h r e e - y e a r - o l d c h i l d h a s g r a d u a t e d
f r o m h i g h s c h o o l . A l t h o u g h t h i s i n d i v i d u a l s h o u l d r e c e i v e h e l p , h e ma y n o t b e i n
i mme d i a t e d a n g e r f o r s u i c i d e .
C. Risk
O n t h e b a s i s o f y o u r a s s e s s me n t , a p laev
t i eeln t of
' s r i s iks d e t e r mi n e d . F o r
e xa mp l e , a p a t i e n t w h o r e p o r t s t h a t s h e h a s h a d t h o u g h t s t h a t s h e w o u l d b e b e t t e r
o f f d e a d , o r e v e n o f a c t u a l l y h a r mi n g h e r s e l f , b u t s a y s s h e h a s n e v e r n o r w i l l e v e r
a t t e mp t s u i c i d e d u e t o p e r s o n a l a n d r e l i g i o u s b e l i e f s a n d b e c a u s e o f f a mi l y
c o mmi t me n t s , i s d i f f e r e n t f r o m t h e p a t i e n t w h o r e p o r t s t h a t h e o w n s f i r e a r ms , h a s
P. 5 0
a t t e mp t e d s u i c i d e i n t h e p a s t , a n d c a n g i v e n o r e a s o n f o r l i v i n g . P a t i e n t s ma y b e
c l a s s i f i e d a s b e i n gmii nni mal , moder at e, or s ev er ,e ar ni sdk t h e t r e a t me n t s h o u l d
be in direct relationship to how the patient is classified.
1. T h e mi ni malr i s k c a t e g o r y i n c l u d e s p a t i e n t s w h o h a v e e xp e r i e n c e d t h o u g h t s o f
s e l f h a r m b u t h a v e n o s p e c i f i c p l a n , n o h i s t o r y o f p a s t a t t e mp t s , a n d w h o s t a t e
t h a t t h e y w o u l d n o t a c t u a l l y a t t e mp t s u i c i d e . T h e s e a r e p a t i e n t s w h o c a n o f f e r
reasons to continue living or at least are active with the P C P in developing a
safety plan.
2. M oder at er i s k p a t i e n t s i n c l u d e i n d i v i d u a l s w h o h a v e c o n s i d e r e d a s u i c i d e p l a n
( w h i c h ma y o r ma y n o t b e r e a l i s t i c ) , b u t s a y t h a t t h e y d o n o t t h i n k t h e y c o u l d
a c t u a l l y h a r m t h e ms e l v e s . M o d e r a t e r i s k p a t i e n t s ma y h a v e ma d e a t t e mp t s i n t h e
d i s t a n t p a s t b u t a r e w i l l i n g t o c r e a t e a s a f e t y p l a n a n d e xp l o r e r e a s o n s t o l i v e .
3. S ev er er i s k p a t i e n t s h a v e a s p e c i f i c p l a n t h a t i s r e a l i s t i c a n d s t a t e t h a t t h e y w i l l
a t t e mp t s u i c i d e o r w i l l n o t c o mmi t t o a n o s u i c i d e c o n t r a c t , e v e n f o r a s h o r t
d u r a t i o n . T h e s e i n d i v i d u a l s ma y o r ma y n o t h a v e a h i s t o r y o f p a s t a t t e mp t s b u t
a r e a t g r e a t e r r i s k i f t h e y h a v e a t t e mp t e d i n t h e p a s t .
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3.3 - Suicide Risk
I V. Diagnosis
I f a p a t i e n t r e s p o n d s t h a t t h e y a r e c o n s i d e r i n g s u i c i d e , t h e s u i c i d e a s s e s s me n t
b e c o me s t h e p r i ma r y f o c u s o f t h e me d i c a l a p p o i n t me n t . A f u l l a s s e s s me n t a n d
c r e a t i n g a s a f e t y p l a n o r a d mi s s i o n t o t h e h o s p i t a l ma y t a k e a s l i t t l e a s t e n mi n u t e s
o r i t ma y t a k e mo r e , d e p e n d i n g o n s t a f f t r a i n i n g a n d t r e a t me n t a c c e s s i b i l i t y.
References
1 . N a t i o n a l I n s t i t u t e o f M e n t a l HIena lhar
t h . m' s way : s ui c i de i n A mer
. i ca
R e v i s e d e d . [ B r o c h u r e ] . B e t h e s d a , M D : U S D e p a r t me n t o f H e a l t h a n d H u ma n
Services, 2003.
2. M urphy G E . T he physician's responsibility for suicide. I. An error of
c o mmi s s i o nA. nn I nt er n M ed
1975;82:301304.
3 . G l i a t t o M F, R a i A K . E v a l u a t i o n a n d t r e a t me n t o f p a t i e n t s w i t h s u i c i d a l i d e a t i o n .
1999;59:15001506.
4 . R u t t e r PA , B e h r e n d t A E . A d o l e s c e n t s u i c i d e r i s k : f o u r p s y c h o l o g i c a l f a c t o r s .
A dol es c enc 2e 0 0 4 ; 3 9 : 2 9 5 3 0 2 .
5 . T h e M a c A r t h u r I n i t i a t i v e o n D e p r e s s i o n a n d P r i ma r y C a r e . T o o l K i t , a t :
h t t p : / / w w w. d e p r e s s i o n - p r i ma r y c a r e . o r g , a c c e s s e d o n J u l y 2 5 , 2 0 0 5 .
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4.1 - Ataxia
> T a b l e o f C o n t e n t s > 4 - P r o b l e ms R e l a t e d t o t h e N e r v o u s S y s t e m > 4 . 1 - A t a xi a
4.1
Ataxia
Robe rt R. Raune r
I . Background
A t a xi a i s t h e l o s s o f t h e a b i l i t y t o c o o r d i n a t e mu s c u l a r mo v e me n t .
A. Etiology
A t a xi a i s c a u s e d b y t h e d y s f u n c t i o n o f t h e c e r e b e l l u m a n d i t s a f f e r e n t a n d e f f e r e n t
p a t h w a y s . A t a xi a h a s a b r o a d l i s t o f c a u s e s , i n c l u d i n g :
1. D r u g s , s u c h a s a l c o h o l , b a r b i t u r a t e s , l i t h i u m, o r c h e mo t h e r a p e u t i c d r u g s
2. T o xi n s , s u c h a s me r c u r y, s o l v e n t s , l e a d , g a s o l i n e , o r g l u e
3. I n f e c t i o n s , s u c h a s c o xs a c k i e v i r u s , h u ma n i mmu n o d e f i c i e n c y v i r u s ( H I V ) ,
L egi onel l, aL y me d i s e a s e , o r v a r i c e l l a
4. N u t r i t i o n a l c o n d i t i o n s , s u c h a s v i t1a, mi
B1n2, Bo r E d e f i c i e n c y
5. E n d o c r i n e c o n d i t i o n s , s u c h a s h y p o t h y r o i d i s m
6. Va s c u l a r c o n d i t i o n s , s u c h a s c e r e b e l l a r i n f a r c t i o n o r h e mo r r h a g e
7. H e r e d i t a r y c o n d i t i o n s , s u c h a s F r i e d r e i c h ' s a t a xi a , a t a xi a t e l a n g i e c t a s i a , o r
M achado- J oseph ( S C A-3)
8. N e o p l a s t i c c o n d i t i o n s , s u c h a s c e r e b e l l a r g l i o ma , me t a s t a t i c t u mo r s , o r
p a r a n e o p l a s t i c s y n d r o me s
9. C o n g e n i t a l c o n d i t i o n s , s u c h a s C h i a r i o r D a n d y - Wa l k e r ma 1)
l f o. r ma t i o n s (
B. Epidemiology
F r i e d r e i c h ' s a t a xi a i s t h e mo s t c o mmo n c a u s e o f h e r e d i t a r y a t a xi a , c o n s t i t u t i n g a b o u
h a l f o f h e r e d i t a r y a t a xi a s . T h e i n c i d e n c e o f F r i e d r e i c h ' s a t a xi a i s a p p r o xi ma t e l y 1 t o
2 p e r 5 0 , 0 0 0 i n t h e U n i t e d S t2)a .t e s (
I I I . Evaluation
A. History
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4.1 - Ataxia
T h e h i s t o r y s h o u l d i n c l u d e t i me o f o n s e t o f t h e d e v e l o p me n t o f a t a xi a , w h e t h e r i t i s
s y mme t r i c o r a s y mme t r i c , a n d w h e t h e r i t i s f o c a l o r d i f f u s e . A g r a d u a l o n s e t w i t h
s y mme t r i c a n d b i l a t e r a l s y mp t o ms s u g g e s t s a b i o c h e mi c a l , i mmu n e , me t a b o l i c , o r
t o xi c c a u s e . F o c a l a n d a s y mme t r i c a t a xi a s w i t h o t h e r s y mp t o ms s u c h a s h e a d a c h e s
a n d c r a n i a l n e r v e p a l s i e s s u g g e s t a c e r e b e l l a r ma s s . T h e h i s t o r y s h o u l d a l s o i n c l u d
q u e s t i o n i n g r e g a r d i n g me d i c a t i o n s t h a t c a n c a u s e a t a xi a ( e . g . , l i t h i u m) , e xp o s u r e t o
t o xi n s ( e . g . , me r c u r y ) , a n d h i s t o r y o f e t h a n o l i n t a k e . T h e a g e o f o n s e t c a n b e h e l p f u
i n d i f f e r e n t i a t i n g s o me o f t h e h e r e d i t a r y a t a xi a s . T h e p a t i e n t s h o u l d b e q u e s t i o n e d
a b o u t r e c e n t o r c h r o n i c i n f e c t i o n s b e c a u s e t h e r e a r e s e v e r a l t h a t c a n c a u s e a t a xi a
( e . g . , v a r i c e l l a , L y me d i s e a s e , o r H I V ) .
T h e e xa mi n a t i o n s h o u l d i n c l u d e a n e v a l u a t i o n o f t h e t y p e o f a t a xi a . T h e p a t i e n t ' s
speech should be observed for difficulties such as dysarthria or scanning speech.
T h e p a t i e n t ' s g a i t s h o u l d b e o b s e r v e d . E y e mo v e me n t s s h o u l d b e e xa mi n e d f o r
n y s t a g mu s o r c r a n i a l n e r v e p a l s i e s . T h e p a t i e n t ' s mo v e me n t s s h o u l d b e e v a l u a t e d
f o r t r e mo r, l o s s o f p r o p r i o c e p t i o n , w e a k n e s s , l o s s o f f i n e mo t o r c o n t r o l , s p a s t i c i t y,
r i g i d i t y, b r a d y k i n e s i a , a n d d y s t o n i a . I n a d d i t i o n , t h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o
s h o u l d s e a r c h f o r o t h e r p r o b l e ms t h a t c a n mi mi c a t a xi a f r o m c e r e b e l l a r c a u s e s , s u c h
as vertigo from vestibular disease, or difficulties in gait that are actually due to leg
w e a k n e s s1,2)
( .
C. Testing
1. C o mp u t e d t o mo g r a p h y o r ma g n e t i c r e s o n a n c e i ma g i n g ( M R I ) o f t h e b r a i n
( p r e f e r a b l y M R I ) t o e v a l u a t e f o r c a u s e s s u c h a s a n e o p l a s m, h e mo r r h a g e , o r
i n f a r c t i o n . T h i s s h o u l d b e d o n e a s s o o n a s p o s s i b l e , b e c a u s e s o me c a u s e s ma y
b e s u r g i c a l e me r g e n c i e s ( e . g . , ma s s e f f e c t l e a d i n g t o b r a i n s t e m h e r n i a t i o n ) .
2. M a n y o t h e r c a u s e s ma y b e d i s c o v e r e d b y l a b o r a t o r y t e s t i n g . P o s s i b l e t e s t s
i n c l u d e t h y r o i d - s t i mu l a t i n g h o r mo n e , v1i t a mi
n dnv B
i t a mi n1 B
2 l e v e l s , H I V,
P. 5 4
r a p i d p l a s ma r e a g i n , L y me , a n d t o xi c o l o g y. A n t i b o d i e s t o Yo , R i , a n d P C D ma y
b e p r e s e n t i n p a r a n e o p l a s t i c s y n d r o me s . I n c h i l d r e n , s c r e e n i n g f o r i n h e r i t e d
me t a b o l i c d i s o r d e r s ma y b e w a r r a n t e d .
3. L u mb a r p u n c t u r e ma y b e h e l p f u l f o r d i a g n o s i n g c a u s e s s u c h a s mu l t i p l e
s c l e r o s i s , v i r a l c e r e b e l l i t i s , a n d o t h e r i n f e c t i o n s . A n e l e c t r o e n c e p h a l o g r a m ma y
r e v e a l s e i zu r e - r e l a t e d a t 1)
a xi. a (
D. Genetics
T h e r e i s a l o n g l i s t o f h e r e d i t a r y a t a xi a s . T h e mo s t c o mmo n o f t h e s e i s F r i e d r e i c h ' s
a t a xi a . T h e h e r e d i t a r y a t a xi a s a r e s u b d i v i d e d b y t h e mo d e o f i n h e r i t a n c e . T h e mo s t
c o mmo n a r e a u t o s o ma l r e c e s s i v e ( e . g . , F r i e d r e i c h ' s a t a xi a ) . A u t o s o ma l d o mi n a n t
a t a xi a s a r e c l a s s i f i e d b y a s e q u e n t i a l n u mb e r i n g s y s t e m ( e . g . , S C A - 3 w h i c h i s mo s t
c o mmo n i n t h e U n i t e d S t a t e s ) . T h e r e a r e a l s o X - l i n k e d a n d mi t o c h o n d r i a l f o r ms o f
h e r e d i t a r y a t a xi a2)s. (
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4.1 - Ataxia
I V. Diagnosis
A t a xi a h a s d i v e r s e c a u s e s , r a n g i n g f r o m t o xi n s ( e . g . , me r c u r y ) , i n f e c t i o n s ( e . g . , L y m
d i s e a s e ) , a n d n e o p l a s ms t o h e r e d i t a r y d e g e n e r a t i v e c a u s e s ( e . g . , F r i e d r e i c h ' s
a t a xi a )1)( . F r i e d r e i c h ' s a t a xi a u s u a l l y s t a r t s b e f o r e 2 5 y e a r s o f a g e . I t mo s t
c o mmo n l y p r e s e n t s w i t h l o w e r e xt r e mi t y s y mp t o ms i n c l u d i n g g a i t d i s t u r b a n c e s ,
d i mi n i s h i n g r e f l e xe s , a n d l o s s o f p r o p r i o c e p t i o n . O t h e r s y mp t o ms ( w h i c h c a n
o c c a s i o n a l l y b e p r e s e n t i n g s y mp t o ms ) i n c l u d e d y s a r t h r i a , s c o l i o s i s , n y s t a g mu s , a n d
f o o t d e f o r mi t i e s . A p p r o xi ma t e l y 2 5 % o f p a t i e n t s h a v e h y p e r t r o p h i c c a r d i o my o p a t h y
a n d 1 0 % h a v e d i a b e t e s me l 1,
l i t u2)s. (
T h e s p e e d o f t h e o n s e t a n d t h e t y p e o f s y mp t o ms ( s y mme t r i c v s . f o c a l ) c a n b e v e r y
h e l p f u l i n d i a g n o s i n g t h e c a u s e o f a t a xi a .
1. A c o mmo n e xa mp l e o f a c u t e s y mme t r i c a t a xi a i s a l c o h o l i n t o xi c a t i o n w i t h i t s
c l u ms i n e s s , s l u r r e d s p e e c h , u n b a l a n c e d g a i t , n y s t a g mu s , a n d i n a c c u r a t e f i n g e r t o - n o s e t e s t i n g . A n a c u t e a n d s y mme t r i c a l o n s e t w o u l d l e a d a c l i n i c i a n t o
s u s p e c t d r u g s , t o xi n s , o r i n f e c t i o n . E xa mp l e s o f mo r e c h r o n i c s y mme t r i c a l
a t a xi a s i n c l u d e h y p o t h y r o i d i s m, p a r a n e o p l a s t i c d i s o r d e r s , i n h e r i t e d d i s o r d e r s , o
v i t a mi n 1B2 d e f i c i e n c y.
2. A r a p i d b u t f o c a l o n s e t w o u l d l e a d a c l i n i c i a n t o s u s p e c t v a s c u l a r o r i n f e c t i o u s
c a u s e s . F o r e xa mp l e , t h e s u d d e n o n s e t o f r i g h t - s i d e d c l u ms y mo v e me n t s , t h e
i n w a r d g a ze o f t h e r i g h t e y e , a n d a h e a d a c h e w o u l d l e a d o n e t o s u s p e c t a r i g h t s i d e d ( c e r e b e l l a r s y mp t o ms a r e i p s i l a t e r a l ) ma s s i n t h e p o s t e r i o r f o s s a s u c h a s
h e mo r r h a g e o r a b s c e s s .
3. A s l o w e r o n s e t o f f o c a l o r a s y mme t r i c f i n d i n g s w o u l d l e a d o n e t o s u s p e c t
n e o p l a s ms , mu l t i p l e s c l e r o s i s , v a s c u l a r l e s i o n s , o r c o n g e n i t a l ma
1) l. f o r ma t i o n s (
References
1 . R o s e n b e r g R N . A t a xi c d i s o r d e r s . I n : B r a u n w a l d E , H a u s e r S L , F a u c i A S , e d s .
H ar r i s on' s pr i nc i pl es of i nt er nal medi
, 1 5ctihnee d N e w Yo r k , N Y : . M c G r a w - H i l l ,
2001:24062412.
2 . B r e s s ma n S B , S a u n d e r s - P u l l ma n R J , R o s e n b e r g R N . H e r e d i t a r y a t a xi a s . I n :
R o w l a n d L P, eM
d .er r i t t ' s neur ol ogy
, 11 t h e d N e w Yo r k , N Y : . L i p p i n c o t t W i l l i a ms
& Wilkins, 2005:783797.
108 / 652
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4.2 - Coma
> T a b l e o f C o n t e n t s > 4 - P r o b l e ms R e l a t e d t o t h e N e r v o u s S y s t e m > 4 . 2 - C o ma
4.2
Coma
Robe rt R. Raune r
I . Background
C o ma i s a s u s t a i n e d p e r i o d ( > 1 h o u r ) o f u n c o n s c i o u s n e s s t h a t i s d i s t i n g u i s h e d f r o m
s l e e p b y t h e i n a b i l i t y t o a r o u s e t h e 1,2)
p a t i.e n t (
C o ma i s a n o n s p e c i f i c ma n i f e s t a t i o n o f c e n t r a l n e r v o u s s y s t e m ( C N S ) i mp a i r me n t
t h a t ma y b e d u e t o a n y n u mb e r o f i n s u l t s . T h e r e i s a l a r g e d i f f e r e n t i a l o f p o s s i b l e
c a u s e s t h a t ma y b e s u b d i v i d e d i n t o f o c a l ( e . g . , s t r o k e ) v e r s u s n o n f o c a l ( e . g . ,
h y p o xi a ) , t r a u ma t i c v e r s u s n o n t r a u ma t i c , o r C N S v e r s u s s y s t e mi c c a u s e s . T h e e n d
r e s u l t i s t h a t t h e r e i s a g l o b a l d y s f u n c t i o n o f b o t h t h e c e r e b r a l h e mi s p h e r e s o r t h e
a s c e n d i n g b r a i n s t e m a n d d i e n c e p h a l o n a c t i v a t i n g2)s.y s t e ms (
I I I . Evaluation
T h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n c a n o f t e n e l i c i t t h e p o t e n t i a l c a u s e s o f c o ma .
A. History
A f t e r e n s u r i n g t h e s t a b i l i t y o f t h e a i r w a y, b r e a t h i n g , a n d c i r c u l a t i o n ( A B C s ) , i t i s
e s s e n t i a l t o g a t h e r p e r t i n e n t h i s t o r y f r o m f r i e n d s , f a mi l y me mb e r s , a n d a n y me d i c a l
personnel. A sudden loss of consciousness would suggest causes such as
i n t r a c e r e b r a l h e mo r r h a g e , s e i zu r e , c a r d i a c a r r h y t h mi a s , o r d r u g o v e r d o s e . A s l o w e r
p r o g r e s s i o n i mp l i e s a mu c h l a r g e r l i s t o f d i f f e r e n t i a l d i a g n o s e s .
T h e e xa mi n a t i o n s h o u l d p a y p a r t i c u l a r a t t e n t i o n t o t h e f o l l o w i n g :
1. M o t o r r e s p o n s eAsr e t h e r e a n y p u r p o s e f u l mo v e me n t s ? I s t h e r e w i t h d r a w a l
f r o m p a i n ? A n y p o s t u r i n g ? A r e t h e mo v e me n t s s y mme t r i c ? A s y mme t r y ma y i mp l y
a s t r u c t u r a l l e s i o n o r a mo r e f o c a l c a u s e . F l e xo r o r e xt e n s o r p o s t u r i n g i mp l i e s a
i mp a i r me n t o f t h e c e r e b r a l h e mi s p h e r e s p o s s i b l y f r o m me t a b o l i c c a u s e s , b r a i n
s t e m l e s i o n s , o r t r a n s t e n t o r i a l h e r n i a t i o n . P a t i e n t s w i t h f l e xo r p o s t u r i n g g e n e r a l
have a better prognosis.
2. R e s p i r a t i o n
Is there a respiratory effort? If so, what pattern (Cheyne-S tokes,
c l u s t e r b r e a t h i n g , h y p e r v e n t i l a t i o n ) ? C h e y n e - S t o k e s r e s p i r a t i o n i s mo r e l i k e l y
109 / 652
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4.2 - Coma
w i t h t r a n s t e n t o r i a l h e r n i a t i o n , u p p e r b r a i n s t e m l e s i o n s , o r me t a b o l i c c a u s e s .
C l u s t e r b r e a t h i n g ma y b e a s s o c i a t e d mo r e w i t h p o s t e r i o r f o s s a l e s i o n s o r
e l e v a t e d i n t r a c r a n i a l p r e s s u r e . H y p e r v e n t i l a t i o n i s mo r e l i k e l y d u e t o me t a b o l i c
a c i d o s i s , h e p a t i c e n c e p h a l o p a t h y, o r a n a l g e s i c d r u g s .
3. P u p i l l a r y r e s p o n A
s ree t h e p u p i l s e q u a l ? A r e t h e y r e a c t i v e ? A n i s o c o r i a i mp l i e s
a s t r u c t u r a l c a u s e . P i n p o i n t p u p i l s ma y b e f r o m p o n t i n e h e mo r r h a g e s o r d r u g
t o xi c i t y. F i xe d d i l a t e d p u p i l s ma y f o l l o w a n o xi c o r i s c h e mi c i n j u r y.
4. E y e m o v e m e n tAsr e t h e e y e l i d s o p e n ? D o e s t h e p a t i e n t b l i n k ? D o e y e
mo v e me n t s c o n j u g a t e o r d o t h e y d e v i a t e ? D o t h e e y e s mo v e w i t h t h e p a s s i v e
mo v e me n t o f t h e p a t i e n t ' s h e a d ( o c u l o c e p h a l i c o r d o l l ' s e y e1) ma
? neuver)(
5. Te m p e r a t u r eI s i t l o w, n o r ma l , o r e l e v a t e d ? L o w t e mp e r a t u r e w o u l d s u g g e s t
d r u g i n t o xi c a t i o n , h y p o g l y c e mi a , h y p o t h y r o i d i s m, o r e n v i r o n me n3)
t a.l e xp o s u r e (
A f e v e r s u g g e s t s i n f e c t i o u s c a u s e s , h e a t s t r o k e , n e u r o l e p t i c ma l i g n a n t
s y n d r o me , s t a t u s e p i l e p t i c u s , o r a n t i c h o l i n e r g i c 2)
o v. e r d o s e (
C. Testing
T h e i ma g i n g o f t h e h e a d u s i n g e i t h e r a c o mp u t e d t o mo g r a p h y ( C T ) s c a n o r a
ma g n e t i c r e s o n a n c e i ma g i n g ( M R I ) s h o u l d b e d o n e a s q u i c k l y a s p o s s i b l e t o r u l e o u
s t r u c t u r a l c a u s e s a n d t o g u i d e e me r g e n t t r e a t me n t ( e . g . , h e mo r r h a g e o r h e r n i a t i o n ) .
L a b o r a t o r y t e s t i n g s h o u l d i n c l u d e a n a r t e r i a l b l o o d g a s , a c o mp l e t e b l o o d c o u n t , a
c o mp r e h e n s i v e me t a b o l i c p r o f i l e , t o xi c o l o g y ( i n c l u d i n g e t h a n o l , c o mmo n l y a b u s e d
d r u g s , a c e t a mi n o p h e n , a n d s a l i c y l a t e s ) , a mmo n i a , a n d l a c t a t e . B l o o d a n d
cerebrospinal fluid should also be cultured. An electroencephalogram ( E E G ) should
b e p e r f o r me d t o l o o k f o r u n r e c o g n i ze d s e i zu r e s . T h e E E G c a n a l s o g i v e c l u e s t o t h e
c a u s e a n d t h e p r o g n o2)
s i .s (
P. 5 6
I V. Diagnosis
T h e d i f f e r e n t i a l d i a g n o s i s o f c o ma i s b r o a d b u t i s u s u a l l y e s t a b l i s h e d b a s e d o n
h i s t o r y, p h y s i c a l , l a b o r a t o r y f i n d i n g s , E E G , a n d i ma g i n g . T h e d i f f e r e n t i a l d i a g n o s i s
s u b d i v i d e d b a s e d o n n o r ma l v e r s u s a b n o r ma l C T s c a n o r M R I , i n c l u d e s :
1. N o r ma l C T s c a n o r M R I
a. D r u g s / o v e r d o s e : a l c o h o l , s e d a t i v e s , o p i a t e s
b. M e t a b o l i c : a n o xi a , e l e c t r o l y t e d i s t u r b a n c e s , g l u c o s e a b n o r ma l i t i e s , t h y r o i d
d i s o r d e r s , h e p a t i c c o ma
c. S e v e r e i n f e c t i o n s : p n e u mo n i a , me n i n g i t i s , e n c e p h a l i t i s , s e p s i s
d. S h o c k
e. S e i zu r e - r e l a t e d c o n d i t i o n s
f. S e v e r e h y p o t h e r mi a o r h y p e r t h e r mi a
g. C o n c u s s i o n .
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2. A b n o r ma l C T s c a n o r M R I
a. H e mo r r h a g e o r i n f a r c t i o n
b. I n f e c t i o n : a b s c e s s , e mp y e ma
c. B r a i n t u mo r
d. T r a u ma t i c i n j u r i e s
e. O t h e r s 3)( .
T h e p a t i e n t l a c k s s e l f - a w a r e n e s s a n d ma k e s n o p u r p o s e f u l mo v e me n t s . Vi t a l s i g n s ,
i n c l u d i n g t h e a b i l i t y t o ma i n t a i n r e s p i r a t o r y f u n c t i o n ma y b e i mp a i r e d ; s o , i mme d i a t e
a t t e n t i o n t o e n s u r i n g s t a b l e A B C s i s e s s e n t i a l . C o ma mu s t b e d i s t i n g u i s h e d f r o m
o t h e r s i mi l a r c l i n i c a l e n t i t i e s s u c h a s v e g e t a t i v e s t a t e , c a t a t o n i a , s e v e r e d e p r e s s i o n
n e u r o mu s c u l a r b l o c k a d e , o r a k i n e s i a p l u s a1,2)
p h .a s i a (
References
1 . B u r s t J C M . C o ma . I n : R o w l a n d LM
P,ererdi .t t ' s neur ol ogy
, 11 t h e d .
2 . M i c h e l s o n D J , A s h w a l S . E v a l u a t i o n o f c o ma a n d b r S
a iemi
n dn
e aPt hedi
. at r
N eur ol 2 0 0 4 ; 11 ( 2 ) : 1 0 5 11 8 .
3 . R o p p e r A H . A c u t e c o n f u s i o n a l s t a t e s a n d c o ma . I n : B r a u n w a l d E , H a u s e r S L ,
e t a l . e d sH. ar r i s on' s pr i nc i pl es of i nt er nal medi
, 1 5ctihnee d . P h i l a d e l p h i a , PA :
McGraw-Hill, 2001:132140.
111 / 652
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4.3 - Delirium
> T a b l e o f C o n t e n t s > 4 - P r o b l e ms R e l a t e d t o t h e N e r v o u s S y s t e m > 4 . 3 - D e l i r i u m
4.3
Delirium
Le s Ve skrna
I . Background
A c c o r d i n g t o t h e A me r i c a n P s y c h i a t r i c A s s oD
c ii aagnos
t i o n ' st i c and S t at i s t i c al
M anual of M ent al D i s or,der
F osu r t h E d i t i o n ( D S M - I V-1)T ,Rd) e( l i r i u m h a s t h e
following key features: disturbance of consciousness with a reduced ability to focus
s u s t a i n , o r s h i f t a t t e n t i o n ; c h a n g e i n c o g n i t i o n o r t h e d e v e l o p me n t o f a p e r c e p t u a l
d i s t u r b a n c e t h a t i s n o t b e t t e r a c c o u n t e d f o r b y a p r e e xi s t i n g , e s t a b l i s h e d , o r e v o l v i n
d e me n t i a ; d i s t u r b a n c e d e v e l o p i n g o v e r a s h o r t p e r i o d o f t i me ( u s u a l l y h o u r s t o d a y s
a n d t e n d i n g t o f l u c t u a t e d u r i n g t h e c o u r s e o f t h e d a y ; e v i d e n c e f r o m t h e h i s t o r y,
p h y s i c a l e xa mi n a t i o n , o r l a b o r a t o r y f i n d i n g s t h a t t h e d i s t u r b a n c e i s c a u s e d b y t h e
d i r e c t p h y s i o l o g i c c o n s e q u e n c e o f a g e n e r a l me d i c a l c o n d i t i o n , s u b s t a n c e
i n t o xi c a t i o n o r w i t h d r a w a l , a me d i c a t i o n s i d e e f f e c t o r t o xi n e xp o s u r e , o r a
c o mb i n a t i o n o f t h e s e f a c t o r s .
A. Etiology
T h e n e u r o b i o l o g i c me c h a n i s m o f d e l i r i u m i s p o o r l y u n d e r s t o o d , b e c a u s e i t i s n o t a
d i s e a s e b u t a s y n d r o me w i t h mu l t i p l e d i s p a r a t e c a u s e s . T h e c a u s e s
P. 5 7
o f d e l i r i u m c a n b e c a t e g o r i ze d a c c o r d i n g t o w h e t h e r t h e y a r e p r e d i s p o s i n g o r
p r e c i p i t a t i n g f a c t o r s . T h e mo s t c o mmo n p r e d i s p o s i n g f a c t o r s a r e a d v a n c e d a g e a n d
d e me n t i a . O t h e r r i s k f a c t o r s i n c l u d e i mmo b i l i t y a n d f u n c t i o n a l d e p e n d e n c e , s e n s o r y
i mp a i r me n t , d e h y d r a t i o n , ma l n u t r i t i o n , a n d a l c o h o l i s m. N e a r l y a n y a c u t e s y s t e mi c
i l l n e s s o r me d i c a l c o n d i t i o n ma y p r e c i p i t a t e d e l i r i u m i n a s u s c e p t i b l e p a t i e n t . T h e
c o mmo n c a u s e s a r e l i s t eTda bi nl e 4 . 3 . 1
B. Epidemiology
Va r i a t i o n i n c a s e i d e n t i f i c a t i o n a n d s a mp l e b i a s ma k e e s t i ma t e s o f t h e p r e v a l e n c e
a n d i n c i d e n c e o f d e l i r i u m d i f f i c u l t t o d e t e r mi n e . C l i n i c i a n s o f t e n f a i l t o r e c o g n i ze
d e l i r i u m, a l t h o u g h i t i s o n e o f t h e mo s t c o mmo n me n t a l d i s o r d e r s e n c o u n t e r e d i n
p a t i e n t s w i t h me d i c a l i l l n e s s e s , e s p e c i a l l y t h o s e w h o a r e c r i t i c a l l y i l l o r e l d e r l y. M o
s t u d i e s o f mi xe d h o s p i t a l i n p a t i e n t p o p u l a t i o n s r e p o r t p r e v a l e n c e s o f 1 0 % t o 2 0 % .
I I I . Evaluation
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A. History
D e t e r mi n i n g t h a t a c o g n i t i v e i mp a i r me n t o r p e r c e p t u a l d i s t u r b a n c e i s n o t d u e t o a
p r e e xi s t i n g o r p r o g r e s s i n g d e me n t i a o r o t h e r me n t a l d i s o r d e r r e q u i r e s k n o w l e d g e o f
t h e p a t i e n t ' s b a s e l i n e me n t a l s t a t u s , a n d l e v e l o f f u n c t i o n i n g . I f t h i s i s n o t k n o w n ,
i n f o r ma t i o n s h o u l d b e s o u g h t f r o m f a mi l y, f r i e n d s , a n d o t h e r c a r e p r o v i d e r s . B e c a u s
i t i s d i f f i c u l t o r i mp o s s i b l e t o o b t a i n a h i s t o r y f r o m a c o n f u s e d o r u n c o o p e r a t i v e
p a t i e n t , i mp o r t a n t c l u e s a s t o t h e c a u s e o f d e l i r i u m ma y a l s o b e e l i c i t e d f r o m t h e s e
h i s t o r i c a l s o u r c e s ( r e c e n t f e b r i l e i l l n e s s , r e c e n t t r a u ma , h i s t o r y o f d r u g a b u s e o r
a l c o h o l i s m) a n d a c a r e f u l r e v i e w o f t h e me d i c a l h i s t o r y. B e c a u s e d r u g t o xi c i t y
a c c o u n t s f o r a s i g n i f i c a n t p e r c e n t a g e o f a l l c a s e s o f d e l i r i u m, c l i n i c i a n s s h o u l d n o t
n e g l e c t c o n s i d e r i n g o v e r - t h e - c o u n t e r d r u g s , d r u g s b e l o n g i n g t o o t h e r f a mi l y
me mb e r s , d r u g s p r e s c r i b e d b y o t h e r p h y s i c i a n s , o r i l l i c i t d r u g s . B e c a u s e t h e f e a t u r e
o f d e l i r i u m f l u c t u a t e d u r i n g t h e c o u r s e o f t h e d a y, a r e v i e w o f n u r s i n g n o t e s ,
especially from the evening and night shifts, can be very helpful for discovering or
d o c u me n t i n g c h a n g e s i n c o n s c i o u s n e s s a n d c o g n i t i o n .
T h e e xa mi n a t i o n mu s t f o c u s o n t w o i s s u e s : ( i ) c o n f i r mi n g t h a t d e l i r i u m i s p r e s e n t ,
a n d ( i i ) u n c o v e r i n g t h e me d i c a l i l l n e s s t h a t h a s l i k e l y c a u s e d t h e d e l i r i u m. A
c o mp r e h e n s i v e e xa mi n a t i o n i s o f t e n d i f f i c u l t i n a c o n f u s e d a n d u n c o o p e r a t i v e p a t i e n
C l i n i c i a n s s h o u l d p e r f o r m a f o c u s e d e xa mi n a t i o n g u i d e d b y t h e h i s t o r y a n d c o n t e xt ,
k e e p i n g i n mi n d t h e mu l t i f a c t o r i a l n a t u r e o f d e l i r i u m.
C. Testing
T h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n s h o u l d g u i d e mo s t o f t h e d i a g n o s t i c
i n v e s t i g a t i o n . F i r s t - l i n e i n v e s t i g a t i o n s s h o u l d i n c l u d e e l e c t r o l y t e s , c o mp l e t e b l o o d
c o u n t , u r i n a l y s i s , l i v e r a n d t h y r o i d f u n c t i o n t e s t s , g l u c o s e , c r e a t i n i n e , c a l c i u m, c h e s
x- r a y, a n d e l e c t r o c a r d i o g r a m. B l o o d g a s d e t e r mi n a t i o n s a r e o f t e n h e l p f u l . D r u g l e v e
can be obtained when appropriate, but the clinician should be aware that delirium
c a n o c c u r e v e n w i t h t h e r a p e u t i c l e v e l s . T h e f o l l o w i n g d i a g n o s t i c t e s t s ma y b e
indicated when a cause of delirium is not apparent after the initial evaluation: urine
a n d b l o o d t o xi c o l o g y s c r e e n , s y p h i l i s s e r o l o g y, h u ma n i mmu n o d e f i c i e n c y v i r u s
a n t i b o d y, a u t o a n t i b o d y s c r e e n , v i t1a2mil env eBl , h e a d c o mp u t e d t o mo g r a p h y o r
ma g n e t i c r e s o n a n c e i ma g i n g , a l u mb a r p u n c t u r e w i t h c e r e b r o s p i n a l a n a l y s i s , a n d
electroencephalogram testing.
I V. Diagnosis
T h e mo s t c o mmo n i s s u e i n t h e d i f f e r e n t i a l d i a g n o s i s i s w h e t h e r t h e p a t i e n t h a s
d e me n t i a r a t h e r t h a n d e l i r i u m, h a s d e l i r i u m o n l y, o r a d e l i r i u m s u p e r i mp o s e d o n a
p r e e xi s t i n g d e me n t i a . C a r e f u l a t t e n t i o n t o t h e k e y f e a t u r e s ( d i s t u r b e d c o n s c i o u s n e s
change in cognition or perceptual disturbance, acute onset, and fluctuating course)
s h o u l d r e a d i l y d i s t i n g u i s h d e l i r i u m f r o m d e me n t i a a n d o t h e r p r i ma r y p s y c h i a t r i c
d i s o r d e r s s u c h a s d e p r e s s i o n , p s y c h o s i s , o r ma n i a . N o n c o n v u l s i v e s t a t u s e p i l e p t i c u
a n d s e v e r a l l o b a r o r f o c a l n e u r o l o g i c s y n d r o me s ( We r n i c k e ' s a p h a s i a , t r a n s i e n t
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4.3 - Delirium
g l o b a l a mn e s i a , A n t o n ' s s y n d r o me , f r o n t a l l o b e t u mo r s ) c a n r e s u l t i n f e a t u r e s t h a t
ma y o v e r l a p w i t h t h o s e o f d e l i r i u m.
Engaging in conversation with a patient in delirium can be difficult because he or s

ma y b e c o me e a s i l y d i s t r a c t e d , u n p r e d i c t a b l y s w i t c h f r o m s u b j e c t t o s u b j e c t , o r
p e r s e v e r e w i t h a n s w e r s t o a p r e v i o u s q u e s t i o n . I n mo r e a d v a n c e d c a s e s o f d e l i r i u m
t h e p a t i e n t ma y b e d r o w s y o r l e t h a r g i c . C o g n i t i v e c h a n g e s ma y i n c l u d e me mo r y
i mp a i r me n t ( mo s t c o mmo n l y s h o r t - t e r m
P. 5 8
P. 5 9
me mo r y ) , d i s o r i e n t a t i o n ( u s u a l l y t o t i me a n d p l a c e ) , d i f f i c u l t y w i t h l a n g u a g e o r
s p e e c h ( d y s a r t h r i a , d y s n o mi a , d y s g r a p h i a , o r a p h a s i a ) , a n d p e r c e p t u a l d i s t u r b a n c e s
( i l l u s i o n s , h a l l u c i n a t i o n s , o r mi s p e r c e p t i o n s ) . T h e p a t i e n t ma y b e s o i n a t t e n t i v e a n d
i n c o h e r e n t t h a t i t ma y b e d i f f i c u l t o r i mp o s s i b l e t o a s s e s s c o g n i t i v e f u n c t i o n . O t h e r
a s s o c i a t e d f e a t u r e s o f d e l i r i u m ma y i n c l u d e s l e e p d i s t u r b a n c e o r a r e v e r s a l o f t h e
n i g h t - d a y s l e e p - w a k e c y c l e , h y p e r s e n s i t i v i t y t o l i g h t a n d s o u n d , a n xi e t y, a n g e r,
d e p r e s s e d a f f e c t , a n d e mo t i o n a l l a b i l i t y. B e c a u s e o f c o n f u s i o n , d i s o r i e n t a t i o n , a n d
a g i t a t i o n , p a t i e n t s w i t h d e l i r i u m ma y h a r m t h e ms e l v e s b y c l i mb i n g o v e r b e d r a i l s o r
p u l l i n g o u t t h e i r i n t r a v e n o u s l i n e o r F o l e y c a t h e t e r.
TAB L E 4.3 Com m on Causes of Delirium

A n e mi a
H y p o xe mi a
Congestive heart failure
C h r o n i c o b s t r u c t i v e p u l mo n a r y d i s e a s e
Shock
Infections
P n e u mo n i a
S e p t i c e mi a
Meningitis
Urinary tract infection
Central nervous system disorders
Cerebrovascular accidents
S e i zu r e s o r p o s t i c t a l s t a t e
H e a d t r a u ma
Increased intracranial pressure
Withdrawal from substances
Alcohol
B e n zo d i a ze p i n e s
Opiates
Metabolic disorders
Renal failure, fluid/electrolyte disorder
Acidbase disorders
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4.3 - Delirium
Endocrinopathy
Hepatic failure
H y p e r g l y c e mi a o r h y p o g l y c e mi a
T h i a mi n e d e f i c i e n c y
Other
F e c a l i mp a c t i o n s
B l a d d e r c a t h e t e r i za t i o n
Urinary retention
Physical restraints
Medications
Psychotropic drugs
Drugs with anticholinergic effects
Tricyclic antidepressants
D i p h e n h y d r a mi n e
B e n zt r o p i n e
References
on.
di s or der, s4 t h e d , T e xt R e v i s i o n . Wa s h i n g t o n , D C : A me r i c a n P s y c h i a t r i c
Association, 2000.
115 / 652
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4.4 - Dementia
> T a b l e o f C o n t e n t s > 4 - P r o b l e ms R e l a t e d t o t h e N e r v o u s S y s t e m > 4 . 4 D e me n t i a
4.4
Dementia
Christian Ky le Hae fe le
I . Background
D e me n t i a i s c h a r a c t e r i ze d b y me mo r y i mp a i r me n t a l o n g w i t h t h e l o s s o f o t h e r
c o g n i t i v e f u n c t i o n s . C o g n i t i v e d e f i c i t s ma y i n c l u d e d i f f i c u l t y w i t h l a n g u a g e ( a p h a s i a
c o mmo n mo t o r t a s k s ( a p r a xi a ) , t h e i d e n t i f i c a t i o n o f c o mmo n o b j e c t s ( a g n o s i a ) , o r
c o mp l e x a n d a b s t r a c t t h i n k i n g ( e xe c u t i v e f u n c t i o n i n g ) . T h e s e d e f i c i t s mu s t b e s e v e r
e n o u g h t h a t s o c i a l b e h a v i o r o r i n d e p e n d e n t l i v i n g i s1)i.mp a i r e d (
A. Etiology
D e me n t i a i s a s y n d r o me r a t h e r t h a n a d i s e a s e , s o e t i o l o g y a n d p a t h o p h y s i o l o g y c a n
v a r y g r e a t l y. M o s t o f t h e c o mmo n d e me n t i a s a r e p r o g r e s s i v e , b u t s o me a r e d u e t o
reversible causes.
1. A l zh e i me r ' s d i s e a s e ( A D ) i s mu l t i f a c t o r i a l . T h e r e i s b o t h a f a mi l i a l p r e d i s p o s i t i o
a n d s o me e v i d e n c e t h a t A D h a s a s i g n i f i c a n t v a s c u l a r c o mp o n e n t , b e c a u s e t h e r e
a r e ma n y r i s k f a c t o r s f o r p r o g r e s s i o n i n c o mmo n w i t h v a s c u l 2)
a r . d e me n t i a (
2. Va s c u l a r d e me n t i a c a n b e d u e t o mu l t i p l e i n f a r c t s o r s ma l l v e s s e l d i s e a s e .
3. D e me n t i a s y n d r o me s c a n b e f o u n d i n c o n n e c t i o n w i t h o t h e r d e g e n e r a t i v e
n e u r o l o g i c d i s e a s e s s u c h a s P a r k i n s o n ' s d i s e a s e w i t h d e me n t i a ( P D D ) ,
f r o n t o t e mp o r a l d e me n t i a ( F T D ) , L e w y b o d y d e me n t i a ( L B D ) , H u n t i n g t o n ' s
disease and progressive supranuclear palsy (P S P).
4. I n f e c t i o u s d i s e a s e s s u c h a s n e u r o s y p h i l i s , a c q u i r e d i mmu n o d e f i c i e n c y s y n d r o me
d e me n t i a , a n d C r e u t zf e l d t - J a c o b d i s e a s e ( C J D ) c a n p r e s e n t w i t h d e me n t i a .
However any infection, such as urinary tract infection, can cause a patient with
d i mi n i s h e d r e s e r v e t o d e c o mp e n s a t e a n d p r e s e n t w i t h d e me n t i a .
5. O t h e r d e me n t i a s a r e f o u n d w i t h me t a b o l i c d i s e a s e s s u c h a s G a u c h e r ' s d i s e a s e
o r w i t h t o xi n s s u c h a s a l c o h o l - r e l a t e d d e me n t i a .
6. N o r ma l p r e s s u r e h y d r o c e p h a l u s i s a p o t e n t i a l l y r e v e r s i b l e d e me n t i a , w h i c h i s
c h a r a c t e r i ze d b y t h e t r i a d o f d e me n t i a , g a i t i n s t a b i l i t y, a n d u r i n a r y i n c o n t i n e n c e
7. O t h e r r e v e r s i b l e d e me n t i a s c a n b e d u e t o a v a r i e t y o f me d i c a l c o n d i t i o n s s u c h
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4.4 - Dementia
a s h y p o t h y r o i d i s m o r v i t a mi
1 2nd eBf i c i e n c y. D e me n t i a c a n b e d u e t o e l e c t r o l y t e
i mb a l a n c e , h y p o g l y c e mi a , h e p a t i c , o r r e n a l d y s f u n c t i o n , h y p o xi a d u e t o c a r d i a c
o r p u l mo n a r y d i s e a s e , d e p r e s s i o n , d r u g s , o r t r a u ma .
B. Epidemiology
1. T h e mo s t c o mmo n c h r o n i c d e me n t i a s y n d r o me i n t h e e l d e r l y i s A D , a c c o u n t i n g
f o r a n e s t i ma t e d 5 0 % t o 6 0 % o f d e me n t i a s o r o v e r f o u r mi l l i o n3) A
. me r i c a n s (
2. C h r o n i c d e me n t i a i s d u e t o v a s c u l a r d e me n t i a i n a b o u t 1 5 % t o 2 0 % o f c a s e s .
T h e o t h e r n e u r o d e g e n e r a t i v e d e me n t i a s a r e l e s s 3)c .o mmo n (
P. 6 0
3. R e v e r s i b l e d e me n t i a s a r e mo r e l i k e l y t o b e f o u n d i n y o u n g e r p a t i e n t s a n d ma y
h a v e a r a p i d o n s e t me a s u r e d i n d a y s o r w e e k s r a t h e r t h a n mo n t h s o r y e a r s .
I I I . Evaluation
A. History and physical examination
T h e h i s t o r y a n d p h y s i c a l f i n d i n g s ma k e t h e d i a g n o s i s o r h e l p d i r e c t t h e w o r k u p o f a
p o t e n t i a l l y r e v e r s i b l e d e me n t i a . A M i n i - M e n t a l S t a t e E xa mi n a t i o n ( M M S E ) ( s e e
C h a p t e r 4 ). ,5 c o mp l e t e n e u r o l o g i c e xa mi n a t i o n , a n d d e p r e s s i o n s c r e e n s h o u l d b e
p e r f o r me d . A r e f e r r a l f o r f o r ma l n e u r o p s y c h o l o g i c t e s t i n g ma y b e c o n s i d e r e d i n
p a t i e n t s w h o a r e d i f f i c u l t t o e v a l u a t e b e c a u s e o f a l a n g u a g e b a r r i e r, s u s p e c t e d
psychiatric diagnosis, education level, or upon request.
B. Testing
1. T h e A me r i c a n A c a d e my o f N e u r o l o g y ( A A N ) r e c o mme n d s r o u t i n e t e s t i n g o n l y f o r
v i t a mi n 1B2 d e f i c i e n c y a n d h y p o t h y r o i d i s m a s c a u s e s o f d4)
e me
. ntia (
2. O t h e r l a b o r a t o r y t e s t s t h a t ma y b e h e l p f u l i f c l i n i c a l l y i n d i c a t e d d u e t o a
s u s p e c t e d me d i c a l c o n d i t i o n c o n t r i b u t i n g t o d e me n t i a , i n c l u d e c o mp l e t e b l o o d
c o u n t , u r i n a l y s i s , l i p i d p a n e l , c o a g u l a t i o n s t u d i e s , c o mp r e h e n s i v e me t a b o l i c
p a n e l , t o xi c o l o g y, h u ma n i mmu n o d e f i c i e n c y v i r u s , L y me d i s e a s e t i t e r, o r
Ve n e r e a l D i s e a s e R e s e a r c h L a b o r a t o r y t e s t . A l u mb a r p u n c t u r e i s n o t r o u t i n e l y
r e c o mme n d e d .
3. A n o n c o n t r a s t h e a d c o mp u t e d t o mo g r a p h y o r ma g n e t i c r e s o n a n c e i ma g i n g i s
r e c o mme n d e d f o r i n i t i a l e v a l u a t i o n . P o s i t r o n e mi s s i o n t o mo g r a p h y, s i n g l e - p h o t o n
e mi s s i o n c o mp u t e d t o mo g r a p h y, a n d o t h e r i ma g i n g a r e i n v e s t i g a t i o n a l a n d a r e
n o t r e c o mme n d e4)
d .(
C. Genetics
T h e mo s t s t u d i e d g e n e t i c ma r k e r f o r d e me n t i a i s a p o l i p o p r o t e i n E e p s i l o n 4
a s s o c i a t e d w i t h A D . T h e A A N g u i d e l i n e s c u r r e n t l y r e c o mme n d a g a i n s t r o u t i n e t e s t i n
f o r a n y g e n e t i c ma r k e r s f o r d e me n t i a s y n d r o me s e xc e p t f o r s p e c i f i c c e r e b r o s p i n a l
f l u i d p r o t e i n s f o u n d i n c a s e s o f s u s p e c t e4)d. C J D (
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4.4 - Dementia
I V. Diagnosis
A n y r e v e r s i b l e d e me n t i a s s h o u l d b e c o n s i d e r e d . T h e d i a g n o s i s o f c h r o n i c d e me n t i a
s y n d r o me s i s b a s e d ma i n l y o n c l i n i c a l ma n i f e s t a t i o n s .
1. T h e d i a g n o s i s o f A D i s c l i n i c a l l y b a s e d o n a s l o w l y p r o g r e s s i v e d e me n t i a n o t
d u e t o o t h e r d i s e a s e s , a n d n o t e xc l u s i v e l y d u r i n g a p e r i o d o f d e l i r i u m.
2. A s t r o k e s y n d r o me o r s u d d e n o r s t e p w i s e d e t e r i o r a t i o n ma y s u g g e s t v a s c u l a r
d e me n t i a .
3. F i n d i n g s o f P a r k i n s o n ' s d i s e a s e ma y i n d i c a t e P D D o r L B D . I n P D D t h e mo t o r
f e a t u r e s a r e mo s t p r o mi n e n t , w h e r e a s i n L B D t h e ma i n f e a t u r e s a r e s l e e p
disturbance, syncope, falls, and hallucinations.
4. I n F T D , a l s o k n o w n a s P i c k ' s d i s e a s e , p a t i e n t s h a v e s o c i a l l y i n a p p r o p r i a t e
u n i n h i b i t e d b e h a v i o r o r p e r s o n a l i t y c h a n g e s , b u t me mo r y ma y b e f a i r l y w e l l
ma i n t a i n e d .
5. P a t i e n t s w i t h P S P h a v e d o w n w a r d g a ze a b n o r ma l i t i e s a n d f r e q u e n t f a l l s .
6. P s e u d o d e me n t i a i s c o mmo n w i t h d e p r e s s i o n , a s w e l l a s w i t h o t h e r p s y c h i a t r i c
diagnoses.
D e me n t i a , w h e t h e r mi l d , o r a d v a n c e d , o f t e n p r e s e n t s a s a c o mp l a i n t f r o m f a mi l y. I f
p a t i e n t p r e s e n t s w i t h t h e c o mp l a i n t o f me mo r y l o s s , i t i s i mp o r t a n t t o c o n s i d e r
d e p r e s s i o n , f a c t i t i o u s d i s o r d e r s s u c h a s ma l i n g e r i n g , mi l d c o g n i t i v e i mp a i r me n t , o r
n o r ma l a g e r e l a t e d c o g n i t i v e d e c l i nCeh (aspet e r 4 ). .5 A h i g h i n d e x o f s u s p i c i o n , a
g o o d h i s t o r y f r o m t h o s e w h o k n o w t h e p a t i e n t w e l l , a n d f o l l o w i n g s e r i a l e xa mi n a t i o n
a n d M M S E s c o r e s h e l p ma k e t h e d i a g n o s i s .
References
on.
di s or der, s4 t h e d . Wa s h i n g t o n , D C : A PA P r e s s , 1 9 9 4 .
2 . S a d o w s k i M , P a n k i e w i c z J , S c h o l t zo v a H , e t a l . L i n k s b e t w e e n t h e p a t h o l o g y
o f A l zh e i me r ' s d i s e a s e a n d v a s c u l a r d eNme
eur
n toc
i a .hem R es
2004;29(6):1257
1266.
3 . A d e l ma n A M , D a l y M P. I n i t i a l e v a l u a t i o n o f t h e p a t i e n t w i t h s u s p e c t e d
d e me n t i aA. m F am P hy s i c i an
2005;71(9):17451750.
4 . K n o p ma n D S , D e K o s k y S T , C u mmi n g s J L , e t a l . P r a c t i c e p a r a me t e r :
d i a g n o s i s o f d e me n t i a ( a n e v i d e n c e - b a s e d r e v i e w ) : r e p o r t o f t h e q u a l i t y
s t a n d a r d s s u b c o mmi t t e e o f t h e A me r i c a n A c a d e my o f NNeeur
u r ooll oogy
g y.
2 0 0 1 ; 5 6 ( 9 ) : 11 4 3 11 5 3 .
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4.4 - Dementia
119 / 652
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4.5 - Memory Impairment
> T a b l e o f C o n t e n t s > 4 - P r o b l e ms R e l a t e d t o t h e N e r v o u s S y s t e m > 4 . 5 - M e mo r y

I mp a i r me n t
4.5
M emory Impairment
Christian Ky le Hae fe le
I . Background
M e mo r y i mp a i r me n t r e f e r s t o t h e i n a b i l i t y t o l e a r n n e w i n f o r ma t i o n o r r e c a l l
p r e v i o u s l y l e a r n e d i n f o r ma t i o n . I t c a n b e a c o mp o n e n t o f d e l i r i u m w h e n a c c o mp a n i e
b y a n a l t e r e d l e v e l o f c o n s c i o u s n e sCsh (aspet e r 4 ). 3o r a c o mp o n e n t o f d e me n t i a
i n p a t i e n t s w i t h d i s t u r b a n c e s i n b e h a v i o r, o t h e r c o g n i t i v e f u n c t i o n s , a n d
i n d e p e n d e n c e ( sC
e eh a p t e r 4 ). .4 T h e r e i s a n o r ma l c o g n i t i v e d e c l i n e w i t h a g i n g t h a t
c o n s i s t s o f a s t a b l e mi l d me mo r y l o s s a n d a d e c l i n e i n t h e r a t e o f p r o c e s s i n g n e w
i n f o r ma t i o n . T h i s n o r ma l c o g n i t i v e d e c l i n e d o e s n o t p r o g r e s s t o t h e p o i n t o f a f f e c t i n
daily function.
A. Etiology
1. M e mo r y d i s o r d e r s w i t h o u t d e l i r i u m o r d e me n t i a aamnes
r e c a ltliecd di s or der
. s
T h e y a r e u s u a l l y d u e t o t h e e f f e c t s o f o t h e r me d i c a l c o n d i t i o n s , me d i c a t i o n s ,
t o xi n s , o r d r u g s o f a b u1)s.e O( f t e n t h e r e i s a p e r i o d o f d e l i r i u m t h a t p r e c e d e s
a mn e s t i c d i s o r d e r s . F o r e xa mp l e , i n f e c t i o u s e n c e p h a l i t i s a n d a l c o h o l u s e c a n
b o t h c a u s e a n i n i t i a l p e r i o d o f d e l i r i u m f o l l o w e d b y me mo r y p r o b l e ms , w h i c h
r e ma i n a f t e r t h e l e v e l o f c o n s c i o u s n e s s h a s r e t u r n e d t o n o r ma l .
2. M e mo r y d i s t u r b a n c e c a n b e c a u s e d b y p h y s i c a l t r a u ma s u c h a s h e a d i n j u r y o r
e mo t i o n a l t r a u ma , w h i c h ma y c a u s e a r a r e , b u t d r a ma t i c p r e s e n t a t i o n w i t h o n e o
the dissociative psychiatric disorders, such as a conversion reaction or fugue
s t a t e 1)
( .
3. M i l d c o g n i t i v e i mp a i r me n t ( M C I ) i s t h e t e r m u s e d f o r me mo r y l o s s t h a t i s mo r e
t h a n i s u s u a l w i t h n o r ma l a g i n g , b u t d o e s n o t me e t t h e c r i t e r i a f o r d e me n t i a . M C
i s c h a r a c t e r i ze d b y i n c r e a s i n g d i f f i c u l t y w i t h me mo r y a n d p r o g r e s s i v e d e c l i n e ,
w h i l e ma i n t a i n i n g o t h e r c o g n i t i v e f u n c t i o n s a n d a c t i v i t i e s o 2)
f d. aAi lsy l i v i n g (
t h e d e f i n i t i v e c r i t e r i a f o r t h i s t e r m i mp r o v e s , i t ma y b e c o me a mo r e u s e f u l c l i n i c
p r e d i c t o r o f p r o g r e s s i o n t o d e me n t i a .
B. Epidemiology
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It is difficult to quantify the prevalence of M C I because the criteria are not uniform
a n d ma n y p a t i e n t s ma y n o t s e e k p r o f e s s i o n a l c a r e . P a t i e n t s w i t h M C I ma y p r o g r e s s
t o d e me n t i a a t a r a t e t h a t i s f o u r t i me s h i g h e r t h a n t h a t o f n o r ma l p e e r s , a l t h o u g h
t h e r e a r e l i mi t e d s t u d i e s t o h e l p i d e n t i f y w h o i s l i k e l y 3)
t o . pTr oh ge r pe rses v (a l e n c e
o f p u r e a mn e s t i c s y n d r o me s d e p e n d s o n t h e e t i o l o g y, b u t t h e y a r e n o t a s c o mmo n a
d e me n t i a s , w h i c h a r e d i s c u s sCehdaipnt e r 4 . 4
I I I . Evaluation
A. History
T h e h i s t o r y s h o u l d i n i t i a l l y f o c u s o n r u l i n g o u t d e me n t i a a n d d e l i r i u m. C a r e f u l d r u g
h i s t o r y mu s t i n c l u d e o v e r - t h e - c o u n t e r me d i c i n e s , w h i c h ma y h a v e c e n t r a l n e r v o u s
s y s t e m s i d e e f f e c t s . I t i s i mp o r t a n t t o o b t a i n c o r r o b o r a t i n g h i s t o r y f r o m f a mi l y a l s o .
P a t i e n t s w i t h d e me n t i a a r e o f t e n b r o u g h t i n b y r e l a t i v e s w h o a r e c o n c e r n e d a b o u t
their function, but the patients have poor insight into their own deficits. T hese
p a t i e n t s a r e d i f f e r e n t f r o m p a t i e n t s w i t h n o r ma l a g e r e l a t e d c o g n i t i v e d e c l i n e , M C I ,
d e p r e s s i o n w h o w i l l o f t e n c o mp l a i n a b o u t t h e i r o w n f o r g e t f u l n e s s .
A c o mp l e t e p h y s i c a l e xa mi n a t i o n , i n c l u d i n g a n e u r o l o g i c e xa mi n a t i o n , s h o u l d b e
p e r f o r me d .
1. A M i n i - M e n t a l S t a t e E xa mi n a t i o n ( M M S E ) t a k e s o n l y a f e w mi n u t e s t o p e r f o r m.
I t c a n h e l p i d e n t i f y me mo r y d e f i c i t s a s w e l l a s d i s c e r n n o n - me mo r y c o g n i t i v e
p r o b l e ms , w h i c h a i d s i n d i f f e r e n t i a l d i a4)g.n oPsai tsi e(n t s w i t h d e p r e s s i o n ma y
s c o r e w e l l o r ma y s h o w p o o r e f f o r t r a t h e r t h a n g i v e i n c o r r e c t a n s w e r s . P a t i e n t s
w i t h d i s s o c i a t i v e d i s o r d e r s ma y b e a b l e t o s c o r e w e l l a s t h e i r me mo r y l o s s ma y
o n l y b e f o r a s p e c i f i c t i me o r s i t u a t i o n . I f c o g n i t i v e d e f i c i t s o t h e r t h a n me mo r y
a r e f o u n d , d e me n t i a mu s t b e s t r o n g l y c o n s i d e r e d . A s c o r e o f 2 4 / 3 0 p o i n t s o r
g r e a t e r i s c o n s i d e r e d n o r ma l , b u t ma y b e a d j u s t e d f o r a g e o r e d u c a t i o n a l l e v e l .
A copy of the
P. 6 2
M M S E a n d a d i s c u s s i o n o f t h e s c o r i n g n o r ms a s t h e y r e l a t e t o a g e a n d
education level is available on-line at
h t t p : / / w w w. a a f p . o r g / a f p / 2 0 0 1 0 2 1 5 / 7 05)
3 ..h t ml (
2. I n s t r u c t i o n s f o r p e r f o r mi n g t h e M M S E :
a. O r i e n t a t i o n t o t i m
F ier s t , a s k t h e p a t i e n t t h e d a y, d a t e , mo n t h , y e a r, a n d
s e a s o n . T h e ma xi mu m s c o r e i s 5 .
b. O r i e n t a t i o n t o p l aSce c o n d , a s k t h e p a t i e n t w h a t b u i l d i n g , t o w n , c o u n t y,
s t a t e , a n d c o u n t r y t h e y a r e i n . T h e ma xi mu m s c o r e i s 5 .
c. M e m o r y r e g i s t r a t i S
o an y t h e n a me o f t h r e e o b j e c t s ( e . g . , c u p , f l a g , d o o r ) ,
a n d a s k t h e p a t i e n t t o r e p e a t t h e m. I f t h e y mi s s a n y, c o n t i n u e u n t i l t h e y a r e
a b l e t o r e p e a t t h e w o r d s s o t h a t r e c a l l c a n b e t e s t e d l a t e r. T h e ma xi mu m
score of 3 is based on the first trial.
d. A t t e n t i o nA s k t h e p a t i e n t t o s p e l l t h e w o r d w o r l d b a c k w a r d s o r t o s u b t r a c t
121 / 652
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7 f r o m 1 0 0 s e r i a l l y b a c k w a r d s ( s t o p a f t e r f i v e a n s w e r s ) . T h e ma xi mu m s c o r e
is 5.
e. M e m o r y r e c aAl ls k t h e p a t i e n t t o r e me mb e r t h e t h r e e o b j e c t s f r o m t h e
r e g i s t r a t i o n p o r t i o n o f t h e t e s t . T h e ma xi mu m s c o r e i s 3 .
f. L a n g u a g e
i. A g n o s i aS h o w t h e p a t i e n t a p e n c i l a n d a s k t h e m t o i d e n t i f y t h e o b j e c t .
T h e ma xi mu m s c o r e i s 2 .
i i. A p h a s i aA s k t h e p a t i e n t t o r e p e a t t h e p h r a s e n o i f s , a n d s , o r b u t s .
A n y mi s t a k e o r s t a r t i n g o v e r o n t h e f i r s t t r y s c o r e s 0 . T h e ma xi mu m
score is 1.
i i i. A p h a s i aWr i t e t h e p h r a s e c l o s e y o u r e y e s o n a b l a n k s h e e t o f p a p e r.
A s k t h e p a t i e n t t o r e a d a n d o b e y t h e c o mma n d . T h e ma xi mu m s c o r e i s
1.
i v. A p r a x i aA s k t h e p a t i e n t t o f o l l o w a 3 - s t e p c o mma n d . P i c k u p t h i s
p a p e r w i t h y o u r r i g h t h a n d , f o l d i t i n h a l f , a n d p l a c e i t o n t h e f l o o r. T h e
ma xi mu m s c o r e i s 3 .
v. A g r a p h i aA s k t h e p a t i e n t t o w r i t e a s e n t e n c e . I t mu s t h a v e a s u b j e c t
a n d a v e r b a n d ma k e s e n s e . T h e ma xi mu m s c o r e i s 1 .
g. V i s u a l - s p a t i a l a w a r e n A
e s ks t h e p a t i e n t t o c o p y a s e t o f i n t e r l o c k i n g
p e n t a g o n s . A l l t e n a n g l e s mu s t b e p r e s e n t , a n d o n e a n g l e o f e a c h f i g u r e
s h o u l d i n t e r s e c t w i t h t h e o t h e r f i g u r e . T h e ma xi mu m s c o r e i s 1 .
C. Testing
T h e r e a r e n o s p e c i f i c l a b o r a t o r y t e s t s f o r me mo r y i mp a i r me n t . A n y t e s t s s h o u l d b e
u s e d t o c o n f i r m o r r u l e o u t s u s p e c t e d me d i c a l c a u s e s o f a mn e s t i c d i s o r d e r s . I f e a r l y
d e me n t i a o r d e l i r i u m i s s u s p e c t e d , t h e w o r k u p c a n b e f o u n d i n C h a p t e r s 4 . 3 o r 4 . 4 .
D. Genetics
A f a mi l y h i s t o r y ma y u n c o v e r a p r e d i l e c t i o n f o r s u b s t a n c e a b u s e , d e p r e s s i o n , o r a
d e me n t i a s y n d r o me . T h e r e a r e n o s p e c i f i c g e n e t i c t e s t s f o r d i s o r d e r s o f me mo r y
i mp a i r me n t . G e n e t i c t e s t i n g i s i n v e s t i g a t i o n a l f o r a n u mb e r o f d e me n t i a s y n d r o me s
i n c l u d i n g A l zh e i me r ' s d e me n t i a , b u t n o n e i s c u r r e n t l y r e c o mme n d e d f o r c l i n i c a l u s e .
I V. Diagnosis
1. N o r ma l a g e r e l a t e d c o g n i t i v e d e c l i n e d o e s n o t p r o g r e s s o v e r t i me t o i mp a i r d a i l y
function. Ask about the onset and course of the problem and follow serial
cognitive function testing when in doubt.
2. D e me n t i a s y n d r o me s i n c l u d e me mo r y i mp a i r me n t , b u t mu s t a l s o a f f e c t o t h e r
c o g n i t i v e f u n c t i o n s t o t h e e xt e n t t h a t a c t i v i t i e s o f d a i l y l i v i n g a r e p r o g r e s s i v e l y
i mp a i r e d ( s eCeh a p t e r 4 ). .4
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3. D e l i r i u m mu s t b e c o n s i d e r e d i f t h e p a t i e n t ' s l e v e l o f c o n s c i o u s n e s s i s a f f e c t e d
( s e e C h a p t e r 4 ). .3
4. P s e u d o d e me n t i a i s s e e n w i t h d e p r e s s i o n . P a t i e n t s ma y s h o w p o o r e f f o r t o n
c o g n i t i v e t e s t i n g a s o p p o s e d t o i n c o r r e c t a n s w e r s . A f o r ma l d e p r e s s i o n s c r e e n
ma y b e h e l p f u l .
5. M a l i n g e r i n g o r f a c t i t i o u s d i s o r d e r ma y p r e s e n t a s me mo r y i mp a i r me n t i n t h e r i g h
s o c i a l o r w o r k s e t t i n g . F o r ma l c o g n i t i v e t e s t i n g ma y r e v e a l i n c o n s i s t e n t d e f i c i t s .
M e mo r y i mp a i r me n t mo s t c o mmo n l y p r e s e n t s w i t h a p a t i e n t c o mp l a i n i n g o f n o r ma l
a g e r e l a t e d c o g n i t i v e d e c l i n e o r M C I i f i t i s p r o g r e s s i v e . M e mo r y l o s s ma y p e r s i s t
a f t e r a n i d e n t i f i e d i l l n e s s , t r a u ma , o r i n t o xi c a t i o n . A
P. 6 3
p a t i e n t w i l l r a r e l y p r e s e n t w i t h a d i s s o c i a t i v e d i s o r d e r i n w h i c h s p e c i f i c me mo r i e s a
l o s t , w h e r e a s c o g n i t i v e t e s t i n g u s u a l l y r e ma i n s n o r ma l .
References
on.
di s or der, s4 t h e d . Wa s h i n g t o n , D C : A PA P r e s s , 1 9 9 4 .
2 . W i n b l a d B , P a l me r K , K i v i p e l t o M , e t a l . M i l d c o g n i t i v e i mp a i r me n t b e y o n d
c o n t r o v e r s i e s , t o w a r d s a c o n s e n s u s : r e p o r t o f t h e I n t e r n a t i o n a l Wo r k i n g G r o u p o n
M i l d C o g n i t i v e I mp a i r me
J nI nt
t . er n M ed
2004;256(3):240246.
3 . G a n g u l i M , D o d g e H H , S h e n C , e t a l . M i l d c o g n i t i v e i mp a i r me n t , a mn e s t i c
t y p e : a n e p i d e mi o l o g i c s tNueur
d y. ol ogy2 0 0 4 ; 6 3 : 11 5 .
4 . F o l s t e i n M F, F o l s t e i n S E , M c H u g h P R . M i n i - me n t a l s t a t e : a p r a c t i c a l me t h o d
f o r g r a d i n g t h e c o g n i t i v e s t a t e o f p a t i e n t s f o r t h eJ cPl si nyicchi
i aat
n .r R es
1975;12:196198.
5 . S a n t a c r u z K S , S w a g e r t y D . E a r l y d i a g n o s i s o f dAemmeFnam
t i a .P hy s i c i an
2001;63(4):620626.
123 / 652
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4.6 - Paresthesia and Dysesthesia
> T a b l e o f C o n t e n t s > 4 - P r o b l e ms R e l a t e d t o t h e N e r v o u s S y s t e m > 4 . 6 Paresthesia and Dysesthesia
4.6
Paresthesia and Dysesthesia
Robe rt R. Raune r
I . Background
Paresthesia is a skin sensation, such as burning, prickling, itching, or tingling, with

n o a p p a r e n t p h y s i c a l c a u s e . D y s e s t h e s i a i s d e f i n e d a s e i t h e r t h e i mp a i r me n t o f
sensation, especially that of touch, or a condition in which an unpleasant sensation
i s p r o d u c e d b y o r d i n a r y s t i mu l i .
A. Etiology
Paresthesias and dysesthesias are due to dysfunction of the nervous system that
can occur anywhere along the pathway of sensation between the cortex and the
s e n s o r y r e c e p t o r. D y s f u n c t i o n c a n b e r e l a t e d t o e i t h e r l a c k o f f u n c t i o n ( e . g . ,
n u mb n e s s d u e t o c a r p a l t u n n e l s y n d r o me ) o r e xc e s s f u n c t i o n ( e . g . , p a i n f r o m
p o s t h e r p e t i c n e u r a l g1)i a. ) (
B. Epidemiology
T h e mo s t c o mmo n s o u r c e o f p a r e s t h e s i a i s p e r i p h e r a l n e u r o p a t h y. T h e mo s t c o mmo n
c a u s e s i n t h e U n i t e d S t a t e s a r e d i a b e t e s a n d a l c o h o l i s m. O t h e r c o mmo n c a u s e s
i n c l u d e h y p o t h y r o i d i s m, v i t a1mi
2 nd eBf i c i e n c y, p o s t h e r p e t i c n e u r a l g i a , a n d n e r v e
e n t r a p me n t s s u c h a s c a r p a l t u n n e l s y n2)
d r. o me (
I I I . Evaluation
A. History
T h e h i s t o r y s h o u l d i n c l u d e t i me o f o n s e t , d u r a t i o n , a n d l o c a t i o n . P a s t me d i c a l h i s t o
should be obtained for illnesses that can cause paresthesias or dysesthesias (e.g.,
d i a b e t e s , h u ma n i mmu n o d e f i c i e n c y v i r u s [ H I V ] , h y p o t h y r o i d i s m, r h e u ma t o i d a r t h r i t i s
S o c i a l h i s t o r y ma y r e v e a l s u b s t a n c e a b u s e ( e . g . , a l c o h o l i s m, o r i n t r a v e n o u s d r u g
u s e , w h i c h w o u l d r a i s e t h e s u s p i c i o n o f H I V ) o r o c c u p a t i o n a l e xp o s u r e s ( e . g . ,
e xp o s u r e t o l e a d o r me r c u r y ) . I n a d d i t i o n , o c c u p a t i o n a l h i s t o r y ma y r e v e a l a n
o c c u p a t i o n a t r i s k f o r r e p e t i t i v e mo t i o n i n j u r i e s a t w o r k ( e . g . , a t r a n s c r i p t i o n i s t w h o
w o u l d b e a t h i g h e r r i s k f o r c a r p a l t u n n e l s y n d r o me ) . F a mi l y h i s t o r y ma y d i s c l o s e a
h e r e d i t a r y n e u r o p a t2)
h y. (
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T h e p a t i e n t s h o u l d h a v e a c o mp l e t e p h y s i c a l e xa mi n a t i o n , p a y i n g p a r t i c u l a r a t t e n t i o
t o t h e s e n s o r y p o r t i o n o f t h e n e u r o l o g i c e xa mi n a t i o n . C o mp l i c a t i n g t h e p h y s i c a l
e xa mi n a t i o n i s t h e f a c t t h a t t h e e xa mi n e r mu s t r e l y o n t h e p a t i e n t ' s s u b j e c t i v e
r e s p o n s e t o t h e e xa mi n a t i o n . T h e e xa mi n a t i o n s h o u l d t e s t f o r p a i n ( u s i n g a p i n o r
needle), light touch (using a cotton-tipped swab or wisp of cotton), vibration (using
t u n i n g f o r k ) , t e mp e r a t u r e , a n d p o s i t i o n s e n s e ( p e r f o r me d w i t h t h e e y e s c l o s e d ) . T h e
e xa mi n a t i o n s h o u l d d e l i n e a t e t h e d i s t r i b u t i o n o f a b n o r ma l
P. 6 4
s e n s a t i o n a s t h i s ma y b e e n o u g h t o e s t a b l i s h a d i a g n o s i s . T h e p a t i e n t ma y b e a s k e
t o ma p t h e a f f e c t e d a r e a . O t h e r a s p e c t s o f t h e n e u r o l o g i c e xa mi n a t i o n s h o u l d i n c l u d
t e s t i n g o f mu s c l e s t r e n g t h a n d r e f l e xe s . M u s c l e w a s t i n g ma1,2)
y b.e Fnl eo xi
t eodn (
o f t h e p a t i e n t ' s n e c k ( L h e r mi t t e ' s s i g n ) c a u s i n g e l e c t r i c s h o c k l i k e p a i n i n t h e b a c k o
e xt r e mi t i e s ma y b e p r e s e n t i n p a t i e n t s w i t h mu l t i p l e s c l e r o s i s , c e r v i c a l s p i n a l c o r d
d i s e a s e , o r v i t a mi1n2 Bd e f i c i e n c y.
C. Testing
I n i t i a l l a b o r a t o r y w o r k - u p s h o u l d i n c l u d e c o mp l e t e b l o o d c o u n t , r e n a l f u n c t i o n , f a s t i
s e r u m g l u c o s e , v i t a mi1n2 lBe v e l , u r i n a l y s i s , t h y r o i d - s t i mu l a t i n g h o r mo n e , a n d
e r y t h r o c y t e s e d i me n t a t i o n r a t e . F u r t h e r l a b o r a t o r y t e s t i n g mi g h t i n c l u d e f o l a t e , t h e
Ve n e r e a l D i s e a s e R e s e a r c h L a b o r a t o r y o r r a p i d p l a s ma r e a g i n t e s t , a n t i n u c l e a r
a n t i b o d y, s e r u m i mmu n o e l e c t r o p h o r e s i s , p u r i f i e d p r o t e i n d e r i v a t i v e , a n d b l o o d l e v e l
o f h e a v y me t a l s ( e . g . , l 2,3)
e a d.) (E l e c t r o my o g r a p h y a n d n e r v e c o n d u c t i o n t e s t i n g a r e
o f t e n h e l p f u l i n d e l i n e a t i n g e i t h e r t h e a n a t o mi c s o u r c e o f t h e n e u r o p a t h y ( e . g . , c a r p
t u n n e l s y n d r o me ) o r t h e s y s t e mi c c a u s e ( e . g . , p a r a n e o p l a s t i c3)s.y n d r o me s ) (
R a d i o l o g i c s t u d i e s s u c h a s c o mp u t e d t o mo g r a p h y o r ma g n e t i c r e s o n a n c e i ma g i n g
ma y b e i n d i c a t e d f o r s p e c i f i c c a u s e s , s u c h a s a s u s p e c t e d l u mb a r d i s c h e r n i a t i o n .
D. Genetics
T here are several hereditary causes of neuropathies. T hese include Charcot-MarieT o o t h d i s e a s e , D e n n y - B r o w n ' s s y n d r o me , a n d f a mi l i a l a my l o i d o t i c p o l y n e u r o p a t h y
(2) .
I V. Diagnosis
T h e d i f f e r e n t i a l d i a g n o s i s o f p a r e s t h e s i a s a n d d y s e s t h e s i a s iTs abbrl oea d ( s e e
4 . 6 . 1) .
T h e c a u s e o f p a r e s t h e s i a s a n d d y s e s t h e s i a s c a n f r e q u e n t l y b e d e t e r mi n e d b y
e xa mi n a t i o n . D i s t a l s e n s o r y l o s s i s t h e mo s t c o mmo n a n d i s f r e q u e n t l y d u e t o
me t a b o l i c o r t o xi c c a u s e s s u c h a s d i a b e t e s , a l c o h o l i s m,
c i enn B
c y, o r
1 2 vdiet fai mi
h e a v y me t a l e xp o s u r e . S o me c a u s e s , s u c h a s d i a b e t e s o r a l c o h o l i s m, c a n h a v e
v a r i o u s c l i n i c a l p a t t e r n s . D i a b e t e s mo s t c o mmo n l y c a u s e s a s y mme t r i c d i s t a l s e n s o r
l o s s , b u t c a n a l s o c a u s e mu l t i f o c a l n e u r o p a t h i e s , a u t o n o mi c n e u r o p a t h i e s , o r e v e n
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s y mme t r i c a l p r o xi ma l mo t o r n e u r o p a3)t .h i e s (
M o s t n e r v e e n t r a p me n t s a r e d i s t i n g u i s h e d b y a n e xa mi n a t i o n c o n s i s t e n t w i t h t h e i r
nerve distribution (e.g., loss of sensation of the fifth finger and adjacent half of the
P. 6 5
f o u r t h f i n g e r i n u l n a r n e u r o p a t h i e s , w h i c h a r e u s u a l l y c a u s e d b y c o mp r e s s i o n a t t h e
cubital tunnel in the elbow).
TAB L E 4.6.1 Causes of Paresthesia and

Dysesthesia
Endocrine
D i a b e t e s , h y p o t h y r o i d i s m, a c r o me g a l y
Nutritional
Vi t a mi n 1B2/ f o l a t e d e f i c i e n c i e s
T o xi c
C h e mo t h e r a p y, h e a v y me t a l s , c h r o n i c o v e r d o s e o f
p y r i d o xi n e , a l c o h o l , me d i c a t i o n s s u c h a s n i t r o f u r a n t o i n
Connective
tissue
disorders
P o l y a r t e r i t i s n o d o s a , r h e u ma t o i d a r t h r i t i s , l u p u s
E n t r a p me n t
s y n d r o me s
C a r p a l a n d c u b i t a l t u n n e l s y n d r o me s , t h o r a c i c o u t l e t
s y n d r o me , l a t e r a l f e mo r a l c u t a n e o u s s y n d r o me , t a r s a l
t u n n e l s y n d r o me , s p i n a l d i s c h e r n i a t i o n s
T r a u ma
Central
nervous
system
C e r e b r o v a s c u l a r a c c i d e n t , t u mo r s
Infectious
S y p h i l i s , L y me d i s e a s e , p o s t h e r p e t i c n e u r a l g i a , h u ma n
i mmu n o d e f i c i e n c y v i r u s , l e p r o s y
Malignancy
P a r a n e o p l a s t i c s y n d r o me s f r o m s ma l l c e l l c a r c i n o ma o f t h e
l u n g a n d c a n c e r s o f t h e b r e a s t , o v a r y, a n d s t o ma c h
M i s c e l l a n e o u s G u i l l a i n - B a r r s y n d r o me , mu l t i p l e s c l e r o s i s , c r i t i c a l i l l n e s s
p o l y n e u r o p a t h2,3)
y (
D e r ma t o ma l d i s t r i b u t i o n s w o u l d p o i n t t o e i t h e r a r a d i c u l o p a t h y o r p o s t h e r p e t i c
n e u r a l g i a . N e u r o p a t h i e s i n v o l v i n g t h e c r a n i a l n e r v e s a r e r a r e , b u t ma y b e c a u s e d b y
G u i l l a i n - B a r r s y n d r o me , d i a b e t e s , H I V, o r L y me3)d. i s e a s e (
126 / 652
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References
1 . A s b u r y A K . N u mb n e s s , t i n g l i n g , a n d s e n s o r y l o s s . I n : B r a u n w a l d E , H a u s e r
S L , e t a l . e dHs .ar r i s on' s pr i nc i pl es of i nt er nal medi
, 1 5ctihnee d . N e w Yo r k ,
N Y: M c G raw H ill, 2001:128132.
2 . M c K n i g h t J T , A d c o c k B B . P a r e s t h e s i a s : a p r a c t i c a l d i a g n o s t iAc ma p p r o a c h .
1997;56(9):22532260.
3 . P o n c e l e t A N . A n a l g o r i t h m f o r t h e e v a l u a t i o n o f p e r i p h e r a l An m
e u r o p a t h y.
1998;57(4):755760.
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4.7 - Seizures
> T a b l e o f C o n t e n t s > 4 - P r o b l e ms R e l a t e d t o t h e N e r v o u s S y s t e m > 4 . 7 S e i zu r e s
4.7
Seizures
Le s Ve skrna
I . Background
A s e i zu r e i s t h e ma n i f e s t a t i o n o f a t r a n s i e n t , u n c o n t r o l l e d , s y n c h r o n o u s d i s c h a r g e o
a p o p u l a t i o n o f n e u r o n s i n t h e c e r e b r a l c o r t e x. S o me s e i zu r e s o c c u r a s a s y mp t o m
d u r i n g t h e c o u r s e o f a n a c u t e n e u r o l o g i c o r me d i c a l i l l n e s s ; t h e y d o n o t r e c u r a f t e r
t h e u n d e r l y i n g d i s o r d e r h a s r e s o l v e d . E p i l e p s y i s a c h r o n i c d i s o r d e r c h a r a c t e r i ze d b
r e c u r r e n t s e i zu r e s t h a t a r e t y p i c a l l y u n p r o v o k e d a n d u n p r e d i c t a b l e .
A. Etiology
M o s t c a s e s o f e p i l e p s y a r e i d i o p a t h i c . S o me c a u s e s o f e p i l e p s y c a n b e a r e s u l t o f
t r a u ma , t u mo r s , p e r i n a t a l e n c e p h a l o p a t h y, c o n g e n i t a l b r a i n ma l f o r ma t i o n s , i n b o r n
e r r o r s o f me t a b o l i s m, g e n e t i c s y n d r o me s , c e n t r a l n e r v o u s s y s t e m i n f e c t i o n s , s t r o k e ,
and neurodegenerative disorders. Epilepsy tends to have age patterns that reflect
underlying causes. A higher proportion of epilepsy in children is idiopathic or due to
c o n g e n i t a l b r a i n ma l f o r ma t i o n s o r d e v e l o p me n t a l n e u r o l o g i c d i s o r d e r s t h a n i n o l d e r
a g e - g r o u p s . T h e o l d e r t h e p a t i e n t , t h e mo r e l i k e l y i t i s t h a t t h e c a u s e i s a
c e r e b r o v a s c u l a r, n e u r o d e g e n e r a t i v e , o r n e o p l a s t i c d i s o r d e r. T h e f o l l o w i n g me d i c a l
d i s o r d e r s a r e k n o w n t o c a u s e n o n e p i l e p t i c s e i zu r e s : h y p o c a l c e mi a , h y p o n a t r e mi a ,
d i s o r d e r s o f p o r p h y r i n me t a b o l i s m, c e r e b r a l a n o xi a , n o n k e t o t i c h y p e r g l y c e mi a , a n d
a d v a n c e d r e n a l f a i l u r e . H i g h f e v e r, d r u g r e a c t i o n s , a n d w i t h d r a w a l s t a t e s c a n a l s o
c a u s e s e i zu r e s .
B. Epidemiology
T he prevalence of epilepsy is in the range of 5 to 10 per 1,000. Age-specific
incidence rates have changed, with a decrease in younger age-groups and an
i n c r e a s e i n p e r s o n s o l d e r t h a n 6 0 1)
y e. a r s (
I I I . Evaluation
A. History
C a r e s h o u l d b e t a k e n t o e l i c i t r i s k f a c t o r s f o r s e i zu r e s p e r t i n e n t t o t h e p a t i e n t ' s a g e
( i n c l u d i n g a f a mi l y h i s t o r y o f s e i zu r e s ) , p o s s i b l e s e i zu r e p r e c i p i t a n t s ( me d i c a t i o n ,
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4.7 - Seizures
a l c o h o l o r d r u g s o f a b u s e , s l e e p d e p r i v a t i o n , s t r o n g e mo t i o n o r s t r e s s , i n t e n s e
e xe r c i s e , f l a s h i n g l i g h t s , f e v e r, o r me n s e s ) , a n d t h e c l i n i c a l f e a t u r e s , s e t t i n g ,
c h r o n o l o g y, a n d d u r a t i o n o f t h e s e i zu r e . I n ma n y c a s e s , i n f o r ma t i o n a b o u t t h e s e i zu
mu s t b e o b t a i n e d f r o m a w i t n e s s b e c a u s e t h e p a t i e n t ma y l o s e a w a r e n e s s o r
c o n s c i o u s n e s s . T h e f o l l o w i n g q u e s t i o n s a r e e s p e c i a l l y i mp o r t a n t i n d e t e r mi n i n g t h e
e t i o l o g y o f t h e s e i zu r e :
1. Wa s t h e r e a n a u r a ?
2. Wa s t h e r e a f a l l o r i n j u r y ?
3. Wa s t h e r e a l o s s o r i mp a i r me n t o f c o n s c i o u s n e s s ?
P. 6 6
4. Wa s t h e r e s t a r i n g , e y e b l i n k i n g , v o c a l i za t i o n s , o r a u t o ma t i s ms ( r e p e t i t i v e
p u r p o s e l e s s mo v e me n t s s u c h a s l i p s ma c k i n g , c h e w i n g , o r f a c i a l g r i ma c i n g ) ?
5. Wa s t h e r e l o s s o f b o w e l o r b l a d d e r c o n t i n e n c e ?
6. Wa s t h e r e r h y t h mi c mu s c u l a r j e r k i n g a n d / o r r i g i d i t y ?
7. Wa s t h e r e a p o s t i c t a l p e r i o d ?
T h e e xa mi n a t i o n i s u s u a l l y n o r ma l i n p a t i e n t s w i t h e p i l e p s y b u t o c c a s i o n a l l y s o me
s i g n s o f t r a u ma , a n u n d e r l y i n g s y s t e mi c o r n e u r o l o g i c d i s o r d e r, o r s t i g ma t a o f
c h r o n i c a l c o h o l i s m ma y b e e v i d e n t . A d d i t i o n a l l y, i t i s i mp o r t a n t t o l o o k f o r c u t a n e o u
ma n i f e s t a t i o n s o f s o me g e n e t i c d i s o r d e r s ( f a c i a l n e v u s f l a mme u s o f S t u r g e - We b e r
s y n d r o me , a d e n o ma s e b a c e u m o f t u b e r o u s s c l ecraf
o si sau
, o lr aima
t cules and
c u t a n e o u s n e u r o f i b r o ma s o f n e u r o f i b r o ma t o s i s ) .
C. Testing
Laboratory studies in adults and children should be ordered on the basis of

s u g g e s t i v e h i s t o r i c a l o r c l i n i c a l f i n d i n g s . I n c h i l d r e n w i t h a f i r s t s i mp l e f e b r i l e s e i zu
( < 1 0 mi n u t e s , i s o l a t e d , g e n e r a l i ze d ) , l a b o r a t o r y t e s t i n g s h o u l d b e d i r e c t e d t o w a r d
i d e n t i f y i n g t h e c a u s e o f t h e f e v e r. S t u d i e s t h a t ma y b e a p p r o p r i a t e f o r e v a l u a t i o n o f
f i r s t s e i zu r e i n c l u d e a c o mp l e t e b l o o d c o u n t , g l u c o s e , e l e c t r o l y t e s , c a l c i u m,
ma g n e s i u m, r e n a l , l i v e r a n d t h y r o i d f u n c t i o n t e s t s , a n d d r u g a n d h e a v y me t a l
t o xi c o l o g y s c r e e n i n g ( i f t h e r e i s a q u e s t i o n o f s u b s t a n c e a b u s e o r p o s s i b i l i t y o f
e xp o s u r e ) .
1. A l u mb a r p u n c t u r e s h o u l d b e p e r f o r me d i f t h e r e i s a c o n c e r n a b o u t me n i n g i t i s o r
e n c e p h a l i t i s o r i f t h e p a t i e n t i s i mmu n o c o mp r o mi s e d . T h e A me r i c a n A c a d e my o f
P e d i a t r i c s s u g g e s t s t h a t a l u mb a r p u n c t u r e b e s t r o n g l y c o n s i d e r e d w h e n a
f e b r i l e s e i zu r e o c c u r s i n a n i n f a n t y o u n g e r t h a n 1 2 mo n t h s o f a g e o r a l l c h i l d r e n
o n p r i o r a n t i b i o t i c t r e a t me n t , c o n s i d e r e d i n i n f a n t s 1 2 t o 1 8 mo n t h s o f a g e , a n
r e c o mme n d e d i f me n i n g e a l s i g n s a r e p r e s e n t i n i n f a n t s o l d e r t h a n 1 8 mo n t h s
o f a g e 2)( . I f i n c r e a s e d i n t r a c r a n i a l p r e s s u r e i s s u s p e c t e d , t h e l u mb a r p u n c t u r e
s h o u l d b e p r e c e d e d b y c o mp u t e d t o mo g r a p h y ( C T ) s c a n o r ma g n e t i c r e s o n a n c e
i ma g i n g ( M R I ) o f t h e h e a d .
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2. A n e l e c t r o e n c e p h a l o g r a m ( E E G ) i s e s s e n t i a l i n t h e e v a l u a t i o n o f e p i l e p t i c
s e i zu r e s . A n a b n o r ma l E E G ma y c o n f i r m t h e d i a g n o s i s b u t a n o r ma l o r
n o n s p e c i f i c a l l y a b n o r ma l E E G d o e s n o t r u l e o u t e p i l e p s y. S l e e p d e p r i v a t i o n a n d
p r o v o c a t i v e me a s u r e s s u c h a s h y p e r v e n t i l a t i o n a n d p h o t i c s t i mu l a t i o n ma y
increase the yield of an E E G.
3. N e u r o i ma g i n g i s o f t e n n e c e s s a r y t o r u l e o u t a s t r u c t u r a l l e s i o n o f t h e b r a i n . A
b r a i n M R I i s mo r e s e n s i t i v e t h a n a C T s c a n ; h o w e v e r, t h e l a t t e r ma y b e
a p p r o p r i a t e i n e me r g e n c y s i t u a t i o n s o r i f a n M R I i s u n a v a i l a b l e o r
c o n t r a i n d i c a t e d . A n E E G a n d n e u r o i ma g i n g n e e d n o t b e p e r f o r me d i n t h e
e v a l u a t i o n o f a n o t h e r w i s e n e u r o l o g i c a l l y h e a l t h y c h i l d w i t h a s i mp l e f e b r i l e
s e i zu r e .
I V. Diagnosis
A v a r i e t y o f e v e n t s , e i t h e r p h y s i o l o g i c o r p s y c h o g e n i c , c a n o f t e n b e mi s t a k e n a s
s e i zu r e s . T h e s e i n c l u d e s y n c o p e , c o mp l e x mi g r a i n e s , b r e a t h - h o l d i n g s p e l l s , t r a n s i e
i s c h e mi c a t t a c k s , s l e e p d i s o r d e r s ( p a r a s o mn i a s , n a r c o l e p s y ) , t r a n s i e n t g l o b a l
a mn e s i a , mo v e me n t d i s o r d e r s , a n d p s y c h i a t r i c d i s o r d e r s ( p a n i c a t t a c k s , a n xi e t y w i t h
h y p e r v e n t i l a t i o n , d i s s o c i a t i v e s t a t e s , p s y c h o g e n i c s e i zu r e s ) .
T h e c l i n i c a l e xp r e s s i o n o f a s e i zu r e d e p e n d s o n t h e l o c a t i o n a n d e xt e n t o f
propagation of the discharging neurons.
1. P a r t i a l s e i z u r eAsp a r t i a l ( f o c a l ) s e i zu r e b e g i n s i n a l o c a l i ze d a r e a o f t h e
c o r t e x. P a r t i a l s e i zu r e s a r e s u b d i v i dsei mpl
d i net po a r t i a l ocrompl exp a r t i a l
s e i zu r e s b a s e d o n t h e i mp a i r me n t o f c o n s c i o u s n e s s ( n o i mp a i r me n t i n s i mp l e
p a r t i a l s e i zu r e s ) . T h e s i g n s a n d s y mp t o ms o f a p a r t i a l s e i zu r e d e p e n d o n t h e
c o r t i c a l r e g i o n i n v o l v e d a n d ma y r a n g e f r o m a s u b j e c t i v e p e r c e p t i o n ( a u r a ) t o
mo t o r, a u t o n o mi c , s o ma t o s e n s o r y, o r p s y c h i c p h e n o me n a . A c o mp l e x p a r t i a l
s e i zu r e i mp l i e s s p r e a d o f t h e s e i zu r e d i s c h a r g e t o a l l o w i mp a i r me n t o f
c o n s c i o u s n e s s . P a t i e n t s w i t h c o mp l e x p a r t i a l s e i zu r e s u s u a l l y e xh i b i t
a u t o ma t i s ms o r s o me o t h e r c o mp l e x mo t o r a c t i v i t y t h a t i s n o t d i r e c t e d o r
p u r p o s e f u l . A s i mp l e p a r t i a l s e i zu r e ma y e v o l v e i n t o a c o mp l e x p a r t i a l s e i zu r e ,
a n d b o t h ma y e v o l v e i n t o a g e n e r a l i ze d s e i zu r e .
P. 6 7
2. G e n e r a l i z e d s e i z u rAe sg e n e r a l i ze d s e i zu r e i n v o l v e s b o t h t h e c e r e b r a l
h e mi s p h e r e s a t t h e o n s e t . G e n e r a l i ze d s e i zu r e s b e g i n w i t h a n a b r u p t l o s s o f
c o n s c i o u s n e s s ( e xc e p t my o c l o n i c s e i zu r e s ) . S u b d i v i s i o n s o f g e n e r a l i ze d
s e i zu r e s a r e b a s e d ma i n l y o n t h e p r e s e n c e o r a b s e n c e a n d c h a r a c t e r o f i c t a l
mo t o r ma n i f e s t a t i o n s : a b s e n c e ( e y e l i d f l u t t e r i n g , n o l o s s o f p o s t u r a l t o n e ,
s t a r i n g ) , t o n i c - c l o n i c ( mu s c u l a r r i g i d i t y f o l l o w e d b y r h y t h mi c mu s c u l a r j e r k i n g ) ,
t o n i c , c l o n i c , a t o n i c ( s u d d e n c o l l a p s e d u e t o l o s s o f p o s t u r a l t o n e ) , my o c l o n i c
( s u d d e n , b r i e f mu s c l e c o n t r a c t i o n s a f f e c t i n g a n y g r o u p o f mu s c l e s ) .
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4.7 - Seizures
References
1 . S a n d e r J W. T h e e p i d e mi o l o g y o f e p i l e p s y r eCvur
i sri tO
e dpi. n N eur ol
2003;16(2):165170.
2 . A me r i c a n A c a d e my o f P e d i a t r i c s . P r a c t i c e p a r a me t e r : t h e n e u r o d i a g n o s t i c
e v a l u a t i o n o f t h e c h i l d w i t h a f i r s t s i mp l ePsedi
e i zu
atrrei c. s1 9 9 6 ; 9 7 ( 5 ) : 7 6 9 7 7 2 ;
discussion 773775.
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4.8 - Stroke
> T a b l e o f C o n t e n t s > 4 - P r o b l e ms R e l a t e d t o t h e N e r v o u s S y s t e m > 4 . 8 - S t r o k e
4.8
Stroke
Ronald D. Craig
I . Background
S t r o k e i s d e f i n e d a s a n a c u t e n e u r o l o g i c d e f i c i t t h a t l a s t s mo r e t h a n 2 4 h o u r s .
E v e n t s l a s t i n g < 2 4 h o u r s a r e r e f e r r e d t o a s t r a n s i e n t i s c h e mi c a t t a c k s ( T I A s ) .
A. Etiology
S t r o k e i s c a u s e d b y o c c l u s i v e v a s c u l a r d i s e a s e 8 5 % o f t h e t i me a n d h e mo r r h a g i c
v a s c u l a r d i s e a s e 1 5 % o f t h e t i me .
B. Epidemiology
R i s k f a c t o r s f o r s t r o k e i n c l u d e u n c o n t r o l l e d h y p e r t e n s i o n , h y p e r l i p i d e mi a , t o b a c c o
s mo k i n g , c a r d i a c d i s e a s e , c o a g u l o p a t h i e s , d i a b e t e s me l l i t u s , a n d h o r mo n e t h e r a p y.
S t r o k e i s t h e t h i r d mo s t c o mmo n c a u s e o1)
f daenadt ht h( e mo s t c o mmo n a c u t e
neurologic event in the United States.
I I I . Evaluation
(2,3)
A. History
Historical factors of note include:
1. A c u t e o n s e t o f s y mp t o ms
2. U n i l a t e r a l c h a n g e i n mo t o r c o n t r o l , v i s i o n , g a i t , s t r e n g t h , o r s e n s a t i o n
3. O t h e r n e u r o l o g i c d i s o r d e r s s u c h a s mi g r a i n e , s y s t e mi c l u p u s , v a s c u l i t i s
4. S u d d e n o n s e t o f u n u s u a l h e a d a c h e
5. P r e s e n c e o f o n e o r mo r e r i s k f a c t o r s
Physical findings include:
1. A l t e r a t i o n o f me n t a l s t a t u s a n d / o r c o n s c i o u s n e s s
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4.8 - Stroke
2. S l u r r e d o r i n a p p r o p r i a t e s p e e c h , a p h a s i a
3. H e mi p a r a l y s i s , h e mi p a r e s i s
4. A l t e r e d s e n s a t i o n
5. Vi s u a l f i e l d d e f e c t , d i p l o p i a , n y s t a g mu s
6. H y p e r t e n s i o n
7. C a r d i a c d y s r h y t h mi a
8. D i zzi n e s s , a t a xi a
9. Va s c u l a r t e n d e r n e s s t o p a l p a t i o n , t e mp o r a l a n d c a r o t i d a r t e r i e s
C. Testing
Studies and laboratory tests include:
1. C o mp u t e d t o mo g r a p h y ( C T ) s c a n o f t h e h e a d t o i d e n t i f y h e mo r r h a g e . O t h e r
i ma g i n g s t u d i e s a r e u n d e r g o i n g c l i n i c a l e v a l u a t i o n
2. C o mp l e t e b l o o d c o u n t , w i t h p l a t e l e t c o u n t , c o mp r e h e n s i v e c h e mi s t r y p a n e l ,
p r o t h r o mb i n t i me , p a r t i a l t h r o mb o p l a s t i n t i me f o r u n d e r l y i n g d i s e a s e s a n d
b a s e l i n e i f t h r o mb o l y t i c o r a n t i c o a g u l a n t t h e r a p y i s a n t i c i p a t e d
P. 6 8
3. S p e c i a l s t u d i e s s u c h a s t o xi c o l o g y s c r e e n , a n t i p h o s p h o l i p i d a n t i b o d i e s , p r o t e i n
S a n d C , a n t i t h r o mb i n I I I , a n d o t h e r s i f i n d i c a t e d b y h i s t o r y o r p h y s i c a l
e xa mi n a t i o n
4. E l e c t r o c a r d i o g r a m t o h e l p d i a g n o s e d y s r h y t h mi a s o r p r e c e d i n g my o c a r d i a l
infarction
5. E c h o c a r d i o g r a m t o v i s u a l i ze s t r u c t u r a l d e f e c t s o r mu r a l t h r o mb i .
T r a n s e s o p h a g e a l e c h o c a r d i o g r a p h y ma y p o s s i b l y b e a p p r o p r i a t e
6. C a r o t i d / i n t r a c r a n i a l D o p p l e r s t u d i e s t o f i n d o c c l u s i v e v a s c u l a r d i s e a s e o r s o u r c
o f a r t e r y t o a r t e r y e mb o l i
I V. Diagnosis
T h e d i f f e r e n t i a l d i a g n o s i s o f s t r o k e i n c l u d e s a b e r r a n t mi g r a i n e , s e i zu r e d i s o r d e r,
me t a b o l i c d i s o r d e r s , p s y c h o g e n i c c o n d i t i o n ( h y s t e r i c a l c o n v e r s i o n r e a c t i o n ,
h y p e r v e n t i l a t i o n ) , a n d t u mo r w i t h h e mo r r h a g e .
B. Clinical approach
W i t h t h e a v a i l a b i l i t y o f t h r o mb o l y t i c t h e r a p y, t h e u r g e n c y o f ma k i n g a r a p i d d i a g n o s
h a s b e c o me mo r e c r i t i c a l . G e t t i n g t h e p a t i e n t t o a me d i c a l c a r e f a c i l i t y a s q u i c k l y a
p o s s i b l e i s v e r y i mp o r t a n t . A h i s t o r y a n d p h y s i c a l e xa mi n a t i o n s h o u l d b e c o mp l e t e d
a s q u i c k l y a s i s p r a c t i c a l , w i t h s p e c i a l a t t e n t i o n t o t h e n e u r o l o g i c a l e xa mi n a t i o n .
Q u a n t i t a t i v e a s s e s s me n t o f s t r e n g t h a n d f u n c t i o n o n t h e a f f e c t e d s i d e s h o u l d b e
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4.8 - Stroke
d o c u me n t e d f o r c o mp a r i s o n w i t h s u b s e q u e n t e xa mi n a t i o n s . C l i n i c a l i mp r o v e me n t i n
t h e f i r s t c o u p l e o f h o u r s w o u l d f a v o r a T I A o r a mo r e l o c a l i ze d s t r o k e . A f t e r t h e i n i t
h i s t o r y a n d p h y s i c a l e xa mi n a t i o n , l a b o r a t o r y s t u d i e s c a n b e o r d e r e d a n d a
n o n c o n t r a s t h e a d C T s c a n c a n b e o b t a i n e d t o r u l e o u t h e mo r r h a g e p r o v i d e d t h e
patient is clinically stable enough. Appropriate interventions to control blood
p r e s s u r e , s e i zu r e s , a n d c a r d i a c d y s r h y t h mi a s s h o u l d p r o c e e d c o n c o mi t a n t l y w i t h t h e
evaluation.
References
1 . C D C F A S T AT S : w w w. c d c . g o v / n c h s / f a s t a t s / s t r o k e . h t m, 2 0 0 5
2 . A me r i c a n H e a r t A s s o c i a t i o n S t r o k e O u t c o me C l a s s i f i c a t i o n . E xe c u t i v e
s u mma r y.C i r c ul at i on
1998:97:24742478.
3. H arrison's O nline: P art 15. N eurological disorders. S ection 2. D iseases of the
C e n t r a l N e r v o u s s y s t e m. C h a p t e r 3 4 9 . C e r e b r o v a s c u l a r d i s e a s e s .
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4.9 - Tremors
> T a b l e o f C o n t e n t s > 4 - P r o b l e ms R e l a t e d t o t h e N e r v o u s S y s t e m > 4 . 9 - T r e mo r s
4.9
Tremors
Ronald D. Craig
I . Background
T r e mo r s , w h i c h r e p r e s e n t t h e mo s t c o mmo n t y p e o f mo v e me n t d i s o r d e r, a r e
a b n o r ma l , i n v o l u n t a r y, r h y t h mi c a l mo v e1,2)
me.n tTsh (e y a l l s t o p d u r i n g s l e e p .
T r e mo r s a r e o f t h r e e b a s i c t y p e s : r e s t , p o s t u r a l , a n d k i n e t i c .
A. Rest tremors
T h e s e o c c u r w h e n t h e l i mb mu s c l e s a r e n o t a c t i v a t e d a n d d i mi n i s h w i t h a c t i v i t y.
T h e y o c c u r s l o w l y i n d i s e a s e s t a t e s s u c h a s P a r k i n s o n ' s d i s e a s e , mu l t i p l e s y s t e ms
a t r o p h y, a n d s u p r a n u c l e a r p a l s y, w h e r e a s t h e y o c c u r r a p i d l y o r s u d d e n l y i n t o xi n o r
d r u g - i n d u c e d a n d p s y c h o g e n i c s t a t e s , i n c l u d i n g t h o s e b r o u g h t o n b y p h e n o t h i a zi n e s
me t o c l o p r a mi d e , a n d a n xi e t y. R e s t t r e mo r s h a v e a r e l a t i v e l y s l o w o s c i l l a t i o n
f r e q u e n c y.
B. Postural tremors
T h e y a r e a l s o r e f e r r e d t o a s e s s e n t i a l , p h y s i o l o g i c , b e n i g n , a n d f a mi l i a l t r e mo r s .
T hey are usually gradual in onset. Benign causes include the essential/physiologic
t r e mo r, a n d a c u t e c a u s e s i n c l u d e t o xi c d r u g a n d a l c o h o l w i t h d r a w a l , s t i mu l a n t u s e ,
a n d me t a b o l i c s t a t e s s u c h a s h y p e r t h y r o i d i s m a n d s t r e s s
P. 6 9
r e a c t i o n s . P o s t u r a l t r e mo r s a r e ma xi ma l w i t h t h e l i mb ma i n t a i n e d a g a i n s t g r a v i t y, a r
r e d u c e d b y r e s t , a n d a r e n o t e n h a n c e d d u r i n g v o l u n t a r y mo v e me n t .
C. Kinetic tremors
S i mp l e k i n e t i c t r e mo r h a s v a r i a b l e f r e q u e n c y a n d d o e s n o t c h a n g e w i t h p u r p o s e f u l
mo v e me n t . I n t e n t i o n t r e mo r h a s a l o w f r e q u e n c y t h a t i n c r e a s e s w i t h d i r e c t e d
mo v e me n t . E t i o l o g y i n c l u d e s c e r e b e l l a r l e s i o n s f r o m c a u s e s s u c h a s s t r o k e , t u mo r,
mu l t i p l e s c l e r o s i s , a n d d r u g s s u c h a s l i t h i u m a n d a l c o h o l . I s o me t r i c t r e mo r h a s a
me d i u m f r e q u e n c y a n d o c c u r s w i t h mu s c l e c o n t r a c t i o n a g a i n s t a s t a t i o n a r y o b j e c t .
T a s k - s p e c i f i c t r e mo r s o c c u r o n l y w i t h s p e c i f i c t a s k s s u c h a s w r i t i n g a n d s e w i n g .
I I I . Evaluation
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4.9 - Tremors
T h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n r e ma i n s t h e mo s t v a l u a b l e t o o l f o r t h e
e v a l u a t i o n o f a t r e mo r.
A. History
A detailed history should include:
1. D u r a t i o n , p r o g r e s s i o n , a n d a g g r a v a t i n g a n d a me l i o r a t i n g f a c t o r s . R e l a t i o n s h i p t
stressful events is particularly useful in evaluating for psychogenic causes
2. F a mi l y h i s t o r y o f n e u r o l o g i c a n d me t a b o l i c d i s o r d e r s
3. M e d i c a t i o n h i s t o r y t o i n c l u d e a l c o h o l , t o b a c c o , a n d i l l i c i t d r u g u s e . O v e r - t h e counter drug use such as decongestants, weight loss preparations, and herbal
p r o d u c t s s h o u l d b e e l i c i t e d , b e c a u s e ma n y p e o p l e b e l i e v e t h e m t o b e f r e e o f
side effects.
T h i s s h o u l d b e g i n w i t h o b s e r v a t i o n w i t h t h e p a t i e n t a t r e s t d u r i n g t h e i n t e r v i e w. T h e
e xa mi n a t i o n s h o u l d d o c u me n t t h e f o l l o w i n g :
1. R e l a t i v e f r e q u e n c y ( f a s t o r s l o w ) a n d a mp l i t u d e ( f i n e o r c o a r s e ) o f t h e t r e mo r a s
w e l l a s s y mme t r y a n d t h e e f f e c t o f p o s i t i o n , r e s t , a n d a c t i o n s
2. A f f e c t e d b o d y p a r t s ( e . g . , h e a d , h a n d s , a r ms , l e g s , f e e t )
3. A n y r e l a t e d f i n d i n g s s u c h a s t a c h y c a r d i a , h y p e r t e n s i o n , e xo p h t h a l mi a ,
t h y r o me g a l y, a b n o r ma l s k i n p i g me n t a t i o n , mu s c l e r i g i d i t y, c o g w h e e l i n g , a n d
h y p e r - r e f l e xi v e n e s s o r h y p o r e f l e xi v e n e s s .
C. Testing
T e s t i n g s h o u l d b e d i r e c t e d b y t h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n . G e n e r a l l y, a
c o mp r e h e n s i v e me t a b o l i c p a n e l w i t h t e s t s o f l i v e r, k i d n e y, t h y r o i d f u n c t i o n s , b l o o d
g l u c o s e , a n d c o mp l e t e b l o o d c o u n t a r e a p p r o p r i a t e f o r mo s t t r e mo r s .
1. C o mp u t e d t o mo g r a p h y o r ma g n e t i c r e s o n a n c e i ma g i n g ma y b e i n d i c a t e d i n
c e r e b e l l a r t r e mo r s .
2. C e r e b r o s p i n a l f l u i d a n a l y s i s , i n c l u d i n g i mmu n i g l o b u l i n G ( I g G ) a n a l y s i s , ma y b e
n e c e s s a r y i f mu l t i p l e s c l e r o s i s i s s u s p e c t e d .
3. A p p r o p r i a t e d r u g a n d a l c o h o l s c r e e n s a r e i n d i c a t e d b y t h e h i s t o r y a n d p h y s i c a l
4. P o s i t r o n e mi s s i o n t o mo g r a p h y a n d s i n g l e p h o t o n e mi s s i o n c o mp u t e d t o mo g r a p h y
are undergoing clinical usefulness trials.
I V. Diagnosis
T h e mo s t c r i t i c a l s t e p i s t o d i f f e r e n t i a t e b e n i g n t r e mo r s f r o m t h o s e w i t h mo r e
o mi n o u s c o n s e q u e n c e s . T r e a t me n t i s b a s e d o n t h e c l a s s i f i c a t i o n o f t h e t r e mo r.
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4.9 - Tremors
T h e r a p e u t i c t r i a l s o f d r u g s s u c h a s - b l o c k e r s ma y b e u s e f u l i n d i f f e r e n t i a t i n g s o me
t y p e s o f t r e mo r.
References
1. M c G raw- H ill's Access M edicine, H arrison's O nline. P art 2. C ardinal
ma n i f e s t a t i o n s a n d P r e s e n t a t i o n o f D i s e a s e s . S e c t i o n 3 . N e r v o u s S y s t e m
D y s f u n c t i o n . C h a p t e r 2 1 . We a k n e s s , D i s o r d e r s o f M o v e me n t a n d I mb a l a n c e .
M o v e me n t D i s o r d e r s .
2 . S h a r o n S ma g a M D . T r e mo
A mr. F am P hy s i c i an
2003;68(8):15451552.
137 / 652
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5.1 - Blurred Vision
> T a b l e o f C o n t e n t s > 5 - E y e P r o b l e ms > 5 . 1 - B l u r r e d Vi s i o n
5.1
Blurred Vision
Norm an Be njam in Fre drick
I . Background
B l u r r e d v i s i o n i s t h e mo s t c o mmo n v i s u a l c o5)mp
. l aBilnutr r(e d v i s i o n i s t h e l o s s o f
s h a r p n e s s o f v i s i o n a n d t h e i n a b i l i t y t o s e e s ma 1)
l l .d e t a i l s (
A. Etiology
T h e c a u s e s o f b l u r r e d v i s i o n r a n g e f r o m mi l d t o p o t e n t i a l l y c a t a s t r o p h i c . M o s t
c a u s e s i n v o l v e t h e o r b i t ( a n t e r i o r a n d p o s t e r i o r s e g me n t s ) ; a l t h o u g h a n u mb e r o f
e xt r a o c u l a r c a u s e s mu s t b e c o n s i d e r e d ( me d i c a t i o n , c e r e b r o v a s c u l a r e v e n t ,
s a r c o i d o s i s , h e r p e s s i mp l e x) .
B. Epidemiology
C e r t a i n a g e - r e l a t e d e y e d i s o r d e r s s u c h a s ma c u l a r d e g e n e r a t i o n , c a t a r a c t s , a n d
t e mp o r a l a r t e r i t i s ma y c a u s e b l u r r e d v i s i o n . I n y o u n g e r p a t i e n t s , b l u r r e d v i s i o n i s
o f t e n a c q u i r e d t h r o u g h t r a u ma , o c c u p a t i o n a l e xp o s u r e s , a n d i n f e c t i o n s .
I I I . Evaluation
A. History
Careful attention should be paid to the rapidity of the onset, associated eye pain,
and whether the blurring is unilateral or bilateral. Blurred vision that worsens at nig
ma y i n d i c a t e a c a t a r 2)
a c.t I(n t e r mi t t e n t l y b l u r r e d v i s i o n ma y b e c a u s e d b y e xc e s s
t e a r i n g , a l l e r g i e s , u n c o n t r o l l e d d i a b e t e s , a c u t e g l a u c o ma , t r a n s i e n t i s c h e mi c a t t a c k
c e r e b r o v a s c u l a r i n s u f f i c i e n c y, a n d mu l t i p l e s3)c .l eO
r ot hs iesr (i mp o r t a n t f a c t o r s
i n c l u d e a f a mi l y h i s t o r y o f e y e d i s o r d e r s ( ma c u l a r d e g e n e r a t i o n , g l a u c o ma ) , a n y w o r
e xp o s u r e s ( c h e mi c a l s ) , me d i c a t i o n s ( s u c h a s c o r t i c o s t e r o i d s , a n t i b i o t i c s ) , a n d p a s t
me d i c a l h i s t o r y ( d i a b e t e s , h y p e r t e4)
n s. i o n ) (
T h e p h y s i c a l e xa mi n a t i o n s h o u l d i n c l u d e t h e f o l l o w i n g e l e me n t s :
1. C a r e f u l d o c u me n t a t i o n o f v i s u a l a c u i t y ( c o r r e c t e d a n d u n c o r r e c t e d ) i s i mp o r t a n t
t o mo n i t o r t h e p r o g r e s s i o n o f t h e d i s e a s e . I f t h e p a t i e n t i s u n a b l e t o d i s c e r n
138 / 652
tmdmss
l e t t e r s o n t h e S n e l l e n e y e c h a r t , t h e e xa mi n e r s h o u l d d e t e r mi n e t h e e xt e n t o f
a c u i t y i mp a i r me n t b y t e s t i n g t h e d i s t a n c e f r o m t h e p a t i e n t ' s e y e s a t w h i c h t h e
p a t i e n t c a n f i r s t s e e t h e e xa mi n e r ' s f i n g e r s .
2. Vi s u a l f i e l d t e s t i n g ma y i n d i c a t e a n u n d e r l y i n g s t r o k e ( h o mo n y mo u s f i e l d d e f e c t )
o r r e t i n a l d e t a c h me n t ( q u a d r a n t o r h e mi s p h e r i c l o s s o f v i s i o n ) .
3. O c u l a r mu s c l e i n v o l v e me n t ma y b e d e t e c t e d b y t e s t i n g t h e c a r d i n a l p o s i t i o n s o f
t h e o r b i t t h r o u g h r a n g e o f mo t i o n .
4. C o n j u n c t i v a l e r y t h e ma a n d d i s c h a r g e s h o u l d b e n o t e d . T h e c o r n e a l l i g h t r e f l e x
s h o u l d b e s y mme t r i c a n d s h a r p ; f l u o r e s c e i n s t a i n i n g s h o u l d b e p e r f o r me d t o
e v a l u a t e f o r t h e e v i d e n c e o f t r a u ma , u l c e r s , o r h e r p e t i c l e s i o n s . T h e a n t e r i o r
c h a mb e r ( s p a c e b e t w e e n t h e c o r n e a a n d t h e i r i s ) s h o u l d b e e v a l u a t e d w i t h a
p e n l i g h t f o r b l o o d ( h y p h e ma ) a n d p u s ( h y p o p y o n ) .
5. I n u p t o 2 0 % o f t h e c a pupi
s e s , l l ar y ex ami nat ima
ony b e t h e o n l y c l u e t o
s e r i o u s u n d e r l y i n g p a t h o l o g y. U s i n g a p e n l i g h t , t h e a b n o r ma l i t i e s o f p u p i l l a r y
s i ze o r s h a p e ( t h e p u p i l s s h o u l d b e s y mme t r i c ; a u n i l a t e r a l mi o t i c p u p i l ma y
i n d i c a t e i r i t i s ) o r c o l o r ( b l a c k i s n o r ma l ) ma y b e d e t e c t e d . O t h e r f i n d i n g s ma y
include cataracts, ruptured globes (with eccentric pupils), and optic nerve
d i s e a s e ( a f f e r e n t p a p i l l a r y d e f e c t p a r a d o xi c a l p a p i l l a r y d i l a t a t i o n i n r e s p o n s e t
light).
6. D i r e c t o p h t h a l mo s c o p y ma y r e v e a l a n a b n o r ma l r e d r e f l e x t h a t s u g g e s t s a
h e mo r r h a g e , c a t a r a c t , o r r e t i n a l d e t a c h me n t . P a p i l l e d e ma w a r r a n t s f u r t h e r
evaluation.
C. Testing
A n e l e v a t e d s e d i me n t a t i o n r a t e ma y s u g g e s t a d i a g n o s i s o f t e mp o r a l a r t e r i t i s .
C o mp u t e d t o mo g r a p h y i s a p p r o p r i a t e t o e v a l u a t e b l u r r e d v i s i o n f o l l o w i n g t r a u ma , o r
w h e n t h e r e i s c o n c e r n f o r ma s s e5)f .f e c t (
P. 7 4
TAB L E 5.1.1 Causes of Blurred Vision

Painle ss
Sudde n onse t
U n i l a t e r a l Vi t r e o u s
h e mo r r h a g e ,
ma c u l a r
degeneration,
retinal
d e t a c h me n t ,
retinal-vein
Gradual onse t
Cataracts, dry
ma c u l a r
degeneration,
t u mo r
Painful
Sudde n
onse t
Gradual
onse t
Corneal
Rare
abrasion,
infection
o r e d e ma ,
uveitis,
t r a u ma t i c
h y p h e ma ,
139 / 652
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occlusion,
a ma u r o s i s f u g a x,
cataracts
Bilateral
acute
g l a u c o ma ,
t e mp o r a l
arteritis,
optic
neuritis,
orbital
cellulitis
P o o r l y c o n t r o l l e dC a t a r a c t s ,
T r a u ma ,
diabetes,
ma c u l a r
c h e mi c a l
me d i c a t i o n s
degeneration,
spill,
( a n t i c h o l i n e r g i c s ,me d i c a t i o n s
welder's
cholinergics,
( h y d r o c h l o r o q u i n e ,e xp o s u r e
c o r t i c o s t e r o i d s ) , e t h a mb u t o l ,
mi g r a i n e s ,
d i g o xi n t o xi c i t y ) ,
psychological
optic chiasm
t r a u ma
ma s s , f a t i g u e ,
refractive errors
( my o p i a ,
hyperopia,
a s t i g ma t i s m,
presbyopia);
incorrect eyewear
Rare
(sarcoidosis,
collagen
vascular
disease)
C o mp i l e d f r o m S h i n g l e t o n B J , O ' D o n o g h u e M W. P r i ma r y c a r e : b lN
urred vision.
E ngl J M ed2 0 0 0 ; 3 4 3 ( 8 ) : 5 5 6 5 6 2 .
D. Genetics
M a c u l a r d e g e n e r a t i o n , g l a u c o ma , c o l l a g e n v a s c u l a r d i s e a s e s , d i a b e t e s , a n d mu l t i p l e
sclerosis (optic neuritis) are potentially heritable conditions.
I V. Diagnosis
( s e e T a b l e 5 . 1 ). 1
A c a r e f u l h i s t o r y a n d p h y s i c a l e xa mi n a t i o n o f t e n l i mi t t h e d i f f e r e n t i a l d i a g n o s i s .
C o n d i t i o n s t h a t r e q u i r e i mme d i a t e o p h t h a l mo l o g i c r e f e r r a l i n c l u d e a c u t e g l a u c o ma ,
r e t i n a l d e t a c h me n t , v i t r e o u s h e mo r r h a g e , r e t i n a l - v e i n o c c l u s i o n , h e r p e s s i mp l e x
infection, and orbital cellulitis.
References
1 . H a r t J A . D i p l o pM
i aedl
. i ne pl us enc y c l opedi
. M ead l i n e P l u s . c o m
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( h t t p : / / w w w. n l m. n i h . g o v / me d l i n e p l u s / e n c y / a r t i c l e / 0 0 3 0 2 9 . h t m) , 2 0 0 4 .
2. Pavan-Langston M
D anual
.
of oc ul ar di agnos i s and t her
. Papy
hiladelphia,
PA : L i p p i n c o t t W i l l i a ms & W i l l i a ms , 2 0 0 2 .
3 . Wr o n g D i a g n o s i s . c o m. B l u r r e d vW
i s ri ongdi
o n . agnos i s s y mpt oms
( h t t p : / / w w w. w r o n g d i a g n o s i s . c o m/ s y m/ b l u r r e d _ v i s i o n . h t m# p o s s i b l e ) , 2 0 0 3 .
4 . Va u g h a n D G , A s b u r y T , R i o r d a n - EGvener
a P. al opht hal mol .ogy
N e w Yo r k ,
N Y: M c G raw- H ill M edical, 2003.
5 . S h i n g l e t o n B J , O ' D o n o g h u e M W. P r i ma r y c a r e : b l u r rNe dE vngl
i s i oJn .M ed
2000;343(8):556562.
141 / 652
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5.2 - Corneal Foreign Body
> T a b l e o f C o n t e n t s > 5 - E y e P r o b l e ms > 5 . 2 - C o r n e a l F o r e i g n B o d y
5.2
Corneal Foreign Body
Peter R. Lewis
I . Background
M a c r o s c o p i c a n d / o r mi c r o s c o p i c ma t e r i a l f r o m t h e e xt e r n a l e n v i r o n me n t ma y l o d g e i
the corneathe anterior and transparent part of the eye that overlies the anterior
c h a mb e r a n d i s c o n t i n u o u s w i t h t h e s c l e r a .
A. Etiology
C o mmo n l y i n v o l v e d ma t e r i a l s i n c l u d e s a n d , d i r t , l e a v e s , a n d o t h e r o r g a n i c ma t e r i a l s
i n t h e e n v i r o n me n t a n d a d d i t i o n a l o c c u p a t i o n a l e xp o s u r e t o ma t e r i a l s s u c h a s me t a l
shavings or glass particles.
B. Epidemiology
A mo n g s y mp t o ma t i c p a t i e n t s , a c o r n e a l f o r e i g n b o d y r e p r e s e n t s a c o mmo n c a u s e o f
e y e - r e l a t e d c o mp l a i n t s f o r a d u l t a n d p e d i a t r i c p a t i e n t s p r e s e n t i n g t o p r i ma r y c a r e
p h y s i c i a n s , o p h t h a l mo l o g i s t s , a n d e me r g e n c y d e p1)
a r. t me
T h ne t sf r e( q u e n t
a s s o c i a t i o n b e t w e e n a c o r n e a l f o r e i g n b o d y a n d o c c u p a t i o n a l o r l e i s u r e - t i me
e xp o s u r e h i g h l i g h t s t h e e mi n e n t l y p r e v e n t a b l e n a t u r e o f t h i s c o n d i t i o n w i t h p r o t e c t i v
e y e w e a r 2)( .
I I I . Evaluation
A. History
A p a t i e n t w i t h a c o r n e a l f o r e i g n b o d y i s f r e q u e n t l y s e l f - e v i d e n t b y h i s t o r y. F o r e i g n b o d y s e n s a t i o n , p a i n , p h o t o p h o b i a , a n d l a c r i ma t i o n a r e c o mmo n l y a s s o c i a t e d
p a t i e n t c o mp l a i n t s . T h e p a t i e n t f r e q u e n t l y n o t e s r e d n e s s i n C
t hhea petyeer ( s e e
5 . 6) . D e c r e a s e d v i s i o n ma y b e r e p o r t e d . I n d i v i d u a l s w i t h c o r n e a l f o r e i g n b o d i e s w h o
a r e v i c t i ms o f s i g n i f i c a n t i n d u s t r i a l a c c i d e n t s , mo t o r v e h i c l e t r a u ma , o r g u n s h o t
w o u n d s ma y b e u n a b l e t o c o mmu n i c a t e o r ma y p r i ma r i l y c o mp l a i n o f p a i n a t s i t e s o f
mo r e s u b s t a n t i a l i n j u r y. T e t a n u s i mmu n i za t i o n s t a t u s s h o u l d b e d e t e r mi n e d i n t h e s e
p a t i e n t s . B y c o n t r a s t , s o me p a t i e n t s w i t h c o r n e a l f o r e i g n b o d i e s ma y h a v e n o
s y mp t o ms . A h i s t o r y o f me t a l g r i n d i n g o r w e l d i n g s h o u l d r a i s e t h e c l i n i c i a n ' s
s u s p i c i o n f o r a n a s y mp t o ma t i c f o r e i g n b o d y ; t h i s i s a n i mp o r t a n t d e t a i l t o c o n s i d e r f
t h e p a t i e n t u n d e r g o i n g ma g n e t i c r e s o n a n c e i ma g i n g ( M R I ) .
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I f t h e i n j u r y i s c l e a r l y c o n f i n e d t o t h e e y e , a c o mp r e h e n s i v e o c u l a r e xa mi n a t i o n
s h o u l d c o mme n c e w i t h g r o s s i n s p e c t i o n t o e v a l u a t e f o r a s y mme t r y i n t h e p e r i o r b i t a l
r e g i o n , c o n j u n c t i v a , c o r n e a , a n d p u p i l s . A s u f f i c i e n t l y l a r g e f o r e i g n b o d y ma y b e
v i s i b l e b y g r o s s i n s p e c t i o n w i t h a p e n l i g h t o n p h y s i c a l e xa mi n a t i o n . M a g n i f i c a t i o n
s h o u l d b e u s e d , i n c l u d i n g a s l i t l a mp , w h e r e a v a i l a b l e . T h e e y e l i d s s h o u l d b e
e v e r t e d , b e c a u s e f o r e i g n b o d i e s ma y l o d g e i n mu l t i p l e l o c a t i o n s . A f u n d u s c o p i c
e xa mi n a t i o n s h o u l d a l s o b e p e r f o r me d .
1. A t o p i c a l a n e s t h e t i c ma y b e r e q u i r e d . Vi s u a l a c u i t y s h o u l d b e d e t e r mi n e d b e f o r e
t h e u s e o f a n y t o p i c a l a n e s t h e t i c . A l t e r e d v i s u a l a c u i t y ma y b e d u e t o a c o r n e a l
f o r e i g n b o d y i n t e r f e r i n g w i t h t h e v i s u a l a xi s , a n d ma y b e a s s o c i a t e d w i t h
p e n e t r a t i n g o c u l a r t r a u ma .
2. S c r e e n i n g f o r a c o r n e a l i n f i l t r a t e o r h y p h e ma ( b l o o d i n t h e a n t e r i o r c h a mb e r ) i s
n e c e s s a r y. T h e p r e s e n c e o f a r u s t s t a i n i s h i g h l y s u g g e s t i v e o f a me t a l l i c
f o r e i g n b o d y.
3. F l u o r e s c e i n s t a i n i n g a n d e xa mi n a t i o n w i t h c o b a l t - b l u e l i g h t o r Wo o d ' s l a mp
s h o u l d b e u s e d t o s e a r c h f o r a n a s s o c i a t e d c o r n e a l3)a.bAr avsei or tni c(a l l y
oriented corneal abrasion suggests the presence of an associated foreign body
in the upper tarsal region.
C. Testing
F o r p a t i e n t s w i t h s u s p e c t e d a s s o c i a t e d p e n e t r a t i n g f o r e i g n b o d y, c o mp u t e d
t o mo g r a p h o r u l t r a s o u n d ma y b e r e q u i r e d . A n M R I s h o u l d b e a v o i d e d i n t h e s e t t i n g
o f a s u s p e c t e d o r a c o n f i r me d me t a l l i c f o r e i g n b o d y.
I V. Diagnosis
I n t h e e v e n t o f s e r i o u s a n d / o r mu l t i p l e i n j u r i e s , a n e xp e d i t e d a n d d e t a i l e d t r a u ma
s u r v e y s h o u l d b e c o n d u c t e d a n d p l a n s ma d e f o r a mb u l a n c e t r a n s p o r t t o
P. 7 6
t h e c l o s e s t e me r g e n c y d e p a r t me n t . S e v e r e c o r n e a l t r a u ma r e l a t e d t o a f o r e i g n b o d y
e s p e c i a l l y i n c h i l d r e n , ma y r e q u i r e e xa mi n a t i o n u n d e r g e n e r a l a n e s t h e s i a i n t h e
o p e r a t i n g r o o m b y a n o p h t h a l mo l o g i s t . O t h e r r e a s o n s f o r p r o mp t o p h t h a l mo l o g i c
referral include corneal infiltrate (increased association with infection), corneal
l a c e r a t i o n , h y p h e ma , a n d a n y s u s p i c i o n f o r a p e n e t r a t i n g i n j u r y. F o r t h e p a t i e n t
suitable for evaluation in the outpatient setting, the differential diagnosis for anteri
eye pain includes corneal ulceration, keratitis (including that caused by shingles),
c l u s t e r o r mi g r a i n e h e a d a c h e , a n d g l a u c o ma .
References
1 . S h i e l d s T , S l o a n e P D . A c o mp a r i s o n o f e y e p r o b l e ms i n p r i ma r y c a r e a n d
o p h t h a l mo l o g y p r a c t i cFeam
s . M ed1 9 9 1 ; 2 3 : 5 4 4 .
2 . Wo r k L o s s D a t a I n s t i t E
u tyee. C o r p u s C h r i s t i , T X : Wo r k L o s s D a t a I n s t i t u t e ,
143 / 652
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Av a i l a b l e o n l i n e a t N a t i o n a l G u i d e l i n e C l e a r i n g h o u s e
w w w. g u i d e l i n e . g o v / s u mma r y / s u mma r y. a s p x? v i e w _ i d = 1 & d o c _ i d = 6 5 5 9 , 2 0 0 4 .
3 . W i l s o n S A , L a s t A . M a n a g e me n t o f c o r n e a l a bAr m
a s iFoam
n s . P hy s i c i an
2004;70(1):123128.
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5.3 - Diplopia
> T a b l e o f C o n t e n t s > 5 - E y e P r o b l e ms > 5 . 3 - D i p l o p i a
5.3
Diplopia
Norm an Be njam in Fre drick
I . Background
D i p l o p i a me a n s d o u b l e v i s i o n . P a t i e n t s c o mp l a i n o f s e e i n g t h e s a me v i e w a s t w o
o v e r l a p p i n g i ma g e1)s. (T h e i ma g e s ma y b e h o r i zo n t a l , v e r t i c a l , o r d i a g o n a l t o o n e
a n o t h e r 2)( .
A. Etiology
D i p l o p i a o c c u r s w h e n t h e s c e n e b e f o r e t h e p a t i e n t i s s e n t a s t w o d i f f e r e n t i ma g e s t
t h e v i s u a l c o r t e x. T h e n o r ma l ma p p i n g p r o c e s s c a n n o t o c c u r a n d t h e b r a i n p e r c e i v e
t w o o v e r l a p p i n g i ma g e s . T h e r e a r e t w o ma i n t y p e s o f d i p l o p i a : mo n o c u l a r a n d
b i n o c u l a r. M o n o c u l a r d i p l o p i a i mp l i e s a p r o b l e m w i t h o n l y o n e o f t h e e y e s . T h e s e a r
o f t e n r e f r a c t i v e a b n o r ma l i t i e s a n d d i s o r d e r s o f t h e g l o b e i t s e l f ( i . e . , c o r n e a , l e n s ,
r e t i n a )1)( . B i n o c u l a r d i p l o p i a i s p r i ma r i l y d u e t o d i s o r d e r s o f o c u l a r mo t i l i t y ( i . e . ,
e i t h e r t h e mu s c l e s o r t h e i n n e r v a t i o n o f t h e o c u l a r mu s c l e s ) . M o n o c u l a r d i p l o p i a i s
readily distinguished from binocular diplopia by the fact that the diplopia persists
d e s p i t e c o v e r i n g t h e u n a f f e c t e d1) e. y e (
B. Epidemiology
P a t i e n t s p r i ma r i l y c o mp l a i n i n g o f d i p l o p i a a r e a d u l t s . C h i l d r e n y o u n g e r t h a n 1 0 y e a
o f a g e t e n d t o c o mp e n s a t e f o r v i s u a l d i s t u r b a n c e s b y s u p p r e s s i n g o n e o f t h e i ma g e s
B i n o c u l a r d i p l o p i a i s mo r e c o mmo n t h a n mo n o c u l a r d i p l o p i a .
I I I . Evaluation
A. History
S t a n d a r d h i s t o r y i s r e q u i r e d w i t h a n e mp h a s i s o n t h e f o l l o w i n g p o i n t s :
1. D i s t i n g u i s h i n g b e t w e e n d o u b l e v i s i o n a n d b l u r r y v i s i o n i s i mp o r t a n t . P a t i e n t s
c o mp l a i n o f s e e i n g t w o i ma g e s w i t h d i p l o p i a .
2. M o s t c o mp l a i n t s o f d i p l o p i a a r e d u e t o a b i n o c u l a r a b n o r ma l i t y a r i s i n g f r o m i n c o o r d i n a t i o n o f t h e o c u l a r mu s c l e s . A s k i n g w h e t h e r t h e i ma g e s a r e s i d e - b y - s i d e ,
v e r t i c a l t o e a c h o t h e r, o r d i a g o n a l t o e a c h o t h e r ma y h e l p d e t e r mi n e w h i c h
o c u l a r mu s c l e g r o u p i s i n v o l v e d . C o mp l a i n t s o f v e r t i c a l d i p l o p i a ( w h i c h i n c l u d e s
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5.3 - Diplopia
d i a g o n a l d i p l o p i a ) a r e d u e t o mu s c l e g r o u p s a s s o c i a t e d w i t h t h e c r a n i a l n e r v e s
( C N s ) I I I a n d I V. T h e a b n o r ma l i t y ma y s t e m f r o m t h e s e n e r v e s o r, mo r e d i s t a l l y,
f r o m t h e mu s c l e s t h e ms e l v e s ( i . e . , my a s t h e n i a g r a v i s , mu s c l e e n t r a p me n t ) .
C o mp l a i n t s o f h o r i zo n t a l d i p l o p i a i n d i c a t e a n a b n o r ma l i t y w i t h t h e l a t e r a l / me d i a l
r e c t u s mu s c l e s a n d / o r C N V I ( b i n o c u l a r v e r t i c a l d i p l o p i a ) .
P. 7 7
3. A s u d d e n o n s e t o f d i p l o p i a ma y s u g g e s t a v a s c u l a r e t i o l o g y.
4. A h i s t o r y o f a n y i n s t i g a t i n g e v e n t s s u c h a s f a c i a l t r a u ma , s i n u s i n f e c t i o n , o r
mi g r a i n e h e a d a c h e ma y p r o v i d e i mp o r t a n t d i a g n o s t i c i n f o r ma t i o n .
5. T h e r e v i e w o f s y mp t o ms s h o u l d i n c l u d e q u e s t i o n s a b o u t a h i s t o r y o f f e v e r,
h e a d a c h e , s i n u s c o n g e s t i o n , a n d a s s o c i a t e d n e u r o l o g i c c o mp l a i n t s .
6. A f a mi l y h i s t o r y o f t h y r o i d d i s o r d e r s , my a s t h e n i a g r a v i s , o r d i a b e t e s ma y s u g g e s
a n a u t o i mmu n e c a u s e .
7. O t h e r s i g n i f i c a n t p a s t me d i c a l h i s t o r y i n c l u d e s d i a b e t e s ( r e t i n o p a t h y, t h i r d C N
p a l s y ) , h y p e r t e n s i o n , a n d u n d e r l y i n g v a s c u l a r 2)
d i .s e a s e s (
8. A s k t h e p a t i e n t w h e t h e r t h e s y mp t o m i mp r o v e s w i t h g a zi n g i n a c e r t a i n d i r e c t i o n
I f t h e g a ze i mp r o v e s ( a l t h o u g h i t i s u n l i k e l y t o r e s o l v e ) , t h e c a u s e i s u s u a l l y d u e
t o a n e u r o mu s c u l a r p r o b l e m o r a me c h a n i c a l r e1)
s t. r i c t i o n (
T h e e xa mi n a t i o n s h o u l d i n c l u d e :
1. O b s e r v a t i o n
L o o k f o r a n y e v i d e n c e o f s t r a b i s mu s ( ma l a l i g n me n t o f t h e g l o b e s
a s i n d i c a t e d b y a n a b n o r ma l c o r n e a l l i g h t r e f l e x) , c a t a r a c t s , c o r n e a l
a b n o r ma l i t i e s s u c h a s s c a r s , e v i d e n c e o f c e l l u l i t i s , e y e l i d p t o s i s ( my a s t h e n i a
g r a v i s , C N I I I p a l s y ) , e y e l i d r e t r a c t i o n ( t h y r o i d o p h t h a l mo p a t h y ) , o r p e r i o r b i t a l
e c c h y mo s e s ( t r a u ma ) . I f t h e p a t i e n t ' s h e a d i s b e i n g h e l d a t a t i l t , c o n s i d e r a
l e s i o n i n v o l v i n g t h e s u p e r i o r o b l i q u e mu s c l e ( a n d i t s c o r r e s p o n d i n g C N V I ) .
2. A u s c u l t a t i o Lni s t e n o v e r t h e c l o s e d e y e f o r a c a r o t i d c a v e r n o u s f i s t u l a b r u i t .
3. P a l p a t i o nP a l p a t e f o r a s t e p - o f f o r a n y t e n d e r n e s s t o s u g g e s t a p e r i o r b i t a l
fracture.
C. Testing
T he following office-based testing should be considered:
1. Vi s u a l a c u i t y s h o u l d i n c l u d e t e s t i n g t h e p a t i e n t ' s u n c o r r e c t e d a n d c o r r e c t e d
vision.
2. C o v e r t e s t c o v e r e a c h e y e s e q u e n t i a l l y. I f t h e d i p l o p i a p e r s i s t s w h e n o n e e y e
is covered (i.e., the diplopia persists with just one eye open) the patient has
mo n o c u l a r d i p l o p i a . M o n o c u l a r i mp l i e s a n a b n o r ma l i t y o f j u s t o n e e y e ( t h e b a d
eye). When the offending eye is covered, the diplopia resolves. When the good
e y e i s c o v e r e d , t h e d i p l o p i a p e r s i s t s . I f t h e p a t i e n t ' s v i s i o n r e t u r n s t o n o r ma l
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5.3 - Diplopia
when either the left or right eye is covered, the patient has binocular diplopia.
T h i s i s a n e xt r e me l y u s e f u l t e s t t o h e l p n a r r o w t h e c a u s e s o f Fd i gp .l o p i a ( s e e
5 . 3 . 1) . F u r t h e r e v a l u a t i o n f o r t h e c a u s e s o f mo n o c u l a r d i p l o p i a c a n b e f o c u s e d
o n t h e a b n o r ma l e 2)
y e. (
3. Vi s u a l a c u i t y w i t h a p i n h o l e i s mo s t h e l p f u l f o r p a t i e n t s w h o h a v e mo n o c u l a r
diplopia. If the offending eye is covered with a pinhole, the visual acuity in that
e y e o f t e n i mp r o v e s . T h i s p o i n t s t o a p r o b l e m w i t h 3)
r e.f r a c t i o n (
4. R a n g e o f o c u l a r mo v e me n t s a n d v i s u a l f i e l d t e s t i n g t h e s e t e s t s ma y b e u s e d t o
f u r t h e r n a r r o w t h e d i f f e r e n t i a l d i a g n o s i s . I n o r b i t a l t r a u ma , f o r e xa mp l e ,
e n t r a p me n t ( o r c o n t u s i o n ) o f t h e i n f e r i o r r e c t u s a n d / o r t h e i n f e r i o r o b l i q u e
mu s c l e s o r t h e i r n e r v e s ma y w o r s e n t h e d4)i p. l o p i a (
5. P u p i l l a r y e v a l u a t i o n ma y r e v e a l a s y mme t r i c p u p i l s . C o n s i d e r a C N I I I p a l s y.
P. 7 8
F I G U R E 5 . 3 . 1T. h e d i p l o p i a c o v e r t e s t .
6. A n a b n o r ma l c o r n e a l r e f l e x i n d i c a t e s a p r o b l e m w i t h t h e a l i g n me n t o f t h e g l o b e s
t h e ms e l v e s . T h i s p r o d u c e s a b i n o c u l a r d i p l o p i a ( a l t h o u g h n o t e xc l u s i v e l y ) .
F u r t h e r i ma g i n g , s u c h a s w i t h a c o mp u t e d t o mo g r a p h y ( C T ) s c a n o r a ma g n e t i c
r e s o n a n c e i ma g i n g ( M R I ; w i t h c o n t r a s t ) ma y b e w2)
a r. r a n t e d (
7. I f a n o c u l a r mu s c l e g r o u p i s s u s p e c t e d i n b i n o c u l a r d i p l o p i a , a P a r k s 3 - s t e p t e s
i s u s e d t o d e t e r mi n e t h e p a r t i c u l a r mu s c l e s . C o n s u l t a n o p h t h a l mo l o g y t e xt , o r
s e e r e f e r e n c e s a2n d3.
8. F a t i g a b i l i t y o f o c u l a r mu s c l e s s u g g e s t s my a s t h e n i a g r a v i s .
9. C o n s i d e r a C T s c a n o r a n M R I o f t h e s k u l l a n d o r b i t s i f t h e r e i s c o n c e r n f o r
ma s s , f r a c t u r e , i n c r e a s e d i n t r a c r a n i a l p r e s s u r e , s i n u s d i s e a s e , o r v a s c u l a r
a b n o r ma l i t y. A T e n s i l o n t e s t ma y b e o r d e r e d i f my a s t h e n i a g r a v i s i s s u s p e c t e d .
I V. Diagnosis
T he differential diagnosis for diplopia, which can often be substantially narrowed
b a s e d o n t h e h i s t o r y a n d t h e p h y s i c a l e xa mi n a t i o n , i s b r o k e n i n t o t w o ma i n g r o u p s
147 / 652
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5.3 - Diplopia
mo n o c u l a r a n d b i n o c u l a r d i p l o p i a .
1. C a u s e s o f m o n o c u l a r d i p l oCpoiran e a l d i s t o r t i o n s ( s c a r s , k e r a t o c o n u s ) ,
mu l t i p l e o p e n i n g s i n t h e i r i s , c a t a r a c t , l e n s d i s p l a c e me n t ( e . g . , M a r f a n ' s
s y n d r o me ) , a d v a n c e d a s t i g ma t i s m, p s e u d o p h a k o s ( a r t i f i c i a l i n t r a o c u l a r l e n s )
s u b l u xa t i o n , v i t r e o u s a b n o r ma l i t i e s , r e t i n a l c o n d i t i o n s , c o n t a c t l e n s c o mp l i c a t i o n
i n t r a o c u l a r f o r e i g n b o d y, h e r p e s zo s t e r o p h t h a l mi c u s , o r b i t a l c e l l u l i t i s , o r b i t a l
f r a c t u r e ( f l o o r, me d i a l w a l l ) , o r b i t a l t u mo r s ( r h a b d o my o s a r c o ma ) , a r t e r i o v e n o u s
ma l f o r ma t i o n s ( c a r o t i d c a v e r n o u s f i s t u l a ) .
2. C a u s e s o f b i n o c u l a r d i p l oNpeirav e p a l s i e s ( a b d u c e n s , o c u l o mo t o r, t r o c h l e a r ) ,
mi g r a i n e h e a d a c h e , my a s t h e n i a g r a v i s , t h y r o i d o p h t h a l mo p a t h y, mo n o n e u r i t i s
mu l t i p l e x ( C N V I a b d u c e n s ) , d i a b e t i c C N I I I p a l s y ( n o r ma l p u p i l , h e a d a c h e , o r
pain around the orbit), diabetic palsies ( C N IV or V).
D i p l o p i a ma y c a u s e d i f f i c u l t y w i t h d e p t h p e r c e p t i o n , e s p e c i a l l y w i t h b a l a n c e , a n d
a c t i v i t i e s s u c h a s d r i v i n g o r o p e r a t i n g ma c h i n e r y. P a t i e n t s w i t h d i p l o p i a s h o u l d n o t
p e r f o r m t h e s e a c t i v i t2)
i e.s (
References
1 . Va u g h a n D G , A s b u r y T , R i o r d a n - EGvener
a P. al opht hal mol .ogy
N e w Yo r k ,
N Y: M c G raw- H ill M edical, 2003.
2 . We s s e l s I . D i p l o pM
i aedl
. i ne pl us enc y c l opedi a
( h t t p : / / w w w. n l m. n i h . g o v / me d l i n e p l u s / e n c y / a r t i c l e / 0 0 3 0 2 9 . h t m) . 2 0 0 4 .
3 . B r a zi s P W, L e e A G . B i n o c u l a r v e r t i c a l dM
i pay
l o op i aC.l i n P r oc
1998;73:55
66.
4 . We b b L AM
. anual of ey e emer genc
. iPes
h i l a d e l p h i a , PA : B u t t e r w o r t h H e i n e ma n , B a r t l e y, 2 0 0 4 .
148 / 652
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5.4 - Nystagmus
> T a b l e o f C o n t e n t s > 5 - E y e P r o b l e ms > 5 . 4 - N y s t a g mu s
5.4
Nystagmus
Peter R. Lewis
I . Background
N y s t a g mu s i s a n i n v o l u n t a r y, r h y t h mi c o s c i l l a t i o n o f o n e o r b o t h e y e s i n a n y o r a l l
f i e l d s o f g a ze . I t ma y b e c o n t i n u o u s o r i n t e r mi t t e n t . N y s t a g mu s c a n b e c l a s s i f i e d i n t
t w o b a s i c t y p e s , j e r k ( t h e mo r e c o mmo n c l a s s i f i c a t i o n ) a n d p e n d u l a r.
A. Jerk nystagmus
T h i s c o n s i s t s o f a n i n i t i a l s l o w p h a s e , f o l l o w e d a n d n a me d f o r t h e d i r e c t i o n ( u p b e a t
d o w n b e a t , h o r i zo n t a l , t o r s i o n a l , o r mi xe d ) b y t h e c o r r e c t i v e s a c c a d e o r f a s t p h a s e
( jerk ) in the opposite direction.
B. Pendular nystagmus
T h i s c o n s i s t s o f s mo o t h b a c k a n d f o r t h ( p e n d u l a r ) mo v e me n t ( h o r i zo n t a l , v e r t i c a l ,
t o r s i o n a l , o r mi xe d ) o f t h e e y e ( s ) .
P. 7 9
T h e a p p r o p r i a t e d e v e l o p me n t , f u n c t i o n , a n d i n t e g r a t i o n o f t h e v i s u a l / o c u l o mo t o r a n d
v e s t i b u l a r s y s t e ms a r e r e q u i r e d f o r o p t i ma l f o c u s a n d t r a c k i n g o f v1)i s. u a l o b j e c t s (
N y s t a g mu s ma y b e a s s o c i a t e d w i t h a b n o r ma l i t i e s o f c e n t r a l o r p e r i p h e r a l o r i g i n i n
t h i s c o mp l e x n e u r o l o g i c n e t w o r k , a l t h o u g h i n ma n y i n s t a n c e s a p r e c i s e c a u s e ma y
n o t b e i d e n t i f i e2)d . (A n y f o r m o f v i s i o n l o s s ma y b e a s s o c i a t e d w i t h n y s t a g mu s .
E xt r e me - g a ze e v o k e d n y s t a g mu s i s e f f e c t i v e l y p h y s i o l o g i c , o c c u r r i n g i n
a p p r o xi ma t e l y o n e h a l f o f t h e p o p u l a t i o n .
A. Etiology
J e r k o r p e n d u l a r n y s t a g mu s ma y b e c h a r a c t e r i ze d a s b e i n g e i t h e r c o n g e n i t a l / i n f a n t i
or acquired.
1. C o n g e n i t a l / i n f a n t i l e n y s t a g mu s i s mo s t c o mmo n l y a s s o c i a t e d w i t h u n d e r l y i n g
s e n s o r y ( e f f e r e n t ) v i s u a l a b n o r ma l i t i e s . I t ma y n o t b e c o me e v i d e n t u n t i l s e v e r a l
mo n t h s o f a g e . I f n o e f f e r e n t d e f e c t i s i d e n t i f i e d t h e n y s t a g mu s i s j u d g e d t o b e
i d i o p a t h i c , p r e s u ma b l y d u e t o a d e f e c t i n t h e o c u l o mo t o r c o mp l e x.
2. A c q u i r e d n y s t a g mu s t h a t d e v e l o p s l a t e r i n l i f e i s mo r e l i k e l y t o b e
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n e u r o p a t h o l o g i c a n d a s s o c i a t e d w i t h a l i f e - t h r e a t e n i n g d i s o r d e r.
a. T w o f o r ms o f a c q u i r e d n y s t a g mu s w h e n s e e n i n t h e y o u n g a r e n o t e w o r t h y.
i. O p s o c l o n u s i s c h a r a c t e r i ze d b y r e p e t i t i v e , i r r e g u l a r, a n d
mu l t i d i r e c t i o n a l ( d a n c i n g e y e s o r s a c c a d o ma n i a ) e y e mo v e me n t s ,
o p s o c l o n u s ma y b e a s s o c i a t e d w i t h c e r e b e l l a r o r b r a i n s t e m d i s e a s e ,
p o s t v i r a l me n i n g i t i s , o r n e u r o b l a s t o ma .
i i. S p a s mu s n u t a n s i s t h e r a r e t r i a d o f h e a d t u r n ( t o r t i c o l l i s ) , n y s t a g mu s ,
a n d h e a d b o b b i n g . T h e n y s t a g mu s c a n b e mo n o c u l a r o r b i n o c u l a r a n d
d i s s o c i a t e d ; o f l o w a mp l i t u d e a n d h i g h f r e q u e n c y ; a n d w i t h h o r i zo n t a l
o r v e r t i c a l p e n d u l a r mo v e me n t s . I t mo s t c o mmo n l y d e v e l o p s b e t w e e n 6
mo n t h s t o 3 y e a r s i n o t h e r w i s e h e a l t h y c h i l d r e n . T h i s u s u a l l y r e s o l v e s
between the ages of 2 and 8 years. Of note, an identical clinical picture
c a n b e p r o d u c e d b y a g l i o ma o f t h e o p t i c c h i a s m o r n e a r b y s t r u c t u r e s .
b. A c q u i r e d f o r ms o f n y s t a g mu s t h a t o c c u r mo r e c o mmo n l y i n a d u l t s i n c l u d e
the following:
i. S e e s a w n y s t a g mu s , i n w h i c h t h e mo v e me n t s a r e p e n d u l a r. O n e e y e
rises and rotates inward, whereas the other descends and rotates
outward. T his is frequently seen with lesions of the optic chiasm or
t h i r d v e n t r i c l e , a s ma y o c c u r w i t h a p a r a s e l l a r ma s s ( e . g . ,
c r a n i o p h a r y n g i o ma o r p i t u i t a r y a d e n o ma ) .
i i. D o w n b e a t i n g n y s t a g mu s , i n w h i c h t h e f a s t p h a s e b e a t s d o w n a n d ma y
b e a s s o c i a t e d w i t h a l e s i o n o f t h e c e r v i c o me d u l l a r y j u n c t i o n a t t h e l e v e l
o f t h e f o r a me n ma g n u m. A r n o l d - C h i a r i ma l f o r ma t i o n a n d
s p i n o c e r e b e l l a r d e g e n e r a t i o n a r e t h e mo s t c o mmo n c a u s e s . O s c i l l o p s i a
( t h e i n t e r mi t t e n t o r c o n s t a n t s e n s a t i o n o f t h e e n v i r o n me n t mo v i n g b a c k
a n d f o r t h ) ma y b e p r e s e n t .
i i i. U p b e a t i n g n y s t a g mu s , i n w h i c h t h e f a s t p h a s e b e a t s u p a n d i s o f l a r g e
o r s ma l l a mp l i t u d e . T h e a s s o c i a t e d l e s i o n c o mmo n l y i n v o l v e s t h e b r a i n
s t e m o r v e r mi s o f t h e c e r e b e l l u m a s o c c u r s i n s t r o k e , t u mo r, o r
degeneration.
i v. C o n v e r g e n c e r e t r a c t i o n n y s t a g mu s , w h i c h i s ma r k e d b y t h e
c o n v e r g e n c e o f t h e e y e s w i t h j e r k n y s t a g mu s a n d t h e r e t r a c t i o n o f t h e
g l o b e o n u p g a ze , e y e l i d r e t r a c t i o n , l i mi t a t i o n i n u p g a ze , a n d l a r g e
u n r e a c t i v e p u p i l s . T h i s i s c a u s e d b y mi d b r a i n a b n o r ma l i t i e s .
v. P e r i o d i c a l t e r n a t i n g n y s t a g mu s , i n w h i c h t h e f a s t p h a s e o c c u r s i n o n e
direction with a head turn for 60 to 90 seconds, and then reverses
d i r e c t i o n w i t h a n i n t e r me d i a t e n e u t r a l zo n e . T h i s c a n b e s e e n w i t h
v e s t i b u l o c e r e b e l l a r d i s e a s e ( s t r o k e , mu l t i p l e s c l e r o s i s , s p i n o c e r e b e l l a r
d e g e n e r a t i o n ) , s e v e r e b i l a t e r a l v i s u a l l o s s ( o p t i c a t r o p h y, d e n s e
v i t r e o u s h e mo r r h a g e ) , o r i t c a n b e c o n g e n i t a l .
v i. G a ze - e v o k e d n y s t a g mu s , w h i c h i s a t y p e o f j e r k n y s t a g mu s t h a t
a p p e a r s o n l y w h e n t h e e y e s l o o k t o t h e s i d e . P a t h o l o g i c f o r ms a r e mo s t
c o mmo n l y s e e n w i t h a l c o h o l o r o t h e r c e n t r a l n e r v o u s s y s t e m ( C N S )
depressants. Cerebellar brain stem disorders can also be associated
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w i t h t h i s t y p e o f n y s t a g mu s .
P. 8 0
v i i. Ve s t i b u l a r n y s t a g mu s , w h i c h i s c a u s e d b y t h e d y s f u n c t i o n o f t h e i n n e r
e a r, a u d i t o r y n e r v e , o r t h e c e n t r a l n u c l e a r c o mp l e x. P e r i p h e r a l
vestibular disease (e.g., labyrinthitis, Mnire's disease, neuronitis,
v a s c u l a r i s c h e mi a , t r a u ma , o r d r u g t o xi c i t y ) p r o d u c e s u n i d i r e c t i o n a l j e r k
n y s t a g mu s w i t h a f a s t p h a s e o p p o s i t e t h e l e s i o n t h a t i s u s u a l l y
h o r i zo n t a l . C o mmo n a s s o c i a t e d s y mp t o ms i n c l u d e v e r t i g o , t i n n i t u s ,
h e a r i n g l o s s , a n d v o mi t i n g . C e n t r a l ( n u c l e a r ) d i s e a s e ( e . g . ,
d e my e l i n a t i n g d i s o r d e r, t u mo r, t r a u ma , o r s t r o k e ) , b y c o n t r a s t , i s
c h a r a c t e r i ze d b y u n i d i r e c t i o n a l o r b i d i r e c t i o n a l n y s t a g mu s t h a t ma y b e
p u r e l y h o r i zo n t a l , v e r t i c a l , o r r o t a t o r y a n d i s c h a r a c t e r i s t i c a l l y t o w a r d
t h e s i d e o f t h e l e s i o n . Ve r t i g o , t i n n i t u s , a n d d e a f n e s s a r e mi l d , i f
p r e s e n t , a n d s y mp t o ms a r e n o t r e l i e v e d w i t h e y e f i xa t i o n a s i n
peripheral disease.
v i i .i T o r s i o n a l n y s t a g mu s , w h i c h i s u s u a l l y c o n s t a n t a n d d u e t o a n
a s s o c i a t e d mi d b r a i n ( e . g . , p o n s , me d u l l a ) l e s i o n i n v o l v i n g t h e v e s t i b u l a r
n u c l e i a s ma y o c c u r i n s t r o k e o r mu l t i p l e s c l e r o s i s . T h e n y s t a g mu s ma y
b e s u p e r i mp o s e d o n h o r i zo n t a l o r v e r t i c a l n y s t a g mu s a n d d i r e c t e d
toward or away from the side of the associated lesion.
i x. D i s s o c i a t e d n y s t a g mu s , i n w h i c h n y s t a g mu s i n o n e e y e i s d i f f e r e n t f r o m
t h e o t h e r. T h i s i s s e e n i n p o s t e r i o r f o s s a l e s i o n s . I f a n a b d u c t i o n
n y s t a g mu s i s p r e s e n t w i t h a n i n t e r n u c l e a r o p h t h a l mo p l e g i a , c o n s i d e r
mu l t i p l e s c l e r o s i s w i t h a l e s i o n i n v o l v i n g t h e me d i a l l o n g i t u d i n a l
fasciculus.
B. Epidemiology
P r e c i s e d a t a r e g a r d i n g t h e p r e v a l e n c e o f n y s t a g mu s a r e u n a v a i l a b l e .
I I I . Evaluation
A. History
A g e o f o n s e t , s e l f - i d e n t i f i e d p r e c i p i t a n t s , t i me c o u r s e , a s s o c i a t e d s y mp t o ms , f a mi l y
h i s t o r y o f v i s u a l i mp a i r me n t a n d / o r a s s o c i a t e d n e u r o l o g i c d i s o r d e r s , a n d f u n c t i o n a l
i mp a i r me n t s h o u l d b e e l i c i t e d f r o m t h e p a t i e n t o r p a r e n t . I n c h i l d r e n , i t i s n e c e s s a r y
t o i n q u i r e a b o u t a h i s t o r y o f p r e ma t u r i t y a n d r e l a t e d v i s u a l i mp a i r me n t ( s ) . B l u r r e d
v i s i o n , i f p r e s e n t , i s c h a r a c t e r i s t i c o f a n a c q u i r e d n y s t a g mu s . Ve r t i g o i mp l i e s
v e s t i b u l a r d i s e a s e . A s s o c i a t e d w e a k n e s s , n u mb n e s s , o r l o s s o f v i s i o n ma y b e
s u g g e s t i v e o f mu l t i p l e s c l e r o s i s . A h i s t o r y o f me d i c a t i o n a n d s u b s t a n c e u s e i s
i mp o r t a n t . M e d i c a t i o n s t h a t c a n i n d u c e n y s t a g mu s ( u s u a l l y d o w n b e a t o r u p b e a t )
i n c l u d e l i t h i u m, b a r b i t u r a t e s , p h e n y t o i n , s a l i c y l a t e s , a n d b e n zo d i a ze p i n e s .
N y s t a g mu s ma y a l s o b e s e e n w i t h p h e n c y c l i d i n e u s e . A c u t e a l c o h o l i n t o xi c a t i o n c a n
p r o d u c e a g a ze - e v o k e d n y s t a g mu s , a s d o e s t h e c h r o n i c a l c o h o l - i n d u c e d t h i a mi n
d e f i c i e n c y c o mmo n t o We r n i c k e ' s e n c e p h a l o p a t h y.
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T h i s s h o u l d b e g i n w i t h a d e v e l o p me n t a l a s s e s s me n t f o l l o w e d b y a v i s u a l
e xa mi n a t i o n . L o s s o f e s t a b l i s h e d v i s u a l a c u i t y i s t y p i c a l l y w o r s e i n a c q u i r e d
n y s t a g mu s . T h e a b i l i t y t o t r a c k mo v i n g o b j e c t s w i t h t h e h e a d i n a f i xe d p o s i t i o n
( o p t o k i n e t i c r e f l e x) t a k e s a n u mb e r o f mo n t h s t o ma t u r e a n d t y p i c a l l y f a i l s t o d e v e l o
i n c o n g e n i t a l n y s t a g mu s . T h e d i r e c t i o n , p l a n e , a n d a mp l i t u d e o f t h e e y e mo v e me n t
s h o u l d b e c h a r a c t e r i ze d . I t i s n e c e s s a r y t o e v a l u a t e a n y c a u s e o f p o o r v i s i o n t h a t
ma y c o n t r i b u t e t o t h e n y s t a g mu s . A n i r i d i a ( a b s e n c e o f i r i s ) o r i r i s t r a n s i l l u mi n a t i o n
seen in albinism should be sought. Congenital cataracts or corneal opacities have
p o o r r e d r e f l e xe s . I t i s i mp o r t a n t t o a n a l y ze t h e o p t i c n e r v e t o a s s e s s f o r h y p o p l a s i a
o r a t r o p h y. L a t e n t n y s t a g mu s ( s e e n o n l y w h e n o n e e y e i s c o v e r e d t h e b a s i s o f t h e
s c r e e n i n g c o v e r - u n c o v e r t e s t ) i s p r e s e n t i n i n f a n t i l e 3)
s t.r aAbl ti es mu
r e ds h( e a d
p o s i t i o n a n d h e a d b o b b i n g ma y b e a s s o c i a t e d w i t h c o n g e n i t a l n y s t a g mu s a n d
s p a s mu s n u t a n s . I f b e n i g n p a r o xy s ma l p o s i t i o n a l v e r t i g o i s s u s p e c t e d , t h e D i xH a l l p i k e ' s ma n e u v e r ma y b e p e r f o r me d i n a n e f f o r t t o r e p r o d u c e t h e p a t i e n t ' s
s y mp t o ms4)( . A c o mp l e t e n e u r o l o g i c a s s e s s me n t s h o u l d b e p e r f o r me d . A p p r o p r i a t e
n e u r o l o g y a n d o p h t h a l mo l o g y c o n s u l t a t i o n ( s ) s h o u l d b e r e q u e s t e d a s i n d i c a t e d .
C. Testing
Urine drug screening for alcohol or barbiturates should be considered when

s i g n i f i c a n t g a ze - e v o k e d n y s t a g mu s i s o b s e r v e d . S e r u m d r u g l e v e l s o f p h e n y t o i n o r
lithium should be obtained as indicated. Additional blood tests to be considered
i n c l u d e v i t a mi n1 2B, ma g n e s i u m, a n d t o xo p l a s mo s i s a n d / o r h u ma n i mmu n o d e f i c i e n c y
v i r u s s e r o l o g i e s . I f a C N S i n f e c t i o n i s s u s p e c t e d ( e . g . , h e r p e s s i mp l e x) , t h e n
c e r e b r o s p i n a l f l u i d a n a l y s i s i s i n d i c a t e d . M a g n e t i c r e s o n a n c e i ma g i n g ( M R I ) ( a s
seen
P. 8 1
i n t h e s u b s e q u e n t t e xt ) s h o u l d b e o b t a i n e d b e f o r e t h e l u mb a r p u n c t u r e . O c u l a r
albinism can be associated with a bleeding disorder secondary to platelet
d y s f u n c t i o n ( H e r ma n s k y - P u d l a k s y n d r o me ) , o r w h i t e b l o o d c e l l d y s f u n c t i o n w i t h
i n c r e a s e d s u s c e p t i b i l i t y t o i n f e c t i o n a n d l y mp h o ma ( C h d i a k - H i g a s h i s y n d r o me ) .
R e s p e c t i v e l y, a b l e e d i n g t i me a n d a p o l y mo r p h o n u c l e a r l e u k o c y t e f u n c t i o n t e s t
s h o u l d b e o r d e r e d . U r i n a r y v a n i l l y l ma n d e l i c a c i d s h o u l d b e o b t a i n e d i n a p a t i e n t w i t
o p s o c l o n u s t o l o o k f o r t h e b y - p r o d u c t s o f a n e u r o b l a s t o ma .
D. Genetics
Va r i a b l e p a t t e r n s o f i n h e r i t a n c e h a v e b e e n a s s o c i a t e d w i t h c o n g e n i t5)
a l. n y s t a g mu s (
C e r t a i n a u t o s o ma l d o mi n a n t a t a xi a s a r e a s s o c i a t e d w i t h d o w n b e a t n y s t a g mu s .
S y mp t o ms u s u a l l y b e g i n i n l a t e a d u l t h o o d .
I V. Diagnosis
Other involuntary ocular oscillations include:
1. O c u l o g y r i c c r i s i s c o n s i s t s o f n o n r h y t h mi c , s u s t a i n e d , a n d i r r e g u l a r e y e
d e v i a t i o n s . T h i s i s s e e n i n p h e n o t h i a zi n e ( e . g . , p r o c h l o r p e r a zi n e ) t o xi c i t y.
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2. O c u l a r b o b b i n g i s c h a r a c t e r i ze d b y f a s t , c o n j u g a t e , d o w n w a r d mo v e me n t o f t h e
e y e , f o l l o w e d b y a s l o w d r i f t t o t h e p r i ma r y p o s i t i o n o f g a ze . T h i s i s s e e n i n
c o ma t o s e p a t i e n t s w i t h l a r g e p o n t i n e l e s i o n s ( e . g . , h e mo r r h a g e , s t r o k e , o r
t u mo r ) . O b s t r u c t i v e h y d r o c e p h a l u s o r me t a b o l i c e n c e p h a l o p a t h y c a n a l s o c a u s e
t h i s t y p e o f e y e mo v e me n t .
3. S u p e r i o r o b l i q u e my o k y mi a i s c h a r a c t e r i ze d b y s ma l l u n i l a t e r a l , v e r t i c a l , a n d
r o t a t o r y mo v e me n t s o f o n e e y e . S y mp t o ms o f o s c i l l o p s i a w o r s e n w h e n l o o k i n g
d o w n w a r d a n d i n w a r d . T h i s i s u s u a l l y b e n i g n a n d s e l f - l i mi t e d , b u t h a s b e e n
n o t e d w i t h mu l t i p l e s c l e r o s i s .
4. N y s t a g mu s ma y o c c u r w i t h e s s e n t i a l h e a d t r e mo r, a n d ma y b e e xt i n g u i s h e d b y
f i xi n g t h e h e a d p o s i t i o n o r b y u t i l i zi n g a - b l o c k e r.
T h e c l i n i c a l ma n i f e s t a t i o n s o f n y s t a g mu s w e r e d i s c u s s e dI I .i nA. sBeecct ai ouns e

ma n y f o r ms o f n y s t a g mu s l o c a l i ze t o t h e p o s t e r i o r f o s s a o r a r e a s s o c i a t e d w i t h a
d e my e l i n a t i n g d i s o r d e r, a n M R I i s t h e i ma g i n g mo d a l i t y o f c h o i c e i f t h e c a u s e i s
o t h e r w i s e n o t i d e n t i f i e d . I n a p a t i e n t w i t h o p s o c l o n u s , a n a b d o mi n a l c o mp u t e d
t o mo g r a p h y ( C T ) s c a n o r a n M R I s h o u l d b e d o n e t o l o o k f o r n e u r o b l a s t o ma i n v o l v i n
t h e a d r e n a l g l a n d s . A n a b d o mi n a l u l t r a s o u n d o r a C T s c a n i s n e e d e d t o e v a l u a t e t h e
k i d n e y s i n a n i r i d i a , b e c a u s e t h e r e i s a s i g n i f i c a n t i n c i d e n c e o f a s s o c i a t e d W i l ms '
t u mo r.
References
1 . L e i g h R J , Z e e DTShe
. neur ol ogy of ey e mov ement
, 3 d se d . O xf o r d : O xf o r d
University Press, 1999.
2 . S e r r a A , L e i g h R J . D i a g n o s t i c v a l u e o f n y s t a g mu s : s p o n t a n e o u s a n d i n d u c e d
o c u l a r o s c i l l a t i o Jn sN. eur ol N eur os ur g P s y c hi 2at0r0y2 ; 7 3 ( 6 ) : 6 1 5 6 1 8 .
3 . S i mo n J W, K a w P. C o mmo n l y mi s s e d d i a g n o s e s i n t h e c h i l d h o o d e y e
e xa mi n a t i o nA. m F am P hy s i c i an
2001;64(4):623628.
4 . S w a r t z R , L o n g w e l l P. T r e a t me n t o f vAem
r t iFg am
o . P hy s i c i an
2 0 0 5 ; 7 1 ( 6 ) : 111 5 11 2 2 .
5 . G o t t l o b I . N y s t a g mu
C sur. r O pi n O pht hal mol
2001;12(5):378383.
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5.5 - Papilledema
> T a b l e o f C o n t e n t s > 5 - E y e P r o b l e ms > 5 . 5 - P a p i l l e d e ma
5.5
Papilledema
Peter R. Lewis
I . Background
P a p i l l e d e ma i s a n o p t i c d i s c s w e l l i n g p r o d u c e d b y i n c r e a s e d i n t r a c r a n i a l p r e s s u r e .
ma y b e d e t e c t e d i n t h e a s y mp t o ma t i c p e d i a t r i c o r a d u l t p a t i e n t d u r i n g t h e c o u r s e o f
s c r e e n i n g f u n d u s c o p i c e xa mi n a t i o n w i t h a n o n l i f e - t h r e a t e n i n g c a u s e . C o n v e r s e l y, i
ma y b e i d e n t i f i e d i n i n i t i a l l y a s y mp t o ma t i c o r g r a v e l y s y mp t o ma t i c p a t i e n t s ( i n c l u d i n
p r e g n a n t w o me n ) a s a ma r k e r o f a l i f e - t h r e a t e n i n g c o n d i t i o n s u c h a s s u b a r a c h n o i d
h e mo r r h a g e , me n i n g i t i s , o r b r a i n 1)
t u.mo r (
A. Etiology
T r u e p a p i l l e d e ma i s a l w a y s a s s o c i a t e d w i t h i n c r e a s e d i n t r a c r a n i a l p r e s s u r e . T h e
mo s t o f t e n c i t e d c a u s e s i n c l u d e t r a u ma , p r i ma r y o r me t a s t a t i c i n t r a c r a n i a l t u mo r,
aqueductal stenosis (as is seen in certain types of congenital hydrocephalus),
p s e u d o t u mo r c e r e b r i ( i d i o p a t h i c i n t r a c r a n i a l h y p e r t e n s i o n ; f r e q u e n t l y mi s d i a g n o s e d
a s mi g r a i n e h e a d a c 2)
h e, ) (s u b d u r a l h e ma t o ma , s u b a r a c h n o i d h e mo r r h a g e ,
a r t e r i o v e n o u s ma l f o r ma t i o n s , b r a i n a b s c e s s , me n i n g i t i s , e n c e p h a l i t i s , a n d s a g i t t a l
s i n u s t h r o mb o s i s .
B. Epidemiology
M o s t p a t i e n t s w i t h p a p i l l e d e ma a r e a d u l t s . M a n y o f t h e c a u s e s o f p a p i l l e d e ma ( e . g .
s u b a r a c h n o i d h e mo r r h a g e a n d c a n c e r ) a r e mo r e c o mmo n w i t h a d v a n c i n g a g e .
P s e u d o t u mo r c e r e b r i i s c h a r a c t e r i s t i c a l l y d i s c o v e r e d i n o v e r w e i g h t a d o l e s c e n t
w o me n . I n d i v i d u a l s w i t h i mmu n o s u p p r e s s i o n ( e . g . , h u ma n i mmu n o d e f i c i e n c y v i r u s
[ H I V ] / a c q u i r e d i mmu n o d e f i c i e n c y s y n d r o me , c h e mo t h e r a p y, c h r o n i c p r e d n i s o n e
therapy) are at heightened risk of central nervous system (C N S) infections (e.g.,
me n i n g i t i s , e n c e p h a l i t i s , a n d b r a i n a b s c e s s ) t h a t ma y b e a s s o c i a t e d w i t h
p a p i l l e d e ma .
I I I . Evaluation
T h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n a r e t a i l o r e d t o t h e a g e , s e t t i n g , a n d u r g e n c y o
the patient presentation. Patients with an acute and rapid increase in intracranial
p r e s s u r e w i t h a s s o c i a t e d p a p i l l e d e ma ma y b e mo r i b u n d a n d c o ma t o s e , r e q u i r i n g
e me r g e n t a n d e xp e d i t e d a s s e s s me n t a n d c a r e . I n l e s s d r a ma t i c p r e s e n t a t i o n s ,
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5.5 - Papilledema
p a t i e n t s p r e s e n t w i t h n o n s p e c i f i c s y mp t o ms ( e . g . , h e a d a c h e ; s e e i n t h e s u b s e q u e n t
t e xt )1)
( that sparks a search, initially on the basis of the history and physical
e xa mi n a t i o n , f o r a s s o c i a t e d i n t r a c r a n i a l p r e s s u r e a n d i t s c a u s e . A l t e r n a t i v e l y, t h e
search for clues to the presence and the cause of increased intracranial pressure
o n l y b e g i n s s u b s e q u e n t t o t h e d i s c o v e r y o f p a p i l l e d e ma d u r i n g t h e c o u r s e o f a
s c r e e n i n g f u n d u s c o p i c e xa mi n a t i o n .
A. History
I n t h e s y mp t o ma t i c p a t i e n t , h e a d a c h e , n a u s e a , v o mi t i n g , d i p l o p i a , f o c a l w e a k n e s s ,
f e v e r, n e c k s t i f f n e s s , a n d p h o t o p h o b i a , a n d / o r f l e e t i n g l o s s o f v i s i o n ( o b s c u r a t i o n s )
especially with the head in dependent positionsraise the index of suspicion for
i n c r e a s e d i n t r a c r a n i a l p r e s s u r e . P a r e n t s o f i n f a n t s o r c h i l d r e n ma y r e p o r t i n c r e a s e d
h e a d s i ze o r d e c r e a s e d a l e r t n e s s . A f t e r p r o b i n g p a t i e n t c o mp l a i n t s a n d a s s o c i a t e d
s y mp t o ms , d e t e r mi n e t h e p r e s e n c e o r r i s k f a c t o r s f o r v a s c u l a r d i s e a s e ( i n c l u d i n g
p r i o r s t r o k e ) , c a n c e r, t r a u ma , o r i mmu n o s u p p r e s s i o n . A t h o r o u g h me d i c a t i o n a n d
d r u g u s e h i s t o r y s h o u l d a l s o b e o b t a i n e d . A f a mi l y h i s t o r y o f c o n d i t i o n s r e l a t e d t o
increased intracranial pressure should be elicited.
1. T h i s c o mme n c e s w i t h v i t a l s i g n s , i n c l u d i n g b l o o d p r e s s u r e a n d v i s u a l a c u i t y.
Rarely is a decrease in visual acuity seen in association with increased
intracranial pressure; if present, it typically suggests other causes (e.g., vein
o c c l u s i o n , a n t e r i o r i s c h e mi c o p t i c n e u r o p a t h y, o r o p t i c n e u r i t i s a s s e e n i n t h e
s u b s e q u e n t t e xt ) .
2. A d d i t i o n a l f e a t u r e s o f a d e t a i l e d o p h t h a l mo l o g i c e xa mi n a t i o n , i n c l u d i n g
f u n d u s c o p i c a s s e s s me n t , s h o u l d b e o b t a i n e d . S i xt h n e r v e p a l s i e s s h o w l i mi t e d
lateral
P. 8 3
g a ze a n d ma y b e a s s o c i a t e d w i t h h o r i zo n t a l d i p l o p i a , w h e r e a s t h i r d n e r v e
p a l s i e s d e mo n s t r a t e a l i mi t a t i o n i n me d i a l g a ze , e l e v a t i o n , a n d d e p r e s s i o n .
R e g a r d i n g t h e f u n d u s c o p i c e xa mi n a t i o n i n t h e a s y mp t o ma t i c p a t i e n t , i t i s
n e c e s s a r y t o t a k e c a r e t o f i r s t d e t e r mi n e i f t h e r e i s t r u e d i s c e d e ma o r o n l y
p s e u d o p a p i l l e d e ma ( t h i s ma y w e l l r e q u i r e c o n s u l t a t i o n w i t h a n o p h t h a l mo l o g i s t ) .
P s e u d o p a p i l l e d e ma i s o p t i c n e r v e h e a d e l e v a t i o n c a u s e d b y h y a l i n e d e p o s i t i o n
( d r u s e n ) w i t h i n t h e o p t i c n e r v e h e a d i t s e l f , a n d i s r e p o r t e d t o b e mo r e c o mmo n
i n w h i t e s3)( . T h e r e i s n o a s s o c i a t e d i n c r e a s e d i n t r a c r a n i a l p r e s s u r e o r C N S
p a t h o l o g y. W h e n t r u e p a p i l l e d e ma i s p r e s e n t , i t i s t y p i c a l l y b i l a t e r a l . D i s c e d e m
p r o d u c e s a n o b s c u r i n g o f t h e b l o o d v e s s e l s ' ma r g i n s . T i n y s p l i n t e r h e mo r r h a g e s
are seen in and around the optic nerve. Spontaneous venous pulsations (S VPs)
s h o u l d b e s o u g h t . I f S V P s a r e p r e s e n t , t h e r e i s n o r ma l i n t r a c r a n i a l p r e s s u r e .
P r o mi n e n t r e t i n a l h e mo r r h a g e s s u g g e s t ma l i g n a n t h y p e r t e n s i o n o r c e n t r a l r e t i n a
v e i n o c c l u s i o n ( a s s e e n i n t h e s u b s e q u e n t t e xt ) .
3. A c o mp l e t e h e a d a n d n e c k e xa mi n a t i o n t o c h e c k f o r n e c k s t i f f n e s s , t e mp o r a l
artery tenderness, pain in and around the eyes, and sinus tenderness is also
i mp o r t a n t . T h o r o u g h v a s c u l a r a n d n e u r o l o g i c e xa mi n a t i o n s a r e i n d i c a t e d .
M e a s u r e h e a d c i r c u mf e r e n c e i n i n f a n t s a n d y o u n g c h i l d r e n ; c h e c k f o r b u l g i n g o r
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5.5 - Papilledema
p r e ma t u r e l y c l o s e d f o n t a n e l s i n t h e f o r me r g r o u p .
C. Testing
I f t r u e p a p i l l e d e ma i s f o u n d , l a b o r a t o r y t e s t i n g a n d c l i n i c a l i ma g i n g s h o u l d b e
d i r e c t e d a t d e t e r mi n i n g t h e c a u s e a n d t h e s e v e r i t y o f t h e a s s o c i a t e d i n c r e a s e d
i n t r a c r a n i a l p r e s s u r e . S u g g e s t e d l a b o r a t o r y t e s t s i n c l u d e s e d i me n t a t i o n r a t e , C reactive protein, and white blood count if C N S infection is suspected. Serologies fo
H I V, s y p h i l i s , a n d h e r p e s s h o u l d b e o b t a i n e d a s i n d i c a t e d . A l u mb a r p u n c t u r e f o r t h
me a s u r e me n t o f o p e n i n g p r e s s u r e a n d a c e r e b r o s p i n a l f l u i d ( C S F ) e xa mi n a t i o n t o
e v a l u a t e f o r e v i d e n c e o f me n i n g i t i s , t u mo r, o r h e mo r r h a g e s h o u l d b e p e r f o r me d o n l y
a f t e r r u l i n g o u t a c o mp r e s s i v e l e s i o n w i t h a c o mp u t e d t o mo g r a p h y ( C T ) s c a n o r
ma g n e t i c r e s o n a n c e i ma g i n g ( M R I ) . I f t r u e p a p i l l e d e ma i s s u s p e c t e d b y t h e p r i ma r y
c a r e c l i n i c i a n a n d / o r c o n f i r me d i n c o n s u l t a t i o n w i t h a n o p h t h a l mo l o g i s t , t h e n
d i a g n o s t i c i ma g i n g i s ma n d a t o r y. A C T s c a n w i t h a n d w i t h o u t c o n t r a s t s h o u l d b e
o r d e r e d . I f t h e C T s c a n i s i n c o n c l u s i v e , a n M R I w i l l b e p a r t i c u l a r l y h e l p f u l i n i ma g i n
brain stem and cerebellar lesions, which can obstruct C S F flow in children and
a d u l t s . A n M R I a n g i o g r a p h y ma y b e r e q u i r e d t o i d e n t i f y a r e l a t e d v a s c u l a r
a b n o r ma l i t y. A C T s c a n i s t h e p r e f e r r e d t e c h n i q u e t o i ma g e a c u t e i n t r a c r a n i a l
b l e e d i n g . A n u l t r a s o u n d o f t h e o p t i c d i s c ma y b e u s e d i f t h e d i a g n o s i s o f
p s e u d o p a p i l l e d e ma i s u n c e r t a i n .
D. Genetics
M a n y o f t h e u n d e r l y i n g c o n d i t i o n s a s s o c i a t e d w i t h p a p i l l e d e ma h a v e g e n e t i c
contributions.
I V. Diagnosis
D i s c s w e l l i n g w i t h o u t i n c r e a s e d i n t r a c r a n i a l p r e s s u r e ma y b e c a u s e d b y t h e f o l l o w i n
conditions:
1. O p t i c n e u r i t iAsn a f f e r e n t p u p i l l a r y d e f e c t e xi s t s a l o n g w i t h d e c r e a s e d v i s i o n
a n d p a i n o n e xt r a o c u l a r mo v e me n t . C o l o r v i s i o n w i l l b e d e c r e a s e d i n t h i s
n o r ma l l y u n i l a t e r a l c o n d i t i o n . I t ma y b e s e e n w i t h mu l t i p l e s c l e r o s i s .
2. M a l i g n a n t h y p e r t e n s (i o fn e s s e n t i a l o r s e c o n d a r y c a u s e s , i n c l u d i n g s e v e r e
p r e e c l a mp s i a ) . B l o o d p r e s s u r e i s ma r k e d l y e l e v a t e d . T h e e y e f i n d i n g s a r e
c h a r a c t e r i s t i c : b i l a t e r a l p r o mi n e n t d i s c e d e ma , f l a me h e mo r r h a g e s t h a t e xt e n d
p e r i p h e r a l l y, a n d c o t t o n w o o l s p o tCs h(aspetee r 7 ). .8
3. C e n t r a l r e t i n a l v e i n o c c l uTs hi oi sn i s c h a r a c t e r i ze d b y a u n i l a t e r a l d i s c
s w e l l i n g w i t h v e r y p r o mi n e n t f l a me a n d b l o t h e mo r r h a g e s , w i t h o u t i n c r e a s e d
blood pressure.
4. A n t e r i o r i s c h e m i c o p t i c n e u r o pTahtihs yma y b e d u e t o a r t e r i t i s ( e . g . ,
t e mp o r a l / g i a n t c e l l ) p r e s e n t i n g w i t h h e a d a c h e , s t i f f n e c k , t e mp o r a l t e n d e r n e s s
j a w c l a u d i c a t i o n , e l e v a t e d s e d i me n t a t i o n r a t e , a n d s e v e r e v i s u a l l o s s i n o n e e y e
followed by visual loss of the other eye in 60% of the cases. When arteritis is
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5.5 - Papilledema
a b s e n t , t y p i c a l l y n o s y mp t o ms a r e p r e s e n t e xc e p t d e c r e a s e d v i s i o n . A s s o c i a t e d
c o n d i t i o n s i n c l u d e s y s t e mi c h y p e r t e n s i o n , d i a b e t e s me l l i t u s , o r c o l l a g e n v a s c u l a
disorders.
P. 8 4
5. I n f i l t r a t i o n o f t h e o p t i c n T
e ur vbe r c u l o s i s g r a n u l o ma , l e u k e mi c i n f i l t r a t e ,
s a r c o i d o s i s , a n d me t a s t a t i c d i s e a s e a r e t h e mo r e c o mmo n e xa mp l e s o f
infiltrative processes. T he infiltration can be unilateral or bilateral and can lead
to rapid loss of vision. Radiation therapy can be helpful to preserve vision.
6. L e b e r ' s h e r e d i t a r y o p t i c n e u r o pTahti hs yu s u a l l y a f f e c t s me n i n t h e s e c o n d
o r t h i r d d e c a d e a n d i s c h a r a c t e r i ze d b y u n i l a t e r a l p r o g r e s s i v e l o s s o f v i s i o n w i t h
disc swelling.
7. D i a b e t i c p a p i l l i tI ti si s a n i s c h e mi c i n f a r c t i o n t o t h e n e r v e i n a d v a n c e d
d i a b e t i c s . T h i s i s o f t e n b i l a t e r a l a n d c a u s e s mi l d d i s c e l eCvhaatpi ot enr ( s e e
1 4 . 1) .
C h r o n i c p a p i l l e d e ma c a n r e s u l t i n o p t i c a t r o p h y ( w i t h d e c r e a s e d o p t i c n e r v e s w e l l i n
a n d p r o g r e s s i v e v i s u a l ( i n i t i a l l y p e r i p h e r a l ) l o s s t h a t ma y p r o g r e s s t o f r a n k b l i n d n e s
S u c h p a t i e n t s s h o u l d b e f o l l o w e d w i t h s e r i a l p e r i me t r y b y a n o p h t h a l mo l o g i s t .
References
1 . C l i n c h C R . E v a l u a t i o n o f a c u t e h e a d a c h e s i nA m
a dFuam
l t s . P hy s i c i an
2001;63(4):685692.
2 . B r a zi s P W, L e e A G . E l e v a t e d i n t r a c r a n i a l p r e s s u r e a n d p s e u d o t u mo r c e r e b r i .
C ur r O pi n O pht hal mol
1998;9(6):2732.
3 . G i o v a n n i n i J , C h r o u s o sP api
G . l l edema
. eMedicine
w w w. e me d i c i n e . c o m/ o p h / t o p i c 1 8 7 . h t m, a c c e s s e d o n A u g u s t 2 , 2 0 0 5 .
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5.6 - Pupillary Inequality
> T a b l e o f C o n t e n t s > 5 - E y e P r o b l e ms > 5 . 6 - P u p i l l a r y I n e q u a l i t y
5.6
Pupillary Inequality
Dav id C. Holub
I . Background
A n i s o c o r i a i s d e f i n e d a s a n i n e q u a l i t y o f p u p i l l a r y s i ze ( d i a me t e r ) .
A. Etiology
1. T h e p u p i l i s a h o l e o r a p e r t u r e i n t h e i r i s t h a t p e r mi t s t h e p a s s a g e o f l i g h t
t h r o u g h t o t h e r e t i n a . T h e r e a r e t w o mu s c l e s w i t h i n t h e i r i s t h a t c o n t r o l p u p i l l a r
s i ze . T h es phi nc t er pupi l l ae
i s a c i r c u l a r mu s c l e t h a t c o n t r o l s p u p i l l a r y
c o n s t r i c t i o n , o r mi o s i s . diT lhate or pupi l l ae
i s a r a d i a l mu s c l e t h a t c o n t r o l s
p u p i l l a r y d i l a t i o n , o r my d r i a s i s .
2. T h e p u p i l l a r y c o n s t r i c t o r i s i n n e r v a t e d b y f i b e r s f r o m t h e p a r a s y mp a t h e t i c
a u t o n o mi c n e r v o u s s y s t e m. T h e s e f i b e r s o r i g i n a t e i n t h e mi d b r a i n a n d t r a v e l t o
t h e E d i n g e r - We s t p h a l n u c l e u s i n t h e d o r s a l mi d b r a i n , a n d t h e n w i t h t h e c r a n i a l
n e r v e ( C N ) I I I ( t h e o c u l o mo t o r n e r v e ) t h r o u g h t h e c a v e r n o u s s i n u s t o t h e o r b i t .
T hey diverge from the C N III to synapse in the orbit with the ciliary ganglion.
Postganglionic short ciliary nerves then innervate the pupillary constrictor
mu s c l e . L e s i o n s a t a n y a n a t o mi c s i t e a l o n g t h i s p a t h w a y l e a d t o p a t h o l o g i c
my d r i a s i s1)( .
P. 8 5
3. T h e p u p i l l a r y d i l a t o r mu s c l e i s i n n e r v a t e d b y f i b e r s f r o m t h e s y mp a t h e t i c
a u t o n o mi c n e r v o u s s y s t e m. T h e s e n e u r o n s o r i g i n a t e i n t h e h y p o t h a l a mu s ,
descend through the brain stem to the C8-T 2 lateral horn, and then travel with
t h e c e r v i c a l s y mp a t h e t i c t r u n k t o t h e s u p e r i o r c e r v i c a l g a n g l i o n . P o s t g a n g l i o n i c
long ciliary nerves travel with the internal carotid artery until the superior orbita
f i s s u r e , w h e r e t h e y d i v e r g e a n d c o n t i n u e t o t h e d i l a t o r p u p i l l a e mu s c l e . T h e s e
f i b e r s b o t h s t i mu l a t e t h e d i l a t o r p u p i l l a e a n d i n h i b i t t h e p u p i l l a r y c o n s t r i c t o r a n d
c i l i a r y mu s c l e s . T h e y a l s o i n n e r v a t e t h e l e v a t o r p a l p e b r a e mu s c l e , a n d t h e r e f o r e
c o n t r i b u t e t o e y e l i d e l e v a t i o n . L e s i o n s a t a n y a n a t o mi c s i t e a l o n g t h i s p a t h w a y
l e a d t o p a t h o l o g i c mi o1)
s i.s (
B. Epidemiology
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P h y s i o l o g i c a n i s o c o r i a i s c o mmo n , a f f e c t i n g p e r h a p s a s mu c h a s 2 0 % o f n o r ma l
i n d i v i d u a l s . T h e d i f f e r e n c e i n p u p i l l a r y s i ze i s s ma l l , u s u2)a.l l y < 1 mm (
I I I . Evaluation
A. History
I n mo s t p a t i e n t s , a n i s o c o r i a i s d i s c o v e r e d i n c i d e n t a l l y ; p r e s e n t i n g s y mp t o ms a r e
r e l a t i v e l y u n c o mmo n . I t i s n e c e s s a r y t o i n q u i r e a b o u t o c u l a r s y mp t o ms s u c h a s p a i n
r e d n e s s , t e a r i n g , o r p h o t o p h o b i a . A n y p a s t h i s t o r y o f e y e d i s e a s e , i n j u r y, s u r g e r y, o
me d i c a t i o n s s h o u l d b e e l i c i t e d .
T h e p u p i l s s h o u l d b e e xa mi n e d i n b o t h d i m a n d b r i g h t l i g h t s , a s s e s s i n g b o t h d i r e c t
a n d c o n s e n s u a l p u p i l l a r y l i g h t r e f l e xe s . A n a f f e r e n t p u p i l l a r y d e f e c t ( M a r c u s G u n n
p u p i l ) p e r mi t s a c o n s e n s u a l l i g h t r e f l e x, b u t n o p u p i l l a r y c o n s t r i c t i o n o r p a r a d o xi c a l
pupillary dilation to direct light. In an efferent pupillary defect, pupillary constrictio
t o b o t h d i r e c t a n d c o n s e n s u a l l i g h t r e f l e xe s i s a b s e n t .
1. T h e c r i t i c a l f i r s t s t e p i n a s s e s s i n g a p a t i e n t w i t h a n i s o c o r i a i s t o d e t e r mi n e
w h i c h i s t h e a b n o r ma l p u p i l ( i . e . , i s o n e p u p i l a b n o r ma l l y c o n s t r i c t e d o r i s o n e
p u p i l a b n o r ma l l y d i l a t e d ? ) . I t i s n e c e s s a r y t o e xa mi n e t h e p u p i l l a r y r e s p o n s e s i n
both bright and dim lighting. T he pupil that does not dilate in dim light or
c o n s t r i c t i n b r i g h t l i g h t i s t h e a b n o r ma l o n e .
2. E xt r a o c u l a r mo v e me n t s s h o u l d b e t e s t e d . I n p a t i e n t s w i t h t h i r d n e r v e p a l s y, t h e
a f f e c t e d e y e s h o w s o u t w a r d d e v i a t i o n d u r i n g p r i ma r y g a ze . I t i s a b l e t o c o me t o
mi d l i n e o n l y w i t h a t t e mp t s a t i n w a r d g a ze , a n d d o w n w a r d g a ze l e a d s t o i n w a r d
rotation.
C. Testing
C o mp u t e d t o mo g r a p h y o r ma g n e t i c r e s o n a n c e i ma g i n g i s v a l u a b l e f o r s u s p e c t e d
i n t r a c r a n i a l ma s s l e s i o n s o r b l e e d i n g . A n g i o g r a p h y i s i n d i c a t e d f o r s u s p e c t e d
cerebral aneurysm or carotid dissection. Obtaining chest radiography for occult lun
ma l i g n a n c y i s r e a s o n a b l e i n p a t i e n t s w i t h H o r n e r ' s s y n d r o me .
I V. Diagnosis
1. T h e d i f f e r e n t i a l d i a g n o s i s o f mi o s i s i n c l u d e s H o r n e r ' s s y n d r o me , u n i l a t e r a l
a n t e r i o r u v e i t i s , L y me d i s e a s e , o r s y p h i l i s .
2. T h e d i f f e r e n t i a l d i a g n o s i s o f my d r i a s i s i n c l u d e s p a l s y o f o r d a ma g e t o C N I I I ,
A d i e ' s p u p i l , t h e u s e o f c e r t a i n me d i c a t i o n s ( w i t h a n t i c h o l i n e r g i c e f f e c t s ) , a n d
a c u t e a n g l e c l o s u r e g l a u c o ma .
159 / 652
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1. P a t h o l o g i c mi o s i s ma y b e c a u s e d b y a l e s i o n o r d i s e a s e s t a t e t h a t a f f e c t s a n y
a n a t o mi c s i t e f r o m t h e b r a i n t o t h e p u p i l i t s e l f . H o r n e r ' s s y n d r o me i s t h e t r i a d o
mi o s i s w i t h p t o s i s a n d i p s i l a t e r a l f a c i a l a n h i d r o s i s ( v a r i a b l y p r e s e n t d e p e n d i n g
o n t h e l e v e l o f t h e l e s i o n ) . I t i s c a u s e d b y d a ma g e t o t h e c e r v i c a l t h o r a c i c
s y mp a t h e t i c f i b e r s . A b r a i n s t e m s t r o k e ma y l e a d t o t h e s e f i n d i n g s . D i s e a s e o f
t h e i n t e r n a l c a r o t i d a r t e r y, s u c h a s d i s s e c t i o n , ma y a l s o i n t e r r u p t t h e s i g n a l s
n o r ma l l y c a r r i e d b y t h e s e n e r v e s . H o r n e r ' s s y n d r o me a l s o f r e q u e n t l y o c c u r s d u e
t o t u mo r s t h a t c o mp r e s s t h e s e n e r v e f i b e r s . T h e s e ma y i n c l u d e p a r o t i d g l a n d
t u mo r s , c a r o t i d b o d y t u mo r s , l y mp h o ma w i t h e n l a r g e d c e r v i c a l a d e n o p a t h y,
me d i a s t i n a l t u mo r s , o r a p i c a l l u n g t u mo r s ( t y p i c a l l y i n t h e s u p e r i o r s u l c u s ) .
D i r e c t t r a u ma c a n a l s o d a ma g e t h e s y mp a t h e t i c n e r3)v .e M
f i iboesriss (ma y
also be caused by unilateral ocular disease such as anterior uveitis, in which
i n f l a mma t i o n l e a d s t o t h e d e v e l o p me n t o f a d h e s i o n s ( s y n e c h i a e ) b e t w e e n t h e
i r i s a n d t h e a n t e r i o r l e n s c a p s u l e . L y me d i s e a s e a n d n e u r o s y p h i l i s ma y i n v o l v e
the eye as well, leading to an Argyll R obertson pupilan irregular pupil that
r e a c t s p o o r l y t o l i g h t b u t n o r ma l l y t o a c c o mmo d a t i o n .
2. P a t h o l o g i c my d r i a s i s i s t y p i c a l l y c a u s e d b y d i s e a s e s t a t e s t h a t a f f e c t t h e
o c u l o mo t o r n e r v e ( C N I I I ) a n d t h e r e b y t h e p a r a s y mp a t h e t i c f i b e r s t h a t t r a v e l w i t
t h i s n e r v e . O t h e r c l i n i c a l ma n i f e s t a t i o n s o f a t h i r d n e r v e p a l s y i n c l u d e
a b n o r ma l i t i e s o f o c u l a r mo v e me n t a n d p t o s i s . N e u r o l o g i c d i s e a s e s s u c h a s
mu l t i p l e s c l e r o s i s ma y c a u s e t h i r d n e r v e p a l s y. N e r v e c o mp r e s s i o n c a n o c c u r
f r o m v a s c u l a r p h e n o me n a ( p o s t e r i o r c o mmu n i c a t i n g a r t e r y a n e u r y s m) , i n c r e a s e d
i n t r a c r a n i a l p r e s s u r e d u e t o h e a d t r a u ma w i t h b l e e d i n g , o r a n i n t r a c r a n i a l ma s s .
I n t h e c o ma t o s e p a t i e n t w i t h h e a d t r a u ma , a t e mp o r a l l o b e h e r n i a t i o n
c o mp r e s s i n g t h e
P. 8 6
mi d b r a i n ma y a l s o l e a d t o u n i l a t e r a l my d r i a s i s . U n i l a t e r a l my d r i a s i s ma y a l s o b e
a n i s o l a t e d f i n d i n g i n A d i e ' s p u p i l , c a u s e d b y d a ma g e t o t h e c i l i a r y g a n g l i o n
t h r o u g h i n f e c t i o n , i s c h e mi a , o r t r a u ma . P a t i e n t s a r e t y p i c a l l y w o me n , 2 0 t o 5 0
y e a r s o f a g e , a n d u s u a l l y a s y mp t o ma t i c . T h e y p r e s e n t w i t h a n i s o c o r i a ,
d i mi n i s h e d o r a b s e n t l i g h t r e f l e xe s ( b o t h d i r e c t a n d c o n s e n s u a l ) , a n d a n
e xa g g e r a t e d a n d p r o l o n g e d ( t o n i c ) p u p i l l a r y c o n s t r i c t i o n d u r i n g a c c o mmo d a t i o n .
3. U n i l a t e r a l p h a r ma c o l o g i c my d r i a s i s ma y o c c u r w i t h a n t i c h o l i n e r g i c me d i c a t i o n s .
4. A c u t e a n g l e c l o s u r e g l a u c o ma i s a c r u c i a l c o n s i d e r a t i o n i n t h e e v a l u a t i o n o f
u n i l a t e r a l my d r i a s i s . P a t i e n t s p r e s e n t w i t h e y e p a i n , r e d n e s s , a n d v i s u a l
i mp a i r me n t . A n e xa mi n a t i o n r e v e a l s a f i xe d , mi d - d i l a t e d p u p i l , a n d e l e v a t e d
intraocular pressure.
References
1 . M o s e n t h a l W.
A t ex t book of neur oanat.omy
London: Parthenon Publishing,
1995.
2 . E g g e n b e r g e r EA .ni s oc or i. ah t t p : / / w w w. e me d i c i n e . c o m/ o p h / t o p i c 1 6 0 . h t m,
accessed on July 2005.
160 / 652
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3 . B a r d o r f CH. or ner ' s s y ndr ome

. h t t p : / / w w w. e me d i c i n e . c o m/ O P H / t o p i c 3 3 6 . h t m,
accessed on July 2005.
161 / 652
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5.7 - Red Eye
> T a b l e o f C o n t e n t s > 5 - E y e P r o b l e ms > 5 . 7 - R e d E y e
5.7
Red Eye
Dav id C. Holub
I . Background
A.
T h e r e d e y e i s o n e o f t h e mo s t c o mmo n o c u l a r c o mp l a i n t s e n c o u n t e r e d i n p r i ma r y
c a r e . A l t h o u g h ma n y c a u s e s o f t h e r e d e y e a r e b e n i g n , s o me a r e t r u e e me r g e n c i e s .
F a i l u r e t o a c t a c c o r d i n g l y ma y p o s e a n i mme d i a t e t h r e a t t o t h e p a t i e n t ' s v i s i o n .
B.
A n a t o mi c a l l y, a l mo s t a n y s t r u c t u r e i n t h e e y e o r i t s s u r r o u n d i n g t i s s u e s ma y ma n i f e s
with redness.
A. Etiology
T h e p r i ma r y c a u s e s o f a r e d e y e a r e i n f e c t i o n o r t r a u ma t o t h e v a r i o u s a n a t o mi c
s t r u c t u r e s o f t h e e y e . O c c a s i o n a l l y, c o n n e c t i v e t i s s u e d i s e a s e o r a p r i ma r y o c u l a r
d i s e a s e c a n a l s o ma n i f e s t w i t h a r e d e y e . C a u s e s i n c l u d e t h e f o l l o w i n g :
1. S c l e r i t i si s a n i n f l a mma t i o n o f t h e s c l e r a , t h e f i b r o u s o u t e r e n v e l o p e o f t h e e y e .
2. E p i s c l e r i t iiss a n i n f l a mma t i o n o f t h e c o n n e c t i v e t i s s u e t h a t l i e s b e t w e e n t h e
sclera and the conjunctiva.
3. C o n j u n c t i v i t i s a n i n f l a mma t i o n o f t h e c o n j u n c t i v a , t h e mu c o u s me mb r a n e s
that line the anterior surface of the eye and the posterior surface of the eyelid.
4. B l e p h a r i t i iss a n i n f l a mma t i o n o f t h e e y e l i d , w h i c h ma y a p p e a r r e d .
5. A h o r d e o l u mi s a s u p e r f i c i a l i n f l a mma t o r y g r a n u l o ma o f t h e e y e l i d t h a t d e v e l o p s
a c u t e l y f r o m a n o b s t r u c t i o n o f t h e o c u l a r s e b a c e o u s g l a n d s ( Z e i s o r me i b o mi a n
glands).
6. A c h a l a z i o nma n i f e s t s s i mi l a r l y, b u t i s a d e e p e r - s e a t e d g r a n u l o ma t o u s
obstruction of these glands.
7. A c u t e d a c r y o c y s t i rt ei ss u l t s f r o m a n o b s t r u c t i o n a n d s e c o n d a r y i n f e c t i o n o f
t h e l a c r i ma l s y s t e m.
8. I r i t i so r a n t e r i o r u v e i tiinsv o l v e s a n i n f l a mma t i o n o f t h e mi d d l e p a r t o f t h e e y e
162 / 652
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5.7 - Red Eye
(iris and ciliary body).

9. K e r a t i t i so c c u r s w h e n t h e c o r n e a , t h e t r a n s p a r e n t o u t e r c o v e r i n g o f t h e a n t e r i o r
e y e , b e c o me s i n f e c t e d o r i s i n j u r e d t r a u ma t i c a l l y, r e s u l t i ncgo er ni tehaelr i n a
a b r a s i o no r ac o r n e a l f o r e i g n b o d y .
1 0. H y p h e m ,a o r h e mo r r h a g e i n t o t h e a n t e r i o r c h a mb e r, w h i c h a l s o r e s u l t s f r o m
t r a u ma , ma y ma n i f e s t w i t h v i s i b l e r e d n e s s i f t h e a mo u n t o f b l e e d i n g
P. 8 7
i s s i g n i f i c a n t . E v e n r e l a t i v e l y mi n o r t r a u ma ma ys ul ebacdo n
t oj uan c t i v a l
h e m o r r h a g, ee v i d e n t a s a p r o mi n e n t r e d a r e a i n t h e n o r ma l l y w h i t e s c l e r a .
11. A c u t e a n g l e c l o s u r e g l a u c loem
a das t o a r a p i d r i s e i n i n t r a o c u l a r p r e s s u r e
a n d ma y ma n i f e s t a s r e d n e s s a l o n g w i t h o c u l a r p a i n , v i s u a l l o s s , h e a d a c h e , a n d
nausea.
1 2. C o n t a c t d e r m a t i, tai st o p i c d e r m a t i,t pi se r i o r b i t a l c e l l u l, i toi rso r b i t a l
c e l l u l i t i sc a u s e t h e s k i n a n d s o f t t i s s u e s s u r r o u n d i n g t h e e y e o r i n v o l v i n g t h e
e y e l i d t o b e c o me i n f l a me d o r i n f e c t e d .
B. Epidemiology
C o n j u n c t i v i t i s i s t h e mo s t c o mmo n e y e c o mp l a i n t i n t h e U n i t e d S t a t e s , a c c o u n t i n g f o
3 0 % o f a l l a c u t e o c u l a r c o mp l 1)
a i .n t s (
I I I . Evaluation
A. History
A n a s s e s s me n t o f t h e r e d e y e b e g i n s w i t h q u e s t i o n s r e g a r d i n g i t s l o c a t i o n
(unilateral, bilateral, or unilateral spreading to bilateral), onset (sudden or gradual
d u r a t i o n ( a c u t e , s u b a c u t e , o r c h r o n i c ) , i n c i t i n g f a c t o r s ( s u c h a s t r a u ma ) , a n d
a s s o c i a t e d s i g n s a n d s y mp t o ms . O c u l a r p a i n , s w e l l i n g , p h o t o p h o b i a , o r d i s t u r b a n c e
i n v i s u a l a c u i t y a r e f r e q u e n t l y s i g n s o f a mo r e e me r g e n t c o n d i t i o n . O t h e r a s s o c i a t e d
s y mp t o ms ma y i n c l u d e c r u s t i n g , d i s c h a r g e , i t c h i n g , b u r n i n g , t e a r i n g , d r y e y e s , o r a
f o r e i g n b o d y s e n s a t i o n . S y s t e mi c s y mp t o ms s u c h a s f e v e r, h e a d a c h e , a b d o mi n a l
p a i n , n a u s e a / v o mi t i n g , r h i n o r r h e a , o r c o u g h s h o u l d b e e l i c i t e d . A p a s t h i s t o r y o f
s i mi l a r o c u l a r c o mp l a i n t s i s i mp o r t a n t . T h e p a t i e n t s h o u l d b e a s k e d a b o u t t h e u s e o
c o n t a c t l e n s e s o r e y e d r o p s . T h e p a s t me d i c a l h i s t o r y ma y i n c l u d e c e r t a i n s y s t e mi c
d i s e a s e s t h a t p r o d u c e o c u l a r i n v o l v e me n t , s u c h a s s y p h i l i s , i n f l a mma t o r y b o w e l
d i s e a s e , a n d s o me c o l l a g e n v a s c u l a r d i s e a s e s ( s a r c o i d o s i s , a n k y l o s i n g s p o n d y l i t i s ,
r e a c t i v e a r t h r i t i s , r h e u ma t o i d a r t h r i t i s , a n d S j g r e n ' s s y n d r o me ) . T h e r e ma y a l s o b e
h i s t o r y o f a l l e r g i c o r a t o p i c d i s o r d e r s s u c h a s a s t h ma , e c ze ma , o r a l l e r g i c r h i n i t i s .
1. T h e p h y s i c a l e xa mi n a t i o n o f t h e e y e s h o u l d a l w a y s b e g i n w i t h a n a s s e s s me n t o f
v i s u a l a c u i t y w i t h a w a l l - mo u n t e d o r h a n d h e l d S n e l l e n ' s e y e c h a r t . Vi s u a l a c u i t y
s h o u l d a l w a y s b e me a s u r e d e i t h e r w i t h t h e p a t i e n t ' s c o r r e c t i v e l e n s e s o r t h r o u g h
p i n h o l e t e s t i n g . Vi s u a l d i s t u r b a n c e s o w i n g s o l e l y t o r e f r a c t i v e e r r o r i mp r o v e w i t h
t h e u s e o f l e n s e s o r p i n h o l e t e s t i n g . Vi s u a l i mp a i r me n t d u e t o o r g a n i c e y e
163 / 652
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5.7 - Red Eye
disease does not.
2. O n c e t h i s h a s b e e n c o mp l e t e d , a s i g n i f i c a n t p o r t i o n o f t h e p h y s i c a l e xa mi n a t i o n
c a n t h e n b e e a s i l y a c c o mp l i s h e d i n t h e p r i ma r y c a r e o f f i c e s e t t i n g w i t h o u t
s p e c i a l i ze d e q u i p me n t . A viins
s upec
a l t i ono f t h e e y e a n d i t s s u r r o u n d i n g s o f t
t i s s u e s i s e s s e n t i a l t o d e t e r mi n e w h e r e t h e r e d n e s s i s l o c a t e d a n a t o mi c a l l y ( i . e .
s c l e r a , c o n j u n c t i v a , a n t e r i o r c h a mb e r, e y e l i d , o r p e r i o r b i t a l t i s s u e s ) . O t h e r
notable findings include the presence of any lid swelling or crusting, purulent
d i s c h a r g e , t e a r i n g , e r y t h e ma o r s w e l l i n g o f t h e p e r i o r b i t a l t i s s u e s , o r p r o p t o s i s .
Ve s i c u l a r s k i n l e s i o n s n e a r t h e e y e s u g g e s t a h e r p e s i n f e c t i o n , w h i c h c a n h a v e
severe consequences on any infected structures within the eye. A palpation of
t h e g l o b e a n d s u r r o u n d i n g s o f t t i s s u e s f o r f i r mn e s s o r t e n d e r n e s s s h o u l d t h e n b
p e r f o r me d . A l i mi t a t i o n o f o r t e n d e r n e s s w i t h e xt r a o c u l a r mo v e me n t s s h o u l d b e
assessed. Eyelid eversion should be carried out to look for retained foreign
b o d i e s . F i n a l l y, a p u p i l l a r y e xa mi n a t i o n s h o u l d b e u n d e r t a k e n t o a s s e s s s i ze ,
s h a p e , a n d r e a c t i v i t y.
3. F u r t h e r e xa mi n a t i o n o f t h e e y e r e q u i r e s b o t h t h e a p p r o p r i a t e e q u i p me n t a n d t h e
s k i l l t o u s e t h i s e q u i p me n t a n d i n t e r p r e t t h e f i n d i n g s c o r r e c t l y. A l t h o u g h t h i s i s
f e a s i b l e i n t h e p r i ma r y c a r e s e t t i n g , c l i n i c i a n s s h o u l d o b t a i n o p h t h a l mo l o g i c
c o n s u l t a t i o n a t t h i s t i me i f n e e d e d .
4. A s l i t l a mp e xa mi n a t i o n s h o u l d b e p e r f o r me d t o a s s e s s f o r a b n o r ma l i t i e s o f t h e
a n t e r i o r c h a mb e r, s u c h a s t h e p r e s e n c e o f r e d o r w h i t e b l o o d c e l l s o r v i s i b l e
f l o a t i n g p a r t i c u l a t e ma t t e r ( f l a r e ) . T h e s l i t l a mp i s a mo r e p r e c i s e me t h o d f o r
me a s u r i n g a n t e r i o r c h a mb e r d e p t h t h a n l a t e r a l v i s u a l i n s p e c t i o n o f t h e e y e w i t h
a penlight. T he cornea, with the application of fluorescein staining and the
c o b a l t b l u e l i g h t s o u r c e o n t h e s l i t l a mp , c a n b e a s s e s s e d f o r a b r a s i o n ,
u l c e r a t i o n , o r a f o r e i g n b o d y. S o me p a t i e n t s ma y b e u n a b l e t o t o l e r a t e t h i s
e xa mi n a t i o n d u e t o
P. 8 8
pain or photophobia. In this instance, a topical anesthetic such as tetracaine
ma y b e a p p l i e d t o t h e e y e .
5. T h e me a s u r e me n t o f i n t r a o c u l a r p r e s s u r e i s i mp o r t a n t i n t h e e v a l u a t i o n f o r a c u t
a n g l e c l o s u r e g l a u c o ma . T h i s c a n b e a c c o mp l i s h e d w i t h a S c h i t z t o n o me t e r i n
t h e p r i ma r y c a r e s e t t i n g . I n t h e o p h t h a l mo l o g i s t ' s o f f i c e , a g o n i o s c o p y o r t h e
me a s u r e me n t o f t h e a n g l e b e t w e e n t h e i r i s a n d c o r n e a , c a n a l s o b e p e r f o r me d .
6. A f u n d u s c o p i c e xa mi n a t i o n s h o u l d b e u n d e r t a k e n , i d e a l l y a f t e r p u p i l l a r y
d i l a t a t i o n h a s b e e n e f f e c t e d t h r o u g h t h e i n s t a l l a t i o n o f a my d r i a t i c s o l u t i o n . A s
pupillary dilatation can precipitate an attack of acute angle closure, it is
e s s e n t i a l t h a t t h i s d i a g n o s i s b e e xc l u d e d p r i o r t o a t t e mp t i n g t h i s . U n d e r d i r e c t
o p h t h a l mo s c o p y, t h e c o r n e a , l e n s , a n d v i t r e o u s h u mo r c a n b e a s s e s s e d f o r
p a t h o l o g y.
C. Testing
Relatively few studies are useful in the differential diagnosis of the red eye. Cultur
of purulent discharge are occasionally helpful if bacterial conjunctivitis is suspecte
B l o o d c u l t u r e s a n d a c o mp l e t e b l o o d c o u n t w i t h d i f f e r e n t i a l a r e p r u d e n t i n c a s e s o f
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5.7 - Red Eye
s u s p e c t e d o r b i t a l c e l l u l i t i s . Wo r k u p f o r r h e u ma t o l o g i c d i s e a s e ma y b e c o n s i d e r e d i n
p a t i e n t s w i t h i r i t i s , e p i s c l e r i t i s , o r s c l e r i t i s . I ma g i n g s t u d i e s a r e r2)a .r e l y o f v a l u e (
I V. Diagnosis
G i v e n t h e p o t e n t i a l s e v e r i t y o f s o me o f t h e c a u s e s o f r e d e y e , t h e p r i ma r y c a r e
p h y s i c i a n ' s f i r s t p r i o r i t y i s t o d e t e r mi n e i f i mme d i a t e i n t e r v e n t i o n o r o p h t h a l mo l o g i c
r e f e r r a l i s w a r r a n t e d . T h e mo s t s e r i o u s c a u s e s o f t h e a c u t e r e d e y e t h a t n e e d t o b e
r u l e d o u t i n c l u d e a c u t e a n g l e c l o s u r e g l a u c o ma , h y p h e ma , o r b i t a l c e l l u l i t i s , a c u t e
k e r a t i t i s , c o r n e a l u l c e r, s c l e r i t i s , a n d i r i t i s / u v e i t i s . L e s s s e r i o u s c a u s e s i n c l u d e
c o n j u n c t i v i t i s , l i d d i s o r d e r s , a n d s u b c o n j u n c t i v a l h e mo r r h a g e s .
1. A c u t e a n g l e c l o s u r e g l a u c porme sa e n t s w i t h s e v e r e u n i l a t e r a l e y e p a i n a n d
b l u r r y v i s i o n , o f t e n a c c o mp a n i e d b y v i s u a l h a l o s , h e a d a c h e , a b d o mi n a l p a i n , a n d
n a u s e a a n d v o mi t i n g . T h e p h y s i c a l e xa mi n a t i o n r e v e a l s a s h a l l o w a n t e r i o r
c h a mb e r e i t h e r b y p e n l i g h t o r s l i t l a mp e xa mi n a t i o n , p a r t i a l l y d i l a t e d p u p i l s t h a t
r e s p o n d p o o r l y t o l i g h t , f i r mn e s s o f g l o b e o n p a l p a t i o n , a n d e l e v a t e d i n t r a o c u l a r
p r e s s u r e o n t o n o me 3)
t r y. (
2. S c l e r i t i st y p i c a l l y p r e s e n t s w i t h p a i n , t e a r i n g , p h o t o p h o b i a , a n d d e c r e a s e d
v i s u a l a c u i t y. Vi s i b l e s w e l l i n g a c c o mp a n i e s t h e r e d n e s s . D i s c h a r g e i s t y p i c a l l y
absent. Fifty percent of the cases are bilateral.
3. I r i t i so r u v e i t i su s u a l l y p r e s e n t s a s b l u r r e d v i s i o n , p a i n , a n d p h o t o p h o b i a
affecting a single eye. Discharge is typically absent, although occasionally
p a t i e n t s ma y h a v e w a t e r y d i s c h a r g e . P h y s i c a l e xa mi n a t i o n r e v e a l s a s l u g g i s h l y
reactive pupil with pain and photophobia on both direct and consensual pupillar
t e s t i n g . S l i t l a mp e xa mi n a t i o n a l s o r e v e a l s p r o t e i n a c e o u s o r c e l l u l a r ma t t e r i n
t h e a n t e r i o r c h a mb e r ( f l a r e ) .
4. K e r a t i t i sc o mmo n l y p r e s e n t s w i t h s e v e r e e y e p a i n , p h o t o p h o b i a , a n d b l u r r y
v i s i o n . A s l i t l a mp e xa mi n a t i o n w i t h f l u o r e s c e i n s t a i n i n g i d e n t i f i e s a n y d i s r u p t i o n
i n t h e c o r n e a l e p i t h e l i u m. O w i n g t o i t s s e r i o u s n a t u r e , t h i s mu s t b e r e g a r d e d a s
a corneal infection until proven otherwise.
5. H y p h e m ama y b e p r e c e d e d b y a h i s t o r y o f o c u l a r t r a u ma . Vi s i o n i s i mp a i r e d
o n l y w i t h a l a r g e q u a n t i t y o f b l o o d i n t h e a n t e r i o r c h a mb e r. P h y s i c a l e xa mi n a t i o n
s h o w s v i s i b l e b l o o d o n s l i t l a mp e xa mi n a t i o n , a d i l a t e d p u p i l , a n d e l e v a t e d
intraocular pressure.
6. O r b i t a lo r p e r i o r b i t a l c e l l u l ipt ri es s e n t s w i t h v i s i b l e e r y t h e ma , s w e l l i n g ,
w a r mt h , a n d t e n d e r n e s s o f t h e s k i n a n d s o f t t i s s u e s s u r r o u n d i n g t h e e y e . T h e
e y e l i d ma y b e i n v o l v e d . O r b i t a l c e l l u l i t i s , a mo r e s e r i o u s i n f e c t i o n , ma y ma n i f e s
w i t h f e v e r, p r o p t o s i s , a n d a b n o r ma l i t i e s o f v i s i o n o r p u p i l l a r y r e s p o n s e .
E xt r a o c u l a r mo v e me n t s ma y a l s o b e l i mi t e d . T h e s e f i n d i n g s a r e a b s e n t i n
periorbital cellulitis.
7. C o n j u n c t i v i t i s t h e mo s t c o mmo n c a u s e o f e y e r e d n e s s a n d ma y b e b a c t e r i a l ,
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5.7 - Red Eye
v i r a l , o r a l l e r g i c i n e t i o l o g y. A l l e r g i c c o n j u n c t i v i t i s u s u a l l y b e g i n s b i l a t e r a l l y,
P. 8 9
as opposed to infectious conjunctivitis, which often begins unilaterally and is
t h e n s p r e a d t o t h e o t h e r e y e d u e t o ma n i p u l a t i o n b y t h e p a t i e n t . I t c h i n g a n d
t e a r i n g a r e c o mmo n . A d i s c h a r g e i s u b i q u i t o u s c l e a r o r w a t e r y i n a l l e r g i c o r
v i r a l e t i o l o g i e s b u t p u r u l e n t i n b a c t e r i a l c a s e s . A l l e r g i c c o n j u n c t i v i t i s ma y b e
a c c o mp a n i e d b y o t h e r a l l e r g i c s y mp t o ms o r p h y s i c a l e xa mi n a t i o n f i n d i n g s .
Although cultures of purulent discharge are often negative, it is virtually
i mp o s s i b l e t o c l i n i c a l l y d i s t i n g u i s h b e t w e e n v i r a l a n d b a c t e r i a l c o n j u n c t i v i t i s .
T h e r e f o r e , e i t h e r c u l t u r e s o r e mp i r i c t r e a t me n t w i t h t o p i c a l a n t i b i o t i c s i s
w a r r a n t e d . N o t a b l e e xc e p t i o n s t o t h e t y p i c a l l y b e n i g n c o u r s e o f i n f e c t i o u s
c o n j u n c t i v i t i s a r e c a s e s o f g o n o c o c c a l a n d c h l a my d i a l c o n j u n c t i v i t i s . A h i s t o r y o
e xp o s u r e t o g e n i t a l s e c r e t i o n s i s c r i t i c a l , a s t h e c o u r s e o f t h e i l l n e s s a n d t h e
t r e a t me n t d i f f e r s s i g n i f i c a n t l y.
8. E p i s c l e r i t iasp p e a r s s i mi l a r t o s i mp l e c o n j u n c t i v i t i s . T h e r e d n e s s ma y b e
d e e p e r a n d mo r e l o c a l i ze d t h a n s e e n i n c o n j u n c t i v i t i s . D i s c h a r g e i s t y p i c a l l y
a b s e n t 1)( .
9. B l e p h a r i t i ps r e s e n t s a s r e d n e s s , c r u s t i n g , a n d o f t e n a s a s w e l l i n g o f t h e e y e l i d .
I t c o mmo n l y o c c u r s i n c o mb i n a t i o n w i t h c o n j u nhc ot irvdi tei so,l uam( w i t h
a s s o c i a t e d e y e l i d t e n d e r n e s s a n d p o s s i b l y a v i s i b l e a b sccheasl sa)z, i o nr a
(without associated eyelid tenderness or abscess).
1 0. D a c r y o c y s t i t ipsr e s e n t s w i t h u n i l a t e r a l r e d n e s s , s w e l l i n g , a n d t e n d e r n e s s o v e r
t h e l a c r i ma l s a c , w h i c h i s l o c a t e d i n t h e i n f e r o n a s a l q u a d r a n t o f t h e e y e . T h i s
c o n d i t i o n i s s e e n a l mo s t e xc l u s i v e l y i n c h i l d r e n a n d i n p a t i e n t s o v e r t h e a g e o f
40.
11. C o r n e a l a b r a s i o, nusl c e r a t i o n, sa n df o r e i g n b o d i ema

s nifest with a foreign
b o d y s e n s a t i o n , e y e p a i n , a n d t e a r i n g . A n e xa mi n a t i o n w i t h a s l i t l a mp u n d e r
fluorescein reveals dark green staining in areas where the corneal epithelium
has been disrupted. Lid eversion is essential to search for retained foreign
b o d i e s . A s s t a t e d i n t h e p r e c e d i n g t e xt , c o r n e a l i n f e c t i o n s mu s t f i r s t b e e xc l u d e d
1 2. S u b c o n j u n c t i v a l h e m o r r haapgpee a r s a s a l o c a l i ze d r e d d i s h d i s c o l o r a t i o n o f
t h e s c l e r a d u e t o mi n o r t r a u ma ( i n c l u d i n g s n e e zi n g o r c o u g h i n g ) .
References
1 . S i l v e r ma n M A , B e s s ma nC onj
E . unc t i v i .t i s
h t t p : / / w w w. e me d i c i n e . c o m/ e me r g / t o p i c 11 0 . h t m, a c c e s s e d o n J u l y 2 0 0 5 .
2 . F a r i n a G A , M a za r i n RGed
. ey e ev al uat .i on
h t t p : / / w w w. e me d i c i n e . c o m/ o p h / t o p i c 2 6 7 . h t m, a c c e s s e d o n J u l y 2 0 0 5 .
3 . B e e r s M H , B e r k o w R , eTdhe
s . M er c k manual of di agnos i s and t,her
1 7apy
th
e d . We s t P o i n t , PA : M e r c k & C o , 1 9 9 9 .
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5.8 - Scotoma
> T a b l e o f C o n t e n t s > 5 - E y e P r o b l e ms > 5 . 8 - S c o t o ma
5.8
Scotoma
Dav id C. Holub
I . Background
A s c o t o ma i s a f o c a l a r e a o f v i s u a l l o s s i n t h e p a t i e n t ' s v i s u a l f i e l d . P a t i e n t s
c o mmo n l y r e f e r t o a s c o t o ma a s a b l i n d s p o t . S c o t o ma t a ma y b e f u r t h e r c a t e g o r i ze
in several different ways.
A.
A s c o t o ma ma y b e c l a s s i f i e d b y i t s l o c a t i o n i n t h e v i scueanl tfri ae l ds. cAo t o m a
o c c u r s a t t h e p o i n t o f f i xa t i o n a n d c a u s e s s i g n i f i c a n t a n d i mme d i a t e l y n o t i c e a b l e
v i s u a l i mp a i r me n t t o t h e p a t i pe an rt .a cAe n t r a l s c o t o m
o cac u r s n e a r t h e p o i n t o f
f i xa t i o n a n d i s a l s o u s u a l l y n o t i c e a b l e t o t h e ppeart i epnhte. rAa l s c o t o m
o cac u r s
a w a y f r o m t h e p o i n t o f f i xa t i o n i n t h e p e r i p h e r y o f t h e v i s u a l f i e l d . P e r i p h e r a l
s c o t o ma t a ma y b e a s y mp t o ma t i c a n d o n l y d i s c o v e r e d o n v i s u a l f i e l d t e s t i n g
p e r f o r me d f o r a d i f f e r e n t r e a s o n .
P. 9 0
B.
A p o s i t i v e s c o t o mma
a nifests as a black spot in the patient's visual field. A
n e g a t i v e s c o t o mma
a nifests as a blank spot in the patient's visual field. P atients
a r e n e a r l y a l w a y s a w a r e o f p o s i t i v e s c o t o ma t a , b u t n e g a t i v e s c o t o ma t a ma y o n l y b e
d e t e c t e d d u r i n g a n o p h t h a l mo l o g i c e xa mi n a t i o n .
C.
A n a b s o l u t e s c o t o mi nad i c a t e s a c o mp l e t e l o s s o f v i s u a l p e r c e p t i o n , w h e r e a s a
r e l a t i v e s c o t o mi nav o l v e s d i mi n i s h e d b u t n o t a b s e n t l i g h t p e r c e p t i o n i n t h e a f f e c t e d
a r e a . Ac o l o r s c o t o mdae f i n e s d i mi n i s h e d o r l o s t c o l o r v i s i o n o n l y.
D.
T h e t e r m s c i n t i l l a t i n g s c o t o ma h a s c o me i n t o c o mmo n u s a g e b y b o t h p a t i e n t s a n d
c l i n i c i a n s , t y p i c a l l y i n t h e c o n t e xt o f a mi g r a i n e w i t h a u r a . T h i s i s s o me t h i n g o f a
mi s n o me r, b e c a u s e s c i n t i l l a t i o n s a n d s c o t o ma t a a r e d i s t i n c t v i s u a l p h e n o me n a . T h a
t h e y f r e q u e n t l y o c c u r t o g e t h e r i n a mi g r a i n e w i t h a u r a h a s l e d t o t h e o r i g i n o f t h i s
t e r m.
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5.8 - Scotoma
A. Etiology
E v e r y o n e h a s a p h y s i o l o g i c s c o t o ma a t t h e o p t i c d i s k , t h e s i t e w h e r e t h e o p t i c n e r v
e n t e r s t h e r e t i n a . D i s e a s e s t h a t a f f e c t t h e o p t i c n e r v e ma y c a u s e a n e n l a r g e me n t o f
t h i s s c o t o ma t o t h e p o i n t t h a t i t i n t e r f e r e s w i t h v i s i o n . T h i s ma n i f e s t s a s a c e n t r a l
s c o t o ma . D i s e a s e s t h a t a f f e c t t h e ma c u l a ma y a l s o p r e s e n t w i t h c e n t r a l s c o t o ma a s
p r o mi n e n t s y mp t o m. P e r i p h e r a l s c o t o ma t a r e s u l t f r o m d i s e a s e p r o c e s s e s t h a t a f f e c t
t h e r e t i n aT. a b l e 5 . 8 . l1i s t s t h e c o mmo n c a u s e s o f b o t h c e n t r a l a n d p e r i p h e r a l
s c o t o ma t a .
B. Epidemiology
T h e i n c i d e n c e o f s c o t o ma v a r i e s d e p e n d i n g u p o n t h e a s s o c i a t e d d i s e a s e s t a t e . T h e
s t r o n g e s t a s s o c i a t i o n i s w i t h o p t i c n e u r i t i s . S i xt y p e r c e n t o f p a t i e n t s w i t h s c o t o ma
d u e t o o p t i c n e u r i t i s w i l l e v e n t u a l l y d e v e l o p mu l t i p l e1) s. c l e r o s i s (
I I I . Evaluation
A. History
P a t i e n t s w i t h a c e n t r a l s c o t o ma r e p o r t a v i s u a l f i e l d d e f e c t a s a p r i ma r y c o mp l a i n t .
E l u c i d a t i n g t h e e xa c t t y p e o f s c o t o ma , a s o u t l i n e d i n t h e d e f i n i t i o n s i n t h e p r e c e d i n
t e xt , i s i mp o r t a n t i n a n a t o mi c a l l y l o c a l i zi n g t h e l e s i o n r e s p o n s i b l e f o r t h e s c o t o ma .
f o c u s e d h i s t o r y s h o u l d a s s e s s f o r o t h e r v i s u a l d i s t u r b a n c e s s u c h a s d i mi n i s h e d
v i s u a l a c u i t y o r d i mi n i s h e d c o l o r v i s i o n , a n d o c u l a r p a i n . N e u r o l o g i c s y mp t o ms ma y
b e f o u n d i n p a t i e n t s w i t h mu l t i p l e s c l e r o s i s . S y s t e mi c s y mp t o ms ma y i n d i c a t e a n
u n d e r l y i n g c o n n e c t i v e t i s s u e d i s e a s e o r v a s c u l i t i s . T h e p a s t me d i c a l h i s t o r y ma y
include known neurologic or connective tissue diseases or vasculitis. A history of
c u r r e n t a n d p a s t me d i c a t i o n s i s v e r y i mp o r t a n t . C e r t a i n me d i c a t i o n s s u c h a s
c h l o r o q u i n e / h y d r o xy c h l o r o q u i n e , i s o n i a zi d , e t h a mb u t o l , d i g i t a l i s g l y c o s i d e s , a n d
p o s s i b l y a mi o d a r o n e c a n b e d i r e c t l y t o xi c t o t h e r e t i n a o r 2)
o p. tIimmu
c n enr vee (
s u p p r e s s a n t me d i c a t i o n s c a n p r e d i s p o s e t o i n f e c t i o n s s u c h a s c y t o me g a l o v i r u s
r e t i n i t i s . A h u ma n i mmu n o d e f i c i e n c y v i r u s ( H I V ) i n f e c t i o n c a n a l s o p r e d i s p o s e t h e
patient to these infections. A history of risk factors for H IV infection should be
o b t a i n e d . A h i s t o r y o f i n g e s t i o n o r e xp o s u r e t o t o xi n s s u c h a s l e a d , me t h a n o l , o r
e t h y l e n e g l y c o l s h o u l d b e o b t a2)i n. eCde(r t a i n d i e t a r y p r a c t i c e s o r p o o r n u t r i t i o n
ma y l e a d t o t h i a mi n e o r v i t a1mi
2 nd eBf i c i e n c y.
1. N o o c u l a r p h y s i c a l e xa mi n a t i o n s h o u l d b e u n d e r t a k e n f o r a n y c o n d i t i o n w i t h o u t
f i r s t p e r f o r mi n g a t e s t o f v i s u a l a c u i t y. T h i s ma y b e d o n e w i t h a h a n d h e l d o r
w a l l - mo u n t e d S n e l l e n e y e c h a r t . O n c e v i s u a l a c u i t y h a s b e e n d o c u me n t e d , t h e
r e s t o f t h e e y e e xa mi n a t i o n ma y b e u n d e r t a k e n .
2. C o l o r v i s i o n s h o u l d b e t e s t e d w i t h p s e u d o i s o c h r o ma t i c p l a t e s ( I s h i h a r a p l a t e s
a r e mo s t c o mmo n l y u s e d ) . D i mi n i s h e d c o l o r v i s i o n i s c o mmo n i n o p t i c n e r v e
disease.
3. P u p i l l a r y t e s t i n g i s i mp o r t a n t , b e c a u s e t h e p r e s e n c e o r a b s e n c e o f a r e l a t i v e
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a f f e r e n t p u p i l l a r y d e f e c t i s h e l p f u l i n d i a g n o s i n g o r e xc l u d i n g u n i l a t e r a l o p t i c
neuritis. A relative afferent pupillary defect is also known as a Marcus Gunn
p u p i l . T h e t e s t i n g i s p e r f o r me d i n a d i ml y l i t r o o m w i t h t h e p a t i e n t ' s e y e s f i xa t e d
o n a d i s t a n t o b j e c t . T h e e xa mi n e r s h i n e s a b r i g h t l i g h t i n t o t h e a s y mp t o ma t i c e y
and should observe bilateral pupillary constriction due to the consensual light
r e f l e x. T h e e xa mi n e r t h e n s w i n g s t h e f l a s h l i g h t a c r o s s t h e n a s a l b r i d g e t o t h e
affected eye. If there is optic nerve dysfunction in the affected eye, the pupil
p a r a d o xi c a l l y d i l a t e s d u e t o t h e i n a d e q u a t e t r a n s mi s s i o n o f d i r e c t l i g h t
P. 9 1
s t i mu l a t i o n t o t h e b r a i n . I n p a t i e n t s w i t h s y s t e mi c d i s e a s e c a u s i n g b i l a t e r a l o p t i
n e u r i t i s , a n a f f e r e n t p u p i l l a r y d e f e c t ma y b e3) a. b s e n t (
TAB L E 5.8.1 Causes of Central and Peripheral

Scotom ata
Cause
Ocular
disease
Optic ne rv e /m acula
Re tina
P r i ma r y o p e n - a n g l e g l a u c o ma R e t i n a l
d e t a c h me n t
A g e - r e l a t e d ma c u l a r d e g e n e r a t i o n
Neurologic
disease
Optic neuritis (secondary to

mu l t i p l e s c l e r o s i s )
Optic neuritis (idiopathic)
Va s c u l a r
disease
T e mp o r a l a r t e r i t i s
Retinal
vasculitis
Retinal artery
occlusion
Retinal vein
occlusion
R h e u ma t o l o g i c S a r c o i d o s i s w i t h o c u l a r i n f i l t r a t i o n
disease
Endocrine
disease
T h y r o i d o p h t h a l mo p a t h y
Diabetic
retinopathy
Infectious
disease
Ocular syphilis
C y t o me g a l o v i r u s
retinitis
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Malignancy
O p t i c n e r v e g l i o ma
O p t i c n e r v e s h e a t h me n i n g i o ma
I n t r a c r a n i a l t u mo r w i t h o p t i c n e r v e
c o mp r e s s i o n
P a r a n e o p l a s t i c s y n d r o me s
Nutritional
deficiency
T h i a mi n e d e f i c i e n c y
Vi t a mi n 1B2 d e f i c i e n c y
T o xi n
e xp o s u r e
L e a d e xp o s u r e
Chloroquine
t o xi c i t y
M e t h a n o l e xp o s u r e
E t h y l e n e g l y c o l e xp o s u r e
E t h a mb u t o l t o xi c i t y
I s o n i a zi d t o xi c i t y
D i g i t a l i s t o xi c i t y
A mi o d a r o n e t o xi c i t y
N u t r i t i o n a l a mb l y o p i a ( d u e t o
chronic tobacco or alcohol use)
4. A f u n d u s c o p i c e xa mi n a t i o n i s b e s t p e r f o r me d a f t e r p u p i l l a r y d i l a t a t i o n . A p a l e o r
w h i t e o p t i c d i s k i n d i c a t e s o p t i c n e u r i t i s . R e t i n a l h e mo r r h a g e s o r e xu d a t e s o r
r e t i n a l v a s c u l a t u r e a b n o r ma l i t i e s ma y b e v i s u a l i ze d .
5. A f t e r t h e o c u l a r e xa mi n a t i o n i s c o mp l e t e , a g e n e r a l p h y s i c a l e xa mi n a t i o n
i n c l u d i n g a c o mp l e t e n e u r o l o g i c e xa mi n a t i o n s h o u l d b e u n d e r t a k e n .
6. Vi s u a l f i e l d a n a l y s i s i s c r i t i c a l i n t h e e v a l u a t i o n o f s c o t o ma . P r i ma r y c a r e
c l i n i c i a n s s e l d o m h a v e t h e o f f i c e e q u i p me n t t o p e r f o r m t h i s t e s t i n g p r o p e r l y.
Vi s u a l f i e l d t e s t i n g b y c o n f r o n t a t i o n o r b y t a n g e n t s c r e e n e xa mi n a t i o n i s
inadequate; patients should be referred to an eye specialist for testing with the
a p p r o p r i a t e e q u i p me n t .
C. Testing
T h e r e i s n o r o l e f o r u n t a r g e t e d d i a g n o s t i c s t u d i e s i n t h e e v a l u a t i o n o f s c o t o ma . T h e
170 / 652
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selection of appropriate laboratory and radiographic studies should arise from the
h i s t o r y a n d p h y s i c a l e xa mi n a t i o n f i n d i n g s , i n c l u d i n g f o r ma l v i s u a l f i e l d t e s t i n g . B l o o
t e s t s s u c h a s a n t i n u c l e a r a n t i b o d i e s , r h e u ma t o i d f a c t o r, o r l e v e l s o f a n g i o t e n s i n c o n v e r t i n g e n zy me s h o u l d b e o r d e r e d i f t h e r e i s a c l i n i c a l s u s p i c i o n f o r a v a s c u l i t i s
c o n n e c t i v e t i s s u e d i s e a s e . T e mp o r a l a r t e r i t i s r e q u i r e s u r g e n t d i a g n o s i s
P. 9 2
a n d a n e r y t h r o c y t e s e d i me n t a t i o n r a t e s h o u l d b e o r d e r e d i mme d i a t e l y w h e n t h i s i s
s u s p e c t e d . S e r u m v i t a mi
1 2n aBn d r e d b l o o d c e l l f o l a t e l e v e l s a r e i n d i c a t e d i n
p a t i e n t s w i t h b i l a t e r a l c e n t r a l s c o t o ma . S e r o l o g i c t e s t i n g f o r s y p h i l i s o r H I V i s
indicated in patients with risk factors for these infections. T hyroid function tests ar
a p p r o p r i a t e i n p a t i e n t s w i t h o c u l a r o r s y s t e mi c p h y s i c a l e xa mi n a t i o n f i n d i n g s t h a t
s u g g e s t t h y r o t o xi c o s i s . S e r u m l e a d l e v e l s s h o u l d b e me a s u r e d i n p a t i e n t s w i t h a
h i s t o r y o f o c c u p a t i o n a l o r d o me s t i c l e a d e xp o s u r e . I n p a t i e n t s w i t h s u s p e c t e d
mu l t i p l e s c l e r o s i s , a l u mb a r p u n c t u r e s h o u l d b e c o n s i d e r e d t o o b t a i n c e r e b r o s p i n a l
f l u i d f o r a n a l y s i s o f my e l i n b a s i c p r o t e i n a n d o l i g o c l o n a l b a n d s . M a g n e t i c r e s o n a n c e
i ma g i n g ( M R I ) w i t h g a d o l i n i u m i s b o t h s e n s i t i v e a n d s p e c i f i c f o r o p t i c n e u r i t i s . A n
M R I ma y a l s o b e u s e f u l i n e s t a b l i s h i n g a d i a g n o s i s o f mu l t i p l e s c l e r o s i s o r i n
v i s u a l i zi n g i n t r a c r a n i a l t u mo r s . S o me c e n t r a l n e r v o u s s y s t e m t u mo r s , s u c h a s o p t i c
n e r v e s h e a t h me n i n g i o ma , a r e a l s o w e l l v i s u a l i ze d b y c o mp u t e d t o mo g r a p h y w i t h
contrast. Again, tests should be deferred unless there is a significant pretest clinic
suspicion for these conditions, based on the patient's history and physical
e xa mi n a t i o n f i n d i n g s .
I V. Diagnosis
T h e a p p r o a c h t o s c o t o ma b e g i n s w i t h c l a s s i f y i n g c e n t r a l v e r s u s p e r i p h e r a l
s c o t o ma t a . C e n t r a l s c o t o ma t a a r e a t t r i b u t a b l e t o o p t i c n e r v e o r ma c u l a r d i s e a s e .
P e r i p h e r a l s c o t o ma t a a r e c a u s e d b y r e t i n a l d i s e a s e .
S c o t o ma p r e s e n t s w i t h a v i s u a l f i e l d d e f e c t a s t h e p r i ma r y c o mp l a i n t . P a t i e n t s w i t h
r e t i n a l d i s e a s e s a n d g l a u c o ma h a v e f e w, i f a n y, s y mp t o ms , e xc e p t f o r v i s i o n l o s s .
P a t i e n t s w i t h mu l t i p l e s c l e r o s i s ma y a l s o p r e s e n t w i t h s y mp t o ms s u c h a s g a i t
a b n o r ma l i t i e s , s p e e c h d i f f i c u l t i e s , d i f f i c u l t i e s w i t h b o w e l a n d / o r b l a d d e r c o n t r o l ,
w e a k n e s s , o r p a r e s t h e s i a s . J a w c l a u d i c a t i o n a n d h e a d a c h e a r e s e e n i n t e mp o r a l
a r t e r i t i s . S a r c o i d o s i s ma y c a u s e f e v e r, a r t h r a l g i a s , l y mp h a d e n o p a t h y, o r s k i n l e s i o n
References
1 . R i o r d a n - E v a P. E y e . I n : T i e r n e y L M , M c P h e e S J , P a p a d a k C
i surMr A
ent
, eds.
medi c al di agnos i s and t r eat, ment
4 4 t h E d . N e w Yo r k : L a n g e M e d i c a l B o o k s ,
2005:166.
2 . Z a f a r A , S e r g o t t RTox
C . i c / nut r i t i onal opt i c neur opat
.
hy
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3 . I n g E .N eur o- opht hal mi c ex ami nat

. i on
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6.1 - Halitosis
> T a b l e o f C o n t e n t s > 6 - E a r, N o s e , a n d T h r o a t P r o b l e ms > 6 . 1 - H a l i t o s i s
6.1
Halitosis
Mark D. Andre w s
I . Background
H a l i t o s i s ( f e t o r o r i s ) r e f e r s t o u n p l e a s a n t o r o f f e n s i v e o d o r s e mi t t e d i n t h e e xp i r e d
a i r. I t ma y me r e l y b e a s o c i a l h a n d i c a p r e l a t e d t o p o o r o r a l h y g i e n e o r o r a l c a v i t y
d i s e a s e . R a r e l y, i t c a n r e p r e s e n t a ma r k e r o f mo r e s e r i o u s s y s t e mi c i l l n e s s r e q u i r i n
d i a g n o s i s a n d t r e a t me1)n.t T( h e G r e e k s a n d R o ma n s w r o t e a b o u t b a d b r e a t h a n d i t
w a s d i s c u s s e d i n t h e J e w i s h T a l mu d . T o d a y, o r a l ma l o d o r h a s b e e n s t i g ma t i ze d ,
g i v i n g r i s e t o a c o mme r c i a l ma r k e t f o r mo u t h f r e s h e n e r s e xc e e d i n g $ 9 0 0 mi l l i o n
a n n u a l l y2)( . D e s p i t e t h i s p u b l i c i t y, p a t i e n t s o n l y s e e k h e l p r a r e l y a n d a r e g e n e r a l l y
u n a w a r e o f t h e p r o b l e m, a l t h o u g h i t c a n s e v e r e l y a f f e c t p e r s o n a l r e l a t i o n s .
A. Etiology
P h y s i o l o g i c h a l i t o s i s , s u c h a s w i t h e a t i n g o n i o n s a n d g a r l i c o r w i t h mo r n i n g b r e a t h ,
t e mp o r a r y. T h e s e o d o r s a r e r e v e r s i b l e , t r a n s i e n t , a n d r e s p o n s i v e t o t r a d i t i o n a l o r a l
h y g i e n e p r a c t i c e3)s . ( I n c o n t r a s t , p a t h o l o g i c h a l i t o s i s i s mo r e i n t e n s e a n d n o t e a s i l y
r e v e r s i b l e . I t ma y a r i s e f r o m s i mi l a r me c h a n i s ms b u t r e s u l t s mo r e f r e q u e n t l y f r o m
r e g i o n a l o r s y s t e mi c p a t h o l o g y, l e a d i n g t o p e r s i s t e n t o d o r s t h a t u l t i ma t e l y r e q u i r e
t r e a t me n t1,2,3)
(
.
P e r s i s t e n t h a l i t o s i s ( u s u a l l y n o t e d b y i n d i v i d u a l s a r o u n d t h e p a t i e n t ) i s mo r e s e v e r e
t h a n p h y s i o l o g i c h a l i t o s i s . T h e i mp o r t a n t t a s k i n i t i a l l y i s t o c a t e g o r i ze t h e h a l i t o s i s
e i t h e r l o c a l i ze d t o t h e o r a l c a v i t y o r o r i g i n a t i n g s y s t e mi c a l l y. I n 8 0 % t o 9 0 % o f
p a t i e n t s , h a l i t o s i s i s d u e t o b a c t e r i a l a c t i v i t y f r o m d i s o r d e r s o f t h e o r a l c a v i t y, a n d
t h e r e ma i n d e r o f p a t i e n t s , t h e c o n d i t i o n i s a t t r i b u t e d t o n o n o r a l o r s y s t e mi c s o u r c e s
Vo l a t i l e s u l f u r c o mp o u n d s a r i s i n g t h r o u g h t h e mi c r o b i a l d e g r a d a t i o n o f a mi n o a c i d s
a r e t h e p r e s u me d s o u r c e o f mo s t o f f e n d i n g o d o r s . B a d b r e a t h ma y o r i g i n a t e f r o m t h
following areas: oral cavity85% to 88%, nasal passages8%, tonsils3%, and
o t h e r s i t e s 2 % t o 4)
3%
. (I n a d d i t i o n , t h e c a u s e s o f h a l i t o s i s c a n b e s u b c a t e g o r i ze d
into pathologic and nonpathologic types.
1. N o n p a t h o l o g i c c a u s e s
a. M o r n i n g b r e a t h i s d u e t o d e c r e a s e d s a l i v a r y f l o w o v e r n i g h t a s s o c i a t e d w i t h
i n c r e a s e d f l u i d p H , e l e v a t e d g r a m- n e g a t i v e b a c t e r i a l g r o w t h , a n d v o l a t i l e
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6.1 - Halitosis
s u l f u r c o mp o u n d s p r o d u c 3)
t i o. n (
b. X e r o s t o mi a o f a n y c a u s e ( e . g . , s l e e p , d i s e a s e s , me d i c a t i o n , mo u t h
breathing, and especially age-related declines in salivary quantity and
quality) can contribute to halitosis.
c. M i s s e d me a l s c a n l e a d t o h a l i t o s i s s e c o n d a r y t o d e c r e a s e d s a l i v a r y f l o w
a n d t h e a b s e n c e o f t h e me c h a n i c a l a c t i o n o f t h e f o o d o n t h e t o n g u e s u r f a c e
to wear down filiform papillae.
d. T o b a c c o o r a l c o h o l c a n b e a c o n t r i b u t i n g c a u s e o f h a l i t o s i s .
e. M e t a b o l i t e s f r o m i n g e s t e d f o o d ( o n i o n s , g a r l i c , a l c o h o l , p a s t r a mi , a n d o t h e r
me a t s ) a r e a b s o r b e d i n t o t h e c i r c u l a t i o n a n d t h e n e xc r e t e d t h r o u g h t h e
lungs.
f. M e d i c a t i o n s s u c h a s a n t i c h o l i n e r g i c d r u g s c a n c a u s e xe r o s t o mi a , e s p e c i a l l y
i n t h e e l d e r l y. O t h e r a g e n t s i n c l u d e a mp h e t a mi n e s , p s y c h i a t r i c d r u g s ,
a n t i h i s t a mi n e s , d e c o n g e s t a n t s , n a r c o t i c s , a n t i h y p e r t e n s i v e s , a n t i p a r k i n s o n i a n a g e n t s , c h e mo t h e r a p y, a n d r a d i a t i o n t h e r a p y.
2. P a t h o l o g i c c a u s e s
a. L o c a l o r o p h a r y nCxh r o n i c p e r i o d o n t a l d i s e a s e a n d g i n g i v i t i s a r e c o mmo n
s o u r c e s t h r o u g h t h e p r o mo t i o n o f b a c t e r i a l o v e r g r o w t h . I n t h e i r a b s e n c e , t h e
mo s t l i k e l y o r a l s o u r c e i s t h e p o s t e r i o r d o r s u m o f t h e t o n g u e w i t h p o s t e r i o r
nasal drainage being a frequent contributing factor to local bacterial
P. 9 6
o v e r g r o w t h . S t o ma t i t i s a n d g l o s s i t i s c a u s e d b y s y s t e mi c d i s e a s e ,
me d i c a t i o n , o r v i t a mi n d e f i c i e n c i e s c a n l e a d t o t r a p p e d f o o d p a r t i c l e s a n d
d e s q u a ma t e d t i s s u e . A n i mp r o p e r l y c l e a n e d p r o s t h e t i c a p p l i a n c e c a n b e a
l o c a l c o n t r i b u t o r, a s c a n p r i ma r y p h a r y n g e a l c a n c e r. O t h e r c o n d i t i o n s
associated with parotid dysfunction (e.g., viral and bacterial infections,
c a l c u l i , d r u g r e a c t i o n s , s y s t e mi c c o n d i t i o n s i n c l u d i n g S j g r e n ' s g r e n ' s
s y n d r o me ) a r e a l s o i mp o r t a n t . T o n s i l s i n f r e q u e n t l y c a u s e h a l i t o s i s ( f o u n d i n
7 % o f t h e p o p u l a t i o n ) , e v e n w i t h c r y p t t o n s i l l i t i s . T h e s e ma y a l a r m p a t i e n t s
b u t a r e u s u a l l y a s y mp t o ma t i c a n d n o t a s s o c i a t e d w i t h a n y p a t h o l o g y.
b. G a s t r o i n t e s t i n a l t r G
a cats t r o i n t e s t i n a l s o u r c e s o c c a s i o n a l l y c o n t r i b u t e t o
i n t e r mi t t e n t b a d b r e a t h . P o t e n t i a l s o u r c e s i n c l u d e g a s t r o e s o p h a g e a l r e f l u x
d i s e a s e , g a s t r o i n t e s t i n a l b l e e d i n g , g a s t r i c c a n c e r, ma l a b s o r p t i o n
s y n d r o me s , a n d e n t e r i c i n f e c t i o n s .
c. R e s p i r a t o r y t r aCc ht r o n i c s i n u s i t i s , n a s a l f o r e i g n b o d i e s o r t u mo r s ,
p o s t n a s a l d r i p , b r o n c h i t i s , p n e u mo n i a , b r o n c h i e c t a s i s , t u b e r c u l o s i s , a n d
ma l i g n a n c i e s ma y c a u s e h a l i t o s i s .
d. P s y c h i a t r i H
c a l i t o p h o b i a i s i ma g i n a r y h a l i t o s i s a s s o c i a t e d w i t h p s y c h i a t r i c
d i s o r d e r s3)( .
e. S y s t e mi c s o u r c e s i n c l u d e d i a b e t i c k e t o a c i d o s i s ( s w e e t , f r u i t y, a c e t o n e
b r e a t h ) , r e n a l f a i l u r e ( a mmo n i a o r f i s h y o d o r ) , h e p a t i c f a i l u r e ( f e t o r
h e p a t i c u s a s w e e t a mi n e o d o r ) , h i g h f e v e r w i t h d e h y d r a t i o n , a n d v i t a mi n
o r mi n e r a l d e f i c i e n c i e s l e a d i n g t o a d r y mo u t h .
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B. Epidemiology
T h e p r e v a l e n c e o f h a l i t o s i s i s n o t k n o w n , b u t ma n y i n d i v i d u a l s w o r r y a b o u t i t . I n o n
s t u d y, 2 0 % o f a d u l t s w o r r i e d a b o u t b a d b r e a t h , w h e n l i t t l e w a5)
s . me a s u r e d (
A p p r o xi ma t e l y 2 5 % o f i n d i v i d u a l s s e e k i n g h e l p f o r h a l i t o s i s ma y b e h a l i t o p h o b i c o r
s u f f e r i n g f r o m p s e u d o h a l i t4)
o s. i s (
I I I . Evaluation
A. History
Focus on the characteristics of the bad breath, although the patient is often unable
t o d e s c r i b e h i s o r h e r c o n d i t i o n a c c u r a t e l y b e c a u s e o f o l f a c t o r y d e s e n s i t i za t i o n . I s
t h e o d o r t r a n s i e n t o r c o n s t a n t ? C o n s t a n t o d o r s u g g e s t s c h r o n i c s y s t e mi c d i s e a s e o r
s e r i o u s d i s o r d e r s o f t h e o r a l c a v i t y. W h a t a r e t h e p r e c i p i t a t i n g , a g g r a v a t i n g , o r
r e l i e v i n g f a c t o r s ? A s k a b o u t s mo k i n g h a b i t s , d i e t , d r u g s , d e n t u r e s , mo u t h b r e a t h i n g
s n o r i n g , h a y f e v e r, a n d n a s a l o b s t r u c t i o n . B e c a u s e t h e t h e r a p y f o r h a l i t o s i s o f o r a l
o r i g i n , b e y o n d t h e l i mi t a t i o n o f a g g r a v a t i n g f a c t o r s , i s p r o p e r o r a l h y g i e n e a n d
vigorous tongue brushing, an evaluation of the patient's tooth brushing and flossing
r e g i me n i s i mp e r a t i v e .
T h i s s h o u l d b e u n d e r t a k e n w i t h a n e mp h a s i s o n t h e o r a l c a v i t y, p a r t i c u l a r l y l o o k i n g
f o r u l c e r a t i o n , d r y n e s s , t r a u ma , p o s t n a s a l d r a i n a g e , i n f e c t i o n s , i n f l a me d c r y p t i c
t o n s i l s , o r n e o p l a s ms . T e c h n i q u e s f o r l o c a l i zi n g t h e o d o r s o u r c e ( s y s t e mi c v s . o r a l
cavity) include:
1. S e a l t h e l i p s a n d b l o w a i r t h r o u g h t h e n o s e . I f a f e t i d o d o r i s n o t e d , t h i s i s
s u g g e s t i v e o f a s y s t e mi c s o u r c e . I f a n o d o r i s o n l y n o t e d f r o m t h e n o s e , t h e n a
n a s a l s o u r c e i s l i k e l y.
2. P i n c h t h e n o s e w i t h t h e l i p s c l o s e d . H o l d r e s p i r a t i o n a n d e xh a l e g e n t l y t h r o u g h
t h e mo u t h . O d o r s d e t e c t e d i n t h i s f a s h i o n g e n e r a l l y a r e l o c a l i n o r i g i n .
3. I f a s i mi l a r o d o r i s n o t e d f r o m b o t h s o u r c e s t h e n a s y s t e mi c s o u r c e ma y b e
suspected.
C. Testing
F o r mo s t p a t i e n t s , c l i n i c a l l a b o r a t o r y t e s t i n g a n d d i a g n o s t i c i ma g i n g a r e
u n n e c e s s a r y, a n d s h o u l d o n l y b e p u r s u e d o n t h e b a s i s o f s p e c i f i c f i n d i n g s i n d i c a t e d
b y t h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n . T h e S c h i r me r ' s t e s t ma y b e u s e f u l i n
i d e n t i f y i n g xe r o p h t h a l mi a a n d a s s o c i a t e d xe r o s t o mi a s e e n w i t h S j g r e n ' s s y n d r o me
a n d s o me o t h e r r h e u ma t o l o g i c c o n dCi thi oanpst e(r 1 2). .1 I f i n d i c a t e d , r a d i o l o g i c
s t u d i e s a n d i ma g i n g p r o c e d u r e s o f t h e s i n u s e s , t h o r a x, a n d a b d o me n ma y b e u s e d t
i d e n t i f y i n f e c t i o u s p r o c e s s e s a n d n e o p l a s ms .
I V. Diagnosis
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T h e k e y i s a t h o r o u g h h i s t o r y a n d f o c u s e d p h y s i c a l e xa mi n a t i o n t o d i s t i n g u i s h l o c a l
o r a l f r o m s y s t e mi c p r o c e s s e ss e( scet ieo nI I . A ). . B e c a u s e 8 0 % t o 9 0 % o f a l l
ma l o d o r o u s c o n d i t i o n s c a n b e t r a c e d t o o r a l c a u s e s , s i mp l e
P. 9 7
ma n e u v e r s c a n b e d i a g n o s t i c a l l y h e l p f u l i n e xc l u d i n g t h e l i k e l i h o o d o f mo r e d i s t a n t
c o mp l e x s y s t e mi c s o u r c e s .
I n a d d i t i o n t o f e t i d o d o r, t h e r e ma y b e u l c e r a t i o n , d r y n e s s , t r a u ma , p o s t n a s a l
d r a i n a g e , i n f e c t i o n s , i n f l a me d c r y p t i c t o n s i l s , o r n e o p l a s ms .
References
1 . R e p l o g l e W M , K e e b e D K . H a l i tAom
s i sF.am P hy s i c i an
1996;53:12151223.
2 . S p i e l ma n A I , B i v o n a P, R e f B R . H a l i t o s i s : a c o mmo n o r aNl Y
prS
o bt at
l eem.
D ent J1 9 9 6 ; 6 2 : 3 6 4 2 .
3 . R o s e n b e r g M . T h e s c i e n c e o f b a d bSr ce ia tAhm
. 2002;286(4):5865.
4. B en- Aryeh H , H orowitz G , N ir D , et al. H alitosis: an interdisciplinary approach.
A m J O t ol ar y ngol
1 9 9 8 ; 1 9 : 8 11 .
5. K naan T, C ohen D , R osenberg M . P redicting bad breath in the nonc o mp l a i n i n g p o p u l a t iOo rnal
. D i s2 0 0 5 ; 11 : 1 0 5 ( a b s t r a c t ) .
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6.2 - Hearing Loss
> T a b l e o f C o n t e n t s > 6 - E a r, N o s e , a n d T h r o a t P r o b l e ms > 6 . 2 - H e a r i n g L o s s
6.2
Hearing Loss
M a r k P. K n u d s o n
I . Background
H e a r i n g i s t h e c o mp l e x s e t o f e v e n t s i n w h i c h s o u n d w a v e s a r e c o n v e r t e d t o
e l e c t r i c a l i mp u l s e s , t r a n s mi t t e d t o t h e b r a i n , a n d i n t e r p r e t e d a s s o u n d . A n y
interruption in this set of events results in hearing loss.
A. Etiology
C a u s e s o f h e a r i n g l o s s c a n b e d i v i d e d i n t o t h r e e c1,2)
a t e :g oc roi ne ds u(c t i v e
h e a r i n g l o s s ( C H L ) , s e n s o r i n e u r a l h e a r i n g l o s s ( S N H L ) , a n d mi xe d h e a r i n g l o s s
( M H L ) . C H L r e s u l t s f r o m t h e o b s t r u c t i o n o f t h e c a n a l , t h e d e r a n g e me n t o f t h e
t y mp a n i c me mb r a n e ( T M ) , o r t h e i n c r e a s e d i mp e d a n c e i n t h e mi d d l e e a r ( e f f u s i o n ) o
o s s i c l e s ( o t o s c l e r o s i s ) . S N H L ma y r e f l e c t d e f e c t s i n t h e i n n e r e a r o r c o c h l e a , t h e
e i g h t h c r a n i a l n e r v e , o r t h e c e n t r a l n e r v o u s s y s t e m. M H L i n v o l v e s a c o n d u c t i o n
i mp a i r me n t c o mp l i c a t i n g a s e n s o r i n e u r a l i mp a i r me n t .
B. Epidemiology
A p p r o xi ma t e l y 2 8 mi l l i o n A me r i c a n s h a v e d o c u me n t e d h e a r i n g l o s s . A t b i r t h , 1 i n
1 , 0 0 0 n e w b o r n s h a v e p r o f o u n d h e a r i n g l o s s , w i t h a n e q u a l n u mb e r h a v i n g mo d e r a t e
h e a r i n g l o s s a f f e c t i n g s p e3)e .c hA mo
( ng individuals older than 65 years of age, 7%
t o 8 % r e p o r t h e a r i n g l o s s ; n e v e r t h e l e s s , mo r e t h a n t w i c e t h a t n u mb e r h a v e e v i d e n c e
o f h e a r i n g l o s s i f s c r e e4)
n e. dA (s ma n y a s 5 0 % o f A me r i c a n s h a v e e v i d e n c e o f
h e a r i n g l o s s b y t h e t i me t h e y r e a c h 7 5 y e a r s o f a g e . S u b g r o u p s a t h i g h e r r i s k o f
h e a r i n g l o s s i n c l u d e t h o s e i n d i v i d u a l s i n a v a r i e t y o f o c c u p a t i o n s ( mi l i t a r y v e t e r a n s
f i r e f i g h t e r s , f a c t o r y w o r k e r s ) , a n d i n ma n y r e c r e a t i o n a l a c t i v i t i e s ( l o u d mu s i c , t a r g e
s h o o t i n g1,2)
)( .
I I I . Evaluation
A. History
A l t h o u g h p a t i e n t s r a r e l y p r e s e n t w i t h a c o mp l a i n t o f d e c r e a s e d h e a r i n g , mo s t h a v e
no specific hearing concern. Instead they present with depression, confusion, socia
i s o l a t i o n , o r p o o r j o b p e r f o r ma n c e , w h i c h ma y b e c a u s e d o r c o mp l i c a t e d b y h e a r i n g
i mp a i r me n t . F a mi l y me mb e r s n o t e a b n o r ma l , s l o w, o r o v e r l y l o u d a n s w e r s , a s u d d e n
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t e n d e n c y t o mo n o p o l i ze o r d i s r u p t c o n v e r s a t i o n , o r t o t i l t t h e h e a d i n c o n v e r s a t i o n .
C H L i s o f t e n o f s u d d e n o n s e t b u t o f a mi l d d e g r e e . C o mp l e t e o c c l u s i o n o r r a p i d
c o l l e c t i o n o f f l u i d i n mi d d l e e a r c a u s e s a n a b r u p t c h a n g e i n h e a r i n g . S N H L c a n b e
a b r u p t a n d s e v e r e ( s t r o k e , i d i o p a t h i c , t r a u ma ) , o r g r a d u a l ( M n i r e ' s s y n d r o me ,
a c o u s t i c n e u r o ma , h y p o t h y r o i d i s m) . C H L o f t e n a f f e c t s t h e q u a l i t y o f h e a r i n g f i r s t .
D e s c r i b e d a s mu f f l e d l i k e a h e a d i n a d r u m, t h e p a t i e n t ma y l o s e h i g h f r e q u e n c y
a n d v o i c e d i s c r i mi n a t i o n ; h o w e v e r,
P. 9 8
they are still capable of detecting subtle sounds. S N H L , when not associated with
t i n n i t u s , c a n h a v e g o o d q u a l i t y b u t d i mi n i s h e d h e a r i n g u s u a l l y mo r e p r o f o u n d t h a n
CHL.
1. A s i mp l e h e a r i n g c h a l l e n g e ma y c o n f i r m h e a r i n g l o s s o r d e t e c t s i g n i f i c a n t
h e a r i n g a s y mme t r y. A s k t h e p a t i e n t t o c o v e r o n e e a r a n d t r y t o d e t e c t s o f t
sounds such as the tick of a watch, the scratching of two fingers rubbed
t o g e t h e r, o r a s o f t l y w h i s p e r e d v o i c e . I n s p e c t t h e c a n a l a n d T M t o r u l e o u t t h e
o b v i o u s c a u s e s o f C H L . C e r u me n i mp a c t i o n i s a r e ma r k a b l y c o mmo n a n d e a s i l y
c o r r e c t e d c a u s e o f h e a r i n g l o s s . P n e u ma t o s c o p y t o c h e c k f o r t h e n o r ma l
mo v e me n t o f t h e T M h e l p s r u l e o u t p e r f o r a t i o n , a t e l e c t a s i s , e u s t a c h i a n t u b e
d y s f u n c t i o n , s t i f f e n e d T M , o s s i c u l a r d i s r u p t i o n , a n d mi d d l e e a r e f f u s i o n .
2. T h e We b e r ' s t e s t i s c o mmo n l y e mp l o y e d w i t h a v i b r a t i n g t u n i n g f o r k p l a c e d o n
the top of the head. T he patient is asked to describe the sound heard and
perceives the sound to be louder in the affected ear in C H L, because the
background noise is absent on that side. T he sound in the unaffected ear is
perceived as louder in S N H L . T he R inne's test is conducted with the vibrating
t u n i n g f o r k p l a c e d o n t h e ma s t o i d , a n d t h e p a t i e n t d e t e c t s b o n e c o n d u c t i o n
( B C ) . T h e t u n i n g f o r k i s r e mo v e d w h e n t h e p a t i e n t c a n n o l o n g e r h e a r t h e
s o u n d . T h e n t h e t u n i n g f o r k i s h e l d n e xt t o t h e e a r t o t e s t f o r a i r c o n d u c t i o n
( A C ) . I n a n i n d i v i d u a l w i t h n o r ma l h e a r i n g , A C i s s i g n i f i c a n t l y b e t t e r t h a n B C .
C H L reduces AC and has little effect on B C.
C. Testing
1. L a b o r a t o r y t e s t i A
n ng a u d i o g r a m p e r f o r me d a t s e v e r a l f r e q u e n c y r e s p o n s e s
ma y d e t e c t i n d i v i d u a l s w h o a r e a t r i s k o f h e a r i n g l o s s . A l t h o u g h t h e s e n s i t i v i t y i
g o o d ( 9 3 % 9 5 % ) , t h e p o o r s p e c i f i c i t y ( 6 0 % 7 4 % ) c a n r e s u l t i n ma n y f a l s e p o s i t i v e f i n d i n g3)s . (A u d i o g r a p h y ma y b e p e r f o r me d t o d e t e c t p u r e t o n e l o s s o r
f o r s p e e c h d e t e c t i o n . P u r e t o n e t e s t i n g d o c u me n t s t h e n u mb e r o f d e c i b e l s h e a r d
a t a g i v e n f r e q u e n c y. U n f o r t u n a t e l y, i t d e s c r i b e s n o t h i n g a b o u t t h e a b i l i t y t o
d i s c r i mi n a t e l a n g u a g e . O n t h e o t h e r h a n d , s p e e c h d e t e c t i o n e s t i ma t e s t h e
i mp a i r me n t o f a c t u a l l a n g u a g e f u n c t i o n b e t t e r, b u t r e q u i r e s a mo r e c o o p e r a t i v e
and attentive patient.
A u d i t o r y - e v o k e d r e s p o n s e d e t e c t s t h e e l e c t r o e n c e p h a l o g r a p h i c s t i mu l a t i o n
caused by repetitive sounds and can be useful in the obtunded, uncooperative,
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or very young patient.
2. I m a g i n gC o mp u t e d t o mo g r a p h y ( C T ) s c a n ma y b e u s e d i n t h e s e t t i n g o f
t r a u ma t i c l o s s o f h e a r i n g , a s i t i s f a s t , l e s s e xp e n s i v e t h a n ma g n e t i c r e s o n a n c e
i ma g i n g ( M R I ) , a n d i s a b l e t o d e t e c t a c u t e b l e e d i n g a n d a b n o r ma l i t i e s w i t h i n t h e
p e t r o u s r i d g e w h e r e f r a c t u r e s c a n a f f e c t 4)
h e. aAr i nCgT (s c a n i s a l s o u s e f u l i n
d e t e c t i n g C H L c a u s e d b y t u mo r s , mi d d l e e a r a n o ma l i e s , my r i n g o s c l e r o s i s , a n d
c h o l e s t e a t o ma . A n M R I i s u s e d i n p a t i e n t s w i t h S N H L . A n M R I w i t h g a d o l i n i u m
i s s u p e r i o r t o a C T s c a n i n i d e n t i f y i n g mu l t i p l e s c l e r o s i s o r v a s c u l a r i n f a r c t s . I n
a d d i t i o n , a c o u s t i c n e u r o ma s a n d l a b y r i n t h d i s o r d e r s , o f t e n t o o s ma l l t o b e s e e n
w i t h a C T s c a n , ma y b e d e t e c t e d w i t h a4)n . M R I (
D. Genetics
Since the first hearing related gene was isolated in 1992, several hundred genetic
l o c i t h a t c o n t r i b u t e t o h e a r i n g l o s s h a v e b e e n1)i s. oTl ahti es dg e( n e t i c h e a r i n g l o s s
c a n b e s y n d r o mi c ( 3 0 % ) o r n o n s y n d r o mi c . M o r e t h a n 4 0 0 s y n d r o me s h a v e b e e n
i d e n t i f i e d , mo s t r e s u l t i n g i n C H L o r M H L t h a t o c c u r s e a r l y i n l i f e . T h e g e n e s t h a t
c a u s e n o n s y n d r o mi c h e a r i n g l o s s a r e u s u a l l y i n h e r i t e d i n a n a u t o s o ma l r e c e s s i v e
ma n n e r, a n d t h e ma j o r i t y r e s u l t i n S N H L d u e t o c o c h l e a r d e f e c t s . A l t h o u g h c o mmo n
f o r ms o f a d u l t h e a r i n g l o s s s u c h a s o t o s c l e r o s i s a n d M n i r e ' s d i s e a s e a r e n o t
l i n k e d t o a s p e c i f i c g e n e d e f e c t , t h e y f o l l o w a g e n e t i c p a t t e r n o3)
f .i n h e r i t a n c e (
I V. Diagnosis
C o mmo n c a u s e s o f S N H L i n c l u d e a c o u s t i c n e u r o ma , mu l t i p l e s c l e r o s i s ,
h y p o t h y r o i d i s m, v e r t e b r o b a s i l a r i n s u f f i c i e n c y, o r s t r o k e , M n i r e ' s s y n d r o me , d r u g
t o xi c i t y, a n d i d i o p a t h i c h e a r i n g l o s s . C H L i s mo s t f r e q u e n t l y c a u s e d b y c e r u me n
i mp a c t i o n , p e r f o r a t i o n o f t h e T M , mi d d l e e a r e f f u s i o n , a t e l e c t a s i s , a n d
P. 9 9
o t o s c l e r o s i s . I n a d d i t i o n , a v a r i e t y o f t u mo r s ( e . g . , s q u a mo u s c e l l c a n c e r, e xo s t o s e s
o r c h o l e s t e a t o ma ) c a n c a u s e C H L . M H L ma y b e s e c o n d a r y t o p r e s b y c u s i s ,
me d i c a t i o n s , a n d n o i s e - i n d u c e d h e a r i n g l o s s .
Adult patients with C H L typically present with sudden onset of partial (often high
f r e q u e n c y ) h e a r i n g l o s s , a t t i me s a s s o c i a t e d w i t h e xt e r n a l e a r s y mp t o ms . P a t i e n t s
w i t h S N H L p r e s e n t mo r e i n s i d i o u s l y ; h o w e v e r, t h e y o f t e n p r o g r e s s t o mo r e c o mp l e t e
and profound hearing loss. As a result, screening is critical to detect and treat thes
p a t i e n t s w h e n p o s s i b5)l e. (
References
1 . I s a a c s o n J E . D i f f e r e n t i a l d i a g n o s i s a n d t r e a t me n t o f h eAamr i nFgam
loss.
P hy s i c i an2 0 0 3 ; 6 8 : 11 2 5 11 3 2 .
2 . We b e r P, K l e i n A . H e a r i n g M
l oed
s s . C l i n N or t h A1m9 9 9 ; 8 3 : 1 2 5 1 3 7 .
179 / 652
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6.2 - Hearing Loss
3 . A g g a r w a l R , S a e e d S R . T h e g e n e t i c s o f h e a rH
i nos
g pl o sMsed
. 2005;66:32
36.
4 . M a g g i S , M i n i c u c i N , M a r t i n i A , e t a l . P r e v a l e n c e r a t e s o f h e a r i n g i mp a i r me n t
a n d c o - mo r b i d c o n d i t i o n s i n o l d e r p e o p l e : t h e Ve n Je t A
o mS tG
u er
d y.i at r S oc
1998;46:10691074.
5 . S h a p i r o Y B , M a c L e a n C H , S h e k e l l e P G . S c r e e n i n g a n d ma n a g e me n t o f a d u l t
h e a r i n g l o s s i n p r i ma r y c a r e : s c i e n t i f i cJ rAeM
v iAe2w.0 0 3 ; 2 8 9 ( 1 5 ) : 1 9 7 6 1 9 8 5 .
180 / 652
tmdmss
6.3 - Hoarseness
> T a b l e o f C o n t e n t s > 6 - E a r, N o s e , a n d T h r o a t P r o b l e ms > 6 . 3 - H o a r s e n e s s
6.3
Hoarseness
L. Gail Curtis
I . Background
H o a r s e n e s s i s a c h a n g e i n n o r ma l v o i c e q u a l i t y a n d i s c a u s e d b y a n a b n o r ma l f l o w
o f a i r p a s t i n c o mp l e t e l y a p p o s e d v o c a l c o r d s . I t i s t h e mo s t c o mmo n s y mp t o m o f
laryngeal disease. Hoarseness occurs early in the process of laryngeal disease and
c a n b e r e a d i l y d i a g n o s e d i n p r i ma r y c a r e s e t t i n g s . B e c a u s e c a n c e r o f t h e l a r y n x h a s
u s u a l l y b e e n p r e s e n t f o r 6 mo n t h s b e f o r e a d i a g n o s i1,2)
s i s, ma
t h ed er e(c o g n i t i o n
a n d p r o mp t e v a l u a t i o n o f h o a r s e n e s s i s p a r a mo u n t .
A. Etiology
F o u r b r o a d e t i o l o g i c c a t e g o r i e s a c c o u n t f o r mo s t v o c a2,3,4,5)
l c h a n:g e s (
1. I n f l a m m a t i o n o r e d e m a o f t h e l aI nr yf encxt i o n ( e s p e c i a l l y v i r a l l a r y n g i t i s ) ,
g a s t r o i n t e s t i n a l r e f l u x, a l l e r g i e s , e xp o s u r e t o i r r i t a n t s ( t o b a c c o , a l c o h o l , t o xi c
f u me s ) , v o i c e a b u s e , a s p i r a t i o n
2. P r o c e s s e s a f f e c t i n g p o s i t i o n o r a p p r o x i m a t i o n o f t h e v oVo
c ac al lc o r d s
p o l y p s o r n o d u l e s , c o n t a c t u l c e r s , g r a n u l o ma t o u s d i s e a s e ( s a r c o i d , f u n g a l ,
s y p h i l i t i c , a u t o i mmu n e ) , n e o p l a s ms ( h e ma n g i o ma , p a p i l l o ma , s q u a mo u s c e l l
c a r c i n o ma )
3. M a l f u n c t i o n o f t h e l a r yI nnt xu b a t i o n , t r a u ma , n e r v e d a ma g e ( r e c u r r e n t
l a r y n g e a l o r v a g a l ) f r o m t u mo r s , s u r g e r y, a g i n g c h a n g e s ( a t r o p h y, b o w i n g )
4. S y s t e m i c p r o c e s s H
e sy p o t h y r o i d i s m, r h e u ma t o i d a r t h r i t i s , P a r k i n s o n ' s
d i s e a s e , mu l t i p l e s c l e r o s i s , a c r o me g a l y, my a s t h e n i a g r a v i s , a n d
psychogenic/psychiatric
B. Epidemiology
L aryngeal cancer is rare in patients younger than 50 years of age. Children with
h o a r s e n e s s mu s t b e e v a l u a t e d f o r j u v e n i l e p a p i l l2,5)
o ma. t C
o seirst a(i n l i f e s t y l e s a n d
o c c u p a t i o n s l e a d t o h o a r s e n e s s a n d d e v e l o p me n t o f v o c a l n o d u l e s . S mo k i n g a n d
a l c o h o l a r e w e l l - k n o w n l a r y n g e a l i r r i t a n t s a n d c a r c i n o g e n s . P a t i e n t s w i t h r e f l u x,
n a s a l a l l e r g i e s , s i n u s i t i s , p r i o r s u r g e r y ( t h y r o i d ) , a n d h y p o t h y r o i d i s m a r e mo r e l i k e l
181 / 652
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6.3 - Hoarseness
to present with hoarseness. L aryngitis, voice abuse, and vocal cord nodules are
a mo n g c o mmo n c a u s e s o f h o a r s e n e s s .
P. 1 0 0
I I I . Evaluation
A. History
A s k p a t i e n t s a b o u t t h e c i r c u ms t a n c e s p r e c e d i n g t h e o n s e t , t h e mo d e o f o n s e t ,
d u r a t i o n , a n d c o n s i s t e n c y o f t h e h o a r s e n e s s . T h e v o i c e o f p a t i e n t s w i t h my a s t h e n i a
g r a v i s b e c o me s h o a r s e r a s t h e d a y p r o g r e s s e s . I n t e r mi t t e n t s y mp t o ms a r g u e a g a i n s
a f i xe d l e s i o n . P r o mi n e n t mo r n i n g s y mp t o ms t h a t i mp r o v e t h r o u g h t h e d a y s u g g e s t
n o c t u r n a l g a s t r o e s o p h a g e a l r e f l u x d i s e a s e (CGhEaR
p tDe)r (9 ). .6 A h i s t o r y o f
indigestion, heartburn, or regurgitation suggests reflux laryngitis from G E R D.
Q u e s t i o n w h e t h e r t a l k i n g e xa c e r b a t e s t h e h o a r s e n e s s . A s k a b o u t p a i n , d y s p h a g i a , o
t r o u b l e mo u n t i n g a n a d e q u a t e r e s p i r a t o r y f o r c e . P a i n i s a l a t e p h e n o me n o n i n
c a r c i n o ma b u t i s p r o mi n e n t i n v i r a l o r r e f l u x l a r y n g i t i s . C h r o n i c c o u g h , s p u t u m
p r o d u c t i o n , o r s i n u s p r o b l e ms p o i n t s t o p o s t n a s a l d r a i n a g e . S p e c i f i c i n c i t i n g e v e n t s
u p p e r r e s p i r a t o r y i n f e c t i o n s , s o r e t h r o a t s , f e v e r s , my a l g i a s , f a t i g u e , o r o t h e r
i n f e c t i o u s e xp o s u r e s ( t u b e r c u l o s i s ) n e e d t o b e e xp l o r e d . E xp o s u r e t o e n v i r o n me n t a
i r r i t a n t s s u c h a s d u s t , f i r e , s mo k e , o r n o xi o u s f u me s s h o u l d b e i n v e s t i g a t e d . A
s mo k i n g a n d a l c o h o l h i s t o r y i s c r i t i c a l . A n y h i s t o r y o f i n t u b a t i o n , p r i o r n e c k s u r g e r y
o r n e c k ma s s s h o u l d b e n o t e d . I n f o r ma t i o n a b o u t v o i c e u s e a n d p o s s i b l e a b u s e
s h o u l d b e o b t a i n e d . D o e s t h e p a t i e n t r a i s e h i s o r h e r v o i c e o v e r c r o w d s o r ma c h i n e
or by yelling? Does his or her profession involve voice overuse or abuse?
1. L i s t e n t o t h e p a t i e n t ' s v o i c e t o e v a l u a t e v o i c1)
e .q N
u aol ist yp e( c i f i c f e a t u r e s o f
hoarseness are definitively diagnostic, but the voice is harsh when turbulence i
c r e a t e d b y t h e i r r e g u l a r i t y o f t h e v o c a l c o r d s , a s i n l a r y n g i t i s o r a ma s s l e s i o n .
r a s p y v o i c e s u g g e s t s c o r d t h i c k e n i n g c a u s e d b y e d e ma o r i n f l a mma t i o n ; a
b r e a t h y v o i c e i n d i c a t e s p o o r v o c a l c o r d p o s i t i o n o r a p p r o xi ma t i o n ; a n d a h i g h ,
s h a k y, o r s o f t v o i c e i s mo s t p r o b a b l y c a u s e d b y t h e ma l f u n c t i o n o f t h e l a r y n x
(1,2) . E xa mi n e t h e s c a l p , n e c k , t h y r o i d g l a n d , c e r v i c a l n o d e s , e a r s , n o s e ,
s i n u s e s , a n d o r a l c a v i t y. T e n d e r n e c k a d e n o p a t h y s u g g e s t s i n f e c t i o n , w h e r e a s
p a i n l e s s e n l a r g e me n t ma y i mp l y ma l i gCnhaanpc tye r( s 1 5 .a1n d1 5 . 2) . I f n o
d i a g n o s i s i s o b v i o u s f r o m t h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n , t h e v i s u a l i za t i o n
o f t h e l a r y n x i s r e c o mme n d e d , e s p e c i a l l y i f t h e h o a r s e n e s s i s o f mo r e t h a n 2 t o
3 w e e k s ' d u r a t i o5)n . ( U s i n g o n e o f t h e t e c h n i q u e s d e s c r i b e d i n t h e s u b s e q u e n t
t e xt , c a r e f u l l y i n s p e c t t h e p o s t e r i o r n a s o p h a r y n x, t o n g u e , l y mp h o i d t i s s u e , a n d
e n t i r e l a r y n x. P e r f o r m v o c a l ma n e u v e r s w h i l e d i r e c t l y o b s e r v i n g v o c a l c o r d
mo v e me n t . A s s e s s f o r mu c o s a l a n d c a r t i l a g i n o u s l e s i o n s , e d e ma , e r y t h e ma , a n d
e xc e s s mu c u s ; t h e l a t t e r f i n d i n g s u g g e s t s p r o mi n e n t a l l e r g i e s . E d e ma t o u s v o c a l
c o r d s a n d g l o t t i s w i t h h y p e r e mi a s u g g e s t s G E R D o r l a r y n g i t i s .
2. T e c h n i q u e s f o r l a r y n g e a l v i s u a l i za t i o n i n c l u d e t h e f o l l o w i n g :
a. I n d i r e c t l a r y n g o s c o p y i s p e r f o r me d b y p l a c i n g a l a r y n g e a l mi r r o r ( w a r me d t o
prevent fogging) against the soft palate while grasping the tongue with
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6.3 - Hoarseness
g a u ze . A b r i g h t l i g h t s o u r c e i s f o c u s e d o n t h e l a r y n g e a l mi r r o r t o r e v e a l a n
i ma g e o f t h e l a r y n x. T h i s t e c h n i q u e , a l t h o u g h s i mp l e , c a n p r o v e d i f f i c u l t d u e
t o a s t r o n g t o n g u e o r g a g r e f l e x.
b. F l e xi b l e n a s o p h a r y n g o l a r y n g o s c o p y p r o v i d e s a n e xc e l l e n t v i e w o f t h e l a r y n x
a n d a v o i d s t h e p r o b l e ms n o t e d i n t h e p r e c e d i n g t e xt . T h e s c o p e i s p a s s e d
intranasally after topical anesthesia is applied (e.g., 2% lidocaine gel). T he
l a r y n x i s v i s u a l i ze d w h i l e t h e p a t i e n t s w a l l o w s a n d p h o n a t e s .
c. Vi d e o - a s s i s t e d r i g i d l a r y n g o s c o p y i s s u p e r i o r t o a l l o t h e r e xa mi n a t i o n
me t h o d s b u t i s r e s e r v e d f o r c o n s u l t a n t u s e w h e n d i a g n o s i s r e ma i n s
u n c e r t a i n5)( .
C. Testing
T h y r o i d f u n c t i o n i s t h e o n l y r o u t i n e b l o o d t e s t i n g r e c o mme n d e d , u n l e s s d i c t a t e d b y
f e a t u r e s o f t h e h i s t o r y o r p h y s i c a l e xa mi n a t i o n . I f i n d i c a t e d , ma g n e t i c r e s o n a n c e
i ma g i n g i s t h e mo d a l i t y o f c h o i c e t o a s s e s s t h e e xt e n t o f s e r i o u s l a r y n g e a l o r n e c k
d i s e a s e 5)( .
I V. Diagnosis
T h e k e y t o d i a g n o s i s i s a t h o r o u g h h i s t o r y c o mb i n e d i n mo s t c a s e s w i t h t h e
v i s u a l i za t i o n o f t h e l a r y n x. H o a r s e n e s s o f l e s s t h a n 2 w e e k s d u r a t i o n i s c o n s i d e r e d
a c u t e a n d i s u s u a l l y s e l f - l i mi t e d . C h r o n i c h o a r s e n e s s ( > 2 3 w e e k s d u r a t i o n )
s u g g e s t s a mo r e s e r i o u s c a u s e a n d a l a r y n g e a l e xa mi n a t i o2,3,4,5)
n i s c r .i t i c a l (
P. 1 0 1
M o s t c a u s e s o f h o a r s e n e s s a r e b e1,2,3)
n i g n. (S o me l a r y n g e a l l e s i o n s h a v e a
p a t h o g n o mo n i c a p p e a r a n1,2)
c e . ( Vo c a l p o l y p s a r e b e n i g n a n d r e s u l t f r o m c h r o n i c
v o i c e a b u s e o r d i r e c t t r a u ma . T h e y o c c u r o n t h e a n t e r i o r p o r t i o n o f o n e v o c a l c o r d .
Vo c a l n o d u l e s r e s u l t f r o m p o o r v o i c e u s e ( e . g . , s i n g e r s , p r e a c h e r s ) , a n d a l w a y s
o c c u r s a t t h e j u n c t i o n o f t h e a n t e r i o r a n d mi d d l e t h i r d o f t h e v o c a l c o r d s b i l a t e r a l l y.
Contact ulcers present as bilateral ulcerations at the tips of the laryngeal cartilage
a n d a r e t h e o n l y c o mmo n l e s i o n s o t h e r t h a n c a n c e r t h a t c a u s e t h r o a t p a i n .
L e u k o p l a k i a p r e s e n t s a s a r a i s e d , w h i t e p l a q u e a t t h e a n t e r i o r e xt r e mi t y o f o n e v o c a
c o r d . I t i s u s u a l l y p r e ma l i g n a n t , r e l a t e d t o a l c o h o l u s e o r s mo k i n g , a n d n e e d s t o b e
b i o p s i e d . L a r y n g e a l c a n c e r p r o d u c e s e a r l y c h a n g e s i n v o i c e q u a l i t y a n d i s t h e mo s t
serious cause of hoarseness. It presents as persistent hoarseness with a lesion in
t h e h y p o p h a r y n x, g l o t t i s , o r s u p r a g l o t t i s . A n y s u s p i c i o u s l e s i o n s e e n o n l a r y n g o s c o p
should be biopsied.
References
1 . R o s e n C A , A n d e r s o n D , M u r r y T . E v a l u a t i n g h o aAr m
s e nFeam
s s .P hy s i c i an
1998;57:27752782.
183 / 652
tmdmss
6.3 - Hoarseness
2 . G a r r e t t C G , O s s o f f R H . H o a r s eM
n eed
s s .C l i n N or t h A1m9 9 9 ; 8 3 : 11 5 1 2 3 .
3 . B a n f i e l d G , T a n d o n P, S o l o mo n s N . H o a r s e v o i c e : a n e a r l y s y mp t o m o f ma n y
c o n d i t i o n sP. r ac t i t i oner2 0 0 0 ; 2 4 4 : 2 6 7 2 7 1 .
4 . S i mp s o n B C , F l e mi n g D J . M e d i c a l a n d v o i c e h i s t o r y i n e v a l u a t i o n o f
d y s p h o n i aO. t ol ar y ngol C l i n N or t h 2A0m
00;33(4):719730.
5 . Va n d e r G o t e n A . E v a l u a t i o n o f p a t i e n t s w i t h h o aErur
s e nReadi
s s .ol
2004;14:14061415.
184 / 652
tmdmss
6.4 - Nosebleed
> T a b l e o f C o n t e n t s > 6 - E a r, N o s e , a n d T h r o a t P r o b l e ms > 6 . 4 - N o s e b l e e d
6.4
Nosebleed
Douglas G. Brow ning
Chad Ezze ll
I . Background
N o s e b l e e d , o r e p i s t a xi s , i s a c o mmo n o t o l a r y n g o l o g i c p r o b l e m, d e f i n e d a s t h e l o s s o
b l o o d f r o m t h e mu c o u s me mb r a n e s l i n i n g t h e n o s e , u s u a l l y f r o m a s i n g l e n o s t r i l .
A. Etiology
E p i s t a xi s r e s u l t s f r o m a n i n t e r a c t i o n o f f a c t o r s t h a t d a ma g e t h e n a s a l e p i t h e l i a l
( mu c o s a l ) l i n i n g a n d v e s s e l w a l l s . T h e c a u s a t i v e f a c t o r s o r p r o c e s s e s i n c l u d e :
1. E n v i r o n m e n t aDl r y a i r, c o l d a mb i e n t t e mp e r a t u r e
2. L o c a lA c c i d e n t a l o r s e l f - i n f l i c t e d t r a u ma , i n f e c t i o n , a l l e r g i e s , f o r e i g n b o d y,
a n a t o mi c a b n o r ma l i t i e s ( d e v i a t e d s e p t u m) , i a t r o g e n i c ( s u r g e r y ) , n e o p l a s ms
3. S y s t e m i cH y p e r t e n s i o n , p l a t e l e t a n d c o a g u l a t i o n a b n o r ma l i t i e s , b l o o d
d y s c r a s i a s , d i s s e mi n a t e d i n t r a v a s c u l a r c o a g u l a t i o n , r e n a l f a i l u r e , a l c o h o l a b u s e
4. D r u g s a f f e c t i n g c l o t tAi ns pg i r i n , n o n s t e r o i d a l a n t i - i n f l a mma t o r y d r u g s ,
w a r f a r i n , h e p a r i n , t i c l o p i d i n e , d i p y r i d a mo l e
5. O t h e r d r u g N
s a s a l s t e r o i d s , t h i o r i d a zi n e , a n t i c h o l i n e r g i c s ( d r y i n g )
6. H e r e d i t a r yH e mo p h i l i a , v o n W i l l e b r a n d ' s d i s e a s e , h e r e d i t a r y h e mo r r h a g i c
t e l a n g i e c t a s i a ( O s l e r - We b e r - R e n d u )
7. I d i o p a t h i cC o l d , d r y a i r i n c r e a s e s c a s e s o f e p i s t a xi s a s d e mo n s t r a t e d i n
c o u n t r i e s w i t h s e a s o n a l c l i ma t e s w h e r e h o s p i t a l a d mi s s i o n s f o r n o s e b l e e d
i n c r e a s e s i g n i f i c a n t l y d u r i n g t h e w i n t e r 1)
mo
. nRtehgs a(r d i n g t h e i mp o r t a n c e o f
a n a t o mi c a b n o r ma l i t i e s , a s t u d y o f r e c u r r e n t e p i s t a xi s s h o w e d t h a t 8 1 % o f
p a t i e n t s h a d s e p t a l d e v i a t i o n v e r s u s 3 1 % o f t h e c o n2)
t r .o lAgl trhoouupg h(
hypertension
P. 1 0 2
i s o f t e n a s s o c i a t e d w i t h e p i s t a xi s , s t u d i e s h a v e h a d c o n f l i c t i n g r e s u l t s a n d t h e
r e l a t i o n s h i p h a s y e t t o b e c o n f 3)
i r me
(C dh a( p t e r 7 ). .8
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B. Epidemiology
A s ma n y a s 1 5 p e r 1 0 , 0 0 0 i n d i v i d u a l s r e q u i r e p h y s i c i a n c a r e a n n u a l l y f o r e p i s t a xi s ,
a n d 1 . 6 / 1 0 , 0 0 0 r e q u i r e a d mi s s i o n t o t h e h o s p i t a l f o r a p e r s i s t e n t n o s e b l e e d . M o s t
cases occur in patients younger than 10 years of age, and the incidence decreases
w i t h a g e4)( .
I I I . Evaluation
A. History
Initial history should include onset, duration, whether one or both nostrils are
a f f e c t e d , a n d t h e q u a n t i t y ( n u mb e r o f s o a k e d t o w e l s ) . A d e t a i l e d h i s t o r y e xp l o r i n g t h
p o s s i b l e p r e c i p i t a t i n g f a c t o r ss e( cs tei eo nI I . A. ) s h o u l d b e t a k e n . I t i s a l s o i mp o r t a n t
t o i n q u i r e a b o u t c h r o n i c me d i c a l c o n d i t i o n s t h a t c o u l d b e c o n t r i b u t o r y, i n c l u d i n g a
history of blood dyscrasias, hypertension, liver disease, and alcohol use. In childre
w i t h e p i s t a xi s w i t h u n i l a t e r a l n a s a l d i s c h a r g e o r f o u l o d o r, b e a l e r t t o t h e p o s s i b i l i t y
o f a n i n t r a n a s a l f o r e i g n b o d y.
T h e b l o o d s u p p l y t o t h e n o s e a r i s e s f r o m t h e i n t e r n a l ma xi l l a r y a n d f a c i a l a r t e r i e s
t h r o u g h t h e e xt e r n a l c a r o t i d a r t e r y a n d t h e a n t e r i o r a n d p o s t e r i o r e t h mo i d a r t e r i e s
through the internal carotid. T he anteroinferior septum (L ittle's area) is supplied by
c o n f l u e n c e o f b o t h s y s t e ms k n o w n a s K i e s s e l b a c h ' s p l e xu s . L i t t l e ' s a r e a i s a
c o mmo n s i t e o f e p i s t a xi s b e c a u s e i t i s i d e a l l y p l a c e d t o r e c e i v e e n v i r o n me n t a l
i r r i t a t i o n ( c o l d , d r y a i r, c i g a r e t t e s mo k e ) , a n d i s e a s i l y a c c e s s i b l e t o d i g i t a l t r a u ma .
F o r t u n a t e l y, t h i s a r e a i s e a s y t o a c c e s s a n d t r e a t . H o w e v e r, a s a p p r o xi ma t e l y 1 0 % o
n o s e b l e e d s o r i g i n a t e f r o m a p o s t e r i o r n a s a 4)
l s, oiut rccaen( b e f a r mo r e d i f f i c u l t t o
i d e n t i f y a s o u r c e o f e p i s t a xi s i n t h i s a r e a . P r o v i d i n g e f f e c t i v e t r e a t me n t f o r o b s t i n a t
b l e e d i n g i n t h i s a r e a ma y a l s o b e mo r e u n c o mf o r t a b l e f o r t h e p a t i e n t a n d mo r e
f o r mi d a b l e f o r t h e h e a l t h p r o v i d e r.
1. W h e n e xa mi n i n g t h e p a t i e n t w i t h e p i s t a xi s , f i r s t a s s e s s t h e v i t a l s i g n s f o r
h y p o t e n s i o n , o r t h o s t a s i s , a n d h e mo d y n a mi c i n s t a b i l i t y. A f t e r e xa mi n i n g t h e f a c e
f o r a n y o b v i o u s s i g n s o f r e c e n t i n j u r y, i t i s i mp o r t a n t t o v i s u a l i ze a s mu c h o f t h e
n a s a l v e s t i b u l e a s p o s s i b l e . I t i s i mp e r a t i v e t o k e e p t h e p a t i e n t ' s h e a d u p r i g h t ,
for if he or she tilts backward, only the roof of the nasal cavity is seen. T he
n a s a l s p e c u l u m s h o u l d b e h e l d i n a h o r i zo n t a l p o s i t i o n t o a l l o w a n o p t i ma l v i e w
o f t h e n a s a l s e p t u m, w h i c h i s t h e s i t e o f mo s t b l e e d i n g .
2. Vi s u a l i za t i o n o f t h e b l e e d i n g c a n b e p e r f o r me d b y t h e d i r e c t i l l u mi n a t i o n o f t h e
a r e a o r s o me t i me s mo r e e a s i l y b y i n d i r e c t i l l u mi n a t i o n u s i n g a h e a d mi r r o r.
S u c t i o n ma y b e n e e d e d t o r e mo v e c l o t s , f r e s h b l o o d , o r mu c u s t o v i s u a l i ze t h e
bleeding. Direct nasopharyngoscopy with endoscopy (using a topical anesthetic
s u c h a s C e t a c a i n e o r l i d o c a i n e g e l ) ma y b e n e c e s s a r y, e s p e c i a l l y i f t h e s o u r c e
o f t h e b l e e d i n g i s e xt r e me l y p o s t e r i o r. T o p i c a l v a s o c o n s t r i c t o r s s u c h a s
p h e n y l e p h r i n e o r o xy me t a zo l i n e c a n b e u s e f u l i n d e c r e a s i n g t h e r a t e o f b l e e d i n g
i n o r d e r t o v i s u a l i ze t h e a r e a ( a n d ma y s o me t i me s h e l p a c h i e v e l o n g - t e r m
cessation of the bleeding).
186 / 652
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6.4 - Nosebleed
3. D e p e n d i n g o n t h e p a t i e n t ' s h i s t o r y, a mo r e g e n e r a l e xa mi n a t i o n ma y b e n e e d e d
w i t h a f o c u s o n t h e s k i n t o l o o k f o r p e t e c h i a e , t e l a n g i e c t a s i a s , h e ma n g i o ma s ,
a n d e c c h y mo s eC
s h( a p t e r 1 5). .3
C. Testing
1. L a b o r a t o r y t e s Itfs b l e e d i n g i s mi n o r a n d n o t r e c u r r i n g , n o t e s t i n g i s n e e d e d .
F o r v i g o r o u s b l e e d i n g o r r e c u r r e n t e p i s t a xi s , o r d e r a c o mp l e t e b l o o d c o u n t
( C B C ) w i t h p l a t e l e t c o u n t , b l e e d i n g t i me , p r o t h r o mb i n t i me , p a r t i a l
t h r o mb o p l a s t i n t i me , a n d p o s s i b l y b l o o d t y p e a n d c r o s s ma t c h f o r h y p o v o l e mi c
s h o c k o r s e v e r e a n e mi a . T e s t i n g t h e s t o o l s f o r o c c u l t b l o o d ma y h e l p a s s e s s
c h r o n i c i t y. T h e C B C c a n d e t e c t b l o o d d y s c r a s i a s a s w e l l a s a n e mi a . A n e l e v a t e d
b l e e d i n g t i me ma y i mp l y a s p i r i n u s e , v o n W i l l e b r a n d ' s d i s e a s e , a n d ma n y
p l a t e l e t - b a s e d b l e e d i n g d i s o r d e r s . C o a g u l a t i o n t i me s c a n b e e l e v a t e d i n
c o a g u l a t i o n f a c t o r d i s e a s e s , b u t mo r e o f t e n t h a n n o t , t h e y i mp l i c a t e l i v e r
disease.
2. I m a g i n gS i n u s r a d i o g r a p h s o r a l i mi t e d c o mp u t e d t o mo g r a p h y o f t h e s i n u s e s
ma y a l s o b e c o n s i d e r e d i f c o n c e r n e xi s t s f o r b e n i g n n e o p l a s ms o r ma l i g n a n c y.
R a r e l y, a n g i o g r a p h y ma y a l s o b e i n d i c a t e d f o r d i a g n o s i n g ( a n d t r e a t i n g )
vascular lesions.
P. 1 0 3
D. Genetics
E p i s t a xi s i s t h e p r e s e n t i n g c o mp l a i n t i n f o u r o f f i v e c a s e s o f t h e r a r e , h e r e d i t a r y
h e mo r r h a g i c t e l a n g i e c t a s i a s ( O s l e r - We b e r - R e n d u3)d.i s e a s e ) (
I V. Diagnosis
F o r p e r s i s t e n t o r r e c u r r e n t n o s e b l e e d s , i t i s i mp o r t a n t t o l o o k f u r t h e r f o r t h e
u n d e r l y i n g c a u s e o f t h e p r o b l e m b y p e r f o r mi n g a c a r e f u l h i s t o r y a n d e v a l u a t i n g t h e
p r o b l e m w i t h e xp e d i e n t l a b o r a t o r y e v a l u a t i o n s , a p p r o p r i a t e i ma g i n g , o r f u r t h e r
c o n s u l t a t i o n w h e n n e c e s s a r y t o r u l e o u t mo r e ma l i g n a n t c a u s e s . R a r e c a u s e s o f
e p i s t a xi s i n c l u d e p o t e n t i a l l y l i f e - t h r e a t e n i n g p o s t t r a u ma t i c p s e u d o a n e u r y s m o f t h e
i n t e r n a l c a r o t i d a r t e r y. T h i s e n t i t y p r e s e n t s f r o m d a y s t o w e e k s a f t e r i n i t i a l t r a u ma
the base of the skull with a classic triad of unilateral blindness, orbital fractures, a
ma s s i v e e p i s t a xi s . F i n a l l y, a l t h o u g h t h e o b s t r u c t i o n o f a i r mo v e me n t i s t h e mo s t
c o mmo n p r e s e n t i n g s y mp t o m o f i n t r a n a s a l n e o p l a s ms , p a t i e n t s ma y a l s o p r e s e n t w i t
e p i s t a xi s a n d / o r n a s a l p5)a .i n (
Bleeding from one or both nostrils occurs, with associated frequent swallowing and
sensation of fluid in the back of the nose and/or throat.
References
187 / 652
tmdmss
6.4 - Nosebleed
1 . T o mk i mo n A , B r e mme r - S mi t h A , C r a v e n C , e t a l . H o s p i t a l e p i s t a xi s a d mi s s i o n s
a n d a mb i e n t t e mp e r a t uCrlei n. O t ol ar y ngol
1995;20:239240.
2 . O ' R e i l l y B J , S i mp s o n D C , D h a r me r a t n a m R . R e c u r r e n t e p i s t a xi s a n d n a s a l
s e p t a l d e v i a t i o n i n y o u n g a dCul il tns .O t ol ar y ngol
1996;21:8284.
3. H erkner H , L aggner A, M ullner M , et al. H ypertension in patients presenting
w i t h e p i s t a xiAs nn
. E mer g M ed
2000;35(2):126130.
4 . M i d d l e t o n P M . E p i s t aEximer
s . g M ed A us t r al 2as
004;6(56):428440.
5 . D y k e w i c z M S . R h i n i t i s a n d s i n uJ sAi tli lser
. gy C l i n I mmunol
2 0 0 3 ; 111 ( S u p p l
2):S520S529.
188 / 652
tmdmss
6.5 - Pharyngitis
> T a b l e o f C o n t e n t s > 6 - E a r, N o s e , a n d T h r o a t P r o b l e ms > 6 . 5 - P h a r y n g i t i s
6.5
Pharyngitis
R i c h a r d W. L o r d
I . Background
P h a r y n g i t i s , c o mmo n l y c a l l e d s o r e t h r o a t ( S T ) , i s a n i n f l a mma t o r y p r o c e s s o f t h e
pharynx that can be caused by viral or bacterial pathogens, and occasionally both.
S T i s o n e o f t h e mo s t c o mmo n r e a s o n s f o r a d u l t s a n d c h i l d r e n t o s e e k c a r e f r o m a
p r i ma r y c a r e p h y s i c i1)
a n. A( l t h o u g h t h e d i f f e r e n t i a l d i a g n o s i s o f S T i n c l u d e s s u c h
entities as epiglottis, peritonsillar abscess, L udwig angina, and gastroesophageal
r e f l u x, t h e mo s t c o mmo n c a u s e i n i mmu n o c o mp e t e n t i n d i v i d u a l s i s a c u t e p h a r y n g i t i s
A c u t e p h a r y n g i t i s i s a f r e q u e n t r e a s o n f o r a n t i b i o t i c p r e s c r i p t i o n s a l t h o u g h t h e mo s
c o mmo n c a u s e s a r e v i2)
r a. l T( h e i n a p p r o p r i a t e u s e o f a n t i b i o t i c s c a n h a v e n e g a t i v e
c o n s e q u e n c e s f o r i n d i v i d u a l a n d p u b l i c h e a l t h . T h e r e f o r e , i t i s i mp o r t a n t f o r
physicians to develop an approach for identifying those individuals with pharyngitis
c a u s e d b y g r o u p A - h e mo l y t i c s t r e p t o c o c c u s ( G A B H S ) .
A. Etiology
O n l y a s ma l l mi n o r i t y ( 1 0 % 2 0 % ) o f p a t i e n t s w i t h S T a r e i n f e c t e d w i t h G A B H S
(3,4,5) . H o w e v e r, t o mi n i mi ze t h e p o t e n t i a l a d v e r s e e f f e c t s o f i n a p p r o p r i a t e
a n t i mi c r o b i a l t h e r a p y, i t i s i mp o r t a n t t o i d e n t i f y G A B H S i n f e c t i o n a c c u r a t e l y b e c a u s
i t i s t h e o n l y c o mmo n l y o c c u r r i n g f o r m o f p h a r y n g i t i s f o r w h i c h a n t i b i o t i c t h e r a p y i s
d e f i n i t i v e l y i n d i c a t2,3)
e d .( T h e e a r l y i d e n t i f i c a t i o n a n d t r e a t me n t o f G A B H S h e l p s
p r e v e n t r h e u ma t i c f e v e r, p r o v i d e s y mp t o ma t i c r e l i e f , r e d u c e s s u p p u r a t i v e
c o mp l i c a t i o n s , a n d d e c r e a s e s i n f e 3,4)
c t i v.i tTya b( l e 6 . 5 . l1i s t s t h e d i f f e r e n t c a u s e s
o f p h a r y n g i t i3,4,5)
s ( .
P. 1 0 4
TAB L E 6.5.1 Causes of Acute Pharyngitis

Cause
Vi r a l
Pe rce nt of case s
50%80%
189 / 652
tmdmss
6.5 - Pharyngitis
G r o u p A - h e mo l y t i c s t r e p t o c o c c u5s% 3 0 % ( me a n 1 5 % i n a d u l t s )
Epstein-Barr virus
1%10%
C hl amy di a pneumoni ae
2%5%
M y c opl as ma pneumoni ae
2%5%
N ei s s er i a gonor r hoeae
1%2%
A c u t e h u ma n i mmu n o d e f i c i e n c y v i R
r uasr e
Other bacteria, fungi, parasites Rare
Referred pain (dental)
Rare
Noninfectious causes
Rare
B. Epidemiology
P h a r y n g i t i s i s t h e s i xt h mo s t c o mmo n r e a s o n f o r s e e i n g a p h y s i c i a n , a n d a c c o u n t s f
a p p r o xi ma t e l y 5 % o f a l l p r i ma r y c a r e o f f i c1)e . vDi sai t as s( h o w t h a t t h e p r o b a b i l i t y
o f G A B H S p h a r y n g i t i s i n p r i ma r y c a r e p e a k s b e t w e e n 5 a n d 1 5 y3,4,5)
e a r s. o f a g e (
I n f e c t i o u s mo n o n u c l e o s i s ( I M ) a s a c a u s e o f S T p e a k s b e t w e e n 1 5 a n d 3 0 y e a r s o f
a g e 3( ,4,5) .
T r a n s mi s s i o n o c c u r s mo s t l y b y h a n d c o n t a c t w i t h n a s a l d i s c h a r g e r a t h e r t h a n b y
c o n t a c t w i t h o r a l s e c r e t i4,5)
o n s. (S y mp t o ms t y p i c a l l y o c c u r 4 8 t o 7 2 h o u r s l a t e r.
M o s t p h a r y n g i t i s i s s e l f - l i mi t e d a n d l a s t s 7 t o 1 0 d a y s . I f p h a r y n g i t i s l a s t s f o r l o n g e
than 3 weeks, noninfectious causes should be considered. Although pharyngitis can
o c c u r a t a n y t i me d u r i n g t h e y e a r, t h e r e i s a p e a k i n t h e w i n t e r a n d s p r i n g .
I I I . Evaluation
T he goal of the evaluation is to differentiate those patients who are likely to have a
infectious cause of pharyngitis and need laboratory testing, and those who have
n o n i n f e c t i o u s c a u s e s . H i s t o r y, p h y s i c a l e xa mi n a t i o n , a n d a p p r o p r i a t e d i a g n o s t i c
l a b o r a t o r y t e s t s a r e u s e d t o a c c o mp l i s h t h i s g o a l .
A. History
1. T h e o n s e t a n d d u r a t i o n o f t h e S T c a n h e l p d i f f e r e n t i a t e i n f e c t i o u s f r o m
n o n i n f e c t i o u s c a u s e s . T y p i c a l l y, t h e o n s e t o f i n f e c t i o u s c a u s e s i s a b r u p t a n d
l a s t s f o r 7 t o 1 0 d a y s . O f t e n w i t h n o n i n f e c t i o u s c a u s e s , t h e o n s e t o f s y mp t o ms i
u n c l e a r a n d o f t e n p e r s i s t s f o r mo r e t h a n 3 w e e k s .
2. A s s o c i a t e d s y mp t o ms c a n a l s o g i v e c l u e s t o t h e c a u s e o f t h e S T . F e v e r, c o u g h ,
h e a d a c h e , a n d o t h e r c o n s t i t u t i o n a l s y mp t o ms a r e t h e h a l l ma r k s o f a n i n f e c t i o u s
190 / 652
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6.5 - Pharyngitis
e t i o l o g y. S y mp t o ms o f a l l e r g i e s , h e a r t b u r n , a n d d e p r e s s i o n p o i n t t o
n o n i n f e c t i o u s c a u s e s . A n y h i s t o r y o f s e a s o n a l a l l e r g i e s , t r a u ma , ma l i g n a n c y,
r a d i a t i o n t h e r a p y, i n h a l a t i o n , i n g e s t i o n , o r t h y r o i d d y s f u n c t i o n p o i n t t o
noninfectious causes.
3. I f i n f e c t i o n i s b e l i e v e d t o b e t h e c a u s e o f t h e S T , t h e f o c u s i s t h e n o n t r y i n g t o
d i f f e r e n t i a t e G A B H S f r o m o t h e r b a c t e r i a l a n d v i r a l c a u s e s . C l a s s i c a l l y, G A B H S
p h a r y n g i t i s i s s e v e r e a n d o f a c u t e ( < 1 d a y ) o n s e t , a n d a c c o mp a n i e d b y f e v e r
( t e mp e r a t u r e > 1 0 1 F [ 3 8 . 3 3 C ] ) , p a i n f u l s w a l l o w i n g , t e n d e r a n t e r i o r c e r v i c a l
a d e n o p a t h y, a n d my a l g i a s , b u t n o t b y c o u g h o r r h i n i t i s . H e a d a c h e , n a u s e a ,
v o mi t i n g , a n d a b d o mi n a l p a i n ma y b e s e e n a s w e l l , e s p e c i a l l y i n c h i l d r e n .
C o n v e r s e l y, t h e g r a d u a l o n s e t o f mi l d S T a c c o mp a n i e d b y r h i n o r r h e a , c o u g h ,
hoarseness, conjunctivitis, or diarrhea in an afebrile patient speaks strongly for
a v i r a l c a u s e . D e s p i t e t h e s e b r o a d g e n e r a l i za t i o n s , c l a s s i c s y mp t o m c o mp l e xe s
alone are neither sensitive nor specific enough to rely on for judging the need
f o r a n t i b a c t e r i a l t r e a t me
2,3,4,5)
nt ( .
T h e p h y s i c a l e xa mi n a t i o n s h o u l d i n c l u d e a s s e s s i n g v i t a l s i g n s ( e s p e c i a l l y
t e mp e r a t u r e ) a n d e xa mi n i n g t h e h e a d , e y e s , e a r s , n o s e , t h r o a t ,
P. 1 0 5
neck, and skin. Findings classically associated with G AB H S infection include palata
p e t e c h i a e , i n t e n s e ( b e e f y r e d ) t o n s i l l o p h a r y n g e a l e r y t h e ma w i t h e xu d a t e s , t e n d e r
a n t e r i o r c e r v i c a l a d e n o p a t h y, a n d a s c a r l a t i n i f o r m r a s h . C o n v e r s e l y, t h e a b s e n c e o f
these features, along with the presence of rhinitis, hoarseness, conjunctivitis,
s t o ma t i t i s , d i s c r e t e u l c e r a t i v e l e s i o n s , o r a t y p i c a l v i r a l e xa n t h e m p o i n t s t o w a r d a
v i r a l c a u s e . I n I M , t h e c l a s s i c f e a t u r e s o f G A B H S a r e o f t e n c o mb i n e d w i t h p o s t e r i o r
c e r v i c a l o r g e n e r a l i ze d l y mp h a d e n o p a t h y a n d h e p a t o s p l e n o me g a l y. H o w e v e r, o n c e
a g a i n , n o n e o f t h e s e p h y s i c a l f i n d i n g s i n a n d o f t h e ms e l v e s h a v e s u f f i c i e n t l y h i g h
s e n s i t i v i t y a n d s p e c i f i c i t y t o r e l y o n f o r a c c u r a t e 3,4,5)
d i a g n. oAsni sa (b d o mi n a l
e xa mi n a t i o n i s d i c t a t e d e i t h e r b y g a s t r o i n t e s t i n a l s y mp t o ms o r b y t h e p r e s e n c e o f
severe fatigue with posterior cervical adenopathy (suggesting IM). A cough or a
f e v e r s h o u l d l e a d t o a p u l mo n a r y e xa mi n a t i o n . A c a r d i a c e xa mi n a t i o n i s i mp o r t a n t f o
t o xi c - a p p e a r i n g p a t i e n t s .
C. Testing
T he two tests typically used in the diagnosis of pharyngitis are the throat culture
(T C) and the rapid streptococcal antigen detection test (R S AD T ).
1. T h e s e n s i t i v i t y o f a n a p p r o p r i a t e l y o b t a i n e d ( v i g o r o u s l y s w a b b i n g b o t h t o n s i l s
a n d p o s t e r i o r p h a r y n x) T C i s 9 0 % t o3,4,5)
9 5 %. U
( n f o r t u n a t e l y, t h e T C d o e s
not reliably distinguish between an acute G AB H S infection and a streptococcal
c a r r i e r s t a t e w i t h c o n c o mi t a n t v i r a l i n f e c t i o n . A n e g a t i v e T C d o e s p e r mi t t h e
w i t h h o l d i n g o f a n t i mi c r o b i a l t h e r a p y ( i . e . , s p e c i f i c 3,4,5)
i t y = .0 . 9 9 ) (
2. A l t h o u g h me t h o d s v a r y, R S A D T s d o h a v e h i g h d e g r e e s o f s p e c i f i c i t y ( 9 2 %
9 5 % ) 3,4,5)
(
. T heir sensitivity in routine clinical practice is low (60%85%)
(3,4,5) . T h e r e f o r e , i n t h e p a s t t h e r e c o mme n d a t i o n h a d b e e n t o f o l l o w u p a
191 / 652
tmdmss
6.5 - Pharyngitis
n e g a t i v e a n t i g e n t e s t w i t h a b a c k u p T C . N e w e r g 2)
u i dheal vi nee bs e(e n
d e v e l o p e d t h a t b a s e t r e a t me n t d e c i s i o n s o n l y o n t h e R S A D T s . A s t u d y o f 3 0 , 0 0 0
patients over 2 years did not find a difference in sequelae from using only
R S A D T s f o r d e c i s i o n ma k i n g . R S A D T s s u f f e r t h e s a me l i mi t a t i o n a s T C s i n t h e
presence of carrier states.
3. S t r e p t o c o c c a l a n t i b o d y t i t e r s a r e o f n o i mme d i a t e v a l u e i n t h e d i a g n o s i s o f a c u t
G A B H S p h a r y n g i t i s . I f I M i s s u s p e c t e d , a c o mp l e t e b l o o d c o u n t a n d h e t e r o p h i l
antibody testing can reliably confirm the diagnosis if the patient is in the secon
week of illness.
4. G e n e r a l l y, n o o t h e r t e s t i n g i s n e e d e d u n l e s s a s e r i o u s s u p p u r a t i v e s e q u e l a i s
s u s p e c t e d ( e . g . , r e t r o p h a r y n g e a l a b s c e s s ) , i n w h i c h c a s e r a d i o l o g i c i ma g i n g
should be pursued.
I V. Diagnosis
1. R e c e n t g u i d e l i n e2,3,4,5)
s (
have called for the use of specific criteria to
e s t i ma t e t h e p r o b a b i l i t y o f d i a g n o s i n g G A B H S . O n e p o p u l a r s e t , t h e C e n t o r
c r i t e r i a2)( , i n c l u d e s t h e f o l l o w i n g : t o n s i l l a r e xu d a t e s , t e n d e r a n t e r i o r c e r v i c a l
l y mp h a d e n o p a t h y, a n d a b s e n c e o f c o u g h a n d h i s t o r y o f f e v e r. T h e p o s i t i v e
predictive value of three or four of these criteria is 40% to 60%. T he absence o
t h r e e o r f o u r o f t h e s e c r i t e r i a h a s a n e g a t i v e p r e d i c t i v e v2,4)
a l u .e Tohf e8 0 % (
following principles are put forth in these guidelines.
a. C l i n i c a l l y, s c r e e n a l l a d u l t p a t i e n t s w i t h p h a r y n g i t i s u s i n g t h e C e n t o r
criteria.
b. D o n o t t e s t o r t r e a t p a t i e n t s w i t h n o n e o r o n l y o n e o f t h e s e c r i t e r i a .
c. F o r p a t i e n t s w i t h t w o o r mo r e c r i t e r i a , t h e f o l l o w i n g o p t i o n s h a v e b e e n
defined.
i. T e s t p a t i e n t s w i t h t w o , t h r e e , o r f o u r o f t h e c r i t e r i a a n d t r e a t o n l y t h o s e
with positive R S AD Ts.
i i. T e s t p a t i e n t s w i t h t w o o r t h r e e c r i t e r i a a n d t r e a t t h o s e w i t h p o s i t i v e
R S AD Ts or those with four criteria.
i i i. D o n o t u s e a n y d i a g n o s t i c t e s t s a n d o n l y t r e a t p a t i e n t s w i t h t h r e e o r
four criteria.
d. D o n o t p e r f o r m f o l l o w - u p T C s o n p a t i e n t s w i t h n e g a t i v e R S A D T s w h e n t h e
sensitivity is >80%.
2. O t h e r s c o r i n g s y s t e ms h a v e a l s o b e e n d e v e l o p e d t h a t i n c o r p o r a t e t h e p a t i e n t ' s
a g e g e n e r a l i zi n g t h e u s e o f t h e s e t o c h i l d r e n a n5)d. aTdhui sl t ss y( s t e m g i v e s
P. 1 0 6
1 point for each of the Centor criteria present, and then adds 1 point for age
less than 15 years, 0 points for age 15 to 45 years, and -1 for age greater than
4 5 y e a r s . T h i s g u i d e l i n e r e c o mme n d s t h e f o l l o w i n g s t r a t e g y : 0 1 p o i n t s , n o
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tmdmss
6.5 - Pharyngitis
t r e a t me n t o r t e s t i n g ; 1 3 p o i n t s , t r e a t t h o s e w i t h p o s i t i v e R S A D T s ; 4 5 p o i n t s ,
t r e a t e mp i r i c a l l y.
3. T h e g o a l o f t h e s e s c o r i n g s y s t e ms i s t o h e l p r e d u c e t h e o v e r u s e o f a n t i b i o t i c s i n
t r e a t i n g p h a r y n g i t i s . S t u d i e s e s t i ma t e d t h a t c l i n i c a l s c r e e n i n g c o u l d d e c r e a s e
i n a p p r o p r i a t e a n t i b i o t i c u s e b y2,4)
8 8. % (
4. A l t h o u g h o b t a i n i n g R S A D T s a n d c o n f i r ma t o r y T C s i s s t i l l a n a p p r o p r i a t e o p t i o n ,
s i mp l y t r y i n g t o u s e c l i n i c a l j u d g me n t w i t h o u t a c l i n i c a l p r e d i c t i o n r u l e i s n o t .
C o n t i n u e d r e s e a r c h i n t h i s a r e a w i l l h e l p d e f i n e t h e mo s t a p p r o p r i a t e me t h o d o f
diagnosis.
T h e p r i ma r y ma n i f e s t a t i o n i s t h r o a t a n d a n t e r i o r n e c k p a i n a c c o mp a n i e d b y s o me
d e g r e e o f p a i n f u l s w a l l o w i n g . O t h e r s y mp t o ms , d e p e n d i n g o n t h e u n d e r l y i n g e t i o l o g
ma y i n c l u d e f e v e r, n a u s e a , v o mi t i n g , f a t i g u e , r a s h , e a r p a i n , a n d e v e n a b d o mi n a l
d i s c o mf o r t . S i g n s ma y r a n g e f r o m mi n i ma l o r n o p h a r y n g e a l e r y t h e ma a n d e xu d a t e t o
s e v e r e s u p p u r a t i v e f i n d i n g s , i n c l u d i n g ma r k e d c e r v i c a l a d e n o p a t h y. T o n s i l l a r
a b s c e s s f o r ma t i o n c a u s e s s w e l l i n g a n d d e v i a t i o n o f t h e s o f t p a l a t e w i t h ma l o d o r o u s
breath.
References
1 . Wo o d w e l l D A , C h e r r y D
NK
at. i onal ambul at or y medi c al c ar e :s ur
2 0v0ey
2
s u mma r y. H y a t t s v i l l e , M D : N a t i o n a l C e n t e r f o r H e a l t hASdv
t a tDi satt iac s ;
2004;346:144.
2 . C o o p e r R J , H o f f ma n J R , B a r t l e t t J G , e t a l . P r i n c i p l e s o f a p p r o p r i a t e a n t i b i o t i c
u s e f o r a c u t e p h a r y n g i t i s i n aAdnn
u l t sE. mer g M ed
2 0 0 1 ; 3 7 : 7 11 7 1 7 .
3 . B i s n o A L . A c u t e p h a r y n gNi t iEs .ngl J M ed2 0 0 1 ; 3 4 4 ( 3 ) : 2 0 5 2 11 .
4 . I n s t i t u t e f o r C l i n i c a l S y s t e ms I mp r o v e me n t ( I C S I ) . A c u t e p h a r y n g i t i s : c l i n i c a l
g u i d e l i n e , M a y 2 0 0 5 u p d a t e , a t h t t p : / / w w w. i c s i . o r g , a c c e s s e d o n J u l y 2 5 , 2 0 0 5 .
5 . Vi n c e n t M T , C e l e s t i n N , H u s s a i n A N . P h a rAym
n gFi tam
i s . P hy s i c i an
2004;69:14651470.
193 / 652
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6.6 - Rhinitis
> T a b l e o f C o n t e n t s > 6 - E a r, N o s e , a n d T h r o a t P r o b l e ms > 6 . 6 - R h i n i t i s
6.6
Rhinitis
Gail S. Marion
I . Background
N o u n i v e r s a l d e f i n i t i o n o r c l a s s i f i c a t i o n e xi s t s f o r r h i n i t i s a c o mmo n p r e s e n t i n g
s y mp t o m. R h i n i t i s i mp l i e s i n f l a mma t i o n o f t h e n a s a l mu c o s a b u t c a n a l s o r e f e r t o a
c o n s t e l l a t i o n o f r h i n o p a t h y s y mp t o ms , i n c l u d i n g mu c o u s d r a i n a g e , s t u f f i n e s s ,
s n e e zi n g , a n d i t c h i n g .
A. Etiology
1. A l l e r g i cS e a s o n a l , p e r e n n i a l ( 5 0 % o f c a s e s )
2. I n f e c t i o nA c u t e v e r s u s c h r o n i c , b a c t e r i a l v e r s u s v i r a l ( 2 0 % 4 0 % o f c a s e s )
3. N o n a l l e r g i c p e r e n n i a l r h i n
E iot si si n o p h i l i c , n o n e o s i n o p h i l i c
4. M i s c e l l a n e o uFso r e i g n b o d y, n a s a l p o l y p , s e p t a l d e v i a t i o n , n e o p l a s m, e n l a r g e d
a d e n o i d s , o r t o n s i l s , r e c e n t h e a d t r a u ma ( w i t h c e r e b r o s p i n a l f l u i d l e a k ) ,
a u t o n o mi c , v a s o mo t o r r h i n i t i s
5. D r u g - i n d u c e O
d r a l c o n t r a c e p t i v e s , h o r mo n e r e p l a c e me n t t h e r a p y,
a n t i h y p e r t e n s i v e s , o p h t h a l mi c - b l o c k e r s , l o c a l d e c o n g e s t a n t s ( r h i n i t i s
me d i c a me n t o s u m) , a s p i r i n a n d n o n s t e r o i d a l a n t i - i n f l a mma t o r y d r u g s , c o c a i n e
6. A t r o p h i c r h i n i tEi sxt e n s i v e s u r g e r y
P. 1 0 7
7. P h y s i c a l / c h e m i c a l e x p o s uOr cecsu p a t i o n a l , p o l l u t i o n , g u s t a t o r y, d r y a i r,
bright light
8. A s s o c i a t e d w i t h a s y s t e m i c d i s o
Hrydpeort h y r o i d i s m, p r e g n a n c y, a c q u i r e d
i mmu n o d e f i c i e n c y s y n d r o me , s y s t e mi c l u p u s , r h e u ma t o i d a r t h r i t i s , S j g r e n ' s
s y n d r o me , p r i ma r y mu c o u s , o r c i l i a r y d e f e c t , c y s t i c f i b r o s i s , a n t i b o d y d e f i c i e n c y
g r a n u l o ma t o u s d i s e a s e .
B. Epidemiology
R h i n i t i s c u r r e n t l y a f f e c t s a b o u t 4 0 mi l l i o n A me r i c a n s , w i t h a n n u a l c o s t s t o s o c i e t y o
mo r e t h a n $ 1 5 b i l l i o n d o l1,2)
l a r s. A
( l t h o u g h r h i n i t i s i s c o n s i d e r e d a mi n o r c o mp l a i n t
194 / 652
tmdmss
6.6 - Rhinitis
a n d i s f r e q u e n t l y l e f t u n t r e a t e d , p r o p e r e v a l u a t i o n a n d t r e a t me n t i mp r o v e s h e a l t h
o u t c o me s a n d t h e q u a l i t y o f l i f e f o r p3,4)
a t i e. nAt sn (i mp o r t a n t e xa mp l e i s t h e
i n c r e a s i n g e v i d e n c e t h a t a g g r e s s i v e a l l e r g i c r h i n i t i s ma n a g e me n t i mp r o v e s t h e
ma n a g e me n t o f a s t h ma b e c a u s e t h e n o s e s e r v e s a s a f i l t e r f o r3,5)
i n h. a l e d a i r (
R h i n i t i s i s a n i mp o r t a n t c a u s e o f mo r b i d i t y ; i t c a n c a u s e f a t i g u e , h e a d a c h e , c o g n i t i v
i mp a i r me n t , a n d o t h e r s y s t e mi c s y mp
4)t.o ms (
I I I . Evaluation
E v a l u a t i o n o f r h i n i t i s r e q u i r e s d e t e r mi n i n g w h e t h e r t h e s y mp t o ms a r e c a u s e d b y ( a )
a l l e r g y, ( b ) i n f e c t i o n , ( c ) a n a t o mi c d e f e c t , ( d ) s e r i o u s s y s t e mi c i l l n e s s , o r ( e ) s o me
c o mb i n a t i o n o f t h e s e .
A. History
W h a t a r e t h e s p e c i f i c s y mp t o ms ( i . e . , s t u f f i n e s s , i t c h i n g , c l e a r o r p u r u l e n t d r a i n a g e
A r e t h e s y mp t o ms u n i l a t e r a l o r b i l a t e r a l ? A l l e r g y a n d i n f e c t i o n p r o d u c e b i l a t e r a l n a s
c o mp l a i n t s , w h e r e a s mo s t r h i n i t i s r e l a t i n g t o t h e s t r u c t u r a l p r o b l e ms o f t h e n o s e i s
u n i l a t e r a l . W h e n d i d t h e s y mp t o m( s ) b e g i n ? A s k w h a t t h e p a t i e n t b e l i e v e s c a u s e s t h
s y mp t o ms . H o w o f t e n a n d w h e n d o t h e s y mp t o ms o c c u r ? D o t h e y p r e d o mi n a t e a t
c e r t a i n t i me s o f t h e y e a r ? W h a t o t h e r s y mp t o ms a r e a s s o c i a t e d ? W h a t ma k e s t h e
s y mp t o ms b e t t e r o r w o r s e ? A s s o c i a t e d c o mp l a i n t s ( e . g . , f r a n k f a t i g u e , i r r i t a b i l i t y,
d e p r e s s i o n , o r c h e s t s y mp t o ms ) t e n d t o p o i n t t o u n t r e a t e d a l l e r g i c c a u s e s , s y s t e mi c
disease, or drug-induced illness. Include questions about atopic disease, upper
r e s p i r a t o r y a l l e r g i e s , a s t h ma , n a s a l s u r g e r y, a n d p r e s c r i p t i o n me d i c a t i o n u s e .
A d d r e s s t o b a c c o ( p e r s o n a l o r s e c o n d a r y e xp o s u r e ) , a l c o h o l o r r e c r e a t i o n a l d r u g u s e
o v e r - t h e - c o u n t e r me d i c a t i o n , h e r b a l r e me d i e s , a n d p e t s i n t h e h o me . A r e t h e r e
s u s p e c t e d e n v i r o n me n t a l i r r i t a n t s ? I s t h e r e a f a mi l y h i s t o r y o f a l l e r g i e s o r o t h e r
r e l e v a n t s y s t e mi c d i s e a s e s ?
A general inspection of the patient frequently offers clues to the cause. Allergic
shiners (infraorbital, bluish discoloration of the skin) or a crease at the lower part
t h e n o s e f r o m r e p e a t e d r u b b i n g a r e c o mmo n p h y s i c a l f i n d i n g s o f a l l e r g i c r h i n i t i s .
E v a l u a t e v i t a l s i g n s ( e s p e c i a l l y t e mp e r a t u r e ) a n d t h e e a r s , n o s e , a n d t h r o a t ,
i n c l u d i n g a n e xa mi n a t i o n f o r l y mp h a d e n o p a t h y a n d t h y r o i d d i s e a s e . A c o mp e t e n t
e xa mi n a t i o n o f n a s a l p a s s a g e s r e q u i r e s a n a s a l s p e c u l u m ( a 4 5 mm e a r s p e c u l u m
on a handheld otoscope is acceptable for children), and a good light source.
Carefully place the nasal speculum vertically into each vestibule. Insert a handheld
o t o s c o p e l i g h t s o u r c e t h r o u g h t h e s p e c u l u m t o s u r v e y f o r n a s a l p a t e n c y, mu c o s a l
c o l o r ( p a l e , r e d , o r b l u i s h ) , t h e d e g r e e a n d t h e l o c a t i o n o f e d e ma , p r e s e n c e a n d t y p
o f n a s a l d r a i n a g e ( t h i n , c l e a r, t h i c k , p u r u l e n t , u n i l a t e r a l , o r b i l a t e r a l ) , a n a t o mi c
d e f o r mi t i e s ( b o n e s p u r s , s e p t a l d e v i a t i o n ) , a n d t h e p r e s e n c e o f p o l y p s o r o t h e r
ma s s e s . I f s w o l l e n n a s a l t u r b i n a t e s b l o c k t h e v i e w, a p p l y a s h o r t - a c t i n g
d e c o n g e s t a n t s p r a y, t h e n r e e xa mi n e i n 1 0 mi n u t e s . A n e v a l u a t i o n o f t h e p o s t e r i o r
p o r t i o n o f t h e n o s e i s o f t e n s u b o p t i ma l w i t h a n a s a l s p e c u l u m a n d l i g h t s o u r c e . A
f l e xi b l e n a s o p h a r y n g o s c o p e p e r mi t s t h e e xa mi n a t i o n o f t h e s t r u c t u r e s b e t w e e n t h e
n a s a l v e s t i b u l e a n d t h e l a1)
r y.n A
x s( s e s s t h e l u n g s a n d t h e s k i n f o r s i g n s o f a t o p i c
d i s e a s e ( w h e e zi n g o r e c ze ma ) . I f s y s t e mi c i l l n e s s i s s u g g e s t e d a f t e r f o c u s e d
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e xa mi n a t i o n , a t h o r o u g h mu l t i s y s t e m e xa m i s n e c e s s a r y.
C. Testing
T h e mo s t c o mmo n c a u s e s o f r h i n i t i s d o n o t r e q u i r e a d d i t i o n a l t e s t i n g t o i n i t i a t e
e f f e c t i v e t r e a t me n t . A mi c r o s c o p i c e xa mi n a t i o n o f n a s a l s e c r e t i o n s c a n b e p e r f o r me d
t o h e l p d e f i n e u n c e r t a i n a l l e r g i c o r b a c t e r i a l c a u s e s o f r h i n i t i s , a l t h o u g h mo s t p r i ma
care clinicians often leave these tests to an otolaryngologist because these are
u s u a l l y d o n e t o c l a r i f y l e s s c o mmo n c a u s e s o3)
f r. hRi nei ft ei sr r (a l i s i n d i c a t e d i f
d o u b t e xi s t s , i f s e r i o u s p a t h o l o g y i s s u s p e c t e d o r f o u n d , i f p h y s i c a l e xa mi n a t i o n i s
d i f f i c u l t s e c o n d a r y t o n a s a l o b s t r u c t i o n , o r i f t h e s y mp t o ms d o n o t
P. 1 0 8
i mp r o v e w i t h t r e a t me n t . I f a n a n a t o mi c a b n o r ma l i t y o r s i n u s p a t h o l o g y i s s u s p e c t e d ,
l i mi t e d c o mp u t e d t o mo g r a p h y o f t h e s i n u s e s i s r e c o1,2,5)
mme n. d e d (
I V. Diagnosis
T o d i s t i n g u i s h b e t w e e n a l l e r g i c a n d n o n a l l e r g i c r h i n i t i s , f o c u s o n t h e s y mp t o ms o f
s n e e zi n g , c l e a r d r a i n a g e , p o s t n a s a l d r i p , i t c h i n g , n a s a l c o n g e s t i o n , g e n e r a l i ze d s i n
p r e s s u r e , s p e c i f i c i r r i t a n t s o r a l l e r g e n s , a n d f a mi l y a n d p e r s o n a l h i s t o r y o f a t o p y a n
a l l e r g y. N e xt , c o n s i d e r s e a s o n a l , p e r e n n i a l , o r g e o g r a p h i c r e l a t i o n s h i p s . T h e
presence of blue or pale boggy turbinates with clear drainage suggests an allergic
p r o c e s s . A p h y s i c a l e xa mi n a t i o n s h o u l d c o n f i r m t h e p a t i e n t ' s s t o r y a n d h e l p i d e n t i f y
a n y a n a t o mi c d e f e c t s o r s y s t e mi c d i s e a s e . S e v e r a l f o l l o w - u p v i s i t s ma y b e n e c e s s a r
to assess, treat, and educate patients with allergic rhinitis and to confirm any need
f o r f u r t h e r e v a l u a t i o n o r t r e a t me n t b y a n o t o l a r y n g o l o g i s t 4)o.r a l l e r g i s t (
P u r u l e n t n a s a l d r a i n a g e i mp l i e s a n i n f e c t i o u s c a u s e , w h e r e a s c l e a r d i s c h a r g e
s u g g e s t s a n o n i n f e c t i o u s c a u s e . Vi r a l i n f e c t i o n p r o d u c e s w h i t i s h t o p a l e y e l l o w
d r a i n a g e w i t h a s s o c i a t e d s y mp t o ms o f g e n e r a l i ze d h e a d o r b o d y a c h e s , n a s a l
c o n g e s t i o n , a n d s n e e zi n g . B a c t e r i a l i n f e c t i o n r e s u l t s i n y e l l o w o r g r e e n d r a i n a g e w i
f o c a l s i n u s p a i n , u p p e r t e e t h c o mp l a i n t s , a n d p o s s i b l y f e v e r. L o o k f o r e d e ma t o u s ,
e r y t h e ma t o u s t u r b i n a t e s . O t h e r l e s s c o mmo n i n f e c t i o u s s o u r c e s ( f u n g a l o r p a r a s i t i c
s h o u l d b e s u s p e c t e d i f t r e a t me n t f a i l s o r i f t h e p a t i e n t h a s a s u g g e s t i v e me d i c a l o r
t r a v e l h i s t o r5)
y .(
References
1 . F o r n a d l e y J A . T h e s t u f f y n o s e a n d rM
h ied
n i t iCs l. i n N or t h A1m9 9 9 ; 8 3 : 2 11
224.
2 . B a c h e r t C . P e r s i s t e n t r h i n i t i s a l l e r g i c o r n o nAal l ler
e rgy
g i2c0?0 4 ; 5 9 : 11 1 5 .
3 . H a d l e y J A . E v a l u a t i o n a n d ma n a g e me n t o f a l l e r g iM
c ed
r h i nCiltii ns . N or t h A m
1999;83:1325.
196 / 652
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6.6 - Rhinitis
4 . D y k e w i c z M S , F i n e ma n S , S k o n e r D P, e t a l . D i a g n o s i s a n d ma n a g e me n t o f
r h i n i t i s : c o mp l e t e g u i d e l i n e s o f t h e j o i n t t a s k f o r c e o n p r a c t i c e p a r a me t e r s i n
a l l e r g y, a s t h ma a n d i mmu n oAl onn
g y.A l l er gy A s t hma I mmunol
1998;81:478518.
5 . B e n n i n g e r M A , A n o n J B , M a b r y R L . T h e me d i c a l ma n a g e me n t o f
r h i n o s i n u s i t iO
s .t ol ar y ngol H ead N ec k S1ur9 9g 7 ; 11 7 : S 4 1 S 4 9 .
197 / 652
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6.7 - Stomatitis
> T a b l e o f C o n t e n t s > 6 - E a r, N o s e , a n d T h r o a t P r o b l e ms > 6 . 7 - S t o ma t i t i s
6.7
Stomatitis
John G. Spangle r
I . Background
S t o ma t i t i s , g e n e r a l l y r e f e r r e d t o a s l e s i o n s i n t h e mo u t h , r e p r e s e n t s a b r o a d c a t e g o
o f o r a l mu c o s a l i n f e c t i o n s , i n f l a mma t o r y c o n d i t i o n s , a n d o t h e r o r a l l e s i o n s . B e c a u s e
o f p o s s i b l e ma l i g n a n c y, p e r s i s t e n t l e s i o n s r e q u i r e d e f i n i t i v e d i a g n o s i s .
A. Etiology
C a u s e s i n c l u d e t h e f o l l o w i n g : ( i ) p r e ma l i g n a n t o r ma l i g n a n t l e s i o n s t o t o b a c c o o r
a l c o h o l u s e ( l e u k o p l a k i a , e r y t h r o p l a s i a , a n d o r a l c a n c e r ) ; ( i i ) h u ma n
i mmu n o d e f i c i e n c y v i r u s ( H I V ) - r e l a t e d l e s i o n s ( e . g . , K a p o s i ' s s a r c o ma , o r a l h a i r y
l e u k o p l a k i a ) ; ( i i i ) i n f e c t i o n s t h a t ma y b e b a c t e r i a l ( e . g . , n e c r o t i zi n g u l c e r a t i v e
g i n g i v i t i s ) , v i r a l ( e . g . , h e r p e s s i mp l e x v i r u s ( H S V ) , h a n d - f o o t - a n d - mo u t h d i s e a s e ) , o
fungal (e.g., thrush, angular cheilitis); (iv) ulcerative and erosive conditions (e.g.,
r e c u r r e n t a p h t h o u s u l c e r s , B e h e t ' s d i s e a s e , a u t o i mmu n e d i s o r d e r s ) ; ( v ) t r a u ma t i c
a n d i r r i t a n t l e s i o n s ( c h r o n i c c h e e k b i t i n g , c h e mi c a l e xp o s u r e s , b u r n s f r o m h o t f o o d )
a n d ( v i ) d r u g - r e l a t e d e r u p t i o n s ( S t e v e n s - J o h n s o n s y n d r o me , c h e mo t h e r a p y a s s o c i a t e d mu c o s i t i s ) .
P. 1 0 9
B. Epidemiology
O r a l l e s i o n s a r e mo r e c o mmo n i n t h e a d u l t p o p u l a t i o n t h a n t e n s i o n h e a d a c h e s ,
p h l e b i t i s , o r a r t h r a l g i a s . I n a ma s s s c r e e n i n g o f mo r e t h a n 2 3 , 0 0 0 a d u l t s , t h e 3 0 mo
c o mmo n l e s i o n s a c c o u n t e d f o r 9 3 % o f a l l o r a l1)l .e s i o n s (
I I I . Evaluation
A. History
T he history should describe the lesion and the potential risk factors for its etiology
Describe the onset: was it abrupt, suggesting an infection, or insidious, suggesting
a n i n f l a mma t o r y o r n e o p l a s t i c o r i g i n ? A r e t h e r e a s s o c i a t e d s i g n s a n d s y mp t o ms ?
M a n y o r a l i n f e c t i o n s a r e a s s o c i a t e d w i t h p a i n , ma l a i s e , a n d f e v e r. B e h e t ' s e t ' s
d i s e a s e h a s a s s o c i a t e d o c u l a r a n d g e n i t a l l e s i o n s , w h e r e a s o t h e r a u t o i mmu n e
d i s e a s e s s u c h a s s y s t e mi c l u p u s e r y t h e ma t o s u s ( S L E ) o r u l c e r a t i v e c o l i t i s ma y h a v e
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6.7 - Stomatitis
s y s t e mi c s y mp t o ms
2,3)( . D e s c r i b e t h e l e s i o n s : a r e t h e y p a i n f u l o r p a i n l e s s ?
I n f e c t i o n s , i n f l a mma t o r y l e s i o n s , a n d a p h t h o u s u l c e r s a r e u s u3)a,l l y p a i n f u l (
w h e r e a s p r e ma l i g n a n t a n d ma l i g n a n t l e s i o n s ma y b e2,4)
p a.i nAl er es st h( e r e
v e s i c l e s o r b u l l a e ? P e mp h i g o i d a n d p e mp h i g u s ma y c a u s e b u l l a e a n d / o r u l c e r s . H S V
s t a r t s a s v e s i c u l a r l e s i o n s a n d t h e n u l c e r a t e s . Va r i c e l l a zo s t e r l e s i o n s c a n o c c u r i n
t h e mo u t h3,4)
( . Did vesicles precede the lesions suggesting H S V or was there
u l c e r a t i o n w i t h o u t v e s i c l e s , s u g g e s t i n g a p h t h o u3)s?uAl cr e r tsh (e l e s i o n s t h a t w i l l
n o t w i p e o f f t h e mu c o s a w h i t e ? L e u k o p l a k i a , a p r e ma l i g n a n t l e s i o n , i s w h i t e , a n d w i
n o t w i p e o f f . A n y c o e xi s t i n g r e d c o mp o n e ner
t , yct ahrl lopl
e d as,i ag r e a t l y i n c r e a s e s
t h e ma l i g n a n t p o t e n t i a l o f t h e l2,4)
e s i o. nL i(c h e n p l a n u s a l s o p r o d u c e s a s t r i a t e d
w h i t e l e s i o n , u s u a l l y o n t h e b u c c a l mu
3) .c oWs ha e(r e a r e t h e l e s i o n s ? H S V t e n d s t o
o c c u r o n p e r i o s t e a l l y b o u n d mu c o s a ( g i n g i v a , h a r d p a l a t e ) , w h e r e a s r e c u r r e n t
a p h t h o u s u l c e r s o c c u r o n n o n p e r i o s t e a l l y b o u n d mu c o s a ( b u c c a l , l i p , o r t o n g u e
mu c o s a )3)( . T h e f l o o r o f t h e mo u t h u n d e r t h e t o n g u e , t h e l a t e r a l a s p e c t s o f t h e
t o n g u e , t h e r e t r o mo l a r r e g i o n s , a n d t h e s o f t p a l a t e a r e w o r r i s o me a r e a s f o r
ma l i g n a n c y t o d e v e l4)
o p, b( u t ma l i g n a n c y c a n o c c u r a n y w h e r e .
P a s t me d i c a l h i s t o r y i s a l s o i mp o r t a n t . S y s t e mi c i n f l a mma t o r y c o n d i t i o n s s u c h a s
S L E or lichen planus can produce oral ulcerations. Recurrence suggests aphthous
u l c e r s a n d H S V. D e n t u r e s i n c r e a s e t h e s u s c e p t i b i l i t y t o d e n t u r e s t o ma t i t i s o r a n g u l a
c h e i l i t i s , b o t h c a u s e dC andi
b y das p e c i e s2,3,4)
(
. H IV increases the likelihood of
o r a l h a i r y l e u k o p l a k i a , K a p o s i ' s s a r c o ma , a n d s e v e r e o r a l c a n d i d i a s i s . E xp o s u r e t o
i n d i v i d u a l s w i t h s i mi l a r s y mp t o ms s u g g e s t s e n t e r o v i r a l i n f e c t i o n s s u c h a s h e r p a n g i n
a n d h a n d - f o o t - a n d - mo u t h d i s e a s e . M e d i c a t i o n s s u c h a s s u l f o n a mi d e s a n d ma n y
o t h e r d r u g s c a n c a u s e S t e v e n s - J o h n s o n s y n d r o me , w h e r e a s c h e mo t h e r a p y f o r t h e
t r e a t me n t o f c a n c e r c a n p r o d u c e s e v e r e mu c o s i t i s . S o c i a l h i s t o r y s h o u l d f o c u s o n
a l c o h o l a n d t o b a c c o u s e , e xp o s u r e s t o o r a l i r r i t a n t s , a n d s e xu a l a c t i v i t y, i n c l u d i n g
o r a l - g e n i t a l s e xu a l c o n t a c t .
1. H e a d , e y e s , e a r s , n o s e , t h r o a t ( H EBEa N
s eTd) o n t h e h i s t o r y, a f o c u s e d
p h y s i c a l e xa mi n a t i o n o f t h e H E E N T i s n e c e s s a r y. L o o k f o r s i g n s o f t r a u ma .
E xa mi n e t h e c o n j u n c t i v a a n d n a s a l mu c o s a f o r i n f l a mma t o r y c h a n g e s o r
u l c e r a t i o n s . E v a l u a t e t h e p a t i e n t f o r c o e xi s t i n g u p p e r r e s p i r a t o r y s i g n s a n d
s y mp t o ms s u c h a s r h i n o r r h e a , s i n u s t e n d e r n e s s t o p a l p a t i o n , a n d o t i t i s me d i a .
I n s p e c t f a c i a l s k i n f o r v e s i c l e s f r o m H S V o r v a r i c e l l a zo s t e r o r o t h e r l e s i o n s s u c
a s e c c h y mo s e s , a ma l a r r a s h , o r a v i r a l e xa n t h e m. L o o k f o r f a c i a l a s y mme t r y.
Va r i c e l l a zo s t e r c a n c a u s e f a c i a l n e r v e p a r a l y s i s , Rcams
a l l e ey
d t hHeunt
s y ndr ome
. E v a l u a t e p r e a u r i c u l a r, p o s t a u r i c u l a r, a n d c e r v i c a l l y mp h n o d e c h a i n s .
F i n a l l y, e v a l u a t e t h e o r a l c a v i t y, d o c u me n t i n g t h e s i ze , l o c a t i o n , a n d a p p e a r a n c
of the lesion.
2. A d d i t i o n a l p h y s i c a l e x a m i n aBt ai os ne d o n t h e r e s u l t s o f t h e H E E N T
e xa mi n a t i o n , a d d i t i o n a l p h y s i c a l e xa mi n a t i o n mi g h t i n c l u d e : ( i ) p u l mo n a r y
e xa mi n a t i o n f o r v i r a l p n e u mo n i t i s o r f i n d i n g s i n a u t o i mmu n e d i s e a s e s ; ( i i )
a b d o mi n a l a n d r e c t a l e xa mi n a t i o n f o r C r o h n ' s d i s e a s e o r u l c e r a t i v e c o l i t i s ; ( i i i )
g e n i t o u r i n a r y e xa mi n a t i o n f o r mu c o s a l u l c e r s i n B e h e t ' s d i s e a s e a n d S t e v e n s J o h n s o n s y n d r o me , a s w e l l a s f o r s i g n s o f s y p h i l i s o r g o n o r r h e a ; ( i v ) a s k i n
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6.7 - Stomatitis
e xa mi n a t i o n l o o k i n g f o r v i r a l e xa n t h e ms , d r u g e r u p t i o n s , l i c h e n p l a n u s ,
p e mp h i g u s , p e mp h i g o i d , a n d S L E ; a n d ( v ) a mu s c u l o s k e l e t a l e xa mi n a t i o n f o r
s i g n s o f S L E , R e i t e r ' s s y n d r o me , o r o t h e r a u t o i mmu n e d i s e a s e s .
P. 11 0
C. Testing
1. C l i n i c a l l a b o r a t o r y t e s t i n g s h o u l d b e g u i d e d b y t h e h i s t o r y a n d p h y s i c a l f i n d i n g s
A p o t a s s i u m h y d r o xi d e w e t mo u n t i s u s e f u l i n t h e d i a g n o s i s o f c a n d i d i a s i s . Vi r a l
and bacterial cultures can be obtained from swabs of oral lesions, but viral
c u l t u r e s a r e u s u a l l y mo r e h e l p f u l t h a n b a c t e r i a l c u l t u r e s . D a r k f i e l d mi c r o s c o p y
c a n b e p e r f o r me d f r o m s w a b s o f s y p h i l i s c h a n c r e s o r p l a q u e s . C y t o l o g i c
s c r a p i n g s o f p r e ma l i g n a n t o r ma l i g n a n t l e s i o n s , p r e p a r e d i n a ma n n e r s i mi l a r t o
a P a p a n i c o l a o u s me a r, a r e n o t a s u b s t i t u t e f o r a b i o p s y o f s u s p e c t e d o r a l
n e o p l a s i a2,3,4)
(
. A n e w b r u s h h a s b e e n d e v e l o p e d t o s a mp l e mu c o s a l l e s i o n s
d o w n t o t h e b a s e me n t me mb r a n e ( O r a l C D x, C D x L a b o r a t o r i e s , S u f f e r n , N e w
Yo r k ) .
2. D i a g n o s t i c i ma g i n g i s i n d i c a t e d o n l y i n s e l e c t e d c a s e s s u c h a s c o e xi s t i n g s i n u s
d i s e a s e ( mi n i s i n u s c o mp u t e d t o mo g r a p h y [ C T ] s c a n ) , c o e xi s t i n g n e c k ma s s o r
l y mp h a d e n o p a t h y s u s p i c i o u s f o r ma l i g n a n t d i s e a s e ( h e a d a n d n e c k C T s c a n ) ,
s u s p e c t e d me t a s t a t i c d i s e a s e ( c h e s t x- r a y, C T s c a n o f t h e h e a d , a b d o me n , a n d
c h e s t ) , o r t r a u ma ( c e r v i c a l s p i n e s e r i e s , c r a n i a l C T s c a n , d e n t a l P a n o r e x f i l ms ) .
I f H S V i s s u s p e c t e d , c r a n i a l ma g n e t i c r e s o n a n c e i ma g i n g ( M R I ) ma y b e u s e f u l .
A c h e s t x- r a y i s i n d i c a t e d i n s u s p e c t e d v i r a l o r a u t o i mmu n e p n e u mo n i t i s o r i n
s e c o n d a r y b a c t e r i a l p n e u mo n i a . I f a s e v e r e l i p l a c e r a t i o n h a s o c c u r r e d , p l a i n
f i l ms c a n h e l p r u l e o u t ma n d i b u l a r c o n d y l a r f r a c t u r e s o r t o o t h f r a c t u r e s .
D. Genetics
ma y p l a y a r o l e i n t h e s u s c e p t i b i l i t y t o a p h t h o u s u l c e r s , a n d a u t o i mmu n e d i s o r d e r s .
I V. Diagnosis
o f s t o ma t i t i s i n c l u d e s l e s i o n s i n t h e s i x c a t e g o r i e s l i s t eIdI . A
i n. Ts he ec t i o n
d i a g n o s i s d e p e n d s o n t h e s y n t h e s i s o f t h e k e y h i s t o r i c a l a n d p h y s i c a l e xa mi n a t i o n s ,
a s w e l l a s t h e l a b o r a t o r y a n d i ma g i n g e lAe lme
l onrtas l. u l c e r s t h a t d o n o t h e a l , a s
w e ll as w hite or re ddish-w hite le sions that do not re solv e in 2 w e e ks, ne e d a
b i o p s y t o r u l e o u t m a l i g n a2,3,4).
ncy (
S t o ma t i t i s ma n i f e s t s a s l u mp s , u l c e r a t i o n s , o r d i s c o l o r e d p a t c h e s t h a t ma y b e
p a i n f u l , o r p a i n l e s s d e p e n d i n g o n t h e u n d e r l y i n g p a t h o l o g y.
References
1 . B o u q u o t J E . C o mmo n o r a l l e s i o n s f o u n d d u r i n g a ma s s s c r e e n i n g e xa mi n a t i o n .
200 / 652
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6.7 - Stomatitis
J A m D ent A s s oc
1 9 8 6 ; 11 2 ( 1 ) : 5 0 5 7 .
2 . S i l v e r ma n SO. r al c anc er
, 4 t h e d n . H a mi l t o n , O H : B C D e c k e r, 1 9 9 8 .
3 . B r u c e A J , R o g e r s R S , I I I . A c u t e o r a l Duer
l c emat
r s . ol C l i2n0 0 3 ; 2 1 : 1 1 5 .
4 . P o r t e r S R , L e a o J C . R e v i e w a r t i c l e : o r a l u l c e r s a n d i t s r e l e v a n c e t o s y s t e mi c
d i s o r d e r sA. l i ment P har mac ol T her
2005;21:295306.
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> T a b l e o f C o n t e n t s > 6 - E a r, N o s e , a n d T h r o a t P r o b l e ms > 6 . 8 - T i n n i t u s
6.8
Tinnitus
Sara Ne al
I . Background
Tinnitus has been described as ear noise and consists of sounds heard by the
p a t i e n t w i t h n o s o u n d s o u r c e e xt e r n a l t o t h e b o d y. A l t h o u g h t h e r e a r e i n f i n i t e
v a r i a t i o n s , p a t i e n t s u s u a l l y d e s c r i b e r i n g i n g , b u zzi n g , c l i c k i n g , w h i s t l i n g , c r i c k e t - l i k
h i s s i n g , o r h u mmi n g s o u 1)
n d. s (
A. Etiology
T i n n i t u s i s n o t a d i s e a s e b u t a n i n d i c a t i o n o f s o me o t h e r o n g o i n g p r o c e s s , w h e t h e r
p a t h o l o g i c o r b e n i g n . T h e f i r s t s t e p i n d e t e r mi n i n g t h e e t i o l o g y i s t o d e f i n e t h e t y p e
of sound heard and any relationship to the pulse or respiration. T hese answers
s u b d i v i d e t i n n i t u s i n t o t w o g e n e r a l c a t e g o r i e s o b j e c t i v e a n d1,2,3)
s u b.j e c t i v e (
P. 111
S u b j e c t i v e t i n n i t u s , w h i c h i s f a r mo r e c o mmo n , i s h e a r d o n l y b y t h e p a t i e n t . O b j e c t i
tinnitus can be heard through a stethoscope placed over the head and neck
s t r u c t u r e s n e a r t h e p a t i e n t ' s3) e. a r (
1. O b j e c t i v e ( v i b r a t o r y , p s e u d o t i n nT iht eusse) s o u n d s a r e o f t e n s y n c h r o n o u s
w i t h t h e p u l s e . T h e y a r e r e a l s o u n d s , me c h a n i c a l i n o r i g i n , a n d c a n b e h e a r d b y
t h e e xa mi n e r a s w e l l a s t h e p a t i e n t . S o u r c e s , e i t h e r v a s c u l a r o r mu s c u l a r,
include:
a. V e n o u s h u mi s d u e t o e d d y c u r r e n t s i n t h e j u g u l a r v e i n . T h e p a t i e n t i s
mo r e a w a r e o f t h e n o i s e w h e n t h e r e i s a c o n c u r r e n t c o n d u c t i v e h e a r i n g
loss.
b. A r t e r i o v e n o u s m a l f o r m a t icoanns b e f o u n d b e t w e e n t h e o c c i p i t a l a r t e r y
and the transverse sinus.
c. M y o c l o n u so f n e a r b y s t r u c t u r e s , mo s t c o mmo n l y t h e p a l a t e , t h e s t a p e d i u s
mu s c l e , a n d t h e t e n s o r t y mp a n i . T h e s e a r e u s u a l l y a s y n c h r o n o u s w i t h t h e
pulse.
d. A p a t u l o u s o r a b n o r m a l l y p a t e n t e u s t a c h i ac an nt uc ba ue s e c h r o n i c o r
i n t e r mi t t e n t t i n n i t u s . S o me t i me s r e l a t e d t o r e s p i r a t i o n s , i t i s f r e q u e n t l y
described as a hissing sound.
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2. S u b j e c t i v e ( n o n v i b r a t oTrhyi)s t y p e i s mo r e c o mmo n , b u t h a s a l e s s e a s i l y
definable etiology than vibratory types. Its causes can be divided into various
s u b c a t e g o r i e 1,3)
s ( .
a. O t o l o g i cN o i s e - i n d u c e d h e a r i n g l o s s , p r e s b y c u s i s , o t o s c l e r o s i s , o t i t i s ,
i mp a c t e d c e r u me n , s u d d e n d e a f n e s s , M n i r e ' s d i s e a s e .
b. N e u r o l o g i cH e a d i n j u r y, w h i p l a s h , mu l t i p l e s c l e r o s i s , a c o u s t i c n e u r o ma ,
a n d o t h e r c e r e b e l l o p o n t i n e a n g l e t u mo r s .
c. I n f e c t i o u sO t i t i s me d i a , s e q u e l a e o f L y me d i s e a s e , me n i n g i t i s , s y p h i l i s .
d. D r u g - r e l a t e S
d e es e c t i o nI I I . A . .5
e. M e t a b o l i cT h y r o i d d i s e a s e , h y p e r l i p i d e mi a , v i1t a2 mi
d enf i B
c i e n c y, zi n c
d e f i c i e n c y.
f. P s y c h o g e n i D
c e p r e s s i o n , a n xi e t y, f i b r o my a l g i a .
g. O t h e r T e mp o r o ma n d i b u l a r j o i n t d y s f u n c t i o n , o t h e r ma n d i b u l o d e n t a l
disorders.
B. Epidemiology
I n t h e U n i t e d S t a t e s , a p p r o xi ma t e l y 9 % o f i n d i v i d u a l s o l d e r t h a n 6 5 y e a r s o f a g e a n
1 % o f i n d i v i d u a l s y o u n g e r t h a n 4 5 y e a r s o f a g e e xp e r i e n4)
c e. W
t i nhni ti et uss a(r e
mo r e f r e q u e n t l y a f f e c t e d t h a n b l a c k s , a n d t h e p r e v a l e n c e i n t h e S o u t h i s a l mo s t t w i
t h a t i n t h e N o r t h e a4)s .t A
( p p r o xi ma t e l y o n e - f o u r t h o f p a t i e n t s w h o e xp e r i e n c e
t i n n i t u s h a v e s i g n i f i c a n t s y mp t o ms t h a t i mp a c t t h e i r l i f e , e s p e c i a l l y b e c a u s e t h e
c o n d i t i o n i s s o f r e q u e n t l y a s s o c i a t e d w i t h h e a1)
r i n. gS pl oescsi a(l a t t e n t i o n s h o u l d
be paid to patients presenting with unilateral tinnitus, associated vertigo, unilatera
s e n s o r i n e u r a l h e a r i n g l o s s , a n d e y e i n f l a mma t i o n .
I I I . Evaluation
A. History
I mp o r t a n t f e a t u r e s o f t h e h i s t o r y s h o u l d 1,3,5)
i n c l u:d e (
1. D a t e o f t i n n i t u s o n sp ea rt t i c u l a r l y a n y r e l a t i o n t o a n i l l n e s s o r c h a n g e i n d r u g
r e g i me n .
2. A d e t a i l e d d e s c r i p t i o n o f t h e t i nma
n i yt uhse l p s u b d i v i d e i t i n t o o b j e c t i v e a n d
s u b j e c t i v e s o u r c e s . A r e t h e r e a n y e xa c e r b a t i n g o r a me l i o r a t i n g f a c t o r s ? A n
association with respirations or pulse points to a vibratory source. Positional
change, such as lowering the head between the knees (causing venous
e n g o r g e me n t ) c a n p o i n t t o w a r d a p a t u l o u s e u s t a c h i a n t u b e a s t h e me c h a n i s m f o
t h e t i n n i t u s . Va r i a t i o n w i t h r e s p i r a t i o n o r d i s t o r t i o n s o f o n e ' s o w n v o i c e ma y a l s o
indicate the eustachian tube. Is the sound constant or episodic, unilateral or
b i l a t e r a l ? Wa s t h e o n s e t s u d d e n o r g r a d u a l ? W h a t a r e t h e p i t c h a n d l o u d n e s s o f
the sound? Is there associated hearing loss, vertigo, or pain? Is there evidence
o f o t h e r c o n d i t i o n s (sseecet i o nI I . A. ) t h a t a r e a s s o c i a t e d w i t h t i n n i t u s ? W h a t
else affects tinnitusbackground noise, alcohol, stress, or sleeplessness? Is
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t h e r e a h i s t o r y o f e xp o s u r e t o l o u d n o i s e , e a r i n f e c t i o n s , o t o l o g i c s u r g e r y, h e a d
i n j u r i e s , a n d u s e o f o t o t o xi c d r u g s ? A r e t h e r e a n y a d v e r s e e f f e c t s o f t h e
tinnitus? How does tinnitus affect daily life and the ability to function?
3. F l u c t u a t i o n o f s y m p t oFml usc t u a t i n g s y mp t o ms a r e c o mmo n l y a s s o c i a t e d w i t h
M n i r e ' s d i s e a s e ( e n d o l y mp h a t i c h y d r o p s ) .
P. 11 2
4. H i s t o r y o f n o i s e e x p o s u r e o r h e a r i nN
g olios ses- i n d u c e d h e a r i n g l o s s
usually causes high-pitched tinnitus, whereas Mnire's disease usually
p r o d u c e s a l o w e r p i t c h e d s o u n d . A l s o , c o n d u c t i v e h e a r i n g l o s s d u e t o c e r u me n
i mp a c t i o n , o t i t i s me d i a , o r o t o s c l e r o s i s ma y h e i g h t e n a w a r e n e s s o f i n t e r n a l
v i b r a t o r y s o u n d s s u c h a s a v e n o u s h u m o r my o c l o n u s . P r e s b y c u s i s , o r
d e g e n e r a t i o n w i t h i n t h e o r g a n o f C o r t i , i s f r e q u e n t l y s e e n i n t h e e l d e r l y. I t i s
associated with a downsloping high frequency hearing loss and a high-pitched
tinnitus.
5. M e d i c a t i o n h i s t oM
r ya n y d r u g s h a v e b e e n a s s o c i a t e d w i t h t1,3)
i n n.i t T
u sh e(
l i s t i n c l u d e s s a l i c y l a t e s , c a f f e i n e , a mi n o g l y c o s i d e s , a l c o h o l , q u i n i d i n e ,
n o n s t e r o i d a l a n t i - i n f l a mma t o r y d r u g s , c a r b a ma ze p i n e , l o o p d i u r e t i c s , c i s p l a t i n ,
v i n c r i s t i n e , l e v o d o p a , p r o p r a n o l o l , a n d a mi n o p h y l l i n e . S o me h o r mo n a l
preparations have also been indicated as has the postpartum state.
6. S i g n i f i c a n t w e i g h t l oc as ns b e a s s o c i a t e d w i t h a p a t u l o u s e u s t a c h i a n t u b e .
7. C o n c u r r e n t m e d i c a l c o n d i t ti o nbse c o n s i d e r e d i n c l u d e h y p e r t e n s i o n ,
d i a b e t e s me l l i t u s , t h y r o i d d i s o r d e r s , h y p e r l i p i d e mi a , a n d i n f e c t i o n . A r t e r i o v e n o u
s o u n d s a r e h e i g h t e n e d b y i n c r e a s e d c a r d i a c o u t p u t . Va s c u l a r d i s e a s e c a n l e a d
t o i s c h e mi a o f t h e a u d i t o r y o r g a n s , i n c l u d i n g t h e c o r t e x. N e u r a l i mp u l s e s c a n b e
affected by neuropathic conditions such as diabetes.
8. P s y c h i a t r i c d i s t u r b a n c ae ns a f f e c t s o u n d p e r c e p t i o n . A n xi e t y o r d e p r e s s i v e
d i s o r d e r s ma y h e i g h t e n t h e a w a r e n e s s o f i n t e r n a l a u d i t o r y s o u n d s . I n t u r n ,
t i n n i t u s ma y e xa c e r b a t e t h e s e u n d e r l y i n g c o n d i t i o n s a s w e l l . A u d i t o r y
h a l l u c i n a t i o n s mu s t b e r u l e d o u t b y h i s t o r y.
T h i s s h o u l d f o c u s o n t h e h e a d , e a r s , e y e s , n o s e , t h r o a t , c a r d i o v a s c u l a r, a n d
n e u r o l o g i c s y s t e ms . A s s e s s v i t a l s i g n s a n d p e r f o r m a c o mp l e t e e a r e xa mi n a t i o n ,
i n c l u d i n g a n e v a l u a t i o n f o r t h e o b s t r u c t i o n o f t h e e xt e r n a l a u d i t o r y c a n a l . L o o k f o r
t y mp a n i c me mb r a n e l a n d ma r k s , t y mp a n i c p u l s a t i o n s , a n d s i g n s o f t u mo r. A u s c u l t a t e
t h e e xt e r n a l a u d i t o r y c a n a l f o r t r a n s mi t t e d s o u n d s a n d u s e t u n i n g f o r k s t o a s s e s s a i
a n d b o n e c o n d u c t i o n ( We b e r a n d R i n n e t e s t i n g ) . O b s e r v e t h e n e c k f o r t h y r o i d
ma s s e s a n d a u s c u l t a t e f o r t h y r o i d o r c a r o t i d b r u i t s . E v a l u a t e e xt r a o c u l a r mo v e me n t s
s p e e c h d i s c r i mi n a t i o n , a n d t h e i n t e g r i t y o f t h e c e n t r a l n e r v o u s s y s t e m ( g a i t ,
e q u i l i b r i u m, s e n s a t i o n ) . I f a p p r o p r i a t e , i n c l u d e a n e v a l u a t i o n o f mo o d , a f f e c t , a n d
perception (e.g., hallucinations).
C. Testing
M o s t p a t i e n t s w i t h t i n n i t u s d o n o t n e e d b l o o d o r u r i1,3,5)
n e t e.s tI fi nign d( i c a t e d b y
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h i s t o r y a n d p h y s i c a l e xa mi n a t i o n , c o n s i d e r t h y r o i d f u n c t i o n t e s t s , e l e c t r o l y t e s , l i p i d
s e d i me n t a t i o n r a t e , t o xi c o l o g y, s y p h i l i s s e r o l o g y, a n d r h e u ma t o l o g5)i c. As nc r e e n i n g (
a u d i o g r a m t o a s s e s s s e n s o r i n e u r a l h e a r i n g l o s s s h o u l d a l w a y s b e p e r f o r me d . A
t y mp a n o g r a m ma y r e v e a l p u l s a t i o n s c o n s i s t e n t w i t h t h e h e a r t r h y t h m o r r e s p i r a t i o n s
P l a i n r a d i o g r a p h s a r e r a r e l y u s e f u l . A n e v a l u a t i o n f o r n e o p l a s m, e s p e c i a l l y a n
a c o u s t i c n e u r o ma , i s b e s t p e r f o r me d w i t h ma g n e t i c r e s o n a n c e i ma g i n g , w h i c h a l s o
d e l i n e a t e s e i g h t h n e r v e l e s i o n s a n d c o r t e x d a ma g e . A u d i t o r y b r a i n s t e m- e v o k e d
r e s p o n s e s c a n b e h e l p f u l a s w e l l , e s p e c i a l l y i n c o n s i d e r a t i o n o f c5)
o r. t A
e xn l e s i o n s (
a n g i o g r a m ma y b e n e c e s s a r y t o e xa mi n e v a s c u l a t u r e n e a r t h e i n n e r e a r.
D. Genetics
T o d a t e t h e r e i s n o e v i d e n c e t h a t t i n n i t u s h a s a g e n e t i c o r f a mi l i a l c a u s e .
I V. Diagnosis
T h e k e y t o t h e d i a g n o s i s o f t i n n i t u s i s d e t e r mi n i n g i f i t i s o b j e c t i v e o r s u b j e c t i v e .
O b j e c t i v e t i n n i t u s s h o u l d p r o mp t a s e a r c h f o r a s t r u c t u r a l s o u r c e o f t h e s o u n d .
C o n s i d e r v a s c u l a r c o mp l e xe s a n d mu s c u l a r c o mp o n e n t s c l o s e t o t h e i n n e r e a r a n d
t h e e u s t a c h i a n t u b e . S u b j e c t i v e t i n n i t u s , a l t h o u g h mo r e c o mmo n , h a s a l e s s e a s i l y
d i s c e r n e d e t i o l o g y i n mo s t c a s e s . D r u g e f f e c t s a n d h e a r i n g l o s s a r e t h e mo s t
c o mmo n s o u r c e s . A l t e r e d me t a b o l i c s t a t e s s u c h a s d i a b e t e s , h y p e r t h y r o i d i s m, o r
infection should then be considered. Evaluate for neurologic contributions, includin
a c o u s t i c n e u r o ma , d a ma g e t o t h e o r g a n o f C o r t i , o r a b r a i n l e s i o n . A l w a y s k e e p i n
mi n d t h e p o s s i b i l i t y o f p s y c h i a t r i c c a u s e s .
B e c a u s e o n l y t h e p a t i e n t h e a r s t i n n i t u s , t h e r e a r e n o o u t w a r d ma n i f e s t a t i o n s n o t e d
b y o t h e r s . T h e i mp a c t o f t i n n i t u s c a n b e s i g n i f i c a n t , h o w e v e r. A p p r o xi ma t e l y o n e f o u r t h o f t i n n i t u s s u f f e r e r s r e p o r t a n i n c r e a s e i n s y mp t o ms o v e r t i me .
P. 11 3
References
1 . L o c k w o o d A A . T i n n i tNu sE. ngl J M ed2 0 0 2 ; 3 4 7 ( 1 2 ) : 9 0 4 9 1 0 .
2 . M o l l e r A R . P a t h o p h y s i o l o g y o f t i nOntiol
t uar
s . y ngol C l i n N or t h A m
2003;36(2):249266.
3 . C r u mme r R W, H a s s a n G A . D i a g n o s t i c a p p r o a c h t oA tmi n F
n iam
t u s .P hy s i c i an
2004;69(1):120.
4 . A d a ms P F, H e n d e r s o t G E , M a r a n o M A . N a t i o n a l C e n t e r f o r H e a l t h S t a t i s t i c s .
C u r r e n t e s t i ma t e s f r o m t h e N a t i o n a l H e a l t h I n t e r v i eVwt als uHr eal
v e y.t h S t at
1999;200:81.
5 . S c h w a b e r M K . M e d i c a l e v a l u a t i o n o f t iOntnoli t ar
u sy. ngol C l i n N or t h A m
205 / 652
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6.8 - Tinnitus
2003;36(2):287292.
206 / 652
tmdmss
6.9 - Vertigo
> T a b l e o f C o n t e n t s > 6 - E a r, N o s e , a n d T h r o a t P r o b l e ms > 6 . 9 - Ve r t i g o
6.9
Vertigo
Frank S. Ce le stino
I . Background
A.
T r u e v e r t i g o i s c h a r a c t e r i ze d b y t h e i l l u s i o n o f mo v e me n t a f e e l i n g t h a t t h e b o d y o
e n v i r o n me n t i s mo v i n g . P a t i e n t s o f t e n r e p o r t r o t a t i o n o r s p i n n i n g , a l t h o u g h
occasionally they describe a sensation of linear acceleration or tilting. T his
s e n s a t i o n o f t e n b e g i n s a b r u p t l y a n d , i f s e v e r e , i t i s a c c o mp a n i e d b y n a u s e a ,
v o mi t i n g , a n d s t a g g e r i n g 1)
g a. i Ve
t ( r t i g o r e p r e s e n t s o n e o f t h e f o u r ma j o r s y mp t o m
c a t e g o r i e s t h a t a c c o u n t f o r p a t i e n t s ' c o mp l a i n t s o f 2)d i (C
zzihnaepst es r ( 2 ). 2. T h e
other three categories are:
1. P r e s y n c o p eT h i s i s a s e n s a t i o n o f i mp e n d i n g f a i n t ( s e v e r e l i g h t h e a d e d n e s s )
a n d i mp l i e s a t e mp o r a r y d e c r e me n t o f c e r e b r a l c i r c u l a t i o n . C o mmo n c a u s e s
i n c l u d e p o s t u r a l h y p o t e n s i o n , v a s o v a g a l r e a c t i o n s , c a r d i a c a r r h y t h mi a s ,
i mp a i r e d c a r d i a c o u t p u t , a n d h y p o g l y c e mi a .
2. D i s e q u i l i b r i u m
T h i s i s a f e e l i n g o f u n s t e a d i n e s s o f g a i t o r i mb a l a n c e i n t h e
a b s e n c e o f a n y a b n o r ma l h e a d s e n s a t i o n i n d i c a t i n g a d i s t u r b a n c e o f t h e mo t o r
c o n t r o l s y s t e m. C a u s e s i n c l u d e a l c o h o l i s m, d r u g s , c e r v i c a l f a c e t j o i n t
a r t h r o p a t h y, mu l t i p l e s c l e r o s i s , a n d mu l t i p l e n e u r o s e n s o r y d e f i c i t s ( e . g . , a
c o mb i n a t i o n o f v i s u a l i mp a i r me n t , v e s t i b u l a r h y p o f u n c t i o n , p e r i p h e r a l
n e u r o p a t h y, a n d me d i c a t i o n s ) .
3. O t h e r o r a t y p i cPaal t i e n t s o f t e n d e s c r i b e a v a g u e o r mi l d w o o zi n e s s ,
l i g h t h e a d e d n e s s , o r h e a v y h e a d e d n e s s , o r a s w i mmi n g o r f l o a t i n g s e n s a t i o n .
T his category has a high association with psychologic disturbances such as
a n xi e t y, d e p r e s s i o n , a n d p2)
a n. i c (
B.
C a r e f u l i n t e r v i e w i n g a l l o w s t h e p l a c e me n t o f p a t i e n t c o mp l a i n t s i n t o o n e o f t h e f o u r
c a t e g o r i e s , e a c h i mp l y i n g c e r t a i n p a t h o p h y s i o l o g i c me c h a n i s ms a n d , t h e r e f o r e ,
s p e c i f i c d i f f e r e n t i a l d i a g n o1,2,3)
s e s .( T h e r e ma i n d e r o f t h i s c h a p t e r w i l l p r i ma r i l y
f o c u s o n e v a l u a t i n g p a t i e n t s w i t h t h e c o mp l e x s y mp t o m o f t r u e v e r t i g o i n a n e f f o r t t o
d e t e r mi n e s p e c i f i c u n d e r l y i n g c a u s e s .
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6.9 - Vertigo
A. Etiology
T h e v e s t i b u l a r s y s t e m mo n i t o r s t h e mo t i o n a n d p o s i t i o n o f t h e h e a d i n s p a c e b y
detecting angular and linear acceleration. T he sensory receptors in the utricle and
s a c c u l e d e t e c t l i n e a r a c c e l e r a t i o n , a n d i t i s t h e c r i s t a e o f t h e s e mi c i r c u l a r c a n a l s t h
d e t e c t a n g u l a r a c c e l e r a t i o n ( h e a d t u r n i n g ) . I n f o r ma t i o n f r o m t h e p e r i p h e r a l r e c e p t o r
is relayed through the vestibular portion of the eighth cranial nerves to the brain
s t e m n u c l e i , a n d p o r t i o n s o f t h e c e r e b e l l u m. A d d i t i o n a l i mp o r t a n t c o n n e c t i o n s a r e
ma d e w i t h t h e o c u l a r mo t o r n u c l e i a n d s p i n a l c o r d . T r u e v e r t i g o r e p r e s e n t s a n
a s y mme t r y o r i mb a l a n c e o f n e u r a l a c t i v i t y b e t w e e n t h e l e f t a n d r i g h t v e s t i b u l a r
n u c l e i . A b n o r ma l a c t i v i t y i n t h i s s y s t e m c a n a r i s e e i t h e r f r o m
P. 11 4
peripheral lesions (labyrinth or vestibular nerve) or from central lesions (brain stem
o r c e r e b e l l u m) .
B. Epidemiology
T h e o v e r a r c h i n g c o mp l a i n t o f d i zzi n e s s a c c o u n t s f o r 1 % t o 2 % o f a l l o f f i c e v i s i t s ,
7% of visits by patients older than 80 years of age, and 20% to 25% of all nonpain
r e l a t e d e me r g e n c y d e p a r t me n t v1,2)
i s i .t sI n( y o u n g a d u l t s ( < 3 0 y e a r s o f a g e ) ,
p s y c h o l o g i c c a u s e s a c c o u n t f o r mo s t c a s e s o f d i zzi n e s s , w h e r e a s v e s t i b u l a r l e s i o n s
b e c o me mo r e c o mmo n i n mi d l i f e . I n t h e e l d e r l y, c e r e b r o v a s c u l a r a n d c a r d i a c
d i s o r d e r s , c o mb i n e d w i t h mu l t i p l e s e n s o r y d e f i c i t s , o u t w e i g h s i mp l e v e s t i b u l a r
c a u s e s . A p p r o xi ma t e l y o n e - f o u r t h o f t h e s e d i zzi n e s s - r e l a t e d v i s i t s a r e r e l a t e d t o t r u
v e r t i g o 2)( . T h e r e f o r e , mo s t p a t i e n t s w i t h d i zzi n e s s h a v e n o n v e s t i b u l a r u n d e r l y i n g
p r o c e s s e s . O f t h o s e w i t h v e r t i g o , c e n t r a l l e s i o n s a r e f o u n d i n a p p r o xi ma t e l y 1 % o f a
p a t i e n t s a n d 1 0 % t o 2 0 % o f e l d e r l y i n d i 2)
v i .d uDai zzi
l s (n e s s a n d v e r t i g o a r e u s u a l l y
b e n i g n , s e l f - l i mi t e d p r o c e s s e s n o t a s s o c i a t e d w i t h e xc e s s mo r t a l i t y, a l t h o u g h s o me
i n d i v i d u a l s s u f f e r i mp a i r e d q u a l i t y o f l i f e b e c a u s e o f r e c u r r e n t o r p e r s i s t e n t
s y mp t o ms .
I I I . Evaluation
A. History
T h e p a t i e n t ' s a g e , u n d e r l y i n g c o mo r b i d i t i e s ( e s p e c i a l l y h y p e r t e n s i o n , d i a b e t e s , h e a
d i s e a s e , a n d p s y c h i a t r i c i l l n e s s ) , a n d s y mp t o m c l a s s i f i c a t i o n c a t e g o r y h e l p l i mi t t h e
diagnostic possibilities. Further specificity is gained by eliciting the following:
1. Te m p o r a l p a t t e A
r nr e t h e s y mp t o ms e p i s o d i c o r c o n t i n u o u s ? I f e p i s o d i c , h o w
l o n g d o t h e y l a s t ? P e r i p h e r a l o r i g i n v e r t i g o i s o f t e n i n t e r mi t t e n t a n d o f s u d d e n
o n s e t c o mp a r e d w i t h t h e u s u a l , mo r e g r a d u a l o n s e t o f c e n t r a l v e r t i g o . A
c o n t i n u o u s h i s t o r y s u g g e s t s c e n t r a l n e r v o u s s y s t e m ( C N S ) p a t h o l o g y, d r u g o r
t o xi n e f f e c t s , me t a b o l i c d y s f u n c t i o n , o r p s y c h i a t r i c d i s e a s e . B e n i g n p a r o xy s ma l
p o s i t i o n a l v e r t i g o ( B P P V ) e p i s o d e s l a s t l e s s t h a n a mi n u t e ; v e r t e b r o b a s i l a r
t r a n s i e n t i s c h e mi c a t t a c k s l a s t f o r s e v e r a l mi n u t e s u p t o a n h o u r ; M n i r e ' s
disease persists for 1 to 24 hours; and vestibular neuronitis or acute labyrinthit
continues for several days.
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2. P r e c i p i t a t i n g o r e x a c e r b a t i n g f aHcat os rtsh e r e b e e n r e c e n t h e a d t r a u ma
( i mp l y i n g p e r i l y mp h a t i c f i s t u l a ) o r v i r a l i l l n e s s ( l a b y r i n t h i t i s ) ? W h a t i s t h e
r e l a t i o n s h i p t o s u d d e n h e a d mo v e me n t o r t u r n i n g o v e r i n b e d ( B P P V ) , c o u g h i n g
o r s n e e zi n g ( p e r i l y mp h a t i c f i s t u l a ) , p o s t u r a l c h a n g e s ( o r t h o s t a s i s ) , e xe r c i s e
( a r r h y t h mi a s ) , f o o d s ( s a l t y me a l s e xa c e r b a t i n g M n i r e ' s ) , w a l k i n g a n d t u r n i n g
( mu l t i p l e s e n s o r y d e f i c i t s ) , mi c t u r i t i o n o r p a i n ( v a s o v a g a l r e a c t i o n ) , a n d
e mo t i o n a l u p s e t ( h y p e r v e n t i l a t i o n ) ?
3. A s s o c i a t e d s y m p t o m
M sa r k e d n a u s e a , v o mi t i n g , d i a p h o r e s i s , a u r a l f u l l n e s s ,
a n d r e c r u i t me n t ( p e r c e p t i o n o f s o u n d s b e i n g t o o l o u d ) a r e t y p i c a l o f p e r i p h e r a l
vestibular disorders. Episodic vertigo associated with tinnitus and gradual
(unilateral) hearing loss involving low frequencies preferentially suggests
M n i r e ' s n i r e ' s d i s e a s e . A s y mme t r i c w e a k n e s s , c r a n i a l n e r v e o r c e r e b e l l a r
d y s f u n c t i o n , d i p l o p i a , n u mb n e s s , o r d y s a r t h r i a s u g g e s t s b r a i n s t e m o r C N S
d i s e a s e . H e a d a c h e , s c o t o ma t a , o r t u n n e l v i s i o n p o i n t s t o mi g r a i n e . N u mb n e s s o
p a r e s t h e s i a s ma y i n d i c a t e n e u r o p a t h y c o n t r i b u t i n g t o mu l t i p l e s e n s o r y d e f i c i t s
(C h a p t e r 4 ). .6 A s i n g l e , a b r u p t e p i s o d e o f s e v e r e v e r t i g o w i t h n e g a t i v e D i xH a l l p i k e ( D H ) t e s t i n g ( s e IcItIi .oBn. )1 t h a t g r a d u a l l y s u b s i d e s o v e r d a y s i mp l i e s
labyrinthitis (if hearing is affected), or vestibular neuronitis (if hearing is
u n a f f e c t e d4)
) (. M i l d v e r t i g o w i t h p r o mi n e n t t i n n i t u s , u n i l a t e r a l h e a r i n g l o s s , a n d
l o s s o f c o r n e a l r e f l e x i s w o r r i s o me f o r a n a c o u s t i c n e u r o ma .
4. M e d i c a t i o n s o r t o x iMnasn y me d i c a t i o n s c a n c a u s e d i zzi n e s s , a l t h o u g h o n l y
a f e w ( a mi n o g l y c o s i d e s , l e a d , me r c u r y ) c a u s e v e r t i g o . A s s e s s t o xi n e xp o s u r e b y
e xp l o r i n g j o b a n d r e c r e a t i o n a l a c t i v i t i e s , i n c l u d i n g i l l i c i t d r u g u s e .
T h i s e mp h a s i ze s o r t h o s t a t i c v i t a l s i g n s , t h e e y e s , e a r s , a n d n e u r o l o g i c a n d
c a r d i o v a s c u l a r s y s t e ms .
1. D e t e c t i o n o f n y s t a g mi su sc r i t i c a l b e c a u s e i t i s t h e o n l y o b j e c t i v e s i g n o f
v e r t i g o 3)( . N y s t a g mu s c a n o c c u r s p o n t a n e o u s l y o r i n r e s p o n s e t o c h a n g e s i n
e y e o r b o d y p o s i t i o n . P e r i p h e r a l v e s t i b u l a r d i s o r d e r s u s u a l l y c a u s e h o r i zo n t a l o
r o t a t o r y n y s t a g mu s , w h e r e a s C N S p a t h o l o g y i s r e f l e c t e d b y v e r t i c a l n y s t a g mu s
a n o mi n o u s s i g n . I n t r u e v e r t i g o c a u s e d b y B P P V, D H ma n e u v e r s o f t e n
confirm
P. 11 5
t h e d i a g n o s i s ( s e n s i t i v i t y 6 0 % 9 0 % , s p e c i f i c i t y 95)
0%
. T 9h 5e %p)a( t i e n t i s
mo v e d r a p i d l y f r o m a s i t t i n g t o a s u p i n e p o s i t i o n w i t h t h e h e a d t u r n e d a t a 3 0
a n g l e , f i r s t t o o n e s i d e a n d t h e n t o t h e o t h e r. A p o s i t i v e D H t e s t i n c l u d e s
precipitation of vertigo, latency of onset by a few seconds, rotational
n y s t a g mu s , r e s o l u t i o n w i t h i n a mi n u t e , a n d l e s s e n e d s y mp t o ms a n d n y s t a g mu s
w i t h p r o l o n g e d l a t e n c y o n r e p e a t e d t e s t i n g ( i . e . , f 3,5)
a t i g. aTb hi l ei t yl a) (c k o f
l a t e n c y a n d f a t i g a b i l i t y c h a r a c t e r i ze v e r t i g o c a u s e d b y s e r i o u s c e n t r a l l e s i o n s .
2. N e u r o l o g i c e x a m i n a t si oenr v e s t o d e t e c t b r a i n s t e m o r C N S p a t h o l o g y. I t i s
i mp o r t a n t t o f o c u s o n t h e p a t i e n t ' s mo t o r c o o r d i n a t i o n a n d s e n s o r y f u n c t i o n t o
d e t e c t t h e p r e s e n c e o f a n u n s t e a d y g a i t , p a s t - p o i n t i n g , a t a xi a , o r a b n o r ma l
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6.9 - Vertigo
R o mb e r g t e s t .
3. O t o s c o p yc a n d e t e c t o t i t i s me d i a o r c h o l e s t e a t o ma . N y s t a g mu s w i t h v e r t i g o
f o l l o w i n g p o s i t i v e o r n e g a t i v e p r e s s u r e a p p l i e d t o t h e t y mp a n i c me mb r a n e
( p n e u ma t i c o t o s c o p y ) s u g g e s t s a p e r i l y mp h a t i c f i s t u l a .
4. O t h e r p r o v o c a t i v e t e(sf tosr c e d h y p e r v e n t i l a t i o n , v e s t i b u l o - o c u l a r r e f l e x
t e s t i n g , v i g o r o u s h o r i zo n t a l h e a d s h a k i n g ) a r e n o t r o u t i n e l y h e l p f u l a n d a r e b e s t
left to consultants.
C. Testing
1. C l i n i c a l l a b o r a t o r y t eMs ot ss t ( 8 0 % 9 0 % ) p a t i e n t s n e e d n o l a b o r a t o r y t e s t i n g
(1,2,3) . A u d i o me t r y i s s u g g e s t e d i f t i n n i t u s o r h e a r i n g l o s s i s p r e s e n t . B l o o d
t e s t s a r e d i c t a t e d b y a p p r o p r i a t e c l i n i c a l i n d i c a t i o n s o n l y. B r a i n s t e m a u d i t o r y e v o k e d r e s p o n s e s c a n h e l p d e t e c t mu l t i p l e s c l e r o s i s o r a c o u s t i c n e u r o ma . H o l t e
mo n i t o r i n g i s i n d i c a t e d i f a r r h y t h mi a s a r e s u s p e c t e d . S p e c i a l i ze d t e s t i n g
p o s t u r o g r a p h y, r o t a t i o n a l c h a i r t e s t i n g , e l e c t r o n y s t a g mo g r a p h y i s b e s t
o r d e r e d b y a c o n s u l t a n t w h e n t h e d i a g n o s i s r e ma i n s u n c l e a r a f t e r i n i t i a l
evaluation.
Cgo n s i d e r D o p p l e r u l t r a s o u n d f o r s u s p e c t e d t r a n s i e n t
i s c h e mi c a t t a c k a n d ma g n e t i c r e s o n a n c e i ma g i n g i f C N S l e s i o n s a r e s u s p e c t e d .
D. Genetics
T h e r e a r e n o s i g n i f i c a n t g e n e t i c o r f a mi l i a l i n f l u e n c e s r e l a t e d t o v e r t i g o .
I V. D I AGN OSI S
P e r i p h e r a l c a u s e s o f v e r t i g o ( i n a p p r o xi ma t e o r d e r o f f r e q u e n c y i n p r i ma r y c a r e )
i n c l u d e B P P V, v i r a l l a b y r i n t h i t i s o r v e s t i b u l a r n e u r o n i t i s ( a c u t e u n i l a t e r a l
v e s t i b u l o p a t h y ) , s e r o u s o t i t i s , p e r i l y mp h a t i c f i s t u l a , M n i r e ' s n i r e ' s d i s e a s e , a n d
d r u g s ( a l c o h o l , a mi n o g l y c o s2)
i d.e sC)e( n t r a l c a u s e s o f v e r t i g o i n c l u d e
v e r t e b r o b a s i l a r t r a n s i e n t i s c h e mi c a t t a c k , c e r e b e l l a r i n f a r c t i o n o r n e o p l a s m,
d e my e l i n a t i n g d i s e a s e , b r a i n s t e m i n f a r c t i o n o r n e o p l a s m, c e r e b e l l o p o n t i n e a n g l e
t u mo r s , mi g r a i n e , h y p e r v e n t i l a t i o n , s e i zu r e s , s p i n o c e r e b e l l a r d e g e n e r a t i o n , a n d
c e r t a i n s y s t e mi c d i s o r d e r s ( i n f e c t i o n s , v a s c u l i t i s , s y p h i l i s ) . T h e c e r v i c a l s p i n e i s
rarely the source of vertigo, either by osteoarthritic spur occlusion of the vertebral
a r t e r i e s o r b y p r o p r i o c e p t i v e o v e r s t i mu l a t i o n b y f a c e t j o i n t a r t h r o p a t h y.
T h e c o mmo n f e a t u r e s o f v e s t i b u l a r d y s f u n c t i o n a r e v e r t i g o , n y s t a g mu s , a n d p o s t u r a
i n s t a b i l i t y. T h e c l i n i c i a n ' s c h i e f t a s k i s t o d i s t i n g u i s h b e t w e e n u s u a l l y b e n i g n
p e r i p h e r a l c a u s e s a n d mo r e o mi n o u s c e n t r a l p r o c e s s e s b y a t t e n d i n g t o t h e h i s t o r i c a
a n d p h y s i c a l e xa mi n a t i o n c h a r a c t e r i s t i c s o u t l i n e d I iInI. sCeecnt ti roanl v e r t i g o i s
a s s o c i a t e d w i t h mo d e r a t e n a u s e a a n d v o mi t i n g , a t a xi a , n e u r o l o g i c s i g n s a n d
s y mp t o ms , r a r e h e a r i n g l o s s , a n d v e r y s l o w c o mp3)
e n. sPaet iroi pnh(e r a l v e r t i g o
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6.9 - Vertigo
ma n i f e s t s c o mb i n a t i o n s o f s e v e r e n a u s e a a n d v o mi t i n g , t i n n i t u s , f l u c t u a t i n g h e a r i n g
l o s s , mi l d e r p o s t u r a l i mb a l a n c e , a l a c k o f n e u r o l o g i c s i g n s a n d s y mp t o ms , a n d r a p i d
c o mp e n s a t i o n .
References
1 . D i e t e r i c h M . D i zzi n eTshe
s . N eur ol ogi 2s 0t 0 4 ; 1 0 : 1 5 4 1 6 4 .
2 . S l o a n e P D , C o e y t a u x R R , B e c k R S , e t a l . D i zzi n e s s : s t a t eA nn
of the art.
I nt er n M ed2 0 0 1 ; 1 3 4 ( 9 P a r t 2 ) : S 8 2 3 S 8 3 2 .
3 . T r a c c i s S , Z o r o d d u G F, Z e c c a M T , e t a l . E v a l u a t i n g p a t i e n t s w i t h v e r t i g o :
b e d s i d e e xa mi n a t i oNneur
. ol S c2i 0 0 4 ; 2 5 ( S u p p l 1 ) : S 1 6 S 1 9 .
4 . B a l o k R W. Ve s t i b u l a r n e uN
r i tE
i sngl
.
J M ed2 0 0 3 ; 3 4 8 : 1 0 2 7 1 0 3 2 .
5 . P a r n e s L S , A g r a w a l S K , A t l a s J . D i a g n o s i s a n d ma n a g e me n t o f b e n i g n
p a r o xy s ma l p o s i t i o n a l v e r t i g o ( BCPM
PV
A )J. 2 0 0 3 ; 1 6 9 ( 7 ) : 6 8 1 6 9 3 .
211 / 652
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7.1 - Atypical Chest Pain
> T a b l e o f C o n t e n t s > 7 - C a r d i o v a s c u l a r P r o b l e ms > 7 . 1 - A t y p i c a l C h e s t P a i n
7.1
Atypical Chest Pain
Thom as J. Hanse n
I . Background
Typical chest pain is pain that is typical of anginal pain. T his pain is usually
d e s c r i b e d a s s u b s t e r n a l , r a d i a t i n g t o t h e l e f t n e c k a n d a r m, a n d i s p r e s s u r e l i k e o r
h a s a s q u e e zi n g s e n s a t i o n . A t y p i c a l c h e s t p a i n i s d e f i n e d a s t h e a b s e n c e o f t h i s
typical presentation.
Atypical chest pain can originate in any of the thoracic organs, as well as from
e xt r a t h o r a c i c s o u r c e s ( e . g . , t h y r o i d i t i s o r p a n i c d i s o r d e r ) .
I I I . Evaluation
T he approach to the evaluation of acute chest pain, whether typical or atypical,
should be to rapidly assess whether the pain is due to cardiac disease. Atypical
c h e s t p a i n d o e s n o t r u l e o u t a n a c u t e my o c a r d i a l i n f a r c t i o n ( A M I ) , e s p e c i a l l y i n
w o me n 1)
( , p a t i e n t s w i t h d i a b e t e s , a n d t h e e l d e r l y, i n w h o m a n A M I ma y p r e s e n t i n
an atypical fashion. A clinical history of the chest pain and an electrocardiogram
( E C G ) s h o u l d b e o b t a i n e d w i t h i n 5 mi n u t e s a f t e r p r e2)s e. nTt ha et i oEnC(G i s
c r i t i c a l f o r g u i d i n g i n i t i a l t h e r a p y a n d d e c i s i o n s r e g a r d i n g d i a g n o s i s a n d t r e a t me n t .
A. History
T h e c l i n i c a l h i s t o r y s h o u l d f o c u s o n t h e t i me o f o n s e t , t h e c h a r a c t e r i s t i c o f t h e p a i n
t h e l o c a t i o n ( r e t r o s u b s t e r n a l , s u b xi p h o i d , d i f f u s e ) , t h e f r e q u e n c y o f t h e p a i n
( c o n s t a n t , i n t e r mi t t e n t , a c u t e o n s e t ) , t h e d u r a t i o n o f t h e p a i n , p r e c i p i t a t i n g f a c t o r s
( e xe r t i o n , s t r e s s , f o o d , r e s p i r a t i o n , mo v e me n t ) , t h e q u a l i t y o f t h e p a i n ( b u r n i n g ,
s q u e e zi n g , d u l l , s h a r p , t e a r i n g , h e a v y ) , a n d a n y a s s o c i a t e d s y mp t o ms ( s h o r t n e s s o f
b r e a t h , d i a p h o r e s i s , n a u s e a , v o mi t i n g , j a w p a i n , b a c k p a i n , r a d i a t i o n , p a l p i t a t i o n s ,
weakness, fatigue).
Other pertinent questions include assessing risk factors for coronary artery disease
( d i a b e t e s , s mo k i n g , h y p e r t e n s i o n , h y p e r c h o l e s t e r e mi a , f a mi l y h i s t o r y ) , a n o r e xi a ,
a n xi e t y, c o u g h a n d / o r w h e e zi n g , d r u g u s e , f e v e r, p r e v i o u s h i s t o r y o f d e e p v e i n
t h r o mb o s i s o r p u l mo n a r y e mb o l i s m, p a i n i n c r e a s e d w i t h r e c u mb e n c y o r r e l i e v e d b y
l e a n i n g f o r w a r d , p r e s e n c e o f a ma s s , l e s i o n , o r r a s h o n t h e c h e s t , p r e v i o u s h i s t o r y
c a n c e r, p r e g n a n c y / p o s t p a r t u m, o r a l c o n t r a c e p t i v e u s e , o r t r a u ma , r e l a t i o n s h i p o f p a
212 / 652
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with eating, and syncopal or near-syncopal episodes.
T h e p h y s i c a l e xa mi n a t i o n s h o u l d i n c l u d e a r a p i d a s s e s s me n t o f v i t a l s i g n s , a s w e l l
a s o xy g e n s a t u r a t i o n a n d e l e c t r o c a r d i o g r a p h i c e v a l u a t i o n . F o l l o w i n g t h i s , a n
e xa mi n a t i o n o f t h e c h e s t s h o u l d b e p e r f o r me d . C a r d i a c e xa mi n a t i o n s h o u l d f o c u s o n
p e r i c a r d i a l r u b s , s y s t o l i c a n d d i a s t o l i c mu r mu r s , t h i r d o r f o u r t h h e a r t s o u n d s , a n d
d i s t e n d e d j u g u l a r v e i n s . A u s c u l t a t i o n o f t h e l u n g s s h o u l d f o c u s o n d i mi n i s h e d b r e a t
s o u n d s , a p l e u r a l r u b , r a l e s , r h o n c h i , a n d w h e e ze s . E xa mi n a t i o n o f t h e l e g s s h o u l d
f o c u s o n e d e ma a n d p o o r p e r f u s i o n o f a l i mb , w h i c h ma y i n d i c a t e a n a o r t i c
d i s s e c t i o n . E xa mi n a t i o n o f t h e mu s c u l o s k e l e t a l s y s t e m s h o u l d f o c u s o n r e p r o d u c i b l e
o r l o c a l i ze d p a i n . E xa mi n a t i o n o f t h e s k i n s h o u l d a s s e s s f o r l e s i o n s , ma s s e s , o r
rashes.
C. Testing
1. O x y g e n s a t u r a t i oOnxy g e n s a t u r a t i o n b e l o w 9 2 % ma y i n d i c a t e a my o c a r d i a l
i n f a r c t i o n , s p o n t a n e o u s p n e u mo t h o r a x, p u l mo n a r y e mb o l i s m, o r p n e u mo n i a . A n
arterial blood gas is warranted.
2. E C G A l w a y s c o mp a r e w i t h a n o l d E C G w h e n a v a i l a b l e . T h e p r e s e n c e o f T w a v e
i n v e r s i o n i s c o n s i s t e n t my
w i toc
h ar di al i s c hemi
. SaT e l e v a t i o n i s c o n s i s t e n t
w i t hmy oc ar di al i nj ur
, ayn d S T d e p r e s s i o n i s c o n s i s t e nstubendoc
with
ar di al
i nf ar c t i on
. A Q w a v e i s d i a g n o s t i c my
o f oc
a ar di al i nf ar c t(3)
i on
. A pul monar y
embol i s mi s c l a s s i c a l l y a s s o c i a t e d w i t h1 Qt h3eT 3Sp a t t e r n r e p r e s e n t i n g a
large S wave in I, an S T depression in II, and a large Q wave in III with T wave
inversion.
P. 1 2 0
S e n s i t i v i t y o f t h i s i s l e s s t h a n 2 0 % , hAo cwut
e vee per
r. i c ar di dt i esmo n s t r a t e s
d i f f u s e S T - s e g me n t e l e v a t i o n , i n w h i c h t h e S T s e g me n t i s f l a t o r s l i g h t l y
concave, and P R depression.
3. O t h e r l a b o r a t o r y t e s t s
a. C o m p r e h e n s i v e m e t a b o l i c p r U
o fsiel ed t o d e t e c t me t a b o l i c a b n o r ma l i t y
a s t h e c a u s e o f c h e s t p a i n a s w e l l a s a b n o r ma l i t i e s o f t h e l i v e r
b. C o m p l e t e b l o o d c o uUnste d t o d e t e c t i n f e c t i o n a n d i n f l a mma t o r y d i s o r d e r s
c. C r e a t i n e k i n a s e - M B ( C K - M B ) a n d t r oHpiognhi np o s i t i v e p r e d i c t i v e v a l u e
f o r a n A M I i f e l e v a t e d b u t ma y b e n e g a t i v e i n i t i a l l y
d. D - d i m e rS e n s i t i v e b u t n o t s p e c i f i c f o r a p u l mo n a r y e mb o l i s m
e. L i v e r f u n c t i o n t e s t s , a m y lHaeslei c, o b a c t e r p y l oUr si e d t o d e t e r mi n e a
gastrointestinal etiology of the pain, such as liver distention, pancreatitis,
g a s t r i c , o r d u o d e n a l u l c e r s dHu.e py
t ol or i
f. To x i c o l o g y s c r e eRne c o mme n d e d i f c o c a i n e u s e i s b e l i e v e d t o b e t h e
cause of the chest pain
4. I m a g i n g s t u d i e s
a. C h e s t x - r a yU s e f u l i n d i a g n o s i n g p n e u mo n i a , p n e u mo t h o r a x, a o r t i c
213 / 652
tmdmss
dissection, acute pericarditis, and esophageal rupture

b. U l t r a s o u n dH e l p f u l t o d i a g n o s e p e r i c a r d i a l , v a l v u l a r d i s e a s e , a n d t o
d e mo n s t r a t e c a r d i a c w a l l mo t i o n a b n o r ma l i t i e s
c. S t r e s s e c h o c a r d i o g r U
am
sed for stable patients who have been ruled out
f o r i n f a r c t i o n t o d e t e r mi n e i f c a r d i a c d i s e a s e i s p r e s e n t
d. C o m p u t e d t o m o g r a p U
h ys e d t o d i a g n o s e a o r t i c d i s s e c t i o n i n s t a b l e
p a t i e n t s a n d ma y i d e n t i f y p u l mo n a r y e mb o l i s m o r c a r d i a c e f f u s i o n
I V. Diagnosis
T he differential diagnosis for atypical chest pain includes:
1. B r e a s t l e s i o nAsb s c e s s , c a r c i n o ma , f i b r o a d e n o s i s , ma s t i t i s
2. C a r d i o v a s c u l aArM I , a n g i n a p e c t o r i s , a o r t i c d i s s e c t i o n , a o r t i c v a l v u l a r d i s e a s e ,
h y p e r t r o p h i c c a r d i o my o p a t h y, mi t r a l v a l v e p r o l a p s e , my o c a r d i t i s , p e r i c a r d i t i s ,
p r i ma r y p u l mo n a r y h y p e r t e n s i o n , t h o r a c i c a o r t i c a n e u r y s m, n e o p l a s m
3. G a s t r o i n t e s t i n a l d i s e aEss eo p h a g e a l r u p t u r e , e s o p h a g i t i s , f o r e i g n b o d y
p r e s e n c e , g a s t r i c d i s t e n t i o n , g a s t r i t i s , l i v e r d i s t e n t i o n , M a l l o r y - We i s s s y n d r o me
p a n c r e a t i t i s , p e p t i c u l c e r d i s e a s e , P l u mme r - Vi n s o n s y n d r o me , s p l e n i c i n f a r c t ,
subphrenic abscess, Z enker's diverticulum
4. T h y r o i dT h y r o i d i t i s
5. P s y c h o g e n i c c a u s A
e ns xi e t y, p a n i c a t t a c k
6. P u l m o n a r y d i s e a sBer o n c h i t i s , n e o p l a s m, p l e u r i t i s , p n e u mo n i a , p u l mo n a r y
h y p e r t e n s i o n , p u l mo n a r y e mb o l i s m
7. M u s c u l o s k e l e t a l d i s o r B
d reur i s e d o r f r a c t u r e d r i b , c e r v i c a l d i s c h e r n i a t i o n ,
c o s t o c h o n d r i t i s , i n t e r c o s t a l mu s c l e c r a mp , i n t e r c o s t a l my o s i t i s , p e c t o r a l s t r a i n ,
o s t e o a r t h r i t i s , t h o r a c i c o u t l e t s y n d r o me
8. N e u r a l g i aH e r p e s zo s t e r, n e u r o f i b r o ma , n e o p l a s m, t a b e s 4)d o r s a l i s (
O n c e a c a r d i a c e t i o l o g y f o r a t y p i c a l c h e s t p a i n h a s b e e n e l i mi n a t e d , a c a r e f u l h i s t o
a n d p h y s i c a l e xa mi n a t i o n u s u a l l y y i e l d s a d i a g n o s i s . T h e a f o r e me n t i o n e d t e s t s a r e
u s e f u l i n ma k i n g t h e d i a g n o s i s a n d d e t e r mi n i n g a n a p p r o p r i a t e t r e a t me n t p l a n .
References
1 . D e C a r a J E . N o n i n v a s i v e c a r d i a c t e s t i n g i n JwAom
meMn ed
. W omens A s s oc
2003 Fall, 58(4):254263.
2 . L e e T H , G o l d ma n L . E v a l u a t i o n o f t h e p a t i e n t w i t h a c u t e JcAhM
e sAt p a i n .
2 0 0 0 ; 3 4 2 : 11 8 7 11 9 5 .
214 / 652
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3 . B r a u n w a l d B , F a u c i A , K a s p e r D , e t aHl ar
. erdi sson'
. s pr i nc i pl es of i nt er nal
medi c i ne
, 1 5 t h e d , N e w Yo r k , N Y : M c G r a w - H i l l , 2 0 0 1 .
4. Adler S N , G asbarra D B , Adler- K lein D
A , poc
e d sk.et manual of di f f er ent i al
di agnos i, s4 t h e d . P h i l a d e l p h i a , PA : L i p p i n c o t t W i l l i a ms & W i l k i n s , 2 0 0 0 .
215 / 652
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7.2 - Chest Pain
> T a b l e o f C o n t e n t s > 7 - C a r d i o v a s c u l a r P r o b l e ms > 7 . 2 - C h e s t P a i n
7.2
Chest Pain
Sanje e v Sharm a
I . Background
C h e s t p a i n a c c o u n t s f o r a p p r o xi ma t e l y 5 . 6 mi l l i o n e me r g e n c y d e p a r t me n t v i s i t s
a n n u a l l y. I t i s s e c o n d o n l y t o a b d o mi n a l p a i n a s t h e mo s t c o mmo n r e a s o n f o r a n
e me r g e n c y d e p a r t me n t v i s i t . M o r e t h a n 3 mi l l i o n p a t i e n t s a r e h o s p i t a l i ze d y e a r l y i n
t h e U n i t e d S t a t e s f o r c h e s t p a i n . A s t a n d a r d i ze d a p p r o a c h t o a d d r e s s i n g t h e
ma n a g e me n t o f t h e s e p a t i e n t s i s e s s e n t i a l , g i v e n t h e a d v e r s e c o n s e q u e n c e s o f
mi s s i n g a l i f e - t h r e a t e n i n g c o n d i t i o n .
C a r d i a c p a i n i s u s u a l l y d u e t o l o w p e r f u s i o n t o t h e my o c a r d i u m t h r o u g h t h e c o r o n a r
a r t e r i e s t h a t a r e c o mp l e t e l y o r p a r t i a l l y b l o c k e d b y a t h e r o s c l e r o t i c p l a q u e . T h i s l e a
t o t i s s u e h y p o xi a , a n a e r o b i c me t a b o l i s m, l a c t i c a c i d o s i s , a n d a b n o r ma l p r o s t a g l a n d i
secretion. Pain originating from the lungs or pleura is caused by the irritation or
i n f l a mma t i o n o f t h e l u n g s a n d / o r t h e p l e u r a o r d i a p h r a g m. G a s t r o i n t e s t i n a l p a i n
a r i s e s f r o m mu c o s a l i n f l a mma t i o n o r s t r u c t u r a l a b n o r ma l i t y, s u c h a s s t r i c t u r e o r
obstruction.
I I I . Evaluation
T h e p a t i e n t s h o u l d b e e v a l u a t e d t o d e t e r mi n e t h e d i a g n o s i s a n d t o f o r mu l a t e a n
i mme d i a t e ma n a g e me n t p l a n . P r i o r i t y s h o u l d b e g i v e n t o r u l e o u t l i f e - t h r e a t e n i n g
c a r d i o v a s c u l a r c a u s e s . I n p a t i e n t s w i t h a c u t e p a i n , t h e c l i n i c i a n mu s t a s s e s s t h e
p a t i e n t ' s h e mo d y n a mi c a n d r e s p i r a t o r y s t a t u s . I f e i t h e r i s c o mp r o mi s e d , ma n a g e me n
s h o u l d f o c u s o n s t a b i l i zi n g t h e p a t i e n t .
A. History
Q u e s t i o n s s h o u l d b e a s k e d a b o u t t h e e xa c t l o c a t i o n , q u a l i t y, a n d n a t u r e o f o n s e t ,
d u r a t i o n , e xa c e r b a t i n g f a c t o r s , a n d r a d i a t i o n o f p a i n . R a d i a t i o n o f p a i n t o t h e l e f t a
i s c o mmo n i n my o c a r d i a l i s c h e mi a . P a i n o f a o r t i c d i s s e c t i o n r a d i a t e s b e t w e e n t h e
s c a p u l a e . I t i s n e c e s s a r y t o i n q u i r e a b o u t c a r d i a c r i s k f a c t o r s , i n c l u d i n g s mo k i n g ,
h y p e r t e n s i o n , d i a b e t e s me l l i t u s , h y p e r l i p i d e mi a , a n d a f a mi l y h i s t o r y o f c o r o n a r y
a r t e r y d i s e a s 1)
e .(
A r a p i d p h y s i c a l a s s e s s me n t o f t h e p a t i e n t s h o u l d i n c l u d e a f u l l s e t o f v i t a l s i g n s ,
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A r a p i d p h y s i c a l a s s e s s me n t o f t h e p a t i e n t s h o u l d i n c l u d e a f u l l s e t o f v i t a l s i g n s ,
d e t e r mi n a t i o n o f t h e p r e s e n c e o r a b s e n c e o f c y a n o s i s , d y s p n e a a n d d i a p h o r e s i s ,
e xa mi n a t i o n o f t h e n e c k , t h o r a x, a n d a b d o me n , a n d p a l p a t i o n o f t h e ma j o r p e r i p h e r a
arteries for the presence of and the characteristics of the pulse.
C. Testing
1. L a b o r a t o r y t e s P
t su l s e o xi me t r y s h o u l d b e o r d e r e d t o a s s e s s o xy g e n s t a t u s .
C a r d i a c e n zy me s , c r e a t i n i n e p h o s p h o k i n a s e ( C K , C K - M B ) a n d t r o p o n i n T o r I
s h o u l d a l s o b e o r d e r e d i n p a t i e n t s s u s p e c t e d o f c a r2)d .i aCc - pr eaai nc t (i v e
p r o t e i n , b r a i n n a t r i u r e t i c p e p t i d e , a n d s e r u m my o g l o b i n h a v e b e e n u s e d i n t h e
ma n a g e me n t o f p a t i e n t s w i t h c h e s t p a i n . T h e p o s s i b i l i t y o f l i f e - t h r e a t e n i n g
c a r d i a c p a i n c a n n o t b e e xc l u d e d b a s e d u p o n a s i n g l e n e g a t i v e v a l u e o f a n y o f
t h e s e ma r k e r s ; t h r e e s e t s o f c a r d i a c e n zy me s s h o u l d3) .b eA uc soemp
d l(e t e
me t a b o l i c p r o f i l e i n c l u d i n g l i v e r f u n c t i o n t e s t s , a my l a s e , a n d l i p a s e s h o u l d b e
o r d e r e d i f a g a s t r o i n t e s t i n a l c a u s e i s s u s4)p.e c t e d (
2. E l e c t r o c a r d i o g r a m ( E CAG
n )e l e c t r o c a r d i o g r a m ( E C G ) i s e s s e n t i a l . A n E C G
s h o w i n g c h a n g e s c o n s i s t e n t w i t h i s c h e mi a o r i n f a r c t i o n i s a s s o c i a t e d w i t h a h i g h
p r o b a b i l i t y o f a c u t e my o c a r d i a l i n f a r c t i o n o r u n s t a b l e a n g i n a .
3. E c h o c a r d i o g r a m
T h i s i s h e l p f u l i f v a l v u l a r a b n o r ma l i t y i s s u s p e c t e d .
4. C h e s t x - r a yP u l mo n a r y d i s e a s e s s u c h a s p n e u mo n i a , p l e u r a l e f f u s i o n , a n d
p n e u mo t h o r a x c a n b e d i a g n o s e d . W i d e n e d me d i a s t i n u m i s s e e n i n p a t i e n t s w i t h
aortic dissection.
5. C o m p u t e d t o m o g r a p h y ( C T ) s c a n o r m a g n e t i c r e s o n a n c eI f itm
h ea g i n g
p a t i e n t ' s h i s t o r y a n d e xa mi n a t i o n a r e c o n s i s t e n t w i t h a o r t i c d i s s e c t i o n , t h e s e
i ma g i n g s t u d i e s s h o u l d b e p e r f o r me d t o e v a l u a t e t h e a o r t a . A t r a n s t h o r a c i c
e c h o c a r d i o g r a m c a n b e p e r f o r me d t o a s s e s s c a r d i a c f u n c t i o n .
P. 1 2 2
6. V e n t i l a t i o n p e r f u s i o n s c a n / s p i r a l C T s c a n o f t h
T eh ecshee asrt e
a p p r o p r i a t e i n i t i a l t e s t s i n p a t i e n t s w i t h a h i s t o r y o f v e n o u s t h r o mb o e mb o l i s m o r
c o a g u l a t i o n a b n o r ma l i t y.
7. O t h e r t e s t i nIgf t h e p a t i e n t w i t h c h e s t p a i n s h o w s n o e v i d e n c e o f l i f e threatening conditions, the clinician should focus on serious chronic conditions
w i t h t h e p o t e n t i a l t o c a u s e ma j o r c o mp l i c a t i o n s i n t h e f u t u r e ; t h e mo s t c o mmo n
of these is stable angina. Cost-effective and noninvasive tests for coronary
d i s e a s e s u c h a s e xe r c i s e e l e c t r o c a r d i o g r a p h y o r s t r e s s e c h o c a r d i o g r a p h y
should
P. 1 2 3
b e p e r f o r me d f o r l o w - r i s k p a t i e n t s . G a s t r o i n t e s t i n a l c a u s e s c a n b e e v a l u a t e d b y
e n d o s c o p y. A s ma n y a s 1 0 % o f p a t i e n t s w i t h c h e s t p a i n ma y h a v e e mo t i o n a l o r
psychiatric conditions. T hese patients should be appropriately evaluated by an
e xp e r t 5)( .
TAB L E 7.2.1 Differential Diagnosis of Chest Pain

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7.2 - Chest Pain
Cardiac
I s c h e mi c d i s e a s e
A c u t e my o c a r d i a l i n f a r c t i o n
Stable angina
Unstable angina
Myocarditis
Pericarditis
Va l v u l a r h e a r t d i s e a s e
Aortic stenosis
Mitral valve prolapse
H y p e r t r o p h i c c a r d i o my o p a t h y
Aortic dissection
Noncardiac
Pulm onary
P n e u mo n i a
Pleuritis
Pleural effusion
P n e u mo t h o r a x
Gastrointe stinal
Esophageal spasm
Esophagitis/gastritis
Peptic ulcer disease
Gall stone
Musculoske le tal
Costochondritis
Muscle spasm
Cervical radiculopathy
Ne urologic
H e r p e s zo s t e r
N e r v e r o o t c o mp r e s s i o n
Psy chiatric
A n xi e t y s t a t e
I V. Diagnosis
T h e d i f f e r e n t i a l d i a g n o s i s o f c h e s t p a i n i s p r eTsaebnltee d7 .i2n. 1
References
1 . R i c h E C , C r o w s o n T W, H a r r i s I B . T h e d i a g n o s t i c v a l u e o f t h e me d i c a l h i s t o r y.
A r c h I nt er n M ed
1987;147:1957.
2 . H a mm C W, G o l d ma n n B U , H e e s c h e n C , e t a l . E me r g e n c y r o o m t r i a g e o f
p a t i e n t s w i t h a c u t e c h e s t p a i n b y me a n s o f r a p i d t e s t i n g f o r c a r d i a c t r o p o n i n T o r
troponin N
I . E ngl J M ed1 9 9 7 ; 3 3 7 ( 2 3 ) : 1 6 4 8 5 3 .
218 / 652
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7.2 - Chest Pain
3. Caragher T E, Fernandez B B, Jacobs F L, et al. Evaluation of quantitative

c a r d i a c b i o ma r k e r p o i n t - o f - c a r e t e s t i n g i n t h e e me r g e n c y Jd eEpmer
a r t me
g nt.
M ed 2 0 0 2 ; 2 2 ( 1 ) : 1 7 .
4 . E s l i c k G D , F a s s R . N o n c a r d i a c c h e s t p a i n : e v a l u a t i o n a n d t r e a t me n t .
G as t r oent er ol C l i n N or t h2A0 0m3 ; 3 2 ( 2 ) : 5 3 1 5 5 2 .
5 . H o K Y, K a n g J Y, Ye o B , e t a l . N o n - c a r d i a c , n o n - o e s o p h a g e a l c h e s t p a i n : t h e
r e l e v a n c e o f p s y c h o l o g i c a l f aG
c tut
o r 1s 9. 9 8 ; 4 3 ( 1 ) : 1 0 5 11 0 .
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7.3 - Bradycardia
> T a b l e o f C o n t e n t s > 7 - C a r d i o v a s c u l a r P r o b l e ms > 7 . 3 - B r a d y c a r d i a
7.3
Bradycardia
Mark D. Goodm an
I . Background
B r a d y c a r d i a , w h i c h i s d e f i n e d a s a h e a r t r a t e l e s s t h a n 6 0 b e a t s / mi n u t e , r e s u l t s f r o
a b n o r ma l i t i e s i n i mp u l s e f o r ma t i o n o r f a i l u r e o f c o n d u c t i o n . I t ma y o r ma y n o t b e a
c a u s e f o r c o n c e r n . F o r t h e w e l l - c o n d i t i o n e d a t h l e t e , b r a d y c a r d i a ma y c a r r y n o
u n d e r l y i n g r i s k o r mo r b i d i t y, b u t f o r t h e p a t i e n t w i t h a c a r d i a c o r n e u r o l o g i c c o n d i t i o
b r a d y c a r d i a c a n b e d e a d l y.
T h e mo s t i mp o r t a n t i n i t i a l a s s e s s me n t i s w h e t h e r t h e s l o w h e a r t r a t e i s p a t h o l o g i c o
i n n o c e n t . I n t h e p r e s e n c e o f s y n c o p e , n e u r o l o g i c c h a n g e s , my o c a r d i a l
i s c h e mi a / a n g i n a , f a t i g u e , o r d y s p n e a , o n e c a n s a f e l y a s s u me t h a t b r a d y c a r d i a
a d v e r s e l y i mp a c t s h e a1)l t .h (
A. Etiology
C o n d i t i o n s t h a t c a n ma n i f e s t a s b r a d y c a r d i a i n c l u d e e xp o s u r e , e l e c t r o l y t e i mb a l a n c e
( h y p o k a l e mi a ) , i n f e c t i o n , h y p o g l y c e mi a , h y p o t h y r o i d / h y p e r t h y r o i d , i n f e r i o r w a l l
my o c a r d i a l i n f a r c t i o n , c a r d i a c i s c h e mi a , i n c r e a s e d i n t r a c r a n i a l p r e s s u r e , me d i c a t i o n
( e . g . , - b l o c k e r s , c a l c i u m c h a n n e l b l o c k e r s , a n t i a r r h y t h mi c s , l i t h i u m, d i g o xi n ) , a t r i a
f i b r i l l a t i o n , l o n g Q T s y n d r o me , a n d s i c k s i n u s s y n d r o me . R e v e r s i b l e c a u s e s i n c l u d e
profound bradycardia, which often develops in patients with obstructive sleep apnea
a n d h y p o xi a , b u t ma y b e e l i mi n a t e d w i t h a p p r o p r i a t e a p n e a t r e a t me n t .
1. L o n g Q T s y n d r o m e
a. T h i s c o n d i t i o n w a s f i r s t d e s c r i b e d i n 1 9 5 7 i n a f a mi l y w i t h r e c u r r e n t s y n c o p e
a n d s u d d e n d e a t h . S e v e r a l d i f f e r e n t f a mi l i a l s y n d r o me s ma n i f e s t a s Q T
prolongation on an electrocardiogram (E C G), with a predilection for
ma l i g n a n t v e n t r i c u l a r a r r h y t h mi a s a n d p o s s i b l e s y n c o p e o r s u d d e n d e a t h . I f
the presenting event is syncope, a clinical evaluation that includes an E C G
a l mo s t a l w a y s r e v e a l s t h e p r o l o n g a t i o n o f t h e Q T i n t e r v a l , t h e c l i n i c a l
h a l l ma r k o f t h i s d i s o r d e r. S e v e r a l d r u g s c a n p r o l o n g t h e Q T c i n t e r v a l , a n d i t
i s i mp o r t a n t t o d i s t i n g u i s h d r u g - i n d u c e d Q T c p r o l o n g a t i o n f r o m t h e i n h e r i t e d
f o r m o f l o n g Q T s y n d r o4)
me
. (
P. 1 2 4
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7.3 - Bradycardia
b. L i f e - t h r e a t e n i n g c a r d i a c e v e n t s t e n d t o o c c u r u n d e r s p e c i f i c c i r c u ms t a n c e s
i n a g e n e - s p e c i f i c ma n n e r : s o me d u r i n g e xe r c i s e , s o me w i t h
a r o u s a l / e mo t i o n , s o me a t s l e e p / r e s t , a n d s o me w i t h l o u d n o i s e s .
2. S i c k s i n u s s y n d r o m e
a. S i c k s i n u s s y n d r o me c o mp r i s e s a v a r i e t y o f c o n d i t i o n s i n v o l v i n g s i n u s n o d e
d y s f u n c t i o n ( mo r e c o mmo n i n t h e e l d e r l y ) . M u l t i p l e ma n i f e s t a t i o n s o n E C G
include sinus bradycardia, sinus arrest, sinoatrial block, and
b r a d y t a c h y c a r d i a s y n d r o me , c h a r a c t e r i ze d b y a l t e r n a t i n g p e r i o d s o f s i n u s
b r a d y c a r d i a a n d s u p r a v e n t r i c u l a r t a c h y c a r d i a . T h e ma i n s t a y o f t r e a t me n t i s
a n a t r i a l o r d u a l - c h a mb e r p a c e ma
2,3)
k e. r (
b. C a u s e s c a n i n c l u d e c a r d i o my o p a t h i e s , c o l l a g e n - v a s c u l a r d i s e a s e , i s c h e mi a ,
i n f a r c t i o n , p e r i c a r d i t i s , my o c a r d i t i s , r h e u ma t i c h e a r t d i s e a s e , e l e c t r o l y t e
d i s o r d e r s ( e s p e c i a l l y h y p o k a l e mi a o r h y p o c a r b i a ) , a n d me d i c a t i o n s . T h e
mo s t c o mmo n c a u s e i s i d i o p a t h i c d e g e n e r a t i v e f i b r o t i c i n f i l t r a t i o n . C o r o n a r y
a r t e r y d i s e a s e c a n c o e xi s t w i t h s i c k s i n u s s y n d r o me . T h e s y n d r o me o c c u r s
in 1/600 cardiac patients older than 65 years and accounts for 50% of
p a c e ma k e r p l a c e me n t s i n t h e U n i t e d S t a t e s .
B. Epidemiology
T h e i n c i d e n c e o f b r a d y c a r d i a i n t h e g e n e r a l p o p u l a t i o n i s u n k n o w n , b u t a mo n g
cardiac patients and those older than 50 years, it is present in 0.6/10000 individual
P e a k i n c i d e n c e o c c u r s i n t h e s i xt h a n d s e v e n t h d e c a d e .
I I I . Evaluation
A. History
1. S y m p t o m sE l i c i t e v i d e n c e o f c a r d i a c n e u r o l o g i c o r r e s p i r a t o r y c o mp r o mi s e ,
s u c h a s d y s p n e a , p a l p i t a t i o n s , a n g i n a , d e c r e a s e d e xe r c i s e t o l e r a n c e ,
tachypnea, lightheadedness, or syncope.
2. C a r d i a c r i s k f a c t oI nr sq u i r e a b o u t f a mi l y h i s t o r y, t o b a c c o u s e , h y p e r l i p i d e mi a ,
d i a b e t e s me l l i t u s , a n d h y p e r t e n s i o n .
3. U n d e r l y i n g c o n d i t i oAnr se u n d e r l y i n g b r a d y c a r d i a r i s k f a c t o r s p r e s e n t ? A s k
a b o u t c a r d i o my o p a t h i e s , a l c o h o l d e p e n d e n c e / mi s u s e , r h e u ma t i c h e a r t d i s e a s e ,
a n d a n y o t h e r c o e xi s t i n g me d i c a l c o n d i t i o n s .
4. M e d i c a t i o n B
s r a d y c a r d i a c a n b e i n d u c e d b y d i g o xi n , p h e n o t h i a zi n e , q u i n i d i n e ,
p r o c a i n a mi d e , - b l o c k e r s , c a l c i u m c h a n n e l b l o c k e r s , c l o n i d i n e , l i t h i u m, a n d
a n t i a r r h y t h mi c me d i c a t i o n s . - B l o c k e r b r a d y c a r d i a i s e s p e c i a l l y c o mmo n ,
b e c a u s e - b l o c k e r s a r e n o t o n l y u s e d f o r h y p e r t e n s i o n a n d a n g i n a p r o p h y l a xi s ,
b u t a l s o f o r mi g r a i n e p r e v e n t i o n , e s s e n t i a l t r e mo r, t h y r o t o xi c o s i s , g l a u c o ma , a n d
a n xi e t y. - B l o c k a d e c a n a l s o ma n i f e s t a s Q T i n t e r v a l p r o l o n g a t i o n .
R e s t i n g p u l s e a n d b l o o d p r e s s u r e , t e mp e r a t u r e , a n d r e s p i r a t o r y r a t e s h o u l d b e
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7.3 - Bradycardia
t a k e n , a n d i f t h e r e i s c o n c e r n f o r h y p o v o l e mi a , b l o o d p r e s s u r e a n d p u l s e i n b o t h
lying and standing positions (orthostatic evaluation). Cardiac auscultation follows t
palpation of the thyroid gland, and the search for other evidence of thyroid disorder
( s k i n , h a i r, e y e p r o p t o s i s ) . T h e p r e s e n c e o r a b s e n c e o f p u l s e s , a n d s e a r c h f o r b r u i t
a n d a b d o mi n a l a n e u r y s m s h o u l d f o l l o w. I s e d e ma p r e s e n t ? A r e l u n g f i e l d s c l e a r ? I s
there jugular venous distention? Cyanosis?
C. Testing
1. O b t a i n c a r d i a c e n zy me s , c o n g e s t i v e h e a r t f a i l u r e p e p t i d e , e l e c t r o l y t e s , c a l c i u m
a n d ma g n e s i u m, t h y r o i d - s t i mu l a t i n g h o r mo n e , t h y r o xi n e , a n d t r i i o d o t h y r o n i n e
l e v e l s , a s w e l l a s d r u g l e v e l s o f d i g o xi n a n d a n t i a r r h y t h mi c s i f i n d i c a t e d .
2. O b t a i n a n E C G , a n d i f n e c e s s a r y a n e v e n t mo n i t o r ( f o r p a t i e n t s r e p o r t i n g r a r e r
i n t e r mi t t e n t s y mp t o ms ) o r a H o l t e r mo n i t o r ( i n t h o s e p a t i e n t s w h o r e p o r t mo r e
f r e q u e n t s y mp t o ms ) .
I V. Diagnosis
T he different types of bradycardia include:
1. S i n u s b r a d y c a r d 5)
ia N
( o r ma l P - Q R S - T s e q u e n c e a t a r a t e l e s s t h a n 6 0
b e a t s / mi n u t e
2. S i n u s n o d a l b l o c k : a m i s s i n g P Twhaivs ec a n b e i n c o mp l e t e , w h e r e t h e
o c c a s i o n a l s e q u e n c e o f P - Q R S - T i s l o s t , o r c o mp l e t e , w h e r e P w a v e s a r e
c o mp l e t e l y a b s e n t , a n d Q R S - T p r o c e e d s a t a s l o w e s c a p e r a t e f r o m t h e
v e n t r i c u l a r p a c e ma k e r.
P. 1 2 5
3. S i c k s i n u s s y n d r o m
G e n e r a l i ze d a b n o r ma l i t y, a s p r e v i o u s l y d e s c r i b e d , o f
c a r d i a c i mp u l s e f o r ma t i o n t h a t c a n ma n i f e s t a s v a r y i n g c o mb i n a t i o n s o f
bradycardia and tachycardia
4. F i r s t - d e g r e e a t r i o v e n t r i c u l a r ( AV ) b l o c k : c o m m o n E CTGh ef i nPdRi n g
i n t e r v a l r e p r e s e n t s t h e c o n d u c t i o n t i me f r o m t h e s i n u s n o d e t h r o u g h t h e a t r i u m,
AV n o d e , a n d H i s - P u r k i n j e s y s t e m t o t h e d e v e l o p me n t o f v e n t r i c u l a r
d e p o l a r i za t i o n . Va l u e s o v e r 0 . 2 q u a l i f y ( b y c o n v e n t i o n ) .
5. S e c o n d - d e g r e e AV b l oTchki s o c c u r s w h e n a n o r g a n i ze d a t r i a l r h y t h m f a i l s t o
conduct to the ventricle in a 1:1 ratio. T here are several types, including the
following:
a. M o b i t z t y p e 1 ( We n c k e b a c h ) , i n w h i c h t h e E C G s h o w s a s t a b l e P P i n t e r v a l
but a progressive increase in the P R interval until a P wave fails to conduct
b. M o b i t z t y p e I I , w h i c h i s c h a r a c t e r i ze d b y a s t a b l e P P i n t e r v a l w i t h n o
me a s u r a b l e p r o l o n g a t i o n o f t h e P R i n t e r v a l b e f o r e a n a b r u p t c o n d u c t i o n
failure
6. T h i r d - d e g r e e AV b l o c k : r e f e r r e d t o a s c o m p l e t e h eAatrrti abl l aoncdk
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7.3 - Bradycardia
v e n t r i c u l a r a c t i v i t y a r e i n d e p e n d e n t o f e a c h o t h e r.
T here are few indications for intervention in patients with bradycardia who are truly
a s y mp t o ma t i c . W h e r e p o s s i b l e , a n d i f s y mp t o ms o r c o n s i d e r a b l e c a r d i a c r i s k i s
p r e s e n t , o f f e n d i n g me d i c a t i o n s c a n b e c h a n g e d o r r e d u c e d , u n d e r l y i n g c o n d i t i o n s
( e . g . , t h y r o i d a b n o r ma l i t i e s ) s h o u l d b e t r e a t e d , p a c i n g c a n b e c o n s i d e r e d , a n d
patient education and prevention strategies should be tried.
References
1 . M a n g r u m J M , D i ma r c o J P. T h e e v a l u a t i o n a n d ma n a g e me n t o f b r a d y c a r d i a .
JAM A2000;342(10):703709.
4 . M o s s A J . L o n g Q T s y n d r oJme
A M. A 2 0 0 3 ; 2 8 9 ( 1 6 ) : 2 0 4 1 2 0 4 4 .
2 . R o t h B .B et a- bl oc k er t ox i c i t y e M edi. c( iwne
w w. e me d i c i n e . c o m) u p d a t e d A p r i l
15, 2005
3 . A d a n V, C r o w n L A . D i a g n o s i s a n d t r e a t me n t o f s i c k s i n u sAsmy nFdam
r o me .
P hy s i c i an2 0 0 3 ; 6 7 ( 8 ) : 1 7 2 5 1 7 3 2 .
5. Baustian G H, Hodgson S
JM
i nus
. br ady c ar .diFaI R S T C o n s u l t
( w w w. f i r s t c o n s u l t . c o m) u p d a t e d M a y 1 9 , 2 0 0 5 .
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7.4 - Cardiomegaly
> T a b l e o f C o n t e n t s > 7 - C a r d i o v a s c u l a r P r o b l e ms > 7 . 4 - C a r d i o me g a l y
7.4
Cardiomegaly
Lou Ann McStay
I . Background
C a r d i o me g a l y i s t r u e e n l a r g e me n t o f t h e h e a r t b e y o n d t h e u p p e r l i mi t s o f n o r ma l .
T his is a physical finding, not a specific disease. It is the result of other diseases
a n d i s a l mo s t a l w a y s a b n o r ma l . H e a r t s i ze i s e a s i l y, r a p i d l y, a n d mo s t c o mmo n l y
d e t e r mi n e d r a d i o g r a p h i c a l l y b y t h e c a r d i o t h o r a c i c r a t i o ( C T R ) . T h i s i s d e f i n e d a s t h
s u m o f t h e d i s t a n c e s f r o m t h e b o d y mi d l i n e ( a v e r t i c a l l i n e t h r o u g h t h e s p i n o u s
processes) to the furthest left and right heart borders, which is then divided by the
w i d e s t t h o r a c i c d i a me t e r me a s u r e d h o r i zo n t a l l y b e t w e e n t h e i n n e r s u r f a c e s o f t h e
r i b s . T h e C T R i s n o r ma l l y 0 5 0 t o 0 5 5 i n a d u l t s a n d u p t o 0 6 i n i n f a n t s a n d c h i l d r e n .
T h e h e a r t ma y t h e r e f o r e a p p e a r f a l s e l y e n l a r g e d d u e t o a b n o r ma l i t i e s o f t h e c h e s t
wall, or to radiographic technique.
A. Etiology
Ve n t r i c u l a r a n e u r y s m a n d p e r i c a r d i a l e f f u s i o n c a n e n l a r g e t h e c a r d i a c s i l h o u e t t e .
H o w e v e r, mo s t c a r d i a c e n l a r g e me n t i s d u e t o p r e s s u r e o v e r l o a d a n d
P. 1 2 6
mu s c l e h y p e r t r o p h y o f o n e o r mo r e c h a mb e r s o f t h e h e a r t , v o l u me o v e r l o a d w i t h
d i l a t i o n o f c a r d i a c c h a mb e r s , o r c a r d i o my o p a t h y. A s s u c h , c a r d i o me g a l y i s u s u a l l y
t h e r e s u l t o f o t h e r c a r d i o v a s c u l a r d i s o r d e r s ( h y p e r t e n s i o n , i s c h e mi c o r v a l v u l a r h e a
d i s e a s e , f a mi l i a l s t r u c t u r a l a b n o r ma l i t i e s ) b u t c a n a l s o b e c a u s e d b y s y s t e mi c
d i s e a s e s ( a n e mi a , v i r a l o r r i c k e t t s i a l i n f e c t i o n s , b i t e o r s t i n g t o xi n s , me d i c a t i o n s ,
h y p e r t h y r o i d i s m, h y p o t h y r o i d i s m, h y p e r p a r a t h y r o i d i s m, a c r o me g a l y, d i a b e t e s ,
a u t o i mmu n e a n d i n f i l t r a t i v e ( a my l o i d ) d i s e a s e s , a n d me t a s t a s e s ) . I n ma n y c a s e s ,
p a r t i c u l a r l y w i t h l e f t v e n t r i c u l a r h y p e r t r o p h y, c a r d i o me g a l y i s r e v e r s i b l e w i t h
t r e a t me n t o f t h e u n d e r l y i n g c a u s e .
B. Epidemiology
C a r d i o me g a l y c a n b e a n i n c i d e n t a l f i n d i n g w h e n d i s c o v e r e d e a r l y i n t h e d i s e a s e
p r o c e s s . I t c a n o c c u r i n c h i l d r e n a n d y o u n g a d u l t s a s a r e s u l t o f r h e u ma t i c h e a r t
d i s e a s e , i n f e c t i o n s , o r f a mi l i a l c a r d i o my o p a t h i e s , w h e r e t h e p r e s e n t i n g s y mp t o m ma
b e s u d d e n d e a t h f r o m a r r h y t h mi a s , o f t e n b r o u g h t o n b y p h y s i c a l e xe r t i o n , a s i n
s p o r t s . L e f t v e n t r i c u l a r ma s s i s i n c r e a s e d i n o b e s e a d1)o.l eDs uc e nt tos t(h e
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7.4 - Cardiomegaly
h i g h e r p r e v a l e n c e o f i s c h e mi a a n d h y p e r t e n s i o n , mo s t c a s e s a r e i n o l d e r a d u l t s .
I I I . Evaluation
A. History
P a s t me d i c a l h i s t o r y ma y i n c l u d e c o n g e s t i v e h e a r t f a i l u r e , c o r o n a r y d i s e a s e ,
h y p e r t e n s i o n , r h e u ma t i c f e v e r, a n d s y s t e mi c d i s e a s e s . F a mi l y h i s t o r y ma y r e v e a l
h y p e r t e n s i o n , h y p e r l i p i d e mi a , o r s u d d e n d e a t h . S o c i a l h i s t o r y mi g h t i n c l u d e a l c o h o l
o r s u b s t a n c e a b u s e o r a n e xp o s u r e t o a c a r d i a c t o xi n . S y s t e m r e v i e w c o u l d i n c l u d e
f a t i g u e , d i zzi n e s s , d y s p n e a , a n g i n a , c o u g h , e d e ma , n o c t u r i a , a n d l o s s o f w e i g h t w i t
c a r d i a c c a c h e xi a , a s w e l l a s s y mp t o ms mo r e s p e c i f i c t o a n y s y s t e mi c u n d e r l y i n g
disease.
1. C a r d i a cE xa mi n a t i o n ma y n o t e a c h e s t d e f o r mi t y c a u s i n g a p p a r e n t
c a r d i o me g a l y. I n s p e c t i o n ma y n o t e a v i s i b l e h e a v e l a t e r a l t o t h e mi d c l a v i c u l a r
l i n e . P a l p a t i o n o f t h e p o i n t o f ma xi ma l i mp u l s e ( P M I ) b e l o w t h e f i f t h i n t e r c o s t a l
s p a c e a n d l a t e r a l t o t h e mi d c l a v i c u l a r l i n e i s h i g h l y s p e c i f i c f o r l e f t v e n t r i c u l a r
e n l a r g e me n t , i n t h e a b s e n c e o f c h e s t w a l l a b n o r ma l i t i e s . T h e P M I ma y b e
d i f f u s e ( 2 3 c m) a n d ma y b e w e a k i n d i l a t e d c a r d i o my o p a t h y, o r i t ma y b e
h y p e r d y n a mi c i n i n c r e a s e d s y mp a t h e t i c s t a t e s . T h e P M I ma y b e n o n p a l p a b l e
w i t h p e r i c a r d i a l e f f u s i o n . P e r i p h e r a l p u l s e s ma y b e w e a k a n d p u l s u s a l t e r n a n s
detectable with decreased left ventricular function. If the P M I cannot be
appreciated by palpation, the location of the left border of the heart can be
a s c e r t a i n e d b y d u l l n e s s t o p e r c u s s i o n . D u l l n e s s b e y o n d t h e mi d c l a v i c u l a r l i n e
a n d f i f t h i n t e r c o s t a l s p a c e i s a b n o r ma l . H e a r t mu r mu r s o f a b n o r ma l v a l v e s
r e s p o n s i b l e f o r t h e c a r d i o me g a l y ma y b e a u s c u l t a t e d , a s w e l l a s t h e r e g u r g i t a n t
mu r mu r s r e s u l t i n g f r o m d i l a t e d c h a mb e r s . D i mi n i s h e d h e a r t s o u n d s o r f r i c t i o n
r u b s ma y b e n o t e d w i t h p e r i c a r d i a l d i s3e a ns de . S4S g a l l o p s ma y b e h e a r d
w i t h h e a r t f a i l u r e r e s u l t i n g f r o m t h e c a r d i o me g a l y. A r r h y t h mi a s a r e c o mmo n w i t h
c a r d i o me g a l y. H y p e r t e n s i o n , a s a c a u s e o f c a r d i o me g a l y, ma y b e p r e s e n t .
2. E x t r a c a r d i aEc xa mi n a t i o n f i n d i n g s ma y r e l a t e t o t h e u n d e r l y i n g c a u s e s o f
c a r d i o me g a l y ( e . g . , a u t o i mmu n e , i n f e c t i o u s , a n d e n d o c r i n e d i s e a s e s , a n d
a l c o h o l i s m) . T h e t y p i c a l e xt r a c a r d i a c f i n d i n g s o f h e a r t f a i l u r e ( c o u g h , d y s p n e a ,
r a l e s , w h e e ze s , e d e ma , h e p a t o j u g u l a r r e f l u x, j u g u l a r v e n o u s d i s t e n s i o n )
r e s u l t i n g f r o m t h e c a r d i o me g a l y ma y a l s o b e p r e s e n t .
C. Testing
C a r d i o me g a l y i s d e t e c t a b l e b y c h e s t x- r a y, e l e c t r o c a r d i o g r a m ( E C G ) , a n d
e c h o c a r d i o g r a p h y. S e r o l o g i c t e s t i n g p r i ma r i l y i d e n t i f i e s s y s t e mi c c a u s a t i v e d i s o r d e r
a n d B - t y p e n a t r i u r e t i c p e p t i d e i n c r e a s e s w i t h v e n t r i c u l a r 2)
w a. l l s t r e t c h (
1. A s t a n d a r d u p r i g h t p o s t e r i o r a n t e r i o r c h e s t x- r a y w i t h t h e 1 0 t h r i b v i s i b l e a t f u l l
i n s p i r a t i o n i n a n o n r o t a t e d p o s i t i o n w i t h o u t c h e s t d e f o r mi t y i s u s e f u l f o r
d e t e r mi n i n g t h e C T R . H o w e v e r, 2 0 % o f e c h o c a r d i o g r a m- c o n f i r me d c a s e s o f
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7.4 - Cardiomegaly
c a r d i o me g a l y a r e n o t s e e n o n c h e s t2)x-. rDaey t e( r mi n a t i o n o f s p e c i f i c c h a mb e r
e n l a r g e me n t b y x- r a y i s mo s t r e l i a b l e w i t h t h e l e f t a t r i u m, w h e r e e n l a r g e me n t
p r o j e c t s i t p o s t e r i o r l y a n d s u p e r i o r l y, r e s u l t i n g i n i n c r e a s e d c a r d i a c d e n s i t y
c e n t r a l l y, d i s p l a c e me n t o f t h e l e f t ma i n b r o n c h u s s u p e r i o r l y g i v i n g i t a mo r e
h o r i zo n t a l t a k e - o f f , a n d a mo r e r e a d i l y v i s i b l e , a n d c u r v e d , l e f t a t r i a l a p p e n d a g e
T h e s e f i n d i n g s a r e s i g n i f i c a n t , b e c a u s e l e f t a t r i a l e n l a r g e me n t i s a s s o c i a t e d w i t
P. 1 2 7
a p o o r p r o g n o s i s a n d w a s t h e o n l y i d e n t i f i e d i n d e p e n d e n t p r e d i c t o r o f mo r t a l i t y
i n a c o h o r t o f p a t i e n t s w i t h i s c h e mi c c a r d i o my
3) o. p a t h y (
2. T h e E C G i s a l mo s t a l w a y s a b n o r ma l w i t h c a r d i o me g a l y, a l t h o u g h t h e c h a n g e s
a r e o f t e n n o n s p e c i f i c . T h e v o l t a g e a mp l i t u d e i s o f t e n i n c r e a s e d o r t h e a xi s
s h i f t e d . A t r i a l a n d v e n t r i c u l a r a r r h y t h mi a s a r e c o mmo n , w i t h a t r i a l f i b r i l l a t i o n
o c c u r r i n g i n 2 5 % o f p a t i e n t s w i t h c a r d i o my4)o.p B
a tehcya u( s e l e a d1 iVs
d i r e c t l y o v e r t h e a t r i a , t h i s l e a d c a n b e s t i n d i c a t e a t r i a l e n l a r g e me n t . A d i p h a s i c
P w a v e i n 1Vi n d i c a t e s a t r i a l h y p e r t r o p h y. I n r i g h t a t r i a l h y p e r t r o p h y, t h e i n i t i a l
h a l f o f t h e w a v e i s t h e l a r g e s t s e g me n t . I f t h e s e c o n d h a l f o f t h e d i p h a s i c P
w a v e i n l e a d1 Vi s l a r g e r o r w i d e r, t h e r e i s l e f t a t r i a l h y p e r t r o p h y.
5 iAs s l e a d V
o v e r t h e l e f t v e n t r i c l e , a t a l l R w a5vien di ni c aVt e s l e f t v e n t r i c u l a r h y p e r t r o p h y.
T h e r e ma y b e i s c h e mi c c h a n g e s n o t e d o n t h e E C G i n c a r d i o me g a l y a s w e l l .
3. E c h o c a r d i o g r a p h y i s t h e t e s t o f c h o i c e f o r a d e f i n i t i v e d i a g n o s i s o f c a r d i a c
e n l a r g e me n t . I t c a n a l s o y i e l d u s e f u l i n f o r ma t i o n a b o u t s y s t o l i c a n d d i a s t o l i c
f u n c t i o n , w a l l h y p e r t r o p h y, i s c h e mi c a r e a s , a n e u r y s ms , p e r i c a r d i a l e f f u s i o n , a n d
the heart valves.
D. Genetics
T h e r e a r e f a mi l i a l d i l a t e d a s w e l l a s o b s t r u c t i v e c a r d i o my o p a t h i e s a n d a f a mi l i a l r i g
a t r i a l e n l a r g e me n t . S t u d i e s h a v e i n d i c a t e d t h a t t h e p r e s e n c e o f c e r t a i n g e n e a l l e l e s
c a n mo d i f y t h e r i s k o f c a r d i a c h y p e r5)
t r .o pMhayn(y a u t o i mmu n e a n d e n d o c r i n e
c a u s e s o f c a r d i o me g a l y a r e k n o w n t o b e f a mi l i a l .
I V. Diagnosis
T h e d i f f e r e n t i a l d i a g n o s i s o f c a r d i o me g a l y c o n s i s t s ma i n l y i n d i s t i n g u i s h i n g t r u e f r o
f a c t i t i o u s c a r d i o me g a l y d u e t o s u b o p t i ma l c h e s t x- r a y t e c h n i q u e . H e r e t h e mo s t
i mp o r t a n t f a c t o r i s a n a d e q u a t e i n s p i r a t i o n r e v e a l i n g t h e 1 0 t h r i b . O t h e r w i s e , t h e
h e a r t a p p e a r s l a r g e r a n d mo r e g l o b u l a r t o w a r d t h e l e f t . A n t e r i o r p o s t e r i o r f i l ms a n d
p o r t a b l e c h e s t x- r a y s , s h o t c l o s e r t h a n t h e s t a n d a r d u p r i g h t v i e w, a l s o f a l s e l y e n l a r
the heart. Supine views preclude adequate inspiration. Trunk rotation, scoliosis, an
p e c t u s e xc a v a t u m c a n c a u s e a p p a r e n t c a r d i o me g a l y.
An enlarged heart is less efficient in providing adequate blood flow to the body and
i t s e l f . T h e r e f o r e , c l i n i c a l ma n i f e s t a t i o n s o f c a r d i o me g a l y a r e p r i ma r i l y t h o s e o f h e a r
f a i l u r e ( e . g . , d y s p n e a , d i zzi n e s s , f a t i g u e ) . A d d i t i o n a l s y mp t o ms a r e a r r h y t h mi a ,
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7.4 - Cardiomegaly
a n g i n a , a n d s u d d e n d e a t h . O t h e r ma n i f e s t a t i o n s a r e s i g n s a n d s y mp t o ms p a r t i c u l a r
t o t h e s p e c i f i c d i s e a s e c a u s i n g t h e c a r d i o me g a l y.
References
1 . D a mi a s P G , T r i t o s N A . I n c r e a s e d l e f t v e n t r i c u l a r ma s s i n o b e s e a d o l e s c e n t s .
E ur H ear t J2 0 0 5 ; 2 6 ( 2 ) : 2 0 1 2 0 2 .
2 . S u t t e r M , D i e r e k s D B . N e w i n s i g h t s i n t o d e c o mp e n s a t e d h eEamer
r t fga i l u r e .
M ed 2 0 0 5 : 1 8 2 5 .
3 . S a b h a r w a l N , C e mi n R , R a j a n K , e t a l . U s e f u l n e s s o f l e f t a t r i a l v o l u me a s a
p r e d i c t o r o f mo r t a l i t y i n p a t i e n t s w i t h i s c h e mi c c a r d i A
o my
m Jo pCaar
t h di
y. ol
2004;94(6):760763.
4 . We n g e r N K , A b e l ma n W H , R o b e r t s W C . C a r d i o my o p a t h y a n d s p e c i f i c h e a r t
mu s c l e d i s e a s e . I n : H u r s t J W,
T he
e dhear
.
t , ar t er i es , and ,v ei
7 tns
h ed. New
Yo r k , N Y : M c G r a w - H i l l , 1 9 9 0 : 1 2 7 8 1 3 4 7 .
5 . D u r s a n o g l u D . A C E p o l y mo r p h i s m i n h e a l t h y y o u n g Ascutbaj eCcar
t sdi
. ol
2005;60(2):153158.
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7.5 - Congestive Heart Failure
> T a b l e o f C o n t e n t s > 7 - C a r d i o v a s c u l a r P r o b l e ms > 7 . 5 - C o n g e s t i v e H e a r t
Failure
7.5
Congestive Heart Failure
He m ant K. Satpathy
Chhabi Satpathy
I . Background
H e a r t f a i l u r e ( H F ) i s d e f i n e d a s a c o mp l e x c l i n i c a l s y n d r o me r e s u l t i n g f r o m a n y
s t r u c t u r a l o r f u n c t i o n a l c a r d i a c d i s o r d e r t h a t i mp a i r s t h e a b i l i t y o f t h e v e n t r i c l e t o f i
with or eject blood. Often, it is associated with features of vascular congestion. T h
t e r mhear t f ai l uries mo r e a p p r o p r i a t e t h a n c o n g e s t i v e h e a r t f a i l u r e , b e c a u s e ma n y
p a t i e n t s w i t h t h i s d i s e a s e ma y n o t s h o w e v i d e n c e o f c o n g e s t i o n , p a r t i c u l a r l y w h e n
t r e a t e d a g g r e s s i v e l y w i t h d i u r e t i c s . H F i s a ma j o r p u b l i c h e a l t h p r o b l e m i n
i n d u s t r i a l i ze d n a t i o n s . I t a p p e a r s t o b e t h e o n l y c o mmo n c a r d i o v a s c u l a r c o n d i t i o n
t h a t i s i n c r e a s i n g i n p r e v a l e n c e a n d i n c i d e n c e i n N o r t h A me r i c a a n d We s t e r n E u r o p
D e s p i t e i mp r o v e me n t s i n t h e r a p y, t h e mo r t a l i t y i n p a t i e n t s w i t h H F h a s r e ma i n e d
u n a c c e p t a b l y h i g h , ma k i n g e a r l y d e t e c t i o n o f s u s c e p t i b l e i n d i v i d u a1)
l s. i mp e r a t i v e (
A. Etiology
1. T w o i n t e r r e l a t e d p r o c e s s e s , c h a mb e r r e mo d e l i n g a n d s y s t o l i c d y s f u n c t i o n , p l a y
c r i t i c a l r o l e s i n H F, w h i c h ma y b e s y s t o l i c o r d i a s t o l i c . I n p a t i e n t s w i t h H F,
a p p r o xi ma t e l y 7 0 % h a v e s y s t o l i c d y s f u n c t i o n , 1 5 % h a v e d i a s t o l i c d y s f u n c t i o n ,
a n d t h e r e ma i n i n g 1 5 % h a v e c o mb i n e d d y s f u n c t i o n . S y s t o l i c H F i s a s s o c i a t e d
w i t h ma r k e d l y d i l a t e d h e a r t c h a mb e r w i t h r e d u c e d w a l l mo t i o n a n d p r e s e r v e d
f i l l i n g . D i a s t o l i c H F i s a s s o c i a t e d w i t h a n o r ma l c h a mb e r s i ze w i t h n o r ma l
e mp t y i n g b u t i mp a i r e d f i l l i n g .
2. C a u s e s o f H F d i f f e r d e p e n d i n g o n w h e t h e r i t i s s y s t o l i c o r d i a s t o l i c , l e f t - s i d e d o
r i g h t - s i d e d , o r a c u t e o r c h r o n i c . RTeaf be lr et o7 . 5 . f1o r t h e c o mmo n
P. 1 2 9
c a u s e s o f H F. I n t h e U n i t e d S t a t e s a n d We s t e r n E u r o p e , i s c h e mi c h e a r t d i s e a s e
i s r e s p o n s i b l e f o r 7 5 % o f a l l c a s e s o f H F f o l l o w e d b y c a r d i o my o p a t h y. T h e mo s t
c o mmo n c a u s e o f r i g h t - s i d e d H F i s l e f t - s i d e d H F. O t h e r c a u s e s o f r i g h t - s i d e d
H F i n c l u d e p u l mo n a r y h y p e r t e n s i o n , c o r p u l mo n a l e , o r d y s f u n c t i o n o f r i g h t - s i d e d
v a l v e s 2)( .
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TAB L E 7.5.1 Types and Causes of Congestive Heart

Failure
Le ft v e ntricular sy stolic dy sfunction
Causes include:
I s c h e mi c h e a r t d i s e a s e , h y p e r t e n s i o n , a l c o h o l t o xi c i t y, o b e s i t y, v a l v u l a r
d i s e a s e ( e . g . , a o r t i c s t e n o s i s ) , o r c h r o n i c t a c h y d y s r h y t h mi a
Pre se rv e d le ft v e ntricular sy stolic function
Causes include:
T r a n s i e n t s y s t o l i c d y s f u n c t i o n d u e t o a c u t e my o c a r d i a l i s c h e mi a
L e f t a t r i a l h y p e r t e n s i o n d u e t o h i g h - o u t p u t s t a t e s ( e . g . , t h y r o t o xi c o s i s ) ,
v o l u me e xc e s s , o r mi t r a l s t e n o s i s
L e f t v e n t r i c u l a r d i a s t o l i c d y s f u n c t i o n d u e t o h y p e r t e n s i o n , i s c h e mi a , a g i n g ,
o b e s i t y, o r s u s t a i n e d t a c h y a r r h y t h mi a s
M i s c e l l a n e o u s d i s o r d e r s s u c h a s r e s t r i c t i v e c a r d i o my o p a t h y d u e t o i n f i l t r a t i v e
d i s e a s e s ( e . g . , a my l o i d o s i s ) , c o n s t r i c t i v e p e r i c a r d i t i s a n d p e r i c a r d i a l
t a mp o n a d e , a n d p u r e r i g h t - s i d e d h e a r t f a i l u r e
B. Epidemiology
1. I n e v a l u a t i n g p a t i e n t s w i t h H F i t i s i mp o r t a n t t o i d e n t i f y n o t o n l y t h e u n d e r l y i n g
c a u s e s , b u t a l s o t h e p r e c i p i t a t i n g f a c t o r s . M o s t c o mmo n p r e c i p i t a t i n g f a c t o r s
i n c l u d e i n a p p r o p r i a t e t h e r a p y ( i n c r e a s e d s a l t a n d w a t e r i n t a k e , n o n c o mp l i a n c e
w i t h me d i c a t i o n s , o r t a k i n g n o n s t e r o i d a l a n t i - i n f l a mma t o r y d r u g s ) , a c u t e
my o c a r d i a l i n f a r c t i o n , t a c h y a r r h y t h mi a s , w o r s e n i n g o f h y p e r t e n s i o n , p u l mo n a r y
e mb o l i s m, h i g h - o u t p u t s t a t e s ( a n e mi a , p r e g n a n c y, t h y r o t o xi c o s i s ) , c a r d i o t o xi n s
( e . g . , a l c o h o l , c o c a i n e , a n t i a r r h y t h mi c s , c a l c i u m c h a n n e l b l o c k e r s , - b l o c k e r s ,
a n t i n e o p l a s t i c s ) , c a r d i a c i n f e c t i o n s ( my o c a r d i t i s , e n d o c a r d i t i s , p e r i c a r d i t i s ) a n d
s y s t e mi c i l l n e s s ( p n e u mo n i a , a c u t e r e n a l f a i l u r e ) .
2. T h e n a t i o n a l h o s p i t a l d i s c h a r g e s u r v e y e s t i ma t e s t h a t 4 . 8 mi l l i o n A me r i c a n s
h a v e H F. I t s p r e v a l e n c e i s s u b s t a n t i a l a n d i n c r e a s e s s t e e p l y w i t h a g e . T h e
p r e v a l e n c e i n A f r i c a n - A me r i c a n s i s r e p o r t e d t o b e 2 5 % h i g h e r t h a n i n
C a u c a s i a n s3)(. T h i s s t u d y w a s b a s e d o n s y mp t o ma t i c H F, w h i c h c o n s i d e r e d
only half of the participants with left ventricular dysfunction. T here has been an
i n c r e a s e i n t h e p r e v a l e n c e o f H F o v e r t i me , s e c o n d a r y t o a g i n g o f t h e
p o p u l a t i o n a n d i mp r o v e d t r e a t me n t o f v a l v u l a r a n d c o r o n a r y a r 4)
t e .r y d i s e a s e (
T h e i n c i d e n c e o f H F, l i k e t h e p r e v a l e n c e , i n c r e a s e s w i t h a g e . I n t h e
F r a mi n g h a m S t u d y, t h e i n c i d e n c e o f H F a p p r o xi ma t e l y d o u b l e d o v e r e a c h
successive decade of life. Diastolic failure has a better prognosis than systolic
failure.
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I I I . Evaluation
A. History
1. T h i s e v a l u a t i o n s h o u l d i n c l u d e s p e c i f i c c o n s i d e r a t i o n o f n o n c a r d i a c d i s e a s e s
such as collagen-vascular diseases, bacterial or parasitic infection, thyroid
e xc e s s o r d e f i c i e n c y, a n d p h e o c h r o mo c y t o ma . A d e t a i l e d f a mi l y h i s t o r y s h o u l d
b e o b t a i n e d n o t o n l y t o d e t e r mi n e w h e t h e r t h e r e i s f a mi l i a l p r e d i s p o s i t i o n t o
a t h e r o s c l e r o t i c d i s e a s e , b u t a l s o t o i d e n t i f y r e l a t i v e s w i t h c a r d i o my o p a t h y,
s u d d e n u n e xp l a i n e d d e a t h , c o n d u c t i o n s y s t e m d i s e a s e , a n d s k e l e t a l my o p a t h y.
T w e n t y p e r c e n t o f c a s e s o f i d i o p a t h i c d i l a t e d c a r d i o my o p a t h y a r e f a mi l i a l .
2. S y mp t o ms o f H F b e l o n g t o t h r e e b r o a d g r o u p s . D y s p n e a o n e xe r t i o n ( D O E ) ,
o r t h o p n e a , a n d p a r o xy s ma l n o c t u r n a l d y s p n e a ( P N D ) r e s u l t f r o m p u l mo n a r y
c o n g e s t i o n , a n d a r e t h e mo s t s p e c i f i c s y mp t o ms o f H F. D y s p n e a i s t h e mo s t
p r e d o mi n a n t s y mp t o m o f H F, w h e r e a s o r t h o p n e a a n d P N D o c c u r i n a mo r e
a d v a n c e d s t a g e o f H F. S y s t e mi c v a s c u l a r c o n g e s t i o n c a u s e s p e r i p h e r a l e d e ma ,
a s c i t e s , a b d o mi n a l p a i n / f u l l n e s s a n d n a u s e a , w h e r e a s l o w c a r d i a c o u t p u t
c a u s e s f a t i g u e a n d c h a n g e i n me n t a l s t a t u s . O l d e r p a t i e n t s w i t h H F ma y n o t
have D O E because of a sedentary baseline status. T hey often present with
a t y p i c a l s y mp t o ms s u c h a s d r y c o u g h , d a y t i me o l i g u r i a w i t h n o c t u r i a , a n d
confusion.
I n g e n e r a l , p h y s i c a l e xa mi n a t i o n i s mo r e s e n s i t i v e i n d e t e c t i n g a c u t e H F t h a n c h r o n
H F. I n mi l d o r mo d e r a t e l y s e v e r e H F, t h e p a t i e n t a p p e a r s i n n o d i s t r e s s a t r e s t
e xc e p t f e e l i n g u n c o mf o r t a b l e w h e n l y i n g f l a t f o r mo r e t h a n a f e w mi n u t e s . I n s e v e r e
H F, t h e p u l s e p r e s s u r e ma y b e d i mi n i s h e d , a n d t h e d i a s t o l i c a r t e r i a l p r e s s u r e ma y b
e l e v a t e d . I n s e v e r e a c u t e H F, s y s t o l i c h y p o t e n s i o n ma y b e p r e s e n t w i t h c o o l ,
d i a p h o r e t i c e xt r e mi t i e s , a n d C h e y n e - S t o k e s r e s p i r a t i o n . T h e r e ma y b e c y a n o s i s i n
l i p s a n d n a i l b e d s a n d s i n u s t a c h y c a r d i a . E l e v a t e d s y s t e mi c v e n o u s p r e s s u r e ma y b
r e f l e c t e d i n t h e d i s t e n t i o n o f t h e j u g u l a r v e i n s , a n d w h e n t h i s p r e s s u r e i s a b n o r ma l l
h i g h , c h a r a c t e r i s t i c a b d o mi n o j u g u l a r r e f l e x i s s e e n . T h i r d a n d f o u r t h h e a r t s o u n d s
a r e o f t e n a u d i b l e b u t a r e n o t s p e c i f i c f o r H F, a n d p u l s u s a l t e r n a n s ma y b e p r e s e n t .
O t h e r p h y s i c a l f i n d i n g s i n c l u d e p u l mo n a r y r a l e s , c a r d i a c e d e ma ( w h i c h i s u s u a l l y
s y mme t r i c a n d d e p e n d a n t o n H F, p i t t i n g i n a c u t e a n d b r a w n y i n c h r o n i c H F ) ,
h y d r o t h o r a x, a s c i t e s , c o n g e s t i v e h e p a t o me g a l y, o c c a s i o n a l s p l e n o me g a l y, j a u n d i c e ,
a n d c a r d i a c c a c h e xi a .
C. Testing
W h i l e o r d e r i n g l a b o r a t o r y t e s t s f o r p a t i e n t s w i t h H F, i t i s i mp o r t a n t t o s c r e e n f o r
d i a b e t e s , k i d n e y d i s e a s e , a n e mi a , t h y r o t o xi c o s i s , l i v e r d y s f u n c t i o n , a n d e l e c t r o l y t e s
i n c l u d i n g c a l c i u m a n d ma g n e s i u m. S c r e e n i n g f o r h e mo c h r o ma t o s i s ma y a l s o b e g i v e
a c o n s i d e r a t i o n . S e r i a l mo n i t o r i n g o f k i d n e y f u n c t i o n a n d e l e c t r o l y t e s
P. 1 3 0
i s n e e d e d a t f o l l o w - u p v i s i t s . A d d i t i o n a l t e s t s ma y b e o r d e r e d w h e n t h e r e i s s u s p i c i
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o f c e r t a i n d i s e a s e s e i t h e r c a u s i n g o r p r e c i p i t a t i n g t h e H F, s u c h a s c a r d i a c ma r k e r s
the presence of chest pain. Although the sodium and potassium values are usually
n o r ma l i n t h e s e p a t i e n t s , h y p o n a t r e mi a a n d h y p e r k a l e mi a c o u l d b e a s s o c i a t e d w i t h
s e v e r e H F. T h e s e p a t i e n t s c o u l d h a v e h y p o k a l e mi a w h e n t r e a t e d a g g r e s s i v e l y w i t h
l o o p d i u r e t i c s . I mp a i r e d l i v e r f u n c t i o n i s o f t e n a s s o c i a t e d w i t h h e p a t i c c o n g e s t i o n o
cardiac cirrhosis.
1. B r ai n nat r i ur et i c pept i de ( Bi sNtPh)e mo s t s e n s i t i v e a n d s p e c i f i c i n d i c a t o r o f a l l

n a t r i u r e t i c p e p t i d e s f o r v e n t r i c u l a r d i s o r d e r s . I t h a s b e e n u s e d mo r e o f t e n o f l a t
i n o r d e r t o s c r e e n f o r H F, i n a d d i t i o n t o i t s r o l e i n p r o g n o s i s a n d mo n i t o r i n g
t h e r a p y. B N P i s a n i n d e p e n d e n t p r e d i c t o r o f h i g h l e f t v e n t r i c u l a r e n d - d i a s t o l i c
p r e s s u r e ( LV E D P ) . I t s r e l e a s e i s p r o p o r t i o n a l t o v e n t r i c u l a r v o l u me e xp a n s i o n
a n d p r e s s u r e o v e r l o a d . A B N P c u t p o i n t o f 1 0 0 p g / mL d i s t i n g u i s h e s p a t i e n t s
with H F from those without it. An elevated B N P on patients with clinical H F and
n o r ma l s y s t o l i c f u n c t i o n i n e c h o s u b s t a n t i a t e s t h e d i a g n o s i s o f d i a s t o l i c h e a r t
dysfunction.
2. C ar di ac c at het er i zatwi on
i t h c o r o n a r y a r t e r i o g r a p h y ma y b e c o n s i d e r e d i n
p a t i e n t s w i t h H F, a n g i n a , a n d k n o w n o r s u s p e c t e d c o r o n a r y a r t e r y d i s e a s e b u t
w i t h o u t a n g i n a , w h o a r e c a n d i d a t e s f o r r e v a s c u l a r i za t i o n .
3. C hes t x - r ayt y p i c a l l y s h o w s c a r d i o me g a l y, e xc e p t i n d i a s t o l i c H F. O t h e r x- r a y
f i n d i n g s o f H F i n c l u d e p u l mo n a r y v a s c u l a r r e d i s t r i b u t i o n , i n t e r s t i t i a l a n d a l v e o l a
p u l mo n a r y e d e ma , a n d l e s s o f t e n , c l o u d l i k e a p p e a r a n c e w i t h c o n c e n t r a t i o n o f
f l u i d a r o u n d t h e h i l u m, g i v i n g b u t t e r f l y o r b a t w i n g a p p e a r a n c e . S e r i a l c h e s t xr a y s a r e n o t r e c o mme n d e d f o r r o u t i n e f o l l o w - u p .
4. E l ec t r oc ar di ogr aphy
(E C G) r e v e a l s a t r i a l f i b r i l l a t i o n i n a p p r o xi ma t e l y 2 0 % t o
3 0 % p a t i e n t s w i t h H F. E C G ma y a l s o s h o w e v i d e n c e o f o l d i n f a r c t i o n , l e f t
v e n t r i c u l a r h y p e r t r o p h y, l e f t a t r i a l e n l a r g e me n t , a r r h y t h mi a s o t h e r t h a n a t r i a l
fibrillation, low voltage, or bundle branch block.
5. E c hoc ar di ogr aphy

i s t h e s i n g l e mo s t e f f e c t i v e d i a g n o s t i c t e s t i n t h e e v a l u a t i o n
o f p a t i e n t s w i t h H F. I t i s o n e o f t h e f a s t e s t g r o w i n g p r o c e d u r e s i n c a r d i o l o g y. I n
s y s t o l i c H F, t h e e j e c t i o n f r a c t i o n i s l e s s t h a n 4 0 % , w h e r e a s i n d i a s t o l i c H F, t h e
e j e c t i o n f r a c t i o n c o u l d b e d e c e i v i n g l y n o r ma l . H i g h LV E D P s u g g e s t s d i a s t o l i c
H F. T h i s a l s o p r o v i d e s i n f o r ma t i o n a b o u t a s s o c i a t e d p e r i c a r d i a l , my o c a r d i a l , o r
v a l v u l a r d i s e a s e . R e a s s e s s me n t o f H F i s o n l y n e e d e d i n p a t i e n t s w h o h a d a
c h a n g e i n c l i n i c a l s t a t u s o r r e c e i v e d t r e a t me n t t h a t mi g h t h a v e h a d a s i g n i f i c a n t
effect on heart function.
6. M a g n e t i c r e s o n a n c e i ma g i n g , r a d i o n u c l i d e v e n t r i c u l o g r a p h y, o r e n d o my o c a r d i a l
biopsy is used for diagnosis or finding the cause of H F less often in clinical
p r a c t i c e . B e c a u s e o f f r e q u e n t c o mo r b i d l u n g pul
d i smonar
e a s e , y f unc t i on
testing should be considered in older patients before dyspnea is attributable to
H F.
References
1 . H o K K , P i n s k y J L , K a n n e l W B . T h e e p i d e mi o l o g y o f H F : t h e F r a mi n g h a m
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S t u d y.J A m C ol l C ar di 1ol9 9 3 ; 2 2 : 6 A 1 3 A .
2 . N o h r i a A , L e w i s E , S t e v e n s o n LW. M e d i c a l ma n a g e me n t o f a d v a n c e d H F.
JAM A2002;287:628640.
3 . A H A . H e a r t & s t r o k e s t a t i s t i c a l uEp cdonomi
a t e . c c as e of c ar di ov as c ul ar
di s eas .e D a l l a s T X : A H A , 2 0 0 1 .
4 . C l e l a n d J G , K h a n d A , C l a r k A . T h e H F e p i d e mi c : e xa c t l y h oEwurb i g i s i t ?
H ear t J2 0 0 1 ; 2 2 : 6 2 3 6 2 6 .
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7.6 - Heart Murmurs, Diastolic
> T a b l e o f C o n t e n t s > 7 - C a r d i o v a s c u l a r P r o b l e ms > 7 . 6 - H e a r t M u r mu r s ,
Diastolic
7.6
Heart M urmurs, Diastolic
J e l y n W. L u
I . Background
A d i a s t o l i c h e a r t mu r mu r o c c u r s d u r i n g h e a r t mu s c l e r e l a xa t i o n s t a r t i n g w i t h o r a f t e r
S 2 a n d e n d i n g b e f o r e o r a f1t e(r* SS2/ ~ mu r mu r ~ /1S* ) . D i a s t o l i c mu r mu r s a r e
t y p i c a l l y d u e t o t h e s t e n o s i s o f t h e mi t r a l o r t r i c u s p i d v a l v e s , o r t h e r e g u r g i t a t i o n o f
t h e a o r t i c o r p u l mo n a r y v a l v e s . T h e d i a s t o l i c mu r mu r s h o u l d mo r e o f t e n t h a n n o t b e
c o n s i d e r e d p a t h o l o g i c a l o r a s s o c i a t e d w i t h s t r u c t u r a l c a r d i a c a b n o r ma l i t i e s a n d
t h e r e f o r e , w a r r a n t s f u r t h e r e v a l u a t i o n . T h e s e mu r mu r s c a n b e c l a s s i f i e d i n t o f o u r
categories:
A. Early diastolic murmur

w h i c h s t a r t s w i t 2h aSn d p e a k s i n t h e f i r s t t h i r d o f d i a s t o l e
B. M id-diastolic murmur
w h i c h s t a r t s a f t e2r aSn d e n d s p r i o r t1o S
C. Late diastolic murmur
which starts in the concluding part of diastole during atrial contraction and persists
t o S1
D. Continuous murmur
w h i c h e xt e n d s f r o m s y s t o l e i n t o d i a s t o l e . S u c h a mu r mu r r e s u l t s f r o m b l o o d f l o w
c o n t i n u i n g f r o m a h i g h - p r e s s u r e t o a l o w e r - p r e s s1)u .r e a r e a (
I I . Pathophysiology/ Evaluation/ Diagnosis

A. Early diastolic murmur
1. A o r t i c r e g u r g i t a t i o n ( A R )
a. P a t h o p h y s i o l o.g yI n s u f f i c i e n t c l o s i n g o f t h e a o r t i c v a l v e l e a f l e t s , w h i c h
can be caused by alteration or dilatation of the aortic root and ascending
a o r t a . A R c a u s e s g r e a t e r r e s u l t a n t e n d - d i a s t o l i c l e f t v e n t r i c u l a r v o l u me ,
r e s u l t i n g i n a r e s i d u a l l e f t v e n t r i c u l a r d i l a t a t i o n a n d h y p e r t r o p h y. E v e n t u a l l y
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peripheral signs include the gradient between systolic and diastolic

pressure increases (widened arterial pulse pressure), observable carotid
p u l s e , s u p r a s t e r n a l p u l s a t i o n s , r a p i d r i s e a n d f a l l o f p u l s e ( w a t e r - h a mme r
pulse), flushing pulsations of nail beds (Q uincke's pulse), to-and-fro
mu r mu r f r o m s l i g h t s t e t h o s c o p e c o mp r e s s i o n o n t h e f e mo r a l a r t e r y
( D u r o zi e z' s mu r mu r ) , h e a d b o b w i t h e a c h p u l s e ( d e M u s s e t ' s s i g n ) , a n d
r a p i d e j e c t i o n o f l a r g e s t r o k e v o l u me ( p i s t o l - s h o t f e mo r a l a r t e r y
s o u n d )2,3)
( .
b. E p i d e m i o l o g. yM o r e c o mmo n i n me n t h a n w o me n ; mo r e c o mmo n i n a d u l t s
o l d e r t h a n 6 0 y e a r s , i f a s s o c i a t e d w i t h M a r f a n ' s s y n d r o me , E h l e r s - D a n l o s
s y n d r o me , a u t o s o ma l r e c e s s i v e o r X - l i n k e d i n h e r i t a n c e , c o l l a g e n - v a s c u l a r
d i s e a s e s , t h e r e ma y b e f a mi l i a l p r e d i s p3)
o s. i t i o n (
c. C a u s e s 2,3,4)
(
R h e u ma t i c h e a r t d i s e a s e , c o n g e n i t a l h e a r t d i s e a s e
(bicuspid aortic valve, prolapsed aortic cusp with ventricular septal defect),
c o l l a g e n - v a s c u l a r d i s e a s e s ( s y s t e mi c l u p u s e r y t h e ma t o s u s ) , c o n n e c t i v e t i s s u e d i s e a s e ( M a r f a n ' s s y n d r o me , T u r n e r ' s s y n d r o me , p s e u d o xa n t h o ma
e l a s t i c u m, a n k y l o s i n g s p o n d y l i t i s , E h l e r s - D a n l o s s y n d r o me , p o l y my a l g i a
r h e u ma t i c a ) , a s c e n d i n g a o r t i c a n e u r y s m, a o r t i t i s ( s y p h i l i s , T a k a y a s u ' s
a r t e r i t i s , g r a n u l o ma t o u s a o r t i t i s ) , c y s t i c me d i a l n e c r o s i s , R e i t e r ' s s y n d r o me ,
my xo ma t o u s a o r t i c v a l v e , c a l c i f i c c h a n g e s i n a o r t i c v a l v e , a n o r e c t i c d r u g s o r
p a t i e n t s t r e a t e d w i t h d e xf e n f l u r a mi n e o r p h e n t e r mi n e / f e n f l u r a mi n e , i n f e c t i v e
e n d o c a r d i t i s , a o r t i c d i s s e c t i o n , t r a u ma , h y p e r t e n s i o n , e n d - s t a g e r e n a l
d i s e a s e ( t r a n s i e n t mu r mu r f r o m f l u i d o v e r l o a d )
d. H i s t o r yI s t h e r e a h i s t o r y o f r h e u ma t i c f e v e r ( R F ) ? C h e c k f o r r e c u r r e n t
s t r e p t h r o a t , s h o r t n e s s o f b r e a t h a t r e s t ( s i g n s o f c a r d i a c d e c o mp e n s a t i o n ) ,
c h e s t p r e s s u r e d u r i n g p h y s i c a l a c t i v i t y, p a l p i t a t i o n s ( s y mp t o ma t i c
a r r h y t h mi a s ) , l o w e r e xt r e mi t y s w e l l i n g , t r a u ma ( r i s k o f a o r t i c d i s s e c t i o n ) ,
intravenous drug use, recent dental work (risk of endocarditis), use of
f e n f l u r a mi n e / d e xf e n f l u r a mi n e ( F e n - P h e n ) o r o t h e r w e i g h t l o s s d r u g s , h i s t o r y
o f l e n s d i s l o c a t i o n / l o n g s l e n d e r l i mb s ( p r e s e n c e o f M a r f a n ' s s y n d r o me ) ,
s e xu a l l y t r a n s mi t t e d d i s e a s e s ( r i s k o f s y p h i l i s ) , a n d l a x j o i n t s ( E h l e r s D a n l o s s y n d r o me
3)).(
P. 1 3 2
e. D i a g n o s i sD e c r e s c e n d o , b l o w i n g c h a r a c t e r, l o w i n t e n s i t y, h i g h - p i t c h e d
d i a s t o l i c mu r mu r a t t h e l e f t s t e r n a l b o r d e r o r o v e r t h e r i g h t s e c o n d
intercostal space while the patient is sitting and leaning forward with breath
h e l d i n d e e p e xp i r a t i o n , r a d i a t i n g t o t h e c a r d i a c a p e x, i n c r e a s e s w i t h
h a n d g r i p o r s q u a t t i n g ; c a n e v e n p r e s e n t w i t h a mu s i c a l q u a l i t y d i a s t o l i c
w h o o p . A d i a s t o l i c r u mb l e a t t h e v e n t r i c u l a r a p e x i s k nAous
w nt i na s t h e
F l i nt mur mur
(1) .
f. P r e s e n t a t i o H
n e a r t f a i l u r e s y mp t o ms , e xe r t i o n a l d y s p n e a , f a t i g u e ,
p a r o xy s ma l n o c t u r n a l d y s p n e a , p u l mo n a r y e d e ma , a n g i n a p e c t o r i s , a t y p i c a l
c h e s t p a i n , e l e v a t e d s y s t o l i c a n d d e c r e a s e d d i a s t o l i c b l o o3)d p r e s s u r e (
2. P u l m o n a r y r e g u r g i t a t i o n ( P R )
a. P a t h o p h y s i o l o.g yI n s u f f i c i e n t c l o s u r e o f t h e p u l mo n a r y v a l v e l e a f l e t s
234 / 652
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b. C a u s e sU s u a l l y s e c o n d a r y t o p u l mo n a r y h y p e r t e n s i o n . H o w e v e r, p u l mo n a r y
regurgitation (P R) can also be caused by the idiopathic dilatation of the
p u l mo n a r y a r t e r y, s t a t u s p o s t s u r g e r y o r p o s t b a l l o o n v a l v u l o p l a s t y, r i g h t s i d e d e n d o c a r d i t i s , o r c o n g e n i t a l a b s e n c e o f t h e p u l mo n a r y v a l v e .
c. D i a g n o s i sD e c r e s c e n d o , b l o w i n g c h a r a c t e r, h i g h - p i t c h e d d i a s t o l i c
(Graham Steell) heard best over the left sternal border and left second and
third intercostal space (vs. AR, which is heard in the right intercostals). T he
P R mu r mu r i n c r e a s e s w i t h i n s p i r a t i o n .
d. P r e s e n t a t i o R
n i g h t v e n t r i c u l a r v o l u me a n d p r e s s u r e o v e r l o a d ; a s i g n i f i c a n t
r i g h t v e n t r i c u l a r h e a v e , e l e v a t e d j u g u l a r v e n o u s 1,3)
pressure (
3. L e f t a n t e r i o r d e s c e n d i n g a r t e r y s t e n
Doosc iks' s mu r mu r i s s i mi l a r t o t h a t o f
AR and is caused by turbulent flow across the stenotic coronary arteries.
A u s c u l t a t e f o r mu r mu r a t t h e l e f t s e c o n d o r t h i r d i n t e r c o s t a l s p a c e a n d t h e l e f t
s t e r n a l b o r d e1)
r .(
4. S 3 g a l l o p / v e n t r i c u l a r g a lTl ho ips c a n b e h e a r d a s a n e a r l y d i a s t o l i c g a l l o p . I n
c h i l d r e n i t i s u s u a l l y n o r ma l ; h o w e v e r, i n a d u l t s i t i s t y p i c a l l y a s i g n o f h e a r t
failure.
B. M id-diastolic murmur
1. M i t r a l s t e n o s i s ( M S )
a. P a t h o p h y s i o l o.g yF i b r o s i s a n d l a c k o f mo b i l i t y o f t h e mi t r a l v a l v e l e a f l e t s ,
w h i c h c a u s e s a n i mp e d i me n t i n f l o w f r o m t h e l e f t a t r i u m t o t h e l e f t v e n t r i c l e ,
r e s u l t i n g i n i n c r e a s e d p r e s s u r e i n t h e l e f t a t r i u m, p u l mo n a r y v a s c u l a t u r e ,
and right side of the heart
b. E p i d e m i o l o g. yP r e d o mi n a t e l y w o me n
c. C a u s e sR F, c o n g e n i t a l d i s e a s e , ma l i g n a n t c a r c i n o i d , me t h y s e r g i d e t h e r a p y,
s y s t e mi c l u p u s e r y t h e ma t o s u s , r h e u ma t o i d a r t h r i t i s
d. H i s t o r yI s t h e r e a h i s t o r y o f R F ( mo s t c o mmo n c a u s e ) , b r e a t h l e s s n e s s w i t h
e xe r t i o n ( mo s t c o mmo n s y mp t o m) , p a l p i t a t i o n s ( a t r i a l f i b r i l l a t i o n a s s o c i a t e d
w i t h mi t r a l s t e n o s i s [ M S ] ) , t r a v e l h i s t o r y ( R F i s c o mmo n i n d e v e l o p i n g
c o u n t r i e s ) , a n d / o r mi g r a i n e me d i c a t i o n s ( me t h y l s e r g i d e ) ?
e. D i a g n o s i sL o u d o p e n i n g s n a p f o l l o w e d b y a l o w - p i t c h e d d i a s t o l i c r u mb l e
h e a r d b e s t a t t h e a p e x w h i l e p a t i e n t l y i n g o n l e f t s i d e ; mu r mu r i n c r e a s e d
w i t h e xp i r a t i o n
f. P r e s e n t a t i onM i t r a l f a c i e s ( v a s o c o n s t r i c t i o n w i t h r e s u l t a n t p i n k i s h - p u r p l e
p a t c h e s o n c h e e k s ) , h e a r t f a i l u r e s y mp t o ms ( e xe r t i o n a l d y s p n e a , e d e ma ) ,
a t r i a l f i b r i l l a t i o n , c h e s t p a i n , h o a r s e n e s s f r o m l e f t a t r i a l e n l a r g e me n t a n d
c o mp r e s s i o n o f r e c u r r e n t l a r y n g e a l n e r v e , 4)f a. t i g u e (
2. T r i c u s p i d s t e n o s i s
a. P a t h o p h y s i o l o.g yF i b r o s i s a n d l a c k o f mo b i l i t y o f t h e t r i c u s p i d v a l v e
leaflets with resultant elevation of pressure in the right atrium and jugular
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v e i n s ; mo r e c o mmo n i n mi d - d i a s t o l e w h e n i n a t r i a l f i b r i l l a t i o n ( i n n o r ma l
s i n u s r h y t h m, mu r mu r i s l a t e d i a s t o l i c )
b. C a u s e sU s u a l l y i n a s s o c i a t i o n w i t h M S , R F, c a r c i n o i d h e a r t d i s e a s e , r i g h t
a t r i a l my xo ma1) (
c. D i a g n o s i sB e g i n s w i t h a t r i c u s p i d o p e n i n g s n a p a n d a s s o c i a t e d w i t h a mi d d i a s t o l i c r u mb l e b e s t a u s c u l t a t e d a t t h e l e f t s t e r n a l b o r d e r ; mu r mu r
increases with inspiration (C arvallo's sign)
P. 1 3 3
d. P r e s e n t a t i o C
n an present with atrial fibrillation, right-sided heart failure,
h e p a t o me g a l y, a s c i t e s , d e p e n d e n t e d e ma .
3. A t r i a l m y x o mTah i s i s t h e mo s t c o mmo n p r i ma r y h e a r t t u mo r c o n s i s t i n g o f a
b e n i g n g e l a t i n o u s g r o w t h . T h e g r o w t h s c a n c a u s e a n o b s t r u c t i o n o f t h e mi t r a l
a n d t r i c u s p i d v a l v e s p r e s e n t i n g w i t h c h e s t p a i n , d y s p n e a , e d e ma , a n d s y n c o p e .
L e f t a t r i a l my xo ma mu r mu r i s s i mi l a r t o t h a t o f M S ( a s r i g h t a t r i a l my xo ma i s
s i mi l a r t o t r i c u s p i d s t e n o s i s ) . A t r i a l my xo ma mu r mu r s c a n c h a n g e w i t h a l t e r a t i o n
o f p o s i t i o n a n d a l s o p r e s e n t w i t h t h e t u mo r p l o p .
4. I n c r e a s e d f l o w a c r o s s t h e a t r i o v e n t r i c u l aTr hvi sa livs eo t h e r w i s e k n o w n a s
a f l ow mur mur
(1) .
C. Late diastolic murmur

1. M i t r a l s t e n o s iTs h. i s mu r mu r b e c o me s a l a t e d i a s t o l i c mu r mu r w h e n a t r i a l
contraction increases the pressure and flow at the end of the diastole.
2. T r i c u s p i d s t e n o s T
i sh. i s l a t e d i a s t o l i c mu r mu r o c c u r s d u r i n g s i n u s r h y t h m.
3. A t r i a l m y x o m aT.h i s mu r mu r c a n a l s o p r e s e n t i n l a t e d i a s t o l e a s w e l l a s mi d diastole.
4. L e f t - t o - r i g h t s h u n t
5. C o m p l e t e h e a r t b l o cT kh.i s i s a s h o r t l a t e mu r mu r cRayl tl and'
e d s mur mur
.
6. S 4/ a t r i a l g a l l o p
T .h i s o c c u r s f r o m t h e r e s i s t a n c e o f a t r i a l f i l l i n g a f t e r a n a t r i a l
c o n t r a c t i o n . I t p r e s e n t s a s a l a t e d i a s t o l i c a t r i a l g a l l o p t h a t ma y b e a s s o c i a t e d
w i t h my o c a r d i a l d i s e a s e , c o r o n a r y a r t e r y d i s e a s e , o r h y p e r t e n s i o n , a n d
i n c r e a s e s s o u n d w i t h d e e p i n s p i r1)a .t i o n (
D. Continuous murmur
T h i s mu r mu r b e g i n s i n s y s t o l e , p e a k s2,n ae na dr Sc o n t i n u e s i n t o a l l o r p a r t o f t h e
diastole.
1. P a t e n t d u c t u s a r t e r i o sT uhsi s c o n t i n u o u s ma c h i n e l i k e mu r mu r i s a u s c u l t a t e d
b e s t a t t h e l e f t mi d d l e a n d l e f t u p p e r s t e r n a l b o r d e r a n d s e c o n d i n t e r c o s t a l
space with radiation to the back.
2. O t h e r c a u s e s o f c o n t i n u o u s m u r Tmhuersse i n c l u d e a o r t o p u l mo n a r y w i n d o w,
shunts (through an atrial septal defect), arteriovenous fistulas, constriction of
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s y s t e mi c / p u l mo n a r y a r t e r i e s , c o a r c t a t i o n o f t h e a o r t a , ma mma r y s o u f f l i n
p r e g n a n c y, v e n o u s h u m, a n d p e r i c a r d i a l f r i c1)
t i .o n r u b (
I I I . Laboratory Tests
A.
T he test of choice is echocardiographytransthoracic or transesophageal (better to

test for endocarditis and evaluate vegetations).
B.
O t h e r p o s s i b l e d i a g n o s t i c t o o l s i n c l u d e e l e c t r o c a r d i o g r a m, c h e s t x- r a y, c a r d i a c
c a t h e t e r i za t i o n , b l o o d c u l t u r e s , c o mp l e t e b l o o d c o u n t w i t h d i f f e r e n t i a l a n d e r y t h r o c y
s e d i me n t a t i o n r a t e i n s u s p e c t e d c a s e s o f e n d o c a r d i t i s , r a d i o n u c l i d e a n g i o g r a p h y
when echocardiography is nonconclusive, aortogram for evaluation of aorta; chest
c o mp u t e d t o mo g r a p h y ( t o d i a g n o s e d i s s e c t i o n ) , a o r t i c ma g n e t i c r e s o n a n c e i ma g i n g
( t o d i a g n o s e d i s s e c t i o n ) , e xe r c i s e t e s t i n g , t i s s u e t e s t i n g / D N A t e s t i n g f o r g e n e t i c
a b n o r ma l i t i e s , a n d s e r o l o g i c t e s t s f o r c o l l a g e n - v a s c u l a r d i s e a s e2,4,5)
a n d .s y p h i l i s (
References
1 . C h a t t e r j e e K a nAuus
. c ul t at i on of c ar di ac mur. mur
U p sTo D at: eo n l i n e 1 3 . 2
( w w w. u p t o d a t e . c o m) ; u p d a t e d o n A p r i l 2 0 0 5 .
2. Cheitlin M D. Surgery for chronic aortic regurgitation: when should it be
c o n s i d e r e dA? m F am P hy s i c i an
2001;64:17091714.
3 . S c h e r g e r J E , O ' H a n l o n K M , J o n e s R CA, or
e tt iacl .r egur gi t at. i on
F I R S T C ons ul( th t t p : / / w w w. f i r s t c o n s u l t . c o m. c u h s l . c r e i g h t o n . e d u / ?
t y p e = me d & i d = 0 1 0 1 4 2 0 2 ) U p d a t e d o n We d n e s d a y, J u n e 2 5 , 2 0 0 5 .
4 . C u n n i n g h a m R , C o r r e t t i M , H e n r iVal
c h vW.
ul ar hear t di s eas e i n pat i ent s wi t h
end- s t age r enal di s eas
. U pe t o D at: eo n l i n e 1 3 . 2 . ( w w w. u p t o d a t e . c o m) ; u p d a t e d
on April 2005.
5 . F e r r i F F, S a v e r D F, H o d g s o n J M ,Mei tt raall . s t enos. iFsI R S T C ons ul t
( h t t p : / / w w w. f i r s t c o n s u l t . c o m. c u h s l . c r e i g h t o n . e d u / ? t y p e = me d & i d = 0 1 0 1 4 2 3 1 )
U p d a t e d o n F r i d a y, M a y 2 7 , 2 0 0 5 .
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tmdmss
7.7 - Heart Murmurs, Systolic
> T a b l e o f C o n t e n t s > 7 - C a r d i o v a s c u l a r P r o b l e ms > 7 . 7 - H e a r t M u r mu r s , S y s t o l i c
7.7
Heart M urmurs, Systolic
C a r l o s A . P r e n d e s J r.
S y s t o l i c mu r mu r s a r e a c o mmo n f i n d i n g d u r i n g t h e p h y s i c a l e xa mi n a t i o n . W i l l i a m
J a me s ' a d v i c e i s c e r t a i n l y a p p l i c a b l e : T h e a r t o f b e i n g w i s e i s t h e a r t o f k n o w i n g
what to overlook. T he reality of today's healthcare system dictates judicious use o
r e s o u r c e s , s o f a mi l i a r i t y w i t h c o mmo n l y e n c o u n t e r e d s y s t o l i c mu r mu r s a n d a n
e v a l u a t i o n p r o t o c o l a i d i n e f f i c i e n t , c o s t - e f f e c t i v e u s e o f me d i c a l i ma g i n g t e c h n o l o g
I . Background
A h e a r t mu r mu r c a n b e d e f i n e d a s t u r b u l e n t f l o w t h r o u g h a v a l v e o r v e s s e l . M u r mu r s
a r i s e w i t h a b n o r ma l b l o o d f l o w p a t t e r n s . S e v e r a l c o n d i t i o n s ma y g i v e r i s e t o a u d i b l e
mu r mu r s : f a s t f l o w t h r o u g h a n o r ma l v a l v e ( s u c h a s a n e mi a o r t a c h y c a r d i a ) , a b n o r m
narrowing of a valve (aortic stenosis), flow into a dilated vessel (dilated aortic root
i n c o mp e t e n t v a l v e s a l l o w i n g b a c k w a r d f l o w ( r e g u r g i t a t i o n [ e . g . , mi t r a l r e g u r g i t a t i o n ]
a n d p e r s i s t e n t o r a c q u i r e d a b n o r ma l c o mmu n i c a t i o n s b e t w e e n h e a r t c h a mb e r s o r
a r t e r i e s ( v e n t r i c u l a r s e p t a l d1)
e f.e c t ) (
S y s t o l i c mu r mu r s a r e g e n e r a l l y l e s s l i k e l y t o b e s i g n i f i c a n t t h a n d i a s t o l i c mu r mu r s ,
h o w e v e r, t h e y a r e a l s o mu c h mo r e f r e q u e n t l y e n c o u n t e r e d . M u r mu r s w i t h c e r t a i n
c h a r a c t e r i s t i c s ma y b e s a f e l y d o c u me n t e d a n d mo n i t o r e d , a n d o t h e r s w a r r a n t u r g e n t
w o r k u p , a s w e l l a s e v e r y t h i n g i n b e t w e e n . K n o w l e d g e o f c o mmo n p h y s i c a l f i n d i n g s
and their associated pathology aid in the prudent and efficient evaluation of new
mu r mu r s . D i a s t o l i c mu r mu r s a r e n e v e r c o n s i d e r e d i n n o c e n t a n d s h o u l d a l w a y s
w a r r a n t i n v e s t i g a t i o n ; t h e y a r e a d d r e s s e d e l s e w h e r e i n t1,2)
h i s . v o l u me (
I I I . Evaluation
A. History
A t h o r o u g h a n d c o mp l e t e h i s t o r y i s p a r a mo u n t w h e n e v a l u a t i n g a p r e v i o u s l y u n h e a r d
mu r mu r.
1. I t i s i mp o r t a n t t o n o t e a n y p r e v i o u s h i s t o r y o f v a l v u l a r d i s e a s e , o r c o n g e n i t a l
heart disease. A social history of intravenous drug use and tobacco abuse, and
o t h e r h i g h - r i s k b e h a v i o r s s h o u l d b e d o c u me n t e d . P a s t me d i c a l h i s t o r y o f
a n e mi a , t h y r o i d d i s o r d e r s , a n d p r e g n a n c y a r e n o t a b l y a s s o c i a t e d w i t h f l o w
238 / 652
tmdmss
mu r mu r s .
2. R e v i e w o f s y s t e ms s h o u l d i n c l u d e a n y c o mp l a i n t s o f d y s p n e a , d y s p n e a o n
e xe r t i o n , d i a p h o r e s i s , c h e s t p a i n , p a l p i t a t i o n s , e d e ma , e xa c e r b a t i n g a n d
r e l i e v i n g f a c t o r s , a s w e l l a s a t i me l i n e o f s y mp t o ms a n d a n y p r o g r e s s i o n . O t h e r
s y s t e mi c c o mp l a i n t s t o a d d r e s s i n c l u d e w e i g h t l o s s , f e v e r s a n d / o r c h i l l s , n e w
r a s h e s , a n d e a s y f a t i g a b i l i t y.
3. I t i s r e a s o n a b l eobs
t o er v emu r mu r s t h a t a r e n o t a s s o c i a t e d w i t h s u b j e c t i v e
c o mp l a i n t o r o t h e r p h y s i c a l f i n d i n g s s u c h a s a t h r i l l . G e n e r a l l y t h e y a r e
mi d s y s t o l i c a n d h a v e s e v e r a l p o s s i b l e e t i o l o g i e s ; i n c l u d i n g , v i b r a t i o n o f t h e
p u l mo n a r y l e a f l e t s , p u l mo n i c l e a f l e t s , o r s y s t e mi c a r t e r i e s . O l d e r p a t i e n t s ma y
a l s o h a v e v i b r a t i o n s o f t h e a o r t i c c u s p s . C h a n g e s i n t h e mu r mu r s o v e r t i me ma y
warrant further workup, especially if associated with worsening of your patient's
c o n d i t i o n2)( .
Medical educators report their belief that teaching cardiac auscultation is of great
i mp o r t a n c e ; h o w e v e r, a n a t i o n w i d e s u r v e y p r e s e n t e d i n 1 9 9 1 r e v e a l e d l e s s t h a n h a l
o f i n t e r n a l me d i c i n e a n d c a r d i o l o g y p r o g r a ms o f f e r e d a n y s t r u c t u r e d t e a c h i n g o f
a u s c u l t a t i o n3) (. A c c u r a c y o f t h e c a r d i a c e xa mi n a t i o n i mp r o v e s w i t h s t r u c t u r e d
t e a c h i n g a n d r e p e t i t 4,5)
i o n .(
1. A l w a y s b e g i n w i t h t h e v i t a l s i g n s a n d o b s e r v i n g y o u r p a t i e n t f o r s i g n s o f
distress.
2. T h e c a r d i a c e xa mi n a t i o n i n v o l v e s a u s c u l t a t i o n a t t h e f i v e c a r d i a c l i s t e n i n g
points, palpation of carotid and peripheral pulses, and description of the heart
s o u n d s . M u r mu r s a r e d e s c r i b e d b y t h e i r l o c a t i o n , l o u d n e s s , a n d t i mi n g . I t s h o u l d
also
P. 1 3 5
b e n o t e d i f t h e mu r mu r c h a n g e s w i t h i n s p i r a t i o n / e xh a l a t i o n . A r e v i e w o f t h e
l i s t e n i n g p o s t s a n d t h e i r a s s o c i a t e d mu r mu r s i sT af ob ul en d7 .i7n. 1
TAB L E 7.7.1 Physical Exam ination Findings of

Systolic Heart Murm urs
Sy stolic
m urm ur
De scription
Mane uv e rs
Location
he ard
Ao r t i c s t e n o s i s M i d s y s t o l i c c r e s c e n d oT
- r a n s mi t s t o
decrescendo
carotids,
thrill with
e xp i r a t i o n
Right
sternal
b o r d e r,
second rib
space
Tricuspid
L ower left
P ansystolic, plateau Increases
239 / 652
tmdmss
regurgitation
with
inspiration
sternal
b o r d e r,
heard
below
zy p h o i d
process
Mitral
regurgitation
M o s t c o mmo n ,
holosystolic
Squatting
ma y
increase
A p e x,
radiates to
a xi l l a
Mitral valve
prolapse
L a t e s y s t o l i c , ma y
hear click
Stand and
i n h a l e a my l
nitrate
A p e x, u s e
diaphragm
P u l mo n i c
stenosis
M i d s y s t o l i c , d i a mo n d -I n c r e a s e s
shaped
with
inspiration,
t r a n s mi t s t o
suprasternal
notch
Ve n t r a l s e p t a l
defect
Harsh holosystolic
Hypertrophic
H a r s h mi d s y s t o l i c ,
c a r d i o my o p a t h y e j e c t i o n mu r mu r
Left sternal
b o r d e r,
second to
third rib
space
R adiates to Apex to
right sternal right
border
sternal
border
Decreases
with
handgrip
and
squatting
Aortic area
t o A p e x,
does not
radiate
beyond
heart
3. M u r mu r s s h o u l d b e g r a d e d a c c o r d i n g t o v o l u me a n d t h e a b s e n c e o r p r e s e n c e o f
a p a l p a b l e t h r 1)
i l l. (
a. G r a d e IF a i n t a n d d i f f i c u l t t o h e a r, w h i c h i s e a s i l y mi s s e d a n d r e q u i r e s
special effort to hear
b. G r a d e I IS o f t mu r mu r mo r e e a s i l y h e a r d , e s p e c i a l l y w i t h e xp e r i e n c e
c. G r a d e I I AI f a i r l y l o u d mu r mu r t h a t i s n o t a s s o c i a t e d w i t h a t h r i l l
d. G r a d e I VA l o u d mu r mu r w h i c h i s a s s o c i a t e d w i t h a t h r i l l
e. G r a d e VA l o u d e r mu r mu r w i t h a t h r i l l , b u t a s t e t h o s c o p e i s s t i l l r e q u i r e d t o
hear it
240 / 652
tmdmss
f. G r a d e V IA mu r mu r w i t h a t h r i l l t h a t i s s o l o u d i t c a n b e h e a r d b e f o r e t h e
stethoscope touches the chest.
C. Testing
G r a d e I I I / V I a n d h i g h e r mu r mu r s , a s w e l l a s mu r mu r s a s s o c i a t e d w i t h s y mp t o ms o f
c a r d i a c d i s e a s e s h o u l d b e r e f e r r e d f o r e c h o c a r2)d .i o g r a m (
1. E l e c t r o c a r d i o g r a p h y ( E C G ) i s r e a d i l y a v a i l a b l e a n d i n e xp e n s i v e . I t p r o v i d e s
u s e f u l i n f o r ma t i o n a b o u t t h e p r e s e n c e o f v e n t r i c u l a r h y p e r t r o p h y, my o c a r d i a l
i s c h e mi a , p r i o r i n f a r c t , a n d a r r h y t2)
h mi
. as (
2. P o s t e r o a n t e r i o r a n d l a t e r a l x- r a y s o f t h e c h e s t y i e l d u s e f u l i n f o r ma t i o n a b o u t
v e n t r i c u l a r s i ze , p u l mo n a r y c o n g e s t i o n , a n d c a l c i f i c a t i o n s o f t h e v a s c u l a t u r e .
T h e y a l s o ma y s h o w f i n d i n g s c o n s i s t e n t w i t h c o n g e s t i v e h e a r t f a i l u r e ( C H F ) ,
c h r o n i c o b s t r u c t i v e p u l mo n a r y d i s e a s e , a n d / o r p n2)e .u mo n i a (
3. U s e f u l l a b o r a t o r y t e s t s ma y i n c l u d e : c o mp l e t e b l o o d c o u n t ( a n e mi a , i n f e c t i o n ,
r e d b l o o d c e l l d e s t r u c t i o n ) , t h y r o i d - s t i mu l a t i n g h o r mo n e
( h y p o t h y r o i d i s m/ h y p e r t h y r o i d i s m) , b l o o d c u l t u r e ( e n d o c a r d i t i s ) , t r o p o n i n I
c r e a t i n e k i n a s e ( C K ) / C K - M B ( ma r k e r s o f my o c a r d i a l d a ma g e ) , a n d C H F
peptide.
P. 1 3 6
4. T r a n s t h o r a c i c e c h o c a r d i o g r a p h y i s n o n i n v a s i v e , a v a i l a b l e , a n d y i e l d s i mp o r t a n t
i n f o r ma t i o n b y i ma g i n g c a r d i a c s t r u c t u r e s a n d me a s u r i n g f l o w r a t e a n d d i r e c t i o n
through cardiac valves.
5. T r a n s e s o p h a g e a l e c h o c a r d i o g r a p h y a n d c a r d i a c c a t h e t e r i za t i o n a r e a l s o
available if the diagnosis is still uncertain after reviewing the transthoracic
e c h o c a r d i o g r a m. T h e s e a r e i n v a s i v e a n d e xp e n s i v e s t u d i e s a n d n o t a p p r o p r i a t e
f o r i n i t i a l w o r k u p o f s y s t o l i c h e a r t mu r mu r s w i t h o u t c o mp e l l i n g e v i d e n c e s u c h a s
e v i d e n c e o f a c u t e c o r o n a r y s y n d r o me .
I V. Diagnosis
A.
S y s t o l i c mu r mu r s a r e c o mmo n l y e n c o u n t e r e d d u r i n g r o u t i n e e xa mi n a t i o n . A l w a y s
ma i n t a i n a h i g h i n d e x o f s u s p i c i o n w h e n a u s c u l t a t i n g c a r d i a c s o u n d s . D i l i g e n t
a t t e n t i o n t o t h e p a t i e n t ' s h i s t o r y a n d t h o r o u g h p h y s i c a l e xa mi n a t i o n o f t e n y i e l d s t h e
i n f o r ma t i o n n e c e s s a r y f o r a n a c c u r a t e d i a g n o s i s , e v e n b e f o r e a d d i t i o n a l s t u d i e s a r e
o b t a i n e d . I f a n y d o u b t r e ma i n s a b o u t w h a t s h o u l d b e d o n e n e xt , r e me mb e r D r.
O s l e r 's a d v i cC
e ar
e mor e par t i c ul ar l y f or t he i ndi v i dual pat i ent t han f or t he
s pec i al f eat ur es of t he di s. eas e
B.
S e v e r a l w e b s i t e s h a v e b e e n d e v e l o p e d t h a t ma y b e u s e f u l f o r l e a r n i n g t o r e c o g n i ze
s p e c i f i c mu r mu r s . S o me i n c l u d e t h e f o l l o w i n g :
241 / 652
tmdmss
1. T h e A u s c u l t a t i o n A s s i s that nt pt : / / w w w. w i l k e s . me d . u c l a . e d u / i n e x. h t m
2. C a r d i a c A u s c u l t a t ihotnt p : / / e g e n e r a l me d i c a l . c o m/ e g e n e r a l me d i c a l /
l i s t o h e a r mu r. h t ml
3. V i r t u a l S t e t h o s c ohptet p : / / s p r o j e c t s . mmi . mc g i l l . c a / mv s / mv s t e t h . h t m.
References
1 . G r e e n b e r g e r N , H i n t h o r nH iDs t. or y t ak i ng and phy s i c al ex ami.nat
S t i. on
L o u i s , M O : M o s b y Ye a r B o o k , 1 9 9 3 .
2 . K a s p e r D , B r a u n w a l d E , W i l s o n J DH, ar
e dr iss. on' s pr i nc i pal s of i nt er nal
medi c i ne
. N e w Yo r k , N Y : M c G r a w - H i l l , 2 0 0 5 .
3 . B o n o w R O , C a r a b e l l o B , d e L e o n A C J r, e t a l . A C C / A H A g u i d e l i n e s f o r t h e
ma n a g e me n t o f p a t i e n t s w i t h v a l v u l a r h e a r t d i s e a s e : a r e p o r t o f t h e A me r i c a n
C o l l e g e o f C a r d i o l o g y / A me r i c a n H e a r t A s s o c i a t i o n T a s k F o r c e o n P r a c t i c e
G u i d e l i n e s ( C o mmi t t e e o n M a n a g e me n t o f P a t i e n t s W i t h Va l v u l a r H e a r t D i s e a s e ) .
J A m C ol l C ar di 1ol9 9 8 ; 3 2 : 1 4 8 6 1 5 8 8 .
4 . M a n g i o n e S , N i e ma n L Z , G r a c e l y E , e t a l . T h e t e a c h i n g a n d p r a c t i c e o f
c a r d i a c a u s c u l t a t i o n d u r i n g i n t e r n a l me d i c i n e a n d c a r d i o l oAgnn
y t Ir nt
a i er
n i nng .
M ed 1 9 9 3 ; 11 9 : 4 7 5 4 .
5. F avrat B , P ecoud A, J aussi A. Teaching cardiac auscultation to trainees in
i n t e r n a l me d i c i n e a n d f a mi l y p r a c t i c e : d o e sBiM
t w
CoM
r ked
? E duc
2004;4:5.
242 / 652
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7.8 - Hypertension
> T a b l e o f C o n t e n t s > 7 - C a r d i o v a s c u l a r P r o b l e ms > 7 . 8 - H y p e r t e n s i o n
7.8
Hypertension
Ray m ond D. He lle r
H y p e r t e n s i o n ( H T N ) i s a l e a d i n g c a u s e o f mo r b i d i t y a n d mo r t a l i t y i n t h e U n i t e d
S t a t e s . E s t i ma t e s s u g g e s t t h a t o f a l l i n d i v i d u a l s w i t h H T N , 3 4 % a r e r e c e i v i n g
a d e q u a t e t h e r a p y, 2 5 % a r e r e c e i v i n g i n a d e q u a t e t h e r a p y, 11 % a r e r e c e i v i n g n o
t h e r a p y, a n d 3 0 % o f i n d i v i d u a l s w i t h H T N d o n o t k n o w t h e y h a v e t1)
h e. c o n d i t i o n (
I . Background
A c c o r d i n g tTohe S ev ent h R epor t of t he J oi nt N at i onal C ommi t t ee on P r ev ent i on,
D et ec t i on, E v al uat i on, and Tr eat ment of H i gh B l ood, tPh er esc lsaur
s sei f i c a t i o n o f
b l o o d p r e s s u r e i n a d u l t s a g e 1 8 y e a r s a n d o l d e r i s a s fToal lbolwe s7(. s8e). 1:e
P. 1 3 7
TAB L E 7.8.1 Classification of Blood Pressure in U S

Adults 18 Years of Age and Older
Blood pre ssure
classification
N o r ma l
Sy stolic blood
pre ssure (m m Hg)
Diastolic blood
pre ssure (m m Hg)
<120
<80
Prehypertension
120139
8089
Stage 1 H T N
140159
9099
Stage 2 H T N
160
100
H T N, hypertension.
D e p a r t me n t o f H e a l t h a n d H u ma n S e r v i c e , N a t i o n a l I n s t i t u t eTs he
of Health,
s ev ent h r epor t of t he J oi nt N at i onal C ommi t t ee on pr ev ent i on, det ec t i on,
ev al uat i on, and t r eat ment of hi gh bl ood pr es s ur e.
w w w. n h l b i . n i h . g o v / g u i d e l i n e s / h y p e r t e n s i o n
243 / 652
tmdmss
7.8 - Hypertension
A.
A s y s t o l i c b l o o d p r e s s u r e o f 1 2 0 mm H g o r l e s s i s c o n s i d e r e d n o r ma l .
B.
A new classification, prehypertension is a systolic blood pressure of 120 to 139

mm H g a n d / o r a d i a s t o l i c b l o o d p r e s s u r e o f 8 0 t o 8 9 mm H g . P r e h y p e r t e n s i o n i s n o t
a d i s e a s e c a t e g o r y a n d i n d i v i d u a l s w h o f a l l i n t o t h iar
s ec anot
t e gc ao nr yd i d a t e s f o r
d r u g t h e r a p y. P a t i e n t s w h o f a l l i n t o t h i s c a t e g o r y a r e i d e n t i f i e d a s b e i n g c o n s i d e r e d
a t h i g h r i s k o f H T N a n d s h o u l d b e c o u n s e l e d r e g a r d i n g l i f e s t y l e mo d i f i c a t i o n t o
reduce their risk of developing H T N in the future.
C.
S t a g e 1 H T N i s d e f i n e d a s a s y s t o l i c b l o o d p r e s s u r e o f 1 4 0 t o 1 5 9 mm H g a n d / o r a
d i a s t o l i c b l o o d p r e s s u r e o f 9 0 t o 9 9 mm H g .
D.
S t a g e 2 H T N i s d e f i n e d a s a s y s t o l i c b l o o d p r e s s u r e o f 1 6 0 mm H g o r g r e a t e r b a s e d
o n t h e a v e r a g e o f 2 o r mo r e r e a d i n g s t a k e n a t e a c h o f 2 o r mo r e v i s i t s a f t e r i n i t i a l
s c r e e n i n g2)( .
A. Etiology
T h e c a u s e o f 9 0 % t o 9 5 % o f t h e c a s e s o f H T N i s n 1)
o t . kPnaotwi enn(t s w i t h n o
i d e n t i f i a b l e c a u s e o f H T N a r e s a i d t o h a v e p r i ma r y o r e s s e n t i a l H T N . P a t i e n t s w i t h
specific structural organ or gene defect responsible for their H T N are classified as
h a v i n g s e c o n d a r y H T3)N. (
B. Epidemiology
A p p r o xi ma t e l y o n e i n t h r e e a d u l t s i n t h e U n i t e d S t a t e s h a v e H T N , a n d a p p r o xi ma t e l
2 8 % o r 5 9 mi l l i o n o f A me r i c a n a d u l t s 1 8 y e a r s o f a g e a n d o l d e r h a v e
p r e h y p e r t e n s i o n1) . (A p p r o xi ma t e l y 5 0 % o f i n d i v i d u a l s w h o s u f f e r a f i r s t my o c a r d i a l
infarction and two-thirds who undergo a first stroke have a blood pressure higher
t h a n 1 6 0 / 9 5 mm H g . C o mp a r e d t o n o r mo t e n s i v e i n d i v i d u a l s , t h e r e l a t i v e r i s k f o r a
s t r o k e i s f o u r t i me s g r e a t e r f o r i n d i v i d u a l s w i t h a s y s t o l i c b l o o d p r e s s u r e o f 1 6 0 mm
H g o r h i g h e r a n d / o r a d i a s t o l i c b l o o d p r e s s u r e o f 9 5 mm H g o r h i g h e r. I n d i v i d u a l s a
t h e l o w e r e d u c a t i o n a l a n d i n c o me l e v e l s t e n d t o h a v e h i g h e r l e v e l s o f b l o o d
pressure.
I I I . Evaluation
A. History
A f t e r t h e d o c u me n t a t i o n o f H T N i s e s t a b l i s h e d , a d e t a i l e d h i s t o r y s h o u l d r e v e a l
i n f o r ma t i o n a b o u t t a r g e t e n d o r g a n d a ma g e , t h e p a t i e n t ' s c a r d i o v a s c u l a r r i s k s t a t u s
a n d s e c o n d a r y c a u s e s o f H T N . Q u e s t i o n s t o a s s e s s t h e e xt e n t o f t a r g e t o r g a n
d a ma g e s h o u l d i n c l u d e a n y h i s t o r y o f a c u t e e p i s o d i c o r p r o g r e s s i v e v i s u a l c h a n g e ,
o r o c c i p i t a l h e a d a c h e s u p o n a r i s i n g i n t h e mo r n i n g t h a t f a d e i n s e v e r a l h o u r s .
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7.8 - Hypertension
R e v i e w a n y s y mp t o ms c o n s i s t e n t w i t h a n g i n a p e c t o r i s , i s c h e mi c h e a r t d i s e a s e ,
peripheral vascular disease, congestive heart failure, cerebral vascular disease,
r e t i n o p a t h y, a n d n e p h r o p a3)t h. yS (y mp t o ms s u g g e s t i v e o f a s e c o n d a r y c a u s e o f
H T N n e e d t o b e e xp l o r e d . T h e p a t i e n t s ' a g e a n d g e n d e r s h o u l d a l s o b e c o n s i d e r e d .
A h i g h e r p e r c e n t a g e o f me n t h a n w o me n h a v e H T N u n t i l 5 5 y e a r s o f a g e . A f t e r 5 5
y e a r s o f a g e , a mu c h h i g h e r p e r c e n t a g e o f w o me n h a v e H T N2) t. hO
a nt h me
er n (
i mp o r t a n t me d i c a l h i s t o r y i n c l u d e s p r e v i o u s d o c u me n t a t i o n o f H T N b y a h e a l t h c a r e
p r o v i d e r. P a r t o f t h e h e a l t h h i s t o r y mu s t a d d r e s s c o mo r b i d h e a l t h c o n d i t i o n s s u c h a
d i a b e t e s me l l i t u s , d y s l i p i d e mi a , o b e s i t y, a n d f a mi l y h i s t o r y.
P. 1 3 8
1. M e d i c a t i o n h i s t oIrt yi s i mp o r t a n t t o r e v i e w c u r r e n t o r p a s t a n t i h y p e r t e n s i v e
me d i c a t i o n u s e . P a t i e n t s s t o p a n t i h y p e r t e n s i v e t h e r a p y f o r v a r i o u s r e a s o n s ,
including stated reasons such as they were feeling better or they could not
a f f o r d t h e me d i c a t i o n . I t i s a l s o n e c e s s a r y t o r e v i e w t h e h e r b a l a n d o v e r - t h e counter (O T C) therapies the patient currently uses or previously used. Many
O T C c o u g h a n d c o l d r e me d i e s a n d w e i g h t l o s s p i l l s c o n t a i n p r o d u c t s t h a t c a n
p o t e n t i a l l y e l e v a t e b l o o d p r e s s u r e . U n f o r t u n a t e l y, s o me O T C h e r b a l t h e r a p i e s
ma y i n t e r a c t w i t h p r e s c r i p t i o n me d i c a t i o n s , a l t e r i n g t h e me t a b o l i s m, s e r u m l e v e l
e f f e c t i v e n e s s , a n d s i d e e f f e c t s o f ma n y p r e s c r i p t i o n me d i c a t i o n s , a n d p o t e n t i a l l y
c o mp l i c a t i n g t r e a t me n t . P o t e n c i e s o f h e r b a l me d i c a t i o n s c a n v a r y d r a ma t i c a l l y
b e t w e e n ma n u f a c t u r e r s . T h e r e f o r e , h e a l t h c a r e p r a c t i t i o n e r s s h o u l d h a v e r e a d y
a c c e s s t o a g u i d e f o r h e r b a l me d i c i n e s t h a t d e s c r i b e s t h e i r o r i g i n , u s e s , s i d e
e f f e c t s , me t a b o l i s m, e xc r e t i o n , t o xi c i t i e s , a n d k n o w n p o t e n t i a l p r e s c r i p t i o n d r u g
i n t e r a c t i o n s . I n a d d i t i o n , ma n y p r e s c r i p t i o n me d i c a t i o n s ma y e l e v a t e b l o o d
pressure.
2. S o c i a l h i s t o rFya c t o r s t o r e v i e w i n c l u d e t o b a c c o , c a f f e i n e , a l c o h o l , a n d i l l i c i t
d r u g u s e . A d i e t a r y h i s t o r y ma y i l l u mi n a t e a p p r o xi ma t e q u a n t i t i e s o f s a t u r a t e d
f a t s a n d s o d i u m i n t a k e . T i me s p e n t i n e xe r c i s e a n d t y p e o f l e i s u r e a c t i v i t i e s
s h o u l d b e d e t e r mi n e d .
1. B l o o d p r e s s u r e m e a s u r e mEernr ot r s c a n b e mi n i mi ze d b y c h o o s i n g t h e p r o p e r
s i ze b l o o d p r e s s u r e c u f f a n d u t i l i zi n g a s t a n d a r d i ze d t e c h n i q u e . G u i d e l i n e s f o r a
p r o p e r s i ze b l o o d p r e s s u r e c u f f a r e lTi satbelde i7n. 8 . 2P a t i e n t p r e p a r a t i o n
g u i d e l i n e s t o mi n i mi ze e r r o n e o u s b l o o d p r e s s u r e r e a d i n g s Taar eb l lei s t e d i n
7 . 8 . 3. T o d e t e r mi n e a p r o p e r c u f f i n f l a t i o n p r e s s u r e , p a l p a t e t h e r a d i a l p u l s e
w h i l e i n f l a t i n g t h e c u f f b l a d d e r. W h e n t h e r a d i a l p u l s e i s n o l o n g e r p a l p a b l e ,
n o t e t h i s p r e s s u r e o n t h e ma n o me t e r a n d a d d 3 0 mm H g t o i t . D e f l a t e t h e c u f f
a n d w a i t a p p r o xi ma t e l y 3 0 s e c o n d s b e f o r e a t t e mp t i n g c u f f r e i n f l a t i o n . P l a c e t h e
s t e t h o s c o p e b e l l l i g h t l y o v e r t h e b r a c h i a l a r t e r y, i n f l a t e t h e c u f f r a p i d l y t o t h e
a b o v e d e t e r mi n e d p r e s s u r e . D e f l a t e i t a t a r a t e o f a b o u t 2 t o 3 mm H g p e r
second. Note the pressure level when at least two consecutive beats are first
heard. T his level is the systolic pressure. Continue lowering the cuff pressure
u n t i l t h e s o u n d s b e c o me mu f f l e d a n d d i s a p p e a r, a g a i n n o t e t h e p r e s s u r e o n t h e
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7.8 - Hypertension
ma n o me t e r. C o n f i r m s o u n d d i s a p p e a r a n c e b y l i s t e n i n g a s t h e p r e s s u r e f a l l s a n
a d d i t i o n a l 2 0 mm H g . I f n o f u r t h e r s o u n d s o c c u r, d e f l a t e t h e c u f f r a p i d l y t o ze r o
T he disappearance point represents the diastolic blood pressure. Measure the
b l o o d p r e s s u r e i n e a c h 4)
a r .m (
2. O t h e r i m p o r t a n t f e a t uTr ehse p h y s i c a l e xa mi n a t i o n s h o u l d i n c l u d e t h e e y e
e xa mi n a t i o n l o o k i n g f o r r e t i n a l c h a n g e s c o n s i s t e n t w i t h H T N . P a l p a t e t h e t h y r o i
t o e v a l u a t e f o r ma s s e s , e n l a r g e me n t , o r a s y mme t r y. T h e c a r d i o v a s c u l a r
e xa mi n a t i o n s h o u l d i n c l u d e p a l p a t i o n a n d a u s c u l t a t i o n o f t h e c a r o t i d a n d f e mo r a
p u l s e s , n o t i n g s y mme t r y o f p u l s e s t r e n g t h o r b r u i t s . P a l p a t e d i s t a l
P. 1 3 9
e xt r e mi t y p u l s e s a n d p e r f o r m a c o mp l e t e c a r d i a c e xa mi n a t i o n , n o t i n g a n y c a r d i a
h e a v e , mu r mu r s , g a l l o p s , r u b s , j u g u l a r v e n o u s d i s t e n s i o n , a n d 1i n t e n s i t y o f S
a n d S2 h e a r t s o u n d s . A b d o mi n a l e xa mi n a t i o n s h o u l d e v a l u a t e f o r b r u i t s ,
p u l s a t i l e a n d n o n p u l s a t i l e ma s s e s , o r r e n a l e n l a r g e me n t . A c o mp l e t e p u l mo n a r y
e xa mi n a t i o n s h o u l d b e p e r f o r me d . E xa mi n a t i o n o f e xt r e mi t i e s s h o u l d n o t e
e d e ma , a n d c h a n g e s c o n s i s t e n t w i t h p e r i p h e r a l v a s c u l a r d i s e a s e . A t h o r o u g h
n e u r o l o g i c a n d s k i n e xa mi n a t i o n s h o u l d b e p e r f o r me d .
TAB L E 7.8.2 Selecting the Correct Blood

Pressure Cuff
W i d t h o f t h e i n f l a t a b l e b l a d d e r o f t h e c u f f s h o u l d b e a p p r o xi ma t e l y
4 0 % o f t h e u p p e r a r m c i r c u mf e r e n c e ( a p p r o xi ma t e l y 1 2 1 4 c m i n t h e
average adult)
L e n g t h o f i n f l a t a b l e b l a d d e r s h o u l d b e a p p r o xi ma t e l y 8 0 % o f u p p e r
a r m c i r c u mf e r e n c e ( a l mo s t l o n g e n o u g h t o e n c i r c l e t h e a r m)
If aneroid, recalibrate periodically before use.
B i c k l e y L S , S zi l a g y i P G . B e g i n n i n g t h e p h y s i c a l e xa mi n a t i o n : g e n e r a l
s u r v e y a n d v i t a l s i g n sG
. ui
I n de
: t o phy s i c al ex ami nat i on and hi s t or y
t ak i ng, 8 t h e d . P h i l a d e l p h i a , PA : L i p p i n c o t t W i l l i a ms & W i l k i n s , 2 0 0 3 : 7 5
78.
TAB L E 7.8.3 Preparing to Measure Blood

Pressure
I d e a l l y, a s k t h e p a t i e n t t o a v o i d s mo k i n g o r d r i n k i n g c a f f e i n a t e d
b e v e r a g e s f o r 3 0 mi n b e f o r e t h e b l o o d p r e s s u r e i s t a k e n a n d t o r e s t
f o r a t l e a s t 5 mi n .
C h e c k t o ma k e s u r e t h e e xa mi n i n g r o o m i s q u i e t , w a r m, a n d
c o mf o r t a b l e .
M a k e s u r e t h e a r m s e l e c t ef rdeei s of c l ot hi .ngT h e r e s h o u l d b e n o
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7.8 - Hypertension
arteriovenous fistulas for dialysis, scarring from prior brachial artery

c u t d o w n s , o r s i g n s o f l y mp h e d e ma ( s e e n a f t e r a xi l l a r y n o d e
dissection or radiation therapy).
Palpate the brachial artery to confirm that it has a viable pulse.
P o s i t i o n t h e a r m s o t h a t t h e b r a c h i a l a r t e r y, a t t h e a n t e c u b i t a l
c r e a s e i sat hear t l ev
elr o u g h l y l e v e l w i t h t h e f o u r t h i n t e r s p a c e a t
i t s j u n c t i o n w i t h t h e s t e r n u m.
If the patient is seated, rest the arm on a table a little above the
patient's waist; if standing, try to support the patient's arm at the
mi d c h e s t l e v e l .
B i c k l e y L S , S zi l a g y i P G . B e g i n n i n g t h e p h y s i c a l e xa mi n a t i o n : g e n e r a l
s u r v e y a n d v i t a l s i g n sG
. ui
I n de
: t o phy s i c al ex ami nat i on and hi s t or y
t ak i ng, 8 t h e d . P h i l a d e l p h i a , PA : L i p p i n c o t t W i l l i a ms & W i l k i n s , 2 0 0 3 : 7 5
78.
C. Testing
1. B a s i c l a b o r a t o r y t e s t s , w h e n e v a l u a t i n g a p a t i e n t w i t h H T N , i n c l u d e u r i c a c i d ,
u r i n a l y s i s i n c l u d i n g s c r e e n i n g f o r mi c r o a l b u mi n u r i a , c o mp l e t e b l o o d c o u n t , s e r u m
c r e a t i n i n e , p o t a s s i u m, c a l c i u m, a n d b l o o d u r e a n i t r o g e n . A f a s t i n g s e r u m g l u c o s
a n d a c o mp l e t e l i p o p r o t e i n p r o f i l e s h o u l d b e o b t a i n e d . T h e l i p o p r o t e i n p r o f i l e
should include a total cholesterol, triglyceride, low-density lipoprotein
c h o l e s t e r o l , a n d h i g h - d e n s i t y l i p o p r o t e i n c h o l e s t e r o l . T h e e s t i ma t e d g l o me r u l a r
filtration rate (G F R) or creatinine clearance should be calculated using availabl
f o r mu l a s ( s eTea b l e 7 . 8 ). .4 I f a s e c o n d a r y c a u s e o f H T N i s s u s p e c t e d , t h e
appropriate specific laboratory workup should be included.
TAB L E 7.8.4 Equations for Estim ation of GF R

from Plasm a Creatinine Concentration
cr) (P
1. E q u a t i o n f r o m t h e M o d i f i c a t i o n o f D i e t i n R e n a l D i s a
ease Study
- 1 . 1 5 4 ( a g e- )
E s t i ma t e d G F R ( mL / mi n / 1 .27) 3=m1 . 8 6 c( P
r)
0.203
M u l t i p l y b y 0 . 7 4 2 f o r w o me n
M u l t i p l y b y 1 . 2 1 f o r A f r i c a n - A me r i c a n s
2.
M u l t i p l y b y 0 . 8 5 f o r w o me n
aK a s p e r D L , B r a u n w a l d E , F a u c i A S ,H eart rai sl .on' s pr i nc i pl es of

i nt er nal medi c ,i ne
16th ed. Part 8; Chapter 230. McGraw-Hill
C o mp a n i e s , 2 0 0 5 .
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7.8 - Hypertension
P. 1 4 0
2. A n e l e c t r o c a r d i o g r a m ma y a s s i s t w i t h r e c o g n i zi n g a p r e v i o u s l y d a ma g e d
my o c a r d i u m, r h y t h m d i s t u r b a n c e s , o r c h a n g e s c o n s i s t e n t w i t h l e f t v e n t r i c u l a r
h y p e r t r o p h y ( LV H ) .
3. I ma g i n g s t u d i e s ma y b e u s e f u l . A c h e s t x- r a y ma y b e u s e f u l w h e n a t t e mp t i n g t o
e v a l u a t e t h e p a t i e n t f o r c a r d i o me g a l y, b u t a l i mi t e d e c h o c a r d i o g r a m w i l l g i v e
mo r e d e t a i l e d i n f o r ma t i o n a b o u t c a r d i a c f u n c t i o n a n d LV H .
D. Genetics
T o d a t e , g e n e t i c s t u d i e s h a v e n o t i d e n t i f i e d a n y s i n g l e g e n e o r c o mb i n a t i o n o f g e n e
that appreciably accounts for H T N in the general population.
I V. Diagnosis
1. E s s e n t i a l H T N
2. S e c o n d a r y H T N
a. R e n o v a s c u l a r H T N
i. S t e n o s i s o f ma i n r e n a l a r t e r i e s a n d / o r ma j o r b r a n c h e s i s r e s p o n s i b l e
for 2% to 5% of patients with H T N.
i i. I n i t i a l w o r k u p i n v o l v e s D o p p l e r u l t r a s o n o g r a p h y w i t h me a s u r e me n t o f
t h e i n t r a r e n a l r e s i s t a n c e i n d e x.
i i i. T h i s d i a g n o s i s i n c l u d e s t h e s u b g r o u p s p r e e c l a mp s i a a n d e c l a mp s i a .
b. R e n a l p a r e n c h y m a l H D
T iNs e a s e s t h a t i n j u r e r e n a l p a r e n c h y ma l t i s s u e
r e s u l t i n i n f l a mma t o r y a n d f i b r o t i c c h a n g e s o f s ma l l i n t r a r e n a l v e s s e l s ,
thereby causing decreased profusion and resultant H T N.
c. P r i m a r y a l d o s t e r o n i A
s mt u mo r o r b i l a t e r a l a d r e n a l h y p e r p l a s i a s h o u l d b e
sought.
d. C u s h i n g ' s s y n d r o m e
i. T r u n c a l o b e s i t y, f a t i g a b i l i t y, p u r p l i s h a b d o mi n a l s t r i a e , a me n o r r h e a ,
H i r s u t i s m, e d e ma , g l u c o s u r i a , mo o n f a c i e s , a n d b u f f a l o h u mp
i i. M o s t c o mmo n i n t h i r d o r f o u r t h d e c a d e o f l i f e
i i i. F a i l u r e t o s u p p r e s s c o r t i s o l l e v e l w i t h d e xa me t h a s o n e c h a l l e n g e
e. P h e o c h r o m o c y t o m a
i. M o s t c o mmo n i n y o u n g t o mi d a d u l t l i f e
i i. H e a d a c h e s , p a l p i t a t i o n s , e xc e s s i v e s w e a t i n g , i mp a i r e d g l u c o s e
t o l e r a n c e , h y p e r c a l c e mi a , w e i g h t l o s s , a n d a n xi e t y
i i i. T w e n t y - f o u r h o u r u r i n e c o l l e c t i o n a s s a y e d f o r v a n i l l y l ma n d e l i c a c i d ,
248 / 652
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7.8 - Hypertension
me t a n e p h r i n e , a n d f r e e c a t e c h o l a mi n e s
f. C o a r c t a t i o n o f t h e a o r t a
i. C a r d i a c mu r mu r, p o s s i b l y h e a r d i n t h e b a c k , o v e r s p i n o u s p r o c e s s e s ,
and lateral thorax
i i. D e c r e a s e d , d e l a y e d , o r a b s e n t f e mo r a l p u l s e s
i i i. C h e s t x- r a y r e v e a l s t h e t h r e e s i g n a t c o a r c t a t i o n s i g h t a n d n o t c h e d
ribs
g. H y p e r p a r a t h y r o i d i s m
i. H y p e r c a l c e mi a
i i. R e n a l p a r e n c h y ma l d a ma g e d u e t o n e p h r o c a l c i n o s i s a n d r e n a l s t o n e s
3. O r a l c o n t r a c e p t i v e me d i c a t i o n s
4. M a l i g n a n t H T N3) (
a. D i a s t o l i c b l o o d p r e s s u r e u s u a l l y g r e a t e r t h a n 1 3 0 mm H
b. P a p i l l e d e ma , r e t i n a l h e mo r r h a g e s , a n d e xu d a t e s
c. P o s s i b l y v o mi t i n g , s e v e r e h e a d a c h e , t r a n s i e n t v i s u a l c h a n g e s o r l o s s ,
s t u p o r, c o ma , o l i g u r i a , a n d c a r d i a c d e c o mp e n s a t i o n
I n d i v i d u a l s ma y h a v e h i g h b l o o d p r e s s u r e f o r y e a r s a n d n o t k n o w i t . S o me t i me s H T N
ma y h a v e s u b t l e s y mp t o ms s u c h a s e p i s t a xi s , h e ma t u r i a , a n d e p i s o d i c w e a k n e s s ,
d i zzi n e s s , p a l p i t a t i o n s , e a s y f a t i g a b i l i t y, a n d i mp o t e n c e . I f H T N i s p a r t o f a d i s e a s e
p r o c e s s , s u c h a s s e c o n d a r y H T N , i t ma y b e j o i n e d b y o t h e r s y mp t o ms o f t h e
influencing disease process.
References
1 . A me r i c a n H e a r t A s s o c i a t i o n I n c . w w w. a me r i c a n h e a r t . o r g , a c c e s s e d o n 11 A p r i l ,
2006.
P. 1 4 1
2 . D e p a r t me n t o f H e a l t h a n d H u ma n S e r v i c e , N a t i o n a l I n s t i t u t e s o f H e a l t h , T h e
S e v e n t h R e p o r t o f t h e J o i n t N a t i o n a l C o mmi t t e e o n P r e v e n t i o n , D e t e c t i o n ,
E v a l u a t i o n , a n d T r e a t me n t o f H i g h B l o o d P r e s s u r e .
w w w. n h l b i . n i h . g o v / g u i d e l i n e s / h y p e r t e n s i o n , a c c e s s e d o n 11 A p r i l , 2 0 0 6 .
3 . K a s p e r D L , B r a u n w a l d E , F a u c i A S ,H eart rai sl .on' s pr i nc i pl es of i nt er nal
medi c i ne
, 1 6 t h e d . P a r t 8 ; C h a p t e r 2 3 0 . N e w Yo r k , N Y : M c G r a w - H i l l C o mp a n i e s ,
2005.
4 . B i c k l e y L S , S zi l a g y i P G . B e g i n n i n g t h e p h y s i c a l e xa mi n a t i o n : g e n e r a l s u r v e y
a n d v i t a l s i g n s . GI nui: de t o phy s i c al ex ami nat i on and hi s t or,y 8ttak
h ieng
d.
249 / 652
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7.8 - Hypertension
250 / 652
tmdmss
7.9 - Palpitations
> T a b l e o f C o n t e n t s > 7 - C a r d i o v a s c u l a r P r o b l e ms > 7 . 9 - P a l p i t a t i o n s
7.9
Palpitations
J o h n Wi n t e r s
I . Background
P a l p i t a t i o n s a r e a n u n p l e a s a n t a b n o r ma l a w a r e n e s s o f t h e h e a r t b e a t . T h e y a r e
usually described as a pounding or racing of the heart.
A. Etiology
P a l p i t a t i o n s a r e a c o mmo n p r e s e n t i n g c o mp l a i n t e n c o u n t e r e d b y t h e p r i ma r y c a r e
physicians and cardiologists. T he list of underlying causes of palpitations is
e xt e n s i v e ( s eTea b l e 7 . 9 ). .1
B. Epidemiology
One prospective cohort study of 190 patients showed that 43% had palpitations due
t o c a r d i a c c a u s e s , 3 1 % d u e t o a n xi e t y o r p a n i c d i s o r d e r, a n d
P. 1 4 2
l 6 % d u e t o a n u n d e t e r mi n e d e t i o1)l o. gRye (me mb e r i n g t h a t t h e h e a r t i s e l e c t r i c a l l y
p a c e d , a g o o d mn e mo n i c t o a s s i s t i n r e c a l l i n g c a u s e s o f p a l p i t a t i o n s i s E - PA C E D
(El e c t r o l y t e sPs
, y c h i a t r i cAn
, e mi a ,Ca r d i a c ,En d o c r i n e a nDr
d u g s El e c t r o l y t e s ,
Ps y c h i a t r i can
, e mi a ,Ca r d i a c ,En d o c r i n e a nDr
d u g s )2)
( .
TAB L E 7.9.1 Som e Causes of Palpitations

Cardiac
Me dications
Alcohol
Atrial fibrillation/ Atrial flutter
Anticholinergics
Multifocal atrial tachycardia
Caffeine
P r e ma t u r e v e n t r i c u l a r c o n t r a c t i oI lnl isc i t d r u g s
251 / 652
tmdmss
7.9 - Palpitations
Nicotine
Sinus node dysfunction
S y mp a t h o mi me t i c s
Sinus tachycardia
Endocrine /m e tabolic
Supraventricular tachycardia
Hyperthyroidism
Ve n t r i c u l a r t a c h y c a r d i a
H y p o g l y c e mi a
Wo l f - P a r k i n s o n - W h i t e s y n d r o meP h e o c h r o mo c y t o ma
Other cardiac causes
Psy chiatric
Cardiac shunts
A n xi e t y d i s o r d e r
C a r d i o my o p a t h y
Panic attacks
P a c e ma k e r
S o ma t i za t i o n d i s o r d e r
Va l v u l a r h e a r t d i s e a s e
Othe rs
A n e mi a
Fever
Strenuous physical activity
I I I . Evaluation
B e c a u s e p a t i e n t s r a r e l y e xp e r i e n c e a n e p i s o d e w h i l e i n t h e p h y s i c i a n ' s o f f i c e , t h e
h i s t o r y a n d p h y s i c a l e xa mi n a t i o n a r e t h e mo s t c r u c i a l e l e me n t s o f t h e i n v e s t i g a t i o n .
M o r e o v e r, o n l y o c c a s i o n a l l y d o e s p h y s i c a l e xa mi n a t i o n p r o v i d e c l u e s o r s u p p o r t i n g
e v i d e n c e t o a f f i r m a d i a g n o s i s , t h e r e b y ma k i n g a t h o r o u g h a n d d e s c r i p t i v e h i s t o r y
a s s u me e v e n g r e a t e r i mp o r t a n c e .
A. History
T h e h i s t o r y s h o u l d f o c u s o n i n f o r ma t i o n s u c h a s t h e c i r c u ms t a n c e s d u r i n g
o c c u r r e n c e , i n t e r mi t t e n c y, d u r a t i o n , p r e c i p i t a t i n g a n d r e s o l v i n g f a c t o r s , a d e t a i l e d
d e s c r i p t i o n o f t h e p a l p i t a t i o n s , a n d a s s o c i a t e d s i g n s a n d s y mp t o ms .
1. T h e c i r c u ms t a n c e s d u r i n g w h i c h p a l p i t a t i o n s o c c u r ma y i n d i c a t e a n a s s o c i a t i o n
w i t h a n xi e t y o r p a n i c a t t a c k s , a l t h o u g h o n e s h o u l d a l s o k e e p i n mi n d e xc e s s
c a t e c h o l a mi n e d u r i n g t i me s o f s t r e s s . Va l i d a t e d s c r e e n i n g t o o l s a r e r e a d i l y
252 / 652
tmdmss
7.9 - Palpitations
a v a i l a b l e t o a s s i s t i n i d e n t i f y i n g p a t i e n t s w i t h s u c h p s y c h i3)
a t. r i c i l l n e s s (
Occurrence at night while lying down could result from atrial or ventricular
p r e ma t u r e c o n t r a c t i o n s . P o s i t i o n a l c h a n g e s mi g h t i n d i c a t e a t r i o v e n t r i c u l a r n o d a
tachycardia.
2. I s o l a t e d o r s k i p p e d b e a t s c o u l d b e p r e ma t u r e c o n t r a c t i o n s o r b e n i g n e c t o p y.
3. P a l p i t a t i o n s p r e c i p i t a t e d b y e xe r c i s e ma y b e a s s o c i a t e d w i t h s u p r a v e n t r i c u l a r
a r r h y t h mi a s o r a t r i a l f i b r i l l a t i o n . P a l p i t a t i o n s t e r mi n a t e d b y v a g a l ma n e u v e r s
p o i n t t o p a r o xy s ma l s u p r a v e n t r i c u l a r t a c h y c a r d i a .
4. P a l p i t a t i o n s d e s c r i b e d a s r a p i d a n d r e g u l a r ma y i n d i c a t e p a r o xy s ma l
supraventricular tachycardia and ventricular tachycardias. Palpitations
d e s c r i b e d a s r a p i d a n d i r r e g u l a r c o u l d i mp l y a t r i a l f i b r i l l a t i o n , mu l t i f o c a l a t r i a l
tachycardia, and atrial flutter with variable block. Flip-flopping or stopping
s t a r t i n g s e n s a t i o n s ma y b e c a u s e d b y s u p r a v e n t r i c u l a r o r v e n t r i c u l a r p r e ma t u r e
c o n t r a c t i o n s . P a l p i t a t i o n s ma y b e s e n s e d a s a p o u n d i n g i n t h e n e c k . N o t a b l y,
atrioventricular nodal reentrant tachycardia is associated with a rapid regular
p o u n d i n g i n t h e n e c k , w h e r e a s s u c h a s e n s a t i o n t h a t i s l e s s r e g u l a r a n d mo r e
i n t e r mi t t e n t ma y b e p r e ma t u r e v e n t r i c u l a r c o n t r a c t i o n s .
5. T h e p h y s i c i a n s h o u l d k e e p i n mi n d t h a t d i zzi n e s s , p r e s y n c o p e , o r s y n c o p e
a c c o mp a n y i n g p a l p i t a t i o n s a r e f a c t o r s t h a t h e l p i d e n t i f y p a t i e n t s a t r i s k f o r f a t a
r e s u l t s . T h e s e s y mp t o ms s h o u l d p r o mp t a n i n v e s t i g a t i o n f o r v e n t r i c u l a r
tachycardias.
6. A g e a t o n s e t i n c h i l d h o o d ma y s u g g e s t s u p r a v e n t r i c u l a r t a c h y c a r d i a . S y mp t o ms
of hyperthyroidism such as heat intolerance should be elicited. Illicit drugs,
c a f f e i n e , a n d n i c o t i n e h a b i t s a s w e l l a s a n e mi a a n d me t a b o l i c d i s o r d e r s a r e a l l
i mp o r t a n t p o s s i b l e a s s o c i a t i o n s t h a t s h o u l d b e c o n s i d e r e d .
T h e f i n d i n g s o f t h e p h y s i c a l e xa mi n a t i o n ma y g i v e c l u e s s u c h a s a h e a r t mu r mu r,
i n d i c a t i n g t h e p o s s i b i l i t y o f v a l v u l a r d i s e a s e o r c a r d i o my o p a t h i e s o r t h e mi d s y s t o l i c
c l i c k o f mi t r a l v a l v e p r o l a p s e . A b r i s k w a l k d o w n a c o r r i d o r mi g h t r e v e a l a p o o r l y
controlled ventricular response and resultant palpitations in a patient with atrial
f i b r i l l a t i o n a n d p a l p i t a t 1)
i o .n sI t ( i s n e c e s s a r y t o s e a r c h f o r s i g n s o f c o n g e s t i v e h e a r t
failure.
C. Testing
T w e l v e - l e a d e l e c t r o c a r d i o g r a p h y ( E C G ) i s i n d i c a t e d a n d s h o u l d b e a n a l y ze d f o r
s h o r t P R i n t e r v a l a n d d e l t a w a v e s ( v e n t r i c u l a r p r e - e xc i t a t i o n ) , Q w a v e s i n d i c a t i n g l
v e n t r i c u l a r h y p e r t r o p h y o r p r i o r i n f a r c t i o n , p r e ma t u r e c o n t r a c t i o n s , l o n g Q T i n t e r v a l
h e a r t b l o c k s , o r a n y o t h e r a b n o r ma l i t y. L a b o r a t o r y t e s t i n g c a n u s u a l l y b e l i mi t e d t o
i n v e s t i g a t i o n o f a n e mi a , t h y r o i d f u n c t i o n , a n d e l e c t r o l y t e d i s t u r b a n c e s ( p o t a s s i u m
a n d ma g n e s i u m) . A mb u l a t o r y E C G c a n b e u s e d f o r f u r t h e r i n v e s t i g a t i o n . H o l t e r
mo n i t o r i n g s h o u l d s u f f i c e f o r p a t i e n t s w i t h d a i l y s y mp t o ms ; o t h e r w i s e , a c o n t i n u o u s l o o p e v e n t r e c o r d e r s h o u l d p r o v i d e a b e t t e r d i a g n o4,5)
s t i c. y i e l d (
253 / 652
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7.9 - Palpitations
I V. Diagnosis
T h e l i s t o f c a u s e s o f p a l p i t a t i o n s i s e xtTeanbsl ei v e7 . (9 ). .1 I n mo s t c a s e s , t h e c a u s e
i s b e n i g n . L i f e - t h r e a t e n i n g c a u s e s a r e s u g g e s t e d b y a s s o c i a t e d s y mp t o ms s u c h a s
d i zzi n e s s o r s y n c o p e . T h e h i s t o r y i s t h e mo s t i mp o r t a n t d i a g n o s t i c t o o l , a n d a
t h o r o u g h a n d d e s c r i p t i v e i n q u i r y s h o u l d h e ma d e . T h e p h y s i c a l e xa mi n a t i o n ma y
P. 1 4 3
provide further clues or supporting evidence. Testing involves 12-lead E C G and a
l i mi t e d l a b o r a t o r y i n v e s t i g a t i o n .
References
1 . A b b o t t AV. D i a g n o s t i c a p p r o a c h t o p a l p iAt amt i oFnam
s . P hy s i c i an
2005;71:743750.
2 . We b e r B E , K a p o o r W N . E v a l u a t i o n a n d o u t c o me s o f p a t i e n t s w i t h p a l p i t a t i o n s .
A m J M ed1 9 9 6 ; 1 0 0 : l 3 8 1 4 8 .
3 . Ta y l o r R B , eT
d .he 10- mi nut e di agnos i s manual
, 1 s t e d . P h i l a d e l p h i a , PA : J B
Lippincott, 1994.
4 . Z i me t b a u m P, J o s e p h s o n M E . E v a l u a t i o n o f p a t i e n t s w i t h pNa l E
p ingl
tations.
J M ed 1 9 9 8 ; 3 3 8 : 1 3 6 9 1 3 7 3 .
5 . Z i me t b a u m P, J o s e p h s o n M E . T h e e v o l v i n g r o l e o f a mb u l a t o r y mo n i t o r i n g i n
g e n e r a l c l i n i c a l p r a c tAi cnn
e . I nt er n M ed
l999;130:848856.
254 / 652
tmdmss
7.10 - Pericardial Friction Rub
> T a b l e o f C o n t e n t s > 7 - C a r d i o v a s c u l a r P r o b l e ms > 7 . 1 0 - P e r i c a r d i a l F r i c t i o n
Rub
7.10
Pericardial Friction Rub
Kam le sh G. Ansingkar
I . Background
T h e p e r i c a r d i u m c o n s i s t s o f a n o u t e r f i b r o u s l a par
y e r i cetaal
l l e per
d i c ar di um
and
a n i n n e r s e r o u s l a y e r c avli lsecder al per i c ar di. um
T he visceral layer folds onto itself
t o f o r m t h e p a r i e t a l l a y e r. I n g e n e r a l , t h e r e i s a p p r o xi ma t e l y 5 0 mL o f u l t r a f i l t r a t e o f
p l a s ma b e t w e e n t h e s e t w o l a y e r s , w h i c h p r i ma r i l y d r a i n s i n t o t h e r i g h t p l e u r a l s p a c e
t h r o u g h t h e t h o r a c i c d1)
u c. t P( e r i c a r d i a l f r i c t i o n r u b ( c a u s e d b y t h e d e p o s i t i o n o f
f i b r i n o u s ma t e r i a l b e t w e e n t h e t w o i n f l a me d l a y e r s ) , a s s o c i a t e d w i t h c h e s t p a i n a n d
s e r i a l e l e c t r o c a r d i o g r a p h i c c h a n g e s , i s t h e mo s t c o mmo n f i n d i n g i n a c u t e p e r i c a r d i t i s
( A P ) 1( ,2) .
A. Etiology of AP
D i s e a s e ma y b e i d i o p a t h i c o r c a u s e d b y a bSatcaphy
t e r i al loc
( oc c us , H aemophi l us ,
P neumoc oc c us , S al monel
, t ul ab e r c u l o s iM
s ,eni ngoc oc c ,uss y p h i l i s ) , v i r a l
( c o xs a c k i e v i r u s , e c h o v i r u s , E p s t e i n - B a r r v i r u s , i n f l u e n za v i r u s , h u ma n
i mmu n o d e f i c i e n c y v i r u s , mu mp s ) , f u n g a l ( h i s t o p l a s mo s i s , b l a s t o my c o s i s ,
c o c c i d i o i d o my c o s i s , a s p e r g i l l o s i s ) , o r p a r a s i t i c ( e c h i n o c o c c o s i s , a me b i a s i s ,
R i c k et t s i a, Tox opl as) ma
i n f e c t i o n . O t h e r c a u s e s i n c l u d e r h e u ma t o i d a r t h r i t i s ,
s y s t e mi c l u p u s e r y t h e ma t o s u s , s c l e r o d e r ma , r h e u ma t i c f e v e r, s a r c o i d o s i s ,
p e r i c a r d i t i s a s s o c i a t e d w i t h o t h e r i n f l a mma t o r y c o n d i t i o n s ( S j g r e n s y n d r o me , mi xe d
c o n n e c t i v e t i s s u e d i s e a s e , R e i t e r ' s s y n d r o me , a n k y l o s i n g s p o n d y l i t i s , i n f l a mma t o r y
b o w e l d i s e a s e , We g e n e r ' s g r a n u l o ma t o s i s , v a s c u l i t i s s u c h a s g i a n t c e l l a r t e r i t i s a n d
p o l y a r t e r i t i s , p o l y my o s i t i s , B e h e t ' s s y n d r o me , W h i p p l e ' s d i s e a s e , f a mi l i a l
M e d i t e r r a n e a n f e v e r, a n d s e r u m s i c k n e s s ) , r e n a l f a i l u r e w i t h a n d w i t h o u t d i a l y s i s ,
h y p o t h y r o i d i s m, c h o l e s t e r o l p e r i c a r d i t i s , a c u t e my o c a r d i a l i n f a r c t i o n ( A M I ) , D r e s s l e r ' s
s y n d r o me , p o s t p e r i c a r d i o t o my s y n d r o me , n e o p l a s ms ( p r i ma r y f r o m p e r i c a r d i a l
me s o t h e l i o ma a n d a n g i o s a r c o ma , a n d me t a s t a t i c f r o m b r e a s t , l u n g , l y mp h o ma ,
me l a n o ma , a n d l e u k e mi a ) , i r r a d i a t i o n , d r u g s ( p e n i c i l l i n , c r o mo l y n s o d i u m,
d o xo r u b i c i n , c y c l o p h o s p h a mi d e , p r o c a i n a mi d e , h y d r a l a zi n e , me t h y l d o p a , i s o n i a zi d ,
me s a l a zi n e , r e s e r p i n e , me t h y s e r g i d e , d a n t r o l e n e , p h e n y t o i n , a n d mi n o xi d i l ) a n d
mi s c e l l a n e o u s c a u s e s , i n c l u d i n g a o r t i c d i s s e c t i o n a n d p e n e t r a t i n g a n d
n o n p e n e t r a t i n g c a r d i a c t r a1,2)
u ma. (
B. Epidemiology of AP
T h e p r e v a l e n c e o f A P i n h o s p i t a l i ze d p a t i e n t s i s e s t i ma t e d t o b e a p p r o xi ma t e l y 0 . 1 %
a n d i s mo r e c o mmo n i n ma l e s a n d a d u l t s t h a n i n f e ma l e s a n d 1,3)
i n c. hUi lpd rteon , (
1 5 % o f p a t i e n t s ma y e xp e r i e n c e r e c u r r e n c e o f s y mp t o ms i n t h e f i r s2)
t .f e w mo n t h s (
B e f o r e t h e e r a o f r e p e r f u s i o n t h e r a p y, t h e i n c i d e n c e o f i n f a r c t i o n - a s s o c i a t e d
p e r i c a r d i t i s r a n g e d f r o m 7 % t o 2 5 % , w h e r e a s i n o n e s t u d y,
255 / 652
tmdmss
P. 1 4 4
a f t e r p e r c u t a n e o u s t r a n s l u mi n a l c o r o n a r y a n g i o p l a s t y ( P T C A ) i n Q w a v e A M I , t h e
p e r i c a r d i a l r u b w a s d e t e c t e d 4)
i n. 8 % (
I I I . Evaluation
A. History
1. P a t i e n t s w i t h A P u s u a l l y p r e s e n t w i t h c h e s t p a i n t h a t i s t y p i c a l l y a s h a r p
p r e c o r d i a l p a i n b u t ma y a l s o p r e s e n t w i t h a d u l l , a c h i n g , b u r n i n g , o r a p r e s s i n g
p a i n . I t ma y b e r e f e r r e d t o t h e t r a p e zi u s r i d g e ( a s y mp t o m c h a r a c t e r i s t i c o f A P )
a n d i s w o r s e w i t h i n s p i r a t i o n , l y i n g d o w n , d u r i n g s w a l l o w i n g , o r w i t h b o d y mo t i o n
and is relieved by sitting upright or leaning forward.
2. A s s o c i a t e d s y mp t o ms ma y i n c l u d e d y s p n e a ( e s p e c i a l l y w i t h t a mp o n a d e ) , f e v e r,
my a l g i a , a n d ma l a i s e ( w i t h v i r a l o r b a c t e r i a l A P ) , w e i g h t l o s s ( ma l i g n a n c y ) , a n d
p r o d u c t i v e c o u g h ( w i t h p n e u mo n i a a n d p u r u l e n t p e r i c a r d i t i s ) o r h e mo p t y s i s
( t u b e r c u l o s i s ) . O t h e r n o n s p e c i f i c s y mp t o ms i n c l u d e h i c c u p s , c o u g h , h o a r s e n e s s ,
p a l p i t a t i o n s , n a u s e a , a n d v o mi t i n g .
3. P a s t me d i c a l h i s t o r y ma y b e s u g g e s t i v e o f a n a s s o c i a t e d i l l n e s s t h a t ma y c a u s e
A P, e xa mp l e s o f w h i c h i n c l u d e h i s t o r y o f r e c e n t p e r i c a r d i o t o my, r e n a l f a i l u r e w i t h
o r w i t h o u t d i a l y s i s , c o l l a g e n - v a s c u l a r d i s e a s e s , ma l i g n a n c y, o r u s e o f
me d i c a t i o n s1,2,3,5)
(
.
Pericardial friction rub, which is like creaking leather or a scratching sound, is
u s u a l l y a u s c u l t a t e d a l o n g t h e l o w e r l e f t s t e r n a l b o r d e r. I t i s b e s t h e a r d w i t h t h e
d i a p h r a g m o f a s t e t h o s c o p e i n a q u i e t r o o m. I t i s u s u a l l y t r i p h a s i c ( a t r i a l s y s t o l e r u b ,
v e n t r i c u l a r s y s t o l e r u b , a n d e a r l y v e n t r i c u l a r d i a s t o l e r u b ) b u t ma y b e b i p h a s i c o r
mo n o p h a s i c , a n d s e r i a l e xa mi n a t i o n s ma y b e n e c e s s a r y b e c a u s e i t c a n b e
e v a n e s c e n t . A s s o c i a t e d p h y s i c a l f i n d i n g s ma y i n c l u d e f e v e r, t a c h y c a r d i a , a n d
t a c h y p n e a . H y p o t e n s i o n , p u l s u s p a r a d o xu s , e l e v a t e d s y s t e mi c v e n o u s p r e s s u r e , a n d
mu f f l e d h e a r t s o u n d s ma y o c c u r w i t h c a r d i a c t a mp
1,2)o.n a d e (
C. Testing
1. L a b o r a t o r y t e sAt sc o mp l e t e b l o o d c o u n t ma y s h o w l e u k o c y t o s i s , a n d
e r y t h r o c y t e s e d i me n t a t i o n r a t e , C - r e a c t i v e p r o t e i n , a n d a n t i s t r e p t o l y s i n t i t e r ma y
b e e l e v a t e d . B l o o d u r e a n i t r o g e n a n d s e r u m c r e a t i n i n e ma y b e u s e f u l t o
e v a l u a t e u r e mi a . C a r d i a c e n zy me s ma y b e e l e v a t e d i f a s s o c i a t e d w i t h
my o c a r d i t i s a n d A M I . R h e u ma t o i d f a c t o r, a n t i n u c l e a r a n t i b o d y t e s t , a n d a n t i D N A v a l u e s ma y b e h e l p f u l i n e v a l u a t i n g a u t o i mmu n e d i s o r d e r s a n d c o n n e c t i v e t i s s u e d i s e a s e s . T u b e r c u l i n t e s t i n g ma y a i d i n r u l i n g o u t t u b e r c u l o u s p e r i c a r d i t i s .
T h y r o i d f u n c t i o n t e s t s ma y b e h e l p f u l t o r u l e o u t h y p o t h y r o i d i s m. A n t i b o d y t i t e r s
to various infectious agents can be tested as clinically indicated.
2. E l e c t r o c a r d i o g r a m ( E CEGC)G i s mo s t u s e f u l i n d i a g n o s i n g A P. E C G c h a n g e s
i n c l u d e d i f f u s e u p w a r d S T e l e v a t i o n , e xc e p t i n a1V
. R
I n iat inadl l y,
V T waves
a r e u p r i g h t i n t h e l e a d s w i t h S T e l e v a t i o n , a n d t h e P R ma y b e d e p r e s s e d i n t h e
e p i c a r d i a l l e a d s . S e v e r a l d a y s l a t e r, t h e S T s e g me n t r e t u r n s t o b a s e l i n e , a n d
t h e T w a v e f l a t t e n s a n d t h e n g r a d u a l l y b e c o me s i n v e r t e d . L o w - v o l t a g e Q R S
c o mp l e xe s i n d i c a t e p r e s e n c e o f a p e r i c a r d i a l e f f u s i o n .
3. I m a g i n g s t u d i eCsh e s t r a d i o g r a p h y ma y s h o w a f l a s k - s h a p e d e n l a r g e d h e a r t i n
t h e p r e s e n c e o f a l a r g e e f f u s i o n . I f c l i n i c a l s i g n s o f p e r i c a r d i a l t a mp o n a d e o r
large effusion are present, then echocardiography is helpful for diagnostic and
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t h e r a p e u t i c p e r i c a r d i o c e n t1,2)
e s i s. (
I V. Diagnosis
D i f f e r e n t i a l d i a g n o s i s i n c l u d e s a o r t i c d i s s e c t i o n , c o r o n a r y a r t e r y v a s o s p a s m,
e s o p h a g e a l r u p t u r e , e s o p h a g i t i s , e s o p h a g e a l s p a s m, a c u t e g a s t r i t i s , my o c a r d i a l
i n f a r c t i o n , my o c a r d i a l i s c h e mi a , a n d p e p t i c u l c e r d i s e a s e . E a c h c o n d i t i o n s h o u l d b e
e v a l u a t e d a p p r o p r i a t 1)
e l .y (
References
1 . P h i l i p J , G e n t l e s k P J , M c C a b e J ,A ec ut
t ael . per i c ar di. t iMs a r c h 2 0 0 5 .
h t t p : / / w w w. e me d i c i n e . c o m. c u h s l . c r e i g h t o n . e d u / me d / t o p i c 1 7 8 1 . h t m, a c c e s s e d o n 1
J u l y, 2 0 0 5 .
2 . M a r i n e l l a M A . E l e c t r o c a r d i o g r a p h i c ma n i f e s t a t i o n s a n d d i f f e r e n t i a l d i a g n o s i s o f
a c u t e p e r i c a r d i tAi sm. F am P hy s i c i an
1998;57:699704.
P. 1 4 5
3 . G r i mm R A , H e s s e PBer
. i c ar di al di s eas e: ac ut e per i c, ar2 di
0 0t3i s.
h t t p : / / w w w. c l e v e l a n d c l i n i c me d e d . c o m/ d i s e a s e ma n a g e me n t / c a r d i o l o g y / p e r i c a r d i a l / p e r i c a r d i a l . h t m
a c c e s s e d o n 1 J u l y, 2 0 0 5 .
4 . S u g i u r a T , T a k e h a n a K , A b e Y, e t a l . F r e q u e n c y o f p e r i c a r d i a l f r i c t i o n r u b
( p e r i c a r d i t i s ) a f t e r d i r e c t p e r c u t a n e o u s t r a n s l u mi n a l c o r o n a r y a n g i o p l a s t y i n Q w a v e a c u t e my o c a r d i a l i n f a r cAtm
i o nJ. C ar di ol1 9 9 7 ; 7 9 ( 3 ) : 3 6 2 3 6 4 .
5 . R o s s A M , G r a u e r S E . A c u t e p e r i c a r d i t i s . E v a l u a t i o n a n d t r e a t me n t o f
i n f e c t i o u s a n d o t h e r c a u s e s . ( R ePv os
i e wt gr
) . ad M ed
2 0 0 4 ; 11 5 ( 3 ) : 6 7 7 0 , 7 3 7 5 .
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7.11 - Raynaud's Disease
> T a b l e o f C o n t e n t s > 7 - C a r d i o v a s c u l a r P r o b l e ms > 7 . 11 - R a y n a u d ' s D i s e a s e
7.11
Raynaud's Disease
Sandra B. Baum be rge r
I . Background
R a y n a u d ' s d i s e a s e i s a v a s o s p a s t i c d i s o r d e r t h a t c a u s e s e p i s o d i c i s c h e mi a o f t h e
d i g i t s o f t h e h a n d s , l a s t i n g a v a r i a b l e a mo u n t o f t i me . I t w a s n a me d a f t e r M a u r i c e
R a y n a u d , w h o f i r s t n o t e d t h e d i s o r d e r i n 1 8 6 2 . A l t h o u g h t h e d i s o r d e r ma i n l y a f f e c t s
the hands, it can also affect the tongue, the nose, the nipples, or the toes. T here a
t w o c l a s s i f i c a t i o n s o f R a y n a u d ' s d i s e a s e : P r i ma r y R a y n a u d ' s d i s e a s e ( P R D ) ,
f o r me r l y k n o w n a s R a y n a u d ' s p h e n o me n o n , i s n o t a s s o c i a t e d w i t h a n u n d e r l y i n g
i l l n e s s . S e c o n d a r y R a y n a u d ' s d i s e a s e ( S R D ) , f o r me r l y k n o w n a s R a y n a u d ' s
s y n d r o me , i s o f t e n a s s o c i a t e d w i t h a n u n d e r l y i n1)g . i l l n e s s (
A. Etiology
E mo t i o n a l s t r e s s a n d c o l d s e e m t o b e t h e b i g g e s t t r i g g e r s o f a t t a c k s . T h e d u r a t i o n
t h e s e a t t a c k s ma y b e a f e w mi n u t e s o r a f e w h o u r s . C l a s s i c a l l y, d u r i n g t h e a t t a c k s
there is a three-stage color change. Stage one involves the pallor of the skin, whic
i s s o me t i me s a s s o c i a t e d w i t h c o l d n e s s , n u mb n e s s , a n d / o r p a r e s t h e s i a s . T h e s e c o n d
s t a g e , i f p r e s e n t , i n v o l v e s c y a n o s i s a n d ma y i n c l u d e s o me o f t h e s y mp t o ms
a s s o c i a t e d w i t h s t a g e o n e . F i n a l l y, t h e t h i r d s t a g e i n v o l v e s e r y t h e ma , w h i c h ma y b e
a s s o c i a t e d w i t h t h r o b b i n g o f t h e 2)
d i.g P
i tR
s D( c a r r i e s a l o w r i s k o f p r o g r e s s i o n t o
a n a u t o i mmu n e d i s o r d e r i f a f t e r 2 y e a r s o f a t t a c k s n o o t h e r s i g n s o r s y mp t o ms o f
a u t o i mmu n e p r o c e s s e s a r e p r e s e n t . C o n v e r s e l y, R a y n a u d ' s c a n b e t h e p r e s e n t i n g
s i g n f o r s c l e r o d e r ma . I t h a s b e e n p r o v e d t h a t t h o s e w h o s u f f e r f r o m P R D o r S R D
h a v e a b n o r ma l b l o o d f l o w t o t h e d i g i t s a n d a b n o r ma l r e c o v e r y f r o m c o l d s t i mu l u s . I n
p a t i e n t s w i t h s c l e r o d e r ma , t h e b l o o d v e s s e l s a r e a l r e a d y n a r r o w e d b e c a u s e o f
p r o l i f e r a t i o n o f t h e i n t i ma l a r e a . T h i s e xa c e r b a t e s t h e i s c h e mi a c a u s e d b y t h e c o l d
s t i mu l u s3)( .
B. Epidemiology
R a y n a u d ' s d i s e a s e a f f e c t s a p p r o xi ma t e l y 3 % t o 4 % o f t h e g e n e r a l p o p u l a t i o n .
A l t h o u g h t h e p r e v a l e n c e v a r i e s , i t a p p e a r s t o b e mo r e c o mmo n i n w o me n t h a n i n
me n . P R D u s u a l l y b e g i n s i n t h e s e c o n d o r t h i r d d e c a d e o f l i f e . S R D ma y b e g i n w i t h
t h e u n d e r l y i n g d i s o r d e r a n d / o r b e t h e p r e s e n t i n g s i g n o f a n a u t o i mmu n e d i s o r d e r.
R a y n a u d ' s d i s e a s e i s mo r e c o mmo n i n c o l d c l i ma t e s a n d c a n b e a s s o c i a t e d w i t h
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c e r t a i n o c c u p a t i o n s s u c h a s t h o s e t h a t r e q u i r e e xp o s u r e t o me c h a n i c a l v i b r a t i o n o n
a n o n g o i n g b a s 4)
i s . ( O t h e r d i s o r d e r s a s s o c i a t e d w i t h S R D i n c l u d e s y s t e mi c l u p u s
e r y t h e ma t o s u s ( S L E ) , r h e u ma t o i d a r t h r i t i s , d e r ma t o my o s i t i s , p o l y my o s i t i s , a n d
h y p e r v i s c o s i t y s y n d r o me s , a s w e l l a s v a s o s p a s t i c p r o c e s s e s s u c h a s v a s c u l a r
h e a d a c h e s , a t y p i c a l a n g i n a , a n d p r i ma r y p u l mo n a r y h y p5)e.r t e n s i o n (
I I I . Evaluation
A. History
W h e n i n t e r v i e w i n g t h e p a t i e n t , i t i s i mp o r t a n t t o d e t e r mi n e w h e n t h e s y mp t o ms
n o r ma l l y a p p e a r. E xp o s u r e t o c o l d t e mp e r a t u r e s a n d s t r e s s a r e t h e mo s t c o mmo n
t r i g g e r s . M e d i c a t i o n s u s e d a n d h i s t o r y o f t o b a c c o u s e a r e a l s o i mp o r t a n t t o p i c s t o b
c o v e r e d i n t h e h i s t o5)r y. Q
( u e s t i o n s a b o u t t h e u s e o f ma c h i n e s o r t o o l s
P. 1 4 6
t h a t p r o d u c e v i b r a t i o n o r o t h e r r e p e t i t i v e a c t i v i t i e s d o n e w i t h t h e f i n g e r s ma y h e l p
d e t e r mi n e i f t h e p a t i e n t h a s a v a r i a n t o f R a y n a u d ' s d i s e a shandar
e k n o w nm a s
v i br at i on s y ndr ome
(1) .
T h e e xa mi n a t i o n s h o u l d f o c u s o n a t h o r o u g h a s s e s s me n t o f t h e d i g i t s . I n s p e c t t h e
d i g i t s c a r e f u l l y f o r s c l e r o d a c t y l y o r d i g i t a l u l c e r s , a n d e xa mi n e t h e n a i l f o l d c a p i l l a r
f o r a b n o r ma l l y l a r g e c a p i l l a r y l o o p s , a l t e r n a t i n g w i t h a r e a s w i t h o u t a n y c a p i l l a r i e s .
T h i s p a t t e r n i s s u g g e s t i v e o f s c l e r o5)
d e. rIma
t i s ( a l s o i mp o r t a n t t o i n s p e c t f o r M a l a r
rash, persistent cyanosis, and/or necrotic tissue of the digits.
C. Genetics
T h e r e i s o f t e n a f a mi l y h i s t o r y o f t h e d5)
i s.o rCduerrr e( n t l y, h o w e v e r, n o g e n e t i c
ma r k e r t h a t c a n b e t e s t e d f o r h a s b e e n i d e n t i f i e d .
I V. Diagnosis
P R D i s t h e mo s t l i k e l y d i a g n o s i s i n a y o u n g w o ma n w i t h l e s s s e v e r e s y mp t o ms a n d
no underlying disease. S R D should be considered in anyone with a known
a u t o i mmu n e d i s e a s e s u c h a s s c l e r o d e r ma o r S L E . O t h e r d i a g n o s e s t h a t c o u l d b e
c o n s i d e r e d i n c l u d e t h r o mb o a n g i i t i s o b l i t e r a n s i n a ma n w h o s mo k e s , t h o r a c i c o u t l e t
s y n d r o me , c a r p a l t u n n e l s y n d r o me r e l a t e d t o r e p e t i t i v e u s e o f t h e h a n d s ,
a c r o c y a n o s i s , a n d c r y o g l o b u l i n e mi a , e s p e c i a l l y a s s o c i a t e d w i t h h e p a t i t i s B o r C .
D r u g s t h a t ma y i n d u c e S R D i n c l u d e , b u t a r e n o t l i mi t e d t o , - b l o c k e r s , a n t i n e o p l a s t
d r u g s , o r a l c o n t r a c e p t i v e s , e r g o t a l k a l o i d s , c y c l o s p o r i n e , a n d 3).- i n t e r f e r o n (
C o l o r c h a n g e o f t h e d i g i t s w h e n e xp o s e d t o t h e c o l d i s t h e c l a s s i c p r e s e n t a t i o n .
P a t i e n t s ma y n o t u n d e r g o a l l t h e t h r e e c o l o r c h a n g e s . S o me l i t e r a t u r e s t a t e s t h a t
p a l l o r f o l l o w e d b y c y a n o s i s i s t h e mo s t c o mmo n p a t t e r n , w h e r e a s o t h e r s o u r c e s n o t e
that in patients who have episodes with cyanosis the severity of the disease is
h i g h e r 1)( . T h e mo s t i mp o r t a n t t h i n g t o k e e p i n mi n d w h e n d i a g n o s i n g p a t i e n t s w i t h
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R a y n a u d ' s d i s e a s e i s t h a t p a t i e n t s w i t h S R D a l mo s t a l w a y s h a v e o t h e r s y mp t o ms o f
connective-tissue disease in addition to P R D.
References
1 . B l o c k J A . R a y n a u d ' s p h e n o meLnanc
o n .et2 0 0 1 ; 3 5 7 : 2 0 4 2 2 0 4 8 .
2. N ational Institute of Arthritis and M usculoskeletal and S kin D iseases.
w w w. n i a ms . n i h . g o v, a c c e s s e d o n 11 A p r i l , 2 0 0 6 .
3 . R ay naud' s phenomenon
, 2 0 0 4 . w w w. i me d i c i n e . c o m.
4. C anada's N ational O ccupational H ealth and S afety R esource.
w w w. c c o h s . c a / o s h a n s w e r s / d i s e a s e s / r a y n a u d / h t ml , a c c e s s e d o n 11 A p r i l , 2 0 0 6 .
5 . R ay naud' s phenomenon
, 2 0 0 4 . w w w. f i r s t c o n s u l t . c o m.
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7.12 - Tachycardia
> T a b l e o f C o n t e n t s > 7 - C a r d i o v a s c u l a r P r o b l e ms > 7 . 1 2 - T a c h y c a r d i a
7.12
Tachycardia
Mike Duke low
I . Background
Tachycardia is defined as a condition in which the heart rate greater than 100
b e a t s / mi n u t e . I t c a n b e c a t e g o r i ze d i n t o t w o ma i n t y p e s , s u p r a v e n t r i c u l a r o r
v e n t r i c u l a r, w i t h t h e f o r me r b e i n g f u r t h e r c a t e g o r i ze d i n t o e i t h e r w i d e c o mp l e x
t a c h y c a r d i a o r n a r r o w c o mp l e x t a c h y 3)
c a. r d i a (
In the evaluation of the patient with tachycardia, one needs to discover the etiology
o f t h e t a c h y c a r d i a a n d t h e n i d e n t i f y t h e s p e c i f i c d y s r h y t h mi a . S y mp t o ms o f
t a c h y c a r d i a i n c l u d e p a l p i t a t i o n s , i r r e g u l a r r h y t h m, l i g h t h e a d e d n e s s o r p r e s y n c o p e ,
s y n c o p e , a n d c o n g e s t i v e h e a r t f aCi hl uarpet e( r s 2 . ,1 72 . 5, a n d7 . 9) .
P. 1 4 7
C o n d i t i o n s t o l o o k f o r i n t h e p r e v i o u s me d i c a l h i s t o r y ma y i n c l u d e my o c a r d i a l
i n f a r c t i o n , c a r d i o my o p a t h y, a r r h y t h mi a s , p u l mo n a r y h y p e r t e n s i o n , c a r d i a c s u r g e r y,
and valvular heart disease. Medications that can precipitate tachycardia and hence
n e e d t o b e w a t c h e d o u t f o r a r e c l a s s I A a n d I I I a n t i a r r h y t h mi c me d i c a t i o n s , o v e r - t h e
c o u n t e r c o l d r e me d i e s , o r e v e n s o me a n t i b i o t i c s c o mb i n e d w i t h s o me o f t h e
n o n s e d a t i n g a n t i h i s t a mi n e s .
I I I . Evaluation
A. History
D e t e r mi n e i f t h e p a t i e n t i s h e mo d y n a mi c a l l y s t a b l e a n d i f h e o r s h e i s s y mp t o ma t i c
( d i s t r e s s e d o r u n c o n s c i o u s ) o r a s y mp t o ma t i c ( a w a k e a n d n o t d i s t r e s s e d ) .
A u s c u l t a t e t h e h e a r t , l i s t e n i n g f o r mu r mu r s , r u b s , a n d g a l l o p s . A r e s p i r a t o r y a n d
v i s u a l e xa mi n a t i o n o f t h e p a t i e n t t h a t d e mo n s t r a t e s l u n g c r a c k l e s , j u g u l a r v e n o u s
d i s t e n t i o n , a n d l o w e r e xt r e mi t y e d e ma i n d i c a t e s h e a r t f a i l u r e .
C. Testing
1. E l e c t r o c a r d i o g r a m ( E COGb)t a i n a 1 2 - l e a d E C G a n d d e t e r mi n e t h e r a t e ,
r h y t h m, a n d Q R S d u r a t i o n o n t h e r h y t h m s t r i p ( mo s t t i me s i t i s l e a d I I ) .
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2. L a b o r a t o r y t e s Lt so o k f o r h y p o ma g n e s e mi a , h y p o k a l e mi a , a n e mi a ,
h y p e r t h y r o i d i s m, h y p o xe mi a , i l l i c i t d r u g s , a n d d i g i t a l i s t o xi c i t y.
I V. Diagnosis
A. Narrow complex tachycardia (QRS < 120
millisecond)
1. S i n u s t a c h y c a r dSi ai n u s t a c h y c a r d i a i s n o r ma l i n i n f a n t s u p t o c h i l d r e n y o u n g e r
t h a n 2 y e a r s . T h e r e a r e n u me r o u s r e a s o n s f o r t a c h y c a r d i a i n a d u l t s : p a t h o l o g i c
( a n e mi a , t h y r o t o xi c o s i s , h y p o xe mi a , h y p o t e n s i o n , p u l mo n a r y e mb o l i s m) ,
p h y s i o l o g i c ( a n e mi a , f e v e r ) , a n d p h a r ma c o l o g i c ( - a g o n i s t ) . N o r ma l P w a v e a n d
P R interval are seen on the E C G. T herapy consists in treating the underlying
cause.
2. A t r i a l f i b r i l l a t iAotnr i a l f i b r i l l a t i o n i s a n i r r e g u l a r l y i r r e g u l a r r h y t h m w i t h a
v a r i a b l e r a t e ( n o r ma l u p t o > 2 0 0 b e a t s / mi n u t e ) w i t h a n a b s e n c e o f P w a v e s o n
the E C G. If there is an aberrant conduction from the atria to ventricles or prior
b u n d l e b r a n c h b l o c k , i t ma y b e d i f f i c u l t t o d i s t i n g u i s h a t r i a l f i b r i l l a t i o n f r o m
v e n t r i c u l a r t a c h y c a r d i a ( V T ) . U s u a l l y, a t r i a l f i b r i l l a t i o n h a s a n i r r e g u l a r r h y t h m,
a n d V T h a s a r e g u l a r r h y t h m.
3. A t r i a l f l u t t eI nr a t r i a l f l u t t e r, c a r d i a c mo n i t o r i n g s h o w s t h e c l a s s i c s a w t o o t h
appearance in leads II, III, and aVF with a rate between 280 and 320
b e a t s / mi n u t e . T h e v e n t r i c l e s h a v e a s l o w e r r a t e , d e mo n s t r a t i n g a b l o c k o f e i t h e r
2 : 1 o r 4 : 1 w i t h a c o r r e s p o n d i n g r a t e o f 1 5 0 t o 7 5 b e a t s / mi n u t e . B y d o i n g a
c a r o t i d ma s s a g e o r u s i n g a d e n o s i n e i f t h e ma s s a g e f a i l s t o s l o w t h e a t r i a l r a t e ,
o n e ma y b e a b l e t o s h o w f l u t t e r w a v e s .
4. P a r o x y s m a l s u p r a v e n t r i c u l a r t a c h y cPaarrdoixy
a s ma l s u p r a v e n t r i c u l a r
t a c h y c a r d i a i s a r h y t h m t h a t i s mo s t c o mmo n l y c a u s e d b y t h e r e e n t r y o f t h e a t r i a
i mp u l s e a t t h e a t r i o v e n t r i c u l a r ( AV ) n o d e . M o r p h o l o g y o f t h e r h y t h m s h o w s a
s u d d e n o n s e t w i t h a n a r r o w a n d r e g u l a r Q R S c o mp l e x w i t h o u t o b v i o u s P w a v e s
a n d a r a t e f r o m 1 2 0 t o 1 9 0 b e a t s / mi n u t e . Wo l f f - P a r k i n s o n - W h i t e s y n d r o me
o c c u r s w h e n t h e r e i s a n t e g r a d e c o n d u c t i o n v i a a n a c c e s s o r y p a t h w a y, p r o d u c i n g
d e l t a w a v e s ( u p s l o p i n g p r i o r t o t h e Q R S c o mp l e x) a n d s h o r t e n e d P R i n t e r v a l s .
5. M u l t i f o c a l a t r i a l t a c h y c a r d i a (M
MuAT
l t i )f o c a l a t r i a l t a c h y c a r d i a i s a n
a r r h y t h mi a t h a t ma y o c c u r i n c a s e s o f me t a b o l i c o r e l e c t r o l y t e d i s o r d e r s ,
p u l mo n a r y d i s e a s e s , o r d i g i t a l i s t o xi c i t y. T h e r a t e o f M AT i s u p t o 1 4 0
b e a t s / mi n u t e w i t h a n i r r e g u l a r l y i r r e g u l a r r h y t h m a n d v a r y i n g P P, R R , a n d P R
intervals.
B. Wide complex tachycardia (QRS > 120 millisecond)
T h e r e c a n b e mu c h d i f f i c u l t y i n d i s t i n g u i s h i n g s u p r a v e n t r i c u l a r t a c h y c a r d i a w i t h a n
aberrant conduction from VT. T he B rugada criteria can help discern the two pattern
(1,2) .
1. I f a n R S c o mp l e x c a n n o t b e i d e n t i f i e d i n a n y p r e c o r d i a l l e a d s , V T c a n b e ma d e
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7.12 - Tachycardia
w i t h 1 0 0 % s p e c i f i c i t y.
2. I f t h e r e a r e R S c o mp l e xe s i n mo r e t h a n o n e l e a d , t h e n t h e R S i n t e r v a l ( t h e s t a r t
o f t h e R w a v e u p t o t h e d e e p e s t p a r t o f t h e S w a v e ) i s me a s u r e d . I f t h i s i s > 1 0 0
mi l l i s e c o n d s , t h e n V T c a n b e d i a g n o s e d w i t h 9 8 % s p e c i f i c i t y.
P. 1 4 8
3. I f t h e R S i n t e r v a l i s < 1 0 0 mi l l i s e c o n d s , t h e n t h e r e ma y b e e i t h e r a v e n t r i c u l a r o r
a s u p r a v e n t r i c u l a r o r i g i n s i t e o f t h e t a c h y c a r d i a a n d AV d i s s o c i a t i o n mu s t b e
e v a l u a t e d . AV d i s s o c i a t i o n i s 1 0 0 % s p e c i f i c f o r V T b u t h a s a l o w s e n s i t i v i t y.
4. I f t h e R S i n t e r v a l i s < 1 0 0 mi l l i s e c o n d s a n d t h e r e i s n o d e mo n s t r a b l e AV
dissociation, then look for a R B B B-like pattern (Q R S polarity is positive in lead
V1 a n d V2 w i t h a n r S c o mp l e x 6i )n oVr a L B B B - l i k e p a t t e r n ( Q R S p o l a r i t y
n e g a t i v e i n l e a d1s aVn d V2 a n d a Q o r Q S w a v e i n l e6a) .d I V
f these are
present, then VT is indicated.
C. Ventricular fibrillation
T h i s i s t e c h n i c a l l y n o t a t a c h y c a r d i a b u t r a t h e r r e p r e s e n t s a b n o r ma l v e n t r i c u l a r
d e p o l a r i za t i o n s i n s e v e r a l a r e a s t h a t p r e v e n t a n u n i f i e d c o n t r a c t i o n o f c a r d i a c
mu s c l e . T h e r h y t h m o n t h e E C G h a s t h e a p p e a r a n c e o f a b a g o f w o r ms , s h o w i n g
v a r y i n g a mp l i t u d e s o f e i t h e r c o a r s e w a v e s o r f i n e w a v e s .
References
1 . S c h e i n ma n M . T a c h y a r r h y t h mi a s i n p r i ma r y c a r d i o l o g y. I n : G o l d ma n L ,
B r a u n w a l d E , e dHsear
. t di s eas e: a t ex t book of c ar di ov as c ul ar medi
.
c i ne
P h i l a d e l p h i a , PA : W B S a u n d e r s , 1 9 9 8 : 3 3 0 3 5 2 .
2 . We l l e n s H J . E l e c t r o p h y s i o l o g y : v e n t r i c u l a r t a c h y c a r d i a : d i a g n o s i s o f b r o a d
Q R S c o mp l e x t a c h y c a r H
d iear
a . t2 0 0 1 ; 8 6 : 5 7 9 .
3 . B r u g a d a P. A n e w a p p r o a c h t o t h e d i f f e r e n t i a l d i a g n o s i s o f a r e g u l a r
t a c h y c a r d i a w i t h a w i d e Q R S c o mp
C i rl ce ul
x. at i on
1991;83:16491659.
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> T a b l e o f C o n t e n t s > 8 - R e s p i r a t o r y P r o b l e ms > 8 . 1 - C o u g h
8.1
Cough
Dsire A. Lie
I . Background
C o u g h i s a mo n g t h e t o p t e n r e a s o n s f o r v i s i t s t o f a mi l y p h y s i c i a n s i n t h e U n i t e d
S t a t e s a n d t h e f i f t h mo s t c o mmo n s y mp t o m p r e s e n t i n g i n o u t p a t i e n t c a r e . I t a c c o u n t
f o r 2 0 0 t o 4 0 0 mi l l i o n e p i s o d e s o f i l l n e s s p e r y e a r.
T hree causal conditions increasing in frequency over the past two decades are
a s t h ma , g a s t r o e s o p h a g e a l r e f l u x d i s e a s e ( G E R D ) , a n d c h r o n i c o b s t r u c t i v e
p u l mo n a r y d i s e a s e ( C O P
1,2)
D ).( B r o n c h i t i s i s o n e o f t h e mo s t c o mmo n c a u s e s o f
c o u g h i n t h e p r i ma r y c a r e s e3)
t t .i nPg o(s t n a s a l d r i p , a s t h ma , a n d G E R D a c c o u n t f o r
a l mo s t 9 0 % o f c a s e s o f c h r o n i c c4)o .u g h (
I I I . Evaluation
A. History
A thorough history is vital to accurate diagnosis.
1. C o u g h c h a r a c t e r i s t iI nc se v a l u a t i n g c o u g h a s a s y mp
5)t,o am d( i s t i n c t i o n h a s
t o b e ma d e a mo n g t h e f o l l o w i n g :
a. N o r ma l v e r s u s p a t h o l o g i c c o u g h
b. A c u t e ( < 3 w e e k s ) v e r s u s c h r o n i c ( > 3 w e e k s ) c o u g h
c. R e s p i r a t o r y v e r s u s n o n r e s p i r a t o r y c a u s e s
d. P e d i a t r i c v e r s u s a d u l t c o n d i t i o n s .
2. C h a r a c t e r i s t i c s o f t h e c oW
u gh ha t i s t h e t y p e o f c o u g h ( b a r k i n g , b r a s s y,
w h e e zy, n o c t u r n a l , o r p a r o xy s ma l ) ? W h a t a r e t h e d u r a t i o n , t i mi n g , a n d t r i g g e r s ?
A r e t h e r e a s s o c i a t e d s y mp t o ms o f f e v e r, s p u t u m p r o d u c t i o n , d y s p n e a ,
h e mo p t y s i s , a n d w e i g h t l o s s ? A r e t h e r e c l e a r r e l i e v i n g f a c t o r s ? A s k s p e c i f i c a l l y
a b o u t p o s t n a s a l d r i p , b e c a u s e p a t i e n t s o f t e n d o n o t v o l u n t e e r t h i s i n f o r ma t i o n . A
good history is the key to diagnosis.
a. U p p e r r e s p i r a t o r y c a u s e s mo s t c o mmo n l y r e l a t e t o p o s t n a s a l d r i p . I n a d u l t s ,
sinusitis, pharyngitis, and allergic rhinitis should be considered. In children
c o n c o mi t a n t o t i t i s me d i a s h o u l d b e e xc l u d e d .
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8.1 - Cough
b. L o w e r r e s p i r a t o r y c a u s e s i n c l u d e l u n g ( b r o n c h i t i s , a s t h ma , p n e u mo n i a , a n d
bronchiectasis, and in children, foreign body aspiration) and cardiac
( c o n g e s t i v e h e a r t f a i l u r e [ C H F ] a n d mi t r a l s t e n o s i s ) c o n d i t i o n s .
c. N o n r e s p i r a t o r y c a u s e s i n c l u d e G E R D , d r u g e f f e c t s ( e . g . , a n g i o t e n s i n c o n v e r t i n g e n zy me [ A C E ] i n h i b i t o r s ) , a n d p s y c h o g e n i c c o n d i t i o n s .
3. S m o k i n gP a t i e n t s w h o s mo k e s h o u l d b e i d e n t i f i e d e a r l y, b e c a u s e o f t h e
p o s s i b l e r i s k o f b r o n c h i t i s a n d l u n g c a n c e r. P a s s i v e s mo k i n g i s a l s o a r i s k f a c t o
e s p e c i a l l y i n c h i l d r e n . O f f i c e v i s i t s f o r c o u g h p r o v i d e a n o p p o r t u n i t y f o r s mo k i n g
c e s s a t i o n e d u c a t i o n . S mo k i n g c e s s a t i o n h a s b e e n s h o w n t o r e d u c e r e s p i r a t o r y
s y mp t o ms b y 5 0 % . I n s mo k e r s , b r o n c h o g e n i c c a n c e r s h o u l d b e c o n s i d e r e d a
p o s s i b l e e t i o l o g y i n t h e p r e s e n c e o f a n e w o r r e c e n t c h a n g e i n c h r o n i c s mo k e r '
c o u g h a c o u g h p e r s i s t i n g f o r mo r e t h a n 1 mo n t h a f t e r s mo k i n g c e s s a t i o n a n d
h e mo p t y s i s w i t h o u t i n f e c t i o n .
4. P s y c h o s o c i a l f a c t oTrhse p s y c h o s o c i a l i mp a c t o f c o u g h r e f l e c t s s e v e r i t y a n d
t h e n e e d f o r f u r t h e r w o r k u p . H a s t h e p a t i e n t mi s s e d s c h o o l o r w o r k ? I s t h e
s l e e p i n g p a r t n e r d i s t u r b e d ? I s t h e r e a v o i d a n c e o f e xe r c i s e b e c a u s e i t t r i g g e r s
c o u g h ? I n c h r o n i c , e p i s o d i c c o u g h , a c o r r e c t d i a g n o s i s o f a s t h ma c a n
c o n s i d e r a b l y i mp r o v e q u a l i t y o f l i f e . A p s y c h o g e n i c c a u s e f o r c o u g h a n d
b e h a v i o r a l p r o b l e ms i n c h i l d r e n ma y b e u n ma s k e d h e r e .
5. O t h e r i n f o r m a t i oAns s o c i a t e d c h e s t p a i n s h o u l d d i r e c t t h e h i s t o r y t o w a r d
p l e u r i s y o r r i b f r a c t u r e s e c o n d a r y t o c h r o n i c c o u g h . O c c u p a t i o n a l e xp o s u r e s
( t o xi c f u me s , c h e mi c a l s , b i r d s , a n d a n i ma l s ) , s y s t e mi c d i s e a s e s ( r h e u ma t o i d
P. 1 5 2
a r t h r i t i s , b r e a s t a n d p r o s t a t e c a n c e r me t a s t a s e s , a n d h u ma n i mmu n o d e f i c i e n c y
v i r u s [ H I V ] d i s e a s e ) , a n d d r u g e xp o s u r e ( A C E i n h i b i t o r s , c y c l o p h o s p h a mi d e , a n d
me t h o t r e xa t e ) a r e i mp o r t a n t f a c t o r s t o c o n s i d e r. C o u g h w i t h s i g n i f i c a n t w e i g h t
l o s s s h o u l d t r i g g e r a w o r k u p f o r t u b e r c u l o s i s ( T B ) , H I V, o r l u n g c a n c e r i n t h e
s mo k e r.
1. F o c u s e d p h y s i c a l e x a m i n aTt ihoi ns s h o u l d i n c l u d e v i t a l s i g n s ( t e mp e r a t u r e ,
p u l s e , r e s p i r a t o r y r a t e , a n d b l o o d p r e s s u r e ) , e a r, n o s e , s i n u s e s , t h r o a t ( E N S T ) ,
a n d a f u l l l u n g e xa mi n a t i o n w i t h t h e c h e s t u n c o v e r e d . N o r ma l l u n g e xa mi n a t i o n
o f t e n e xc l u d e s p n e u mo n i a b u t n o t a s t h ma , b r o n c h i t i s , C O P D , G E R D , o r l u n g
c a n c e r. I t i s mo r e e f f e c t i v e t o e xa mi n e t h e l u n g b e f o r e t h e E N S T i n y o u n g
c h i l d r e n b e c a u s e t h e E N S T e xa mi n a t i o n i s mo r e t r a u ma t i c a n d c a n i n d u c e
c r y i n g . I n t h e o l d e r p a t i e n t , e s p e c i a l l y t h e p o s t me n o p a u s a l w o ma n , r i b p a l p a t i o n
ma y b e i n c l u d e d t o i s o l a t e f r a c t u r e s e c o n d a r y t o o s t e o p o r o s i s .
2. A d d i t i o n a l p h y s i c a l e x a m i n aCt ai or dni o v a s c u l a r e xa mi n a t i o n i s d i r e c t e d a t a
d i a g n o s i s o f C H F. A s s o c i a t e d l y mp h a d e n o p a t h y s u g g e s t s i n f e c t i o n o r n e o p l a s m.
Wa s t i n g c a n b e o mi n o u s ( c a n c e r o r H I V ) . A b d o mi n a l e xa mi n a t i o n ma y r e v e a l a
t e n d e r e n l a r g e d l i v e r i n C H F o r e p i g a s t r i c t e n d e r n e s sCihna G
p tEe R
r sD7(. 5
a n d 9 . 6) .
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C. Testing
1. C l i n i c a l l a b o r a t o r y t eMs ot ss t a c u t e p r e s e n t a t i o n s o f c o u g h d o n o t r e q u i r e
blood, urine, or other laboratory tests. White blood count with differential count
a n d b l o o d c u l t u r e s a r e i n d i c a t e d f o r p n e u mo n i a . G r a m' s s t a i n a n d c u l t u r e o f
sputum are rarely practical in the office. A purified protein derivative test shoul
be placed early if T B is suspected, unless the patient is known to be anergic or
t h o u g h t t o h a v e o v e r w h e l mi n g a c t i v e T B . S y s t e mi c c a u s e s r e q u i r e t e s t i n g
specific to the disease in question.
2. R a d i o l o g i c t e sAt sc h e s t x- r a y i s n o t i n d i c a t e d f o r u p p e r r e s p i r a t o r y c a u s e s o r
b r o n c h i t i s . I t i s o n l y u s e f u l w h e n p n e u mo n i a , T B , C O P D , C H F, o r c a n c e r
( p r i ma r y o r me t a s t a t i c ) i s b e i n g c o n s i d e r e d . C o mp u t e d t o mo g r a p h y o f t h e
s i n u s e s i s mo r e s e n s i t i v e a n d s p e c i f i c t h a n p h y s i c a l e xa mi n a t i o n t o d i f f e r e n t i a t e
sinusitis from other causes of cough.
3. P u l m o n a r y f u n c t i o n t eAs tssi mp l e p e a k f l o w me t e r u s e d w i t h a t h e r a p e u t i c t r i a l
o f b r o n c h o d i l a t o r s w i l l i d e n t i f y mo s t c a s e s o f a s t h ma . T h i s i mp o r t a n t t e s t s h o u l d
b e s u p e r v i s e d b y t h e p h y s i c i a n o r a n e xp e r i e n c e d n u r s e . A d d i t i o n a l t e s t i n g i s
s u g g e s t e d f o r C O P D a n d p u l mo n a r y f i b r o s i s .
4. I n v a s i v e t e s tBsr o n c h o s c o p y i s u s e f u l f o r f o r e i g n b o d y a s p i r a t i o n , c a n c e r, o r
c h r o n i c i n t e r s t i t i a l l u n g d i s e a s e . E s o p h a g e a l p H mo n i t o r i n g mo s t l i k e l y c o n f i r ms
suspected G E R D.
I V. Diagnosis
A c ut e c ough
is likely to be infectious. A pertinent observation is that physicians
o v e r t r e a t a c u t e b r o n c h i t i s w i t h a n t i b i o t i c s . T h e l i t e r a t u r e s u g g e s t s t h a t mo s t c a s e s
a r e v i r a l i n o r i g i n , a n d a n t i b i o t i c s a r e i n eCf hr
f e oni
c t i vce .c ough
has a longer list of
d i f f e r e n t i a l d i a g n o s e s . A s t h ma t e n d s t o b e u n d e r d i a g n o s e d i n a d u l t s a n d c h i l d r e n .
S mo k i n g - r e l a t e d c a u s e s s h o u l d p r o mp t e d u c a t i o n a l i n t e r v e n t i o n a n d w o r k u p ,
e s p e c i a l l y i n o l d e r p a t i e n t s . G E R D i s a d i a g n o s i s o f t e n mi s s e d b e c a u s e i t i s n o t
c o n s i d e r e d . O f t e n , mo r e t h a n o n e o f f i c e v i s i t i s n e e d e d t o u n r a v e l t h e c a u s e o f
c h r o n i c c o u g h . U p t o 8 0 % o f c a s e s h a v e mu l t i p l e6) .c aMuas keisn g( a n a c c u r a t e
d i a g n o s i s i s e s s e n t i a l t o s u c c e s s f u l t r e a t me n t . O f t h o s e p r e s e n t i n g w i t h c o u g h , 9 0 %
c a n b e a d e q u a t e l y ma n a g e d i n t h e f a mi l y p h y s i c i a n ' s o f f i c e , a l t h o u g h i t c a n t a k e 3 t
5 mo n t h s t o a r r i v e a t a c o r r e c t d i a g n o s i s i n s o me
2) . cRaesf e sr r a( l t o a p u l mo n a r y
s p e c i a l i s t i s n e e d e d o n l y i n c o mp l i c a t e d c a s e s ( e . g . , c a n c e r, o c c u p a t i o n a l a n d
connective tissue diseases, and failed therapy).
B. Special concerns
F a i l u r e t o i mp r o v e w i t h a p p r o p r i a t e ma n a g e me n t o v e r 4 w e e k s s i g n a l s a n e e d f o r
mo r e e xt e n s i v e w o r k u p t o e xc l u d e T B , a d u l t - o n s e t a s t h ma , p e n i c i l l i n - r e s i s t a n t
pneumoc oc c us
, l u n g c a n c e r, a n d i mmu n o s u p p r e s s i o n .
P. 1 5 3
266 / 652
tmdmss
8.1 - Cough
References
1 . We i s s B D20
. c ommon pr obl ems i n pr i mar .y Nc ar
e we Yo r k , N Y : M c G r a w - H i l l ,
1999.
2 . L a w l e r W R . A n o f f i c e a p p r o a c h t o t h e d i a g n o s i s o f c h r oAnm
i c Fcam
ough.
P hy s i c i an1 9 9 8 ; 5 8 ( 9 ) : 2 0 1 5 2 0 2 2 .
3 . H e a t h J M . C h r o n i c b r o n c h i t i s : p r i ma r y c a r e ma nAa m
g eF
me
am
n t .P hy s i c i an
1998;57(10):23652372, 23762378.
4 . M e l l o C J , I r w i n R S , C u r l e y F J . P r e d i c t i v e v a l u e s o f t h e c h a r a c t e r, t i mi n g a n d
c o mp l i c a t i o n s o f c h r o n i c c o u g h i n d i a g n o s i n g iAt sr ccha uI nt
s eer
. n M ed
1996;156:997.
5 . I r w i n R S . M a n a g i n g c o u g h a s a d e f e n s e me c h a n i s m a n d a s a s y mp t o m. A
c o n s e n s u s r e p o r t o f t h e A me r i c a n c o l l e g e o f c h e s t pChhes
y s i ct 1i a9n9s8. ; 11 4 :
133S181S.
6 . I r w i n R S . S i l e n c i n g c h r o n i c cH
o uos
g hp . P r ac1t 9 9 9 ; 3 4 : 5 3 6 0 .
267 / 652
tmdmss
8.2 - Cyanosis
> T a b l e o f C o n t e n t s > 8 - R e s p i r a t o r y P r o b l e ms > 8 . 2 - C y a n o s i s
8.2
Cyanosis
J a n i s F. N e u m a n
I . Background
C y a n o s i s i s a b l u i s h d i s c o l o r a t i o n o f t h e s k i n a n d mu c o u s me mb r a n e s c a u s e d b y
i n c r e a s e d a mo u n t s o f u n s a t u r a t e d h e mo g l o b i n i n t h e b l o o d .
F o r c y a n o s i s t o a p p e a r, 5 g / 1 0 0 mL o f r e d u c e d b l o o d h e mo g l o b i n i s r2e q u i r e d . A n O
s a t u r a t i o n < 7 5 % o r a 2P ao O
f 4 0 mm H g r e s u l t s i n c y a n1)
o s. i O
s xy
( gen delivery to
t h e t i s s u e s d e p e n d s o n a n i n t a c t r e s p i r a t o r y s y s t e m t o p r o v i d e o xy g e n f o r
h e mo g l o b i n s a t u r a t i o n , t h e c o n c e n t r a t i o n o f h e mo g l o b i n , t h e c a r d i a c o u t p u t a n d
r e g i o n a l mi c r o v a s c u l a t u r e , a n d a n o xy h e mo g l o b i n u n l o a d i n g 2)
me
. chanism (
Cyanosis can be considered central or peripheral, based on the underlying
a b n o r ma l i t y.
A.
Central cyanosis includes conditions that lead to arterial desaturation such as

d e c r e a s e d i n s p i r e d o xy g e n t e n s i o n , p u l mo n a r y d i s e a s e , a n d c o n d i t i o n s c a u s i n g r i g h
t o l e f t s h u n t s ( e . g . , c o n g e n i t a l h e a r t d i s e a s e a n d i n t r a p u l mo n a r y s h u n t s ) . A b n o r ma l
h e mo g l o b i n l e v e l s a r e a l s o c o n s i d e r e d c e n t r a l . D e c r e a s e d b l o o d o xy g e n a t i o n ( c e n t r
cyanosis) is usually caused by one of the following:
1. O b s t r u c t i o n t o t h e i n t a k e o f o xy g e n ( e p i g l o t t i t i s a n d a c u t e
l a r y n g o t r a c h e o b r o n c h i t i s , a s t h ma , c h r o n i c b r o n c h i t i s o r e mp h y s e ma , a n d f o r e i g n
body aspiration).
2. D e c r e a s e d a b s o r p t i o n o f o xy g e n , a s o c c u r s w i t h a n a l v e o l a r c a p i l l a r y b l o c k
( s a r c o i d , p u l mo n a r y f i b r o s i s , p n e u mo n i a , p u l mo n a r y e d e ma , a l v e o l a r
p r o t e i n o s i s ) . Ve n t i l a t i o n p e r f u s i o n ( [ V w i t h d o t a b o v e ] / [ Q w i t h d o t a b o v e ] )
d e f e c t s f r o m e mp h y s e ma , p n e u mo c o n i o s e s , a n d s a r c o i d a l s o2d e c r e a s e O
absorption.
3. D e c r e a s e d p e r f u s i o n o f t h e l u n g w i t h b l o o d ( s h o c k , s e p t i c , o r c a r d i o g e n i c ;
p u l mo n a r y e mb o l u s ; p u l mo n a r y v a s c u l a r s h u n t s f r o m p u l mo n a r y h e ma n g i o ma ;
congenital heart disease).
4. R e d u c e d i n t a k e o f o xy g e n f r o m a n a t mo s p h e r e w i t h a d e c r e a s e d o xy g e n
268 / 652
tmdmss
8.2 - Cyanosis
concentration.
5. A d e f e c t i v e h e mo g l o b i n u n a b l e t o a t t a c h t o o xy g e n ( me t h e mo g l o b i n e mi a ,
s u l f h e mo g l o b i n e mi a , c a r b o n mo n o xi d e p o i s o n i n g , a n d o t h e r
h e mo g l o b i n o p a t h i e s ) .
B.
P e r i p h e r a l c y a n o s i s i s c a u s e d b y r e d u c e d c a r d i a c o u t p u t , c o l d e xp o s u r e , a n d a r t e r i a
or venous obstruction. Peripheral cyanosis occurs with the following:
1. R e d u c e d c a r d i a c o u t p u t f r o m a c u t e my o c a r d i a l i n f a r c t i o n o r o t h e r c a u s e s o f
p u mp f a i l u r e
2. L o c a l o r r e g i o n a l p h e n o me n o n f r o m c o l d e xp o s u r e , a r t e r i a l o b s t r u c t i o n f r o m
e mb o l u s o r t h r o mb o s i s , a n d v e n o u s s t a s i s o r o b s t r u c t i o n
3. C o l d e xp o s u r e ( R a y n a u d ' s d i s eCahs ae p) t(e r 7 . 11
)
P. 1 5 4
I I I . Evaluation
A. History
1. W h e n d i d t h e c y a n o s i s a p p e a r ? I s t h e c y a n o s i s o f r e c e n t o n s e t o r h a s i t b e e n
present since birth? A history of squatting episodes in childhood and
congenital cyanosis suggest congenital heart disease. Chronic cyanosis caused
b y me t h e mo g l o b i n e mi a c a n b e c o n g e n i t a l o r a c q u i r e d . O t h e r c a u s e s o f c h r o n i c
c y a n o s i s i n c l u d e c h r o n i c o b s t r u c t i v e p u l mo n a r y d i s e a s e ( C O P D ) , p u l mo n a r y
f i b r o s i s , a n d p u l mo n a r y a t r i o v e n t r i c u l a r f i s t u l a . A c u t e a n d s u b a c u t e c y a n o s i s a r e
c a u s e d b y a c u t e my o c a r d i a l i n f a r c t , p n e u mo t h o r a x, p u l mo n a r y e mb o l u s ,
p n e u mo n i a , o r u p p e r a i r w a y o b s t r u c t i o n .
2. I s t h e p a t i e n t s y mp t o ma t i c o r a s y mp t o ma t i c ? A s y mp t o ma t i c p a t i e n t s ma y h a v e
me t h e mo g l o b i n e mi a ( c o n g e n i t a l o r d r u g - i n d u c e d ) , o r s u l f h e mo g l o b i n e mi a .
E xp o s u r e t o d r u g s ( p r e s c r i b e d a n d i l l i c i t ) o r e n v i r o n me n t a l f a c t o r s a r e i mp o r t a n t
i n t h e s e p a t i e n t s . I n t e r mi t t e n t c y a n o s i s , s k i n c o l o r c h a n g e s , a n d p a i n w i t h c o l d
e xp o s u r e s u g g e s t R a y n a u d ' s p h e n o me n o n . S y mp t o ma t i c p a t i e n t s , e s p e c i a l l y
w i t h c h e s t p a i n a n d r e s p i r a t o r y d i s t r e s s , a r e mo r e l i k e l y t o h a v e a c a r d i a c o r
p u l mo n a r y c a u s e o f c y a n o s i s .
3. D o e s t h e p a t i e n t h a v e k n o w n r i s k f a c t o r s f o r c a r d i a c o r p u l mo n a r y d i s e a s e ,
i n c l u d i n g s mo k i n g , h y p e r l i p i d e mi a , a s t h ma , d r u g a b u s e ( e s p e c i a l l y
me t h a mp h e t a mi n e s ) , s e v e r e o b e s i t y ( s l e e p a p n e a ) , n e u r o mu s c u l a r d i s e a s e , o r
a u t o i mmu n e d i s e a s e ? D o e s t h e p a t i e n t h a v e c h e s t p a i n o r i n t e r mi t t e n t c y a n o s i s
w i t h e xe r c i s e , s u g g e s t i n g a n g i n a ? C h e s t p a i n c a n b e p r e s e n t w i t h a c u t e
p u l mo n a r y e mb o l i o r p n e u mo t h o r a x. I s t h e r e a c o u g h a n d f e v e r s u g g e s t i n g
p n e u mo n i a ? H a s t h e p a t i e n t h a d a n y o c c u p a t i o n a l o r e n v i r o n me n t a l e xp o s u r e s
t h a t mi g h t c a u s e p u l mo n a r y p r o b l e ms ?
4. I s t h e r e a f a mi l y h i s t o r y o f a b n o r ma l h e mo g l o b i n s o r p u l mo n a r y d i s e a s e ? H a s
269 / 652
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8.2 - Cyanosis
the patient suffered an episode of hypotension that could produce adult

r e s p i r a t o r y d i s t r e s s s y n d r o me ( A R D S ) , s u c h a s s e p s i s o r h e a r t f a i l u r e ?
1. I n i t i a l a s s e s s m eVi
n t a l s i g n s a r e v e r y i mp o r t a n t ; t a c h y c a r d i a s u g g e s t s c a r d i a c
a r r h y t h mi a , s h o c k , v o l u me d e p l e t i o n , a n e mi a , Cohr afpetveer r 7(. 1) .2 A n
i n c r e a s e d o r d e c r e a s e d r e s p i r a t o r y r a t e a n d u s e o f a c c e s s o r y mu s c u l a t u r e
s u g g e s t h y p o xi a f r o m a n y c a u s e . H y p o t e n s i o n c a n s i g n a l v a s c u l a r c o l l a p s e f r o m
my o c a r d i a l i n f a r c t i o n , s e p t i c s h o c k , o r p u l mo n a r y e mb o l u s .
2. A d d i t i o n a l p h y s i c a l e x a m i n aSt ti roi dn o r s u g g e s t s u p p e r a i r w a y o b s t r u c t i o n .
E xa mi n e t h e p h a r y n x f o r e v i d e n c e o f o b s t r u c t i o n . I f e p i g l o t t i t i s o r t h e p r e s e n c e
of a foreign body is suspected, be prepared to intubate the patient. Check the
neck for evidence of jugular venous distention. Auscultate the chest for rales,
s u g g e s t i v e o f p u l mo n a r y e d e ma ; w h e e zi n g a n d r h o n c h i c o n s i s t e n t w i t h r e a c t i v e
a i r w a y d i s e a s e ; o r a b s e n c e o f b r e a t h s o u n d s , s u g g e s t i v e o f p n e u mo n i a , o r
p n e u mo t h o r a x. A u s c u l t a t e t h e h e a r t f o r mu r mu r s , a r r h y t h mi a s , a n d a b n o r ma l
h e a r t s o u n d s . F e e l t h e p u l s e s i n t h e e xt r e mi t i e s t o a s s e s s f o r a r t e r i a l e mb o l u s o
v e n o u s t h r o mb o s i s , e s p e c i a l l y i f c y a n o s i s i s l o c a l i ze d t o o n e e xt r e mi t y. E xa mi n e
t h e a b d o me n f o r e v i d e n c e o f i n t r a a b d o mi n a l c a t a s t r o p h e o r a n e u r y s m. E xa mi n e
t h e n a i l s f o r e v i d e n c e o f c l u b b i n g , w h i c h i s s u g g e s t i v e o f c h r o n i c p u l mo n a r y
disease.
C. Testing
1. P u l s e o xi me t r y e s t i ma t e s o xy g e n s a t u r a t i o n b u t d o e s n o t me a s u r e i t d i r e c t l y.
D i r e c t me a s u r e me n t s u s i n g a r t e r i a l b l o o d g a s e s ( A B G s ) a r e n e c e s s a r y t o
a s s e s s a p a t i e n t w i t h c y a n o s i s . P a t i e n t s w i t h a b n o r ma l h e mo g l o b i n s h a v e a
n o r ma l P a2O b u t d e c r e a s e d h e mo g l o b2i ns aOt u r a t i o n . P a t i e n t s w i t h c y a n o s i s
h a v e O2 s a t u r a t i o n < 7 5 % a n d 2P a<O
4 0 mm H g i f t h e y h a v e a n o r ma l
h e mo g l o b i n c o n c e n t r a t i o n . A l o w
2 iPs acOa u s e d b y r e s p i r a t o r y o r c a r d i a c
p r o b l e ms i n mo s t c i r c u ms t a n c e s .
2. A c h e s t r a d i o g r a p h h e l p s a s s e s s h e a r t s i ze a n d l u n g p a r e n c h y ma . I n f i l t r a t e s
s u g g e s t p n e u mo n i a , A R D S , o r p u l mo n a r y e d e ma . E xc l u d e p n e u mo t h o r a x. L o o k
for evidence of interstitial lung disease. Pleural effusion can represent infection
ma l i g n a n c y, o r p u l mo n a r y e dCe hma
a p (t e r 8 ). .4
3. A n e l e c t r o c a r d i o g r a m ma y d e mo n s t r a t e a c u t e my o c a r d i a l i n f a r c t i o n , a r r h y t h mi a ,
o r p e r i c a r d i a l p r o c e s s . P p u l mo n a l e , r i g h t v e n t r i c u l a r h y p e r t r o p h y, a n d R a xi s
s u g g e s t c h r o n i c p u l mo n a r y d i s e a s e .
P. 1 5 5
4. O t h e r t e s t s i n c l u d e v e n t i l a t i o n p e r f u s i o n s c a n n i n g , w h i c h ma y d e mo n s t r a t e
p u l mo n a r y e mb o l u s . P u l mo n a r y a r t e r y c a t h e t e r i za t i o n a n d p r e s s u r e
me a s u r e me n t s h e l p d i s t i n g u i s h c a r d i a c c a u s e s f r o m p u l mo n a r y c a u s e s o f
c y a n o s i s . P u l mo n a r y f u n c t i o n t e s t i n g c a n h e l p i n t h e d i a g n o s i s o f v a r i o u s
270 / 652
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8.2 - Cyanosis
p u l mo n a r y d i s e a s e s .
I V. Diagnosis
A.
F o c u s e d h i s t o r y, p h y s i c a l e xa mi n a t i o n , a n d d i a g n o s t i c t e s t i n g e l u c i d a t e t h e c a u s e o
c y a n o s i s i n a f f e c t e d p a t i e n t s . R e s p o n s e t o s u p p l2e me
c a nn taalls O
o help pinpoint
t h e c a u s e o f c y a n o s2)i s. D
( e c r e a s e d o xy g e n a t i o n s e c o n d a r y t o mi l d t o mo d e r a t e
v e n t i l a t i o n p e r f u s i o n mi s ma t c h e s c a u s e d b y p n e u mo n i a , p u l mo n a r y e mb o l u s , a n d
a s t h ma ma y b e r e v e r s i b l e w i t h s u p p l e me n t a l o xy g e n . S e v e r e [ V w i t h d o t a b o v e ] / [ Q
w i t h d o t a b o v e ] mi s ma t c h c a u s e d b y i n t r a p u l mo n a r y s h u n t i n g f r o m s e v e r e p u l mo n a r y
e d e ma o r A R D S ma y b e r e f r a c t o r y t o s u p p l e 2me
. M
n toadl eOr a t e [ V w i t h d o t
a b o v e ] / [ Q w i t h d o t a b o v e ] mi s ma t c h a s s o c i a t e d w i t h v e n t i l a t o r y f a i l u r e C O P D ma y
r e s p o n d t o s u p p l e me n t2a, l bOu t b e a w a r e o f i n c r e a s i n2g l eCvOe l s . A B G s d i r e c t l y
me a s u r e P a2Oa n d O2 s a t u r a t i o n . A b n o r ma l h e mo g l o b i n s w i l l a l s o b e me a s u r e d a n d
t h e y h e l p g u i d e t h e r a p y. H y p o xi a w i t h a n e l e2v astuegdg C
e sOt s C O P D o r a s t h ma ,
w h e r e a s h y p o xi a w i t h a n o r ma l o r d e c r e a2s seudg C
g eOs t s p n e u mo n i a , A R D S ,
p u l mo n a r y e d e ma , p u l mo n a r y e mb o l i , o r i n t e r s t i t i a l l u n1)g. d i s e a s e (
B.
O n c e t h e c a u s e o f t h e c y a n o s i s i s d e t e r mi n e d , t h e o b j e c t i v e i s t o t r e a t t h e u n d e r l y i n
p r o c e s s . C a u s e s o f p s e u d o c y a n o s i s i n c l u d e a r g y r i a o r b i s mu t h p o i s o n i n g ( s l a t e b l u e
g r a y c o l o r ) , h e mo c h r o ma t o s i s ( b r o w n i s h c o l o r ) , o r p o l y c y t h e mi a ( r u d d y r e d c o l o r ) .
For peripheral cyanosis caused by decreased cardiac output, correct the causes of
h y p o v o l e mi a ( e . g . , d e h y d r a t i o n , s h o c k , h e a r t f a i l u r e f r o m w h a t e v e r c a u s e ) . S u r g i c a l
c o n s u l t a t i o n ma y b e r e q u i r e d f o r a c u t e e mb o l i za t i o n o f a n e xt r e mi t y, a n d
a n t i c o a g u l a t i o n f o r v e n o u s t h r o mb o s i s .
References
1 . K h a n M GC
. ar di ac and pul monar y management
. P h i l a d e l p h i a , PA : L e a &
F e b i g e r, 1 9 9 3 : 8 1 8 8 2 5 .
2 . Wo o d l e y M , W h e l a nMAanual
.
of medi c al t her apeut
. Bi cos t o n , M A : L i t t l e ,
B r o w n a n d C o mp a n y, 1 9 9 3 : 1 7 9 1 8 1 .
Suggested Reading
H u r s t J W.M edi c i ne f or t he pr ac t i c i ng phy.s iBcoi an
ston, M A: B utterworthH e i n e ma n , 1 9 8 3 : 9 7 3 9 7 5 .
C o l l i n s R DD. y nami c di f f er ent i al di agnos
. P hi isl a d e l p h i a , PA : J . B . L i p p i n c o t t ,
1981:386388.
271 / 652
tmdmss
8.3 - Hemoptysis
> T a b l e o f C o n t e n t s > 8 - R e s p i r a t o r y P r o b l e ms > 8 . 3 - H e mo p t y s i s
8.3
Hemoptysis
Kathry n M. Larse n
Mary D. Knudtson
I . Background
H e mo p t y s i s i s d e f i n e d a s t h e c o u g h i n g u p o r e xp e c t o r a t i o n o f b l o o d f r o m t h e
t r a c h e o b r o n c h i a l t r e e , w h i c h c a n b e f r o m t h e t r a c h e a , t h e ma j o r a i r w a y s , o r t h e l u n g
p a r e n c h y ma . I t i s a n a l a r mi n g s y mp t o m t h a t u s u a l l y p r o mp t s t h e p a t i e n t t o s e e k
i mme d i a t e me d i c a l a t t e n t i o n .
H e mo p t y s i s c a n r a n g e i n s e v e r i t y f r o m t r i v i a l t o l i f e - t h r e a t e n i n g , a n d i s d u e t o
n u me r o u s c a u s e s . T h e c l i n i c i a n mu s t d e t e r mi n e t h e a n a t o mi c b l e e d i n g s i t e a n d t h e
u n d e r l y i n g c a u s e f o r t h e b l e e1)d .i nBg l e( e d i n g o r i g i n a t i n g f r o m t h e n a s o p h a r y n x o r
b l e e d i n g f r o m t h e g a s t r o i n t e s t i n a l t r a c t c a n mi mi c h C
e mo
h a p t ey rs i9s). 7.( A
t h o r o u g h e v a l u a t i o n i s n e c e s s a r y b e c a u s e t h e a mo u n t o f b l o o d e xp e c t o r a t e d d o e s
n o t c o r r e l a t e w i t h t h e s e r i o u s n e s s o f t h e c a u s e . A f t e r e xt e n s i v e e v a l u a t i o n , t h e r e i s
no
P. 1 5 6
i d e n t i f i a b l e c a u s e o f h e mo p t y s i s i n u p t o 3 0 % o f a f f e c t e d p a t i e n t s ; t h e s e p a t i e n t s a
c l a s s i f i e d a s h a v i n g c r y p t o g e n i c h e mo p t y s i s .
A. Etiology
T h e p a t h o g e n e s i s o f h e mo p t y s i s g e n e r a l l y r e s u l t s f r o m i n f l a mma t i o n o r i n j u r y t o t h e
t r a c h e o b r o n c h i a l mu c o s a ( e . g . , b r o n c h i t i s , b r o n c h i e c t a s i s , t u b e r c u l o s i s , s a r c o i d o s i s
b r o n c h o g e n i c c a r c i n o ma ) ; i n j u r y t o t h e p u l mo n a r y v a s c u l a t u r e ( e . g . , l u n g a b s c e s s ,
n e c r o t i zi n g p n e u mo n i a , p u l mo n a r y i n f a r c t i o n s e c o n d a r y t o e mb o l i za t i o n ) ; o r e l e v a t i o
o f t h e p u l mo n a r y c a p i l l a r y p r e s s u r e ( e . g . , p u l mo n a r y e d e ma , We g e n e r ' s
g r a n u l o ma t o s i s , G o o d p a s t u r e ' s s y n d r o me ) .
B. Epidemiology
T h e mo s t c o mmo n c a u s e s a r e a c u t e a n d c h r o n i c b r o n c h i t i s , f o l l o w e d b y
b r o n c h o g e n i c c a r c i n o ma a n d p n e u mo
2) . nLi au n( g t u mo r s a c c o u n t f o r 2 0 % o f t h e
c a s e s o f h e mo p t y s i s ; t h e y a r e u s u a l l y a s s o c i a t e d w i t h s mo k e r s o l d e r t h a n 4 0 y e a r s
who have had a change in cough pattern with an ache or pain in the chest. A
b l e e d i n g d i a t h e s i s o r t h e u s e o f a n t i c o a g u l a n t me d i c i n e ma y p r e s e n t w i t h h e mo p t y s i
272 / 652
tmdmss
8.3 - Hemoptysis
b u t u n d e r l y i n g p u l mo n a r y d i s e a s e mu s t a l w a y s b e e xc l u d e d . C h e s t t r a u ma i s a l e s s
c o mmo n c a u s e o f h e mo p t y s i s .
I I I . Evaluation
A. History
1. I d e n t i f i c a t i o n o f t h e b l e e d i n gWshiat te i s t h e s o u r c e o f t h e b l e e d i n g ? I s t h e
p r o b l e m t r u l y h e mo p t y s i s o r c o u l d t h e b l e e d i n g o r i g i n a t e i n a n o n p u l mo n a r y
location such as the nose and oropharynx or the gastrointestinal tract? Blood
t h a t i s c o u g h e d f r o m t h e r e s p i r a t o r y t r a c t i s b r i g h t r e d i n c o l o r a n d ma y b e f r o t h
o r mi xe d w i t h s p u t u m. H e mo p t y s i s i s mo r e l i k e l y a s s o c i a t e d w i t h a h i s t o r y o f
u n d e r l y i n g p u l mo n a r y d i s e a s e , s mo k i n g , o r mi t r a l v a l v e d i s e a s e . H e ma t e me s i s i s
a s s o c i a t e d w i t h b l o o d t h a t i s d a r k r e d , b r o w n , o r g r o u n d c o f f e e i n c o l o r a n d ma y
b e mi xe d w i t h f o o d p a r t i c l e s . H e ma t e me s i s i s f a v o r e d i n t h e p r e s e n c e o f a
p r e e xi s t i n g g a s t r o i n t e s t i n a l c o n d i t i o n , e s p e c i a l l y w i t h a h i s t o r y o f l i v e r d i s e a s e ,
alcohol use, or peptic ulcer disease. Sputum that is blood-streaked often arises
f r o m t h e n a s a l mu c o s a a n d o r o p h a r y n x.
2. C h a r a c t e r i s t i c s o f t h e s p uW
t uhm
at are the characteristics of the sputum in
t e r ms o f c o l o r, o d o r, a n d c o n s i s t e n c y ? A d e s c r i p t i o n o f t h e s p u t u m c a n a s s i s t i n
d e f i n i n g t h e d i s e a s e p r o c e s s c a u s i n g t h e h e mo p t y s i s : ( a ) f r o t h y, p i n k s p u t u m i s
s u g g e s t i v e o f p u l mo n a r y e d e ma f l u i d ; ( b ) p u t r i d o r f o u l - s me l l i n g s p u t u m s u g g e s t
a l u n g a b s c e s s ; ( c ) c u r r a n t j e l l y s p u t u m ma y s u g g e s t a n e c r o t i zi n g p n e u mo n i a ;
( d ) t h e s p u t u m o f p n e u mo c o c c a l p n e u mo n i a i s t y p i c a l l y r u s t - c o l o r e d a n d c a n b e
c o n f u s e d w i t h t r u e h e mo p t y s i s ; ( e ) l a r g e a mo u n t s o f b l o o d - s t r e a k e d s p u t u m
often suggest bronchiectasis.
3. O t h e r i n f o r m a t i oDno e s t h e p a t i e n t h a v e o t h e r a s s o c i a t e d s y mp t o ms ? C o u g h ,
d y s p n e a , a n d s p u t u m p r o d u c t i o n o v e r s e v e r a l y e a r s ma y s u g g e s t c h r o n i c
b r o n c h i t i s o r b r o n c h i e c t a s i s . We i g h t l o s s a n d f a t i g u e ma y s u g g e s t a n u n d e r l y i n g
ma l i g n a n c y, a n d f e v e r a n d n i g h t s w e a t s mi g h t i n d i c a t e t u b e r c u l o s i s . D o e s t h e
p a t i e n t h a v e a h i s t o r y o f k n o w n p u l mo n a r y, c a r d i a c , o r h e ma t o l o g i c p r o b l e ms ?
D o e s t h e p a t i e n t h a v e h e ma t u r i a , w h i c h mi g h t s u g g e s t a p u l mo n a r y r e n a l
s y n d r o meC(h a p t e r 1 0). ?2 D o e s t h e p a t i e n t s mo k e o r h a v e s p e c i f i c
e n v i r o n me n t a l e xp o s u r e s ? I s t h e p a t i e n t t a k i n g me d i c a t i o n s , e s p e c i a l l y
a n t i c o a g u l a n t s , t h a t mi g h t c o n t r i b u t e t o t h e b l e e d i n g ?
A f o c u s e d p h y s i c a l e xa mi n a t i o n s h o u l d i n c l u d e v i t a l s i g n s a n d e xa mi n a t i o n s o f t h e
n o s e , s i n u s e s , o r o p h a r y n x, n e c k , l u n g s , a n d h e a r t . T h e n e c k s h o u l d b e p a l p a t e d f o r
t h e p r e s e n c e o f l y mp h a d e n o p a t h y a n d i n s p e c t e d f o r j u g u l a r v e n o u s d i s t e n s i o n . T h e
l o w e r e xt r e mi t i e s s h o u l d b e c h e c k e d f o r e d e ma . E xa mi n a t i o n o f t h e s k i n ma y r e v e a l
l e s i o n s a s s o c i a t e d w i t h s y s t e mi c l u p u s e r y t h e ma t o s u s ; K a p o s i ' s s a r c o ma ; c l u b b i n g
( c o n s i s t e n t w i t h n e o p l a s m, b r o n c h i e c t a s i s , o r l u n g a b s c e s s ) ; o r e c c h y mo s i s r e l a t e d
t o a c o a g u l o p a t h y.
C. Testing
273 / 652
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8.3 - Hemoptysis
1. E v a l u a t i o n s h o u l d b e g i n w i t h a c h e s t x- r a y t o l o o k f o r p o s s i b l e c l u e s t o t h e
d i a g n o s i s : a ma s s l e s i o n , f o c a l o r d i f f u s e p a r e n c h y ma l d i s e a s e , p n e u mo n i t i s ,
a b s c e s s , i n f i l t r a t e , h i l a r a d e n o p a t h y, e n l a r g e d h e a r t , p u l mo n a r y e d e ma , c o i n
l e s i o n o f a s p e r g i l l o ma , o r t h e p e r i b r o n c h i a l c u f f i n g s u g g e s t i v e o f b r o n c h i e c t a s i s
A c o mp u t e d t o mo g r a p h y s c a n ma y b e n e c e s s a r y t o d e f i n e a l e s i o n s e e n o n
c h e s t x- r a y
P. 1 5 7
f i l m 3)
( . A d d i t i o n a l b a s i c t e s t i n g s h o u l d i n c l u d e a c o mp l e t e b l o o d c o u n t w i t h
differential and a coagulation profile. For patients in whom infection is
s u s p e c t e d , s k i n t e s t i n g , a G r a m' s s t a i n , a c i d - f a s t s t a i n , o r s p u t u m c u l t u r e s ma y
b e a p p r o p r i a t e . C y t o l o g i c e xa mi n a t i o n o f t h e s p u t u m i s i n d i c a t e d i n c a s e s o f
s u s p e c t e d ma l i g n a n c y.
2. O t h e r s p e c i a l t e s t s i n c l u d e f i b e r o p t i c b r o n c h o s c o p y, w h i c h i s u s e d t o l o c a l i ze t h
b l e e d i n g s i t e o f s p e c i f i c l e s i o n s n o t e d o n x- r a y f i l m. I t i s a l s o u s e d i n c a s e s o f
p e r s i s t e n t o r r e c u r r e n t b l e e d i n g a n d f o r s mo k e r s o l d e r t h a n 4 0 y e a r s w i t h a
n e g a t i v e c h e s t x- r a y s t u d y. Ve n t i l a t i o n p e r f u s i o n s c a n n i n g i s i n d i c a t e d i f
p u l mo n a r y e mb o l i s m i s s u s p e c t e d .
I V. Diagnosis
D e t e r mi n i n g t h e s i t e o f b l e e d i n g i s t h e f i r s t s t e p . I f t h e b l e e d i n g i s f r o m t h e
n a s o p h a r y n x o r g a s t r o i n t e s t i n a l t r a c t , t h e n i t i s n o t c l a s s i f i e d a s h e mo p t y s i s . T h e
basic approach depends on the severity of the bleeding. Most cases of blood-tinged
s p u t u m a r e u p p e r r e s p i r a t o r y i n n a t u r e a n d d o n o t r e q u i r e e xt e n s i v e w o r k u p .
B r o n c h i t i s i s t h e mo s t c o mmo n c a u s e . H o w e v e r, b r o n c h o g e n i c c a r c i n o ma , a n d
b r o n c h i e c t a s i s a r e a l s o c o mmo n c a u s e s t h a t r e q u i r e f u r t h e r4)e. v M
a liul da t i o n (
h e mo p t y s i s c a n b e e v a l u a t e d w i t h e l e c t i v e b r o n c h o s c o p y o f t h e r e s p i r a t o r y t r a c t .
M a s s i v e h e mo p t y s i s ( d e f i n i t i o n s i n t h e l i t e r a t u r e r a n g e f r o m 1 0 0 mL i n 2 4 h o u r s t o
1 , 0 0 0 mL o v e r s e v e r a l d a y s ) r e q u i r e s a n e me r g e n t d i a g n o s t i c a p p r o a c h , t y p i c a l l y
w i t h r i g i d b r o n c h o s c o5)p .y I(f h e mo p t y s i s p e r s i s t s d e s p i t e t r e a t me n t o f a p r e s u me d
i n f e c t i o n , b r o n c h i a l a r t e r i o g r a p h y w i t h e mb o l i za t i o n o r r e s e c t i o n o f t h e i n v o l v e d
s e g me n t ma y b e n e c e s s a r y.
References
1 . C o l i c e G L . H e mo p t y s i s : t h r e e q u e s t i o n s t h a t c a n d i r e c t ma
P nosa tggr
e me
ad n t .
M ed 1 9 9 6 ; 1 0 0 ( 1 ) : 2 2 7 2 3 6 .
2 . D i L e o M D , A me d e e R G , B u t c h e r R B . H e mo p t y s i s a n d p s e u d o h e mo p t y s i s : t h e
p a t i e n t e xp e c t o r a t i n g b lEo ar
o d .N os e T hr oat 1J9 9 5 ; 7 4 ( 1 2 ) : 8 2 2 8 2 4 , 8 2 6 8 2 8 .
3. Marshall T J , Flower C D, J ackson J E. T he role of radiology in the
i n v e s t i g a t i o n a n d ma n a g e me n t o f p a t i e n t s w i t h h eCmo
l i np tR
y sadi
i s .ol
1996;51(6):391400.
4 . M a r w a h O S , S h a r ma O P. B r o n c h i e c t a s i s : h o w t o i d e n t i f y, t r e a t , a n d p r e v e n t .
P os t gr ad M ed
1995;97(2):149150, 153156, 159.
274 / 652
tmdmss
8.3 - Hemoptysis
5 . C a h i l l B C , I n g b a r D H . M a s s i v e h e mo p t y s i s : a s s e s s me n t a n d ma n a g e me n t .
C l i n C hes t M ed
1994;15(1):147167.
275 / 652
tmdmss
8.4 - Pleural Effusion
> T a b l e o f C o n t e n t s > 8 - R e s p i r a t o r y P r o b l e ms > 8 . 4 - P l e u r a l E f f u s i o n
8.4
Pleural Effusion
M a r k F. G i g l i o
I . Background
Pleural effusions occur in a variety of illnesses. T he underlying causes range from

b e n i g n a t e l e c t a s i s t o ma l i g n a n c y. P l e u r a l e f f u s i o n s d e v e l o p i n 1 mi l l i o n p a t i e n t s e a c
y e a r i n t h e U n i t e d S t a1)
t e.s A(l t h o u g h e f f u s i o n s o c c u r w i t h i n t h e l u n g s a n d p l e u r a ,
t h e s o u r c e i s o f t e n f r o m o u t s i d e t h e p u l mo n a r y s y s t e m.
P l e u r a l e f f u s i o n s a r e o f t w o d i s t i n c t t y p e s : t r a n s u d a t i v e a n d e xu d a t i v e .
A.
Transudative effusions result from an elevated net hydrostatic pressure gradient.
C o mmo n c a u s e s i n c l u d e c o n g e s t i v e h e a r t f a i l u r e ( C H F ) , n e p h r o t i c s y n d r o me , a n d
c i r r h o s i s . G e n e r a l l y, t h e y r e q u i r e n o f u r t h e r w o r k u p w h e n i d e n t i f i e d a n d r e s p o n d t o
t r e a t me n t o f t h e p r i ma r y p r o b l e m.
B.
E xu d a t i v e e f f u s i o n s r e s u l t f r o m i n c r e a s e d p e r me a b i l i t y o f t h e p l e u r a l v e s s e l s . T h e
d i f f e r e n t i a l d i a g n o s i s e n c o mp a s s e s a b r o a d e r r a n g e o f c o n d i t i o n s , i n c l u d i n g
ma l i g n a n c y a n d i n f e c t i o n s .
P. 1 5 8
I I I . Evaluation
A. History
T h e p a t i e n t ' s h i s t o r y c a n o f t e n s u g g e s t t h a t a p l e u r a l e f f u s i o n i s p r e s e n t . S ma l l
e f f u s i o n s , h o w e v e r, ma y c a u s e n o s y mp t o ms . F r e q u e n t l y, a n u n d e r l y i n g d i s e a s e
c a u s e s t h e p a t i e n t ' s i n i t i a l s y mp t o ms .
1. P u l m o n a r yD y s p n e a i s t h e mo s t c o mmo n s y mp t o m. D i d i t d e v e l o p a c u t e l y o r
g r a d u a l l y ? I s t h e r e a d r y c o u g h p r e s e n t ? D o e s t h e p a t i e n t e xp e r i e n c e c h e s t
p a i n , e s p e c i a l l y p l e u r i t i c p a i n ? D o e s t h e p a i n v a r y Ci hn aqput ea rl i t8y). 5?(
T hs e ma i n g o a l h e r e i s t o t h i n k a b o u t u n d e r l y i n g
i l l n e s s e s t h a t mi g h t p r o d u c e a p l e u r a l e f f u s i o n . O r t h o p n e a , p a r o xy s ma l n o c t u r n a
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d y s p n e a , d y s p n e a o n e xe r t i o n , a n d p e d a l e d e ma s u g g e s t C H F. D o e s t h e p a t i e n t
h a v e e xe r t i o n a l c h e s t p a i n t h a t ma y b e a n g i n a ? H e mo p t y s i s , w e i g h t l o s s , a n d
a n o r e xi a p o i n t t o ma l i g n a n c y. H a s t h e p a t i e n t b e e n a c u t e l y i l l ? P r o d u c t i v e
c o u g h , f e v e r, c h i l l s , a n d n i g h t s w e a t s s u g g e s t p n e u mo n i a . D o e s t h e p a t i e n t h a v e
r i s k f a c t o r s f o r d e e p v e n o u s t h r o mb o s i s ( e . g . , r e c e n t t r a v e l , p r o l o n g e d
i mmo b i l i za t i o n , o r f r a c t u r e ) ? S y mp t o ms o f p u l mo n a r y e mb o l i s m i n c l u d e
t a c h y c a r d i a , h e mo p t y s i s , a n d d y s p n e a .
3. P a s t m e d i c a l h i s t oHr ay s t h e p a t i e n t h a d p r i o r p u l mo n a r y d i s e a s e s ? A r e
cardiac risk factors present? Is there a history of hepatic or renal disease? Has
the patient had cancer?
4. F a m i l y h i s t o rAyr e t h e r e f a mi l y me mb e r s w i t h p r e ma t u r e c o r o n a r y a r t e r y
d i s e a s e , t u b e r c u l o s i s , o r ma l i g n a n c y ?
5. S o c i a l h i s t o rDyo e s t h e p a t i e n t s mo k e ? D o e s t h e p a t i e n t u s e a l c o h o l
e xc e s s i v e l y ? W h e r e d o e s t h e p a t i e n t w o r k ? I s t h e r e s o l v e n t o r a s b e s t o s
e xp o s u r e a t w o r k ?
1. F o c u s e d p h y s i c a l e x a m i n aO
t i bosne r v e t h e p a t i e n t ' s a p p e a r a n c e a n d
respiratory effort. Is the patient splinting or showing signs of respiratory
d i s t r e s s ? Vi t a l s i g n s s h o u l d i n c l u d e r e s p i r a t o r y r a t e . I s t a c h y c a r d i a p r e s e n t
(C h a p t e r 7 . 1) ?2 T y p i c a l f i n d i n g s o n p u l mo n a r y e xa mi n a t i o n i n c l u d e d e c r e a s e d o r
absent breath sounds over the affected side, dullness to percussion, decreased
t a c t i l e f r e mi t u s , a n d p o s s i b l y s p l i n t i n g o f t h e a f f e c t e d s i d e . F i n d i n g s c a n b e
b i l a t e r a l ( e . g . , C H F ) o r u n i l a t e r a l . E xa mi n a t i o n c a n a l s o v a r y w i t h t h e s e v e r i t y o
t h e e f f u s i o n . F i n d i n g s a r e u s u a l l y n o r ma l w h e n l e s s t h a n 3 0 0 mL o f f l u i d i s
p r e s e n t . A p l e u r a l r u b ma y b e n o t e d . W i t h a l a r g e e f f u s i o n ( > 1 , 5 0 0 mL ) , t h e
a f f e c t e d h e mi t h o r a x i s o f t e n l a r g e r w i t h b u l g i n g i n2)
t e.r s p a c e s (
2. A d d i t i o n a l p h y s i c a l e x a m i n aI t i iosnn e c e s s a r y t o t h i n k i n t e r ms o f
differential diagnosis when looking for signs of underlying causes. Cardiac
e xa mi n a t i o n s h o u l d l o o k f o r s i g n s o f c o n g e s t i v e h e a r t f a i l u r e , i n c l u d i n g
c a r d i o me g a l y, d i s p l a c e d p o i n t o f ma xi ma l i mp u l s e3, ga an ldl oapn. S
Is a heart
mu r mu r p r e s e n t ? A b d o mi n a l e xa mi n a t i o n ma y r e v e a l h e p a t o me g a l y, l i v e r
tenderness, a fluid wave, and other signs of ascites. Are there signs of
ma l i g n a n c y, i n c l u d i n g g e n e r a l i ze d o r r e g i o n a l l y mp h a dCehnaoppt ae tr hs y1(5 . 1
a n d 1 5 . 2) ?
C. Testing
1. R a d i o g r a p h yI n i t i a l t e s t i n g f o c u s e s o n c o n f i r mi n g t h a t a p l e u r a l e f f u s i o n i s
p r e s e n t . A c h e s t x- r a y s t u d y i s t h e t y p i c a l s t a r t i n g p o i n t . O n t h e u p r i g h t
a n t e r o p o s t e r i o r v i e w, a s ma l l e f f u s i o n ma y s h o w u p a s b l u n t i n g o f t h e
c o s t o p h r e n i c a n g l e . L a r g e r e f f u s i o n s s h o w a me n i s c u s s i g n a t t h e a i r f l u i d
b o r d e r. L a t e r a l d e c u b i t u s v i e w s h e l p e s t i ma t e t h e s i ze o f t h e e f f u s i o n .
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2. U l t r a s o u n d a n d o t h e r m o d a l U
i t ni ef osr t u n a t e l y, c h e s t x- r a y s c a n f a i l t o s h o w
s ma l l e f f u s i o n s , e v e n w i t h d e c u b i t u s v i e w s . U l t r a s o u n d c a n d e t e c t a s l i t t l e a s 5
t o 5 0 mL o f f l u i d . I t i s a l s o h e l p f u l i n l o c a t i n g p o c k e t s o f f l u i d a n d g u i d i n g
t h o r a c e n t e s i s f o r s ma l l e f f u s i o n s . C o mp u t e d t o mo g r a p h y, w h i c h i s v e r y s e n s i t i v e
c a n d i f f e r e n t i a t e p l e u r a l f l u i d f r o m p l e u r a l t h i c k e n i n g a n d f o c a l ma s s e s .
3. T h o r a c e n t e s iTsh o r a c e n t e s i s a l l o w s e v a l u a t i o n o f a n y u n d i a g n o s e d p l e u r a l
effusion. Note that not all effusions require diagnostic thoracentesis. If the
c a u s e i s a p p a r e n t f r o m t h e c l i n i c a l p r e s e n t a t i o n ( e . g . , C H F ) , o b s e r v a t i o n ma y b
a p p r o p r i a t e3)(. I n g e n e r a l , p a r a p n e u mo n i c e f f u s i o n s r e q u i r e t h o r a c e n t e s i s t o
c o n f i r m d i a g n o s i s a n d a s s e s s t h e n e e d f o r c h e s t t u b e p l a c e me n t .
P. 1 5 9
a. R e l a t i v e c o n t r a i n d i c a t i o n s i n c l u d e b l e e d i n g d i a t h e s i s , s y s t e mi c
a n t i c o a g u l a t i o n , s ma l l v o l u me o f p l e u r a l f l u i d , me c h a n i c a l v e n t i l a t i o n ,
inability of the patient to cooperate, and cutaneous disease at the needle
entry site.
b. T r a n s u d a t e o r e xu d a t e ? O n t h e b a s i s o f a r e v i s i o n o f t h e mo d i f i e d L i g h t ' s
c r i t e r i a , p l e u r a l f l u i d i s a n e xu d a t e i f i t me e t s o n e o r mo r e o f t h e f o l l o w i n g
p a r a me t e r s4)(:
i. P l e u r a l f l u i d s e r u m l a c t a t e d e h y d r o g e n a s e ( L D H ) l e v e l > 0 . 4 5 t h e u p p e r
l i mi t o f n o r ma l L D H
i i. P l e u r a l .S e r u m L D H > 0 . 6
i i i. P l e u r a l .S e r u m p r o t e i n > 0 . 5
c. O t h e r me a s u r e s u s e d t o t e s t f o r a n e xu d a t e i n c l u d e p l e u r a l f l u i d c h o l e s t e r o l
f l u i d : s e r u m a l b u mi n g r a d i e n t a n d f l u i d : s e r u m b i l i r u b i n r a t i o . C e l l c o u n t , p H ,
g l u c o s e , G r a m' s s t a i n , a n d c u l t u r e h e l p a s s e s s f o r i n f e c t i o n .
I V. Diagnosis
I n d e v e l o p i n g a d i a g n o s t i c a s s e s s me n t f o r t h e p a t i e n t w i t h p l e u r a l e f f u s i o n , i t i s
i mp o r t a n t t o c o n s i d e r t h a t p l e u r a l f l u i d a n a l y s i s d o e s n o t e s t a b l i s h a s p e c i f i c
d i a g n o s i s , b u t s u p p o r t s a c l i n i c a l i mp r e s s i o n . O r d e r i n g a n d i n t e r p r e t i n g t e s t s mu s t b
g u i d e d b y p r e t e s t c l i n i c a l i mp r e s5)
s i.o Innsi t (i a l l y, i t ma y b e a p p r o p r i a t e t o o r d e r
o n l y p l e u r a l f l u i d L D H a n d p r o t e i n l e v e l s t o d e t e r mi n e t h e p r e s e n c e o r a b s e n c e o f a
e xu d a t e . A d d i t i o n a l f l u i d c a n b e r e s e r v e d f o r f u r t h e r t e s t i n g i f a n e xu d a t e i s f o u n d .
A.
I f t h e p l e u r a l f l u i d a n a l y s i s s h o w s a t r a n s u d a t e , t h e mo s t l i k e l y d i a g n o s i s i s C H F.
A d d i t i o n a l p o s s i b i l i t i e s i n c l u d e c i r r h o s i s w i t h a s c i t e s , n e p h r o t i c s y n d r o me ,
h y p o a l b u mi n e mi a , a n d a c u t e a t e l e c t a s i s . F u r t h e r d i a g n o s t i c e v a l u a t i o n o f t h e p l e u r a
f l u i d i s n o t n e c e s s a r y.
B.
I f t h e p l e u r a l f l u i d i s e xu d a t i v e , t h e mo s t l i k e l y d i a g n o s t i c p o s s i b i l i t i e s a r e
ma l i g n a n c y, i n f e c t i o n , o r t u b e r c u l o s i s , b u t t h e d i f f e r e n t i a l d i a g n o s i s i s q u i t e b r o a d .
o n e s t u d y, ma l i g n a n c y a c c o u n t e d f o r 2 5 % o f a l l p l e u r a l e f f u s i o n s s e e n i n t h e g e n e r a
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h o s p i t a l s e t t i n g . C y t o l o g y i s h e l p f u l i n l o o k i n g f o r ma l i g n a n c y. I n 5 4 % t o 6 3 % o f
p a t i e n t s w i t h ma l i g n a n t e f f u s i o n s , p l e u r a l f l u i d c y t o l o g y5)i .s G
p ol usci toi sv e l(e v e l ,
c e l l c o u n t , a n d p H h e l p g u i d e ma n a g e me n t i n t h e s e t t i n g o f p a r a p n e u mo n i c e f f u s i o n
a n d a i d i n d e t e r mi n i n g t h e n e e d f o r c h e s t t u b e p l a c e me n t . T u b e r c u l o u s e f f u s i o n s ma
r e q u i r e p l e u r a l b i o p s y t o c o n f i r m t h e d i a g n o s i s . A my l a s e c a n b e e l e v a t e d i n
p a n c r e a t i t i s , p a n c r e a t i c p s e u d o c y s t , ma l i g n a n c y, a n d e s o p h a g e a l r u p t u r e .
T r i g l y c e r i d e s a r e e l e v a t e d i n t h e s e t t i n g o f c h y l o t h o r a x.
C.
S t u d i e s o n p l e u r a l f l u i d y i e l d a d e f i n i t i v e o r p r e s u mp t i v e d i a g n o s i s i n 7 4 % o f c a s e s
(5) . T h o s e c a s e s t h a t a r e u n d i a g n o s e d ma y r e q u i r e r e p e a t t h o r a c e n t e s i s , P l e u r a l
b i o p s y, b r o n c h o s c o p y, o r t h o r a c o s c o p y t o a s c e r t a i n t h e c a u s e .
References
1 . S t a g n e r S W, C a mp b e l l G D . P l e u r a l e f f u s i o n : w h a t c a n y o u l e a r n f r o m t h e
r e s u l t s o f t h e a t aPp os
? t gr ad M ed
1992;91:439454.
2. Jay S J. Diagnostic procedures for pleural C
d il si ne aCs hes
e . t M ed
1985;6:33
48.
3 . B u r g h e r LW, J o n e s F L , P a t t e r s o n J R , e t a l . G u i d e l i n e s f o r t h o r a c e n t e s i s a n d
n e e d l e b i o p s y o f t h e p l eAum
r a .R ev R es pi r D1i s9 8 9 ; 1 4 0 : 2 5 7 2 5 8 .
4 . H e f f n e r J E , B r o w n L K , B a r b i e r C . D i a g n o s t i c v a l u e o f t e s t s t h a t d i s c r i mi n a t e
b e t w e e n e xu d a t i v e a n d t r a n s u d a t i v e p l e u r a l eCf fhes
u s i ot 1n9s9. 7 ; 111 : 9 7 0 9 8 0 .
5. B artter T, S antarelli R , Akers S , et al. T he evaluation of pleural effusion.
C hes t 1 9 9 4 ; 1 0 6 : 1 2 0 9 1 2 1 4 .
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8.5 - Pleuritic Pain
> T a b l e o f C o n t e n t s > 8 - R e s p i r a t o r y P r o b l e ms > 8 . 5 - P l e u r i t i c P a i n
8.5
Pleuritic Pain
Hal S. Shim azu
I . Background
P l e u r i t i c p a i n i s t h e c h a r a c t e r i s t i c p a i n o f i n f l a me d p l e u r a ( i . e . , p l e u r i s y a t e r m o f t
u s e d s y n o n y mo u s l y w i t h p l e u r i t i c p a i n ) . P l e u r i t i c p a i n a r i s e s f r o m p a r i e t a l p l e u r a a n
is typically sharp, stabbing, unilateral, and aggravated by deep inspiration and
coughing. T he visceral pleura is insensitive.
A. Etiology
I t i s i mp o r t a n t t o d i s t i n g u i s h t r u e p l e u r i t i c Tpa bi nl e( s8e. 5e). 1f r o m c o n d i t i o n s t h a t

mi mi c t h i s p a i n ( sTeaeb l e 8 . 5 ). .2 I n a c u t e p l e u r i t i c p a i n , u r g e n t e xc l u s i o n o f
p u l mo n a r y e mb o l i s m ( P E ) i s p a r a mo u n t ; mo r t a l i t y f r o m P E i s < 1 0 % i n t r e a t e d
p a t i e n t s a n d 3 0 % i n u n t r e a t e d p a1)
t i e. nBt se c(a u s e o f t h e l o w s p e c i f i c i t y o f t h e
p r e s e n t i n g s i g n s a n d s y mp t o ms o f P E , i n t h e a b s e n c e o f a n o b v i o u s c a u s e f o r a c u t e
p l e u r i t i c p a i n , s u s p i c i o n f o r P E mu s t r e ma i n h i g h ; f u r t h e r e v a l u a t i o n i s r e q u i r e d ,
typically with a ventilationperfusion ([V with dot above]/[Q with dot above]) lung
s c a n 2,3)
( .
B. Epidemiology
P l e u r i t i c p a i n a s a c l i n i c a l ma n i f e s t a t i o n w i t h a mu l t i t u d e o f e t i o l o g i e s i s n o t a s s u c
a r e p o r t a b l e c l i n i c a l e n t i t y. T h e r e f o r e , s p e c i f i c d a t a o n t h e p r e v a l e n c e a n d i n c i d e n c
of pleuritic pain are lacking.
I I I . Evaluation
A. History
1. I n i t i a l h i s t o rHyi s t o r y b e g i n s w i t h a s s e s s i n g t h e c h a r a c t e r i s t i c s o f t h e p l e u r i t i c
p a i n t h e a c u i t y, i t s l o c a t i o n , a n d e xa c e r b a t i n g f e a t u r e s . A c u t e o n s e t
P. 1 6 1
s u g g e s t s s u d d e n d e v e l o p me n t a s v i r a l o r i d i o p a t h i c p l e u r i s y, P E , p n e u mo n i a , o r
p n e u mo t h o r a x. I n s i d i o u s o n s e t s u g g e s t s a s l o w e r i n f l a mma t o r y o r i r r i t a t i v e
process, usually resulting in a pleural effusion with the pain generally
d i mi n i s h i n g a s f l u i d a c c u mu lCa ht easp t(e r 8 ). .4 P l e u r i t i c c h e s t p a i n l o c a l i ze s
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above the underlying pleural pathology through intercostal innervation. Shoulde

p a i n t h r o u g h p h r e n i c i n n e r v a t i o n c a n i n d i c a t e i p s i l a t e r a l d i a p h r a g ma t i c
i n v o l v e me n t , u s u a l l y b y a b d o mi n a l p a t h o l oTgayb l(es e8e. 5 ). 2. S u b s t e r n a l p a i n
t h a t d e c r e a s e s b y l e a n i n g f o r w a r d , s u g g e s t s p eC
r i hc a pr dt ei tri s7 ).( 1.
P r o v o c a t i o n o f p a i n b y s h o u l d e r mo v e me n t i n d i c a t e s a mu s c u l o s k e l e t a l c a u s e .
TAB L E 8.5.1 Causes of Pleuritic Pain

P l e u r i t i s / p l e u r i sIyn :f e c t i o u s ( v i r a l , e s p e c i a l l y c o xs a c k i e v i r u s B ;
p r i ma r y o r r e a c t i v a t e d t u b e r c u l o s i s , f u n g a l ) , a u t o i mmu n e ( e . g . , s y s t e mi c
l u p u s , r h e u ma t o i d a r t h r i t i s , D r e s s l e r ' s s y n d r o me p l e u r o p e r i c a r d i t i s ) ,
u r e mi c , r a d i a t i o n - i n d u c e d , d r u g r e a c t i o n , a n d i d i o p a t h i c
P n e u mo n i a
S p o n t a n e o u s p n e u mo t h o r a x
T r a u ma ( h e mo t h o r a x, p n e u mo t h o r a x)
Neoplasia
Asbestos
TAB L E 8.5.2 Conditions that Mim ic Pleuritic Pain

C h e s t w a l lC: o s t o c h o n d r i t i s , r i b f r a c t u r e , mu s c l e s t r a i n / s p a s m, h e r p e s
zo s t e r
A b d o m i n a lP
: ancreatitis, abscess (hepatic, splenic, subphrenic),
splenic infarction
C a r d i a c :P e r i c a r d i t i s
2. R e v i e w o f s y m p t o m
A sf o c u s e d r e v i e w o f s y s t e ms mi g h t s u g g e s t a r e s p i r a t o r y
i n f e c t i o n , P E , o r ma l i g n a n c y a s t h e e t i o l o g y f o r t h e p l e u r i t i c p a i n . N o n p r o d u c t i v
c o u g h i s n o n s p e c i f i c , a n d p r o d u c t i v e c o u g h s u g g e s t s i n f e c t i o n . H e mo p t y s i s
s u g g e s t s ma l i g n a n c y, t u b e r c u l o s i s , o r p u l mo n a r y e mb o l i s m. F e v e r s u g g e s t s
i n f e c t i o n b u t c a n o c c u r w i t h P E . R e c e n t s u r g e r y o r l o w e r e xt r e mi t y t r a u ma o r
s w e l l i n g i n c r e a s e s t h e r i s k o f P E . U n e xp l a i n e d w e i g h t l o s s s u g g e s t s ma l i g n a n c y
or tuberculosis (T B).
3. P a s t m e d i c a l h i s t oTr hyi s c a n p r o v i d e c l u e s t o t h e c a u s e i n c l u d i n g
ma l i g n a n c y, r e c e n t my o c a r d i a l i n f a r c t i o n , u r e mi a , l u p u s , a n d r h e u ma t o i d a r t h r i t i s
4. O t h e r i n q u i r i eAsd d i t i o n a l h i s t o r y c o u l d b e h e l p f u l w i t h i n q u i r i e s a b o u t o r a l
c o n t r a c e p t i v e s ( P E r i s k ) , T B , o r a s b e s t o s e xp o s u r e .
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1. A f o c u s e d e xa mi n a t i o n s h o u l d i n c l u d e v i t a l s i g n s w i t h a t t e n t i o n t o t e mp e r a t u r e ,
r e s p i r a t o r y r a t e , a n d e xa mi n a t i o n o f t h e c h e s t . T e n d e r n e s s t o p a l p a t i o n i n d i c a t e
a mu s c u l o s k e l e t a l c a u s e . D u l l n e s s t o p e r c u s s i o n s u g g e s t s p l e u r a l e f f u s i o n o r
p a r e n c h y ma l p a t h o l o g y a n d h y p e r r e s o n a n t p e r c u s s i o n i n d i c a t e s p n e u mo t h o r a x.
On auscultation, a pleural friction rub is the only sign of pleurisy; crackles
s u g g e s t p n e u mo n i a ; a n d d e c r e a s e d b r e a t h s o u n d s i n d i c a t e p n e u mo t h o r a x o r
e f f u s i o n . T h e r e s u l t o f t h e e xa mi n a t i o n i s f r e q u e n t l y n o r ma l .
2. A d d i t i o n a l p h y s i c a l f i n d i n g s s u c h a s a b d o mi n a l t e n d e r n e s s c a n s u g g e s t a
s u b d i a p h r a g ma t i c p r o c eTsasb l(e 8 . 5 ). .2 L o w e r e xt r e mi t y e d e ma , t e n d e r n e s s , o r
H o ma n s ' s i g n c a n i mp l y d e e p v e i n t h r o mb o s i s ( D V T ) a n d P E . L y mp h a d e n o p a t h y
c a n r e p r e s e n t l y mp h o ma o r me t a s t a t i c d i s e a s e .
C. Testing
1. R o u t i n e l a b o r a t o r y s t u d i e s ( e . g . , c o mp l e t e b l o o d c o u n t a n d me t a b o l i c p a n e l s )
a r e o f l i mi t e d u s e f u l n e s s ; l e u k o c y t o s i s i n p n e u mo n i a , u r e mi a , o r h e p a t i c
a b n o r ma l i t i e s ma y s u g g e s t t h e c a u s e .
2. I ma g i n g s t u d i e s
a. A c h e s t x- r a y ( C X R ) i s e s s e n t i a l , p o t e n t i a l l y r e v e a l i n g p n e u mo n i a ,
n e o p l a s m, p n e u mo t h o r a x, o r p l e u r a l e f f u s i o n ; t h e d e c u b i t u s v i e w i s
s e n s i t i v e a t 1 0 0 mL e f f u s i o n . N o n s p e c i f i c f i n d i n g s o f a t e l e c t a s i s , p u l mo n a r y
p a r e n c h y ma l a b n o r ma l i t i e s , o r b o t h a r e s e e n i n 6 8 % o f P E ; p l e u r a l e f f u s i o n
i s f o u n d i n 4 8 % o f P3)E. T
( h e C X R i s g e n e r a l l y n o r ma l . N o t a b l y, P E a n d
v i r a l p l e u r i s y f r e q u e n t l y h a v e a n o r ma l C X R .
b. U l t r a s o u n d h a s d i a g n o s t i c a n d t h e r a p e u t i c a d j u n c t i v e r o l e s w i t h p l e u r a l
effusions.
c. C o mp u t e d t o mo g r a p h y ( C T ) s c a n h a s a r o l e w i t h b o t h e f f u s i o n s a n d
p a r e n c h y ma l a b n o r ma l i t i e s .
3. D i a g n o s t i c t h o r a c e n t e s i s i s i n d i c a t e d f o r p l e u r a l e f f u s i o n i f t h e c a u s e o f t h e
e f f u s i o n a n d p a i n i s n o t a p p a r e n t ( d i a g n o s t i c a s s e s s me n t o f p l e u r a l e f f u s i o n i s
d i s c u s s e d iCn h a p t e r 8 ). .4 E f f u s i o n a s s o c i a t e d w i t h p l e u r i t i c p a i n i s n e a r l y
a l w a y s a n e xu d a t e w i t h a n o t a b l e e xc e p t i o n o f P E , w h i c h c a n b e a t r a n s u d a t e
(4) .
4. A d d i t i o n a l s t u d i e s a r e i n d i c a t e d f o r e v a l u a t i o n o f a p o t e n t i a l P E . T h e s t a n d a r d
o r r a p i d e n zy me - l i n k e d i mmu n o s o r b e n t a s s a y o f D - d i me r, t h e d e g r a d a t i o n
product of cross-linked fibrin, is a highly sensitive but nonspecific test for
v e n o u s t h r o mb o e mb o l i s m t h a t , w h e n c o u p l e d w i t h l o w c l i n i c a l p r o b a b i l i t y o f P E ,
can safely rule out P
CE
h a(p t e r 1 7). (5)
5 . [V with dot above]/[Q with dot above]
lung scan is typically obtained for any significant suspicion of P E . If the [V with
d o t a b o v e ] / [ Q w i t h d o t a b o v e ] s c a n h a s l o w o r i n t e r me d i a t e p r o b a b i l i t y
P. 1 6 2
282 / 652
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f o r P E , t h e n e v a l u a t i n g f o r p o s s i b l e D V T w i t h l e g i mp e d a n c e p l e t h y s mo g r a p h y
o r u l t r a s o u n d i s u s e f u l . I f t h e D V T s t u d y i s n e g a t i v e , t h e n a p u l mo n a r y
a n g i o g r a m c a n b e o b t a i n e d i f t h e r e i s i n t e r me d i a t e o r h i g h c l i n i c a l s u s p i c i o n f o r
P E (5) . O p t i o n a l l y, s e r i a l l e g v e n o u s s t u d i e s c a n b e o b t a i n e d i f t h e r e i s o n l y
i n t e r me d i a t e c l i n i c a l s u s p i3)
c i.o nT h( e h e l i c a l C T a n g i o g r a m h a s a n e me r g i n g
role in suspected P E evaluation, especially in patients with conditions that
would cause a nondiagnostic [V with dot above]/[Q with dot above] scan. T he
s e n s i t i v i t y a n d s p e c i f i c i t y o f t h e C T a n g i o g r a m a r e b o t h 9 0 % i n ma i n a n d l o b a r
p u l mo n a r y e mb o l i ; h o w e v e r, s e n s i t i v i t y i s 7 1 % t o 8 4 % i n s u b s e g me n t a l
p u l mo n a r y a r t e r y e mb o l i , w h i c h c a n r e p r e s e n t f r o m 6 % t o 3 0 % o f p a t i e n t s w i t h
p u l mo n a r y e mb o5)
l i .(
D. Genetics
E xc e p t f o r a h e r e d i t a r y h y p e r c o a g u l a b l e s t a t e i n c r e a s i n g t h e r i s k o f P E , g e n e t i c s h a
n o r e l e v a n c e t o t h e c l i n i c a l ma n i f e s t a t i o n o f p l e u r i t i c p a i n .
I V. Diagnosis
A f t e r r u l i n g o u t mi mi c s o f p l e u r i t i Tc apbal ei n 8(. 5 ). ,2 t h e r e a r e ma n y p o t e n t i a l
c a u s e s o f p l e u r i t i c p a i n t o c o nTsai dbel er (8 . 5 ). .1 C o n s i d e r i n g t h e p o s s i b i l i t y o f P E
i s o f p a r a mo u n t i mp o r t a n c e .
T h e C X R s t u d y ma y r e v e a l a n o b v i o u s c a u s e ( e . g . , n e o p l a s m, p n e u mo n i a , o r
p n e u mo t h o r a x) o r ma y n o n s p e c i f i c a l l y r e v e a l a p l e u r a l e f f u s i o n . C o mmo n l y, t h e C X R
ma y b e n o r ma l . C a u s e s o f p l e u r i t i c p a i n i n a p a t i e n t w i t h a n o r ma l C X R r e s u l t a r e
P E , v i r a l o r i d i o p a t h i c p l e u r i s y, a n d s e r o s i t i s , e s p e c i a l l y f r o m s y s t e mi c l u p u s . Vi r a l
p l e u r i s y, u s u a l l y c o xs a c k i e v i r u s B , i s c h a r a c t e r i ze d b y u n i l a t e r a l a c u t e p l e u r i t i c p a i
v a r i a b l e l o w - g r a d e f e v e r, a n d n o n p r o d u c t i v e c o u g h w i t h t y p i c a l l y a n o r ma l C X R ,
which is an indistinguishable presentation from P E 20% presenting with acute
p l e u r i t i c p a i n t o t h e e me r g e n c y d e p a r t me n t h a v e P E a n d a p p r o xi ma t e l y 5 0 % h a v e
v i r a l o r i d i o p a t h i c p l e u1,2)
r i s y. (H e n c e , a c u t e p l e u r i t i c p a i n w i t h o u t a n o b v i o u s
c a u s e o n h i s t o r y a n d p h y s i c a l e xa mi n a t i o n a n d C X R f i l m r e q u i r e s e xc l u s i o n o f P E . A
p o s s i b l e e xc e p t i o n t o t h i s t e n e t i s t h e y o u n g a d u l t ( a g e < 4 0 y e a r s ) w h o i s h i g h l y
u n l i k e l y t o h a v e a P E i f a l l t h r e e o f t h e f o l l o w i n g c l i n i c a l abs
f e aent
t u:r e( sa )ar ies k
f a c t o r s f o r o r p a s t h i s t o r y o f v e n o u s t h r o mb o e mb o l i c d i s e a s e , ( b ) p h y s i c a l f i n d i n g s
p h l e b i t i s a n d ( c ) p l e u r a l e f f u s i o n o n2) .C IXnRt h( e p r e s e n c e o f a n e f f u s i o n w i t h o u t
a clear cause of the pleuritic pain and P E either ruled out or clinically highly unlike
a diagnostic thoracentesis is indicated. T he effusion associated with pleuritic pain
n e a r l y a l w a y s a n e xu d a t e . T h e mo s t c o mmo n c a u s e s o f e xu d a t e s i n d e s c e n d i n g
o r d e r o f f r e q u e n c y a r e ma l i g n a n c y ( mo s t c o mmo n l y l u n g c a n c e r, b r e a s t c a n c e r,
l y mp h o ma ) , P n e u mo n i a , P E , a n d v i r a l i n f e c t i o n s , w h i c h , t o g e t h e r c o n s t i t u t e 9 5 % o f
t h e s e e f f u s i o n4)
s .(
References
283 / 652
tmdmss
1 . P a l e v s k y H I , K e l l e y M A , F i s h ma n A P. P u l mo n a r y t h r o mb o e mb o l i c d i s e a s e . I n :
F i s h ma n A P, eFd i. s hman' s pul monar y di s eas es and di .s or
N eder
w sYo r k , N Y :
McGraw-Hill, 1997:12971329.
2 . H u l l R D , R a s k o b G E . P u l mo n a r y e mb o l i s m i n o u t p a t i e n t s w i t h p l e u r i t i c c h e s t
p a i n .A r c h I nt er n M ed
1988;148:838844.
3 . S t e i n P D . A c u t e p u l mo n a r y e mb oDl i s m.
M on1 9 9 4 ; 4 0 ( 9 ) : 4 6 7 5 1 5 .
4 . L i g h t R W.P l eur al di s eas, es
3 r d e d . B a l t i mo r e , M D : W i l l i a m & W i l k i n s ,
1995:7582, 187191.
5 . F e d u l l o P F, T a p s o n V F. T h e e v a l u a t i o n o f s u s p e c t e d p u l mo n a rNy e mb o l i s m.
E ngl J M ed2 0 0 3 ; 3 4 9 : 1 2 4 7 1 2 5 6 .
284 / 652
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8.6 - Pneumothorax
> T a b l e o f C o n t e n t s > 8 - R e s p i r a t o r y P r o b l e ms > 8 . 6 - P n e u mo t h o r a x
8.6
Pneumothorax
Charle s Ve ga
I . Background
P n e u mo t h o r a x o c c u r s w h e n a i r e n t e r s t h e p l e u r a l s p a c e , t h e a r e a b e t w e e n t h e
v i s c e r a l a n d p a r i e t a l p l e u r a . I t i s a c o mmo n p r o b l e m w i t h a v a r i e t y o f d i f f e r e n t
causes. In all cases, air enters the space because of a disruption or break in either
the visceral or parietal pleura.
P n e u mo t h o r a x c a n b e c l a s s i f i e d i n t o t w o ma j o r c a t e g o r i e s s p o n t a n e o u s a n d
t r a u ma t i c .
A. Spontaneous pneumothorax
1. P r i ma r y s p o n t a n e o u s p n e u mo t h o r a x o c c u r s i n p r e v i o u s l y h e a l t h y i n d i v i d u a l s . I t
c a n b e f o u n d mo r e f r e q u e n t l y i n t a l l , s l e n d e r i n1)
d i.v iI dt ui sa l mo
s ( s t c o mmo n i n
i n d i v i d u a l s i n t h e i r e a r l y t w e n t i e s a n d i s u n c o mmo n i n t h o s e o l d e r t h a n 4 0 y e a r s
o f a g e . P r i ma r y s p o n t a n e o u s p n e u mo t h o r a x i s o f t e n c a u s e d b y a r u p t u r e o f
a p i c a l b l e b s o r b u l l a e . C i g a r e t t e s mo k i n g i n c r e a s e s t h e p o s s i b i l i t y o f p r i ma r y
s p o n t a n e o u s p n e u mo t h o1)
r a.x T( h e e s t i ma t e d a n n u a l i n c i d e n c e o f p r i ma r y
s p o n t a n e o u s p n e u mo t h o r a x i s 7 . 4 t o 1 8 c a s e s p e r 1 0 0 , 0 0 0 me n a n d 1 . 2 t o 6
c a s e s p e r 1 0 0 , 0 0 0 w o me
2) .n (
2. S e c o n d a r y s p o n t a n e o u s p n e u mo t h o r a x o c c u r s a s a c o mp l i c a t i o n i n i n d i v i d u a l s
w i t h u n d e r l y i n g p u l mo n a r y d i s e a s e . I t i s mo s t c o mmo n i n i n d i v i d u a l s w i t h c h r o n i c
o b s t r u c t i v e p u l mo n a r y d i s e a s e ( C O P D ) . S e c o n d a r y s p o n t a n e o u s p n e u mo t h o r a x
is also seen in individuals with interstitial lung disease, with infections,
p a r t i c u l a r lPy neumoc y s t i s c ar i pninie u mo n i a a n d t u b e r c u l o s i s , a n d w i t h
n e o p l a s ms , e i t h e r p r i ma r y l u n g o r me t a s t a t i c t u mo r s . T h e i n c i d e n c e i s s i mi l a r t o
t h a t o f p r i ma r y s p o n t a n e o u s p n e u mo t h o r a x, w i t h a n e s t i ma t e d 1 0 , 0 0 0 c a s e s p e r
y e a r i n t h e U n i t e d S t a3)
t e.s (
B. Traumatic pneumothorax
1. I a t r o g e n i c p n e u mo t h o r a x o c c u r s a s a c o mp l i c a t i o n o f me d i c a l p r o c e d u r e s s u c h
a s t r a n s t h o r a c i c n e e d l e b i o p s y, c e n t r a l v e n o u s c a t h e t e r p l a c e me n t ,
285 / 652
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8.6 - Pneumothorax
t h o r a c e n t e s i s , a n d b r o n c h o s c o p y, o r a s a c o mp l i c a t i o n o f me c h a n i c a l v e n t i l a t i o n
2. P e n e t r a t i n g a n d b l u n t t r a uPme na e. t r a t i n g t r a u ma , s u c h a s a s t a b w o u n d , a s
t h e c a u s a t i v e f a c t o r o f p n e u mo t h o r a x i s o b v i o u s : t h e w o u n d a l l o w s a i r t o e n t e r
t h r o u g h t h e c h e s t w a l l . P n e u mo t h o r a x c a n a l s o b e a r e s u l t o f b l u n t t r a u ma , a s
s o me t i me s o c c u r s w h e n a r i b f r a c t u r e p i e r c e s t h e v i s c e r a l p l e u r a . M o r e o f t e n ,
h o w e v e r, d e c e l e r a t i n g f o r c e s o f b l u n t c h e s t t r a u ma c a n l e a d t o c h e s t
c o mp r e s s i o n t h a t c a n d i r e c t l y c a u s e p n e u mo t h o r a x.
I I I . Evaluation
A. History
1. S p o n t a n e o u s p n e u m o t h oC
r ahxe s t p a i n , mo s t o f t e n p l e u r i t i c a n d l o c a l i ze d t o
t h e s i d e o f t h e p n e u mo t h o r a x, a n d d y s p n e a a r e t h e ma j o r s y mp t o ms . O n s e t i s
g e n e r a l l y s u d d e n . I n p r i ma r y s p o n t a n e o u s p n e u mo t h o r a x, t h e s y mp t o ms a r e
o f t e n mi l d , o n s e t i s u s u a l l y a t r e s t , a n d p a t i e n t s o f t e n d o n o t i mme d i a t e l y s e e k
me d i c a l a t t e n t i o4)n . ( S y mp t o ms a r e g e n e r a l l y mo r e s e v e r e i n p a t i e n t s w i t h
u n d e r l y i n g l u n g d i s e a s e ( s e c o n d a r y s p o n t a n e o u s p n e u mo t h o r a x) w h o h a v e
i mp a i r e d p u l mo n a r y r e s e5)
r v. e (
2. T r a u m a t i c p n e u m o t h o rTahxe s y mp t o ms a r e t h e s a me a s i n s p o n t a n e o u s
p n e u mo t h o r a x; a l t h o u g h , i n i a t r o g e n i c p n e u mo t h o r a x, t h e y ma y n o t o c c u r f o r 2 4
h o u r s o r mo r e a f t e r t h e d i a g n o s t i c o r t h e r a p e u t i c p5)r o. cTehdeu rcel i n( i c a l
deterioration of patients on ventilators should raise the suspicion of
p n e u mo t h o r a x. T h i s i s mo r e l i k e l y i n p a t i e n t s w i t h a c u t e r e s p i r a t o r y d i s t r e s s
s y n d r o me , n e c r o t i zi n g o r a s p i r a t i o n p n e u mo n i a , C O P D , o r i n t e r s t i t i a l l u n g
d i s e a s e 5)( .
P. 1 6 4
Vi t a l s i g n s c a n b e n o r ma l , b u t t a c h y c a r d i a i s t h e mo s t c o mmo n s i g n o f s p o n t a n e o u s
p n e u mo t h o r a 2)
x (. S i g n i f i c a n t t a c h y p n e a c a n o c c u r i n p a t i e n t s w i t h l a r g e
p n e u mo t h o r a c e s , o r i n p a t i e n t s w i t h u n d e r l y i n g p u l mo n a r y d i s e a s e . H y p o t e n s i o n a n d
s e v e r e t a c h y c a r d i a c a n b e p r e s e n t i n p a t i e n t s w i t h t e n s i o n p n e u mo t h o r a x o r
s e c o n d a r y s p o n t a n e o u s p n e u mo t h o r a x. O n c h e s t a n d l u n g e xa mi n a t i o n ma y b e f o u n d
u n i l a t e r a l e n l a r g e me n t o f t h e c h e s t c a v i t y, l o s s o f t a c t i l e f r e mi t u s , h y p e r r e s o n a n c e
percussion, and decreased, or absent, breath sounds on the affected side. Trachea
d e v i a t i o n ma y b e s e e n , e s p e c i a l l y w i t h t e n s i o n p n e u mo t h o r a x.
C. Testing
1. A r t e r i a l b l o o d g a s t y p i c a l l y s h o w s h y p o xi a a n d , o c c a s i o n a l l y, h y p o c a r b i a
secondary to hyperventilation.
2. E l e c t r o c a r d i o g r a p h i c c h a n g e s ma y b e s e e n , e s p e c i a l l y w i t h l e f t - s i d e d
p n e u mo t h o r a x, i n c l u d i n g a xi s d e v i a t i o n , n o n s p e c i f i c S T - a n d T - w a v e c h a n g e s ,
S T d e p r e s s i o n , a n d T - w a v e i n v e5)
r s.i o n (
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8.6 - Pneumothorax
3. C h e s t x- r a y i s g e n e r a l l y p a r a mo u n t i n t h e d i a g n o s i s , w i t h v i s u a l i za t i o n o f t h e
v i s c e r a l p l e u r a l l i n e a n d t h e a b s e n c e o f l u n g ma r k i n g s d i s t a l t o t h i s l i n e . T h i s i s
b e s t s e e n o n a n u p r i g h t i n s p i r a t o r y f i l m, a n d t h e v a l u e o f a d d i n g a d d i t i o n a l
e xp i r a t o r y f i l ms h a s b e e n q u e s t 3)
i o .n eLda t(e r a l d e c u b i t u s f i l ms ma y b e h e l p f u l
i n c r i t i c a l l y i l l p a t i e n t s w h o c a n n o t s i t 6)u.p r i g h t (
4. C o mp u t e d t o mo g r a p h y c a n a l s o b e u s e f u l w h e n t h e c h e s t x- r a y i s n o t d i a g n o s t i c
I V. Diagnosis
1. P n e u mo t h o r a x c a n b e d i a g n o s e d b y t h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n i n
slender patients who have had acute onset of chest pain and dyspnea, and
c o n f i r me d w i t h a c h e s t r a d i o g r a p h v i s u a l i zi n g t h e v i s c e r a l p l e u r a l l i n e . P a t i e n t s
w h o h a v e h a d p r i ma r y s p o n t a n e o u s p n e u mo t h o r a x a r e a t r i s k o f r e c u r r e n c e .
2. S e c o n d a r y s p o n t a n e o u s p n e u mo t h o r a x c a n b e a b i t mo r e d i f f i c u l t t o d i a g n o s e .
A l t h o u g h t h e s y mp t o ms a r e mo r e p r o mi n e n t , t h e s i g n s o n p h y s i c a l e xa mi n a t i o n
are often subtle, especially in patients with C O P D who tend to have decreased
b r e a t h s o u n d s a n d d e c r e a s e d t a c t i l e f r e mi t u s , b e c a u s e o f t h e i r u n d e r l y i n g
d i s e a s e . R a d i o g r a p h i c e v a l u a t i o n c a n a l s o b e mo r e d i f f i c u l t . B e c a u s e o f t h e l a c
o f i n t e r s t i t i a l ma r k i n g s i n t h e e mp h y s e ma t o u s l u n g , l i t t l e d i f f e r e n c e i s s e e n i n t h
a p p e a r a n c e p r o xi ma l a n d d i s t a l t o t h e v i s c e r a l l i n e . A l s o , a n e mp h y s e ma t o u s
b l e b mi g h t b e mi s t a k e n f o r a v i s c e r a l l i n e .
1. I t i s c o mmo n t o o b t a i n a c h e s t x- r a y a f t e r p r o c e d u r e s t h a t mi g h t l e a d t o
p n e u mo t h o r a x. P l e u r i t i c c h e s t p a i n a n d d y s p n e a , a f t e r a s s o c i a t e d d i a g n o s t i c o r
t h e r a p e u t i c p r o c e d u r e s , s h o u l d a l e r t p r a c t i t i o n e r s , e v e n i f t h e s e s y mp t o ms o c c u
ma n y h o u r s a f t e r t h e p r o c e d u r e . A l l p a t i e n t s w i t h s i g n i f i c a n t b l u n t t r a u ma t o t h e
c h e s t s h o u l d b e e v a l u a t e d f o r p n e u mo t h o r a x, i n c l u d i n g a l l p a t i e n t s w i t h r i b o r
scapula fractures.
2. T e n s i o n p n e u mo t h o r a x o c c u r s w h e n a o n e - w a y v a l v e a l l o w s a i r i n t o , b u t n o t o u t
o f t h e p l e u r a l s p a c e . A s p r e s s u r e i n t h e p l e u r a l s p a c e e xc e e d s t h e a t mo s p h e r i c
p r e s s u r e , t h e i p s i l a t e r a l l u n g , me d i a s t i n u m, a n d c o n t r a l a t e r a l l u n g a r e
c o mp r e s s e d . T h i s i s a me d i c a l e me r g e n c y. T h e d i a g n o s i s o f t e n s i o n
p n e u mo t h o r a x mu s t b e ma d e c l i n i c a l l y, b e c a u s e t h e r e i s n o t e n o u g h t i me f o r
i ma g i n g s t u d i e s . T h e d i a g n o s i s c a n b e c o n f i r me d b y t r e a t me n t : a l a r g e b o r e
n e e d l e i s p l a c e d t h r o u g h t h e s e c o n d i n t e r c o s t a l s p a c e a p p r o xi ma t e l y 2 t o 3 c m
f r o m t h e e d g e o f t h e s t e r n u m. A r u s h o f a i r a n d r e l i e f o f s y mp t o ms c o n f i r m t h e
diagnosis.
References
1 . B a u m G L , Wo l i n s k y Tex
E . t book of pul monar y di s eas
, 5 tes
h e d . Vo l . I I .
287 / 652
tmdmss
8.6 - Pneumothorax
B o s t o n , M A : L i t t l e , B r o w n a n d C o mp a n y, 1 9 9 4 : 1 8 7 1 1 8 7 5 .
2 . S a h n S A , H e f f n e r J E . S p o n t a n e o u s p n e u moNt hEongl
r a x.J M ed
2000;342:868874.
P. 1 6 5
3 . S c h r a me l F M N H , P o s t mu s P E , Va n d e r s c h u e r e n R G J R A . C u r r e n t a s p e c t s o f
s p o n t a n e o u s p n e u mo t h oEr aurx. R es pi r J1 9 9 7 ; 1 0 : 1 3 7 2 1 3 7 9 .
4 . L i g h t R W.P l eur al di s eas, es
3 r d e d . B a l t i mo r e , M D : W i l l i a ms & W i l k i n s ,
1995:242277.
5 . J a n t z M A , P i e r s o n D J . P n e u mo t h o r a x a n d b a r oCtlri anu C
ma
hes
. t M ed
1994;15(1):7591.
6 . S p i l l a n e R M , S h e p a r d J O , D e l u c a S A . R a d i o g r a p h i c a s p e c t s o f p n e u mo t h o r a x.
1995;51(2):459464.
288 / 652
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8.7 - Shortness of Breath
> T a b l e o f C o n t e n t s > 8 - R e s p i r a t o r y P r o b l e ms > 8 . 7 - S h o r t n e s s o f B r e a t h
8.7
Shortness of Breath
Robe rt M. The al
I . Background
S h o r t n e s s o f b r e a t h , o r d y s p n e a , a c c o u n t s f o r 3 . 7 % o f a l l v i s i t s t o me d i c a l c l i n i c s
(1) .
F o r t u n a t e l y, o n l y a h a n d f u l o f d i s o r d e r s c a u s e mo s t o f t h e c a s e s ; t h e r e f o r e , t h e mo s
e c o n o mi c a l a p p r o a c h i s t o e xc l u d e a c u t e l i f e - t h r e a t e n i n g p r o b l e ms d u r i n g t h e i n i t i a l
c l i n i c a l e xa mi n a t i o n . T h e s e i n c l u d e p n e u mo n i a , p u l mo n a r y e mb o l u s , a c u t e h e a r t
f a i l u r e , t o xi c e xp o s u r e o r i n g e s t i o n , my o c a r d i a l i n f a r c t i o n , p n e u mo t h o r a x, l i f e t h r e a t e n i n g n e u r o mu s c u l a r d i s e a s e , o r a i r w a y o b s t r u c t i o n . I f t h e s e a r e u n l i k e l y, t h e
n e xt s t e p i s t o t h e n s y s t e ma t i c a l l y e v a l u a t e t h e p a t i e n t f o r t h e mo s t f r e q u e n t
d i s o r d e r s u s i n g c o mmo n t e s t s .
Shortness of breath has a long differential diagnosis, but respiratory and cardiac
d i s e a s e s a c c o u n t f o r 8 5 % o f t h e c a s e s ; i n t h e r e ma i n i n g 1 5 % , o n l y a f e w i l l n e s s e s
a r e u s u a l l y f o u n2)d . ( O f a l l t h e f i n a l d i a g n o s e s i n p a t i e n t s w i t h d y s p n e a , t h e
f r e q u e n c y o f a s t h ma i s 1 8 % t o 3 3 % a n d c h r o n i c o b s t r u c t i v e p u l mo n a r y d i s e a s e i s 9 %
t o 1 9 % 1,2,3,4)
(
. C o n g e s t i v e h e a r t f a i l u r e ( C H F ) o r p u l mo n a r y e d e ma r e p r e s e n t s
11 % t o 6 3 % o f t h e c a s1,2,3,4)
e s ( . O t h e r i mp o r t a n t d i a g n o s e s a r e d e c o n d i t i o n i n g o r
o b e s i t y i n 3 % t o 51,2,3,4)
% (
. Final diagnoses ranging between 0% and 10% include
i n t e r s t i t i a l l u n g d i s e a s e a n d i s c h e mi c h e a r t 1,2,3,4)
d i s e a s. eT a( b l e 8 . 7 . l1i s t s l e s s
c o mmo n d i a g n o s e s .
I I I . Evaluation
T h e i n i t i a l h i s t o r y, p h y s i c a l e xa mi n a t i o n , a n d c h e s t x- r a y ( C X R ) a r e d i a g n o s t i c i n
6 6 % t o 9 2 % o f t h e p a t i e1)
n t. s (
A. History
Are historical features helpful? H istorical findings are neither sensitive nor specific
h o w e v e r, s o me s y mp t o ms a r e a s s o c i a t e d w i t h s p e c i f i c d i s e a s e s . R e g a r d l e s s o f t h e
cause, individuals associate shortness of breath with words that describe a sense o
w o r k o r e f f o r t t o b r e a t h e . A s t h ma i s a s s o c i a t e d w i t h w o r d s t h a t d e n o t e a s e n s e o
t i g h t n e s s . P a t i e n t s w i t h i n t e r s t i t i a l l u n g d i s e a s e c h o o s e t e r ms e mp h a s i zi n g t h e
s e n s e o f r a p i d b r e a t h i n g . D o e s t h e p a t i e n t s e l e c t t e r ms i n d i c a t i n g d i f f i c u l t y w i t h
289 / 652
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b o t h i n h a l a t i o n a n d e xh a l a t i o n ? T h i s i s o f t e n r e p o r t e d b y p a t i e nCt sh awpittehr C H F (
7 . 5) . P a t i e n t s w h o a r e d e c o n d i t i o n e d s e l e c t r a p i d , b r e a t h i n g mo r e , o r h e a v y t o
d e s c r i b e t h e i r d y s p n e a . P a t i e n t s s u f f e r i n g f r o m n e u r o mu s c u l a r d i s o r d e r s s e l e c t t e r m
denoting rapid breathing or difficulty with inhalation. Is the patient younger than 40
y e a r s o f a g e ? A r e t h e p a t i e n t ' s s y mp t o ms e p i s o d i c ? R e a c t i v e a i r w a y d i s e a s e a n d
h y p e r v e n t i l a t i o n a r e a s s o c i a t e d w i t h t h e s3)
e .t e r ms (
I n t h e p h y s i c a l e xa mi n a t i o n , f o c u s o n s i g n s o f r e s p i r a t o r y o r c a r d i a c d i s e a s e . F o r t h
r e s p i r a t o r y s y s t e m, t h i s me a n s a c a r e f u l e xa mi n a t i o n s t a r t i n g a t t h e n o s e .
S p e c i f i c a l l y, o n h e a d , e y e s , e a r, n o s e , a n d t h r o a t e xa mi n a t i o n l o o k f o r e v i d e n c e o f
o b s t r u c t i o n , i n f e c t i o n , o r p o s t n a s a l d r i p . E xc l u d e o b s t r u c t i o n , s u b c u t a n e o u s
e mp h y s e ma , o r t r a c h e a l d e v i a t i o n . O n c a r d i a c e xa mi n a t i o n , l o o k f o r e v i d e n c e o f
c a r d i o me g a l y,3 Sg a l l o p , o r h e p a t o j u g u l a r r e f l u x ( H J R ) . I n t h i s s e t t i n g , H J R i s v e r y
s p e c i f i c f o r C H1)
F . ( A s s e s s t h e l u n g s f o r a b n o r ma l b r e a t h s o u n d i n t e n s i t y, r a l e s ,
w h e e zi n g , r h o n c h i , o r t a c h y p n e a . E xa mi n e t h e c h e s t f o r a b n o r ma l
P. 1 6 6
mo v e me n t s o r d e f o r mi t i e s . E xc l u d e a b d o mi n a l ma s s e s , a s c i t e s , p r e g n a n c y, o r
a b d o mi n a l d i s t e n t i o n . E v a l u a t e t h e e xt r e mi t i e s f o r e d e ma , t e n d e r n e s s , o r a s y mme t r y
P e r f o r m a c o mp l e t e n e u r o l o g i c e xa mi n a t i o n , a n d s c r e e n f o r w e a k n e s s a t r o p h y,
sensory loss, and fasciculations.
TAB L E 8.7.1 Rare Diagnoses of Shortness of

Breath
A n e mi a A c i d o s i s
T hyroid disease
N e u r o mu s c u l a r d i s e a s e
P u l mo n a r y i n f e c t i o n
P u l mo n a r y e mb o l u s
P u l mo n a r y h y p e r t e n s i o n
P u l mo n a r y e f f u s i o n
Neoplasia
Airway obstruction
A r r h y t h mi a
A c u t e my o c a r d i a l i n f a r c t i o n
Atrial septal defect
Ve n t r i c u l a r s e p t a l d e f e c t
Mitral stenosis
Pericardial disease
C h e s t w a l l d e f o r mi t y
Postnasal drip
Sleep apnea
Hyperventilation
290 / 652
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aF r e q u e n c i e s b e t w e e n 0 % a n d 5 % .
C. Testing
1. M o s t p a t i e n t s r e q u i r e a C X R a n d p u l s e o xi me t r y t o s c r e e n f o r c a r d i a c a n d
p u l mo n a r y d i s e a s e s . U s e a n a r t e r i a l b l o o d g a s ( A B G ) a n a l y s i s t o c o n f i r m
h y p o xi a , h y p e r c a p n i a , h y p o c a p n i a , a n d a c i d o s i s . C o mp l e t e b l o o d c o u n t ( C B C ) ,
e l e c t r o l y t e s , t h y r o i d - s t i mu l a t i n g h o r mo n e ( T S H ) , a n d d r u g s c r e e n s a r e u s e f u l f o
s u s p e c t e d c a s e s o f a n e mi a , a c i d o s i s , h y p e r t h y r o i d i s m, h y p o t h y r o i d i s m, o r d r u g
ingestions.
2. P u l mo n a r y f u n c t i o n t e s t s ( P F T s ) a r e i mp o r t a n t t o d o c u me n t t h e p r e s e n c e o f
o b s t r u c t i v e o r r e s t r i c t i v e l u n g d i s e a s e s . A me t h a c h o l i n e c h a l l e n g e t e s t i s u s e d i
t h e s y mp t o ms a r e i n t e r mi t t e n t , i f t h e p a t i e n t i s y o u n g e r t h a n 4 0 y e a r s o f a g e , o r
i f l u n g d i s e a s e i s s u s p e c t e d a n d t h e P F T s a r e n o r ma l . I n t h i s s e t t i n g , t h e r e s u l t
w i l l c o n f i r m o r e xc l u d e a s t2)
h ma
. I n( p a t i e n t s w i t h d y s p n e a , a d i f f u s i n g c a p a c i t y
o f l u n g f o r c a r b o n mo n o xi d e ( D L C O ) h a s a h i g h p o s i t i v e p r e d i c t i v e v a l u e a n d a
h i g h n e g a t i v e p r e d i c t i v e v a l u e f o r i n t e r s t i t i a l l u n2)
g .d Li soewa sma
e (xi ma l
i n s p i r a t o r y a n d e xp i r a t o r y p r e s s u r e s s u g g e s t n e u r o mu s c u l a r d i s e a s e .
3. A n e l e c t r o c a r d i o g r a m ( E C G ) o r e xe r c i s e s t r e s s t e s t ( E S T ) s c r e e n s f o r
a r r h y t h mi a s a n d i s c h e mi c h e a r t d i Wa
s e arsnei .n g :A n e g a t i v e E S T d o e s n o t
e xc l u d e i s c h e mi a i n p a t i e n t s w i t h d y2)
s p(C
n ehaa p( t e r 7 ). .1 T h e c a r d i a c c a u s e s
o f d y s p n e a a r e C H F, i n t r a c a r d i a c s h u n t s , v a l v u l a r h e a r t d i s e a s e , p u l mo n a r y
h y p e r t e n s i o n , a n d p e r i c a r d i a l d i s e a s e . T h e y h a v e a b n o r ma l o r c h a r a c t e r i s t i c
f i n d i n g s o n e c h o c a r d i o g r a p h y a n d D o p p l e r e c h o c a r d i o g r a p h y.
P. 1 6 7
4. O t h e r t e s t s a r e u s e d i n s e l e c t e d p a t i e n t s . A h i g h - r e s o l u t i o n c o mp u t e d
t o mo g r a p h y ( C T ) s c a n o f t h e c h e s t d e t e c t s e a r l y i n t e r s t i t i a l l u n g d i s e a s e i n
p a t i e n t s w i t h n o r ma l C X R f i l ms . E l e c t r o my o g r a m ( E M G ) a n d n e r v e c o n d u c t i o n
s t u d i e s a r e u s e f u l f o r c o n f i r mi n g a n d d i f f e r e n t i a t i n g t h e mo s t c o mmo n
n e u r o mu s c u l a r p r o b l e ms : my a s t h e n i a g r a v i s a n d G u i l l a i n - B a r r s y n d r o me . A
t h e r a p e u t i c r e s p o n s e 2t ob lHo c k e r s c o n f i r ms g a s t r o e s o p h a g e a l r e f l u x d i s e a s e
( G E R D ) i n mo s t p a t i e n t s w i t h d y s3)p .n eSac r(e e n f o r a c u t e o r c h r o n i c
p u l mo n a r y e mb o l i s m w i t h a n u c l e a r me d i c i n e v e n t i l a t i o n a n d p e r f u s i o n ( [ V w i t h
dot above]/[Q with dot above]) scan.
I V. Diagnosis
T h e i n i t i a l a s s e s s me n t u s u a l l y r e q u i r e s a c l i n i c a l e v a l u a t i o n , C X R , a n d p u l s e
o xi me t r y. T h i s i d e n t i f i e s a b o u t 7 0 % o f t h e u n d e r l y i n g1)d. i sFeoar stehse (
r e ma i n d e r, a s y s t e ma t i c e v a l u a t i o n f o r t h e mo s t c o mmo n d i s e a s e s c o r r e c t l y i d e n t i f i e
the cause. If appropriate, consider obtaining an E C G, C B C, T S H, and electrolytes.
If these are nondiagnostic, further testing is then indicated.
A.
291 / 652
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E xc l u d e p u l mo n a r y d i s e a s e s i f t h e i n i t i a l e v a l u a t i o n i s n o n d i a g n o s t i c , o r i f p u l mo n a r
d i s e a s e s a r e s u s p e c t e d , w h i c h a c c o u n t f o r 7 5 % o f t2)h .e Sc taasret sw(i t h P F T s
a n d a n A B G . I f t h e P F T s a r e n o r ma l , o r d e r a me t h a c h o l i n e c h a l l e n g e t e s t t o r u l e o u
a s t h ma . I f i n t e r s t i t i a l l u n g d i s e a s e i s s u s p e c t e d o r i f t h e P F T s s h o w a r e s t r i c t i v e
p a t t e r n , t h e n o r d e r a D L C O . A b n o r ma l l y l o w ma xi ma l i n s p i r a t o r y a n d e xp i r a t o r y
p r e s s u r e s s u g g e s t n e u r o mu s c u l a r d i s e a s e . C o n f i r m t h e d i a g n o s i s w i t h a n E M G .
B.
W h e n p u l mo n a r y d i s e a s e h a s b e e n e xc l u d e d , o r i f c a r d i a c d i s e a s e i s s u s p e c t e d , t h e
n e xt s t e p s h o u l d b e a c a r d i a c e v a l u a t i o n . A n e c h o c a r d i o g r a m s u g g e s t s o r i d e n t i f i e s
mo s t o f t h e c a r d i a c c a u s e s . I f t h e e c h o c a r d i o g r a m i s n o r ma l , c o n s i d e r e xe r c i s e
s t r e s s t e s t i n g o r a H o l t e r mo n i t o r. I f t h e s e a r e n o r ma l , mo s t p a t i e n t s w i l l h a v e e i t h e
G E R D or deconditioning, or psychogenic disorders. Other low frequency causes of
s h o r t n e s s o f b r e a t h t h a t n e e d f u r t h e r e v a l u a t i o n i n c l u d e n e u r o mu s c u l a r d i s e a s e s ,
p u l mo n a r y e mb o l i , p o s t n a s a l d r i p , a n d s l e e p a p n e a . W i t h a c l i n i c a l s u s p i c i o n o f
these disorders, obtain an E M G, [V with dot above]/[Q with dot above] scan, or
p o l y s o mn o g r a m. O t h e r w i s e , t h e y a r e n o t i n d i c a t e d .
References
1 . M u l r o w C D , L u c e y C R , F a r n e t t L E . D i s c r i mi n a t i n g c a u s e s o f d y s p n e a t h r o u g h
c l i n i c a l e xa mi n a t i Jo nG. en I nt er n M ed
1993;8:383392.
2. Pratter M R, Curley F J, Dubois J, et al. Cause and evaluation of chronic
d y s p n e a i n a p u l mo n a r y d i s e a s e Ac rl icnhi cI.nt er n M ed
1989;149:22772282.
3 . D e P a s o W J , W i n t e r b a u e r R H , L u s k J A , e t a l . C h r o n i c d y s p n e a u n e xp l a i n e d b y
h i s t o r y, p h y s i c a l e xa mi n a t i o n , c h e s t r o e n t g e n o g r a m, a n d Cshes
p i r ot me t r y.
1991;100:12931299.
4 . S c h mi t t B P, K u s h n e r M S , W i e n e r S L . T h e d i a g n o s t i c u s e f u l n e s s o f t h e h i s t o r y
o f t h e p a t i e n t w i t h d y s pJn eGaen
. I nt er n M ed
1986;1:386393.
Suggested Reading
M ahler D A, H arver A, L entine T, etal. D escriptors of breathlessness in
c a r d i o r e s p i r a t o r y d i s e aAs ems .J R es pi r C r i t C ar e M1ed
996;154:13571363.
292 / 652
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8.8 - Stridor
> T a b l e o f C o n t e n t s > 8 - R e s p i r a t o r y P r o b l e ms > 8 . 8 - S t r i d o r
8.8
Stridor
Ale x andra Duke
Ta h a n y M a u r i c e - H a b a s h y
I . Background
S t r i d o r i s a c o mmo n t y p e o f w h e C
e zi
h anpgt e( r 8 ). .9 I t i s c h a r a c t e r i ze d b y a h a r s h ,
r a s p y, me d i u m- p i t c h e d s o u n d , p r o d u c e d a s a i r f l o w s t h r o u g h a p a r t i a l l y b l o c k e d
a i r w a y. I t i s u s u a l l y s e e n i n e a r l y c h i l d h o o d .
S t r i d o r c a n b e i n s p i r a t o r y, i n d i c a t i n g a n o b s t r u c t i o n a t o r a b o v e t h e l a r y n x; o r
e xp i r a t o r y, i n d i c a t i n g a n o b s t r u c t i o n b e l o w t h e l a r y n x. B i p h a s i c
P. 1 6 8
s t r i d o r i s a n o b s t r u c t i o n i n t h e t r a c h e a ; i t i s h e a r d w i t h i n s p i r a t i o n a n d e xp i r a t i o n .
W h e n h o a r s e n e s s o r a p h o n i a a c c o mp a n i e s s t r i d o r, t h e v o c a l c o r d s a r e i n v o l v e d ( s e e
T a b l e 8 . 8 ). 1(1,2,3,4,5) .
TAB L E 8.8.1 Com m on Causes of Stridor

C o n g e n i t aa l
L a r y n g o ma l a c i a
I nflam m atory
Laryngotracheobronchitis (croup)
L a r y n g e a l c y s t s a n d w e bEs p i g l o t t i t i s , b a c t e r i a l t r a c h e i t i s
L a r y n g e a l h e ma n g i o ma s R e t r o p h a r y n g e a l a b s c e s s
T u mo r s
A l l e r g i c e d e ma
Subglottic stenosis
Diphtheria, tetanus
Vo c a l c o r d d y s f u n c t i o n
Micrognathia
Noninflam m atory
293 / 652
tmdmss
8.8 - Stridor
Va s c u l a r r i n g
Foreign body
Ectopic thyroid
Cri du chat
Hysterical stridor
Macroglossia
aM o r e c o mmo n i n c h i l d r e n y o u n g e r t h a n 6 mo n t h s o f a g e .
I I I . Evaluation
A. History
1. C h a r a c t e r i s t i c s o f s t r i d
Wohr.e n c o n f r o n t e d w i t h s t r i d o r, c h e c k t h e a g e o f t h e
p a t i e n t a n d t h e d u r a t i o n o f t h e s y mp t o ms . A c h i l d y o u n g e r t h a n 6 mo n t h s o f a g e
w i t h s t r i d o r l a s t i n g a f e w w e e k s t o mo n t h s h a s a c o n g e n i t a l c a u s e o f s t r i d o r. A
p a t i e n t o l d e r t h a n 6 mo n t h s o f a g e w i t h s t r i d o r l a s t i n g h o u r s t o d a y s u s u a l l y h a s
a n a c q u i r e d c a u s e o f s t r i d o r, mo s t c o mmo n l y v i r a l c r o u p , e p i g l o t t i t i s , o r
a s p i r a t i o n o f a f o r e i g n b o d y.
a. A t y p i c a l h i s t o r y i s a c h i l d y o u n g e r t h a n 6 y e a r s o f a g e w i t h a 2 - t o 3 - d a y
history of upper respiratory infection (U R I) and gradually worsening cough,
especially at night. A barking cough with the inspiratory stridor heralds the
d i a g n o s i s o f c r o u p , w h i c h a c c o u n t s f o r 9 0 % o f a l l c a s e s o f s t r i d o r. T h i s
c o n d i t i o n c l a s s i c a l l y i mp r o v e s w i t h mo1,3)
i s t. a i r (
b. A h i s t o r y o f c h o k i n g , c o u g h i n g , o r g a g g i n g p o i n t s t o a s p i r a t i o n o r i n g e s t i o n
o f a f o r e i g n b o d y.
c. I n o l d e r c h i l d r e n a n d a d u l t s , a c o n c o mi t a n t s o r e t h r o a t a n d f e v e r ma y
i n d i c a t e a c u t e s u p r a g l o t t i t i s , w h i c h c o n s t i t u t e s a n e me r g e n c y.
2. O t h e r i n f o r ma t i o n
a. I t i s n e c e s s a r y t o l e a r n w h e t h e r t h e s t r i d o r i s a c u t e , r e c u r r e n t , o r c h r o n i c .
b. A p e r s o n a l o r f a mi l y h i s t o r y o f a t o p y s u g g e s t s s p a s mo d i c c r o u p , w h i c h
presents with stridor at night, not necessarily associated with a U R I.
1. F o c u s e d p h y s i c a l e xa mi n a t i o n
a. T h e e xa mi n a t i o n s h o u l d i n c l u d e v i t a l s i g n s , n o t a b l y t e mp e r a t u r e a n d
r e s p i r a t o r y r a t e , a n d p u l s e , w i t h a n e mp h a s i s o n g e n e r a l a p p e a r a n c e , a n d
e xa mi n a t i o n o f t h e h e a d a n d n e c k , i n c l u d i n g e a r s , n o s e , a n d t h r o a t .
b. S i g n s o f r e s p i r a t o r y d i s t r e s s ma y b e p r e s e n t , i n c l u d i n g d y s p n e a , t a c h y p n e a ,
294 / 652
tmdmss
8.8 - Stridor
c h e s t r e t r a c t i o n s , n a s a l f l a r i n g , a n d s t r i d o r. I f c y a n o s i s i s p r e s e n t , t h i s i s a n
o mi n o u s s i g n2,4)
( (C h a p t e r 8 ). .2
2. A d d i t i o n a l p h y s i c a l e xa mi n a t i o n
a. A t o xi c - a p p e a r i n g c h i l d w i t h h i g h f e v e r h a s d r o o l i n g , s e v e r e r e s p i r a t o r y
d i s t r e s s , a n d a p r e f e r e n c e f o r a s i t t i n g a n d f o r w a r d - l e a n i1,4)
n g .p o s i t i o n (
P. 1 6 9
b. Va r y i n g d e g r e e s o f a n xi e t y, w h i c h i n c r e a s e d u r i n g e xa mi n a t i o n , c a u s e a
w o r s e n i n g o f s t r i d1,4)
o r (.
C. Testing
1. T h e b e s t t e s t i s a l a t e r a l n e c k x- r a y s t u d y t o a s s i s t w i t h a d i a g n o s i s t h a t i s
mo s t l y ma d e o n c l i n i c a l g r o u n d s . F i l ms o f t h e l a r y n x a n d t r a c h e a i n
a n t e r o p o s t e r i o r a n d l a t e r a l n e c k v i e w s ma y s h o w a n a r r o w i n g o f t h e t r a c h e a o r
e xt r i n s i c p r e s s u r e o n t h e t r a c h e o b r o n c h i a l a i r w a y. A c u t e l y, l a t e r a l n e c k
r a d i o g r a p h s s h o w i n g t h e c l a s s i c s w o l l e n g l o t t i s d e s c r i b e d b y s o me a s a
t h u mb p r i n t a s s i s t w i t h t h e d i a g n o s i s o f a c u t e s u p r a g l o t t i t i s a n d e mi n e n t
r e s p i r a t o r y c o l l a p s e . C h e s t x- r a y s t u d i e s a r e o f l i t t l e v a l u e . F i l ms s h o w i n g
h y p e r i n f l a t i o n o r b r o n c h i a l t h i c k e n i n g ma y h e l p t o ma k e a d i a g n o s i s o f a s t h ma ,
r a t h e r t h a n s t r i d o r. A d d i t i o n a l l y, f o r e i g n b o d y a s p i r a t i o n o r ma s s i s e l u c i d a t e d i n
x- r a y s t u d i e 2)
s .(
2. T o mo g r a ms o r a c o mp u t e d t o mo g r a p h y ( C T ) s c a n o f t h e n e c k ma y p r o v i d e
a d d i t i o n a l i n f o r ma t i o n , e s p e c i a l l y i n c h r o n i 2)
c .s t r i d o r (
3. B l o o d t e s t s ( e . g . , c o mp l e t e b l o o d c o u n t ) c a n b e u s e f u l i n t h e a c u t e l y i l l p a t i e n t ,
especially if viral or bacterial infection is suspected.
4. I f i t i s s u s p e c t e d t h a t t h e s t r i d o r i s a r e s u l t o f a l a r y n g o ma l a c i a o r l a r y n g e a l
l e s i o n s s u c h a s p a p i l l o ma , d i r e c t l a r y n g o s c o p y i s t h e t e s t o f c h o i c e f o r a c c u r a t e
diagnosis. Direct observation through fiber-optic bronchoscope positioned in the
p h a r y n x p r o v i d e s d i a g n o s t i c v i e w s o f t h e2,4)
l a r. y n x (
I V. Diagnosis
I n ma k i n g t h e d i a g n o s i s o f s t r i d o r, t w o k e y e l e me n t s e xi s t : a c u t e o n s e t i n a t o xi c appearing patient versus chronic stridor in a relatively stable patient.
A. Acute stridor
1. T h e mo s t l i k e l y c a u s e o f a c u t e s t r i d o r i n t h e f e b r i l e c h i l d w i t h t h e a d d i t i o n a l
f e a t u r e s o f b a r k i n g c o u g h a n d a n t e c e d e n t c o r y za i s l a r y n g o t r a c h e o b r o n c h i t i s o r
croup. Acute stridor is a nonlife-threatening condition accounting for 90% of
s t r i d o r c a s e s . C l a s s i c a l l y, i t i mp r o v e s w i t h e xp o s u r e t o mo i s t a i r. I t h a s a v i r a l
cause, usually from one of the following: respiratory syncytial virus, rhinovirus,
a d e n o v i r u s , p a r a i n f l u e n za v i r u s , o r i n f l u e n za v i r u s . G e n e r a l l y, t h i s d i a g n o s i s i s
ma d e o n c l i n i c a l g r o u n1)d.s T( h e c h i l d i s l e s s i l l a n d , a l t h o u g h o f t e n f e b r i l e , n o t
t o xi c a p p e a r i n g . T h e e n t i r e i l l n e s s u s u a l l y a b a t e s i n 5 d a y s . H o s p i t a l i za t i o n ,
295 / 652
tmdmss
8.8 - Stridor
u n l i k e w i t h e p i g l o t t i t i s , i s r a r e l y n2)
e e. d e d (
2. I n t h e t o xi c p a t i e n t w i t h f e v e r, r e s p i r a t o r y d i s t r e s s , s o r e t h r o a t , o r d r o o l i n g ,
e s p e c i a l l y i n t h e y o u n g e r a g e - g r o u p , e p i g l o t t i t i s a me d i c a l e me r g e n c y s h o u l d
b e c o n s i d e r e d . U s e o f Ht aemophi
he
l us i nf l uenzae
vaccine has increased in
r e c e n t y e a r s ; t h e r e f o r e , a c u t e e p i g l o t t i t i s i s b e c o mi n g i n c r eHa.s i n g l y r a r e .
i nf l uenzaei s t h e mo s t c o mmo n b a c t e r i a l c a u s e o f s t r i d o r, a l t h o u g h
streptococcus, staphylococcus, and viral agents are also possible causes.
3. T h e p a t i e n t w i t h a h i s t o r y o f s u s p e c t e d f o r e i g n b o d y a s p i r a t i o n h a s s i mi l a r
s y mp t o ms w i t h o u t f e v e r. F o r e i g n b o d y a s p i r a t i o n i s c o mmo n i n 1 - t o 2 - y e a r - o l d
children, although it does occur in adults. It can be a cause of chronic stridor
(3) .
4. A d d i t i o n a l l y, a n a c u t e a l l e r g i c r e a c t i o n c a n c a u s e s t r i d o r. T h e h i s t o r y s h o u l d
h e r a l d a p o s s i b l e o f f e n d i n g a g e n t a n d , a l t h o u g h r e s p i r a t o r y c o l l a p s e ma y b e
e mi n e n t , t h e p a t i e n t i s n o t t o xi c , a s n o i n f e c t i o u s a g e n t i s i n v o l v e d .
5. T r a u ma c a n a l s o c a u s e l a r y n g e a l d a ma g e ; h o w e v e r, t h e h i s t o r y a s s i s t s w i t h t h i s
diagnosis.
B. Chronic stridor
F o r t h e mo s t p a r t , t h e s e c a u s e s o f s t r i d o r o c c u r i n e a r l y c h i l d h o o d . W i t h t h e
e xc e p t i o n o f l a r y n g e a l p a p i l l o ma s , t u mo r s , a n d s u b g l o t t i c s t e n o s i s a f t e r
i n s t r u me n t a t i o n , a s i n i n t u b a t i o n ( t h e r e i s a c o n g e n i t a l f o r m a l s o ) , f o r e i g n b o d y
aspiration with partial obstruction and hysterical stridor can occur at any age.
L a r y n g o ma l a c i a a n d l a r y n g e a l l e s i o n s a r e c a u s e d b y w e b s , h e ma n g i o ma s , a n d c y s t s
t h e y a r e u s u a l l y i d e n t i f i e d e a r l y 1,2,3)
i n l i f .e (
References
1 . P r y o r M P. N o i s y b r e a t h i n g i n c h iPl dos
r et gr
n . ad M ed
1 9 9 7 ; 1 0 1 : 1 0 3 11 2 .
2 . B e h r ma n R E , K l i e g ma n R M , A r v i nN el
AM
s on
. t ex t book of pedi at. r i c s
P h i l a d e l p h i a , PA : W B S a u n d e r s , 1 9 9 6 : 2 4 1 , 11 7 3 , 11 9 8 , 1 2 3 8 .
P. 1 7 0
3 . B e h r ma n R E , Va u g h a n N
VC
el. s on t ex t book of pedi at. rPi chsi l a d e l p h i a , PA :
WB Saunders, 1983:10311032, 10761077.
4 . T i n t i n a l l i J E , R u i z E , K r o meE mer
R L . genc y medi c i ne: a c ompr ehens i v e s t udy
gui de. N e w Yo r k : M c G r a w - H i l l , 1 9 9 6 : 2 4 7 2 5 1 .
5 . C a mp b e l l A G M , M a c I n t o s hTex
N .t book of pedi at r. i cL so n d o n : P e a r s o n L t d ,
1998:508513, 563.
296 / 652
tmdmss
8.9 - Wheezing
> T a b l e o f C o n t e n t s > 8 - R e s p i r a t o r y P r o b l e ms > 8 . 9 - W h e e zi n g
8.9
Wheezing
Thom as C. Be nt
I . Background
W h e e zi n g i s o n e o f t h e mo s t c o mmo n r e s p i r a t o r y c o mp l a i n t s t o p r e s e n t t o p r i ma r y
care physicians.
A l t h o u g h a s t h ma a n d c h r o n i c o b s t r u c t i v e p u l mo n a r y d i s e a s e ( C O P D ) a c c o u n t f o r
mo s t c a s e s o f w h e e zi n g , t h e r e a r e mu l t i p l e c a u s e s . T h e N a t i o n a l A s t h ma E d u c a t i o n
a n d P r e v e n t i o n P r o g r a m ( N A E P P ) r e c o mme n d s a n a c c u r a t e d i a g n o s i s b e ma d e b y
me d i c a l h i s t o r y, p h y s i c a l , a n d s p i r o1)me
. try (
A. Wheezing in infants, children, and adults
T h e r e a s o n s w h y p a t i e n t s w h e e ze v a r y d r a ma t i c a l l y, d e p e n d i n g o n a g e . F o r e xa mp l e
w h e r e a s a s t h ma i s t h e mo s t c o mmo n c h r o n i c p e d i a t r i c d i s e a s e i n i n d u s t r i a l i ze d
n a t i o n s , i n h a l a n t a l l e r g e n s a p p e a r t o b e u n i mp o r t a n t p r e c i p i t a n t s o f w h e e zi n g i n
i n f a n c y 2)( ( s e eT a b l e 8 . 9 ). .1 W h e e zi n g t h a t b e g i n s i n i n f a n c y o r e a r l y c h i l d h o o d i s
a s s o c i a t e d w i t h p r o g r e s s i v e a n d p e r s i s t e n t 3)
d i.s e a s e (
B. Wheezing versus stridor
S t r i d o r, d i s c u s s e dC ihna p t e r 8 ,. 8i s c h a r a c t e r i ze d a s a n i n s p i r a t o r y w h e e ze t h a t
i mp l i e s ma j o r o b s t r u c t i o n o f t h e u p p e r a i r w a y. W h e e zi n g , i n c o n t r a s t , i s d e f i n e d a s
h i g h - p i t c h e d , c o n t i n u o u s ( o r l o n g d u r a t i o n ) l u n g s o u n d s t h a t a r e s u p e r i mp o s e d o n
t h e n o r ma l b r e a t h s o u n4)d.s T( h e i n s p i r a t o r y p h a s e o f r e s p i r a t i o n i s u s u a l l y n o r ma l ,
a n d t h e e xp i r a t o r y p h a s e i s p r o l o n g e d . U n f o r t u n a t e l y, t h e d i f f e r e n c e i s n o t a l w a y s
o b v i o u s t o t h e c l i n i c i a n . Vo c a l c o r d d y s f u n c t i o n , w h i c h i s a p s y c h o s o ma t i c d i s o r d e r,
c a n b e d i f f i c u l t t o d i f f e r e n t i a t e f r o m a s t h ma . T h e s e e p i s o d e s c a n i n c l u d e b o t h
i n s p i r a t o r y a n d e xp i r a t o r y w h e e zi n g a n d a n u p p e r a i r w a y c a u s e5)i s. n o t c l e a r (
TAB L E 8.9.1 Etiology of Wheezing by Age-Group

I nfants
Bronchiolitis, pertussis, recurrent aspiration during feeding,
297 / 652
tmdmss
8.9 - Wheezing
g a s t r o e s o p h a g e a l r e f l u x d i s e a s e , f o r e i g n b o d y i n h a l a t i o n , b r o n c h o p u l mo n a r y
dysplasia, cystic fibrosis, tracheoesophageal fistula, congenital
ma l f o r ma t i o n s
Childre n
A s t h ma , b r o n c h i o l i t i s , t r a c h e o ma l a c i a , g a s t r o e s o p h a g e a l r e f l u x, s i n u s i t i s ,
f o r e i g n b o d i e s , c y s t i c f i b r o s i s , a n d p u l mo n a r y h e mo s i d e r o s i s
Adults
A s t h ma , c h r o n i c o b s t r u c t i v e p u l mo n a r y d i s e a s e , a c u t e i n f e c t i o n s , f o r e i g n
b o d y i n h a l a t i o n , i n t r a - a i r w a y t u mo r, e xt r i n s i c t u mo r w i t h a i r w a y c o mp r e s s i o n ,
interstitial lung disease
P. 1 7 1
I I I . Evaluation
A. History
1. O n s e t I s t h i s t h e f i r s t e p i s o d e ? I f s o , w e r e t h e r e p r o b l e ms w i t h w h e e zi n g o r
a s t h ma i n c h i l d h o o d ?
2. E x p o s u r e sT r i g g e r s t o w h e e zi n g s h o u l d b e i d e n t i f i e d a n d c1)
o n. t r o l l e d (
a. C i g a r e t t e s mo k e i s o n e o f t h e mo s t p o t e n t a n d u b i q u i t o u s a v o i d a b l e
allergens.
b. O c c u p a t i o n a l e xp o s u r e s c a n f r e q u e n t l y b e i d e n t i f i e d , e s p e c i a l l y b e t w e e n
agricultural and industrial workers.
c. F a mi l y o r h o u s e h o l d e xp o s u r e t o t u b e r c u l o s i s o r p e r t u s s i s c a n i n d i c a t e a n
infectious cause.
3. C o m o r b i d c o n d i t i oH
n as s t h e p a t i e n t r e c e n t l y s u f f e r e d a n u p p e r r e s p i r a t o r y
infection or sinusitis? Is there a history of gastroesophageal reflux disease?
C ongestive heart failure? Aspirin sensitivity? Allergic rhinitis?
4. F a m i l y h i s t o rAy h i s t o r y o f a s t h ma , a l l e r g i e s , o r a t o p i c d i s e a s e i n f a mi l y
me mb e r s c a n s u p p o r t t h e d i a g n o s i s o f a s t h ma .
5. P a s t h i s t o rAy c h i l d h o o d h i s t o r y o f a t o p i c d i s e a s e o r a l l e r g i e s s u g g e s t s a d u l t
o n s e t a s t h ma . P a s t h i s t o r y o f e xe r c i s e - i n d u c e d w h e e zi n g a l s o s u p p o r t s t h i s
diagnosis.
6. P s y c h o s o c i a l a s p e cEtmo
s t i o n a l s t r e s s c a n l e a d t o e xa c e r b a t i o n o f c h r o n i c
a s t h ma . P s y c h o g e n i c w h e e zi n g i s a c o n v e r s i o n d i s o r d e r, w h i c h c a n c o e xi s t w i t h
o t h e r p s y c h o p a t h o l o g y.
298 / 652
tmdmss
8.9 - Wheezing
1. V i t a l s i g n A
s c o mp l e t e s e t o f v i t a l s i g n s i s e s s e n t i a l t o t h e a s s e s s me n t o f t h e
p a t i e n t w i t h w h e e zi n g . T h e r e s p i r a t o r y r a t e a n d t h e p u l s e a r e a mo r e o b j e c t i v e ,
a n d o f t e n mo r e a c c u r a t e , a s s e s s me n t o f t h e s e v e r i t y o f w h e e zi n g , r a t h e r t h a n
t h e a u d i t o r y v o l u me o f t h e w h e e zi n g i t s e l f . F e v e r s u g g e s t s a c o n c u r r e n t
r e s p i r a t o r y i n f e c t i o n . H y p o t e n s i o n i s a n o mi n o u s s i g n t h a t p o i n t s t o a
d e c o mp e n s a t i n g p a t i e n t .
2. L u n g e x a m i n a t i oDnu r i n g a u s c u l t a t i o n , n o t e t h e l o c a t i o n , i n t e n s i t y, a n d t h e
d u r a t i o n o f w h e e zi n g . W h e e zi n g c a u s e d b y a s t h ma , C O P D , o r i n t e r s t i t i a l
d i s e a s e s h o u l d b e d i f f u s e a n d s y mme t r i c a n d p r e s e n t d u r i n g e xp i r a t i o n . T h e
e xp i r a t o r y p h a s e i s p r o l o n g e d . F o c a l o b s t r u c t i o n ( e . g . , t u mo r s a n d f o r e i g n
b o d i e s ) c a n g i v e a s y mme t r i c f i n d i n g s a n d i n s p i r a t o r y w h e e zi n g . M u c o u s
plugging changes with cough. Rhonchi and crackles suggest a concurrent
infectious process. Percussion and egophony can be present with consolidation
C. Testing
1. P u l m o n a r y f u n c t i A
o np e a k f l o w me t e r i s a v a l u a b l e i n i t i a l a s s e s s me n t o f
airway obstruction, and can be done quickly and cheaply in the office. It is also
a n e xc e l l e n t me a s u r e me n t o f p r o g r e s s i o n o f d i s e a s e o r s u c c e s s o f t r e a t me n t .
P u l s e o xi me t r y i s a n o t h e r q u i c k , n o n i n v a s i v e o f f i c e t e c h n i q u e t o a s s e s s t h e
s e v e r i t y o f b o t h c h r o n i c d i s e a s e a n d a c u t e r e s p i r a t o r y d i s t r e s s . F u l l s p i r o me t r y,
a l t h o u g h n o t a v a i l a b l e i n a l l p r i ma r y c a r e o f f i c e s , g i v e s a d d i t i o n a l d i a g n o s t i c
i n f o r ma t i o n t h a t c a n d i f f e r e n t i a t e a mo n g a s t h ma , C O P D , a n d f i xe d a i r w a y
obstruction.
2. C h e s t x - r a yP l a i n c h e s t f i l ms i d e n t i f y c o n s o l i d a t i o n , ma s s e s , me d i a s t i n a l s h i f t s ,
and hyperaeration.
3. C l i n i c a l l a b o r a t o r y t eAs tcso mp l e t e b l o o d c o u n t ma y d e mo n s t r a t e s i g n s o f a n
a c u t e b a c t e r i a l i n f e c t i o n . P o l y c y t h e mi a i s a s i g n o f c h r o C
n ihcahpyt epro xi a (
1 6 . 5) . E o s i n o p h i l i a c a n i n d i c a t e a s t h ma o r a l l e r g i cCdhiaspetaesr e1(6). .2
A n g i o t e n s i n - c o n v e r t i n g e n zy me l e v e l s a r e e l e v a t e d i n s a r c o i d o s i s . A t u b e r c u l i n
s k i n t e s t s h o u l d b e c o n s i d e r e d i n a l l p a t i e n t s w i t h w h e e zi n g o r c h r o n i c c o u g h .
I V. Diagnosis
T h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n a r e t h e k e y e l e me n t s t o a n a c u t e d i a g n o s i s . A
c o n s i s t e n t e xp o s u r e o r r e a c t i o n h i s t o r y, c o u p l e d w i t h a n e l e v a t e d s e r u m
i mmu n o g l o b u l i n E o r e o s i n o p h i l i a , i n d i c a t e s a l l e r g i c d i s e a s e . W h e e zi n g i n t h e s e t t i n
o f a c u t e b r o n c h i t i s o r s i n u s i t i s i s n o t t r u e a s t h ma , a n d t h e p a t i e n t c a n b e r e a s s u r e d
t h a t t h i s i s n o t t h e b e g i n n i n g o f a c h r o n i c d i s e a s e . I n s p i r a t o r y w h e e zi n g , o r s t r i d o r,
i n d i c a t e s u p p e r a i r w a y o b s t r u c t i o n o r p s y c h o g e n i c w h e e zi n g . A n o r ma l , o r n e a r l y
n o r ma l , p e a k f l o w i s r e a s s u r a n c e t h a t g o o d a i r
P. 1 7 2
e xc h a n g e i s o c c u r r i n g , r e g a r d l e s s o f t h e l o u d n e s s o f t h e w h e e zi n g . T h e p u l s e
o xi me t r y d i f f e r e n t i a t e s b e t w e e n s e v e r e o b s t r u c t i o n a n d p o o r c o o p e r a t i o n w i t h t h e
299 / 652
tmdmss
8.9 - Wheezing
p e a k f l o w t e s t i n g . W h e n c o n f u s i o n s t i l l e xi s t s , s p i r o me t r y c l a r i f i e s t h e d i a g n o s i s i n
mo s t c a s e s .
T h e i mme d i a t e a s s e s s me n t o f t h e p a t i e n t w i t h a c u t e w h e e zi n g i s e s s e n t i a l .
Regardless of whether the patient presents with an initial episode or a chronic
c o n d i t i o n , i t i s n e c e s s a r y t o d e t e r mi n e t h e d e g r e e o f a i r w a y o b s t r u c t i o n a n d t h e
p o t e n t i a l d e t e r i o r a t i o n o f t h e p a t i e n t q u i c k l y. T h e r e d u c t i o n i n t h e i n t e n s i t y o f
w h e e zi n g c a n i n d i c a t e a c u t e d e c o mp e n s a t i o n , a s a i r o b s t r u c t i o n b e c o me s t o o s e v e r e
t o a l l o w t h e me c h a n i c a l s o u n d s o f w h e e zi n g .
References
1 . K e y c l i n i c a l a c t i v i t i e s f o r q u a l i t y a s t h ma c a r e : r e c o mme n d a t i o n s o f t h e N a t i o n a l
A s t h ma E d u c a t i o n a n d P r e v e n t i o n P r o g r a m.
w w w. g u i d e l i n e s . g o v / s u mma r y / s u mma r y. a s p x?
d o c _ i d = 3 7 3 4 & = 2 9 6 0 & s t r i n g = a s t h ma , a c c e s s e d o n M a y, 2 0 0 6 .
2 . M a r t i n a t i L C , B o n e r A L . C l i n i c a l d i a g n o s i s o f w h e e zi n g i n e a r l y c h i l d h o o d .
A l l er gy1 9 9 5 ; 5 0 : 7 0 1 7 1 0 .
3 . C a s t r o - R o d r g u e z J A , e t a l . A c l i n i c a l i n d e x t o d e f i n e r i s k o f a s t h ma i n y o u n g
c h i l d r e n w i t h r e c u r r e n t w h e eAzimn gJ. R es pi r C r i t C ar e M2ed
000;162:1403
1 4 0 6 . h t t p : / / w w w. a a f p . o r g / a f p / 2 0 0 1 0 5 0 1 / t i p s / 1 2 . h t ml .
4 . M e s l i e r N , C h a r b o n n e a u G , R a c i n e u x J L . WEhur
e e ze
R es
s . pi r J
1995;8:19421948.
5 . G o l d ma n J . A l l t h a t w h e e ze s i s n o t aPs rt ac
h ma
t i t.i oner1 9 9 7 ; 2 4 1 : 3 5 3 8 .
300 / 652
tmdmss
9.1 - Abdominal Pain
> T a b l e o f C o n t e n t s > 9 - G a s t r o i n t e s t i n a l P r o b l e ms > 9 . 1 - A b d o mi n a l P a i n
9.1
Abdominal Pain
Dav id R. Congdon
I . Background
A b d o mi n a l p a i n i s a c o mmo n c o mp l a i n t a n d c o mp r i s e s u p t o 5 % o f t o t a l v i s i t s t o t h e
e me r g e n c y d e p a r t me n t . T h e e t i o l o g y ma y b e q u i t e v a r i e d a n d ma y r e s u l t f r o m e xt r a a b d o mi n a l p a t h o l o g y o r i n t r a - a b d o mi n a l s o u r c e s .
T h e c o mmo n r e a s o n s f o r a b d o mi n a l p a i n t h a t c a u s e s c o n c e r n a r e Tpar belsee n t e d i n
9 . 1 . 1(1) . S e l e c t e d c a u s e s o f s e v e r e a b d o mi n a l p a i n a r eT al ibsl tee d9 . i1n. 2
I I I . Evaluation
A. History
1. T h e h i s t o r y s h o u l d i n c l u d e t h e f o l lPa
o wl li inagt :i v e / a l l e v i a t i n g f a cQu
t o rasl,i t y o f
p a i n ,Ra d i a t i o n o r r e f e r r e d p a i n p aSe
t t evr enr, i t y, a nTi
d me o f o n s e t / t e mp o r a l
P. 1 7 6
r e l a t i o n s h i p s . T h e c h a r a c t e r o f p a i n i s p a r t i c u l a r l y h e l p f u l ( e . g . , c o l i c k y, s t e a d y
s h a r p , b u r n i n g , t e a r i n g , g n a w i n g ) . T h e a s s o c i a t e d s y mp t o ms s h o u l d b e
i d e n t i f i e d ( e . g . , d y s u r i a , h e ma t u r i a , h e ma t o c h e zi a , c h a n g e i n b o w e l h a b i t s ,
p e r s i s t e n t v o mi t i n g ) . T h e e xa c t s e q u e n c e o f s y mp t o ms ma y b e e s p e c i a l l y
h e l p f u l . F o r e xa mp l e , i n a c u t e a p p e n d i c i t i s , p a i n a n d a n o r e xi a t y p i c a l l y p r e c e d e
point tenderness.
TAB L E 9.1.1 Com m on Causes of Abdom inal

Pain
Diagnosis
Etiology
Epide m iology
Acute
Appendiceal obstruction, Adolescence to young
a p p e n d i c i t i s i n f l a mma t i o n , i s c h e mi a
adulthood
Higher perforation rate in
c h i l d r e n , w o me n , a n d
301 / 652
tmdmss
elderly
Mortality rate of 0.1%
(2%6% with perforation)
B i l i a r y t r a c t S t o n e i n c y s t i c o r c o mmo n P e a k a g e o f 3 5 6 0 y
disease
duct is the typical cause.
Rare in patients <20 y of
age
F e ma l e - t o - ma l e r a t i o o f
3:1
R i s k f a c t o r s : mu l t i p a r i t y,
o b e s i t y, a l c o h o l i n t a k e ,
birth control pills
Ureteral
colic
F a mi l y h i s t o r y, d e h y d r a t i o nAv
, e r a g e a g e o f 3 0 4 0 y,
u r i n a r y t r a c t i n f e c t i o n s , l e s s c o mmo n i n c h i l d r e n
c e r t a i n me d i c a t i o n s
Diverticulitis Diverticular
Incidence increases with
i n f e c t i o n / i n f l a mma t i o n o r a d v a n c i n g a g e ; o c c u r s i n
p e r f o r a t i o n ; p e r i t o n i t i s ma me
y n mo r e t h a n i n w o me n
develop.
Recurrences are
c o mmo n .
P e p t i c u l c e r S o me t i me s a s s o c i a t e d w i t hO c c u r s i n a l l a g e - g r o u p s ;
H el i c obac t er py l or i
peaks at the age of 50 y
infection.
Risk factors include
nonsteroidal antii n f l a mma t o r y d r u g u s e ,
tobacco and alcohol use.
Men affected twice as

mu c h a s w o me n . S e v e r e
bleeding or perforation is
rare.
K i n g K E , W i g h t ma n J M . A b d o mi n a l p a i n . I n : M a rRx os
J Aen'
, esd .
emer genc y medi c i ne: c onc ept s and c l i ni c al, pr
5 tac
h et idc.e S t . L o u i s ,
M O : M o s b y, 2 0 0 2 : 1 8 5 1 9 4 .
TAB L E 9.1.2 Selected Life-threatening Causes of

302 / 652
tmdmss
- Abdominal Pain
TAB L E 9.1.2 Selected Life-threatening9.1 Causes
of
Abdom inal Pain
Ruptured
aortic
aneurysm
A t h e r o s c l e r o s i s , i n t i ma l
M o r e f r e q u e n t i n me n
dissection; leakage causes
shock.
Risk factors:
hypertension, diabetes
me l l i t u s , s mo k i n g , c h r o n i c
o b s t r u c t i v e p u l mo n a r y
disease, coronary artery
disease
Acute
O b s t r u c t i v e b i l i a r y s t o n e , M o r e f r e q u e n t i n me n ,
pancreatitis alcohol,
rare in childhood
h y p e r t r i g l y c e r i d e mi a ,
h y p e r c a l c e mi a .
M e s e n t e r i c O f t e n mu l t i f a c t o r i a l ,
M o s t c o mmo n i n e l d e r l y
i s c h e mi a
including transient
people with
h y p o t e n s i o n i n t h e p r e s e n ccee r e b r o v a s c u l a r d i s e a s e ,
of atherosclerosis.
congestive heart failure,
T h r o mb o s i s o c c u r s i n u p t od i a b e t e s me l l i t u s
65% of cases.
2. A t h o r o u g h g y n e c o l o g i c h i s t o r y s h o u l d b e p e r f o r me d i n a l l w o me n w i t h
a b d o mi n a l p a i n , a n d p r e g n a n c y ( i n c l u d i n g e c t o p i c p r e g n a n c y ) s h o u l d b e
c o n s i d e r e d . T h e l a s t n o r ma l me n s t r u a l p e r i o d s h o u l d b e d e t e r mi n e d .
3. S ma l l b o w e l o b s t r u c t i o n i n p a t i e n t s w i t h p r i o r a b d o mi n a l s u r g e r y s h o u l d b e
c o n s i d e r e d . A l a r mi n g s y mp t o ms g e n e r a l l y i n v o l v e e s c a l a t i n g s y mp t o ms , f e v e r,
p r o f o u n d i l l n e s s , a n d e xt r e me s o f a g e .
1. A n a l y s i s o f t h e g e n e r a l a p p e a r a n c e i s e s p e c i a l l y h e l p f u l . P a t i e n t s w i t h c o l i c k y
( h o l l o w v i s c u s o b s t r u c t i o n ) p a i n o f t e n w r i t h e a b o u t , s e e k i n g a c o mf o r t a b l e
position. Peritonitis causes patients to be still and jarring the bed or heel tap
ma y e xa c e r b a t e t h e p a i n . C h e c k c o mp l e t e v i t a l s i g n s , i d e n t i f y f e v e r, t a c h y c a r d i a
o r h y p o t e n s i o n . T h e a b d o me n s h o u l d b e i n s p e c t e d f o r d i s t e n s i o n , p u l s a t i o n s , o r
e c c h y mo s i s ( G r e y T u r n e r ' s o r C u l l e n ' s s i g n ) . I t i s n e c e s s a r y t o a u s c u l t a t e f o r t h
p r e s e n c e o f b o w e l s o u n d s o r b r u i t s s u g g e s t i v e o f a n e u r y s ms . B o w e l s o u n d s a r e
s o me t i me s a b s e n t i n a p p e n d i c i t i s .
2. P a l p a t i o n b e g i n s d i s t a l t o a r e a s o f p a i n a n d s h o u l d b e g e n t l e . G u a r d i n g a n d
r i g i d i t y s h o u l d b e n o t e d . M a s s e s a n d o r g a n o me g a l y s h o u l d b e a s s e s s e d .
M u r p h y ' s s i g n ma y h e l p i d e n t i f y g a l l b l a d d e r p a t h o l o g y. T h e i l i o p s o a s a n d
o b t u r a t o r s i g n s ma y i n d i c a t e a p p e n d i c i t i s . G e n i t a l e xa mi n a t i o n i s i mp o r t a n t t o
303 / 652
tmdmss
e xc l u d e h e r n i a a n d a r e c t a l e xa mi n a t i o n ma y h e l p i d e n t i f y a r e t r o c e c a l
appendicitis.
C. Testing
1. L a b o r a t o r y e v a l u a t iIot ni s n e c e s s a r y t o o b t a i n a s e r u m h u ma n c h o r i o n i c
gonadotropin from any patient who could potentially be pregnant. Electrolytes
c a n r u l e o u t me t a b o l i c a b n o r ma l i t i e s s u c h a s h y p e r c a l c e mi a . A c o mp l e t e b l o o d
count should be considered in infectious etiologies and for the purpose of
mo n i t o r i n g h e ma t o c r i t s t a b i l i t y a n d p l a t e l e t c o u n t . E l e v a t e d l i v e r t e s t s c a n
i d e n t i f y h e p a t o c e l l u l a r o r o b s t r u c t i v e b i l i a r y d i s e a s e . E l e v a t e d a my l a s e ma y b e
f o u n d i n p a n c r e a t i t i s , s a l i v a r y d i s e a s e , me s e n t e r i c i s c h e mi a , a n d f a l l o p i a n
p a t h o l o g y. L i p a s e i s v e r y s p e c i f i c t o t h e p a n c r e a s . U r i n a l y s i s i s u s e f u l t o
e xc l u d e u r i n a r y t r a c t i n f e c t i o n s . H e ma t u r i a i s f o u n d i n 9 0 % o f r e n a l s t o n e s .
P y u r i a c a n b e p r e s e n t w h e n a n i n f l a mma t o r y ma s s ( e . g . , a p p e n d i c i t i s ) i s c l o s e t
t h e u r i n a r y t r a 2)
c t. (
P. 1 7 7
TAB L E 9.1.3 Types of Abdom inal Pain

Vi s c e r a l
pain
Results from stretching nerve fibers surrounding an organ;

ma y b e c r a mp y o r c o l i c k y, o f t e n i n t e r mi t t e n t ; ma n y t i me s
l o c a t e d i n mi d l i n e . T h i s t y p e o f p a i n i s o f t e n i l l - d e f i n e d a n d
d i f f u s e . E xa mp l e s i n c l u d e a p p e n d i c i t i s , c h o l e c y s t i t i s , b o w e l
obstruction, and renal colic. Foregut structures often refer to
t h e e p i g a s t r i u m ( s t o ma c h , d u o d e n u m, p a n c r e a t i c - b i l i a r y
t r e e ) , a n d mi d g u t s t r u c t u r e s ( s ma l l b o w e l , a s c e n d i n g c o l o n )
r e f e r t o p e r i u mb i l i c a l a r e a . H i n d g u t s t r u c t u r e s ( d e s c e n d i n g
colon) often refer to the suprapubic area or the back.
S o ma t i c
pain
A r i s e s f r o m p a i n f i b e r s i n p a r i e t a l p e r i t o n e u m; u s u a l l y
s h a r p e r, l o c a l i ze d , a n d mo r e c o n s t a n t ; ma y d e v e l o p a f t e r
v i s c e r a l p a i n . U s u a l l y i t r e p r e s e n t s p e r i t o n e a l i n f l a mma t i o n
f r o m v a r i o u s s o u r c e s ( b l e e d i n g , c h e mi c a l i r r i t a t i o n ,
i n f e c t i o u s c a u s e s ) a n d mo s t o f t e n c a u s e s g r e a t c o n c e r n .
Referred Defined as pain felt at a distance from the diseased organ.

pain
D i a p h r a g ma t i c i r r i t a t i o n o f t e n r a d i a t e s t o s h o u l d e r s , a n d
ureteral colic often radiates to groin. Referred pain is often
l o c a l i ze d t o t h e d e v e l o p me n t a l d e r ma t o me a n d c a n b e
mi s l e a d i n g i n l o c a l i zi n g t h e s o4)u.r c e (
2. I m a g i n gP l a i n f i l m a c u t e a b d o mi n a l s e r i e s ( u p r i g h t a n d s u p i n e ) i d e n t i f y f r e e a i r
a n d a b n o r ma l g a s p a t t e r n s . P l a i n f i l ms ma y i d e n t i f y c a l c i f i c a t i o n s a s s o c i a t e d
304 / 652
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w i t h r e n a l s t o n e s ( 8 5 % ) , b i l i a r y l i t h i a s i s ( 1 5 % ) , a n d p n e u mo n i a a s a n e xt r a a b d o mi n a l c a u s e f o r p a i n . U l t r a s o u n d i s u s e f u l t o r u l e o u t e c t o p i c p r e g n a n c y,
a p p e n d i c i t i s , b i l i a r y t r a c t d i s e a s e , a b d o mi n a l a o r t i c a n e u r y s m, a n d h y d r o u r e t e r.
C o mp u t e d t o mo g r a p h y ( C T ) s c a n i s l a r g e l y r e p l a c i n g o t h e r mo d a l i t i e s , b e c a u s e
i t p r o v i d e s a q u i c k a c c u r a t e i ma g i n g mo d a l i t y t o d e t e c t a p p e n d i c i t i s , u r o l i t h i a s i s
c h o l e c y s t i t i s , d i v e r t i c u l i t i s , a n d p a n c r e a t i t i s . I n e l d e r l y p a t i e n t s w i t h a b d o mi n a l
p a i n , C T s c a n a l t e r s t h e i n i t i a l d i a g n o s i s i n 4 5 % o f c a s e s a n d a f f e c t s a d mi s s i o n
d e c i s i o n s i n 2 6 % o f c a 3)
s e.s (
I V. Diagnosis
T he differential diagnosis is usually broad and frequently reconsidered. If a chosen

t r e a t me n t mo d a l i t y i s u n s u c c e s s f u l , t h e w o r k i n g d i a g n o s i s mu s t b e q u e s t i o n e d a n d
t h e d i f f e r e n t i a l d i a g n o s i s e xp a n d e d . S e r i a l e xa mi n a t i o n a n d c l o s e f o l l o w - u p a r e
necessary if the diagnosis is in question.
P a t t e r n s a s s o c i a t e d w i t h t h e d i f f e r e n t t y p e s o f a b d o mi n a l p a i n a r e p r e s e n t e d i n
T a b l e 9 . 1 . 3M u l t i p l e c a u s e s n e c e s s i t a t e a b r o a d d i f f e r e n t i a l d i a g n o s i s . N u me r o u s
d i a g n o s t i c mo d a l i t i e s e xi s t , i n c l u d i n g p h y s i c a l e xa mi n a t i o n , l a b o r a t o r y s t u d i e s , a n d
ancillary studies.
References
1 . K i n g K E , W i g h t ma n J M . A b d o mi n a l p a i n . I n : M a rRx os
J Aen'
, esd .emer genc y
medi c i ne: c onc ept s and c l i ni c al pr
, ac
5 t ht i ceed . S t . L o u i s , M O : M o s b y,
2002:185194.
2 . K a mi n R A , N o w i c k i T A , C o u r t n e y D S , e t a l . P e a r l s a n d p i t f a l l s i n t h e
e me r g e n c y d e p a r t me n t e v a l u a t i o n o f a b d o mi nEamer
l p agi nM. ed C l i n N or t h A m
2003;21:6172.
3 . E s s e s D , B i r n b a u m A , B i j u r P, e t a l . A b i l i t y o f C T t o a l t e r d e c i s i o n ma k i n g i n
e l d e r l y p a t i e n t s w i t h a c u t e a b d o mi n aAl mp aJi nE. mer g M ed
2004;22:270272.
4 . F a l e s W D , O v e r t o n D T . A b d o mi n a l p a i n . I n : T i n t i n aEl lmer
i J Egenc
, e d .y
medi c i ne
, 4 t h e d . N e w Yo r k , N Y : M c G r a w - H i l l , 1 9 9 6 : 2 1 7 2 2 1 .
305 / 652
tmdmss
9.2 - Ascites
> T a b l e o f C o n t e n t s > 9 - G a s t r o i n t e s t i n a l P r o b l e ms > 9 . 2 - A s c i t e s
9.2
Ascites
Wi l l i a m M . L u c a s
I . Background
A s c i t e s i s a f l u i d c o l l e c t i o n i n t h e p e r i t o n e a l c a v i t y. I n a p p r o xi ma t e l y 8 5 % o f t h e
p a t i e n t s i n t h e U n i t e d S t a t e s w i t h a s c i t e s , h e p a t i c c i r r h o s i s i s1)t ,h ea nedt i o l o g y (
t h e s e c o n d mo s t c o mmo n c a u s e i s c a r c i n o ma t o s i s . T h e 5 - y e a r s u r v i v a l r a t e f o r
p a t i e n t s w i t h a s c i t e s i s 3 0 % t o2) 4. 0L%
i v(e r t r a n s p l a n t i n c r e a s e s t h e c h a n c e s o f
s u r v i v a l b y 4 02)
%.(
T h e mo s t c o mmo n c a u s e o f a s c i t e s c i r r h o s i s l e a d s t o i n c r e a s e d p o r t a l p r e s s u r e s
f o l l o w e d b y d e v e l o p me n t o f c o l l a t e r a l f l o w t h r o u g h l o w e r p r e s s u r e p a t h w a y s . P o r t a l
h y p e r t e n s i o n t r i g g e r s t h e r e l e a s e o f n i t r i c o xi d e , c a u s i n g v a s o d i l a t a t i o n a n d a n
e n l a r g e d i n t r a v a s c u l a r s p a c e . T h e b o d y a t t e mp t s t o c o r r e c t t h i s p e r c e i v e d
h y p o v o l e mi a b y t r i g g e r i n g v a s o c o n s t r i c t o r a n d a n t i n a t u r e t i c f a c t o r s t h a t l e a d t o s a l t
a n d f l u i d r e t e n t i o n , t h e r e b y i n t e r r u p t i n g t h e b a l a n c e a mo n g t h e S t a r l i n g f o r c e s
P. 1 7 9
t h a t ma i n t a i n f l u i d h e mo s t a s i s . T h e n , f l u i d s w e a t s f r o m t h e s u r f a c e o f t h e l i v e r a n
c o l l e c t s i n t h e a b d o mi n a l c a v i t y. T h e k i d n e y s e v e n t u a l l y l o s e t h e i r a b i l i t y t o c l e a r f r
w a t e r, c a u s i n g a d i l u t i o n a l h y p o n a t r e mi a a n d u l t i ma t e l y h e p a t o r e n a l s y n d r o me .
TAB L E 9.2.1 Paracentesis Fluid Findings and T heir

Significance
Characte ristic Spe cific findings
A ppear anc e
Significance
Clear
Cirrhosis
Turbid/cloudy
Infection
Opalescent
Elevated triglyceride concentration
Milky
Elevated triglyceride concentration

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9.2 - Ascites
C el l c ount
and
di f f er ent i al
Pink/bloody
T r a u ma t i c t a p , ma l i g n a n c y
Brown
Patient with jaundice, ruptured

g a l l b l a d d e r, d u o d e n a l u l c e r
P o l y mo r p h o n u c l e a r S p o n t a n e o u s b a c t e r i a l p e r i t o n i t i s
leukocytes count
>250
S er um t o as c i t es al bumi n gr adi
( s eent
r u m a l b u mi n a s c i t e s a l b u mi n ) ( g / d L )
>1.1
P o r t a l h y p e r t e n s-i coinr r h o s i s ,
a l c o h o l i c h e p a t i t i s , c o mp r e s s i o n f r o m
l a r g e l i v e r me t a s t a s e s , p o r t a l v e i n
t h r o mb o s i s , B u d d - C h i a r i S y n d r o me ,
cardiac ascites, acute fatty liver of
p r e g n a n c y, my xe d e ma , mi xe d a s c i t e s
<1.1
N o p o r t a l h y p e r t e n s- ipor ne s e n t ,
p e r i t o n e a l c a r c i n o ma t o s i s ( mo s t
c o mmo n ) , t u b e r c u l o s i s , p a n c r e a t i c o r
biliary ascites, bowel infarction or
o b s t r u c t i o n , n e p h r i t i c s y n d r o me
I I I . Evaluation
A. History
T h e h i s t o r y s h o u l d f o c u s o n a l c o h o l c o n s u mp t i o n , t r a n s f u s i o n s , h u ma n
i mmu n o d e f i c i e n c y v i r u s a n d h e p a t i t i s r i s k f a c t o r s , t a t t o o s , f a mi l y h i s t o r y o f l i v e r
d i s e a s e , o b e s i t y, d i a b e t e s , h y p e r l i p i d e mi a , a n d t u b e r c u l o s i s r i s k f a c t o r s .
D e t e c t i n g a s c i t e s b y p h y s i c a l e xa mi n a t i o n i s v a r i a b l y s e n s i t i v e a n d e xa mi n e r
d e p e n d e n t . A p p r o xi ma t e l y 1 , 5 0 0 mL o f f l u i d mu s t b e p r e s e n t t o i d e n t i f y a s c i t e s
r e l i a b l y. O f t h e s p e c i f i c p h y s i c a l e xa mi n a t i o n t e c h n i q u e s d e s c r i b e d t o d e t e c t a s c i t e s
f l a n k d u l l n e s s i s t h e mo s t r e l i a b l e p r e d i c t o r. F l u i d w a v e a n d p u d d l e s i g n s a r e o f l i t t
c l i n i c a l v a l u e . S p i d e r a n g i o ma s , p a l me r e r y t h e ma , mu s c l e w a s t i n g , l a r g e a b d o mi n a l
wall collateral veins, and jaundice are suggestive of portal hypertension.
C. Testing
F o l l o w i n g c o n f i r ma t i o n o f a s c i t e s b y u l t r a s o u n d , a l l p a t i e n t s w i t h a s c i t e s s h o u l d b e
evaluated with a diagnostic paracentesis. Other indications of paracentesis include
f e v e r, a b d o mi n a l p a i n , t e n d e r n e s s , h y p o t e n s i o n , i l e u s , r e n a l f a i l u r e , me n t a l s t a t u s
c h a n g e s , a n d t h e r a p e u t i c r e l i e f o f e xc e s s f l u i d . C o a g u l o p a t h y i s n o t a
c o n t r a i n d i c a t i o n t o t h i s p r o c e d u r e a n d r a r e l y c a u s e s c o mp l i c a t i o n s e xc e p t i n t h e
307 / 652
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9.2 - Ascites
s e t t i n g o f d i s s e mi n a t e d i n t r a v a s c u l a r c o a g u l o p a t h y. P a r a c e n t e s i s f i n d i n g s a n d t h e i r
s i g n i f i c a n c e a r e d e s c r i b eTda bi nl e 9 . 2 ,. 1w h i l e t h e i r a s s o c i a t i o n i s p r e s e n t e d i n
T a b l e 9 . 2 . 2P a r a c e n t e s i s f l u i d a n a l y s i s o f p H , l a c t a t e , c h o l e s t e r o l , a n d f i b r o n e c t i n
a r e u n h e l p f u l i n d i a g n o3)s .i sW( h e n o b t a i n i n g c u l t u r e s , 1 0 t o 2 0 c c o f p e r i t o n e a l
fluid should be collected at the bedside for best results.
I V. Diagnosis
T h e d i f f e r e n t i a l d i a g n o s i s o f a s c i t e s i s Tl iasbt el ed 9i n. 2 . 3U n c o mmo n c a u s e s
i n c l u d e t r a u ma t o l y mp h a t i c s o r u C
r ehlt eamy
r s , di,an e p h r o t i c s y n d r o me , s e r o s i t i s i n
c o n n e c t i v e t i s s u e d i s e a s e , my xe d e ma , a c q u i r e d i mmu n o d e f i c i e n c y s y n d r o me , a n d
F i t z- H u g h - C u r t i s s y n d r o me .
T h e s y mp t o ms o f a s c i t e s a r e n a u s e a , a n o r e xi a , e a r l y s a t i e t y, h e a r t b u r n , i n c r e a s e d
a b d o mi n a l g i r t h , s h o r t n e s s o f b r e a t h , a b d o mi n a l p a i n , o r t h o p n e a , w e i g h t g a i n , a n d
l e g s w e l l i n g . P h y s i c a l f i n d i n g s i n c l u d e f e v e r, e n c e p h a l o p a t h y, j a u n d i c e , j u g u l a r
v e n o u s d i s t e n s i o n , g a s t r o i n t e s t i n a l b l e e d i n g , b u l g i n g f l a n k s , a b d o mi n a l d i s t e n s i o n ,
h e p a t o me g a l y, s p l e n o me g a l y, p e n i l e a n d s c r o t a l e d e ma , o r u mb i l i c a l h e r n i a .
TAB L E 9.2.2 Clinical Association of Paracentesis

Fluid Results
Characte ristic
Association
A my l as e
E l e v a t e d i n s ma l l b o w e l o r p a n c r e a t i c i n j u r y
LDH
I n u n c o mp l i c a t e d a s c i t e s , L D H i s f e w e r t h a n h a l f t h e s e r u m
values. Peritonitis > S B P > serum L D H.
G l uc os e
M i l d l y d e p r e s s e d i n c a r c i n o ma t o s i s a n d S B P. I n b o w e l
c onc ent r at i on p e r f o r a t i o n o r l a t e S B P t h e g l u c o s e l e v e l ma y b e a s l o w a s
0.
C y t ol ogy
T h i s i s o f b e n e f i t w h e n c a r c i n o ma t o s i s i s p r e s e n t . T h i s t e s t
r e q u i r e s t h e r e l e a s e o f ma l i g n a n t c e l l s i n t o t h e a s c i t i c f l u i d ,
s e n s i t i v i t y 6 7 % o f a l l ma l i g n a n c y r e l a t e d c a s e s .
R enal panel
A s s e s s f o r h e p a t o r e n a l s y n d r o me .
L D H , l a c t a t e d e h y d r o g e n a s e ; S B P, s p o n t a n e o u s b a c t e r i a l p e r i t o n i t i s .
P. 1 8 0
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9.2 - Ascites
TAB L E 9.2.3 Differential Diagnosis of Ascites

Cause
Cirrhosis
Com m e nt
E t i o l o g y i n ~ 8 0 % o f a s c 3)
ites (
Alcoholic hepatitis
C a r c i n o ma t o s i s
T w o t h i r d s o f ma l i g n a n c y - r e l a t e d a s c i t e s
Metastases
O n e t h i r d o f ma l i g n a n c y - r e l a t e d a s c i t e s
Congestive heart
failure
History of heart failure or severe lung disease
Tuberculosis
C a u s e i n ~ 1 % o f a s c i t3)e s (
Hepatitis B and C
Leads to cirrhosis
P a n c r e a t i c d i s e a s e A s c i t e s a my l a s e i s f i v e t i me s t h e s e r u m v a l u e s
Wilson's disease
E f f e c t s r a n g e f r o m l i v e r t e s t a b n o r ma l i t i e s t o h e p a t i c
failure
H e mo c h r o ma t o s i s
Iron deposition causes cirrhosis
Constrictive
pericarditis
Leads to hepatic congestion
M i xe d a s c i t e s
~5% (cirrhosis + another etiology)
References
1 . R u n y o n B A . M a n a g e me n t o f a d u l t p a t i e n t s w i t h a s c i t e s d u e t o c i r r h o s i s .
A A S L L D p r a c t i c e g u i d e l iH
n eepat
s . ol ogy2 0 0 4 ; 3 9 : 1 1 6 .
2 . G i n e s P, C a r d e n a s A , A r r o y o V, e t a l . C u r r e n t c o n c e p t s : ma n a g e me n t o f
c i r r h o s i s a n d a s c i tNe sE. ngl J M ed2 0 0 4 ; 3 5 0 : 1 6 4 6 1 6 5 4 .
3 . H a u b r i c h W S , S c h a f f n e r F, B r e k J B
, oc
e t kaus
l . gas t r oent er ol. ogy
5th ed.
P h i l a d e l p h i a , PA : W B S a u n d e r s , 1 9 9 4 : 2 0 0 9 .
309 / 652
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9.2 - Ascites
310 / 652
tmdmss
9.3 - Constipation
> T a b l e o f C o n t e n t s > 9 - G a s t r o i n t e s t i n a l P r o b l e ms > 9 . 3 - C o n s t i p a t i o n
9.3
Constipation
J o s e p h T. L a V a n
C o n s t i p a t i o n i s a s y mp t o m, a n d n o t a d i a g n o s i s . M o s t c a s e s o f c o n s t i p a t i o n a r e
functional or idiopathic in nature, so evaluation is targeted at identifying the few
c a u s e s t h a t c a n b e c o r r e c t e d . O n c e t h e s e c a u s e s h a v e b e e n r u l e d o u t , t r e a t me n t i s
s y mp t o ma t i c .
I . Background
T h e me a n i n g o f c o n s t i p a t i o n v a r i e s f r o m p a t i e n t t o p a t i e n t a n d p r o v i d e r t o p r o v i d e
I n 1 9 9 9 , a n i n t e r n a t i o n a l p a n e l d e v e l o p e d a c o n s e n s u s d e f i n i t i o n f o 1)
r c. o n s t i p a t i o n (
T h e R o me - I I c r i t e r i a (Tsaebel e 9 . 3 ). 1h e l p e d s t a n d a r d i ze r e s e a r c h a n d o f f e r a n
objective path to clinical diagnosis.
T h e c o l o n a n d r e c t u m a r e r e s p o n s i b l e f o r b o t h d i g e s t i v e a n d e xc r e t i v e f u n c t i o n s ,
i n c l u d i n g c o n t i n u e d mi xi n g o f t h e o u t p u t o f t h e i l e u m, f u r t h e r p r o c e s s i n g o f
undigested carbohydrate (including digestion and absorption), absorption of water t
c r e a t e s e mi s o l i d o r s o l i d s t o o l , a n d u l t i ma t e l y e v a c u a t i o n o f s t o o l f r o m t h e b o d y.
T h e s e p r o c e s s e s a r e c o o r d i n a t e d t h r o u g h a v a r i e t y o f n e u r o t r a n s mi t t e r s , a
p a r a s y mp a t h e t i c n e u r a l p l e xu s a n d a v a r i e t y o f o t h e r v o l u n t a r y a n d i n v o l u n t a r y
me c h a n i s ms .
A. Etiology
C h a n g e s i n t h e t e xt u r e o f t h e s t o o l , t h e p e r i s t a l t i c f u n c t i o n o r i n t e r n a l d i a me t e r o f t
c o l o n o r t h e e xp u l s i v e f u n c t i o n o f t h e r e c t u m a n d p e l v i c f l o o r c a n l e a d t o c o n s t i p a t i
(2,3) . T a b l e 9 . 3 . l2i s t s s o me o f t h e mo r e c o mmo n c a u s e s .
P. 1 8 1
TAB L E 9.3.1 Rom e-I I Criteria for the Diagnosis of

Functional Constipation
Adults
311 / 652
tmdmss
9.3 - Constipation
T w o o r mo r e o f t h e f o l l o w i n g s y mp t o ms a r e p r e s e n t a t l e a s t 1 2 w k i n 1 2
mo ( n o t r e q u i r e d t o b e c o n s e c u t i v e ) :
S t r a i n i n g d u r i n g mo r e t h a n o n e i n f o u r b o w e l mo v e me n t s
L u mp y o r h a r d s t o o l s d u r i n g mo r e t h a n o n e i n f o u r b o w e l mo v e me n t s
S e n s a t i o n o f i n c o mp l e t e d e f e c a t i o n i n mo r e t h a n o n e i n f o u r b o w e l
mo v e me n t s
S e n s a t i o n o f b l o c k a g e o r o b s t r u c t i o n i n a n u s o r r e c t u m d u r i n g mo r e
t h a n o n e i n f o u r b o w e l mo v e me n t s
U s e o f ma n u a l ma n e u v e r s ( e . g . , d i g i t a l e xp r e s s i o n o f s t o o l , p e l v i c
f l o o r s u p p o r t ) w i t h mo r e t h a n o n e i n f o u r b o w e l mo v e me n t s
F e w e r t h a n t h r e e b o w e l mo v e me n t s p e r w e e k
Absence of diarrhea or loose stools
S y mp t o ms d o n o t me e t t h e c r i t e r i a f o r i r r i t a b l e b o w e l s y n d r o me
I nfants and y oung childre n
T w o o r mo r e w e e k s o f o n e o f t h e f o l l o w i n g s y mp t o ms :
H a r d s t o o l s ( p e b b l e l i k e = s c y b a l o u s ) i n mo s t b o w e l mo v e me n t s
L E S S T H AN three F IR M stools per week
S y mp t o ms n o t e xp l a i n e d b y a me t a b o l i c , e n d o c r i n e , o r a n a t o mi c c a u s e
A d a p t e d w i t h p e r mi s s i o n f r o m T h o mp s o n W G , L o n g s t r e t h G F, D r o s s ma n D A ,
e t a l . F u n c t i o n a l b o w e l d i s o r d e r s a n d f u n c t i o n a l a b d o mi n a l p a i n ( R o me I I : a
mu l t i n a t i o n a l c o n s e n s u s d o c u me n t o n f u n c t i o n a l g a s t r o i n t e s t i n a l d i s o r d e r s ) .
G ut 1 9 9 9 ; 4 5 ( s u p p l 2 ) : 11 4 3 11 4 7 .
TAB L E 9.3.2 Causes of Constipation (Rated by the

Likelihood of Being a Cause of Constipation)
Functional
(com m on)
Me dications (le ss com m on)
L ow-fiber diet
Antacids
Clonidine
S edentary lifestyle Anticholinergics
Diuretics
Dehydration
Antidepressants
S i n e me t
S l o w t r a n s i t t i me
C a l c i u m c h a n n e l b l o c k e r sN a r c o t i c s
Outlet delay
C h o l e s t y r a mi n e
S y mp a t h o mi me t i c s
Irritable bowel
Nonsteroidal antiPsychotropics
s y n d r o me
i n f l a mma t o r y d r u g s
I n f l a mma t o r y b o w e l
disease
E xc e s s i v e mi l k i n t a k e
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9.3 - Constipation
Stool withholding
Structural (le ss
com m on)
Ne uroge nic (rare )
Endocrine /m e tabolic
(rare )
Anal fissures
H e mo r r h o i d s
Colonic strictures
Diverticulitis
I s c h e mi a
Radiation colitis
A d e n o c a r c i n o ma
I mp e r f o r a t e a n u s
P e l v i c ma s s e s
Cerebrovascular events
Multiple sclerosis
H irschsprung's disease
S p i n a l c o r d t u mo r s o r
a b n o r ma l i t i e s
Cerebral palsy
Chagas disease
Botulism
D o w n s y n d r o me
P r u n e - b e l l y s y n d r o me
D i a b e t e s me l l i t u s
H y p e r c a l c e mi a
Hyperparathyroidism
H y p o k a l e mi a
Hypothyroidism
U r e mi a
Celiac disease
Cystic fibrosis
Pregnancy
Psy choge nic (le ss Conne ctiv e tissue (rare )

com m on)
A n xi e t y
Depression
S o ma t i za t i o n
A my l o i d o s i s
S c l e r o d e r ma
S y s t e mi c l u p u s
P. 1 8 2
TAB L E 9.3.3 History and Physical Exam ination

Findings
Com pone nt
Finding
Condition
sugge ste d
History
Age at onset
Childhood onset
Congenital
causes
Duration
Acute onset
Correctable
causes
Longer duration
Functional
cause
T h e mo s t t r o u b l i n g s y mp t o m S t r a i n i n g
Pelvic floor
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9.3 - Constipation
dysfunction
N e e d f o r ma n u a l
ma n e u v e r s f o r
evacuation
C r a mp i n g / b l o a t i n g
between bowel
mo v e me n t s
Medication history
Irritable
bowel
s y n d r o me
S e e t h e l i s t o f c o mmo n o f f e n d e r s i n
Table 9.3.2
Phy sical e x am ination
D i g i t a l r e c t a l e xa mi n a t i o n
Tender puborectalis
Puborectalis
spasm
I n a b i l i t y t o e xp e l r e c t a l P e l v i c f l o o r
finger
dysfunction
R e c t a l ma s s , a n a l
stricture
Obstruction
Anal fissure
Cause or
effect?
I mp a c t e d s t o o l
Slow transit
constipation
S i mu l a t e d d e f e c a t i o n
L a xi t y o f a n a l v e r g e
Neurogenic
causes
Perineal function during

s i mu l a t e d e xp u l s i o n a n d
retention
Low activity
Pelvic floor
dysfunction
Va g i n a l e xa mi n a t i o n ( w o me n ) O b s e r v e f o r r e c t o c e l e
Pelvic
r e l a xa t i o n
N e u r o l o g i c e xa mi n a t i o n
Other focal neurologic Neurogenic

deficits
causes
A b d o mi n a l e xa mi n a t i o n
Masses, scars
Causes of
obstruction
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9.3 - Constipation
B. Epidemiology
Va r i o u s s t u d i e s h a v e f o u n d p r e v a l e n c e r a t e s r a n g i n g b e t w e e n 4)
2%
. and 28%(
C o n s t i p a t i o n i s t h e r e a s o n f o r 2 . 5 mi l l i o n p h y s i c i a n v i s i t s p e r y e a r. P r e v a l e n c e i s
h i g h e r i n w o me n a n d t h e e l d e r l y. R i s k f a c t o r s i n c l u d e i n a c t i v i t y, l o w - c a l o r i e d i e t s ,
h i g h e r n u mb e r o f me d i c a t i o n s ( i n d e p e n d e n t o f s i d e e f f e c t p r o f i l e s ) a n d l o w e r
s o c i o e c o n o mi c s t a t 5)
u s. (L o w - f i b e r d i e t s d o n o t s e e m t o b e a r i s k f a c t o r.
I I I . Evaluation
A. History and physical examination
T he initial focus of the evaluation should be on distinguishing idiopathic constipatio

f r o m s e c o n d a r y c a u sTeasb. l e 9 . 3 . p3 r e s e n t s s i g n i f i c a n t f i n d i n g s i n t h e h i s t o r y a n d
p h y s i c a l e xa mi n a t i o n t h a t a i d i n d i s t i n g u i s h i n g i d i o p a t h i c o r f u n c t i o n a l c a u s e s f r o m
s e c o n d a r y c a u s e s . M o s t s e c o n d a r y c a u s e s c a n b e i d e n t i f i e d b y h i s t o r y, p h y s i c a l
e xa mi n a t i o n , a n d a l i mi t e d l a b o r a t o r y e v a l u a t i o n .
B. Testing
I n i t i a l l a b o r a t o r y t e s t s s h o u l d i n c l u d e s e r u m c h e mi s t r i e s ( i n c l u d i n g c a l c i u m a n d
g l u c o s e ) , c o mp l e t e b l o o d c o u n t a n d t h y r o i d f u n c t i o n t e s t s . A d d i t i o n a l l a b o r a t o r y t e s
s u c h a s s e r u m p r o t e i n e l e c t r o p h o r e s i s , u r i n e p o r p h y r i n s , o r p a r a t h y r o i d h o r mo n e a r
i n d i c a t e d o n l y i f t h e i n i t i a l e v a l u a t i o n i s a b n o r ma l . F l e xi b l e s i g mo i d o s c o p y,
c o l o n o s c o p y, o r b a r i u m e n e ma a r e i n d i c a t e d w h e n o b s t r u c t i v e s y mp t o ms a r e p r e s e n t
or when the patient is older than 50 years.
C. Genetics
A l t h o u g h s o me c a u s e s o f c o n s t i p a t i o n h a v e g e n e t i c c o mp o n e n t s , a d e t a i l e d
d i s c u s s i o n i s o u t s i d e t h e s c o p e o f t h i s r e v i e w.
I V. Diagnosis
T h e d i f f e r e n t i a l d i a g n o s i s o f f u n c t i o n a l c o n s t i p a t i o n i s pTraebsleen 9t e. 3d. 1i n
P. 1 8 3
A l t h o u g h p h y s i c i a n s s e e m t o f o c u s o n a d e c r e a s e d f r e q u e n c y o f b o w e l mo v e me n t s ,
p a t i e n t s c o mp l a i n o f s t r a i n i n g , i n f r e q u e n c y, h a r d s t o o l s , t h e n e e d t o d e f e c a t e w i t h o
b e i n g a b l e t o , a n d t h e p e r s i s t e n t u r g e t o d e f e c a t e i mme d i a t e l y a f t e r a b o w e l
mo v e me n t .
References
1 . T h o mp s o n W G , L o n g s t r e t h G F, D r o s s ma n D A , e t a l . F u n c t i o n a l b o w e l
d i s o r d e r s a n d f u n c t i o n a l a b d o mi n a l p a i n . ( R o me I I : a mu l t i n a t i o n a l c o n s e n s u s
d o c u me n t o n f u n c t i o n a l g a s t r o i n t e s t i n a l d i sGout
r d1e9r s9)9. ; 4 5 ( s u p p l 2 ) : 11 4 3
11 4 7 .
315 / 652
tmdmss
9.3 - Constipation
2 . A r c e D A , E r mo c i l l a C A , C o s t a H . E v a l u a t i o n o f c o nAsm
t i pFaam
tion.
P hy s i c i an2 0 0 2 ; 6 5 : 2 2 8 3 2 2 9 0 .
3 . F a i g e l D O . A c l i n i c a l a p p r o a c h t o c o n s tCi pl ai nt i oCnor
. ner s t one
2 0 0 2 ; ( 4 ) : 11
21.
4 . S a t i s h - R a o S C . C o n s t i p a t i o n : e v a l u a t i o n a n d t rGeas
a t tme
r oent
n t . er ol C l i n
N or t h A m2 0 0 3 ; 3 2 : 6 5 9 6 8 3 .
5. AG A Technical R eview: C onstipation. U p To D ate 2005.
h t t p : / / w w w. u p t o d a t e o n l i n e . c o m/ a p p l i c a t i o n / t o p i c . a s p ?
file= gihepgui/26493&type= A&selected T itle= 7~ 62> .25, accessed on M ay-2005.
316 / 652
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9.4 - Diarrhea
> T a b l e o f C o n t e n t s > 9 - G a s t r o i n t e s t i n a l P r o b l e ms > 9 . 4 - D i a r r h e a
9.4
Diarrhea
Dav id C. Krulak
I . Background
T h e c l i n i c a l l y mo s t u s e f u l d e f i n i t i o n o f d i a r r h e a i s a c t u a l l y t h e mo s t v a g u e a n d
p a t i e n t - c e n t r i c , n a me l y, f r e q u e n t , l o o s e s t o o l s . T h e s e s t o o l s a r e o f t e n w a t e r y,
e xc e s s i v e , a n d c a n b e u n c o n t r o l l e d . D i a r r h e a c a n b e a s s o c i a t e d w i t h f e v e r,
a b d o mi n a l c r a mp i n g , w e i g h t l o s s , p a i n f u l d e f e c a t i o n , a n d b l o o d y s t o o l s . A d d i t i o n a l l y
t h e d u r a t i o n o f d i a r r h e a i s o f s i g n i f i c a n t c l i n i c a l i mp o r t a n c e . D i a r r h e a o f < 1 4 d a y s
d u r a t i o n i s t e r me
acdut e, w h e r e a s d i a r r h e a t h a t e xt e n d s b e y o n d 3 0 d a y s i s t e r me d
c hr oni .c
A. Etiology
1. A c u t e d i a r r h e a c a n b e i n f e c t i o u s o r n o n i n f e c t i o u s . I n f e c t i o u s o r g a n i s ms a f f e c t
e i t h e r t h e c o l o n ( o f t e n a f e b r i l e , b l o o d y d i a r r h e a ) o r s ma l l i n t e s t i n e ( t y p i c a l l y
l a r g e v o l u me , w a t e r y s t o o l s ) .
2. C h r o n i c d i a r r h e a i s c a t e g o r i ze d a s o n e o f f o u r t y p e s : f a t t y, i n f l a mma t o r y,
s e c r e t o r y, o r o s mo t i c .
a. F a t t y d i a r r h e a c o n t a i n s e xc e s s s t o o l f a t , s h o w n b y d i r e c t me a s u r e me n t o r
Sudan staining.
b. I n f l a mma t o r y d i a r r h e a d e mo n s t r a t e s mu c u s , b l o o d , a n d f e c a l l e u k o c y t e s i n
the stool.
c. S e c r e t o r y d i a r r h e a i s w a t e r y a n d d o e s n o t h a v e a s i g n i f i c a n t o s mo t i c g a p
( < 5 0 mO s m/ k g ) .
d. O s mo t i c d i a r r h e a i s a l s o l i q u i d i n n a t u r e b u t i s c h a r a c t e r i ze d b y a n o s mo t i c
g a p ( > 1 2 5 mO s m/ k g ) .
B. Epidemiology
1. T h e e p i d e mi o l o g y o f d i a r r h e a i n t h e U n i t e d S t a t e s h a s n o t b e e n w e l l s t u d i e d . I t
is well established that acute diarrhea is the leading cause of childhood death
w o r l d w i d e . I n t h e U S p o p u l a t i o n , v i r u s e s a r e l i k e l y t h e mo s t c o mmo n c a u s e , w i t h
317 / 652
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9.4 - Diarrhea
b a c t e r i a l i n f e c t i o n p r o d u c i n g mo s t o f t h e s e v e r e c a s e s . I n t h e U n i t e d S t a t e s ,
acute diarrhea rarely requires a significant diagnostic evaluation and usually no
mo r e t h a n s y mp t o ma t i c o r a l r e h y d r a t i o n a n d r e a s s u r a n c e . H o w e v e r, i n s e l e c t e d
clinical
P. 1 8 4
s e t t i n g s ( e . g . , i mp o v e r i s h e d o r i mmu n o c o mp r o mi s e d p a t i e n t s ) , t h i s a i l me n t c a n
be life threatening.
2. C h r o n i c d i a r r h e a i s a w o r l d w i d e c h i l d h o o d p r o b l e m, w i t h i n f e c t i o n b e i n g t h e mo s
c o mmo n c a u s e . I n d e v e l o p e d c o u n t r i e s , c h r o n i c d i a r r h e a i s mo s t f r e q u e n t l y
b e c a u s e o f i r r i t a b l e b o w e l s y n d r o me , i n f l a mma t o r y b o w e l d i s e a s e , a n d
ma l a b s o r p t i o n s y n d r o me s .
I I I . Evaluation
M o s t p a t i e n t s w i t h a c u t e d i a r r h e a e xp e r i e n c e a mi l d , s e l f - l i mi t e d i l l n e s s a n d n o
l a b o r a t o r y t e s t i n g i s n e c e s s a r y. T h e c h a l l e n g e o f t h e i n i t i a l e v a l u a t i o n i s t o
d i s t i n g u i s h t h e s e p a t i e n t s f r o m t h o s e w i t h mo r e s e r i o u s d i s o r d e r s . R e d f l a g s
a s s o c i a t e d w i t h s e v e r e i l l n e s s i n c l u d e h y p o v o l e mi a ; b l o o d y d i a r r h e a ; f e v e r ; mo r e
t h a n f i v e u n f o r me d s t o o l s i n 2 4 h o u r s ; s e v e r e a b d o mi n a l p a i n ; r e c e n t a n t i b i o t i c u s e
o r h o s p i t a l i za t i o n ; o r d i a r r h e a i n a n i n f a n t , g e r i a t r i c , o r i mmu n o c o mp r o mi s e d p a t i e n t
(1) .
A. History
Evaluation of acute diarrhea begins with a history regarding occupation, travel,

s e xu a l p r a c t i c e s , p e t s , a n d h o b b i e s t h a t c o u l d s i g n a l e xp o s u r e t o p o t e n t i a l
p a t h o g e n s . O t h e r k e y p o i n t s i n c l u d e i n q u i r i e s a b o u t r e c e n t f e v e r s , me d i c a t i o n u s e ,
d i e t a r y h i s t o r y, a n d a c o mp l e t e p a s t me d i c a l h i s t o r y ( t o d e t e r mi n e r i s k o f n o s o c o mi a
i n f e c t i o n o r i mmu n o c o mp r o mi s e d s t a t u s ) . A c o mp l a i n t o f c h r o n i c d i a r r h e a r e q u i r e s a
d e t a i l e d h i s t o r y t h a t s h o u l d t h o r o u g h l y e xp l o r e t h e o n s e t , d u r a t i o n , p a t t e r n , a n d
characteristics of the stools. Major findings include significant weight loss, diarrhe
f o r l o n g e r t h a n o n e y e a r, n o c t u r n a l d i a r r h e a , a n d s t r a i n i n g w i t h s t o o l . O t h e r
q u e s t i o n s s h o u l d f o c u s o n s o c i a l a n d e p i d e mi o l o g i c h i s t o r y ( t r a v e l , f o o d s , s e xu a l
p r a c t i c e s , l i v i n g c o n d i t i o n s , w a t e r s o u r c e s ) , f a mi l y h i s t o r y, a s s o c i a t e d s y mp t o ms
( f e v e r, f e c a l i n c o n t i n e n c e , a b d o mi n a l p a i n ) , a n d a g g r a v a t i n g o r p a l l i a t i v e
P. 1 8 5
f a c t o r s ( d i e t , s t r e s s , me d i c a t i o n ) . A d e t a i l e d me d i c a l h i s t o r y e v a l u a t e s f o r i a t r o g e n i
c a u s e s ( me d i c a t i o n , s u r g e r y ) , i mmu n o c o mp r o mi s e d s t a t e s , a n d p o s s i b l e p s y c h i a t r i c
p a t h o l o g y. A t h o r o u g h r e v i e w o f s y s t e ms p r o v i d e s e v i d e n c e o f s y s t e mi c p r o c e s s e s
t h a t c a n c a u s e d i a r r h e a s u c h a s d i a b e t e s me l l i t u s , h y p e r t h y r o i d i s m, r h e u ma t o l o g i c
d i s e a s e , a n d t u mo r s . ( T h e p h y s i c a l e xa mi n a t i o n i n t h e s e c a s e s i s u s u a l l y n o r ma l ;
h o w e v e r, a n e v a l u a t i o n o f v o l u me a n d n u t r i t i o n a l s t a t u s i s e s s e n t i a l . )
318 / 652
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9.4 - Diarrhea
F I G U R E 9 . 4 . 1L.a b o r a t o r y e v a l u a t i o n o f a c u t e d i a r r h e a . ( A d a T
p thei de l ma
f r o nm
N M , G u e r r a n t R L . C l i n i c a l p r a c t i c e . A c u t e i n f e c t i o u sN dEi angl
r r h Je aM
. ed
2 0 0 4 ; 3 5 0 : 3)8 .
E xa mi n a t i o n s h o u l d f o c u s o n s i g n s o f h y p o v o l e mi a ( e . g . , d r y mu c o u s me mb r a n e s ,
orthostatic hypotension). Observation for fever or peritoneal signs can indicate an
i n v a s i v e s o u r c2)
e .( A b d o mi n a l ( ma s s e s , a s c i t e s , l i v e r c h a n g e s ) a n d a n o r e c t a l
f i n d i n g s ( l o s s o f t o n e , p r e s e n c e o f d i s e a s e ) , l y mp h a d e n o p a t h y, mo u t h u l c e r s , s k i n
c h a n g e s , o r t h y r o i d ma s s e s c a n p r o v i d e d i a g n o s3)
t i .c c l u e s (
C. Testing
S e e F i g u r e s 9 . 4 .a1n d9 . 4 . 2f o r e v a l u a t i o n o f a c u t e a n d c h r o n i c d i a r r h e a .
319 / 652
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9.4 - Diarrhea
F I G U R E 9 . 4 . 2L.a b o r a t o r y e v a l u a t i o n o f c h r o n i c d i a r r h e a . A C T H ,
a d r e n o c o r t i c o t r o p i c h o r mo n e ; C T , c o mp u t e d t o mo g r a p h y ; O & P, o v a a n d
p a r a s i t e s ; S P E P, s e r u m p r o t e i n e l e c t r o p h o r e s i s ; T S H , t h y r o i d - s t i mu l a t i n g
h o r mo n e . ( A d a p t e d f rFoi m
ne K D, Schiller L R. AG A technical review on the
e v a l u a t i o n a n d ma n a g e me n t o f c h r o n i c d G
i aas
r r ht reoent
a . er ol ogy
1 9 9 9 ; 11 6 : 1 4 6) 4 .
P. 1 8 6
I V. Diagnosis
S e e T a b l e 9 . 4 . f1o r a d i f f e r e n t i a l d i a g n o s i s o f d i a r r h e a .
1. A c u t e d i a r r h e a f e a t u r e s a f e w w e l l - d e s c r i b e d p a t t e r n s . T r a v e l e r ' s d i a r r h e a
c o mmo n l y b e g i n s 3 t o 1 0 d a y s a f t e r a r r i v a l i n a f o r e i g n l o c a t i o n w h e r e t h e
p a t i e n t i s e xp o s e d t o f o o d s o r w a t e r c o n t a mi n a t e d w i t h d i a r r h e a g e n i c
E s c her i c hi a c ol i , S al monel
, o rlCaampy l obac t ,era mo n g o t h e r s . D i a r r h e a t h a t
d e v e l o p s w i t h i n 6 h o u r s o f f o o d i n g e s t i o n i s l i k e l y b e c a u s e o f a p r e f o r me d
b a c t e r i a l t o xi n . S y mp t o ms t h a t b e g i n a f t e r mo r e t h a n 8 h o u r s s u g g e s t a b a c t e r i a
or viral infection. A febrile diarrhea is suggestive of invasive bacteria, enteric
320 / 652
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9.4 - Diarrhea
v i r u s e s , o r a c y t o t o xi c o r g a n i s m. I f d i a r r h e a o c c u r s i n t h e s e t t i n g o f a r e c e n t
c o u r s e o f a n t i b i o t i c t h e rCalpos
y, t r i di um di f f i ctiol xi
e n should be considered.
2. C h r o n i c d i a r r h e a h a s f e w e r r e c o g n i za b l e p a t t e r n s s i mp l y b e c a u s e o f t h e my r i a d
p o s s i b l e u n d e r l y i n g e t i o l o g i e s . H o w e v e r, c r a mp y a b d o mi n a l p a i n w i t h
P. 1 8 7
b o w e l h a b i t s v a r y i n g b e t w e e n d i a r r h e a a n d c o n s t i p a t i o n i s c o mmo n i n i r r i t a b l e
b o w e l s y n d r o me . D i a r r h e a w i t h a b d o mi n a l p a i n , w e i g h t l o s s , a n d f e v e r a r e
h a l l ma r k s o f i n f l a mma t o r y b o w e l d i s e a s e . F i n a l l y, ma l a b s o r p t i o n c l a s s i c a l l y
p r e s e n t s w i t h w e i g h t l o s s a n d v o l u mi n o u s , f a t t y, ma l o d o r o u s s t o o l s .
TAB L E 9.4.1 Differential Diagnosis of Acute and

Chronic Diarrhea
Acute diarrhe a
I nfe ctious
B a c t e r i aS(al monel l a, C ampy l obac t er, S hi gel l a, E s c her)i c hi a c ol i
Vi r u s ( r o t a v i r u s , n o r o v i r u s )
P r o t o zo aC(r y pt os por i di um, G i ar di a, E nt amoeba hi s) t ol y t i c a
Noninfe ctious
Food intolerance
Medications
Carcinoid
T hyroid disease
Chronic diarrhe a
Fatty
I n t e s t i n a l ma l a b s o r p t i o n
Maldigestion
I nflam m atory
Infection
I s c h e mi a
Neoplasia
321 / 652
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9.4 - Diarrhea
Se cre tory
Medications
Motility disorder
Neoplasia
T o xi n
Osm otic
C a r b o h y d r a t e ma l a b s o r p t i o n
I n g e s t i o n o f ma g n e s i u m, s u l f a t e s , o r p h o s p h a t e s
References
1 . T h i e l ma n N M , G u e r r a n t R L . C l i n i c a l p r a c t i c e . A c u t e i n f e c t i oNu s d i a r r h e a .
E ngl J M ed2 0 0 4 ; 3 5 0 : 3 8 .
2. DuPont H L. Guidelines on acute infectious diarrhea in adults. T he practice
p a r a me t e r s c o mmi t t e e o f t h e A me r i c a n c o l l e g e o f g a s t r oAe m
n t eJr o l o g y.
G as t r oent er ol
1997;92:1962.
3 . S c h i l l e r L R . C h r o n i c d i a r rGhas
e at.r oent er ol ogy
2004;127:287.
4 . G u e r r a n t R L , Va n G i l d e r T , S t e i n e r T S , e t a l . P r a c t i c e g u i d e l i n e s f o r t h e
ma n a g e me n t o f i n f e c t i o u s d i a rCr hl iena .I nf ec t D 2i s0 0 1 ; 3 2 : 3 3 1 .
5. Fine K D, Schiller L R. AG A technical review on the evaluation and
ma n a g e me n t o f c h r o n i c d i a r rGhas
e at.r oent er ol ogy
1 9 9 9 ; 11 6 : 1 4 6 4 .
322 / 652
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9.5 - Dysphagia
> T a b l e o f C o n t e n t s > 9 - G a s t r o i n t e s t i n a l P r o b l e ms > 9 . 5 - D y s p h a g i a
9.5
Dysphagia
Michae l R. Spie ke r
I . Background
D y s p h a g i a ( d i f f i c u l t y i n s w a l l o w i n g ) i s a c o mmo n c o mp l a i n t i n p r i ma r y c a r e p r a c t i c e
especially in aging persons. Up to 10% of adults older than 60 years, 25% of
h o s p i t a l i ze d p a t i e n t s , a n d 3 0 % t o 4 0 % o f n u r s i n g h o me p a t i e n t s e xp e r i e n c e
s w a l l o w i n g p r o b l e ms .
O r g a n i c s w a l l o w i n g p r o b l e ms o c c u r a t t w o p r i ma r y s i t e s : a t t h e i n i t i a t i o n o f t h e
swallow reflex in the oropharynx or with propulsion of food through the esophagus.
Dysfunctional transfer of the food past the upper esophageal sphincter into the
e s o p h a g u s c a u s e s o r o p h a r y n g e a l d y s p h a g i a s y mp t o ms , a n d d i s o r d e r e d p e r i s t a l s i s o
conditions that obstruct the flow of a food bolus through the esophagus cause
e s o p h a g e a l d y s p h a g i a s y mp t o ms . M e c h a n i c a l o b s t r u c t i o n o r n e u r o mu s c u l a r mo t i l i t y
d i s o r d e r s o c c u r i n b o t h t h e o r o p h a r y n x a n d t h e e s o p h a g u s . C o mmo n c a u s e s o f
o r o p h a r y n g e a l a n d e s o p h a g e a l d y s p h a g i a a r e Tlai sbtleed9i.n5 (1)
.1 .
I I I . Evaluation
C h o o s i n g t h e b e s t s t u d y d e p e n d s o n ma n y f a c t o r s i n c l u d i n g a g e , a c u i t y o f o n s e t ,
c o mo r b i d i t i e s , a n d a v a i l a b i l i t y o f t e s t i n g mo d a l i t i e s i n t h e c o mmu n i t y. T h e A g e n c y f o
H e a l t h C a r e P o l i c y a n d R e s e a r c h ( A H C P R ) h a s p u b l i s h e d a l i mi t e d r e v i e w t h a t
e v a l u a t e s d i a g n o s t i c a n d t h e r a p e u t i c mo d a l i t i e s , p r i ma r i l y f o c u s i n g o n s t r o k e p a t i e n
w i t h d y s p h a g i 2)
a .(
A. History
A careful history identifies 80% to 85% of causes by differentiating whether the

dysphagia is oropharyngeal or esophageal in location and whether it is obstructive
n e u r o mu s c u l a r i n n a t u r e . T h e o n s e t , s e v e r i t y, a n d d u r a t i o n o f d y s p h a g i a c o mb i n e d
w i t h q u e s t i o n s a b o u t a s s o c i a t e d s y mp t o ms c a n h e l p n a r r o w t h e d i f f e r e n t i a l d i a g n o s i
( s e e T a b l e 9 . 5 ). (3)
2 . A l c o h o l a n d t o b a c c o c o n s u mp t i o n h i s t o r i e s p r o v i d e i mp o r t a n t
i n f o r ma t i o n . M e d i c a t i o n s c a n c a u s e d i r e c t e s o p h a g e a l i n j u r y, d y s mo t i l i t y, d e c r e a s e d
l o w e r e s o p h a g e a l s p h i n c t e r t o n e w i t h r e f l u x, o r xe r o s t o mi a w i t h s u b s e q u e n t
d y s p h a g i a ( s eTea b l e 9 . 5 ). (4)
3 .
323 / 652
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9.5 - Dysphagia
E xa mi n a t i o n , i n c l u d i n g g a g r e f l e x t e s t i n g , t y p i c a l l y p r o v i d e s l i mi t e d i n f o r ma t i o n
u n l e s s t h e p a t i e n t h a s e xp e r i e n c e d a n o b v i o u s c e r e b r o v a s c u l a r a c c i d e n t . G a g
r e f l e xe s a r e a b s e n t i n u p t o 1 3 % o f n o n d y s p h a g i c , n o r ma l p a t i e n t s . H o w e v e r, t w o
c l i n i c a l i t e ms c o mb i n e d ( c o u g h o n s w a l l o w i n g a n d p o s i t i v e t h r e e - o u n c e w a t e r t e s t
provide a useful and potentially cost-effective screening tool in aspiration risk
e v a l u a t i o n . P a t i e n t s s u b j e c t i v e l y l o c a l i ze e s o p h a g e a l s t r i c t u r e s w i t h i n 4 c m
a p p r o xi ma t e l y 7 5 % o f t h e t i me .
P. 1 8 8
TAB L E 9.5.1 Differential Diagnosis of Dysphagia

Orophary nge al dy sphagia
Ne urom uscular dise ase (dise ase s of the ce ntral ne rv ous sy ste m )
Cerebrovascular accident
B r a i n s t e m t u mo r s
Degenerative diseases
A my o t r o p h i c l a t e r a l s c l e r o s i s
Multiple sclerosis
H untington's disease
Postinfectious
P o l i o my e l i t i s
Syphilis
Peripheral nervous system
Peripheral neuropathy
Motor end-plate dysfunction
Myasthenia gravis
S k e l e t a l mu s c l e d i s e a s e ( my o p a t h i e s )
P o l y my o s i t i s
D e r ma t o my o s i t i s
M u s c u l a r d y s t r o p h y ( my o t o n i c d y s t r o p h y, o c u l o p h a r y n g e a l d y s t r o p h y )
Cricopharyngeal (upper esophageal sphincter), achalasia
Obstructiv e le sions
T u mo r s
I n f l a mma t o r y ma s s e s
T r a u ma / s u r g i c a l r e s e c t i o n
Z enker's diverticulum
Esophageal webs
E xt r i n s i c s t r u c t u r a l l e s i o n s
324 / 652
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9.5 - Dysphagia
A n t e r i o r me d i a s t i n a l ma s s e s
Cervical spondylosis
Esophage al dy sphagia
Ne urom uscular disorde rs
Achalasia
S p a s t i c mo t o r d i s o r d e r s
Diffuse esophageal spasm
Hypertensive lower esophageal sphincter
Nutcracker esophagus
Obstructiv e le sions
Intrinsic structural lesions
T u mo r s
Strictures
Peptic
Radiation-induced
C h e mi c a l - i n d u c e d
Medication-induced
L o w e r e s o p h a g e a l r i n g s ( S c h a t zk i ' s r i n g )
Esophageal webs
Foreign bodies
Ex trinsic structural le sions
Va s c u l a r c o mp r e s s i o n
Enlarged aorta or left atrium
Aberrant vessels
M e d i a s t i n a l ma s s e s
L y mp h a d e n o p a t h y
Substernal thyroid
F r o m C a s t e l l D O . A p p r o a c h t o t h e p a t i e n t w i t h d y s p h a g i a . I n : Ya ma d a T , e d .
Tex t book of gas t r oent er ol, ogy
2 n d e d . P h i l a d e l p h i a , PA : L i p p i n c o t t W i l l i a ms
& Wilkins, 1995.
P. 1 8 9
C. Testing
Few screening laboratory tests are indicated in the evaluation of dysphagia unless
h i s t o r y o r c l i n i c a l e xa mi n a t i o n f i n d i n g s d i c t a t e o t h e r w i s e . H o w e v e r, s p e c i a l s t u d i e s ,
325 / 652
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9.5 - Dysphagia
i n c l u d i n g b a r i u m s w a l l o w f o r s u s p e c t e d i n t r i n s i c a n d e xt r i n s i c o b s t r u c t i v e l e s i o n s ,
e s o p h a g o g a s t r o s c o p y f o r a s s e s s i n g e s o p h a g e a l mu c o s a o r ma s s e s i d e n t i f i e d b y
b a r i u m s w a l l o w, a n d v i d e o r a d i o g r a p h y i n s t r o k e p a t i e n t s w i t h s u s p e c t e d s w a l l o w i n g
disorders, are typically required for definitive diagnosis. In patients with acute onse
d y s p h a g i a w h i l e e a t i n g , u p p e r e n d o s c o p y c a n d i r e c t l y r e mo v e a n i mp a c t e d f o o d
b o l u s a n d d i l a t e s t r i c t u r e s . F o r a l l d y s p h a g i a d i a g n o s e s , e n d o s c o p y i s mo r e s e n s i t i v
( 9 2 % v s . 5 4 % ) a n d mo r e s p e c i f i c ( 1 0 0 % v s . 9 1 % ) t h a n d o u b l e - c o n t r a s t u p p e r
g a s t r o i n t e s t i n a l r a d i o g r a p h y.
I V. Diagnosis
T he diagnosis of dysphagia centers on the answers to two questions: is the

d y s p h a g i a o r o p h a r y n g e a l o r e s o p h a g e a l i n l o c a t i o n ? I s i t n e u r o mu s c u l a r o r
o b s t r u c t i v e i n n a t u rTea?b (l e 9 . 5 ). 1P a t i e n t s w i t h o r o p h a r y n g e a l d y s p h a g i a p r e s e n t
with difficulty in initiating swallowing and have associated coughing, choking or nas
r e g u r g i t a t i o n . S p e e c h q u a l i t y ma y h a v e a n a s a l t o n e . O r o p h a r y n g e a l d y s p h a g i a i s
a s s o c i a t e d w i t h s t r o k e , P a r k i n s o n ' s d i s e a s e , o r o t h e r l o n g - t e r m n e u r o mu s c u l a r
d i s o r d e r s . L o c a l s t r u c t u r a l l e s i o n s a r e l e s s c o mmo n . E s o p h a g e a l d y s p h a g i a o f t e n
causes the sensation of food sticking in the throat or chest. Motility disorders and
me c h a n i c a l o b s t r u c t i o n s a r e c o mmo n . S e v e r a l me d i c a t i o n s h a v e b e e n a s s o c i a t e d
w i t h d i r e c t e s o p h a g e a l mu c o s a l i n j u r y, w h e r e a s o t h e r s c a n d e c r e a s e l o w e r
e s o p h a g e a l s p h i n c t e r p r e s s u r e s a n d c a u s eT ar belfel u9x . 5( ). (4,5)
3
.
P. 1 9 0
TAB L E 9.5.2 Associated Sym ptom s and Possible

Causes of Dysphagia
Condition
Diagnose s to conside r
P r o g r e s s i v e d y s p h a g i aN e u r o mu s c u l a r d y s p h a g i a
Sudden dysphagia
Obstructive dysphagia, esophagitis
Difficulty in initiating Oropharyngeal dysphagia

swallow
Food sticks after
swallow
Esophageal dysphagia
Cough
Early in swallow
N e u r o mu s c u l a r d y s p h a g i a
L ate in swallow
Obstructive dysphagia
326 / 652
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9.5 - Dysphagia
We i g h t l o s s
In the elderly
C a r c i n o ma
With regurgitation
Achalasia
P r o g r e s s i v e s y mp t o ms
Heartburn
P e p t i c s t r i c t u r e , s c l e r o d e r ma
I n t e r mi t t e n t s y mp t o ms R i n g s a n d w e b s , d i f f u s e e s o p h a g e a l s p a s m,
nutcracker esophagus
Pain with dysphagia
Esophagitis
Postradiation
I n f e c t i o u s : h e r p e s s i mp l e x v i r u s , mo n i l i a
Pill-induced
P a i n ma d e w o r s e b y
Solid food only
Obstructive dysphagia
Solids and liquids
N e u r o mu s c u l a r d y s p h a g i a
Regurgitation of old
food
We a k n e s s a n d
dysphagia
C e r e b r o v a s c u l a r a c c i d e n t s , mu s c u l a r d y s t r o p h i e s ,
my a s t h e n i a g r a v i s , mu l t i p l e s c l e r o s i s
Halitosis
Dysphagia relieved
Achalasia
with repeated swallows
D y s p h a g i a ma d e w o r s eN e u r o mu s c u l a r mo t i l i t y d i s o r d e r s
with cold foods
F r o m J o h n s o n A . D e g l u t i t i o n . I n : S c o t t - B r o w n W G , K e rSr cAotGt,- e d s .
B r own' s ot ol ar y ngol,ogy
6 t h e d . B o s t o n , M A : B u t t e r w o r t h - H e i n e ma n n , 1 9 9 7 .
327 / 652
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9.5 - Dysphagia
P a t i e n t s w i t h n e u r o mu s c u l a r d y s p h a g i a e xp e r i e n c e g r a d u a l l y p r o g r e s s i v e d i f f i c u l t y i
s w a l l o w i n g s o l i d f o o d a n d l i q u i d s . C o l d f o o d s o f t e n a g g r a v a t e t h e p r o b l e m. P a t i e n t s
ma y s u c c e e d i n p a s s i n g t h e f o o d b o l u s b y r e p e a t e d s w a l l o w i n g , b y p e r f o r mi n g t h e
Va l s a l v a ma n e u v e r, o r b y ma k i n g a p o s i t i o n a l c h a n g e . T h e y a r e mo r e l i k e l y t o
e xp e r i e n c e p a i n w h e n s w a l l o w i n g t h a n p a t i e n t s w i t h s i mp l e o b s t r u c t i o n . A c h a l a s i a ,
s c l e r o d e r ma , a n d d i f f u s e e s o p h a g e a l s p a s m a r e t h e mo s t c o mmo n c a u s e s o f
n e u r o mu s c u l a r mo t i l i t y d i s o r d e r s . O b s t r u c t i v e p a t h o l o g y i s t y p i c a l l y a s s o c i a t e d w i t h
d y s p h a g i a o f s o l i d f o o d b u t n o t l i q u i d s . P a t i e n t s ma y b e a b l e t o f o r c e f o o d t h r o u g h
t h e e s o p h a g u s b y p e r f o r mi n g a Va l s a l v a ma n e u v e r, o r t h e y ma y r e g u r g i t a t e
u n d i g e s t e d f o o d . R a p i d l y p r o g r e s s i n g d y s p h a g i a o f a f e w mo n t h s ' d u r a t i o n s u g g e s t s
e s o p h a g e a l c a r c i n o ma . We i g h t l o s s i s mo r e p r e d i c t i v e o f a me c h a n i c a l o b s t r u c t i v e
l e s i o n . P e p t i c s t r i c t u r e , c a r c i n o ma , a n d S c h a t zk i ' s r i n g a r e t h e p r e d o mi n a n t
obstructive lesions.
P. 1 9 1
TAB L E 9.5.3 Medications Associated with

Dysphagia
Me dications that can cause dire ct e sophage al m ucosal injury
Antibiotics
D o xy c y c l i n e ( Vi b r a my c i n )
Tetracycline
C l i n d a my c i n ( C l e o c i n )
T r i me t h o p r i m- s u l f a me t h o xa zo l e ( B a c t r i m, S e p t r a )
A l e n d r o n a t e ( F o s a ma x)
Zidovudine (Retrovir)
Ascorbic acid
Potassium chloride tablets (Sa
low-K)
T heophylline
Quinidine gluconate
Ferrous sulfate
Me dications, horm one s, and foods associate d w ith re duce d low e r
e sophage al sphincte r tone and re flux
B u t y l s c o p o l a mi n e
T heophylline
Nitrates
Calcium antagonists
328 / 652
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9.5 - Dysphagia
Alcohol, fat, chocolate

Me dications associate d w ith x e rostom ia
A n t i c h o l i n e r g i c s : a t r o p i n e , s c o p o l a mi n e ( T r a n s d e r m S c o p )
- Adrenergic blockers
A n g i o t e n s i n - c o n v e r t i n g e n zy me i n h i b i t o r s
Angiotensin IIreceptor blockers
A n t i a r r h y t h mi c s
D i s o p y r a mi d e ( N o r p a c e )
M e xi l e t i n e ( M e xi t i l )
I p r a t r o p i u m b r o mi d e ( A t r o v e n t )
Diuretics
Opiates
Antipsychotics
aE s p e c i a l l y t h e s l o w - r e l e a s e f o r mu l a t i o n .
F r o m B o y c e H W. D r u g - i n d u c e d e s o p h a g e a l d a ma g e : d i s e a s e s o f me d i c a l
pr ogr ess. [ E di t orG
i aas
l ] t r oi nt es t E ndos
1 9c9 8 ; 4 7 : 5 4 7 5 5 0 ; S t o s c h u s B ,
Al l e s c h e r H D . D r u g - i n d u c e d d y s pD
h aygs iphagi
a.
a1 9 9 3 ; 8 : 1 5 4 1 5 9 .
P. 1 9 2
References
1 . C a s t e l l D O . A p p r o a c h t o t h e p a t i e n t w i t h d y s p h a g i a . I n : Ya ma d a T , e d .
Tex t book of gas t r oent er ol, ogy
2 n d e d . P h i l a d e l p h i a , PA : L i p p i n c o t t W i l l i a ms &
Wilkins, 1995.
2 . A g e n c y f o r H e a l t h C a r e P o l i c y a n d R e sDei agnos
a r c h . i s and t r eat ment of
s wal l owi ng di s or der s ( dy s phagi a) i n ac ut e- c ar e s t r ok
. eE vpat
i d ei nent
c es
R e p o r t / T e c h n o l o g y A s s e s s me n t : N u mb e r 8 . A H C P R P u b l i c a t i o n N o . 9 9 - E 0 2 4 .
Rockville, M D: AH C P R, J uly 1999:9.
3 . J o h n s o n A . D e g l u t i t i o n . I n : S c o t t - B r o w n W G , K e r rSAcG
ot,t -eBdrsown'
.
s
ot ol ar y ngol ogy
, 6 t h e d . B o s t o n , M A : B u t t e r w o r t h - H e i n e ma n , 1 9 9 7 .
4 . B o y c e H W. D r u g - i n d u c e d e s o p h a g e a l d a ma g e : d i s e a s e s o f me d i c a l p r o g r e s s
[ E d i t o r i a lG
] . as t r oi nt es t E ndos
1 9c9 8 ; 4 7 : 5 4 7 5 5 0 .
5 . S t o s c h u s B , A l l e s c h e r H D . D r u g - i n d u c e d d y sDpyhsaphagi
g i a . a1 9 9 3 ; 8 : 1 5 4
159.
329 / 652
tmdmss
9.6 - Epigastric Distress
> T a b l e o f C o n t e n t s > 9 - G a s t r o i n t e s t i n a l P r o b l e ms > 9 . 6 - E p i g a s t r i c D i s t r e s s
9.6
Epigastric Distress
Pam e la L. Pe ntin
I . Background
E p i g a s t r i c d i s c o mf o r t i s c a u s e d b y a v a r i e t y o f c o n d i t i o n s o c c u r r i n g i n mu l t i p l e
o r g a n s a c r o s s t h e g a s t r o i n t e s t i n a l , c a r d i o v a s c u l a r, a n d p u l mo n a r y s y s t e ms . S e r i o u s
causes, such as gastric, esophageal, bowel, and pancreatic cancers are rare but
mu s t a l s o b e c o n s i d e r e d . O v e r l a p p i n g s y mp t o ms ma k e i n i t i a l d i a g n o s i s d i f f i c u l t , a n d
i n ma n y p a t i e n t s a d e f i n i t e c a u s e i s n e v e r e s t a b l i s h e d . P u l mo n a r y, c a r d i a c , a n d
v a s c u l a r c a u s e s o f e p i g a s t r i c d i s c o mf o r t a r e r e v i e w e d i n o t h e r c h a p t e r s .
A. Etiology
Gastroesophageal reflux disease (G E R D) is caused by weakness of the lower

e s o p h a g e a l s p h i n c t e r, a l l o w i n g s t o ma c h c o n t e n t s t o mo v e i n a r e t r o g r a d e d i r e c t i o n ,
irritating the esophagus. Esophagitis can be related to G E R D but also can be
c a u s e d b y h y p e r s e c r e t o r y c o n d i t i o n s . E s o p h a g i t i s a n d g a s t r i t i s c a n a l s o b e p r i ma r i l
i n f l a mma t o r y c o n d i t i o n s , r e l a t e d t o me d i c a t i o n s s u c h a s n o n s t e r o i d a l a n t i i n f l a mma t o r y d r u g s ( N S A I D s ) a n d t o xi n s s u c h a s a l c o h o l . M o s t p e p t i c u l c e r d i s e a s e
( P U D s ) a r e r e l a t e dHtel
o i c obac t er py l or
i nif e c t i o n . G a l l s t o n e s , f o r me d b y
supersaturation of bile, cause pain by obstructing the cystic duct. Obstruction of th
c y s t i c d u c t b y g a l l s t o n e s c a n c a u s e c h o l e c y s t i t i s , a n a c u t e i n f l a mma t o r y a n d o f t e n
condition process.
B. Epidemiology
E p i g a s t r i c d i s c o mf o r t a n d d y s p e p s i a o c c u r i n 2 5 % o f t h e p o p u l a t i o n e a c h y e a r, b u t
mo s t a f f e c t e d p e r s o n s d o n o t s e e k me d i c1)
a l. cPaar tei e(n t s a t i n c r e a s e d r i s k f o r
g a l l s t o n e s i n c l u d e t h e e l d e r l y, t h e o b e s e ( e s p e c i a l l y w i t h r a p i d w e i g h t l o s s ) , t h e
p r e g n a n t , a n d t h o s e o n me d i c a t i o n s s u c h a s f i b r a t e s , e s t r o g e n s , a n d c o n t r a c e p t i v e s
A l s o a t i n c r e a s e d r i s k f o r g a l l s t o n e s a r e c e r t a i n e t h n i c i t i e s , t h o s e w i t h ma t e r n a l
f a mi l y h i s t o r y, t h e f e ma l e g e n d e r, a n d t h o s e w i t h me t a b o l i c d i s e a s e s s u c h a s
d i a b e t e s , c i r r h o s i s , a n d h y p e r t r i g l y c e r i d e mi a .
I I I . Evaluation
A. Clinical approach
330 / 652
tmdmss
T y p i c a hl i s t o r y, p h y s i c a l e x a m i n a t,i oann dl a b o r a t o r y f i n d i nagsss o c i a t e d w i t h

c o mmo n c a u s e s o f e p i g a s t r i c d i s t r e s s a r e Tl iasbt leed 9i .n6 . 1
B. Genetics
M o s t c a u s e s o f e p i g a s t r i c d i s c o mf o r t r e s u l t f r o m e n v i r o n me n t a l f a c t o r s , b u t s o me ,
s u c h a s c h o l e l i t h i a s i s , e xh i b i t h e r e d i t a r y c o mp o n e n t s . C o l o n i c a n d e s o p h a g e a l
ma l i g n a n c i e s a r i s e i n c e r t a i n i n h e r i t e d d i s o r d e r s . H e r e d i t a r y p a n c r e a t i t i s i s c a u s e d
b y a mu t a t i o n i n t h e c a t i o n i c t r y p s i n o g e n g e n e .
I V. Diagnosis
T h e d i f f e r e n t i a l d i a g n o s i s o f g a s t r o i n t e s t i n a l c a u s e s o f e p i g a s t r i c d i s c o mf o r t i s
p r e s e n t e d iTna b l e 9 . 6 . 2
TAB L E 9.6.1 Typical Historical, Physical

Exam ination, and Laboratory Findings for
Gastrointestinal Etiologies of Epigastric Discom fort
History
Phy sical
e x am ination
Laboratory te sts
GERD
Heartburn,
Dental
regurgitation,
erosions,
sour belches,
e xa mi n a t i o n
p a i n r a d i a t i n g t o o f t e n n o r ma l
throat, pain
worsened by lying
supine, chronic
cough,
hoarseness,
beneficial trial of
t r e a t me n t w i t h
p r o t o n - p u mp
inhibitors
U s u a l l y n o r ma l
PUD
P e r s o n a l o r f a mi l yH y p o t e n s i o n o r
history P U D,
tachycardia (G I
s mo k i n g h i s t o r y, b l e e d i n g ) ,
n o n s t e r o i d a l a n t i - me l e n a
i n f l a mma t o r y d r u g
use, pain reduced
by the intake of
me a l s
Positive
H el i c obac t er
py l or i t e s t i n g ,
h e me p o s i t i v e
stools
331 / 652
tmdmss
GI
ma l i g n a n c y
Dysphagia,
We i g h t l o s s ,
A n e mi a , h e me
weight loss,
p a l p a b l e ma s s , p o s i t i v e s t o o l s
c o n t i n u o u s p a i n , Vi r c h o w ' s
a n o r e xi a ,
nodes,
protracted
hypotension or
v o mi t i n g , a g e > 5 0 t a c h y c a r d i a ( G I
y, s mo k i n g o r
bleeding),
a l c o h o l h i s t o r y, me l e n a ,
f a mi l y h i s t o r y
acanthosis
nigricans,
brittle nails,
cheilosis,
conjunctival
pallor
Pancreatitis
Abrupt onset
D i f f u s e , s e v e r e E l e v a t e d a my l a s e ,
pain, stabbing,
pain
lipase
severe, and
radiating to back;
history of alcohol
use
Cholelithiasis Rapid onset pain, No palpable

increasing
ma s s
i n t e n s i t y, < 3 - h
duration,
radiation to
scapula or right
s h o u l d e r,
sweating,
v o mi t i n g , p a i n
brought on by
me a l s , mo r e
c o mmo n a t n i g h t ,
genetic
c o mp o n e n t
U s u a l l y n o r ma l
C h o l e c y s t i t i s > 3 - h d u r a t i o n o f P a l p a b l e ma s s
pain, shifts from (30%40%),
epigastrium to
f e v e r, j a u n d i c e
right upper
(15%), positive
quadrant, acholic Murphy's sign
stools, dark urine
L eukocytosis (with
left shift), elevated
s e d i me n t a t i o n
rate, elevated
bilirubin, elevated
a mi n o t r a n s f e r a s e s ,
elevated alkaline
phosphatase,
e l e v a t e d a my l a s e
332 / 652
tmdmss
Irritable
bowel
Constipation
and/or diarrhea,
pain relieved by
defecation
N o r ma l
N o r ma l
G E R D, gastroesophageal reflux disease; G I, gastrointestinal; P U D, peptic

ulcer disease
F r o m B a za l d u a O V, S c h n e i d e r F D . E v a l u a t i o n a n d ma n a g e me n t o f
d y s p e p s i aA. m F am P hy s i c i an
1999;60:17731788; Dyspepsia and G E R D
P r a c t i c e G u i d e l i n e . I n s t i t u t e f o r C l i n i c a l S y s t e ms I mp r o v e me n t ( I C S I ) ; 2 0 0 4
J ul; Marshall B J . Gastritis and peptic ulcer disease. In: Rakel R E , ed.
C onn' s c ur r ent t her,apy
5 7 t h e d . E l s e v i e r, 2 0 0 5 : 6 0 0 6 0 3 ; A h ma d M , e t a l .
D i f f e r e n t i a l d i a g n o s i s o f g a l l s t o n e i n d u c e d c o mpSl out
i c a thi oM
n sed
. J
2000;93:261264.
P. 1 9 3
1. A v e r y u s e f u l d i a g n o s t i c t e s t f o r G E R D i s a s i mp l e t r i a l o f t r e a t me n t w i t h a
d o u b l e - d o s e d p r o t o n - p u mp i n h i b i t o r, s u c h a s o me p r a zo l e . I f s y mp t o ms r e mi t ,
f u r t h e r w o r k u p ma y n o t b e n e c e s s a r y. I f s y mp t o ms d o n o t r e mi t , t h e n a n u p p e r
endoscopy is warranted. Dysphagia (difficulty in swallowing) suggests an early
s y mp t o m o f e s o p h a g e a l c a n c e r o r c a n b e a n a c i d - i n d u c e d s t r i c t u r e . T h e b i l i a r y
colic of gallstones causes epigastric or right upper quadrant pain of rapid onset
t h a t i n c r e a s e s i n i n t e n s i t y o v e r a 1 5 - mi n u t e i n t e r v a l a n d l a s t s a s l o n g a s 3
hours,
P. 1 9 4
u s u a l l y w i t h o u t a n y p a l p a b l e ma s s . P a i n ma y r a d i a t e t o t h e i n t e r s c a p u l a r r e g i o n
o r t o t h e r i g h t s h o u l d e r. A s s o c i a t e d s w e a t i n g a n d v o mi t i n g a r e c o mmo n . A t t a c k s
o f b i l i a r y c o l i c a r e o f t e n b r o u g h t o n b y e a t i n g , e s p e c i a l l y a f a t t y me a l , a n d a r e
mo r e c o mmo n a t n i g h t , p o s s i b l y b e c a u s e t h e g a l l b l a d d e r s h i f t s t o a h o r i zo n t a l
p o s i t i o n , f a c i l i t a t i n g e n t r y o f s t o n e s i n t o t h e c 2)
y s.t i c d u c t . (
TAB L E 9.6.2 Differential Diagnosis of

Gastrointestinal Causes of Epigastric
Discom fort
Esophagus
Esophagitis
Medications
333 / 652
tmdmss
Lung
P n e u mo n i a
P n e u mo t h o r a x
Pancre as
Pancreatitis
He art
C a r d i a c i s c h e mi a
Pericarditis
Stom ach
Gastritis
Peptic ulcer disease
Bleeding
Gastroparesis
Medications
Gallbladde r
Cholelithiasis
Cholecystitis
Bow e l
I r r i t a b l e b o w e l s y n d r o me
Bleeding
2. A c u t e c h o l e c y s t i t i s c a u s e s e p i g a s t r i c o r r i g h t u p p e r q u a d r a n t t e n d e r n e s s o r
ma s s a n d f e v e r. T h e p a i n t y p i c a l l y l a s t s l o n g e r t h a n 3 h o u r s , a n d o v e r t i me ,
s h i f t s f r o m t h e e p i g a s t r i u m t o t h e r i g h t u p p e r q u a d r a n t . I n t h e e l d e r l y, l o c a l i ze d
t e n d e r n e s s ma y b e t h e o n l y p r e s e n t i n g s i g n . T h e g a l l b l a d d e r i s p a l p a b l e 3 0 % t o
4 0 % o f t h e t i me , a n d j a u n d i c e i s s e e n i n a b o u t 1 5 % o f p a t i e n t s w i t h a c u t e
c h o l e c y s t i t i s . P a t i e n t s w i t h c h o l e c y s t i t i s ma y a l s o h a v e a c h o l i c s t o o l s .
3. M a l i g n a n c i e s t h a t c a u s e e p i g a s t r i c d i s c o mf o r t a r e r a r e . H o w e v e r, s y mp t o ms o f
g a s t r i c a n d e s o p h a g e a l c a n c e r s a r e s i mi l a r t o t h o s e o f o t h e r c a u s e s o f
e p i g a s t r i c d i s t r e s s . D y s p h a g i a , u n e xp l a i n e d w e i g h t l o s s , h i s t o r y o f
g a s t r o i n t e s t i n a l b l e e d i n g , o r c l i n i c a l s i g n s o f a n e mi a c a n d i f f e r e n t i a t e p a t i e n t s
w i t h a mo r e s e r i o u s d i s e a s e . N u me r o u s me d i c a t Ti oanbsl e( s9e. 6e ). 3i n d u c e
334 / 652
tmdmss
n o n u l c e r d y s p e p s i a . B y i d e n t i f y i n g me d i c a t i o n - a s s o c i a t e d d y s p e p s i a , c o s t l y
d i a g n o s t i c s t u d i e s c a n b e a v o i d e d . I r r i t a b l e b o w e l s y n d r o me i s g e n e r a l l y
a s s o c i a t e d w i t h a b n o r ma l b o w e l h a b i t s .
P. 1 9 5
TAB L E 9.6.3 Medications Associated with

Epigastric Discom fort
Acarbose
Bisphosphonates
I r o n s u p p l e me n t s
Oral antibiotics
P o t a s s i u m s u p p l e me n t s
S y s t e mi c c o r t i c o s t e r o i d s
4. T h o u g h d i s c u s s e dCi hna p t e r 7 ,. 5i t mu s t b e me n t i o n e d h e r e t h a t t h e p a i n o f
i s c h e mi c h e a r t d i s e a s e ma y o r i g i n a t e i n t h e e p i g a s t r i u m a n d mu s t b e r u l e d o u t .
M e t a b o l i c d i s o r d e r s a r e a r a r e c a u s e o f e p i g a s t r i c d i s c o mf o r t , b u t a c o mp l e t e
d i f f e r e n t i a l s h o u l d i n c l u d e ma l a b s o r p t i o n s y n d r o me s , c o l l a g e n v a s c u l a r
d i s o r d e r s , Z o l l i n g e r - E l l i s o n s y n d r o me a n d C r o h n ' s d i s e a s e .
References
1 . B a za l d u a O V, S c h n e i d e r F D . E v a l u a t i o n a n d ma n a g e me n t o f Admy s p e p s i a .
1999;60:17731788.
2 . A h me d A , C h e u n g R C , K e e f f e E B . M a n a g e me n t o f g a l l s t o n e s a n d t h e i r
c o mp l i c a t i o n A
s .m F am P hy s i c i an
2000;61:16731680, 16871688.
335 / 652
tmdmss
9.7 - Upper Gastrointestinal Bleeding
> T a b l e o f C o n t e n t s > 9 - G a s t r o i n t e s t i n a l P r o b l e ms > 9 . 7 - U p p e r G a s t r o i n t e s t i n a l
Bleeding
9.7
Upper Gastrointestinal Bleeding
Charle s S. Blackadar
I . Background
U p p e r g a s t r o i n t e s t i n a l ( G I ) b l e e d i n g , d e f i n e d a s b l e e d i n g p r o xi ma l t o t h e l i g a me n t o
T r e i t z, i s r e s p o n s i b l e f o r 3 5 0 , 0 0 0 h o s p i t a l a d mi s s i o n s i n t h e U n i t e d S t a t e s , w i t h a
mo r t a l i t y o f 1 01)
%.( B l e e d i n g c a n b e e i t h e r a c u t e o r c h r o n i c , a n d t h e s o u r c e c a n b e
o v e r t o r o c c u l t . T h e p a t i e n t ma y b e e i t h e r h e mo d y n a mi c a l l y s t a b l e o r u n s t a b l e o n
presentation.
Bleeding from the upper G I tract usually results when disruption occurs between the
p r o t e c t i v e b a r r i e r s t o t h e v a s c u l a t u r e a n d t h e h a r s h e n v i r o n me n t o f t h e d i g e s t i v e
t r a c t . T h e mo s t c o mmo n c a u s e s ( a s p e r c e n t a g e s ) a rTea lbi lset e9d. 7i .n1
I I I . Evaluation
T h e k e y t o s u c c e s s f u l e v a l u a t i o n a n d d i a g n o s i s i s a s y s t e ma t i c a p p r o a c h w i t h
e mp h a s i s o n t h e o v e r a l l h e mo d y n a mi c s t a t u s o f t h e p F
a ti gi eunr te ( 9s .e7e). .1
A. History
C l i n i c a l h i s t o r y a c c u r a t e l y p o i n t s t o t h e s o u r c e o f b l e e d i n g i n o n l y 2)
4 0. % o f c a s e s (
H e ma t e me s i s a n d me l e n a a r e t h e mo s t c o mmo n p r e s e n t a t i o n s o f a c u t e u p p e r G I
b l e e d i n g . T h e e xa mi n e r mu s t g e t a n s w e r s t o s e v e r a l i mp o r t a n t q u e s t i o n s : I s t h e r e a
p r i o r h i s t o r y o f b l e e d i n g ( 6 0 % r e b l e e d f r o m t h e s2)a.me
D osei st e t) h( e p a t i e n t h a v e
a n y c o mo r b i d d i s e a s e s ( p e p t i c u l c e r d i s e a s e , p a n c r e a t i t i s , c i r r h o s i s , c a n c e r ) ?
P a r t i c u l a r a t t e n t i o n mu s t b e g i v e n t o t h e p a t i e n t ' s c a r d i o p u l mo n a r y s t a t u s b e c a u s e
t h i s a f f e c t s u r g e n c y a n d d e g r e e o f r e s u s c i t a t i vTea belfef o9r t. s7. l1i s t s o t h e r
d i a g n o s t i c c l u e s t o c o mmo n c a u s eTs a, balned9 . 7 . l2i s t s t h e l e s s c o mmo n c a u s e s .
P. 1 9 6
TAB L E 9.7.1 Com m on Causes of Gastrointestinal

Bleeding and Diagnostic Clues
Cause
Diagnostic clue
336 / 652
tmdmss
Peptic ulcer
(55%)
N o n s t e r o i d a l a n t i - i n f l a mma t o r y d r uHgeluisceobac
,
t er
py l or i i n f e c t i o n , s t r e s s
E s o p h a g o g a s t r i c E t h a n o l u s e , u mb i l i c a l o r r e c t a l v a r i c e s , p a l ma r
varices (14%)
e r y t h e ma , a s c i t e s , s p i d e r h e ma n g i o ma s
Arteriovenous
ma l f o r ma t i o n s
(6%)
History of previous episodes
M a l l o r y - We i s s
tears (5%)
Vo mi t i n g p r i o r t o h e ma t e me s i s , e t h a n o l u s e , o c c u r s i n
y o u n g me n
T u mo r s ( 4 % )
E t h a n o l u s e , s mo k i n g , s mo k e d f o o d s , g a s t r o e s o p h a g e a l
reflux disease, B arrett's esophagus, weight loss
F rom J utabha R J , J ensen D M . Approach to the patient with upper

gastrointestinal bleeding. U pToD ate J une 2005.
1. V i t a l s i g n T
s h e s i n g l e mo s t i mp o r t a n t a s p e c t o f t h e i n i t i a l p h y s i c a l e xa mi n a t i o n
i s d e t e r mi n i n g t h e p a t i e n t ' s h e mo d y n a mi c s t a b i l i t y. U n s t a b l e p a t i e n t s s h o u l d b e
ma n a g e d a s t r a u ma p a t i e n t s . P l a c e me n t o f a n a s o g a s t r i c ( N G ) t u b e i s
c o n s i d e r e d t h e f i f t h v i t a l s i g n i n p a t i e n t s w i t h a c u t e u p p e r 3)G. I b l e e d i n g (
A f t e r e n s u r i n g h e mo d y n a mi c s t a b i l i t y, t h e i n i t i a l p h y s i c a l e xa mi n a t i o n s h o u l d
e l i mi n a t e a n a s a l o r o r o p h a r y n g e a l s o u r c e o f b l e e d i n g .
2. S k i n e x a m i n a t i oEnc c h y mo s e s , p e t e c h i a e , a n d v a r i c e s s h o u l d b e n o t e d .
C o n j u n c t i v a l p a l l o r i s a s i g n o f c h r o n i c a n e mi a . N u me r o u s mu c o s a l
t e l a n g i e c t a s i a s c a n p o i n t t o a n u n d e r l y i n g v a s c u l a r a b n o r ma l i t y.
3. A b d o m i n a l e x a m i n a t iLoono k f o r s t i g ma t a o f c h r o n i c l i v e r d i s e a s e
( h e p a t o s p l e n o me g a l y, s p i d e r a n g i o ma t a , a s c i t e s , p a l ma r e r y t h e ma , c a p u t
me d u s a e , g y n e c o ma s t i a , a n d t e s t i c u l a r a t r o p h y ) .
4. R e c t a l e x a m i n a t i o
Rne c t a l v a r i c e s , h e mo r r h o i d s , a n d f i s s u r e s s h o u l d b e n o t e d .
C. Testing
B a s i c l a b o r a t o r y s t u d i e s s h o u l d i n c l u d e a c o mp l e t e b l o o d c o u n t ( C B C ) w i t h p a r t i c u l
a t t e n t i o n t o t h e h e ma t o c r i t , c o a g u l a t i o n s t u d i e s ( p r o t h r o mb i n t i me [ P T ] ) a n d p a r t i a l
t h r o mb o p l a s t i n t i me [ P T T ] ) , l i v e r f u n c t i o n t e s t s ( L F T s ) , s e r u m c h e mi s t r i e s ( b l o o d
urea nitrogen is elevated disproportionate to creatinine in patients with G I blood
l o s s ) , e l e c t r o c a r d i o g r a m ( E C G ) , a n d N G a s p i r a t e a n a l y s i s . I n i t i a l l y, t h e h e ma t o c r i t
a p o o r i n d i c a t o r o f b l o o d l o s s ; h o w e v e r, s e r i a l h e ma t o c r i t s c a n b e u s e f u l i n a s s e s s i
ongoing blood loss. T he C B C indices, particularly an elevated red cell distribution
337 / 652
tmdmss
w i d t h a n d l o w me a n c o r p u s c u l a r v o l u me , p o i n t t o w a r d a c h r o n i c b l e e d . A p r o l o n g e d
P T o r P T T s u g g e s t s a n u n d e r l y i n g c o a g u l o p a t h y. E l e v a t e d L F T s s u g g e s t u n d e r l y i n g
l i v e r d i s e a s e . A n E C G i s i mp o r t a n t , e s p e c i a l l y i n e l d e r l y p a t i e n t s , t o s e a r c h f o r
e v i d e n c e o f c a r d i a c i s c h e mi a . I f t h e a s p i r a t e i s b r i g h t r e d , o r c o f f e e g r o u n d s i n
a p p e a r a n c e , a n u p p e r G I s o u r c e o f b l e e d i n g i s l i k e l y. A d d i t i o n a l l y, N G l a v a g e
r e mo v e s b l o o d a n d h e l p s w i t h t h e p e r f o r ma n c e o f e n d o s c o p y. I n c a s e s i n w h i c h n o
source can be identified, lower endoscopy should be considered in the search for a
lower G I bleed.
I V. Diagnosis
T he differential diagnosis of an upper G I bleed should include bleeding from the
upper airways or pharynx that is swallowed and regurgitated and lower G I bleeding
t h a t ma y b e ma n i f e s t e d a s me l a n i c s t o o l s d u e t o d e l a y i n t r a n s i t t h r o u g h t h e c o l o n .
mo s t c a s e s , d i a g n o s i s c a n b e c o n f i r me d w i t h l a v a g e a n d t h e s o u r c e l o c a l i ze d a n d
t r e a t e d w i t h u p p e r e n d o s c o p y. I n r a r e c a s e s i n w h i c h e n d o s c o p y i s u n a b l e t o
a d e q u a t e l y i d e n t i f y t h e s o u r c e o f G I b l e e d i n g , s p e c i a l i ze d n u c l e a r me d i c i n e a n d
angiographic studies can be used.
P. 1 9 7
F I G U R E 9 . 7 . 1A.p p r o a c h t o u p p e r g a s t r o i n t e s t i n a l b l e e d i n g . ( M o d i f i e d f r o m
E i s e n G M , D o mi n i t z J A , F a i g e l D O . A n a n n o t a t e d a l g o r i t h mi c a p p r o a c h t o u p p e r
g a s t r o i n t e s t i n a l b l e e dG
i nas
g .t r oi nt es t E ndos
2 0c0 1 ; 5 3 : 8 5 3 8 )5 8 .
338 / 652
tmdmss
TAB L E 9.7.2 Uncom m on Causes of Upper

Gastrointestinal Bleeding
Cause
D ieulafoy's
lesion
Diagnostic clue
Congenital lesion usually diagnosed during endoscopy
G a s t r i c a n t r a l A s s o c i a t e d w i t h c i r r h o s i s a n d e l d e r l y w o me n
vascular
ectasia
H e mo b i l i a
Usually associated with recent biliary tree injury
Aortoenteric
fistulas
P r i ma r y c a s e s a s s o c i a t e d w i t h A A A , s e c o n d a r y c a s e s w i t h
f i s t u l a s f r o m A A A r e p a i r s ; ma y p r e s e n t w i t h b a c k p a i n a n d
fever
A A A , a b d o mi n a l a o r t i c a n e u r y s m.
F rom J utabha R J , J ensen D M . Approach to the patient with upper
gastrointestinal bleeding. U pToD ate J une 2005.
References
1 . E i s e n G M , D o mi n i t z J A , F a i g e l D O . A n a n n o t a t e d a l g o r i t h mi c a p p r o a c h t o
u p p e r g a s t r o i n t e s t i n a l b l e eG
d ias
n gt .r oi nt es t E ndos
2 0c0 1 ; 5 3 : 8 5 3 8 5 8 .
2 . M c G u i r k T D , C o y l e W J . U p p e r g a s t r o i n t e s t i n a l t r a c tE bmer
l e egd iM
n ged
.
C l i n N or t h A 1m9 9 6 ; 1 4 : 5 2 3 5 4 5 .
3 . L a i n e L . A c u t e a n d c h r o n i c g a s t r o i n t e s t i n a l b l e e d i n g . I n : F e l d ma n M ,
S l e i s i n g e r M H , S c h a r s c h mi d t B F,G as
e dtsr.oi nt es t i nal and l i v er di s eas e:
pat hophy s i ol ogy, di agnos i s , and management
. P h i l a d e l p h i a , PA : W B S a u n d e r s ,
1998:198218.
339 / 652
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9.8 - Hepatitis
> T a b l e o f C o n t e n t s > 9 - G a s t r o i n t e s t i n a l P r o b l e ms > 9 . 8 - H e p a t i t i s
9.8
Hepatitis
Brian A. Sm ole y
I . Background
H e p a t i t i s i s a n i n f l a mma t i o n o f t h e l i v e r t h a t i s u s u a l l y c a u s e d b y a v i r a l i n f e c t i o n b u t
c a n r e s u l t f r o m a n u mb e r o f d i f f e r e n t e t i o l o g i e s .
T h e i n f l a mma t i o n d u e t o h e p a t i t i s p r o d u c e s c e l l u l a r i n j u r y a n d n e c r o s i s . E v e n w i t h
f u l mi n a n t a c u t e h e p a t i t i s , r e c o v e r y i s p o s s i b l e . F i b r o s i s d i s t o r t s t h e l i v e r ' s
architecture in chronic hepatitis and can cause cirrhosis and portal hypertension.
Whether acute or chronic, liver failure ensues when enough necrosis has occurred to
c o mp r o mi s e h e p a t i c f u n c t i o n .
A. Etiology
Vi r a l h e p a t i t i s h a s mu l t i p l e c a u s e s . T h e h i g h l y c o n t a g i o u s h e p a t i t i s A a n d E v i r u s e s
( H AV, H E V ) a r e s p r e a d b y t h e f e c a l - o r a l r o u t e a n d t y p i c a l l y p r o d u c e a n a c u t e , s e l f l i mi t e d , i n f e c t i o u s h e p a t i t i s . H e p a t i t i s B , C , a n d D v i r u s e s ( H B V, H C V, H D V ) a r e
s p r e a d p a r e n t e r a l l y, v e r t i c a l l y, o r b y i n t i ma t e c o n t a c t a n d c a u s e a c u t e a n d c h r o n i c
s e r u m h e p a t i t i s . H D V r e q u i r e s t h e p r e s e n c e o f H B V, c a u s i n g c o i n f e c t i o n w h e n t h e y
a r e t r a n s mi t t e d t o g e t h e r a n d s u p e r i n f e c t i o n w h e n H D V f o l l o w s H B V.
1. T o xi n - o r d r u g - i n d u c e d h e p a t i t i s c a n b e d i f f i c u l t t o d i a g n o s e a n d i s p r o b a b l y
u n d e r r e p o r t e d . A n o f f e n d i n g d r u g ma y h a v e b e e n s t a r t e d mo n t h s e a r l i e r a n d
e xp o s u r e t o t o xi n s ma y n o t b e k n o w n . A c e t a mi n o p h e n i s t h e b e s t k n o w n a n d
mo s t c o mmo n d r u g c u l p r i t , b u t o t h e r k n o w n o f f e n d e r s i n c l u d e n o n s t e r o i d a l a n t i i n f l a mma t o r y d r u g s , a n t i c o n v u l s a n t s , a n d v a r i o u s a n t i mi c r o b i a l s . H e p a t o t o xi n s
i n c l u d e o r g a n i c s o l v e n t s , mu s h r o o m a ma t o xi n , r a t p o i s o n s , a n d y e l l o w
p h o s p h o r u s ( f o u n d i n f i r e w o r k s ) . A l c o h o l i s a l s o a n i mp o r t a n t a g e n t , e i t h e r a s a
t o xi n o r t h r o u g h i t s e f f e c t o n t h e me t a b o l i s m o f o t h e r t o xi n s .
2. F a t t y l i v e r d i s e a s e s t a r t s w i t h a n a c c u mu l a t i o n o f l i p i d s w i t h i n h e p a t o c y t e s
(steatosis) and can progress to frank steatohepatitis and even cirrhosis. Alcohol
c o n s u mp t i o n i s a c o mmo n c a u s e , b u t ma n y d r u g s a n d me t a b o l i c d i s o r d e r s
( e s p e c i a l l y i n s u l i n - r e s i s t a n t s y n d r o me s ) a r e a s s o c i a t e d w i t h n o n a l c o h o l i c
steatosis and steatohepatitis.
3. A s w i t h mo s t a u t o i mmu n e d i s e a s e s , a u t o i mmu n e h e p a t i t i s o c c u r s mo r e
c o mmo n l y i n w o me n . I t p r e s e n t s i n a n u mb e r o f w a y s b u t i s p r o g r e s s i v e a n d
u l t i ma t e l y l e a d s t o e n d - s t a g e l i v e r d i s e a s e i f n o 1t -tAr enat itter yd p. si n ( A 1 AT )
i s a h e p a t i c a l l y p r o d u c e d p r o t e i n a s e i n h i b i t o r. A 1 AT - d e f i c i e n t i n d i v i d u a l s w h o
p r o d u c e a p a r t i c u l a r a b n o r ma l f o r m o f t h e e n zy me a n d l a c k t h e a b i l i t y t o d e g r a d e
i t d e v e l o p h e p a t i t i s b e c a u s e o f i t s a c c u mu l a t i o n w i t h i n h e p a t o c y t e s . I r o n
overload leads to iron deposition in the liver and other tissues. Hepatic iron
o v e r l o a d u s u a l l y t a k e s a t l e a s t 4 0 y e a r s t o b e c o me s y mp t o ma t i c . H e r e d i t a r y
h e mo c h r o ma t o s i s , i n e f f e c t i v e e r y t h r o p o i e s i s , mu l t i p l e b l o o d t r a n s f u s i o n s , a n d
e xc e s s i v e d i e t a r y i n t a k e i r o n a r e k n o w n c a u s e s . A d e f i c i e n t c o p p e r - t r a n s p o r t i n g
e n zy me c a u s e s W i l s o n ' s d i s e a s e . C o p p e r f i r s t a c c u mu l a t e s w i t h i n h e p a t o c y t e s
a n d l a t e r i n o t h e r t i s s u e s a s i t s p i l l s i n t o t h e s e r u m. I s c h e mi a c a n a l s o c a u s e
h e p a t i t i s1,2)
( .
P. 1 9 9
B. Epidemiology
A c u t e h e p a t i t i s a f f e c t s u p t o 1 % o f A me r i c a n s e a c h y e a r. H AV, H B V, a n d H C V a r e
the causes in 97% of cases (48%, 34%, and 15%, respectively). T here were an
e s t i ma t e d a v e r a g e o f 9 2 , 0 0 0 n e w c a s e s o f h e p a t i t i s A , 7 8 , 0 0 0 o f h e p a t i t i s B , a n d
30,000 of hepatitis C each year in the United States from 2000 to 2003. T he
e s t i ma t e d p r e v a l e n c e o f c h r o n i c h e p a t i t i s i s 2 % . A p p r o xi ma t e l y, 5 % o f H B V
i n f e c t i o n s a n d 7 5 % o f H C V i n f e c t i o n s r e s u l t i n c h r o n i c d i s e a s e , y i e l d i n g a n e s t i ma t e d
1 . 2 5 mi l l i o n c a s e s o f c h r o n i c h e p a t i t i s B a n d 2 . 7 mi l l i o n c a s e s o f c h r o n i c h e p a t i t i s C
i n t h e U n i t e d S t a t 1,3)
e s (. A s y mp t o ma t i c e l e v a t i o n s o f a mi n o t r a n s f e r a s e s o c c u r i n
1% to 4% of individuals, but at least half likely represent the 2.5% whose values are
e xp e c t e d t o f a l l a b o v e t h e n o r ma l r a n g e . F a t t y l i v e r d i s e a s e i s b e l i e v e d t o b e t h e
mo s t c o mmo n c a u s e o f mi l d ( l e s s t h a n f i v e t i me s t h e u p p e r l i mi t o f n o r ma l ) l i v e r
e n zy me e l e v a t i o n2)s . (
340 / 652
tmdmss
9.8 - Hepatitis
I I I . Evaluation
A. History
Initial history should focus on identifying risk factors for viral and alcoholic hepatitis.
H i s t o r y o f c o n t a c t w i t h a p e r s o n w i t h j a u n d i c e o r h e p a t i t i s , t r a v e l t o e n d e mi c a r e a s ,
ingestion of shellfish or other suspect foods, work in a health care or other high-risk
s e t t i n g , d a y - c a r e a t t e n d a n c e , i n s t i t u t i o n a l i za t i o n , h i s t o r y o f b l o o d t r a n s f u s i o n , s e xu a l
p r o mi s c u i t y, o r a n y u s e o f i n j e c t e d i l l i c i t d r u g s a l l s u g g e s t v i r a l h e p a t i t i s . S c r e e n i n g
for alcohol abuse is done with questionnaires (e.g., C AG E) or by looking for red
f l a g s ( e . g . , s o c i a l o r o c c u p a t i o n a l d i f f i c u l t i e s , d e p r e s s i o n o r a n xi e t y, p o o r l y
e xp l a i n e d t r a u ma , c o n v i c t i o n s f o r d r i v i n g w h i l e b e i n g i n t o xi c a t e d ) . R e v i e w o f a l l
me d i c a t i o n s ( p r e s c r i b e d , o v e r t h e c o u n t e r, t r a d i t i o n a l , a n d c o mp l e me n t a r y ) a n d
o c c u p a t i o n a l e xp o s u r e s c a n i d e n t i f y p o t e n t i a l h e p a t o t o xi n s . F a mi l y h i s t o r y c a n y i e l d
e v i d e n c e o f i n h e r i t e d d i s o r d e r s s u c h a s A 1 AT d e f i c i e n c y, h e r e d i t a r y
h e mo c h r o ma t o s i s , a n d W i l s o n ' s d i s1,4)
e a s. e (
E xa mi n a t i o n s e r v e s t o c o n f i r m o r r e f u t e i n i t i a l s u s p i c i o n s a n d r e v e a l o t h e r c l u e s t o
the cause and severity of disease. Icterus, jaundice, and liver tenderness are
e xp e c t e d f i n d i n g s i n a c u t e h e p a t i t i s ; h e p a t o s p l e n o me g a l y c a n o c c u r i n s e v e r e c a s e s .
P a t i e n t s w i t h c h r o n i c h e p a t i t i s c a n h a v e o n l y mi l d l i v e r t e n d e r n e s s . T h e l i v e r c a n b e
s ma l l o r e n l a r g e d ; a n d s mo o t h , n o d u l a r, o r h a r d . P a t i e n t s w i t h a d v a n c e d d i s e a s e
p r e s e n t w i t h s i g n s o f c h r o n i c d i s e a s e ( e . g . , mu s c l e w a s t i n g ) , a l t e r e d h o r mo n e
me t a b o l i s m ( e . g . , a l o p e c i a , g y n e c o ma s t i a , p a l ma r e r y t h e ma , t e s t i c u l a r a t r o p h y ) , a n d
p o r t a l h y p e r t e n s i o n ( e . g . , a s c i t e s , c a p u t me d u s a , s p l e1)n.o me g a l y ) (
C. Testing
1. T h e l i k e l i h o o d a n d s e v e r i t y o f d i s e a s e mu s t b e c o n s i d e r e d w h e n o r d e r i n g o r
i n t e r p r e t i n g l a b o r a t o r y t e s t s . C o mmo n l y u s e d tTeas bt sl e( s9e. 8e). 1i n c l u d e
s e r u m a l a n i n e a n d a s p a r t a t e a mi n o t r a n s f e r a s e s ( A LT a n d A S T ) , a l k a l i n e
p h o s p h a t a s e , a n d - g l u t a my l t r a n s p e p t i d a s e ( G G T ) ; s e r u m a l b u mi n a n d b i l i r u b i n
l e v e l s ; a n d p r o t h r o mb i n t i me ( P T ) . S p e c i f i c a n t i g e n s , n u c l e i c a c i d s , o r
antibodies used to diagnose viral hepatitis and laboratory tests for rare causes
o f h e p a t i t i s a r e l i s t eTda ibnl e 9 . 8 . 2
2. I ma g i n g s t u d i e s a r e n o t g e n e r a l l y i n d i c a t e d i n t h e i n i t i a l e v a l u a t i o n e xc e p t t o
e xc l u d e b i l i a r y t r a c t o b s t r u c t i o n o r s c r e e n f o r f a t t y l i v e r d i s e a s e . L i v e r b i o p s y i s
s o me t i me s n e c e s s a r y f o r b o t h d i a g n o s i s a n d e s t i ma t i o n o f p r o g n o s i s . A r e v i e w
(2) a n d a l g o r i t h 5)
m (f o r t h e e v a l u a t i o n o f a b n o r ma l l i v e r c h e mi s t r i e s a r e
available on the Internet.
D. Genetics
Genetic studies are not used in the initial evaluation of hepatitis but can be used if
a n i n h e r i t e d c o n d i t i o n i s s u s p e c t e d ( e . g . , h e r e d i t a r y h e mo c h r o ma t o s i s ) .
I V. Diagnosis
A d i f f e r e n t i a l d i a g n o s i s o f h e p a t i t i s i s Tgai vbel en 9i n. 8 . 3
1. A c u t e h e p a t i t T
i sh e c l i n i c a l p r e s e n t a t i o n c a n b e q u i t e v a r i a b l e . S o me p a t i e n t s
e xp e r i e n c e n o s y mp t o ms , w h e r e a s o t h e r s ( 1 % 2 % ) s u f f e r f u l mi n a n t d i s e a s e w i t h
l i f e - t h r e a t e n i n g l i v e r f a i1)
l u.rTea(b l e 9 . 8 . o4 u t l i n e s a c l a s s i c p r e s e n t a t i o n .
2. C h r o n i c h e p a t i tAi sn y h e p a t i t i s t h a t p e r s i s t s b e y o n d 6 mo n t h s i s c o n s i d e r e d t o
b e c h r o n i c . S y mp t o ms t e n d t o b e l e s s s e v e r e o r a b s e n t . W h e n p r e s e n t , t h e y a r e
n o n s p e c i f i c , i n t e r mi t t e n t , a n d mi l d a n d i n c l u d e f a t i g u e , ma l a i s e , p o o r
P. 2 0 0
concentration, sleeping difficulties, and right upper quadrant pain. More severe
a n o r e xi a , n a u s e a , w e a k n e s s , w e i g h t l o s s , p r u r i t u s , i c t e r u s , a n d j a u n d i c e c a n
o c c u r i n t h e s e t t i n g o f a d v a n c e d d i s e a s e o r a n a c u t e e xa c e r b a t i o n . A l t h o u g h
t y p i c a l l y l e s s s e v e r e i n p r e s e n t a t i o n , t h e i n f l a mma t i o n o f c h r o n i c h e p a t i t i s c a n
l e a d t o c i r r h o s i s a n d e n d - s t a g e l i v e r d i s e a s e . S u c h p a t i e n t s s u f f e r a b d o mi n a l
s w e l l i n g , e d e ma , e a s y b r u i s i n g , g a s t r o i n t e s t i n a l b l e e d i n g , a n d me n t a l c o n f u s i o n
( w i t h t h e o n s e t o f h e p a t i c e n c e p h a l o p a t h y ) . S e r u m a mi n o t r a n s f e r a s e l e v e l s a r e
P. 2 0 1
u s u a l l y l e s s t h a n f i v e t i me s t h e u p p e r l i mi t o f n o r ma l b u t a r e h i g h l y v a r i a b l e .
B e c a u s e o f t h e v a r y i n g s e v e r i t y o f i n f l a mma t i o n a n d t h e g r a d u a l d e c r e a s e i n
l i v e r f u n c t i o n t h a t o c c u r s i n t h e c o u r s e o f c h r o n i c h e p a t i t i s , s e r i a l e xa mi n a t i o n o f
l a b o r a t o r y t e s t s i s p r o b a b l y mo r e i mp o r t a n t t h a n t h e a b s o l u t e v a l u e s a t a n y
g i v e n t i me1)(.
341 / 652
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9.8 - Hepatitis
TAB L E 9.8.1 Com m on Laboratory Tests for Evaluating Liver

Disease
Te s t
I m plication
Note s
A LT a n d A S T
Hepatocellular
injury or necrosis
D o n o t c o r r e l a t e w i t h d i s e a s e s e v e r i t y ; A S T / A LT 2 i n
alcoholic liver disease
Direct bilirubin
Hepatobiliary
disease
Bilirubinuria occurs with conjugated (direct)

h y p e r b i l i r u b i n e mi a a n d i s a l w a y s p a t h o l o g i c
GGT
Hepatobiliary
disease
Increased by alcohol ingestion; used to confirm hepatic

origin of alkaline phosphatase
Alkaline
phosphatase
C h o l e s t a t i c d i s e a s eN o t l i v e r - s p e c i f i c ; u s e d t o r u l e o u t c h o l e s t a s i s
P r o t h r o mb i n t i me H e p a t i c f u n c t i o n
a n d a l b u mi n
Short-term and long-term indicators; not specific
A LT , a l a n i n e a mi n o t r a n s f e r a s e ; A S T , a s p a r t a t e a mi n o t r a n s f e r a s e ; G G T , - g l u t a my l t r a n s p e p t i d a s e .
F r o m G o l d ma n L , A u s i e l l o D ,Ceec
d si .l t ex t book of medi ,c i2ne
2 n d e d . P h i l a d e l p h i a , PA : S a u n d e r s ,
2 0 0 3 ; G r e e n R M , F l a mm S . A G A t e c h n i c a l r e v i e w o n t h e e v a l u a t i o n o f l i v e r c h e mi s t r y t e s t s .
G as t r oent er ol ogy
2 0 0 2 ; 1 2 3 : 1 3 6 7 1 3 8 4 . h t t p : / / w w w 2 . u s . e l s e v i e r h e a l t h . c o m/ i n s t / s e r v e ?
action=searchD B&searchD Bfor=art&artType=abs&id=agast1231367&nav=abs&special=hilite&query=
[ a l l _ f i e l d s ] ( l i v e r + c h e mi s t r y + t e s t s , ) .
TAB L E 9.8.2 Diagnostic Tests for Suspected Hepatitis

Hepatitis A
A n t i - H AV I g M
Hepatitis B
Hepatitis B surface antigen, antihepatitis B core IgM
Hepatitis C
Anti- H C V antibody ( E L IS A or R IB A), H C V R N A
U n c o mmo n
A 1 AT d e f i c i e n c y
S e r u m A 1 AT a c t i v i t y, A 1 AT p h e n o t y p i n g
A u t o i mmu n e h e p a t i t i s
A n t i n u c l e a r, a n t i s mo o t h mu s c l e , l i v e r k i d n e y mi c r o s o ma l a n t i b o d i e s
Iron overload
Serum ferritin and transferrin saturation
Wilson's disease
S e r u m c e r u l o p l a s mi n , 2 4 - h u r i n e c o p p e r
A 1 AT , 1- a n t i t r y p s i n ; E L I S A , e n zy me - l i n k e d i mmu n o s o r b e n t a s s a y ; H AV, h e p a t i t i s A v i r u s ; H C V, h e p a t i t i s C v i r u s ; R I B A , r e c o mb i n
i mmu n o b l o t a s s a y.
0 0 2 ; 1 2 3 : 1 3 6 7 1 3 8 4 . h t t p : / / w w w 2 . u s . e l s e v i e r h e a l t h . c o m/ i n s t / s e r v e ?
a c t i o n = s e a r c h D B & s e a r c h D B f o r = a r t & a r t T y p e = a b s & i d = a g a s t 1 2 3 1 3 6 7 & n a v = a b s & s p e c i a l = h i l i t e & q u e r y = [ a l l _ f i e l d s ] ( l i v e r + c h e mi s t r y + t
TAB L E 9.8.3 Differential Diagnosis of Hepatitis

Biliary
C o n d i t i o n s s u c h a s c h o l e d o c h o l i t h i a s i s , n e o p l a s m, p a n c r e a t i t i s , p r i ma r y s c l e r o s i s c h o l a n g i t i s
tract
o b s t r u c t i o n S U R G I C A L C A U S E S M U S T B E E X C L U D E D E A R LY
Liver
G r a n u l o ma t o u s l i v e r d i s e a s e : i mmu n o l o g i c a n d h y p e r s e n s i t i v i t y d i s o r d e r s ; f u n g a l , my c o b a c t e r i a l , p a r a s i t i c , o t h e r
i n f i l t r a t i o n i n f e c t i o n s ; i n f l a mma t o r y b o w e l d i s e a s e ; l y mp h o ma ; p r i ma r y b i l i a r y c i r r h o s i s ; d r u g , f o r e i g n b o d y, t o xi c r e a c t i o n s ; r h e u
diseases; sarcoidosis
I n f i l t r a t i n g ma l i g n a n c y
Other
infections
B a c t e r i a l : e h r l i c h i o s i s , g o n o c o c c a l p e r i h e p a t i t i s , L e g i o n n a i r e s ' d i s e a s e , l e p t o s p i r o s i s , l i s t e r i o s i s , L y me d i s e a s e , p y o
a b s c e s s , Q - f e v e r, R o c k y M o u n t a i n s p o t t e d f e v e r, s a l mo n e l l o s i s , s e c o n d a r y s y p h i l i s , t u l a r e mi a
342 / 652
tmdmss
9.8 - Hepatitis
Fungal: candidiasis
P a r a s i t i c : a me b i c a b s c e s s , b a b e s i a s i s , ma l a r i a , t o xo p l a s mo s i s
Vi r a l : a d e n o v i r u s , c y t o me g a l o v i r u s , E p s t e i n - B a r r v i r u s , h e r p e s s i mp l e x v i r u s
P r e g n a n c y A c u t e f a t t y l i v e r o f p r e g n a n c y, H E L L P s y n d r o me , h y p e r e me s i s g r a v i d a r u m
H E L L P s y n d r o me , h e mo l y s i s , e l e v a t e d l i v e r e n zy me s , a n d l o w p l a t e l e t c o u n t .
2 n d e d . P h i l a d e l p h i a , PA : S a u n d e r s , 2 0 0 3 ; G r e e n R M , F l a mm S . A G A
t e c h n i c a l r e v i e w o n t h e e v a l u a t i o n o f l i v e r c h e miG
s tas
r y t rt oent
e s t s .er ol ogy
2002;123:13671384.
h t t p : / / w w w 2 . u s . e l s e v i e r h e a l t h . c o m/ i n s t / s e r v e ?
a c t i o n = s e a r c h D B & s e a r c h D B f o r = a r t & a r t T y p e = a b s & i d = a g a s t 1 2 3 1 3 6 7 & n a v = a b s & s p e c i a l = h i l i t e & q u e r y = [ a l l _ f i e l d s ] ( l i v e r + c h e mi s t r y + t
TAB L E 9.8.4 Phases of Classic Acute Hepatitis

Phase
Sy m ptom s
Findings
Duration
Preicteric
A n o r e xi a , n a u s e a ,
f a t i g u e , ma l a i s e ,
vague right upper
quadrant pain
Icteric
Wo r s e n i n g o f
Jaundice, liver
13 wk
p r e i c t e r i c s y mp t o ms , t e n d e r n e s s ,
d a r k u r i n e , j a u n d i c e h y p e r b i l i r u b i n e mi a
Convalescent Gradual resolution
Greater than
310 d
tenfold elevation
o f A LT a n d A S T
G r a d u a l r e s o l u t i o n 5 mo
A LT , a l a n i n e a mi n o t r a n s f e r a s e ; A S T , a s p a r t a t e a mi n o t r a n s f e r a s e .
2nd ed.
P h i l a d e l p h i a , PA : S a u n d e r s , 2 0 0 3 .
P. 2 0 2
References
1 . G o l d ma n L , A u s i e l l o D , C
e dec
s .i l t ex t book of medi ,c i2ne
2nd ed.
P h i l a d e l p h i a , PA : S a u n d e r s , 2 0 0 3 .
2 . G r e e n R M , F l a mm S . A G A t e c h n i c a l r e v i e w o n t h e e v a l u a t i o n o f l i v e r
c h e mi s t r y t e s tGs as
. t r oent er ol ogy
2002;123:13671384.
a c t i o n = s e a r c h D B & s e a r c h D B f o r = a r t & a r t T y p e = a b s & i d = a g a s t 1 2 3 1 3 6 7 & n a v = a b s & s p e c i a l = h i l i t e & q u e r y = [ a l l _ f i e l d s ] ( l i v e r + c h e mi s t r y + t e s t s , )
3. N ational C enter for Infectious D iseases. B urden from hepatitis A, B , and C in
the United States. 2005.
h t t p : / / w w w. c d c . g o v / n c i d o d / d i s e a s e s / h e p a t i t i s / r e s o u r c e / d z_ b u r d e n 0 2 . h t m.
4 . M e r s y D J . R e c o g n i t i o n o f a l c o h o l a n d s u b s t a n cAema bFuam
s e .P hy s i c i an
2 0 0 3 ; 6 7 : 1 5 2 9 1 5 3 2 . h t t p : / / w w w. a a f p . o r g / a f p / 2 0 0 3 0 4 0 1 / 1 5 2 9 . h t ml .
5 . A me r i c a n G a s t r o e n t e r o l o g i c a l A s s o c i a t i o n me d i c a l p o s i t i o n s t a t e me n t :
e v a l u a t i o n o f l i v e r c h e mi s t r y G
t eas
s ttsr.oent er ol ogy
2002;123:13641366.
a c t i o n = s e a r c h D B & s e a r c h D B f o r = a r t & a r t T y p e = a b s & i d = a g a s t 1 2 3 1 3 6 4 & n a v = a b s & s p e c i a l = h i l i t e & q u e r y = [ a l l _ f i e l d s ] ( l i v e r + c h e mi s t r y + t e s t s , )
343 / 652
tmdmss
9.9 - Hepatomegaly
> T a b l e o f C o n t e n t s > 9 - G a s t r o i n t e s t i n a l P r o b l e ms > 9 . 9 - H e p a t o me g a l y
9.9
Hepatomegaly
Darry l G. White
I . Background
H e p a t o me g a l y i s a p h y s i c a l f i n d i n g a s s o c i a t e d w i t h h e p a t o b i l i a r y d i s e a s e . I t i s n o t
s p e c i f i c o r s e n s i t i v e t o o n e c a u s e a n d d e f i n i n g i t c a n b e e n i g ma t i c b e c a u s e o f t h e
h i g h l y v a r i a b l e l i v e r s i ze t h a t ma k e s e s t a b l i s h me n t o f w h a t c o n s t i t u t e s n o r ma l
s o me w h a t d i f f i c u l t . T h e n o r ma l a d u l t l i v e r h a s a mi d c l a v i c u l a r r a n g e o f 8 t o 1 2 c m f o
me n a n d 6 t o 1 0 c m f o r w o me n , w i t h mo s t s t u d i e s d e f i n i n g h e p a t o me g a l y a s a l i v e r
s p a n g r e a t e r t h a n 1 5 c m i n t h e mi d c l a v i c u1)l a. r l i n e (
A. Etiology
L i v e r e n l a r g e me n t c a n h a v e mu l t i p l e p a t h o p h y s i o l o g i c p a t h w a y s . E n l a r g e me n t o c c u r
i n t h e e a r l y h e p a t i c i n f l a mma t o r y r e s p o n s e t o v i r a l p a t h o g e n s , t o xi c s u b s t a n c e s , a n d
o t h e r s t i mu l a n t s a n d i s t h e n f o l l o w e d b y s c a r r i n g a n d s h r i n k i n g i n c h r o n i c c o n d i t i o n
F a t t y i n f i l t r a t i o n c a u s e s e n l a r g e me n t i n o b e s i t y a n d me t a b o l i c s y n d r o me . Va s c u l a r
congestion results in swelling in acute and chronic heart diseases as well as in
c o n d i t i o n s o f d e c r e a s e d v a s c u l a r o u t f l o w. F o c a l l y e n l a r g i n g l e s i o n s s u c h a s
v a s c u l a r c y s t s , i n f e c t i o u s c y s t s , a n d c a n c e r o u s g r o w t h s a l s o o c c u r. A b n o r ma l
d e p o s i t i o n o f a my l o i d , l i p i d s , o r i r o n c a n a l s o r e s u l t i n l i v e r e n l a r g e me n t .
B. Epidemiology
T h e i n c i d e n c e o f h e p a t o me g a l y h a s n o t b e e n s t u d i e d i n a n y l a r g e p o p u l a t i o n g r o u p .
H o w e v e r, a r e c e n t s u mma r y a r t i c l e o f mu l t i p l e s t u d i e s f o u n d t h a t a p a l p a b l e l i v e r
ma r g i n h a d a l i k e l i h o o d r a t i o o f 2 . 5 ( c o n f i d e n c e i n t e r v a l [ C I ] , 2 . 2 2 . 8 ) f o r
h e p a t o me g a l y a n d a n o n p a l p a b l e l i v e r ma r g i n h a d a n e g a t i v e l i k e l i h o o d r a t i o o f 0 . 4 5
( C I , 0 . 3 8 0 . 5 22)) ..(
I I I . Evaluation
A. History
I n i t i a l e v a l u a t i o n o f h e p a t o me g a l y c e n t e r s o n t w o q u e s t i o n s : D o e s t h e p a t i e n t h a v e
k n o w n r i s k f a c t o r s f o r l i v e r d i s e a sTea b( sl ee e9 . 9 ). ?1 D o e s t h e p a t i e n t h a v e
s y mp t o ms a s s o c i a t e d w i t h l i v e r d i s e a sTea b( lsee e9 . 9 ). ?2
344 / 652
tmdmss
9.9 - Hepatomegaly
E xa mi n a t i o n o f t h e l i v e r i s d i f f i c u l t g i v e n i t s i r r e g u l a r s h a p e a n d i t s l o c a t i o n w i t h i n
a b d o me n .
1. I t i s t y p i c a l t o u s e o n e o f t h e t w o d i r e c t i o n s f o r p a l p a t i o n o f t h e r i g h t u p p e r
quadrant: palpate from below using the fingertips to palpate superiorly or from
a b o v e w i t h t h e f i n g e r t i p s h o o k e d o v e r t h e l o w e r r i b . E i t h e r me t h o d i s f a c i l i t a t e d
b y t h e p a t i e n t ' s d e e p i n s p i r a t i o n . P a l p a t i o n mu s t i n c l u d e t h e mi d l i n e t o i d e n t i f y
a n e n l a r g e d l e f t l o b e o f t h e l i v e r. O n p a l p a t i o n , n o t e t h e l i v e r p o s i t i o n , t h e e xt e
o f i t s p a l p a t i o n b e l o w t h e c o s t a l ma r g i n , a n d i t s t e xt u r e a n d c o n s i s t e n c y. P a l p a t
P. 2 0 3
f o r t h e l o w e r e d g e a n d p e r c u s s f o r t h e u p p e r ma r g i n . T h e s e t w o p o i n t s g i v e t h e
h i g h e s t a c c u r a c y i n e s t i ma t i n g l i v e3)r sI fi ze
t h.e( ma r g i n i s n o t p a l p a t e d b u t
h e p a t o me g a l y i s s u s p e c t e d , a t t e mp t d i r e c t p e r c u s s i o n o f b o t h ma r g i n s . T h e
s c r a t c h me t h o d ( g e n t l y s t o k i n g o r s c r a t c h i n g t h e s k i n s u r f a c e i n a p a r a l l e l
plane while listening with the stethoscope for change in sound and intensity of
f r e q u e n c y ) h a s b e e n u s e d t o i d e n t i f y ma r g i n s : h o w e v e r, a r e c e n t s t u d y
c o mp a r i n g u l t r a s o u n d t o t h e r e s u l t s o f t h e s c r a t c h t e s t f o u n d t h a t t h i s t e s t w a s
u n r e l i a b l e a n d i n a c c u r4)
ate.(
TAB L E 9.9.1 Risk Factors for Liver Disease

Acupuncture
Alcoholism
B i s e xu a l i t y
Blood product
transfusion
Dietary
s u p p l e me n t s
F a mi l y h i s t o r y l i v e r
disease
Gallstones
Gastrointestinal
bleeding
Irritable bowel
disease
S e xu a l l y t r a n s mi t t e d
disease
M a l e h o mo s e xu a l i t y I n t e r n a t i o n a l t r a v e l
Tattoos
2. A u s c u l t a t i o n o f t h e r i g h t u p p e r q u a d r a n t h a s p o o r c l i n i c a l u t i l i t y. O t h e r p h y s i c a l
e xa mi n a t i o n f i n d i n g s c o n s i s t e n t w i t h l i v e r d i s e a s e i n c l u d e j a u n d i c e , v a s c u l a r
s p i d e r s , p a l ma r e r y t h e ma , g y n e c o ma s t i a , a s c i t e s , s p l e n o me g a l y, t e s t i c u l a r
a t r o p h y, p e r i p h e r a l e d e ma , D u p u y t r e n ' s c o n t r a c t u r e , p a r o t i d e n l a r g e me n t , a n d
e n c e p h a l o p a t h y. A l t h o u g h n o n e o f t h e s e p h y s i c a l e xa mi n a t i o n s i g n s a r e
p a t h o g n o mo n i c f o r h e p a t o b i l i a r y d i s e a s e , t h e i r p r e s e n c e p r o mp t s f u r t h e r
diagnostic tests for hepatic disease.
345 / 652
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9.9 - Hepatomegaly
C. Testing
D i a g n o s t i c t e s t s s h o u l d i n c l u d e c o mp u t e d t o mo g r a p h y ( C T ) s c a n o f t h e r i g h t u p p e r
q u a d r a n t a n d i n i t i a l l a b o r a t o r y t e s t s ( c o mp l e t e b l o o d c o u n t , s e r u m b l o o d t e s t s
[ C H E M - 7 ] ) , l i v e r e n zy me t e s t s ( a s p a r t a t e a mi n o t r a n s f e r a s e , a l a n i n e
a mi n o t r a n s f e r a s e , - g l u t a my l t r a n s p e p t i d a s e , a l k a l i n e p h o s p h a t a s e ) , a n d t r u e l i v e r
f u n c t i o n t e s t s ( a l b u mi n , p r o t h r o mb i n t i me , p a r t i a l t h r o mb o p l a s t i n t i me , a n d b i l i r u b i n )
I f l i v e r e n zy me s a r e e l e v a t e d , p r o c e e d w i t h h e p a t i t i s s e r o l o g y. U l t r a s o u n d c a n b e
used when C T scan is contraindicated or not available. Further testing used to
e l u c i d a t e t h e d i f f e r e n t i a l d i a g n o s i s i s Tl iasbt el ed 9i n. 9 . 3
I V. Diagnosis
T h i s c a n b e a p p r o a c h e d o n t h e b a s i s o f t h e f i n d i n g s o f p h y s i c a l e xa mi n a t i o n .
1. S mo o t h n o n t e n d e r l i v e r. S u s p e c t f a t t y i n f i l t r a t i o n , c o n g e s t i v e h e a r t f a i l u r e
( C H F ) , p o r t a l c i r r h o s i s , l y mp h o ma , p o r t a l o b s t r u c t i o n , h e p a t i c v e n o u s
t h r o mb o s i s , h e p a t i c v e i n t h r o mb o s i s , l y mp h o c y s t i c l e u k e mi a , a my l o i d o s i s ,
s c h i s t o s o mi a s i s , o r k a l a - a za r.
2. S mo o t h t e n d e r l i v e r. S u s p e c t e a r l y C H F, a c u t e h e p a t i t i s , a mo e b i c a b s c e s s , o r
hepatic abscess.
3. N o d u l a r l i v e r. S u s p e c t l a t e p o r t a l c i r r h o s i s , t e r t i a r y s y p h i l i s , h y d a t i d c y s t , o r
me t a s t a t i c c a r c i n o ma .
4. Ve r y h a r d n o d u l a r l i v e r. T h i s n e a r l y a l w a y s i n d i c a t e s me t a s t a t i c c a r c i n o ma .
TAB L E 9.9.2 Sym ptom s of Liver Disease

A b d o mi n a l p a i n P r u r i t u s
Nausea
Loss of appetite
Confusion
Arthralgias
Rashes
Night sweats
Fatigue
Diarrhea
Chills
Sleep
disturbance
Gastric
bleeding
Fever
E a s y b r u i s a b i l i t y Vo mi t i n g
Myalgia
Heavy
me n s e s
Frequent
e p i s t a xi s
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TAB L E 9.9.3 Specific Tests to Evaluate

hepatom egaly
Sugge ste d
diagnosis
Te s t
A u t o i mmu n e
hepatitis
S mo o t h mu s c l e a n t i b o d y, a n t i l i v e r k i d n e y
mi c r o s o ma l a n t i b o d y
Biliary cirrhosis
A n t i mi t o c h o n d r i a l a n t i b o d y
Ferritin
Wilson's disease
C e r u l o p l a s mi n
Hepatocellular
cancer
- F etoprotein
1 - A n t i t r y p s i n
deficiency
E n zy me a s s a y o f t h e s a me
P. 2 0 4
T h e c l i n i c a l ma n i f e s t a t i o n s o f h e p a t o me g a l y, a l t h o u g h s o me w h a t i l l d e f i n e d , s h o u l d
p r o mp t a t h o r o u g h e v a l u a t i o n a n d w o r k u p . A p p r o p r i a t e d i a g n o s t i c t e s t s g e n e r a l l y
provide a quick and accurate diagnosis. New therapies for chronic liver diseases
ma k e e a r l y d i a g n o s i s c r i t i c a l f o r o b t a i n i n g g o o d r e s u l t s .
References
1 . U n a l B , B i l g i l i Y, K o c a c i k l i E , e t a l . S i mp l e e v a l u a t i o n o f l i v e r s i ze o n e r e c t
a b d o mi n a l p l a i n r a d i o g r aCp lhi y.
n R adi ol2 0 0 4 ; 5 9 : 11 3 2 11 3 5 .
2 . S e u p a u l R A , C o l l i n s R . P h y s i c a l e xa mi n a t i o n o f At nn
he E
l i mer
v e r. g M ed
2005;45:553555.
3 . N a y l o r C D . P h y s i c a l e xa mi n a t i o n o f t hJeA lM
i vAe1r.9 9 4 ; 2 7 : 1 8 5 9 1 8 6 5 .
4. Tucker WN, Saab S, Leland S R, et al. T he scratch test is unreliable for
d e t e r mi n i n g t h e l i v e r e Jd gCel. i n G as t r oent er1ol
997;25:410414.
347 / 652
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9.10 - Jaundice
> T a b l e o f C o n t e n t s > 9 - G a s t r o i n t e s t i n a l P r o b l e ms > 9 . 1 0 - J a u n d i c e
9.10
Jaundice
R o n a l d F. D o m m e r m u t h
I . Background
J a u n d i c e r e f e r s t o a y e l l o w i s h d i s c o l o r a t i o n o f t h e s k i n , s c l e r a e , a n d mu c o u s
me mb r a n e s c a u s e d b y t h e d e p o s i t i o n o f b i l e p i g me n t s . T y p i c a l l y, p h y s i c a l
e xa mi n a t i o n i s u n a b l e t o d e t e c t e l e v a t i o n s o f b i l i r u b i n u n t i l t h e s e r u m l e v e l e xc e e d s
2 . 0 t o 3 . 0 mg / d L .
A. Etiology
B i l i r u b i n i s f o r me d p r i ma r i l y t h r o u g h t h e me t a b o l i c b r e a k d o w n o f h e me r i n g s ,
p r e d o mi n a n t l y f r o m t h e c a t a b o l i s m o f r e d b l o o d c e l l s . D y s f u n c t i o n o f a n y o f t h e
p h a s e s o f b i l i r u b i n me t a b o l i s m ( p r e h e p a t i c , i n t r a h e p a t i c , o r p o s t h e p a t i c ) c a n l e a d t o
j a u n d i c e . N o r ma l l y, 9 6 % o f p l a s ma b i l i r u b i n i s u n c o n j u g a t e d ( i n d i r e c t ) . W h e n t h e
p l a s ma e l e v a t i o n i s c a u s e d p r e d o mi n a n t l y b y u n c o n j u g a t e d b i l i r u b i n , t h e d e f e c t i s
l i k e l y t o b e t h e r e s u l t o f o v e r p r o d u c t i o n , i mp a i r e d h e p a t i c u p t a k e , o r a b n o r ma l i t i e s
c o n j u g a t i o n . W h e n t h e p l a s ma e l e v a t i o n i n c l u d e s a s u b s t a n t i a l c o n t r i b u t i o n f r o m
conjugated (direct) bilirubin, then hepatocellular disease, defective canalicular
e xc r e t i o n , a n d b i l i a r y o b s t r u c t i o n a r e t h e mo r e l i k e l y c a u s e s .
P. 2 0 5
TAB L E 9.10.1 Medications Associated with the

Developm ent of Jaundice
Alcohol
Diuretics
A c e t a mi n o p h e n
Cholesterol-lowering agents
Anticonvulsants
S e x h o r mo n e s
P s y c h o t r o p i c me d i c a t i o n s
Antibiotics
Diuretics
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9.10 - Jaundice
O r a l h y p o g l y c e mi c s
Inhaled anesthetics
C h e mo t h e r a p y a g e n t s
H e r b a l c o mp o u n d s
B. Epidemiology
A mo n g a d u l t s p r e s e n t i n g w i t h j a u n d i c e , s e r i o u s u n d e r l y i n g d i s e a s e i s c o mmo n . I n
o n e s t u d y, 2 0 % o f p a t i e n t s w i t h j a u n d i c e h a d p a n c r e a t i c o r b i l i a r y c a r c i n o ma , 1 3 %
e xp e r i e n c e d g a l l s t o n e d i s e a s e a n d 1 0 % s u f f e r e d f r o m a l c o h o 1)
l i c. c i r r h o s i s (
J a u n d i c e r a r e l y r e p r e s e n t s a me d i c a l e me r g e n c y b u t c a n p r e s e n t a s ma s s i v e
h e mo l y s i s , k e r n i c t e r u s , a s c e n d i n g c h o l a n g i t i s , a n d f u l mi n a n t h e p a t i c f a i l u r e .
I I I . Evaluation
A. History
1. S u d d e n o n s e t i s c o n s i s t e n t w i t h v i r a l h e p a t i t i s , a c u t e b i l i a r y o b s t r u c t i o n , t r a u ma
o r t o xi n - me d i a t e d f u l mi n a n t l i v e r f a i l u r e . M o r e g r a d u a l o n s e t i s i n d i c a t i v e o f
c h r o n i c l i v e r d i s e a s e ( i n c l u d i n g a l c o h o l i c c i r r h o s i s ) o r ma l i g n a n c y. A l i f e l o n g
h i s t o r y s u g g e s t s a n i n h e r i t e d me t a b o l i c o r h e mo l y t i c c a u s e . A c h o l i c s t o o l s , d a r k
u r i n e , a n d r i g h t u p p e r q u a d r a n t ( R U Q ) p a i n , e s p e c i a l l y f o l l o w i n g a f a t t y me a l ,
should suggest cholestasis or cholelithiasis. A history of fever or prior biliary
s u r g e r y, e s p e c i a l l y i f R U Q p a i n i s p r e s e n t , p o i n t s t o c h o l a n g i t i s . C h a r c o t ' s t r i a d
o f c h o l a n g i t i s i n c l u d e s f e v e r, R U Q p a i n , a n d j a u n d i c e . I n t h e p r e s e n c e o f a
h i s t o r y o f a n o r e xi a , ma l a i s e , o r my a l g i a s , a v i r a l e t i o l o g y s h o u l d b e c o n s i d e r e d .
T y p i c a l l y, p r u r i t u s o r w e i g h t l o s s i s a s s o c i a t e d w i t h n o n i n f e c t i o u s e t i o l o g i e s s u c
a s p r i ma r y b i l i a r y c i r r h o s i s i n w h i c h p r u r i t u s i s c o mmo n l y t h e i n 2)
i t i.a l s y mp t o m (
N u me r o u s me d i c a t i o n s c a n i n d u c e h e p a t o c e l l u l a r i n j u r y o r c h o l e s t a s i s ( s e e
T a b l e 9 . 1 0). .1
2. B l o o d t r a n s f u s i o n s , i n t r a v e n o u s d r u g u s e , s e xu a l c o n t a c t s , t r a v e l t o e n d e mi c
r e g i o n s , o r i n g e s t i o n o f c o n t a mi n a t e d f o o d s c a n a l s o e xp o s e p a t i e n t s t o v i r a l r e l a t e d h e p a t o c e l l u l a r i n j u r y. P a s t me d i c a l h i s t o r y a n d s u r g i c a l h i s t o r y, p r i o r
e p i s o d e s o f j a u n d i c e , a n d a h i s t o r y o f a r h e u ma t o l o g i c d i s e a s e o r i n f l a mma t o r y
b o w e l d i s e a s e s h o u l d b e e xa mi n e d . F a mi l y h i s t o r y ma y r e v e a l a n i n h e r i t e d
d e f e c t i n b i l i r u b i n c o n j u g a t i o n o r t r a n s p o r t , W i l s o n ' s1- dainstei tar sy ep,s in
d e f i c i e n c y, o r h e mo c h r o ma t o s e s .
I n a d d i t i o n t o i c t e r u s , e xa mi n a t i o n f i n d i n g s o f a s c i t e s , s p l e n o me g a l y, s p i d e r
a n g i o ma s , c a p u t me d u s a , g y n e c o ma s t i a , t e s t i c u l a r a t r o p h y, p a l ma r e r y t h e ma , o r
D upuytren's contracture suggest chronic liver disease and portal hypertension.
A l t e r e d c o n s c i o u s n e s s a n d a s t e r i xi s p o i n t t o w a r d l i v e r. K a y s e r - F l e i s c h e r r i n g s a r e
seen in Wilson's disease, and C ourvoisier's sign (a painless palpable gall bladder)
s u g g e s t i v e o f p a n c r e a t i c c a n c e r.
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P. 2 0 6
C. Testing
1. I n i t i a l l a b o r a t o r y t e s t i n g f o r a j a u n d i c e d p a t i e n t i n c l u d e s a c o mp l e t e b l o o d c o u n
a n d p e r i p h e r a l s me a r, a f r a c t i o n a t e d s e r u m b i l i r u b i n ( t o t a l a n d d i r e c t ) , a n d a
u r i n a l y s i s . A d d i t i o n a l t e s t i n g s h o u l d i n c l u d e a s p a r t a t e a mi n o t r a n s f e r a s e / a l a n i n e
a mi n o t r a n s f e r a s e ( A S T / A LT ) , - g l u t a my l t r a n s p e p t i d a s e , a n d a l k a l i n e
p h o s p h a t a s e . P u r e l i v e r f u n c t i o n s h o u l d b e e v a l u a t e d b y o b t a i n i n g a n a l b u mi n
a n d a p r o t h r o mb i n t i me .
2. F u r t h e r l a b o r a t o r y t e s t i n g a n d i ma g i n g a r e p r e d i c a t e d o n o b t a i n i n g t h e i n i t i a l
r e s u l t s a n d ma y i n c l u d e s e r o l o g i c t e s t i n g f o r v i r a l h e p a t i t i s , a u t o i mmu n e ma r k e r
( a n t i mi t o c h o n d r i a l a n t i b o d i e s : p r i ma r y b i l i a r y c i r r h o s i s , o r a n t i s mo o t h mu s c l e
a n t i b o d i e s a n d a n t i mi c r o s o ma l a n t i b o d i e s : a u t o i mmu n e h e p a t i t i s ) , s e r u m i r o n ,
t r a n s f e r r i n g a n d f e r r i t i n ( h e mo c h r o ma t o s i s ) , c e r u l o p l a s mi n ( W i l s o n ' s d i s e a s e ) ,
a n d 1- a n t i t r y p s i n a c t i v i t1y- a( nt i t r y p s i n d e f i c i e n c y ) . U l t r a s o u n d a n d c o mp u t e d
t o mo g r a p h y c a n h e l p d i s t i n g u i s h o b s t r u c t i v e d i s e a s e f r o m h e p a t o c e l l u l a r
disease.
I V. Diagnosis
H i s t o r i c a l f e a t u r e s c o r r o b o r a t e d b y p h y s i c a l e xa mi n a t i o n a n d l a b o r a t o r y s t u d i e s
should distinguish obstructive causes from nonobstructive causes, differentiate
a c u t e l y p r e s e n t i n g c o n d i t i o n s f r o m mo r e c h r o n i c d i s e a s e s , a n d d i s c r i mi n a t e
u n c o n j u g a t e d a n d c o n j u g a t e d s o u r c e s o f b i l i r uTbaibnl e( s9e. e1 0). .2 D u b i n - J o h n s o n
a n d R o t o r ' s s y n d r o me a r e i n h e r i t e d d i s o r d e r s t h a t a f f e c t t r a n s p o r t a t i o n o f c o n j u g a t e
bilirubin.
R e s u l t s o f t h e p a t i e n t ' s a mi n o t r a n s f e r a s e s f u r t h e r r e f i n e t h e d i f f e r e n t i a l d i a g n o s i s .
Hepatocellular injury is distinguished from cholestasis by substantial elevations in
t h e a mi n o t r a n s f e r a s e s ( A S T / A LT ) . A r a t i o o f A S T / A LT o f > 2 . 0 s u g g e s t s a l c o h o l i c
l i v e r d i s e a s e . N o r ma l a mi n o t r a n s f e r a s e s a r g u e a g a i n s t h e p a t o c e l l u l a r i n j u r y a n d
increase the
P. 2 0 7
l i k e l i h o o d t h a t h e mo l y s i s o r a d i s o r d e r o f b i l i r u b i n p r o c e s s i n g e xi s t s . H o w e v e r, t h i s
c a n b e mi s l e a d i n g i n c h r o n i c l i v e r d i s e a s e w h e n t h e r e i s l i t t l e l i v e r p a r e n c h y ma l e f t
for being injured.
TAB L E 9.10.2 Differential Diagnosis of Jaundice

Disorde rs of unconjugate d hy pe rbilirubine m ia
Ov e rproduction
Intravascular
h e mo l y s i s
I m paire d uptake
Congestive heart
failure
I m paire d conjugation
C r i g l e r - N a j j a r s y n d r o me ,
types I and II
350 / 652
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9.10 - Jaundice
E xt r a v a s c u l a r
h e mo l y s i s
Cirrhosis
D yserythropoiesis G ilbert's disease

Medication
Advanced cirrhosis
Wilson's disease
Antibiotics
Disorde rs of conjugate d hy pe rbilirubine m ia

Ex trahe patic
chole stasis
Sclerosing
cholangitis
I ntrahe patic
chole stasis
Vi r a l h e p a t i t i s
He patoce llular injury

S eeTable 9.10.1
C h o l e d o c h o l i t h i a s i sA l c o h o l i c h e p a t i t i s
Pancreatitis
Nonalcoholic
steatohepatitis
T u mo r s
Biliary cirrhosis
AID S
cholangiopathy
T o xi n s
Strictures
Infiltrating diseases
P a r a s i t i c i n f e c t i o n sT o t a l p a r e n t e r a l
nutrition
Pregnancy
End-stage hepatic
disease
A I D S , a c q u i r e d i mmu n o d e f i c i e n c y s y n d r o me .
References
1 . R e i s ma n Y, G i p s G H , L a v e l l e S M , e t a l . C l i n i c a l p r e s e n t a t i o n o f ( s u b c l i n i c a l )
j a u n d i c eH. epat ogas t r oent er ol ogy
1 9 9 6 ; 4 3 : 11 9 0 .
2 . L e u s c h n e r U . P r i ma r y b i l i a r y c i r r h o s i s - p r e s e n t a t i o n a n dCdl ii ni
a gcnso isni s .
L i v er D i s eas2e0 0 3 ; 7 : 7 4 1 .
351 / 652
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352 / 652
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9.11 - Rectal Bleeding
> T a b l e o f C o n t e n t s > 9 - G a s t r o i n t e s t i n a l P r o b l e ms > 9 . 11 - R e c t a l B l e e d i n g
9.11
Rectal Bleeding
Christine Rom ascan
I . Background
P r i ma r y c a r e p h y s i c i a n s a r e f r e q u e n t l y f a c e d w i t h t h e c l i n i c a l q u a n d a r y o f r e c t a l
b l e e d i n g i n t h e o u t p a t i e n t o r e me r g e n c y d e p a r t me n t s e t t i n g . T h e s p e c t r u m o f r e c t a l
b l e e d i n g c a n r a n g e f r o m j u s t a f e w d r o p s o f b l o o d o n t h e t o i l e t p a p e r t o mo r e
o mi n o u s s y mp t o ms o f h e ma t o c h e zi a o r me l e n a . M a n y p a t i e n t s w i t h r e c t a l b l e e d i n g d
n o t s e e k h e a l t h c a r e f o r t h i s p r o1)b.l e m (
Re ctal ble e ding is ty pically a sign of local m ucosal or v ascular dise ase , but it
c a n a l s o s i g n i f y p r o x i m a l i n t e s t i n a l d i s e a s e i n t h e s m a l l o r. lS
a rcgaen t b o w e l
o r mi n i ma l r e c t a l b l e e d i n g i s a c o mmo n p r o b l e m p r e s e n t i n u p t o 1 5 % o f t h e
p o p u l a t i o n . H a r d , c o mp a c t e d s t o o l s a s s o c i a t e d w i t h p o o r d i e t a n d b o w e l h a b i t s
t r a u ma t i ze l o c a l v e n o u s a n d mu c o s a l t i s s u e s . P h y s i o l o g i c s t a t e s s u c h a s p r e g n a n c y
c a n i n h i b i t v e n o u s b l o o d f l o w i n t h e p e l v i s a n d l e a d t o h e mo r r h o i d f o r ma t i o n .
B l e e d i n g f r o m s o u r c e s p r o xi ma l t o t h e d u o d e n u m t e n d t o p r o d u c e s t o o l s t h a t a r e
b l a c k a n d t a r r y b e c a u s e o f me t a b o l i s m o f h e me .
I I I . Evaluation
T h e i n i t i a l e v a l u a t i o n s h o u l d d e t e r mi n e t h e a c u i t y a n d h e mo d y n a mi c s t a b i l i t y o f t h e
patient and establish the origins of the bleeding as upper or lower gastrointestinal
tract in nature. Characteristics of anorectal versus other gastrointestinal sources o
b l e e d i n g a r e c o mp a r e T
d ai bnl e 9 . 11 .(2,3,4)
1
.
TAB L E 9.11.1 Factors Associated with Anorectal

versus Other Sources of Gastrointestinal Bleeding
Suspe cte d anore ctal dise ase (he m orrhoids, anal fissure , proctitis,
m alignancy )
Age < 50 y
A mo u n t o f b l e e d i n g mi n i ma l ( b r i g h t r e d d r o p s : o n t o i l e t p a p e r, s t o o l o r i n t o i l e t
bowl only)
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Constipation or diarrhea
Pain while defecating
A n a l s y mp t o ms ( p a i n , p r u r i t u s , p r o l a p s e , p r o t r u s i o n s )
F r o m B o u n d s B , F r i e d ma n L . L o w e r g a s t r o i n t e s t i n a lGbas
l eterdoent
i n g .er ol
C l i n 2 0 0 3 ; 3 2 : 11 0 7 11 2 5 ; G o p a l D . D i s e a s e s o f t h e r e c t u m a n d a n u s : a
c l i n i c a l a p p r o a c h t o c o mmo n d i s oCr dl ienr sC. or ner s t one
2002;4:3448;
Peter D, Dougherty J . Evaluation of the patient with gastrointestinal
b l e e d i n g : a n e v i d e n c e - b a s e d a p p rEomer
a c hg. M ed C l i n N or t h A m
1999;17:239261.
P. 2 0 8
A. History
I n v e s t i g a t e a b o u t a n y c o mo r b i d i l l n e s s s u c h a s l i v e r d i s e a s e , k i d n e y d i s e a s e , u l c e r s
o r c a n c e r. W h a t a r e t h e d i e t a r y i n f l u e n c e s , s u c h a s f i b e r c o n t e n t o r f o o d i n g e s t i o n
( t o xi n s o r i n f e c t i o n s ) , t h a t c o u l d b e o c c u r r i n g ? I s t h e p a t i e n t t a k i n g a n y me d i c a t i o n s
known to increase the risk of gastrointestinal bleed such as nonsteroidal antii n f l a mma t o r y d r u g s , a s p i r i n , w a r f a r i n , t o b a c c o , o r a l c o h o l ? H a s t h e p a t i e n t e n g a g e d
i n a n o r e c e p t i v e i n t e r c o u r s e o r b e e n e xp o s e d t o s e xu a l l y t r a n s mi t t e d d i s e a s e s ? A r e
g e n i t o u r i n a r y s y mp t o ms s u c h a s u r i n a r y p a i n o r p r o s t a t i3,4)
t i s ?p r e s e n t (
1. I t i s i mp o r t a n t t o c h e c k t h e f o l l o w i n g :
a. Vi t a l s i g n s , o r t h o s t a t i c a s s e s s me n t t i l t s
b. S k i n p a l l o r, j a u n d i c e , o r t u r g o r
c. A b d o mi n a l t e n d e r n e s s , ma s s e s
d. A s c i t e s a n d / o r h e p a t o s p l e n o me g a l y
e. G e n i t o u r i n a r y e xa mi n a t i o n
f. R e c t a l e xa mi n a t i o n : e xt e r n a l v i s u a l i n s p e c t i o n , d i g i t a l r e c t a l e xa mi n a t i o n ,
s t o o l a s s e s s me n t
2. P o t e n t i a l h e mo d y n a mi c c o mp r o mi s e w a r r a n t s v o l u me r e s u s c i t a t i o n w i t h t w o
large-bore intravenous lines, preferably in an intensive care setting.
C. Testing
1. I n i t i a l l a b o r a t o r y t e s t s a s s e s s h e mo d y n a mi c s t a b i l i t y a n d c l o t t i n g a b i l i t y.
C o n s i d e r p e r f o r mi n g t h e f o l l o w i n g t e s t s : c o mp l e t e b l o o d c o u n t t o a s s e s s f o r
a n e mi a , t y p e a n d s c r e e n , s t o o l H e mo c c u l t t e s t , s e r u m b l o o d u r e a n i t r o g e n a n d
creatinine, and coagulation panel.
2. I ma g i n g s t u d i e s c a n b e d i r e c t e d a t t h e mo s t l i k e l y s o u r c e o f b l e e d i n g . Yo u n g e r
p a t i e n t s w i t h i n t e r mi t t e n t mi l d r e c t a l b l e e d i n g c a n b e e v a l u a t e d w i t h a n o s c o p y o
f l e xi b l e s i g mo i d o s c o p y. P e r s o n s o l d e r t h a n 5 0 y e a r s o f a g e w i t h p a i n l e s s
354 / 652
tmdmss
b l e e d i n g a r e a s s u me d t o h a v e c o l o n c a n c e r u n t i l p r o v e d o t h e r w i s e b y
c o l o n o s c o p y, e v e n i f t h e r e c t a l e xa mi n a t i o n i s a b n o r ma l .
D. Genetics
A f a mi l y h i s t o r y o f g a s t r o i n t e s t i n a l i l l n e s s s u c h a s i n f l a mma t o r y b o w e l d i s e a s e
(C rohn's or ulcerative colitis), polyposis, or cancer significantly increases the
l i k e l i h o o d o f i n h e r i t e d r e c t a l d i 4)
s e.a s e (
I V. Diagnosis
Differential diagnosis includes any upper or lower gastrointestinal tract bleeding
s o u r c e f r o m i n f l a mma t o r y b o w e l d i s e a s e , d i v e r t i c u l a r b l e e d i n g , p o l y p o s i s ,
ma l i g n a n c y, i n f e c t i o n , t r a u ma , o r c o a g u l o p a t h y.
C l i n i c a l a s s e s s me n t o f t h e p a t i e n t s h o u l d a l w a y s b e g i n w i t h e v a l u a t i o n o f
h e mo d y n a mi c s t a t u s . I f r e c t a l b l e e d i n g i s mi n i ma l o r h a s s t o p p e d , a n d t h e p a t i e n t i s
s t a b l e , e v a l u a t i o n c a n o c c u r i n t h e o u t p a t i e n t s e t t i n g . B y f a r, t h e mo s t c o mmo n
a n o r e c t a l b l e e d i n g s o u r c e i s h e mo r r h o i d a l d i s e a s e . R i s k s o f h e mo r r h o i d s i n c r e a s e
w i t h a d v a n c i n g a g e , p r e g n a n c y, p r o l o n g e d s i t t i n g o r s t a n d i n g , c h r o n i c d i a r r h e a o r
c o n s t i p a t i o n . A s s o c i a t e d c o mp l a i n t s f r o m h e mo r r h o i d s i n c l u d e a n a l p r u r i t u s , p a i n a n
p r o l a p s e . O t h e r a n o r e c t a l s o u r c e s o f mi n i ma l b l e e d i n g i n c l u d e a n a l f i s s u r e s , p o l y p s
p r o c t i t i s , r e c t a l u l c e r s , a n d c a3)n.c e r (
References
1. Talley N J , J ones M . S elf-reported rectal bleeding in a U nited S tates
c o mmu n i t y : p r e v a l e n c e , r i s k f a c t o r s , a n d h e a l t h c a rAe ms eJe k i n g .
G as t r oent er ol
1998;93:21792183.
2 . B o u n d s B , F r i e d ma n L . L o w e r g a s t r o i n t e s t i n a l G
b las
e etdr oent
i n g . er ol C l i n
2 0 0 3 ; 3 2 : 11 0 7 11 2 5 .
3 . G o p a l D . D i s e a s e s o f t h e r e c t u m a n d a n u s : a c l i n i c a l a p p r o a c h t o c o mmo n
d i s o r d e r sC. l i n C or ner s t one
2002;4:3448.
4. Peter D, Dougherty J . Evaluation of the patient with gastrointestinal bleeding:
a n e v i d e n c e b a s e d a p p r oE
a cmer
h . g M ed C l i n N or t h 1A9m9 9 ; 1 7 : 2 3 9 2 6 1 .
355 / 652
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9.12 - Steatorrhea
> T a b l e o f C o n t e n t s > 9 - G a s t r o i n t e s t i n a l P r o b l e ms > 9 . 1 2 - S t e a t o r r h e a
9.12
Steatorrhea
C h a r l e s L . B r y n e r J r.
I . Background
S t e a t o r r h e a , t h e t h i r d mo s t c o mmo n c a u s e o f c h r o n i c d i a r r h e a , a r i s e s f r o m t h e
ma l a b s o r p t i o n o f f a t . B y d e f i n i t i o n , i t i s t h e e xc r e t i o n o f mo r e t h a n 7 g o f f a t i n t h e
s t o o l d u r i n g a 2 4 - h o u r p e r i o d w h i l e t h e p a t i e n t i s o n a d i e t c o n t a i n i n g n o mo r e t h a n
1 0 0 g o f f a t p e r d a y. I t ma n i f e s t s a s a h i s t o r y o f g r e a s y, f o u l - s me l l i n g s t o o l s t h a t
leave an oily residue in the toilet bowl, increased flatulence, and weight loss.
A. Etiology
T h e p r o b l e m o f f a t ma l a b s o r p t i o n ma y s t e m f r o m a n i n t r a l u mi n a l d i g e s t i v e d i s o r d e r,
mu c o s a l b l o c k a g e o f a b s o r p t i o n a t t h e e n t e r o c y t e l e v e l , o r a p o s t mu c o s a l b l o c k a g e .
T h e p o s t mu c o s a l b l o c k a g e s ( e . g . , i n t e s t i n a l l y mp h a n g i e c t a s i s o r a c a n t h o c y t o s i s ) a r
q u i t e u n c o mmo n a n d a r e b e y o n d t h e s c o p e o f t h i s b r i e f d i s c u s s i o n .
1. A c u t e o r c h r o n i c l i v e r d i s e a s e ma y c a u s e s t e a t o r r h e a . I mp a i r e d s y n t h e s i s o r
i mp a i r e d e xc r e t i o n o f c o n j u g a t e d b i l e s a l t s r e s u l t s i n a b i l e s a l t d e f i c i e n c y a n d
ma l d i g e s t i o n o f f a t s . H e p a t i t i s , p r i ma r y b i l i a r y c i r r h o s i s , a l c o h o l - i n d u c e d l i v e r
d i s e a s e s , o r e xt r a h e p a t i c b i l i a r y o b s t r u c t i o n ( i n c l u d i n g c a n c e r o f t h e h e a d o f t h e
p a n c r e a s ) ma y b e t h e c a u s e s . P r o l o n g e d , s e v e r e c a r d i a c f a i l u r e o r
h e mo c h r o ma t o s i s c a n c a u s e c i r r h o s i s , w h i c h c a n p r o d u c e s t e a t o r r h e a .
2. T h e s h o r t b o w e l s y n d r o me i s a w e l l - k n o w n c a u s e o f s t1)
e aat onrdr hceaan (
follow actual resection of the ileum or arise from conditions that render large
p o r t i o n s o f t h e s ma l l b o w e l n o n f u n c t i o n a l , s u c h a s C r o h n ' s d i s e a s e ,
c h e mo t h e r a p y, o r r a d i a t i o n e n t e r i t i s . L o s s o f a f u n c t i o n a l i l e u m l e a d s t o
inadequate reabsorption of bile acid salts and causes a faulty digestion of fats.
P a n c r e a t i c e n zy me s ma y b e i n a c t i v a t e d b y e xp o s u r e t o e xc e s s g a s t r i c a c i d s
s u c h a s w o u l d o c c u r i n t h e Z o l l i n g e r - E l l i s o n s y n d r o me . S e g me n t a l s t a s i s o f
i n t e s t i n a l c o n t e n t s c a n a l s o o c c u r w i t h t h e d y s mo t i l i t y o f d i a b e t e s o r
s c l e r o d e r ma . S t a s i s p e r mi t s b a c t e r i a l o v e r g r o w t h o f t h e a f f e c t e d s e g me n t . T h e
b a c t e r i a s u b s e q u e n t l y d e c o n j u g a t e a n d me t a b o l i ze t h e b i l e a c i d s i n t h e s ma l l
bowel rather than the colon.
3. I n g e s t i o n o f c e r t a i n f o o d s h a s b e e n s h o w n t o c a u s e s t e a t o r r h e a . I n g e s t i o n o f a
356 / 652
tmdmss
9.12 - Steatorrhea
l a r g e a mo u n t o f p e a n u t s o r t h e u s e o f l i q u i d p a r a f f i n t o t r e a t c o n s t i p a t i o n ma y
r e s u l t i n s i g n i f i c a n t s t e a t o r r h e a . I n p a t i e n t s a t t e mp t i n g t o l o s e w e i g h t , i n g e s t i o n
o f t h e f a t s u b s t i t u t e O l e s t r a c a n c a u s e s t e a2)t o. r r h e a (
4. L a xa t i v e a b u s e h a s t e n s g a s t r o i n t e s t i n a l ( G I ) t r a n s i t a n d ma y p r e c l u d e a d e q u a t e
absorption of fats. Other drugs that can cause steatorrhea through a variety of
me c h a n i s ms i n c l u d e b i l e a c i d s e q u e s t e r i n g r e s i n s ( c h o l e s t y r a mi n e a n d
c o l e s t i p o l ) , c o l c h i c i n e , p a r a - a mi n o s a l i c y l i c a c i d , p a r o my c i n s u l f a t e ( H u ma t i n ) ,
a n d a n t i b i o t i c s s u c h a s t e t r a c y c l i n e a n d n e o my c i n . T h y r o t o xi c o s i s c a n c a u s e a
secondary steatorrhea due to the rapid transit of intestinal contents, which
p r e c l u d e s p r o p e r d i g e s t i o n a n d a b s o r p t i o n o f f a t s . I n fGe icar
t i odinawl iambl
th i a
c a n l e a d t o f a t ma l a b s o r p t i o n . T r a v e l w i t h i n t h e t r o p i c s ( e xc l u d i n g A f r i c a )
suggests tropical sprue, a chronic diarrheal disease, possibly of infectious
origin.
5. E v e n s e e mi n g l y u n r e l a t e d e v e n t s h a v e b e e n s h o w n t o t r i g g e r s t e a t o r r h e a ; 3 % o f
p a t i e n t s d e v e l o p s t e a t o r r h e a a s a c o mp l i c a t i o n o f b o n e ma r r o w t r a n s p l a n t a t i o n .
T h e me c h a n i s m i s b e l i e v e d t o b e g r a f t v e r s u s h o s t d i s e a s e t h a t a f f e c t s t h e
e xo c r i n e p a n c r e a t i c f u n c 3)
t i o. n (
B. Epidemiology
T h e i n c i d e n c e a n d p r e v a l e n c e o f s t e a t o r r h e a a r e d i f f i c u l t t o e s t i ma t e b e c a u s e t h i s
condition arises from an array of diverse underlying conditions that are difficult to
a ma s s i n t o a n o v e r a l l f i g u r e . T h e s e o t h e r c o n d i t i o n s a r e n o n r e p o r t a b l e i l l n e s s e s o r
s u r g i c a l p r o c e d u r e s p e r f o r me d f o r o t h e r r e a s o n s a n d s o a r e n o t r e a d i l y t a b u l a t e d i n
the literature.
P. 2 1 0
I I I . Evaluation
A. History
A careful history often provides clues to probable diagnoses and guides the astute
c l i n i c i a n t o t e s t s mo s t l i k e l y t o p r o v i d e a d e f i n i t i v e d i a g n o s i s . T h e f r e q u e n c y o f s t o
a n d d u r a t i o n o f t h e c o mp l a i n t a r e i mp o r t a n t , a s w e l l a s w h e t h e r t h e r e h a s b e e n a n
actual loss of weight. Recent travel to the tropics or a history of pancreatitis can b
i mp o r t a n t c l u e s . A f a mi l y h i s t o r y o f s i mi l a r p r o b l e ms c a n h e l p p i n p o i n t a c a u s e . D o e
t h e p a t i e n t e xh i b i t a n y s i g n s o r s y mp t o ms o f h y p e r t h y r o i d i s m?
P h y s i c a l f i n d i n g s a s s o c i a t e d w i t h s t e a t o r r h e a a r e l i mi t e d . A t h o r o u g h e xa mi n a t i o n o
t h e a b d o me n t o e xc l u d e p a l p a b l e ma s s e s s h o u l d b e u n d e r t a k e n a l o n g w i t h a s e a r c h
for signs of alcoholic liver disease.
C. Testing
1. T h e g o l d s t a n d a r d f o r d i a g n o s i s r e ma i n s t h e q u a n t i t a t i v e d e t e r mi n a t i o n o f f e c a l
f a t f r o m s t o o l s a mp l e s c o l l e c t e d o v e r 7 2 h o u r s w h i l e t h e p a t i e n t i n g e s t s a d i e t
w i t h a l i mi t e d f a t c o n t e n t . C o l l e c t i n g t h e s t o o l i s c u mb e r s o me a n d d i f f i c u l t f o r t h
357 / 652
tmdmss
9.12 - Steatorrhea
patient and the test requires strict adherence to the prescribed diet. Spot tests
o f a s i n g l e s t o o l s a mp l e w i t h S u d a n I I I o r t h e n e w e r f e c a l e l a s t a s e d e t e r mi n a t i o
a r e s i mp l e r t e s t s t o a d mi n i s t e r. T h e v a l i d i t y o f t h e f e c a l e l a s t a s e t e s t i s s t i l l
c o n t r o v e r s i a l b u t mo s t a g r e e t h a t i t h a s a v e r y s t r o n g n e g a t i v e p r e d i c t i v e v a l u e
(4) . O n c e t r u e s t e a t o r r h e a i s d e mo n s t r a t e d , f u r t h e r t e s t i n g h e l p s d e t e r mi n e t h e
u n d e r l y i n g d i s o r d e r.
2. L i v e r f u n c t i o n t e s t i n g a n d a p p r o p r i a t e u s e o f u l t r a s o u n d o r c o mp u t e d
t o mo g r a p h y ( C T ) s c a n o f t h e l i v e r a n d p a n c r e a s ma y d e mo n s t r a t e t h e s o u r c e .
S e r u m a n t i b o d y t e s t i n g c a n n o w i d e n t i f y g l u t e n e n 5)
t e.r oSpwaet hayt c( h l o r i d e
testing is indicated in young children; cystic fibrosis is the leading cause of
steatorrhea in this age group.
3. T h e D - xy l o s e t e s t - xy l o s e t e s t me a s u r e s t h e a b s o r p t i v e c a p a c i t y o f t h e p r o xi ma l
s ma l l b o w e l mu c o s a . D - xy l o s e d o e s n o t r e q u i r e p a n c r e a t i c e xo c r i n e f u n c t i o n t o
b e a b s o r b e d . D i s o r d e r s o f t h e i n t e s t i n a l mu c o s a t h a t i mp e d e a b s o r p t i o n l e a d t o
l o w l e v e l s o f t h e s u g a r i n b o t h s e r u m a n d u r i n e s a mp l e s . I n a d e q u a t e r e n a l
function, dehydration, or hypothyroidism can also depress urine levels, so
d e t e r mi n a t i o n s o f s e r u m t h y r o i d - s t i mu l a t i n g h o r mo n e , b l o o d u r e a n i t r o g e n , a n d
c r e a t i n i n e a r e w a r r a n t e d . B a c t e r i a l o v e r g r o w t h o f t h e s ma l l i n t e s t i n e c a n a l s o
p r o d u c e a n a b n o r ma l D - xy l o s e t e s t .
a. A n a b n o r ma l D - xy l o s e t e s t s h o u l d p r o mp t r e f e r r a l f o r s ma l l i n t e s t i n e b i o p s y
t o s e a r c h f o r e v i d e n c e o f mu c o s a l d i s e a s e s i n c l u d i n g g l u t e n e n t e r o p a t h y,
W h i p p l e ' s d i s e a s e , g i a r d i a s i s , t r o p i c a l s p r u e , o r i n t e s t i n a l l y mp h o ma . S o me
o f t h e s e e n t i t i e s ma y a l s o b e d i a g n o s e d b y t h e i r c h a r a c t e r i s t i c a p p e a r a n c e
on an upper G I series.
b. A n o r ma l D - xy l o s e t e s t i n d i c a t e s p r o p e r mu c o s a l f u n c t i o n a n d s o t h e
p r o b l e m i s u s u a l l y t h e d i g e s t i o n o f f a t s w i t h i n t h e i n t e s t i n a l l u me n . T h e mo s
f r e q u e n t e t i o l o g y i s p a n c r e a t i c i n s u f f i c i e Tn ac by l e( s 9e .e1 2). .1 A s e c r e t i n
t e s t ma y b e r e q u i r e d t o me a s u r e p a n c r e a t i c f u n c t i o n . A t h e r a p e u t i c t r i a l o f
p a n c r e a t i c e n zy me s w i t h i mp r o v e me n t i n s y mp t o ms i s c o n s i d e r e d
p r e s u mp t i v e p r o o f o f t h e d i a g n o s i s . A b d o mi n a l u l t r a s o n o g r a p h y, C T s c a n ,
o r e n d o s c o p i c r e t r o g r a d e c h o l a n g i o p a n c r e a t o g r a p h y ma y a l s o b e u s e f u l i n
the evaluation of suspected pancreatic disease.
4. T h e b i l e s a l t b r e a t h t e s t , a n u c l e a r me d i c i n e s t u d y, me a s u r e s b i l e a c i d
a b s o r p t i o n . B e c a u s e t h e t e r mi n a l i l e u m i s a l s o t h e s i t e 1o2f v i t a mi n B
a b s o r p t i o n , t h e S c h i l l i n g t e s t ma y a l s o b e u t i l i ze d t o s e a r c h f o r d i s o r d e r s o f
a b s o r p t i o n . D i s o r d e r s o f t h e t e r mi n a l i l e u m ( C r o h n ' s d i s e a s e , g r a n u l o ma t o u s
ileitis, prior ileal resection) result in poor absorption of bile salts, which then
p a s s i n t o t h e c o l o n w h e r e b a c t e r i a d e c o n j u g a t e t h e m. P o o r r e a b s o r p t i o n
d e p l e t e s t h e s u p p l y o f b i l e s a l t s , r e s u l t i n g i n ma l d i g e s t i o n o f f a t s .
5. N o n i n v a s i v e t e s t i n g f o r s ma l l b o w e l b a c t e r i a l o v e r g r o w t h ( S B B O ) i s a v a i l a b l e b y
t h e C - xy l o s e b r e a t h t e s t , t h e b i l e a c i d b r e a t h t e s t , o r t h e b r e a t h h y d r o g e n t e s t .
t h e r a p e u t i c t r i a l o f o r a l t e t r a c y c l i n e w i t h r e s o l u t i o n o f s t e a t o r r h e a i s p r e s u mp t i v
c o n f i r ma t i o n o f b a c t e r i a l o v e r g r o w t h , a v o i d i n g a mo r e c o s t l y t e s t i n g . T h e u p p e r
s ma l l i n t e s t i n e i s n o r ma l l y b a c t e r i o l o g i c a l l y s t e r i l e e xc e p t f o r c o n t a mi n a n t s f r o m
P. 2 11
358 / 652
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t h e mo u t h a n d u p p e r r e s p i r a t o r y t r a c t . A s p i r a t i o n o f f l u i d t h r o u g h a n e n d o s c o p e
o r a s ma l l - i n t e s t i n e t u b e p l a c e d u n d e r f l u o r o s c o p i c g u i d a n c e t h a t y i e l d s a
b a c t e r i a l c o l o n y c o u n t g r e a t e r t h a n 1 0 0 , 0 0 0 / mL i s d i a g n o s t i c o f S B B O .
TAB L E 9.12.1 Causes of Pancreatic Exocrine

I nsufficiency
A l c o h o l - i n d u c e d l i v e r d i s e a s eCs y s t i c f i b r o s i s
Chronic pancreatitis
Z o l l i n g e r - E l l i s o n s y n d r o me
H y p e r t r i g l y c e r i d e mi a
G a s t r e c t o my
Cancer of the pancreas
Va g o t o my
Resection of the pancreas
B l o c k a g e o f t h e p a n c r e a t i c d uScht w a c h ma n - D i a mo n d s y n d r o me
T r a u ma t i c p a n c r e a t i t i s
Trypsinogen deficiency
Hereditary pancreatitis
1 - A n t i t r y p s i n d e f i c i e n c y
Enterokinase deficiency
S o ma t o s t a t i n o ma
Graft vs. host disease
D. Genetics
G e n e t i c s p l a y s l i t t l e r o l e i n t h i s d i s o r d e r w i t h t h e e xc e p t i o n o f t h o s e c o n d i t i o n s w i t h
f a mi l i a l t e n d e n c i e s s u c h a s c y s t i c f i b r o s i s , g l u t e n e n t e r o p a t h y, a n d a c a n t h o c y t o s i s .
a p p r o xi ma t e l y 2 5 % o f c a s e s , Z o l l i n g e r - E l l i s o n s y n d r o me o c c u r s i n a s s o c i a t i o n w i t h
g e n e t i c s y n d r o me , mu l t i p l e e n d o c r i n e n e o p l a s i a t y p e 1 .
I V. Diagnosis
A s i n d i c a t e d i n t h e p r e c e d i n g t e xt , t h e d i f f e r e n t i a l d i a g n o s i s o f s t e a t o r r h e a i s
e xt e n s i v e . T h e d e g r e e o f s t e a t o r r h e a c a n l e n d c l u e s t o t h e s o u r c e . M i l d s t e a t o r r h e a
can occur with any disorder that causes rapid transit of intestinal contents, because
t h e s h o r t e n e d e xp o s u r e o f t h e f a t s p r e v e n t s p r o p e r a b s o r p t i o n . T h e e xp l o s i v e u r g e n
d i a r r h e a o f i r r i t a b l e b o w e l s y n d r o me c a n mi mi c s t e a t o r r h e a , b u t t h e f e c a l f a t c o n t e n
d o e s n o t u s u a l l y me e t t h e c r i t e r i a f o r t r u e s t e a t o r r h e a . T h e p r o p e r w o r k u p o f t h i s
359 / 652
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9.12 - Steatorrhea
s y mp t o m f r e q u e n t l y r e q u i r e s s p e c i a l i ze d t e s t i n g a n d p r o c e d u r e s , n e c e s s i t a t i n g
c o n s u l t a t i o n w i t h a g a s t r o e n t e r o l o g i s t i f i n i t i a l h i s t o r y a n d t h e mo r e r e a d i l y a v a i l a b l
tests fail to reveal its source.
T h e c l i n i c a l e xp r e s s i o n a s f o u l , g r e a s y l o o s e s t o o l s t h a t a r e d i f f i c u l t t o f l u s h
a c c o mp a n i e d b y w e i g h t l o s s i s u n c h a n g e d n o ma t t e r w h a t t h e u n d e r l y i n g e t i o l o g y o f
t h e s t e a t o r r h e a i s . T h e u r g e n c y a n d o c c a s i o n a l i n c o n t i n e n c e ma y l e a d t h e p a t i e n t t o
a v o i d s o c i a l g a t h e r i n g s , c e a s e p h y s i c a l e xe r c i s e , o r a t t i me s w i t h d r a w f r o m e v e n
f r i e n d s a n d f a mi l y r a t h e r t h a n e xp e r i e n c e t h e s h a me o f a n a c c i d e n t . A g g r e s s i v e l y
seeking the underlying condition and treating the diarrhea until the cause is found
helps avoid this situation.
References
1 . G r u y - K a p r a l C , L i t t l e K H , F o r d t r a n J S , e t a l . C o n j u g a t e d b i l e a c i d r e p l a c e me n t
t h e r a p y f o r s h o r t - b o w e l s y n d rGoas
met r. oent er ol ogy
1 9 9 9 ; 11 6 : 1 5 .
2. Balasekaran R, Porter J L. Positive results on tests for steatorrhea in persons
c o n s u mi n g o l e s t r a p o t a t o cAh nn
i p s .I nt er n M ed
2000;132(4):279282.
3 . G r i g g A P, A n g u s P W, H o y t R , e t a l . T h e i n c i d e n c e , p a t h o g e n e s i s a n d n a t u r a l
h i s t o r y o f s t e a t o r r h e a a f t e r b o n e ma r r o w t r a n s pBl aone
n t a tM
i oar
n .r ow
Tr ans pl ant2 0 0 3 ; 3 1 ( 8 ) : 7 0 1 7 0 3 .
4. Beharry S, Ellis L, Corey M, et al. How useful is fecal pancreatic elastase 1 as
a ma r k e r o f e xo c r i n e p a n c r e a t i c d i sJe P
a sedi
e ?at r2 0 0 2 ; 1 4 1 ( 1 ) : 8 4 9 0 .
5 . M u r r a y J A. S e r o d i a g n o s i s o f c e l i a c dC
i slei n
a sLeab
. R es
1997;17:445464.
360 / 652
tmdmss
10.1 - Dysuria
> T a b l e o f C o n t e n t s > 1 0 - R e n a l a n d U r o l o g i c P r o b l e ms > 1 0 . 1 - D y s u r i a
10.1
Dysuria
Dav id M. Quille n
I . Background
D ysuria is defined as painful urination. Acute dysuria is a frequent problem seen

a mb u l a t o r y p r a c t i c e s , a c c o u n t i n g f o r > 3 mi l l i o n o f f i c e v i s i t s a y e a r. T h e mo s t c o mmo
diagnosis given for patients with dysuria is a urinary tract infection (U T I). T he
e s t i ma t e d c o s t o f t r a d i t i o n a l ma n a g e me n t o f a c u t e U T I s a p p r o a c h e s $ 1 b i l l i o n p e r
y e a r i n t h e U n i t e d S t a t e s . A l t h o u g h a U T I i s t h e mo s t c o mmo n c a u s e o f d y s u r i a
s y mp t o ms , ma n y o t h e r c a u s e s n e e d t o b e a c c u r a t e l y d i a g n o s e d . T h e d i f f e r e n t i a l
diagnosis for patients with dysuria can be separated into broad categories. With a
f e w n o t a b l e e xc e p t i o n s , t h e d i f f e r e n t i a l d i a g n o s e s f o r me n a n d w o me n a r e s i mi l a r,
a l t h o u g h t h e i n c i d e n c e s a r e g r e a t l y d i f f e r e n t a n d c h a n g1)
e .w i t h a g e (
A.
C a u s e s o f d y s u r i a f e ma
5)l e (
1. I n f e c t i o u s
a. C y s t i t i s , l o w e r U T I , w i t h o r w i t h o u t p y e l o n e p h r i t i s
b. U r e t h r i t i s c a u s e d b y a s e xu a l l y t r a n s mi t t e d d i s e a C
s ehl (amy
S T Ddi) a,
:
, h e r p e s s i mp l e x v i r u s ( H S V )
c. Vu l v o v a g i n i t i s b a c t e r i a l v a g i n o s i s , t r i c h o mo n i a s i s , y e a s t , g e n i t a l H S V
2. N o n i n f e c t i o u s t r a u ma , i r r i t a n t , a l l e r g y, s e xu a l a b u s e
B.
C a u s e s o f d y s u r i a ma2)l e (
1. I n f e c t i o u s
a. U r e t h r i t i s c a u s e d Cbhl
y amy di a, N . gonor r hoeae
, y e a s t ( u n c i r c u mc i s e d
balanitis), H S V
b. C y s t i t i s ( i f c u l t u r e p o s i t i v e , p o s s i b l e a n a t o mi c a b n o r ma l i t y, f u r t h e r w o r k u p
indicated)
361 / 652
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10.1 - Dysuria
c. P r o s t a t i t i s , a c u t e mo r e c o mmo n t h a n c3)
hronic (
2. N o n i n f e c t i o u s
a. P e n i l e l e s i o n s , t r a u ma , s e xu a l a b u s e
b. B e n i g n p r o s t a t i c h y p e r t r o p h y ( B P H ) p a r t i c u l a r l y i n 4)
o l .d eI nr f me
e c tni o(n
c a n b e e v i d e n t b u t i s p r i ma r i l y a n o b s t r u c t i v e p r o c e s s .
I I I . Evaluation
A. History
A good general history is critical and can help direct further questions. Careful
q u e s t i o n i n g a b o u t o t h e r a s s o c i a t e d s y mp t o ms a n d r i s k f a c t o r s i s t h e k e y t o s o r t i n g
out the diagnosis.
1. I n t e r n a l d y s u r i a v e r s u s e x t e r n a l d yI nst ue r ni aa l d y s u r i a i s w h e r e t h e
d i s c o mf o r t s e e ms t o b e c e n t e r e d i n s i d e t h e b o d y a n d b e g i n s b e f o r e o r w i t h t h e
initiation of voiding.
a. I n f l a mma t i o n o f t h e b l a d d e r o r u r e t h r a
E xt e r n a l d y s u r i a i s w h e n t h e d i s c o mf o r t a p p e a r s a f t e r v o i d i n g h a s i n i t i a t e d
b. Va g i n i t i s , v u l v a r i n f l a mma t i o n , o r e xt e r n a l p e n i l e l e s i o n s .
2. O t h e r i mp o r t a n t h i s t o r y i t e ms
a. N e w s e x p a r t n e r S T D c a u s e
b. D i a p h r a g m u s a g e b l a d d e r i n f e c t i o n
c. G r a d u a l o n s e t u r e t h r i t i s a n d e xt e r n a l c a u s e s
d. S u p r a p u b i c p a i n , c o s t o v e r t e b r a l a n g l e t e n d e r n e s s , f e v e r, a n d f l a n k p a i n
pyelonephritis
e. O l d e r me n q u e s t i o n i n g a b o u t B P H
T h e e xa mi n a t i o n i s e s s e n t i a l i n n a r r o w i n g t h e d i a g n o s i s .
1. R u l e o u t p y e l o n e p h r i t i s
a. F e v e r
P. 2 1 6
b. F l a n k t e n d e r n e s s
c. S u p r a p u b i c t e n d e r n e s s
2. G e n i t a l e xa mi n a t i o n
a. S p e c u l u m e xa mi n a t i o n i n w o me n
b. F o r e s k i n r e t r a c t i o n i n u n c i r c u mc i s e d me n
c. P r o s t a t e e xa mi n a t i o n
362 / 652
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3. C o l l e c t i o n o f s a mp l e s f o r t e s t i n g
a. H S V l e s i o n s
b. D i s c h a r g e y e a s t , b a c t e r i a l v a g i n o s i s , g o n o r r h e a , a n d t r i c h o mo n i a s i s
c. T r a u ma
C. Testing
1. U r i n e a n a l y s i s d i p s t i c k t e s t n i t r a t e s a n d l e u k o e s t e r a s e ( u r e a - f i xi n g b a c t e r i a
and leukocytes)
2. D i r e c t mi c r o s c o p i c e xa mi n a t i o n o f t h e u r i n e c a n d e t e c t t h e f o l l o w i n g :
a. L e u k o c y t e s , b a c t e r i a , a n d b l o o d
b. P y u r i a ( d e f i n e d a s w h i t e b l o o d c e l l c o u n t3 >o1f 0u/ rmm
ine)
3. U r i n e c u l t u r e : t a k e s u p t o 4 8 h o u r s
I V. Diagnosis
G i v e n t h e ma n y c a u s e s o f d y s u r i a , a n a c c u r a t e d i a g n o s i s c a n b e d i f f i c u l t w i t h o u t a
t h o r o u g h a p p r o a c h t o e a c h p a t i e n t . B e c a u s e mo s t c a u s e s h a v e o t h e r a s s o c i a t e d
s y mp t o ms a n d f i n d i n g s , a d i a g n o s i s c a n u s u a l l y b e ma d e w i t h a c a r e f u l l y t a k e n
h i s t o r y, a f o c u s e d p h y s i c a l e xa mi n a t i o n , a n d a p p r o p r i a t e l a b o r a t o r y t e s t s . S e p a r a t i n
a n u n c o mp l i c a t e d U T I o r S T D f r o m t h e mo r e s e r i o u s p y e l o n e p h r i t i s a n d o t h e r
possible diagnoses is the challenge in these patients.
References
1 . J o h n s o n J R , S t a mm W E . D i a g n o s i s a n d t r e a t me n t o f a c u t e u r i n a r y t r a c t
i n f e c t i o n sI .nf ec t D i s C l i n N or t h 1A9m8 7 ; 4 ( 1 ) : 7 7 3 7 9 1 .
5 . K u r o w i s k i K . T h e w o ma n w i t h d yAs m
u r iFa am
.
P hy s i c i an
1998;57(9):2155
2164, 21692170.
2 . A i n s w o r t h J G , We a v e r T , M u r p h y S , e t a l . G e n e r a l p r a c t i t i o n e r s ' i mme d i a t e
ma n a g e me n t o f me n p r e s e n t i n g w i t h u r e t h r a l s yGmp
enit ot our
ms .i n M ed
1996;72(6):427430.
3. Roberts R O, Lieber M M, Rhodes R, et al. Prevalence of a physician-assigned
d i a g n o s i s o f p r o s t a t i t i s : t h e O l ms t e d C o u n t y s t u d y o f u r i n a r y s y mp t o ms a n d
h e a l t h s t a t u s a mo n g me
U rnol. ogy1 9 9 8 ; 5 1 ( 4 ) : 5 7 8 5 8 4 .
4 . B r e mn o r J D , S a d o v s k y R . E v a l u a t i o n o f d y s u r i a Ai nma Fd am
u l t sP
. hy s i c i an
2002;65(8):15891596.
363 / 652
tmdmss
10.2 - Hematuria
> T a b l e o f C o n t e n t s > 1 0 - R e n a l a n d U r o l o g i c P r o b l e ms > 1 0 . 2 - H e ma t u r i a
10.2
Hematuria
S i e g f r i e d O . F. S c h m i d t
Ku-Lang Chang
I . Background
H e ma t u r i a , d e f i n e d a s b l o o d i n t h e u r i n e , i s f r e q u e n t l y e n c o u n t e r e d i n f a mi l y
p r a c t i c e . I t c a n b e ma n i f e s t e d a s g r o s s ( ma c r o s c o p i c ) h e ma t u r i a w i t h o b v i o u s r e d d i s
d i s c o l o r a t i o n , o r i t c a n o c c u r a s mi c r o s c o p i c h e ma t u r i a d e t e c t e d w i t h a d i p s t i c k
f o l l o w e d b y a mi c r o s c o p i c e xa mi n a t i o n . A l t h o u g h t h e r e i s e xi s t i n g c o n t r o v e r s y
p e r t a i n i n g t o w h a t c o n s t i t u t e s mi c r o h e ma t u r i a , mo s t c l i n i c i a n s c o n s i d e r t h r e e o r mo r
r e d b l o o d c e l l s p e r h i g h - p o w e r f i e l d ( 4 0 0 ) a s a b n o r ma l .
A. Etiology
T h e l i s t o f t h e p o t e n t i a l c a u s e s o f h e ma t u r i a i s l e n g t h y a n d i n c l u d e s d i s e a s e s o f t h e
u r i n a r y t r a c t , a s w e l l a s n o n u r o l o g i c c a u s e s . S o me o f t h e s e a r e l i f e t h r e a t e n i n g ( e . g
renal and bladder cancer), whereas others are insignificant (e.g.,
P. 2 1 7
e xe r c i s e - i n d u c e d h e ma t u r i a , b l a d d e r p o l y p s , r e n a l c y s t s ) . I t i s i mp o r t a n t t o n o t e t h a
h e ma t u r i a i n a n a d u l t i s mo r e o f t e n u r o l o g i c t h a n r e n a l i n o r i g i n . T h e e xt e n t o f t h e
w o r k u p s h o u l d b e d e t e r mi n e d b y c o n s i d e r i n g f a c t o r s s u c h a s t h e l i k e l i h o o d o f
c o e xi s t i n g i l l n e s s e s , p o t e n t i a l c o mp l i c a t i o n s f r o m p r o c e d u r e s , a n d t h e c o s t t o t h e
p a t i e n t . B e n i g n c a u s e s s u c h a s me n s t r u a t i o n , i n f e c t i o n , a n d t r a u ma s h o u l d a l w a y s
b e e xc l u d e d b e f o r e f u r t h e r e v a l u a t i o n a n d r e f e r r a l t o u r o l o g y o r n e p h r o l o g y i s
initiated.
B. Epidemiology
O v e r a l l p r e v a l e n c e v a r i e s g r e a t l y ( 0 . 1 % 1 3 % ) a n d d e p e n d s o n ma n y v a r i a b l e s ( e . g .
t h e n u mb e r o f s c r e e n i n g t e s t s p e r f o r me d , t h e t y p e o f p o p u l a t i o n s t u d i e d ) . H o w e v e r,
s c r e e n i n g o f t h e g e n e r a l p o p u l a t i o n f o r mi c r o s c o p i c h e ma t u r i a i s n o t r e c o mme n d e d .
T h e d e c i s i o n t o c h e c k f o r h e ma t u r i a s h o u l d d e p e n d o n v a r i o u s r i s k f a c t o r s f o r
s i g n i f i c a n t u n d e r l y i n g d i s e a s e , a n d r e ma i n s a p h y s i c i a n ' s j u d g me n t c a l l . R i s k f a c t o r
f o r s i g n i f i c a n t u r o l o g i c d i s e a s e i n c l u d e a g e > 4 0 y e a r s , c i g a r e t t e s mo k i n g ( p a s t o r
p r e s e n t , i n c l u d i n g s e c o n d - h a n d s mo k e ) , h i s t o r y o f u r i n a r y t r a c t i n f e c t i o n s , h i s t o r y o
h e ma t u r i a , e xp o s u r e t o v a r i o u s d r u g s ( a n a l g e s i c , a n t i - i n f l a mma t o r y d r u g s ,
c y c l o p h o s p h a mi d e , h u ma n i mmu n o d e f i c i e n c y v i r u s t h e r a p y ) , e xp o s u r e t o
364 / 652
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10.2 - Hematuria
o c c u p a t i o n a l h a za r d s ( b e n ze n e s , 2 - n a p h t h y l a mi n e , a r o ma t i c a mi n e s , a n d a n i l i n e
d y e s ) , a n d p e l v i c r a d i a t i o n . I n ma n y c a s e s o f mi c r o h e ma t u r i a , n o c a u s e c a n b e f o u n
d e s p i t e a c o mp l e t e e v a l u a t i o n .
I I I . Evaluation
A. History
A t h o r o u g h h i s t o r y i s o f u t mo s t i mp o r t a n c e .
1. G e n e r a l q u e s t i o n s s h o u l d c o v e r t h e t y p e o f h e ma t u r i a
( ma c r o s c o p i c / g r o s s / mi c r o s c o p i c ) , a n d t h e r e l a t i o n s h i p b e t w e e n u r i n a t i o n a n d t h e
t i mi n g o f h e ma t u r i a . T h e t h r e e - c o n t a i n e r me t h o d a s s i s t s i n s e p a r a t i n g t h e
mi c t u r i t i o n i n t o t h r e e p o r t i o n s i n i t i a l , mi d d l e , a n d f i n a l p o r t i o n s . A n a t o mi c a l l y,
i n i t i a l h e ma t u r i a u s u a l l y o r i g i n a t e s f r o m a n t e r i o r u r e t h r a l d i s e a s e , a n d f i n a l
h e ma t u r i a r e s u l t s f r o m d i s e a s e o f t h e b l a d d e r n e c k , p o s t e r i o r u r e t h r a , o r
p r o s t a t e . H e ma t u r i a t h r o u g h o u t t h e mi c t u r i t i o n s u g g e s t s p a t h o l o g y mo r e
p r o xi ma l . Wo r ml i k e c l o t s s u g g e s t a l o c a t i o n a b o v e t h e b l a d d e r n e c k . U r i n e c o l o r
s h o u l d b e q u e s t i o n e d , w h i c h c a n b e a f f e c t e d b y t h e f o l l o w i n g : p h e n a zo p y r i d i n e
( o r a n g e ) , n i t r o f u r a n t o i n ( b r o w n ) , r i f a mp i n ( y e l l o w - o r a n g e ) , L - d o p a , me t h y l d o p a ,
a n d me t r o n i d a zo l e ( r e d d i s h b r o w n ) , p h e n o l p h t h a l e i n i n l a xa t i v e s , r e d b e e t a n d
r h u b a r b c o n s u mp t i o n , f o o d c o l o r i n g , a n d v e g e t a b l e d y e s ( r e d ) .
2. A s s o c i a t e d s y mp t o ms c a n h i n t a t v a r i o u s p a r t i c u l a r p r o b l e ms ; f o r e xa mp l e , a
r e c e n t s o r e t h r o a t , f e v e r, c h i l l s , a n d f l u l i k e s y mp t o ms ma y b e t h e f i r s t s i g n s o f
i mmu n o g l o b u l i n A n e p h r o p a t h y o r p o s t i n f e c t i o u s g l o me r u l o n e p h r i t i s . U r i n a r y
f r e q u e n c y, u r g e n c y, d y s u r i a , f e v e r, a n d c h i l l s p o i n t t o a n i n f e c t i o u s p r o c e s s .
D i mi n i s h e d u r i n e f l o w a n d a b d o mi n a l o r f l a n k p a i n r a d i a t i n g i n t o t h e g r o i n c a n
i n d i c a t e t h e p r e s e n c e o f u r i n a r y t r a c t o b s t r u c t i o n . Va g i n a l d i s c h a r g e o r b o w e l
mo v e me n t c h a n g e s ma y h i n t a t a n o n u r i n a r y t r a c t c a u s e s u c h a s a f o r e i g n b o d y
( e s p e c i a l l y i n c h i l d r e n ) . A r a s h , j o i n t p a i n , p h o t o s e n s i t i v i t y, f l u l i k e s y mp t o ms , a n
R a y n a u d ' s p h e n o me n o n p o i n t t o a c o l l a g e n v a s c u l a r d i s e a s e .
3. P a s t me d i c a l h i s t o r y s h o u l d a l s o i n c l u d e t r a v e l h i s t o r y. I f t h e p a t i e n t h a s
t r a v e l e d t o a r e a s w h e r e b i l h aSrczihias t( os oma haemat obi
) um
i s e n d e mi c ,
p a r a s i t i c i n f e s t a t i o n i s h i g h l y p r o b a b l e . F u r t h e r mo r e , i f t h e h i s t o r y i n c l u d e s t h e
u s e o f a n a l g e s i c s , e s p e c i a l l y a n t i - i n f l a mma t o r y d r u g s , r e n a l p a p i l l a r y n e c r o s i s
s h o u l d b e c o n s i d e r e d . P a s t e xp o s u r e t o c y c l o p h o s p h a mi d e ma y c a u s e c h e mi c a l
c y s t i t i s , b e c a u s e p a s t e xp o s u r e t o a n t i b i o t i c s ( e . g . , p e n i c i l l i n , c e p h a l o s p o r i n s )
ma y c a u s e i n t e r s t i t i a l n e p h r i t i s . O f s p e c i a l n o t e i s t h a t t h e me r e u s e o f o r a l
a n t i c o a g u l a n t s d o e s n o t c a u s e h e ma t u r i a . T o t h e c o n t r a r y, t h e s e p a t i e n t s ma y i n
f a c t p r e s e n t e a r l i e r i n t h e i r d i s e a s e p r o c e s s a n d s h o u l d b e e v a l u a t e d p r o mp t l y
(1) .
4. F a mi l y h i s t o r y ma y l e a d t o t h e s u s p i c i o n o f p o l y c y s t i c k i d n e y d i s e a s e , s i c k l e c e l
t r a i t a n d d i s e a s e , n e p h r o l i t h i a s i s , v a r i o u s g l o me r u l a r d i s e a s e s , t u b e r c u l o s i s , a n
b e n i g n f a mi l i a l h e ma t u r i a . T h e c o mb i n a t i o n o f r e n a l f a i l u r e , d e a f n e s s , a n d
h e ma t u r i a s u g g e s t s A l p o r t ' s h e r e d i t a r y n e p h r i t i s .
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10.2 - Hematuria
E xa mi n a t i o n s h o u l d f o c u s o n s i g n s o f s y s t e mi c d i s e a s e ( e . g . , f e v e r, r a s h ,
l y mp h a d e n o p a t h y, j o i n t s w e l l i n g , a n d a b d o mi n a l o r p e l v i c ma s s ) a n d u n d e r l y i n g
me d i c a l o r r e n a l d i s e a s e ( e . g . , h y p e r t e n s i o n , p e r i p h e r a l a n d g e n e r a l i ze d e d e ma ) .
M u l t i p l e t e l a n g i e c t a s i a s a n d mu c o u s me mb r a n e l e s i o n s i n d i c a t e
P. 2 1 8
h e r e d i t a r y h e mo r r h a g i c t e l a n g i e c t a s i a ( R e n d u - O s l e r - We b e r d i s e a s e ) . T h e f i n d i n g o f
a n a b d o mi n a l ma s s ma y i n d i c a t e a W i l ms ' t u mo r i n c h i l d r e n o r a b d o mi n a l c a n c e r a n d
a n e u r y s m i n a d u l t s . O n r e c t a l a n d g e n i t o u r i n a r y e xa mi n a t i o n , o n e c a n f i n d s i g n s o f
p r o s t a t i t i s , p r o s t a t e h y p e r t r o p h y, p r o s t a t e c a n c e r, v a g i n a l a n d u r e t h r a l c h a n g e s , a n
p e l v i c ma s s e s .
C. Testing
1. L a b o r a t o r y t e s t i n g i n i t i a l l y b e g i n s w i t h e xa mi n i n g a f r e s h l y v o i d e d , c l e a n c a t c h
u r i n e s a mp l e b y a d i p s t i c k . I t i s i mp o r t a n t t o c o n f i r m h e ma t u r i a b y a mi c r o s c o p i c
e xa mi n a t i o n o f t h e u r i n e s e d i me n t . T h i s s e d i me n t i s o b t a i n e d b y c e n t r i f u g a t i o n
o f a f i xe d v o l u me o f u r i n e ( 5 mL ) f o r 5 mi n u t e s a t 3 , 0 0 0 r o t a t i o n s / mi n u t e .
A f t e r w a r d , t h e s u p e r n a t a n t i s p o u r e d o f f , a n d t h e r e ma i n i n g s e d i me n t i s
resuspended in the centrifuge tube by gently tapping the bottom of the tube. A
p i p e t t e i s u s e d t o s a mp l e t h e r e s i d u a l f l u i d a n d t r a n s f e r i t t o a g l a s s s l i d e , a n d
c o v e r s l i p i s a p p l i e d t o t h e s l i d e f o r mi c r o s c o p i c e2)v .a lTu ha et i osnp e( c i me n i s
e xa mi n e d u n d e r h i g h ma g n i f i c a t i o n ( 4 0 0 ) t o d e t e r mi n e t h e c e l l t y p e a n d
d i s t i n c t mo r p h o l o g i c f e a t u r e s . R e s u l t s a r e r e c o r d e d a s t h e n u mb e r o f r e d b l o o d
c e l l s p e r h i g h - p o w e r f i e l d . I f a p a t i e n t i s a s y mp t o ma t i c a n d h a s n o p a r t i c u l a r r i s
factors, two additional urine analyses should be obtained. If one of them is
a b n o r ma l , i t i s n o t n e c e s s a r y t o p e r f o r m o t h e r s t u d i e s . I f a t l e a s t t w o o u t o f t h r e
u r i n e a n a l y s e s a r e a b n o r ma l , f u r t h e r w o r k u p i s n3)
e c. eW
s shaerny t(h e d i p s t i c k
t e s t i n g i s p o s i t i v e f o r b l o o d , b u t u r i n e mi c r o s c o p y r e v e a l s n o r e d b l o o d c e l l s ,
h e mo g l o b i n u r i a o r my o g l o b i n u r i a s h o u l d b e c o n s i d e r e d . T h e u s e o f a b e n zi d i n e
d i p s t i c k a l l o w s t h e d i f f e r e n t i a t i o n o f t h e s e d i s c o l o r a t i o n s f r o m t h o s e o f h e ma t u r i
a n d my o g l o b i n u r i a . A s a n e xt s t e p , a u r i n e c u l t u r e c a n b e o b t a i n e d t o r u l e o u t a
infection.
2. B l o o d t e s t s i n c l u d e a r e n a l p a n e l a n d a c o mp l e t e b l o o d c o u n t w i t h d i f f e r e n t i a l ,
s e d i me n t a t i o n r a t e , p r o t h r o mb i n t i me , a n d p a r t i a l t h r o mb o p l a s t i n t i me .
3. A n y f u r t h e r e v a l u a t i o n i s h i g h l y d e p e n d e n t o n t h e s u s p e c t e d c a u s e . F u r t h e r
b l o o d t e s t s ma y i n c l u d e s e r u m c o mp l e me n t t i t e r ( s i g n i f i c a n t i f l o w ) ,
a n t i s t r e p t o l y s i n - O t i t e r ( s i g n i f i c a n t i f h i g h ) , a n t i n u c l e a r a n t i b o d y a n d e xt e n d e d
p a n e l s w i t h a n t i d e o xy r i b o n u c l e a s e B t i t e r ( s i g n i f i c a n t i f h i g h ) , a n d h e mo g l o b i n
e l e c t r o p h o r e s i s . T h e u r i n e c a n a l s o b e s e n t f o r c y t o l o g y. A t u b e r c u l i n s k i n t e s t
o r c h e s t x- r a y c a n b e p e r f o r me d t o d e t e c t t u b e r c u l o s i s . I n t r a v e n o u s p y e l o g r a m,
a b d o mi n a l a n d p e l v i c u l t r a s o u n d , c o mp u t e r t o mo g r a p h y, o r ma g n e t i c r e s o n a n c e
i ma g i n g ma y d e t e c t b e n i g n c o n d i t i o n s s u c h a s u r o l i t h i a s i s , o b s t r u c t i v e u r o p a t h y,
r e n a l c y s t s , p a r e n c h y ma l a b n o r ma l i t i e s , a n d n o n u r i n a r y t r a c t l e s i o n s , a s w e l l a s
ma l i g n a n c i e s o f v a r i o u s a n a t o mi c a l a r e a s . O n e s h o u l d t h e n p r o c e e d w i t h a
c y s t o s c o p y, l o o k i n g f o r a b n o r ma l i t i e s o f t h e u r e t h r a a n d b l a d d e r. B i o p s i e s o f
366 / 652
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10.2 - Hematuria
v a r i o u s a r e a s , a s w e l l a s i n v a s i v e v a s c u l a r s t u d i e s , ma y b e n e c e s s a r y.
4. R e c e n t l y d e v e l o p e d u r i n a r y t u mo r ma r k e r s ( e . g . , B T A T R A K a n d t e l o me r a s e )
h a v e b e e n u s e d i n t u mo r s u r v e i l l a n c e , b u t t h e i r r o l e i n s c r e e n i n g o f p a t i e n t s w i t
h e ma t u r i a i s u n c l e a r a t t h i s 1)t i.me
A t (a n y t i me d u r i n g a n e v a l u a t i o n , i t r e ma i n s
w i t h i n t h e p h y s i c i a n ' s j u d g me n t t o r e f e r t h e p a t i e n t t o a s u b s p e c i a l i s t .
I V. Diagnosis
T h e k e y s t o t h e d i a g n o s i s o f h e ma t u r i a a r e f o u n d i n t h e c l i n i c a l h i s t o r y a n d t h e
p h y s i c a l e xa mi n a t i o n . L a b o r a t o r y a n d i ma g i n g s t u d i e s o n l y h e l p c o n f i r m o r r u l e o u t
initial suspicions. T he goal is to diagnose a variety of serious illnesses, including
ma l i g n a n c i e s a n d r e n a l p a r e n c h y ma l d i s e a s e s . I n g e n e r a l , t h e d e g r e e o f h e ma t u r i a
o f l i t t l e d i a g n o s t i c o r p r o g n o s t i c 2)v .a lAuse l (i t t l e a s 1 mL o f b l o o d c a n c a u s e a
visible color change. In addition, a variety of drugs, foods, and food coloring can
d i s c o l o r t h e u r i n e . A l s o , i t i s n e c e s s a r y t o r e me mb e r t h a t t r a n s i e n t h e ma t u r i a ,
e s p e c i a l l y i n a y o u n g i n d i v i d u a l , i s q u i t e c o mmo n a n d r a r e l y i n d i c a t i v e o f s i g n i f i c a n
p a t h o l o g y4)( . W h e n p r e s e n t i n p a t i e n t s o l d e r t h a n 4 0 y e a r s o f a g e , h o w e v e r,
t r a n s i e n t h e ma t u r i a w a r r a n t s a c o mp r e h e n s i v e e v a l u a t i o n t o r u l e o u t ma l i g n a n c y.
S i mi l a r l y, a d i a g n o s t i c w o r k u p s h o u l d b e p e r f o r me d w h e n p e r s i s t e n t h e ma t u r i a i s
f o u n d i n p a t i e n t s o f a n y a g e . A s u mma r y o f t h e b e s t p r a c t i c e p o l i c y r e c o mme n d a t i o n
b y t h e A me r i c a n U r o l o g i c A s s o c i a t i o n i s c o n t a i n e d i n t h e a r t i c l e , A s y mp t o ma t i c
M i c r o s c o p i c H e ma t u r i a i n A d u l t s , a v a i l a b l e a t t h e w e b s i t e
h t t p : / / w w w. a a f p . o r g / a f p / 2 0 0 1 0 3 1 5 / 11 4 5 . h t ml . I t c o n t a i n s a n e xc e l l e n t f l o w c h a r t o f
t h e w o r k u p o f a s y mp t o ma t i c mi c r o s c o p i c h e ma t u r i a , p l u s a s t r a t e g y t o i d e n t i f y
P. 2 1 9
p a t i e n t s w i t h s i g n i f i c a n t d i s e a s e , w h i l e mi n i mi zi n g c o s t a n d mo r b i d i t y a s s o c i a t e d w i
unnecessary tests.
Repeat evaluations are indicated for those patients with a negative evaluation, and
i n w h o m a ma l i g n a n c y i s s u s p e c t e d ( ma i n l y o l d e r a d u l t s ) . A r e a s o n a b l e t i me f r a me
a p p e a r s t o b e 3 t o 6 mo n t h s f o r l e s s i n v a s i v e t e s t s a n d 1 y e a r f o r mo r e i n v a s i v e t e s
(5) .
A. Clinical manifestations
O t h e r i mp o r t a n t c l i n i c a l ma n i f e s t a t i o n s s h o u l d a l s o b e c o n s i d e r e d . U n t i l o t h e r w i s e
p r o v e n , p a i n l e s s g r o s s h e ma t u r i a i n t h e a b s e n c e o f i n f e c t i o n i n e l d e r l y me n i s
c a u s e d b y ma l i g n a n c y, j u s t a s h e ma t u r i a a s s o c i a t e d w i t h s t e r i l e p y u r i a i s c a u s e d
b y g e n i t o u r i n a r y t u b e r c u l o s i s o r i n t e r s t i t i a l n e p h r i t i s . F i n a l l y, o t h e r me d i c a l p r o b l e m
s u c h a s p r o s t a t e h y p e r t r o p h y, d i a b e t e s me l l i t u s ( n e p h r o s c l e r o s i s ) , n e p h r o l i t h i a s i s ,
t r a u ma ( i n c l u d i n g v i g o r o u s ma s t u r b a t i o n ) , p r e v i o u s u r i n a r y t r a c t ma l i g n a n c i e s w i t h
r e c u r r e n c e , a n d s i c k l e c e l l d i s e a s e ( p a p i l l a r y n e c r o s i s ) ma y c a u s e h e ma t u r i a .
References
1 . Y u n E J , M e n g M V, C a r r o l l P R . E v a l u a t i o n o f t h e p a t i e n t w i t hM hed
e ma t u r i a .
C l i n N or t h A m
2004;88:329343.
2 . T h a l l e r T R , Wa n g L P. E v a l u a t i o n o f a s y mp t o ma t i c mi c r o s c o p i c h e ma t u r i a i n
367 / 652
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10.2 - Hematuria
a d u l t s .A m F am P hy s i c i an
1 9 9 9 ; 6 0 : 11 4 3 11 5 4 .
3 . G r o s s f e l d G D , Wo l f J S , L i t w i n M S , e t a l . A s y mp t o ma t i c mi c r o s c o p i c h e ma t u r i a
i n a d u l t s : s u mma r y o f t h e A U A b e s t p r a c t i c e p o l i c y r e c o mme
A mn dFaam
tions.
P hy s i c i an2 0 0 1 ; 6 3 ( 6 ) : 11 4 5 11 5 4 .
4 . M u r a k a mi S , I g a r a s h i T , H a r a S , e t a l . S t r a t e g i e s f o r a s y mp t o ma t i c
mi c r o s c o p i c h e ma t u r i a : a p r o s p e c t i v e s t u d y o f 1 0 3 4J pUartol
i e1n9t 9s 0. ; 1 4 4 : 9 9
106.
5 . M e s s i n g E M , Yo u n g T B , H u n t V B , e t a l . H e ma t u r i a h o me s c r e e n i n g : r e p e a t
t e s t i n g r e s u l tJs .U r ol 1 9 9 5 ; 1 5 4 ( 1 ) : 5 7 6 1 .
368 / 652
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10.3 - Impotence
> T a b l e o f C o n t e n t s > 1 0 - R e n a l a n d U r o l o g i c P r o b l e ms > 1 0 . 3 - I mp o t e n c e
10.3
Impotence
Louis Kuritzky
I . Background
I mp o t e n c e , d e f i n e d a s t h e c o n s i s t e n t i n a b i l i t y t o g e t o r ma i n t a i n a n e r e c t i o n s u f f i c i e
f o r i n t e r c o u r s e , i s , i n e s s e n c e , a p a t i e n t - d e f i n e d 1,2)
d i a .g nTohsei sr o( l e o f t h e
c l i n i c i a n i s t o c o n f i r m i mp o t e n c e , r u l e o u t c o r r e c t a b l e s e c o n d a r y c a u s e s , a n d
e xp e d i t i o u s l y r e s t o r e s e xu a l f u n c t i o n . T o e n c o u r a g e h o p e f u l n e s s , c l i n i c i a n s w o u l d d
w e l l t o e xp l a i n a t t h e o u t s e t t h a t e s s e n t i a l l y 1 0 0 % o f me n c a n h a v e r e s t o r a t i o n o f
s e xu a l f u n c t i o n u s i n g c u r r e n t l y a v a i l a b l e t r e a t me n t s .
T h e mo s t r e c e n t l a r g e e p i d e mi o l o g i c s u r v e y o f A me r i c a n me n f o u n d s o me d e g r e e o f
i mp o t e n c e i n 5 2 % o f me n o l d e r t h a n 4 0 y e a r s o f a g e . M o s t o r g a n i c i mp o t e n c e i s o n
vascular basis. Because integrity of the endothelium is necessary to provide
a d e q u a t e p e n i l e e n g o r g e me n t , d i s o r d e r s t h a t c a u s e e n d o t h e l i a l d y s f u n c t i o n a r e
p r e d i c t a b l y a s s o c i a t e d w i t h e r e c t i l e d y s f u n c t i o n ( E D ) . D i a b e t e s , s mo k i n g ,
h y p e r t e n s i o n , d y s l i p i d e mi a , a n d p e r i p h e r a l v a s c u l a r d i s e a s e a r e a l l a s s o c i a t e d w i t h
i mp a i r e d e n d o t h e l i a l f u n c t i o n , a n d ma y h e n c e i n d u c e o r c o n t r i b u t e t o E D . W h e t h e r
t h e c o r r e c t i o n o f v a s c u l o p a t h i c f a c t o r s i mp r o v e s e r e c t i l e f u n c t i o n i n me n w i t h E D
r e ma i n s t o b e d e t e r mi n e d .
I I I . Evaluation
A. History
1. A l t h o u g h w r i t t e n q u e s t i o n n a i r e s ma y e l i c i t s e xu a l d y s f u n c t i o n , mo s t p a t i e n t s
p r e f e r t o c o mmu n i c a t e s u c h i s s u e s i n t h e p r i v a c y o f v e r b a l c o mmu n i c a t i o n w i t h
t h e i r p r i ma r y c a r e p r o v i d e r. I n i t i a l i n q u i r y c a n s i mp l y b e A r e y o u s e xu a l l y
P. 2 2 0
a c t i v e ? F o r s e xu a l d y s f u n c t i o n e v a l u a t i o n , g e n d e r o r i e n t a t i o n i s n o t r e l e v a n t t o
d i a g n o s i s o r t h e r a p y, s o t h a t w h e t h e r t h e p a t i e n t i s h o mo s e xu a l , h e t e r o s e xu a l , o
b i s e xu a l h a s n o d i s t i n c t b e a r i n g o n t h e d i a g n o s t i c o r t h e r a p e u t i c d i r e c t i o n . F o r
i n d i v i d u a l s w h o a r e n o t s e xu a l l y a c t i v e , t h e n e xt i n q u i r y s h o u l d b e t o d e t e r mi n e
w h e t h e r t h i s i s a ma t t e r o f c h o i c e , o r a n o b s t a c l e t h a t p r e v e n t s s e xu a l a c t i v i t y
( e . g . , l a c k o f p a r t n e r, i mp o t e n c e , p h y s i c a l d i s o r d e r ) .
2. F o r i n d i v i d u a l s w h o a r e s e xu a l l y a c t i v e , a s e r i e s o f f o l l o w - u p q u e s t i o n s u n c o v e r
369 / 652
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10.3 - Impotence
mo s t r e l e v a n t p s y c h o s e xu a l p a t h o l o g y. Yo u c o u l d b e g i n w i t h H o w w o u l d y o u
r a t e y o u r s e x l i f e o n a s c a l e o f 1 t o 1 0 ? I f t h e r e s p o n s e i s 1 0 , s e xu a l
d y s f u n c t i o n i s d e c i d e d l y u n l i k e l y. H o w e v e r, mo s t i n d i v i d u a l s r e s p o n d O h , a b o u t
a 7 . Yo u c a n f o l l o w w i t h , W h a t w o u l d h a v e t o b e d i f f e r e n t t o c h a n g e y o u r s e x
life from a 7 to a 10? T his forced-choice inquiry often produces responses that
d i r e c t l y i n d i c a t e p r o b l e ma t i c u n d e r l y i n g i s s u e s s u c h a s We l l , i f I c o u l d j u s t g e t
g o o d e r e c t i o n o r I f my e r e c t i o n c o u l d l a s t > 3 0 s e c o n d s . I n q u i r y a b o u t l i b i d o i
a c r u c i a l d i a g n o s t i c p o i n t f o r t e s t o s t e r o n e d e f i c i e n c y. M e n w h o p r e s e n t w i t h
g o o d l i b i d o o n l y h a v e a r e mo t e p o s s i b i l i t y o f h a v i n g t e s t o s t e r o n e d e f i c i e n c y.
3. A me d i c a t i o n h i s t o r y s h o u l d b e t a k e n . M o s t me d i c a t i o n - i n d u c e d i mp o t e n c e i s
e v i d e n t b y t h e t e mp o r a l r e l a t i o n s h i p b e t w e e n t h e o n s e t o f i mp o t e n c e a n d
me d i c a t i o n i n i t i a t i o n . O n t h e o t h e r h a n d , a g e n t s s u c h a s t h i a zi d e s ma y p r o d u c e
i mp o t e n c e a f t e r mo n t h s o f u s e . S i mi l a r l y, s o me a n t i d e p r e s s a n t s ma y p r o d u c e
s e xu a l d y s f u n c t i o n e a r l y, o r a f t e r w e e k s o f t h e r a p y. R e l a t i o n s h i p o f me d i c a t i o n s
t o i mp o t e n c e c a n o f t e n b e c l a r i f i e d b y a d r u g h o l i d a y.
A l t h o u g h p h y s i c a l e xa mi n a t i o n i s u s u a l l y n o t e n l i g h t e n i n g , t h e r e i s g e n e r a l
a g r e e me n t t h a t t h e g e n i t a l s s h o u l d b e e xa mi n e d f o r e v i d e n c e o f o v e r t t e s t i c u l a r
atrophy and the penis for P eyronie's disease. P eyronie's disease produces palpable
plaques in the corpora cavernosa that can lead to angulation upon erection, pain, o
b o t h . T r e a t me n t i s s u r g i c a l . A r e c t a l e xa mi n a t i o n t o d o c u me n t r e c t a l s e n s a t i o n a s
w e l l a s t o n e c a n b e c o mp l e me n t e d b y t h e b u l b o c a v e r n o s u s r e f l e x. T h i s r e f l e x i s
e l i c i t e d b y b r i s k l y s q u e e zi n g t h e g l a n s p e n i s i n o n e h a n d w h i l e a s i n g l e d i g i t f r o m t h
o t h e r i s i n t h e r e c t u m. A n o r ma l e xa mi n a t i o n , i n d i c a t i n g a n i n t a c t r e f l e x a r c , i s
ma n i f e s t a s a r e c t a l c o n t r a c t i o n i n r e s p o n s e t o t h e g l a n s s q u e e ze . P r o s t a t e
e xa mi n a t i o n i s p e r t i n e n t a t t h i s p o i n t , i n t h e e v e n t t e s t o s t e r o n e t h e r a p y i s r e q u i r e d .
C. Testing
R e a s o n a b l e s c r e e n i n g t e s t s f o r i mp o t e n c e i n c l u d e p l a s ma g l u c o s e , l i p i d p r o f i l e
( s e e k i n g v a s c u l o p a t h i c r i s k f a c t o r s ) , t o t a l mo r n i n g t e s t o s t e r o n e , a n d a u r i n a l y s i s . I f
t o t a l mo r n i n g t e s t o s t e r o n e i s l o w, l u t e i n i zi n g a n d f o l l i c l e - s t i mu l a t i n g h o r mo n e l e v e l s
s h o u l d b e me a s u r e d , b e c a u s e a n i n c r e a s e i n t h e s e i n d i c a t e s g o n a d a l f a i l u r e , f o r
w h i c h t e s t o s t e r o n e r e p l a c e me n t i s i n d i c a t e d ; a d e c r e a s e i n d i c a t e s p o t e n t i a l
h y p o t h a l a mi c o r p i t u i t a r y i n s u f f i c i e n c y, n e c e s s i t a t i n g c e n t r a l n e r v o u s s y s t e m ( C N S )
i ma g i n g t o r u l e o u t a ma s s l e s i o n . S i mi l a r l y, t e s t o s t e r o n e ma y b e s u p p r e s s e d b y a n
e l e v a t e d p r o l a c t i n , w h e t h e r i n d u c e d b y a C N S l e s i o n , h y p o t h y r o i d i s m, me d i c a t i o n s ,
o r o t h e r f a c t o r s . A l o w ( o r l o w n o r ma l ) t o t a l t e s t o s t e r o n e s h o u l d b e r e p e a t e d a n d
c o n f i r me d b y me a n s o f me a s u r i n g t h e f r e e t e s t o s t e r o n e , w h i c h i s mo r e s e n s i t i v e , b u
s u b s t a n t i a l l y mo r e e xp a n s i v e t h a n t o t a l t e s t o s t e r o n e .
I V. Diagnosis
I mp o t e n c e i s b r o a d l y d i v i d e d i n t o p s y c h o g e n i c a n d o r g a n i c c a t e g o r i e s , a l t h o u g h
t h e r e i s o f t e n a s u b s t a n t i a l d e g r e e o f o v e r l a p . M e n w h o r e p o r t s u d d e n , c o mp l e t e l o s
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10.3 - Impotence
o f s e xu a l f u n c t i o n , o r c i r c u ms t a n t i a l i mp o t e n c e : ( i ) g o o d f u n c t i o n w i t h o n e p a r t n e r
b u t n o t a n o t h e r ; ( i i ) g o o d e r e c t i o n s w i t h ma s t u r b a t i o n b u t n o t w i t h a p a r t n e r ; ( i i i )
g o o d mo r n i n g e r e c t i o n s , b u t n o t w i t h a p a r t n e r, a r e mu c h mo r e l i k e l y t o h a v e
p s y c h o g e n i c i mp o t e n c e . B e c a u s e o r g a n i c i mp o t e n c e g e n e r a l l y l e a d s t o p s y c h o l o g i c a
c o n s e q u e n c e s , ma n y p a t i e n t s s u f f e r a c o mb i n a t i o n o f p s y c h o g e n i c a n d o r g a n i c
i mp o t e n c e .
1. P s y c h o g e n i c i mp o t e n c e ma y r e f l e c t d e p r e s s i o n , r e l a t i o n s h i p c o n f l i c t ,
p e r f o r ma n c e a n xi e t y, o r p a r t n e r - d i r e c t e d h o s t i l i t y. T h e h i s t o r y i s d e f i n i t i v e i n
mo s t c a s e s . S u d d e n c o mp l e t e l o s s o f f u n c t i o n , s i t u a t i o n o r p a r t n e r v a r i a b i l i t y,
a l o n g w i t h ma i n t e n a n c e o f mo r n i n g o r ma s t u r b a t o r y e r e c t i o n s , i s t y p i c a l .
O c c a s i o n a l l y,
P. 2 2 1
p a t i e n t s w i t h d y s f u n c t i o n s o t h e r t h a n i mp o t e n c e s e e k a d v i c e ; f o r i n s t a n c e ,
b e l i e v i n g t h a t p r e ma t u r e e j a c u l a t i o n i s i mp o t e n c e . I n s u c h c a s e s , c o r r e c t i v e
e d u c a t i o n c o mb i n e d w i t h a p p r o p r i a t e a t t e n t i o n t o t h e a l t e r n a t e d i a g n o s i s i s t h e
l o g i c a l n e xt s t e p .
2. O r g a n i c s e xu a l d y s f u n c t i o n i s c h a r a c t e r i ze d b y i n c r e me n t a l l o s s o f e r e c t i l e
f u n c t i o n . I n mi d d l e a g e , me n w i t h o r g a n i c E D n o t e r e d u c e d e r e c t i l e t u r g i d i t y,
i n c r e a s i n g r e q u i r e me n t f o r t a c t i l e s t i mu l a t i o n t o p r o d u c e a n e r e c t i o n , a n d a
l e n g t h e n i n g r e f r a c t o r y p e r i o d ( i . e . , t h e a mo u n t o f t i me r e q u i r e d a f t e r e j a c u l a t i o n
b e f o r e t h e ma l e i s r e c e p t i v e t o r e s t i mu l a t i o n a n d e r e c t i o n ) . S u c h s t e p w i s e l o s s
o f s e xu a l f u n c t i o n c o r r o b o r a t e s o r g a n i c i t y.
3. I n p r i ma r y c a r e , a s ma n y a s 9 8 % o f p a t i e n t s h a v e n o c o r r e c t a b l e c a u s e o f
i mp o t e n c e d i s c e r n e d a f t e r a p p r o p r i a t e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n . T h a t
n e e d n o t d e l a y t h e i mme d i a t e p r o v i s i o n o f h i g h l y e f f e c t i v e o r a l a g e n t s ( P D E S
inhibitor [phosphodiesterase type 5 inhibitors]), vacuum constriction devices, or
i n t r a c o r p o r e a l i n j e c t i o n , a n y o f w h i c h ma y b e p r o v i d e d i n t h e p r i ma r y c a r e
s e t t i n g . P a t i e n t s w h o f a i l t o r e s p o n d t o t h e s t a n d a r d t r e a t me n t t o o l s s h o u l d b e
referred.
References
1 . K u r i t zk y L . P r i ma r y c a r e i s s u e s i n t h e ma n a g e me n t o f e r e c t i l e d y s f u n c t i o n . I n :
S e f t e l A D , eM
d . al e and f emal e s ex ual dy s f unc
. Et di on
i n b u r g h : M o s b y, 2 0 0 4 .
2 . K u r i t zk y L , A h me d O , K o s c h S . M a n a g e me n t o f i mp o t e n c e i n p r i ma r y c a r e .
C omp T her1 9 9 8 ; 2 4 ( 3 ) : 1 3 7 1 4 6 .
371 / 652
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10.4 - Urinary Incontinence in Adults
> T a b l e o f C o n t e n t s > 1 0 - R e n a l a n d U r o l o g i c P r o b l e ms > 1 0 . 4 - U r i n a r y
Incontinence in Adults
10.4
Urinary Incontinence in Adults
Richard Rathe
I . Background
U r i n a r y i n c o n t i n e n c e ( U I ) i s t h e i n v o l u n t a r y l o s s o f u r i n e a t t i me s a n d i n a mo u n t s t h
i n t e r f e r e w i t h h y g i e n e a n d a c t i v i t i e s o f d a i l y l i v i n g . U I i s o n e o f t h e mo s t p r e v a l e n t
a n d u n d e r d i a g n o s e d a f f l i c t i o n s i n t h e U n i t e d S t a t e s . I t i s a ma j o r c a u s e o f s o c i a l
w i t h d r a w a l a n d l o s s o f i n d e p e n d e n t l i v i n g . Vi c t i ms a r e o f t e n t o o e mb a r r a s s e d t o
d i s c u s s t h i s p r o b l e m, e v e n w i t h t h e i r p h y s i c i a n . S o me e v e n v i e w i t a s a n a t u r a l p a r t
o f a g i n g , b u t t h i s i s n o t t h e c a s e . U I i s a s y mp t o m, n o t a d i s e a s e . U n d e r s t a n d i n g t h
types of disorders that cause incontinence is the key to correct diagnosis and
e f f e c t i v e t r e a t me n t .
A. Etiology
( S e e T a b l e s 1 0 . 4 .a1n d1 0 . 4 . 2). T h e D R I P mn e mo n i c i s o f t e n c i t e d t o r e me mb e r t h e
reversible (and curable) causes of U I:
1. D d e l i r i u m a n d d r u g s
2. R r e s t r i c t e d mo b i l i t y a n d r e t e n t i o n
3. I i n f e c t i o n , i n f l a mma t i o n , a n d i mp a c t i o n
4. P p o l y u r i a f r o m u n c o n t r o l l e d d i a b e t e s a n d o t h e r c o n d i t i o n s
B. Epidemiology
1. T h i r t e e n mi l l i o n A me r i c a n s a r e i n c o n t i n e n t ; 11 mi l l i o n a r e w o me n .
2. O n e i n f o u r w o me n a g e s 3 0 t o 5 9 h a v e e xp e r i e n c e d a n e p i s o d e o f U I .
3. M o r e t h a n 5 0 % o f e l d e r l y i n d i v i d u a l s a r e i n c o n t i n e n t .
4. E v e r y y e a r $ 1 6 . 4 b i l l i o n i s s p e n t o n i n c o n t i n e n c e - r e l a t e d c a r e .
5. E v e r y y e a r $ 1 . 1 b i l l i o n i s s p e n t o n d i s p o s a b l e p r o d u c t s1)f.o r a d u l t s (
372 / 652
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I I I . Evaluation
A. History
1. V o i d i n g h i s t o rI ty i s i mp o r t a n t t o f u l l y c h a r a c t e r i ze t h e p a t i e n t ' s p r o b l e m b y
t a k i n g a d e t a i l e d h i s t o r y, i n c l u d i n g t h e d u r a t i o n o f t h e s y mp t o ms , t i mi n g o f
P. 2 2 2
v o l u n t a r y / i n v o l u n t a r y v o i d i n g , a mo u n t s v o i d e d i n v o l u n t a r i l y, a n d t h e r e l a t i o n s h i p
to voluntary voiding. Focus on the following areas:
TAB L E 10.4.1 Classification of Urinary

incontinence
Ty p e
De finition
Me chanism
Disorde rs
Urge
Inability to
Detrusor
delay voiding hyperactivity
once the urge
occurs
I d i o p a t h i c c o mmo n i n t h e
elderly)
Genitourinary conditions
(cystitis, stones)
Stress
Loss of urine Sphincter

with
failure
increased
a b d o mi n a l
pressure
We a k o r i n j u r e d p e l v i c
mu s c l e s
Sphincter weakness
Overflow
Partial
retention of
urine behind
an
obstruction
Outlet
obstruction
Loss of
innervation
Obstruction (prostate,
cystocele)
Neuropathic (diabetes,
nerve injury)
F unctional Inability to
get to the
t o i l e t i n t i me
Physical or
cognitive
i mp a i r me n t
D e me n t i a o r d e l i r i u m
P h y s i c a l l i mi t a t i o n s ( l a c k
o f mo b i l i t y )
Psychological/behavioral
M i xe d
Any
A n y c o mb i n a t i o n o f t h e
c o mb i n a t i o n a b o v e
of the above
Any
c o mb i n a t i o n
of the above
a. N e e d f o r p a d s o r d i a p e r s ( me a s u r e o f s e v e r i t y )
b. L o s s o f u r i n e w i t h c o u g h i n g o r l a u g h i n g ( s u g g e s t s s t r e s s t y p e )
c. I n a b i l i t y t o h o l d u r i n e a f t e r h a v i n g t h e u r g e t o u r i n a t e ( s u g g e s t s u r g e t y p e )
373 / 652
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d. P a i n o r d i s c o mf o r t ( s u g g e s t s i n f e c t i o n o r i n f l a mma t i o n )
e. I n a b i l i t y t o f u l l y e mp t y b l a d d e r ( s u g g e s t s o b s t r u c t i o n )
f. D e c r e a s e d u r i n a r y s t r e a m ( s u g g e s t s o b s t r u c t i o n )
g. I mp a c t o f U I o n t h e p a t i e n t ' s l i f e
h. W h a t t h e p a t i e n t t h i n k s i s g o i n g o n
2. V o i d i n g j o u r n A
a l v o i d i n g j o u r n a l i s a g o o d w a y t o g e t a d d i t i o n a l i n f o r ma t i o n
a b o u t t h e p a t i e n t ' s p r o b l e m. H a v e t h e p a t i e n t r e c o r d t h e t i me a n d a p p r o xi ma t e
a mo u n t o f e a c h v o i d i n g , a n d w h e t h e r t h e y w e r e w e t o r d r y.
TAB L E 10.4.2 Major causes of Urinary

I ncontinence
Acute
P r i ma r y d i s o r d e r s
Chronic
Local processes
Infection,
Pelvic floor weakness following childbirth,
me d i c a t i o n s , d e l i r i u mb l a d d e r t u mo r o r d e f o r mi t y, t u mo r s , o b s t r u c t i o n
by an enlarged prostate, cystocele or fecal
i mp a c t i o n
Postsurgical
E xa c e r b a t i o n s o f
s y s t e mi c d i s e a s e s
S y s t e mi c p r o c e s s e s
D i a b e t e s me l l i t u s ,
diabetes insipidus,
congestive heart
failure, stroke
M e n o p a u s e , n e u r o p a t h y ( d i a b e t e s , a l c o h o l i s m) ,
d e me n t i a , d e p r e s s i o n , s t r o k e , t u mo r,
P. 2 2 3
3. M a j o r m e d i c a l p r o b l e D
mose s t h e p a t i e n t h a v e a n y k n o w n c o n d i t i o n t h a t i s
a s s o c i a t e d w i t h U I ? T h e s e i n c l u d e d i a b e t e s , h e a r t f a i l u r e , me n o p a u s e , a n d
n e u r o l o g i c p r o b l e ms . D o e s t h e p a t i e n t h a v e o t h e r g e n i t o u r i n a r y s y mp t o ms ? I n
f e ma l e p a t i e n t s , b e s u r e t o t a k e a d e t a i l e d o b s t e t r i c a l h i s t o r y.
4. M e d i c a t i o n h i s t oBr ye c a u s e me d i c a t i o n s a r e a ma j o r c a u s e o f i n c o n t i n e n c e , b e
s u r e t o t a k e a t h o r o u g h me d i c a t i o n h i s t o r y. O f f e n d i n g a g e n t s i n c l u d e d i u r e t i c s ,
older antidepressants, antihypertensives, narcotics, and alcohol.
5. D i a b e t e s i n s i p i d C
u se n t r a l o r n e p h r o g e n i c d i a b e t e s i n s i p i d u s c a n p r e s e n t w i t h
U I d u e t o i n c r e a s e d u r i n e o u t p u t ( ma n y l i t e r s p e r d a y ) . T h e s e p a t i e n t s f r e q u e n t l
h a v e a c o n c o mi t a n t p o l y d i p s i a t h a t c l o s e l y ma t c h e s t h e i r w a t e r l o s s . Yo u s h o u l d
c o n s i d e r t h i s d i a g n o s i s w h e n t h e p a t i e n t g i v e s a h i s t o r y o f v o i d i n g l a r g e v o l u me
374 / 652
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of urine.
T h e e xa mi n a t i o n i s o f t e n n o r ma l i n c a s e s o f U I . Yo u s h o u l d f o c u s y o u r e f f o r t s i n a n
a t t e mp t t o u n c o v e r t h e u n d e r l y i n g c a u s e ( s ) :
1. G e n e r a l ( i s t h e p a t i e n t p h y s i c a l l y c a p a b l e o f g e t t i n g t o t h e t o i l e t ? )
2. M e n t a l s t a t u s ( c a n t h e p a t i e n t u n d e r s t a n d a n d a c t o n t h e i r u r g e t o v o i d ? )
3. N e u r o l o g i c i n c l u d i n g t h e a n a l r e f l e x ( f o c a l s i g n s s u g g e s t a n e u r o l o g i c c a u s e )
4. A b d o mi n a l ( i s t h e b l a d d e r d i s t e n d e d ? )
5. R e c t a l / p r o s t a t e ( i mp a c t i o n o r e n l a r g e d p r o s t a t e p r e s e n t ? )
6. P e l v i c ( a t r o p h i c v a g i n i t i s , p r o l a p s e , o r ma s s p r e s e n t ? )
C. Testing
1. U r i n a l y s i sB e c a u t i o u s i n t e r p r e t i n g t h e u r i n a l y s i s ; i n t h e a b s e n c e o f o t h e r
s y mp t o ms , b a c t e r i u r i a i s s e l d o m t h e p r i ma r y c a u s e o f U I . T r e a t c y s t i t i s o r
u r e t h r i t i s w h e n t h e y a r e c o n f i r me d b y t h e r e s t o f t h e c l i n i c a l p i c t u r e . A u r o l o g i s t
s h o u l d i n v e s t i g a t e u n e xp l a i n e d , p e r s i s t e n t mi c r o h e ma t u r i a .
2. P o s t v o i d i n g u r i n e v o l uTmhee p a t i e n t s h o u l d b e s t r a i g h t c a t h e t e r i ze d
i mme d i a t e l y a f t e r v o i d i n g . I n g e n e r a l , t h e p o s t v o i d u r i n e v o l u me s h o u l d b e < 5 0
mL . Vo l u me s i n t h e r a n g e o f 1 0 0 t o 2 0 0 mL ma y s u g g e s t i mp a i r e d b l a d d e r
c o n t r a c t i l i t y o r o b s t r u c t i o n . Vo l u me s > 2 0 0 mL s t r o n g l y s u g g e s t o b s t r u c t i o n .
3. B l o o d u r e a n i t r o g e n , c r e a t i n i n e , a n d gTl uhceos se es i mp l e b l o o d t e s t s h e l p
rule out underlying renal disease and diabetes.
4. S p e c i a l t e s tCs e r t a i n t e s t s a r e a v a i l a b l e t h r o u g h u r o l o g i c c o n s u l t a t i o n t o
f u r t h e r d e l i n e a t e t h e c a u s e o f U I . T h e s e i n c l u d e c y s t o s c o p y, c y s t o me t r y, a n d
other voiding studies. Up to two-thirds of patients can be successfully treated
w i t h o u t u r o l o g i c r e f e r2)r a. l (
D. Genetics
R e c e n t r e s e a r c h i n d i c a t e s t h a t t h e r e ma y b e a g e n e t i c p r e d i s p o s i t i o n f o r U I i n
w o me n . F o r a w o ma n w i t h a h i s t o r y o f i n c o n t i n e n c e i n h e r mo t h e r o r o l d e r s i s t e r s t h
r e l a t i v e r i s k w a s 1 . 3 . T h i s i n c r e a s e d t o 1 . 9 i f t h e s y mp t o ms w e r e r e p o r t e d a s
severe. T hese results were consistent, regardless of the type(s) of incontinence
p r e s e n t 3)( .
I V. Diagnosis
C l i n i c a l h i s t o r y i s t h e mo s t i mp o r t a n t f a c t o r l e a d i n g t o t h e c o r r e c t d i a g n o s i s a n d
s u c c e s s f u l t r e a t me n t o f U I . H o w e v e r, i t i s a n i mp e r f e c t t o o l a t b e s t . I n o n e r e v i e w,
clinical history had a sensitivity and specificity for stress incontinence of 0.90 and
0 . 5 0 r e s p e c t i v e l y. F o r d e t r u s o r i n s t a b i l i t y t h e f i g u r e s w e r e 0 .4)7 .4 Tahned 0 . 5 5 (
375 / 652
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t a s k b e c o me s e v e n mo r e p r o b l e ma t i c w h e n o n e c o n s i d e r s t h e r e l u c t a n c e o f p a t i e n t s
t o t a l k a b o u t t h e i r s y mp t o ms , a n d t h e t e n d e n c y f o r U I t o b e o f a mi xe d t y p e .
R e s p o n s e t o t h e r a p y ( o r l a c k t h e r e o f ) o f t e n d r i v e s t h e p r a c t i c a l ma n a g e me n t o f t h i s
c o n d i t i o n . L a c k o f r e s p o n s e t o mu l t i p l e t r i a l s o f t h e r a p y i s a g o o d i n d i c a t i o n f o r
c o n s u l t i n g a n u r o l o g i s t . R e me mb e r t h a t y o u r i n i t i a l a s s e s s me n t i s o f t e n i n c o r r e c t , s
k e e p a n o p e n mi n d a n d d o n o t d i s c o u n t a n y p o s s i b l e d i a g n o s i s . F i n a l l y, r e me mb e r
t h a t t h e r e a r e o f t e n mu l t i p l e s i mu l t a n e o u s f a c t o r s l e a d i n g t o U I . F o r e xa mp l e , ma n y
o t h e r w i s e h e a l t h y e l d e r l y i n d i v i d u a l s h a v e p h y s i c a l l i mi t a t i o n s t h a t c a n c o n t r i b u t e a
f u n c t i o n a l c o mp o n e n t t o t h e i r p r o b l e m.
References
1 . O v er v i ew: ur i nar y i nc ont i nenc e i n adul t s , c l i ni c al pr ac t i c e gui .del i ne updat e
( M a r c h 1 9 9 6 ) h t t p : / / w w w. a h r q . g o v / c l i n i c / u i o v e r v w. h t m, a c c e s s e d o n J u l y 2 0 0 5 .
P. 2 2 4
2 . We i s s B D . D i a g n o s t i c e v a l u a t i o n o f u r i n a r y i n c o n t i n e n c e i n g e r i a t r i c p a t i e n t s .
1 9 9 8 ; 5 7 ( 11 ) : 2 6 6 5 2 6 8 7 .
h t t p : / / w w w. a a f p . o r g / a f p / 9 8 0 6 0 0 a p / w e i s s . h t ml , a c c e s s e d o n J u l y 2 0 0 5 .
3 . H a n n e s t a d Y S . F a mi l i a l r i s k o f u r i n a r y i n c o n t i n e n c e i n w o me n : p o p u l a t i o n
b a s e d c r o s s s e c t i o n a l s tBu M
d y.
J 2004;329:889891.
h t t p : / / b mj . b mj j o u r n a l s . c o m/ c g i / c o n t e n t / f u l l / 3 2 9 / 7 4 7 1 / 8 8 9 / , a c c e s s e d o n J u l y 2 0 0 5 .
4. Jensen J K, Nielsen F R, Ostergard D R. T he role of patient history in the
d i a g n o s i s o f u r i n a r y i n c o n t i nO
e nbs
c et et
. G y nec ol
1994;83(5):904910.
376 / 652
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10.5 - Nocturia
> T a b l e o f C o n t e n t s > 1 0 - R e n a l a n d U r o l o g i c P r o b l e ms > 1 0 . 5 - N o c t u r i a
10.5
Nocturia
Vince nt H. Obe r
I . Background
N o c t u r i a i s u r i n a t i o n d u r i n g t h e t i me t h a t a p a t i e n t i s u s u a l l y s l e e p i n g . N o c t u r i a i s a
u n i q u e c l i n i c a l s y n d r o me b u t ma y b e a s s o c i a t e d w i t h ma n y d i f f e r e n t me d i c a l
p r o b l e ms1)( .
A. Etiology
T he causes of nocturia are usually divided into three groups: conditions that
i n c r e a s e u r i n e v o l u me , c o n d i t i o n s a s s o c i a t e d w i t h d i s o r d e r e d s l e e p , a n d
d i s t u r b a n c e s o f t h e l o w e r u r i n a r y2)t.r aHcot w( e v e r, a p a t i e n t ma y h a v e mu l t i p l e
contributing etiologies.
1. Vo l u me - r e l a t e d c o n d i t i o n s
a. E xc e s s i v e i n t a k e o f f l u i d s
b. D i e t a r y s u b s t a n c e s a n d me d i c a t i o n s t h a t i n c r e a s e u r i n e o u t p u t , i n c l u d i n g
a l c o h o l , c a f f e i n e , d i u r e t i c s , l i t h i u m, a n d t h e o p h y l l i n e
c. D i s e a s e s a s s o c i a t e d w i t h i n c r e a s e d s o l u t e e xc r e t i o n s u c h a s d i a b e t e s
me l l i t u s a n d h y p e r c a l c e mi a
d. D i a b e t e s i n s i p i d u s ( c e n t r a l a n d n e p h r o g e n i c )
e. P e r i p h e r a l e d e ma f r o m e xc e s s i v e s o d i u m i n g e s t i o n , c o n g e s t i v e h e a r t f a i l u r e ,
h e p a t i c d i s e a s e , s l e e p a p n e a , r e n a l i n s u f f i c i e n c y, h y p o a l b u mi n e mi a , a n d
me d i c a t i o n s ( e . g . , d i h y d r o p y r i d i n e c a l c i u m c h a n n e l b l o c k e r s a n d
t h i a zo l i d i n e d i o n e s )
f. I n c r e a s e d n i g h t t i me u r i n e s e c r e t i o n ( n o c t u r n a l p o l y u r i a ) d u e t o a g e - r e l a t e d
d e c r e a s e d r e n a l c o n c e n t r a t i n g a b i l i t y, d e c r e a s e d r e n a l s o d i u m c o n s e r v a t i o n ,
d e c r e a s e d n o c t u r n a l a n t i d i u r e t i c h o r mo n e s e c r e t i o n , a n d i n c r e a s e d a t r i a l
n a t r i u r e t i c h o r mo n e s e c r e3)
tion (
2. S l e e p - r e l a t e d c o n d i t i o n s
a. I n s o mn i a
b. R e s t l e s s l e g s y n d r o me
377 / 652
tmdmss
10.5 - Nocturia
c. C h r o n i c p a i n s y n d r o me s
d. S h o r t n e s s o f b r e a t h a s s o c i a t e d w i t h p u l mo n a r y o r c a r d i a c d i s e a s e
e. D i e t a r y s u b s t a n c e s s u c h a s c a f f e i n e o r d r u g s ( e . g . , v e r y s h o r t a c t i n g
hypnotics)
f. N e u r o l o g i c d i s o r d e r s s u c h a s d e me n t i a a n d P a r k i n s o n ' s d i s e a s e
3. L o w e r u r i n a r y t r a c t c o n d i t i o n s
a. U r i n a r y t r a c t i n f e c t i o n , i n c l u d i n g c y s t i t i s a n d p r o s t a t i t i s
b. C h r o n i c i n t e r s t i t i a l c y s t i t i s
c. S ma l l b l a d d e r c a p a c i t y b e c a u s e o f p e l v i c ma s s , a d h e s i o n s , o r s u r g e r y
d. D e t r u s o r h y p e r a c t i v i t y ( b o t h n e u r o g e n i c a n d n o n - n e u r o g e n i c )
e. U r i n a r y r e t e n t i o n d u e t o b l a d d e r o u t l e t o b s t r u c t i o n o r d e t r u s o r w e a k n e s s
f. D e c r e a s e d b l a d d e r c o mp l i a n c e d u e t o a g i n g , t u mo r, o r r a d i a t i o n
B. Epidemiology
T h e i n c i d e n c e o f n o c t u r i a i s r e p o r t e d i n 4 % o f c h i l d r e n 7 4)
t o a1n5dy iena rusp (t o
9 1 % o f me n o l d e r t h a n 8 0 y e a r s o f a g e . O v e r a l l , t h e i n c i d e n c e
P. 2 2 5
i s h i g h e r i n me1,n 5)
( . T h e r e a r e ma n y c a u s e s f o r t h e i n c r e a s e d i n c i d e n c e w i t h a g e ,
b u t i n c r e a s e d n o c t u r i a i s ma i n l y r e l a t e d t o a d e c r e a s e i n b l a d d e r c a p a c i t y a n d a n
increase in nocturnal urine secretion (with no change in 24-hour urine production).
I I I . Evaluation
A. History
T he history should include the following: (i) a voiding diary (for 2472 hours), with
me a s u r e me n t o f u r i n e v o l u me s t o e s t a b l i s h b l a d d e r c a p a c i t y ( a v e r a g e v o i d i n g
v o l u me ) , t o t a l u r i n e o u t p u t , a n d d i u r n a l v o i d i n g p a t t e r n s ; ( i i ) t h e v o l u me o f f l u i d
i n t a k e ; ( i i i ) t h e me d i c a l h i s t o r y ; ( i v ) t h e s u r g i c a l h i s t o r y ; ( v ) t h e me d i c a t i o n s u s e d ;
d i e t a r y h a b i t s ; ( v i i ) s l e e p h i s t o r y ; a n d ( v i i i ) r e s p o n s e t o s e l f t r e a t me n t s s u c h a s f l u i
caffeine, and alcohol restriction.
T h e e xa mi n a t i o n s h o u l d i n c l u d e p u l mo n a r y, c a r d i a c , a b d o mi n a l , f e ma l e p e l v i c , r e c t a
and neurologic evaluations.
C. Testing
1. L a b o r a t o r y t e s t s s h o u l d i n c l u d e r o u t i n e d i p s t i c k u r i n a l y s i s a n d b l o o d c h e mi s t r i e
( t o i n c l u d e b l o o d u r e a n i t r o g e n [ B U N ] , c r e a t i n i n e , g l u c o s e , c a l c i u m, a l b u mi n ) .
P o s t v o i d r e s i d u a l u r i n e s h o u l d b e me a s u r e d ( < 5 0 mL i s n o r ma l ) . U r i n e c u l t u r e
ma y b e u s e f u l i f t h e p a t i e n t h a s s y mp t o ms o f f r e q u e n c y a n d d y s u r i a a n d a
n o r ma l u r i n a l y s i s .
378 / 652
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10.5 - Nocturia
2. S p e c i a l i ze d t e s t i n g s u c h a s c y s t o s c o p y, u r o d y n a mi c t e s t i n g , a n d s l e e p s t u d i e s
are rarely needed.
I V. Diagnosis
A.
Vo l u me - r e l a t e d c a u s e s f o r n o c t u r i a a r e i n d i c a t e d b y a t o t a l d a i l y u r i n e o u t p u t > 4 0
mL / k g / d a y. E xc e s s i v e f l u i d , c a f f e i n e , a n d a l c o h o l i n t a k e a r e d e t e c t e d b y t h e p a t i e n t
h i s t o r y. S e r u m g l u c o s e i s e l e v a t e d i n d i a b e t e s me l l i t u s . S e r u m c a l c i u m i s e l e v a t e d i
h y p e r c a l c e mi a . U r i n e s p e c i f i c g r a v i t y i s < 1 . 0 1 0 i n d i a b e t e s i n s i p i d u s . T h e h i s t o r y a
p h y s i c a l e xa mi n a t i o n s h o u l d d e t e c t me d i c a l o r s u r g i c a l p r o b l e ms a n d me d i c a t i o n s
associated with increased nocturnal urine production. Renal insufficiency is
diagnosed by an elevated serum creatinine and B U N. Nocturnal polyuria is indicate
b y n o c t u r n a l u r i n e v o l u me > 3 3 % o f t h e t o t a l 2 4 - h o u r u r i n e v o l u me .
B.
S l e e p - r e l a t e d c a u s e s a r e s u g g e s t e d b y a p a t i e n t h i s t o r y o f i n s o mn i a , a c u t e o r
chronic pain, or breathlessness. Dietary substances or drugs that disrupt sleep, as
w e l l a s d e me n t i a a n d n e u r o l o g i c d i s e a s e s , s h o u l d b e d e t e c t e d b y t h e h i s t o r y a n d
p h y s i c a l . S l e e p s t u d i e s a r e u s u a l l y n o t r e q u i r e d . N i g h t t i me v o i d e d v o l u me w i t h e a c h
mi c t u r i t i o n l e s s t h a n a v e r a g e d a y t i me v o i d e d v o l u me ( b l a d d e r c a p a c i t y ) s u g g e s t s
d i s o r d e r e d s l e e p o r u r i n a r y t r a c t a b n o r ma l i t i e s .
C.
L o w e r u r i n a r y t r a c t c a u s e s a r e i d e n t i f i e d b y t h e h i s t o r y, u r i n a l y s i s , a n d me a s u r e me n
of postvoid residual urine. Pyuria usually indicates urinary tract infection. L ower
u r i n a r y t r a c t i r r i t a t i v e s y mp t o ms ( f r e q u e n c y a n d d y s u r i a ) a n d a n o r ma l u r i n a l y s i s a n
u r i n e c u l t u r e s u g g e s t i n t e r s t i t i a l c y s t i t i s ( a n d i s c o n f i r me d b y c y s t o s c o p y ) . S ma l l
v o i d e d v o l u me t h r o u g h o u t t h e d a y a n d n i g h t i n d i c a t e s s ma l l b l a d d e r c a p a c i t y,
d e c r e a s e d b l a d d e r c o mp l i a n c e , o r d e t r u s o r h y p e r a c t i v i t y. P o s t v o i d r e s i d u a l u r i n e > 5
mL i n d i c a t e s u r i n a r y r e t e n t i o n t h a t ma y b e c a u s e d b y p r o s t a t i c o b s t r u c t i o n o r
i n a d e q u a t e d e t r u s o r c o n t r a c t i o n s . U r o d y n a mi c s t u d i e s c a n b e h e l p f u l i n c o mp l e x
cases.
References
1 . Va n K e r r e b r o e c k P, A b r a ms P, C h a i k i n D , e t a l . T h e s t a n d a r d i za t i o n o f
t e r mi n o l o g y i n n o c t u r i a : r e p o r t f r o m t h e s t a n d a r d i za t i o n s u b c o mmi t t e e o f t h e
i n t e r n a t i o n a l c o n t i n e n c e s oB
c iJeUt y.I nt2 0 0 2 ; 9 0 ( S 3 ) : 11 1 5 .
2. Resnick N M. Urinary incontinence. In: Cassel C K , Cohen H J , L arson E B ,
e t a l . , e d sG. er i at r i c M edi c,i ne
3 r d e d . N e w Yo r k , N Y : S p r i n g e r - Ve r l a g , 1 9 9 7 : 5 6 2
566.
3. Miller M. Nocturnal polyuria in older people: pathophysiology and clinical
i mp l i c a t i o n sJ . A m G er i at r S oc
2000;48(10):13211329.
379 / 652
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10.5 - Nocturia
4 . M a t t s s o n S . U r i n a r y i n c o n t i n e n c e a n d n o c t u r i a i n h e a l t h s c hAocotlac h i l d r e n .
P aedi at r1 9 9 4 ; 8 3 : 9 5 0 9 5 4 .
5 . M a l ms t e n U G H , M i l s o n I , M o l a n d e r U , e t a l . U r i n a r y i n c o n t i n e n c e a n d l o w e r
u r i n a r y t r a c t s y mp t o ms : a n e p i d e mi o l o g i c a l s t u d y o f me n a g e d 4 5J t o 9 9 y e a r s .
U r ol 1 9 9 7 ; 1 5 8 ( 5 ) : 1 7 3 3 1 7 3 7 .
380 / 652
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10.6 - Oliguria and Anuria
> T a b l e o f C o n t e n t s > 1 0 - R e n a l a n d U r o l o g i c P r o b l e ms > 1 0 . 6 - O l i g u r i a a n d
Anuria
10.6
Oliguria and Anuria
M a r c i a W. F u n d e r b u r k
I . Background
O l i g u r i a a n d a n u r i a s h o u l d b e r e c o g n i ze d p r o mp t l y s o t h a t t h e c a u s e c a n b e
i d e n t i f i e d a n d t r e a t me n t i n i t i a t e d t o p r e s e r v e r e n a l f u n c t i o n a n d p r e v e n t l i f e t h r e a t e n i n g c o mp l i c a t i o n s . O l i g u r i a i s d e f i n e d a s u r i n e v o l u me < 5 0 0 mL / 2 4 h o u r o r
< 2 0 mL / h o u r ( i n s ma l l c h i l d r e n , < 0 . 8 mL / k g / h o u r ) . A n u r i a i s s t r i c t l y d e f i n e d a s t h e
t o t a l a b s e n c e o f u r i n e ; i n t h e c l i n i c a l s e t t i n g , h o w e v e r, a u r i n e o u t p u t o f < 5 0 t o 1 0 0
mL / 2 4 h o u r i s o f t e n c o n s i d e r e d a1)
n u. rTi ah e( a mo u n t o f u r i n e o u t p u t mu s t b e
r e l i a b l y e s t a b l i s h e d ( mo s t r e l i a b l y me a s u r e d u s i n g a n i n d w e l l i n g c a t h e t e r ) .
A. Etiology
1. O l i g u r i aI t i s h e l p f u l t o t h i n k o f o l i g u r i a a s p r e r e n a l , r e n a l , o r p o s t r e n a l .
a. P r e r e n a l d i s o r d e r s a r e c h a r a c t e r i ze d b y d e c r e a s e d r e n a l p e r f u s i o n , l e a d i n g
t o d e c r e a s e d g l o me r u l a r f i l t r a t i o n r a t e s u c h t h a t t h e d a i l y e n d o g e n o u s l o a d
o f n i t r o g e n o u s w a s t e s c a n n o t b e e xc r e t e d , t h e r e f o r e t h e t e r m p r e r e n a l
a zo t e mi a .
b. R e n a l d i s o r d e r s a r e c h a r a c t e r i ze d b y p a t h o l o g y w i t h i n t h e k i d n e y
p a r e n c h y ma i t s e l f , w h i c h c a n b e t h e e n d r e s u l t o f p r o l o n g e d d e c r e a s e d
renal perfusion.
c. P o s t r e n a l d i s o r d e r s c a u s i n g o l i g u r i a a r e c h a r a c t e r i ze d b y t h e p a r t i a l
o b s t r u c t i o n o f t h e u r i n a r y t r a c t a t a n a n a t o mi c p o s i t i o n d i s t a l t o t h e k i d n e y.
T his can be confusing, because these disorders can also cause polyuria.
2. A n u r i a r e s u l t s f r o m t h e t o t a l o b s t r u c t i o n o f t h e u r i n a r y t r a c t , o r a s a n e n d r e s u l t
of the prerenal and renal causes of oliguria. A sudden cause of anuria,
e s p e c i a l l y i n t h e e l d e r l y, i s b i l a t e r a l r e n a l a r t e r y o c c l u s i o n ( o r u n i l a t e r a l
o c c l u s i o n i n a s i n g l e k i d n e y ) t y p i c a l l y c a u s e d b y e mb o l i s m. E a r l y r e c o g n i t i o n o f
t h i s e n t i t y i s i mp e r a t i v e t o r e s t o r e b l o o d f l o w t o t h e i s c h e mi c k i d n e y ( s ) .
I I I . Evaluation
381 / 652
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A. History
1. P e r t i n e n t p r e s e n t h i s tAo rpya t i e n t ma y c o mp l a i n o f d e c r e a s e d u r i n e o u t p u t i n
s o me c l i n i c a l s i t u a t i o n s . M o r e o f t e n , h o w e v e r, t h e c l i n i c a l s i t u a t i o n a n d p e r t i n e n
history should lead to an evaluation of the presence of oliguria or anuria.
a. A r e t h e r e s y mp t o ms o f i l l n e s s o r t r a u ma l e a d i n g t o h y p o t e n s i o n ?
i. H y p o v o l e mi a ( e . g . , h e mo r r h a g e , d i u r e t i c o v e r u s e , g a s t r o i n t e s t i n a l f l u i d
l o s s , s k i n f l u i d l o s s o w i n g t o b u r n s o r h e a t e xp o s u r e , t h i r d s p a c i n g
s e c o n d a r y t o b u r n s , p e r i t o n i t i s , p a n c r e a t i t i s , o r t r a u ma ) ?
i i. D e c r e a s e d c a r d i a c o u t p u t ( e . g . , c o n g e s t i v e h e a r t f a i l u r e , my o c a r d i a l
i n f a r c t i o n , p e r i c a r d i a l t a mp o n a d e , o r a c u t e p u l mo n a r y e mb o l u s ) ?
i i i. P e r i p h e r a l v a s o d i l a t a t i o n ( e . g . , s e p t i c s h o c k , a n a p h y l a c t i c s h o c k ) ?
b. A r e t h e r e s y mp t o ms o f v a s c u l a r d i s e a s e ? C o n s i d e r b i l a t e r a l r e n a l v a s c u l a r
o b s t r u c t i o n d u e t o s e v e r e r e n a l a r t e r y s t e n o s i s , t h r o mb o s i s , o r e mb o l i s m.
c. I s t h e r e a n y h i s t o r y c o n s i s t e n t w i t h r e n a l p a r e n c h y ma l i n j u r y ( e . g . , r e c e n t
r a d i o c o n t r a s t a g e n t , n e p h r o t o xi n e xp o s u r e s u c h a s e t h y l e n e g l y c o l , n o n s t e r o i d a l a n t i - i n f l a mma t o r y d r u g o v e r d o s e , a c u t e n e p h r i t i s , a c u t e v a s c u l i t i s ,
pyelonephritis [in the elderly], papillary necrosis [in patients with diabetes],
or prolonged hypotension with hypoperfusion of the kidney)?
d. I s t h e r e a n y h i s t o r y c o n s i s t e n t w i t h u r i n a r y t r a c t o b s t r u c t i o n ?
i. B l a d d e r n e c k o b s t r u c t i o n ( e . g . , b e n i g n p r o s t a t i c h y p e r t r o p h y, p r o s t a t e
c a n c e r, b l a d d e r c a n c e r, o r f u n c t i o n a l o b s t r u c t i o n d u e t o d r u g s i d e
effects)?
i i. O b s t r u c t i o n o f t h e u r e t h r a o r b i l a t e r a l u r e t e r s i n t e r n a l l y ( s e c o n d a r y
b l o o d c l o t s , s t o n e s , s u l f o n a mi d e o r u r i c a c i d c r y s t a l s , p y o g e n i c d e b r i s ,
n e c r o t i zi n g p a p i l l i t i s o r e d e ma ) , o r e xt e r n a l l y ( s e c o n d a r y t u mo r s ,
periureteral
P. 2 2 7
f i b r o s i s , a c c i d e n t a l u r e t e r a l l i g a t i o n d u r i n g p e l v i c s u r g e r y, a s c i t e s ,
p r e g n a n c y, p e l v i c a b s c e s s , o r h e ma t o ma ) .
e. M e d i c a t i o n u s e mu s t b e c o n s i d e r e d d i u r e t i c s , a n t i h y p e r t e n s i v e s ,
a n t i c h o l i n e r g i c s , a mi n o g l y c o s i d e s , a mp h o t e r i c i n B , o r c h e mo t h e r a p e u t i c
drugs.
2. P e r t i n e n t p a s t h i s t oI sr yt h e r e a h i s t o r y o f c a n c e r, r e c e n t s u r g e r y, k i d n e y
s t o n e s , n e u r o l o g i c d i s o r d e r, v a s c u l a r d i s e a s e , c h r o n i c l i v e r d i s e a s e ( h e p a t o r e n a
s y n d r o me ) , o r k i d n e y t r a n s p l a n t ?
1. F o c u s e d p h y s i c a l e x a m i n aTt ihoi ns s h o u l d i n c l u d e v i t a l s i g n s ( n o t a b l y b l o o d
p r e s s u r e , p u l s e , a n d t e mp e r a t u r e ) . O r t h o s t a t i c b l o o d p r e s s u r e a n d p u l s e ma y b e
n e c e s s a r y. S i g n s o f h y p o v o l e mi a , h y p o t e n s i o n , a n d d e h y d r a t i o n s k i n t u r g o r
a n d c o l o r, mu c o u s me mb r a n e s , c a p i l l a r y r e f i l l , w a r mt h o f e xt r e mi t i e s s h o u l d b e
382 / 652
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noted.
2. A d d i t i o n a l p h y s i c a l e x a m i n aDt ei opne n d i n g o n t h e h i s t o r y ( e . g . , s k i n r a s h ,
c a r d i a c e xa mi n a t i o n , b r u i t s o v e r k i d n e y s ) , p a l p a t e f o r a d i s t e n d e d b l a d d e r ; i f a
c a n c e r o r o u t l e t o b s t r u c t i o n i s s u s p e c t e d , p e r f o r m a r e c t a l o r p e l v i c e xa mi n a t i o n
C. Testing
1. A n i n d w e l l i n g u r i n a r y c a t h e t e r s e r v e s a s a d i a g n o s t i c t o o l ( i f o b s t r u c t i o n h a s
o c c u r r e d a t t h e b l a d d e r n e c k o r u r e t h r a ) a n d f o r a c c u r a t e u r i n e v o l u me
me a s u r e me n t . U r i n e o u t p u t a n d b l o o d p r e s s u r e mo n i t o r i n g c a n o f t e n l e a d t o
e xp e d i e n t c o r r e c t i o n o f p r e r e n a l c a u s e s , t h e r e b y a v o i d i n g f u r t h e r c o mp l i c a t i o n s .
2. U r i n a l y s i s i s o f t e n n o r ma l i n p r e r e n a l c a u s e s o f o l i g u r i a o r a n u r i a , e xc e p t b e i n g
highly concentrated with possible qualitative proteinuria because of the high
c o n c e n t r a t i o n . M i c r o s c o p i c a n a l y s i s i s u s u a l l y u n r e ma r k a b l e ( o r r e v e a l s f e w
hyaline or granular casts) in prerenal causes; whereas proteinuria, casts, and
h e ma t u r i a c a n p o i n t t o r e n a l c a u s e s .
3. U r i n e o s mo l a l i t y i s t y p i c a l l y h i g h i n p r e r e n a l c a u s e s ( > 5 0 02 OmO
) s m/ k g H
v e r s u s i mp a i r e d i n r e n a l c a u s e s ( < 3 5 0 mO2sOm/
) (2)
k g. H
4. U r i n e s o d i u m i s t y p i c a l l y < 2 0 mE q / L i n p r e r e n a l c a u s e s ( u n l e s s d i u r e t i c s h a v e
b e e n u s e d ) v e r s u s > 4 0 mE q / L i n r e n a l c1)a.u s e s (
5. B l o o d u r e a n i t r o g e n a n d c r e a t i n i n e l e v e l s a r e e l e v a t e d . T h e r a t i o mu s t b e
i n t e r p r e t e d c o n s i d e r i n g t h e e n t i r e c l i n i c a l s i t u a t i o n . U r i n e : p l a s ma c r e a t i n i n e r a t
(U:P Cr) is calculated to help differentiate between prerenal (U:P Cr >40) and
r e n a l ( U : P C r < 2 0 ) c a u 1)
s e.s (
6. D i a g n o s t i c i ma g i n g , w h i c h ma y b e n e c e s s a r y i n s o me c a s e s , i s g u i d e d b y t h e
h i s t o r y a n d p h y s i c a l e xa mi n a t i o n f i n d i n g s ( e . g . , u l t r a s o u n d , c o mp u t e d
t o mo g r a p h y [ C T ] , r e t r o g r a d e p y e l o g r a m, r e n a l b i o p s y ) .
I V. Diagnosis
T he key to a diagnosis of oliguria or anuria is to actively anticipate when it is likely

t o ma n i f e s t a n d a c c u r a t e l y me a s u r e u s i n g a n i n d w e l l i n g c a t h e t e r. O n c e r e c o g n i ze d
a n d a c a u s e i s s u g g e s t e d , ( i ) p r e r e n a l c a u s e s c a n b e f u r t h e r a s s e s s e d b y me a s u r i n
h e mo d y n a mi c s t a t u s a n d a d mi n i s t e r i n g f l u i d s ; ( i i ) r e n a l c a u s e s c a n b e f u r t h e r
assessed with urinalysis (qualitative and quantitative), renal ultrasound, or renal
biopsy; and (iii) postrenal causes can be further assessed using ultrasound, C T
s c a n , o r r e t r o g r a d e p y e l o g r a p h y.
References
1 . M o l i t o r i s B A . O l i g u r i a a n d a n u r i a . I n : M a s s r y S G , G l a s s oM
c kasRsJr y, e d s .
and G l as s oc k ' s t ex t book of nephr
, ol
4 t ogy
h e d . P h i l a d e l p h i a , PA : L i p p i n c o t t ,
W i l l i a ms & W i l k i n s , 2 0 0 1 : 4 8 9 4 9 1 .
2 . G l a s s o c k R J , M a s s r y S G , H u me s H D . A c u t e r e n a l f a i l u r e i n c l u d i n g c o r t i c a l
383 / 652
tmdmss
n e c r o s i s , P a r t 2 d i a g n o s i s , c l i n i c a l p r e s e n t a t i o n , a n d ma n a g e me n t . I n : M a s s r y
S G , G l a s s o c k R J , eM
d sas
. s r y and G l as s oc k ' s t ex t book of nephr
, 4olt hogy
ed.
P h i l a d e l p h i a , PA : L i p p i n c o t t , W i l l i a ms & W i l k i n s , 2 0 0 1 : 9 7 1 .
384 / 652
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10.7 - Priapism
> T a b l e o f C o n t e n t s > 1 0 - R e n a l a n d U r o l o g i c P r o b l e ms > 1 0 . 7 - P r i a p i s m
10.7
Priapism
Dav id B. Fe lle r
I . Background
Priapism is defined as a persistent, often painful, penile erection not associated wi
s e xu a l s t i mu l a t i o n . N o t i me c o u r s e i s s p e c i f i c a l l y d e f i n e d , b u t p r i a p i s m i s u s u a l l y
d i a g n o s e d w h e n t h e e r e c t i o n l a s t s > 4 h o u r s . A l t h o u g h r e l a t i v e l y u n c o mmo n
( i n c i d e n c e 1 . 5 / 1 0 0 , 0 0 0 p e r s o n - y e a r s a n d 2 . 9 / 1 0 0 , 0 0 0 p e r s o n - y e a r s f o r me n 4 0 y e a r
o f a g e a n d o l d e1)r ), ( p r i a p i s m r e p r e s e n t s a u r o l o g i c e me 2)
r g.e n c y (
A. Etiology
Two types of priapism (low-flow or veno-occlusive and high-flow or arterial) have

b e e n d e s c r i b e d b a s e d o n t h e u n d e r l y i n g p r e c i p i t a t3)
i n.g Aer vt e rni ta l( p r i a p i s m
usually occurs after injury to the cavernous artery from perineal or direct penile
t r a u ma . T h i s i n j u r y t h e n l e a d s t o u n c o n t r o l l e d h i g h a r t e r i a l i n f l o w w i t h i n t h e c o r p o r a
c a v e r n o s a . Ve n o - o c c l u s i v e p r i a p i s m i s c h a r a c t e r i ze d b y i n a d e q u a t e o u t f l o w a n d i s f
a n d a w a y t h e mo s t c o mmo n . D i s t i n c t i o n b e t w e e n t h e t w o i s i mp e r a t i v e b e c a u s e
u l t i ma t e t r e a t me n t v a r i e s s i g n i f i c a n t l y.
B. Epidemiology
A h i s t o r y o f p e n i l e o r p e r i n e a l t r a u ma a l mo s t a l w a y s p r e c e d e s a r t e r i a l p r i a p i s m, a n d
i s t h e mo s t i mp o r t a n t h i s t o r i c a l i n f o r ma t i o n t h a t d i s t i n g u i s h e s b e t w e e n t h e t w o t y p e
o f p r i a p i s m. S t u d i e s h a v e s u g g e s t e d t h a t u p t o 4 1 % o f p a t i e n t s w h o p r e s e n t w i t h
p r i a p i s m ( v e n o - o c c l u s i v e ) h a v e t a k e n s o me t y p e o f p s y c h o t r o p i c me d i c a t i o n , u s u a l l y
n e u r o l e p t i c s , o r t r a zo d o n e , a n d e v e n w i t h t h e - b l o c k e4)r .p P
r ar zo
i a ps i snm( h a s
b e e n c o mmo n l y r e p o r t e d ( 1 % 1 7 % ) a f t e r i n t r a c a v e r n o u s i n j e c t i o n w i t h
p r o s t a g l a n d i n s f o r t h e t r e a t me n t o f e r e c t i l e d y s f u n c t i o n . S u b s e q u e n t l y,
t h e r a p e u t i c a l l y i n d u c e d p r o l o n g e d e r e c t i o n h a s b e c o me t h e p r i ma r y a n d mo s t
c o mmo n c a u s e o f p r i a p i s m. P a t i e n t s w i t h a n y h i s t o r y o f ma l i g n a n c y, e s p e c i a l l y
g e n i t o u r i n a r y o r p e l v i c c a r c i n o ma a n d n e w - o n s e t p r i a p i s m, s h o u l d b e e v a l u a t e d f o r
p e n i l e me t a s t a s i s . I n o n e r e v i e w, 2 0 % t o 5 3 % o f c a s e s w i t h p e n i l e me t a s t a s e s
p r e s e n t e d i n i t i a l l y w i t h p r i a5)
p i.s m (
T h e mo s t c o mmo n c a u s e o f p r i a p i s m i n c h i l d r e n i s s i c k l e c e l l d i s e a s e . I t h a s b e e n
reported that over 60% of all children with this disease eventually develop priapism
385 / 652
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10.7 - Priapism
I I I . Evaluation
A. History
S p e c i f i c q u e s t i o n s ma y h e l p i d e n t i f y t h e t y p e o f p r i a p i s m, c a u s e , a n d u r g e n c y o f
t r e a t me n t . A l w a y s i n q u i r e h o w l o n g t h e p r i a p i s m h a s b e e n p r e s e n t . H o w mu c h p a i n
d o e s t h e p a t i e n t e xp e r i e n c e ? M o d e r a t e t o s e v e r e , p e r s i s t e n t p a i n i s c h a r a c t e r i s t i c o
v e n o - o c c l u s i v e p r i a p i s m a n d r e s u l t s f r o m t i s s u e i s c h e mi a . P a i n i s g e n e r a l l y mu c h
mo r e mi l d o r t r a n s i e n t w i t h a r t e r i a l p r i a p i s m. I s t h e r e a h i s t o r y o f p e n i l e o r p e r i n e a l
t r a u ma ? T r a u ma mo r e c o mmo n l y p r e c e d e s a r t e r i a l p r i a p i s m. D o e s t h e p a t i e n t t a k e
a n y me d i c a t i o n s t h a t ma y p r e d i s p o s e t o p r i a p i s m? I s t h e r e a n y h i s t o r y o f
ma l i g n a n c y ? I s t h e r e a n y h i s t o r y o f s i c k l e c e l l d i s e a s e ?
T h e e xa mi n a t i o n s h o u l d i n c l u d e a t h o r o u g h g e n i t o u r i n a r y e xa mi n a t i o n t o l o o k f o r
t r a u ma o r ma l i g n a n c y. T h e c o r p o r a c a v e r n o s a b u t n o t t h e c o r p o r a s p o n g i o s u m i s
i n v o l v e d w i t h p r i a p i s m a n d , t h e r e f o r e , t h e g l a n s r e ma i n s f l a c c i d w h i l e t h e s h a f t i s
e r e c t a n d t e n d e r. T h e e xa mi n a t i o n s h o u l d a l s o i n c l u d e p a l p a t i o n f o r i n g u i n a l
l y mp h a d e n o p a t h y ( g e n i t o u r i n a r y ma l i g n a n c y ) , a n d a n a b d o mi n a l e xa mi n a t i o n
( a b d o mi n a l o r g e n i t o u r i n a r y ma l i g n a n c y a n d t r a u ma ) .
C. Testing
1. I n mo s t i n s t a n c e s , t h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n d e t e r mi n e t h e c a u s e o f
p r i a p i s m. A c o mp l e t e b l o o d c o u n t a n d s i c k l e c e l l s c r e e n ma y b e u s e f u l , l o o k i n g
f o r ma l i g n a n c y a n d s i c k l e c e l l d i s e a s e r e s p e c t i v e l y. C o a g u l a t i o n s t u d i e s a r e a l s
r e c o mme n d e d ( i n c a s e a s p i r a t i o n i s c o n t e mp l a t e d f o r t r e a t me n t ) . B l o o d g a s
me a s u r e me n t f r o m a c a v e r n o s a l s a mp l e ma y b e u s e f u l i f d i f f e r e n t i a t i o n b e t w e e n
l o w - f l o w v e r s u s h i g h - f l o w p r i a p i s m i s d2)i f. f i c u l t (
P. 2 2 9
2. I n mo s t c a s e s , f u r t h e r d i a g n o s t i c t e s t i n g i s n o t n e e d e d . I f o b j e c t i v e s t u d i e s a r e
n e e d e d , c o l o r f l o w D o p p l e r c a v e r n o s o n o g r a p h y i s t h e me t h o d o f c h o i c e f o l l o w e d
b y t e c h n e t i u m- 9 9 m p e n i l e s c a n n i n g a n d ma g n e t i c r e s o n a n c e i ma g i n g . I f p e l v i c
ma l i g n a n c y i s s u s p e c t e d , c o mp u t e d t o mo g r a p h y i s g e n e r a l l y t h e n e xt s t e p . I f
t r a u ma p r e c e d e d p r i a p i s m, a r t e r i o g r a p h y ma y b e i n d i c a t e d .
D. Genetics
S i c k l e c e l l a n e mi a , a n a u t o s o ma l r e c e s s i v e d i s o r d e r i s a s s o c i a t e d w i t h a h i g h
incidence of priapism (>40% of affected adults and >60% of affected children).
I V. Diagnosis
T h e k e y t o d e t e r mi n i n g t h e c a u s e o f p r i a p i s m i s t h e c l i n i c a l h i s t o r y. E xa mi n a t i o n
reveals an erect, usually tender penis, with flaccid glans. Early distinction between
a r t e r i a l a n d v e n o - o c c l u s i v e p r i a p i s m s h o u l d b e ma d e ; t h e f o r me r i s o f t e n a s s o c i a t e d
w i t h t r a u ma a n d l e s s p a i n f u l o r p a i n l e s s e r e c t i o n s . E v a l u a t i o n o f p r i a p i s m i s a i me d
386 / 652
tmdmss
10.7 - Priapism
d e t e r mi n i n g h o w l o n g i t h a s b e e n p r e s e n t , b e c a u s e p e r ma n e n t d a ma g e ma y o c c u r
w i t h i n a s l i t t l e a s 4 h o u r s , a n d a t d e t e r mi n i n g t h e c a u s e . T h e mo s t c o mmo n c a u s e s
a r e d u e t o p s y c h o t r o p i c me d i c a t i o n s o r me d i c a t i o n s f o r e r e c t i l e d y s f u n c t i o n . L e s s
c o mmo n c a u s e s i n c l u d e t r a u ma , s i c k l e c e l l d i s e a s e , a n d p e l v i c ma l i g n a n c y.
P r i a p i s m i s c o n s i d e r e d a u r o l o g i c e me r g e n c y a n d s h o u l d b e ma n a g e d a g g r e s s i v e l y.
W i t h o u t p r o mp t r e c o g n i t i o n a n d t r e a t me n t , p r i a p i s m ma y r e s u l t i n u r i n a r y r e t e n t i o n ,
c a v e r n o s a f i b r o s i s , i mp o t e n c e , o r e v e n g a n g r e n e . T r e a t me n t w i t h i n 4 t o 6 h o u r s o f
o n s e t h a s b e e n s h o w n t o d e c r e a s e mo r b i d i t y, t h e n e e d f o r i n v a s i v e p r o c e d u r e s , a n d
d e c r e a s e i mp o t e n c4)e. (
References
1. E land IA, van der L ei J , S trickler B H , et al. Incidence of priapism in the
g e n e r a l p o p u l a t i oUnr.ol ogy2 0 0 1 ; 5 7 : 9 7 0 9 7 2 .
2 . Vi l k e G M , H a r r i g a n R A , U f b e r g J W, e t a l . E me r g e n c y e v a l u a t i o n a n d t r e a t me n t
o f p r i a p i s m.
J E mer g M ed2 0 0 3 ; 2 6 ( 3 ) : 3 2 5 3 2 9 .
3 . M o n t a g u e D K , J a r o w J , B r o d e r i c k G A , e t a l . A me r i c a n U r o l o g i c a l A s s o c i a t i o n
g u i d e l i n e o n t h e ma n a g e me n t o f p r iJa pUi sr ol
m.2 0 0 3 ; 1 7 0 : 1 3 1 8 1 3 2 4 .
4 . T h o mp s o n J W J r, Wa r e M R , B l a s h f i e l d R K . P s y c h o t r o p i c me d i c a t i o n a n d
p r i a p i s m: a c o mp r e h e n s i v e r eJv iC
e w.
l i n P s y c hol
1990;51:430433.
5 . C h a n P T , B e g i n L R , A r n o l d D , e t a l . P r i a p i s m s e c o n d a r y t o p e n i l e me t a s t a s i s :
a r e p o r t o f t w o c a s e s a n d a r e v i e w o f t h e l iJt eSr aurt ugr eO. nc ol1 9 9 8 ; 6 8 : 5 1
59.
387 / 652
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10.8 - Scrotal Mass
> T a b l e o f C o n t e n t s > 1 0 - R e n a l a n d U r o l o g i c P r o b l e ms > 1 0 . 8 - S c r o t a l M a s s
10.8
Scrotal M ass
Ke ndall M. Cam pbe ll
Robe rt L. Hatch
I . Background
S c r o t a l ma s s e s a r e c o mmo n , o c c u r r i n g i n a l l a g e - g r o u p s , f r o m i n f a n t s t o e l d e r l y me n
I n f a c t , u p t o 2 0 % o f a d u l t ma l e s h a v e a v a r i c o c e l e . M a n y s c r o t a l ma s s e s a r e b e n i g
a n d r e q u i r e n o t r e a t me n t , w h e r e a s o t h e r s r e q u i r e i mme d i a t e r e c o g n i t i o n a n d
e me r g e n t t r e a t me n t .
T e s t i c u l a r t o r s i o n i s a t r u e e me r g e n c y, b e c a u s e t h e b e s t r e s u l t s o c c u r i f p a t i e n t s a r
i n t h e o p e r a t i n g r o o m w i t h i n 6 h o u r s o f o n s e t o f s y mp t o ms . S t r a n g u l a t e d i n g u i n a l
hernias and testicular ruptures also present urgent situations, whereas testicular
c a n c e r s a n d i n c a r c e r a t e d h e r n i a s r e q u i r e p r o mp t b u t l e s s u r g e n t t r e a t me n t .
H y d r o c e l e s i n c h i l d r e n a n d c e r t a i n v a r i c o c e l e s i n a d o l e s c e n t s ma y r e q u i r e s u r g e r y t
p r e s e r v e f u t u r e f e r t i l i t y. W h e n e v a l u a t i n g s c r o t a l ma s s e s , t h e p r i ma r y o b j e c t i v e i s t o
r a p i d l y i d e n t i f y a n d r e f e r p a t i e n t s w h o r e q u i r e i mme d i a t e i n t e r v e n t i o n .
P. 2 3 0
I I I . Evaluation
A. History
1. P a i n I s t h e ma s s p a i n f u l ? H o w p a i n f u l ? T e s t i c u l a r t o r s i o n u s u a l l y p r e s e n t s w i t h
severe pain, although several other conditions are also quite painful. T hese
i n c l u d e s t r a n g u l a t e d h e r n i a s , e p i d i d y mi t i s , o r c h i t i s , a n d t o r s i o n o f a t e s t i c u l a r o
e p i d i d y ma l a p p e n d a g e . Va r i c o c e l e , h y d r o c e l e , s p e r ma t o c e l e , e p i d i d y ma l c y s t s ,
a n d t e s t i c u l a r t u mo r s a r e t y p i c a l l y p a i n l e s s b u t ma y p r e s e n t w i t h a d u l l a c h e o r
h e a v i n e s s o f t h e s c r o t u m.
2. I n c i t i n g e v e nDti d t h e ma s s f i r s t a p p e a r a f t e r v i g o r o u s a c t i v i t y o r t e s t i c u l a r
t r a u ma ? T o r s i o n i s o f t e n p r e c i p i t a t e d b y o n e o f t h e s e f a c t o r s . A n o t h e r s u r g i c a l
e me r g e n c y, t e s t i c u l a r r u p t u r e , c a n r e s u l t f r o m h i g h f o r c e i n j u r i e s s u c h a s a t h l e t i
i n j u r i e s o r c a r a c c i d e n t s . A n e w s w e l l i n g f o l l o w i n g mi n o r t r a u ma , o n t h e o t h e r
h a n d , s u g g e s t s b l e e d i n g a s s o c i a t e d w i t h a t u mo r. T u mo r s c a n a l s o b e
a s s o c i a t e d w i t h me t a s t a s e s o r g y n e c o ma s t i a .
388 / 652
tmdmss
10.8 - Scrotal Mass
3. P a t i e n t a g eI n a r e v i e w o f 2 3 8 t e s t i c u l a r ma s s e s i n c h i l d r e n , t o r s i o n o f a n
a p p e n d a g e w a s t h e mo s t c o mmo n c a u s e o f a c u t e ma s s e s i n c h i l d r e n a g e d u p t o
1 3 y e a r s . I n me n o l d e r t h a n 1 3 y e a r s , e p i d i d y mi t i s a n d t e s t i c u l a r t o r s i o n b e c o me
mo r e c o mmo n1)(. T e s t i c u l a r t o r s i o n p e a k s i n me n b e t w e e n t h e a g e s o f 1 3 a n d
1 5 y e a r s1)( b u t o c c u r s f r o m i n f a n c y t o mi d d l e a g e . I n d e e d , t o r s i o n a c c o u n t s f o r
8 3 % o f a c u t e s c r o t a l ma s s e s i n c h i l d r e n a g e d 1)<.1 Eypeiadri d(y mi t i s i s t h e
mo s t c o mmo n c a u s e o f a c u t e s c r o t u m i n a d o l e s c e n t s a n d a d u l t s . I n me n y o u n g e r
t h a n 3 5 y e a r s o f aC
g ehl, amy di aa n d g o n o r r h e a a r e t h e mo s t c o mmo n
p a t h o g e n s . I n i n f a n t s a n d me n o l d e r t h a n 3 5 y e a r s o f a g e , e p i d i d y mi t i s u s u a l l y
r e s u l t s f r o m a n u n d e r l y i n g u r i n a r y t r a c t i n f e cEt si ocnher
, aincdhi a c ol
a ni d
P r ot eus mi r abi lpi rse d o mi n a t e2) (. T h e a v e r a g e a g e f o r p a t i e n t s w i t h t e s t i c u l a r
c a n c e r i s 3 2 y e a r s . H y d r o c e l e , e p i d i d y mi t i s , v a r i c o c e l e , a n d h e r n i a s a r e mo r e
c o mmo n i n a d u l t s , b u t t h e y t o o o c c u r o v e r a w i d e r a n g e o f a g e s .
4. D u r a t i o nH o w l o n g h a s t h e ma s s b e e n p r e s e n t ? T o r s i o n t y p i c a l l y p r e s e n t s w i t h
s u d d e n o n s e t o f s y mp t o ms , l e a d i n g p a t i e n t s t o s e e k c a r e s o o n a f t e r o n s e t .
Other acute conditions can also have an abrupt onset. Many benign scrotal
ma s s e s h a v e b e e n n o t e d f o r s o me t i me b y t h e p a t i e n t . A b r u p t a p p e a r a n c e o f a
v a r i c o c e l e i n a n o l d e r ma n c a n s i g n a l v e n o u s o b s t r u c t i o n ( s p e r ma t i c v e i n i f o n
the left, vena cava if on the right).
5. S y m p t o m s o f i n f e c t iIos nt h e r e a h i s t o r y o f f e v e r, p e n i l e d i s c h a r g e , mu mp s , o r
a n y o t h e r r e c e n t i n f e c t i o n ? I n f e c t i o n i s t h e mo s t c o mmo n c a u s e o f a c u t e
t e s t i c u l a r p a i n . E p i d i d y mi t i s o f t e n p r e s e n t s w i t h d i s c h a r g e a n d mi l d f e v e r. A h i g h
f e v e r o f t e n a c c o mp a n i e s o r c h i t i s . M u mp s o r c h i t i s , t h e mo s t c o mmo n c a u s e o f
o r c h i t i s w i t h o u t e p i d i d y mi
2) t, i st y(p i c a l l y o c c u r s 3 t o 4 d a y s a f t e r t h e p a r o t i t i s . I t
i s b i l a t e r a l i n 1 4 % t o 3 5 % o f c2)a .s eSsc r( o t a l w a l l c e l l u l i t i s i s c o mmo n i n
p a t i e n t s w h o a r e i mmu n o c o mp r o mi s e d , o b e s e , o r 2)d.i aMb ae nt i yc o( t h e r
i n f e c t i o n s , i n c l u d i n g t u b e r c u l o s i s a n d s y p h i l i s , c a n p r o d u c e e p i d i d y mi t i s o r
orchitis.
6. P r e v i o u s h i s t o rHya v e t h e s y mp t o ms p r e v i o u s l y a p p e a r e d ? P a t i e n t s w i t h
t o r s i o n ma y h a v e h a d s i mi l a r, mi l d e r s y mp t o ms i n t h e p a s t ( t o r s i o n t h a t
s p o n t a n e o u s l y r e s o l v e d ) . P a t i e n t s w i t h c h r o n i c e p i d i d y mi t i s g e n e r a l l y d e s c r i b e
a n i n i t i a l s e v e r e b o u t t h a t h a s b e e n f o l l o w e d b y mi l d e r r e c u r r e n c e s .
7. O t h e r a s s o c i a t e d s y m p t oAm
r es t h e r e a n y o t h e r s y mp t o ms ? N a u s e a o f t e n
a c c o mp a n i e s t o r s i o n a n d o r c h i t i s .
1. P a l p a t e t h e s c r o t u m a n d c o n t e n t s
a. D e t e r m i n e t h e o r i e n t a t i o n o f t h e t eAs t oi cr sl ee d t e s t i c l e i s u s u a l l y
r e t r a c t e d u p w a r d a n d r o t a t e d t o a n a b n o r ma l p o s i t i o n . T h i s ma y b e
i n d i c a t e d b y a n e p i d i d y mi s t h a t a p p e a r s t o l i e i n a n a b n o r ma l l o c a t i o n
( n o r ma l l y, t h e h e a d o f t h e e p i d i d y mi s l i e s a t t h e s u p e r i o r p o l e o f t h e t e s t i c l e
a n d i t s b o d y e xt e n d s p o s t e r o l a t e r a l a l o n g t h e t e s t i c l e ) . C o mp a r i s o n w i t h t h e
o t h e r t e s t i c l e ma y h e l p w i t h t h i s d e t e r mi n a t i o n . N o r ma l p o s i t i o n d o e s n o t
r u l e o u t t o r s i o n , h o w e v e r, b e c a u s e t h e t e s t i c l e ma y h a v e r o t a t e d a f u l l 3 6 0 .
389 / 652
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10.8 - Scrotal Mass
I n e p i d i d y mi t i s a n d o r c h i t i s , t e s t i c u l a r l i e c a n b e d i f f i c u l t t o d e t e r mi n e d u e t o
s w e l l i n g3)( .
P. 2 3 1
b. A s s e s s f o r s w e l l i n g a n d t e n d e rTnoer s si o n , o r c h i t i s , a n d e p i d i d y mi t i s a l l
develop swelling and tenderness soon after onset.
c. D e t e r m i n e l o c a t i o n o f mCa os ns s i d e r w h e t h e r t h e ma s s i s i n t r a t e s t i c u l a r
o r e xt r a t e s t i c u l a r. T h e mo s t c o mmo n e xt r a t e s t i c u l a r s c r o t a l ma s s e s i n c l u d e
i n g u i n a l h e r n i a s , e p i d i d y ma l c y s t s , a n d v a r i c o c e l e s . T h e mo s t c o n c e r n i n g
i n t r a t e s t i c u l a r l e s i o n s a r e ma l i g n a n t g e r m c e l l t u mo r s , w h i c h ma k e u p 9 0 %
t o 9 5 % o f i n t r a t e s t i c u l a r p r i ma r y t u mo r s . A p p e n d i c e s o f t h e e p i d i d y mi s a n d
t e s t i c l e c a n e xt e n d f r o m t h e s u p e r i o r p o l e o f e i t h e r s t r u c t u r e . S p e r ma t o c e l e
i s mo s t c o mmo n l y f o u n d s u p e r i o r a n d p o s t e r i o r t o t h e t e s t i c l e . Va r i c o c e l e
o c c u r s i n a s i mi l a r l o c a t i o n , u s u a l l y o n t h e l e f t s i d e . I n e p i d i d y mi t i s , t h e
s w o l l e n e p i d i d y mi s i s o f t e n d i f f i c u l t t o d i s t i n g u i s h f r o m t h e t e s t i c l e .
d. A s s e s s t h e c o n s i s t e n c y o f t h e A
m av as rsi c o c e l e t y p i c a l l y h a s t h e
c o n s i s t e n c y o f a b a g o f w o r ms . H y d r o c e l e a n d s p e r ma t o c e l e u s u a l l y h a v e a
c y s t i c c o n s i s t e n c y. H y d r o c e l e c a n b e c o me mo r e t e n s e a s t h e d a y
progresses (because of the dependent position).
2. A s s e s s t h e c r e m a s t e r i c r eW
f l he exn t h e i n n e r t h i g h i s l i g h t l y s t r o k e d , t h e
t e s t i c l e o n t h a t s i d e s h o u l d r i s e n o t i c e a b l y. A b s e n c e o f t h i s r e f l e x s u g g e s t s
testicular torsion.
3. E l e v a t e t h e t e s t i c l e a b o v e t h e s y m p h y s i sT hpi us bui ssu a l l y r e l i e v e s t h e
p a i n o f e p i d i d y mi t i s b u t n o t o f t o r s i o n ( P r e h2)n .' s s i g n ) (
4. T r a n s i l l u m i n a t e t h e m H
a sy sd r o c e l e s a n d s p e r ma t o c e l e s t r a n s i l l u mi n a t e .
5. E x a m i n e t h e p a t i e n t i n b o t h t h e s u p i n e a n d s t a n d i n g Hpeorsniitai so nasn d
v a r i c o c e l e s u s u a l l y b e c o me mo r e p r o mi n e n t o n s t a n d i n g , e s p e c i a l l y w i t h
Va l s a l v a .
C. Testing
Reliance on clinical findings alone produces false-positive rates for testicular torsi
o f a p p r o xi ma t e l y 5 2)
0%
. (D o p p l e r u l t r a s o u n d h a s r e p l a c e d r a d i o i s o t o p e s c a n s a s t h e
i ma g i n g mo d a l i t y o f c h o3)i c. eI t( a v o i d s f a l s e - p o s i t i v e r e s u l t s f r o m s k i n h y p e r e mi a
a n d i s f a r s u p e r i o r f o r d e t e c t i n g o t h e r c a u s e s o f t e s t i c u l a r p a 3)
i n . oIrn ma
t h es s e s (
p r e s e n c e o f a c u t e o r s t u t t e r i n g s y mp t o ms t h a t s u g g e s t t o r s i o n , a D o p p l e r u l t r a s o u n d
s h o u l d b e o b t a i n e d a s s o o n a s p o s s i b l e ( c o mp a r i n g t h e a f f e c t e d t e s t i c l e t o t h e
u n a f f e c t e d o n e ) . P a r e n t e r a l n a r c o t i c s ma y b e a d mi n i s t e r e d i f i t i s n e c e s s a r y t o
a l l e v i a t e p a i n a n d a l l o w a b e t t e r e xa mi n a t i o n . S p e c i f i c i t y o f 9 7 % i s r e p o r t e d f o r
d e t e c t i n g t o r s i o1)n . ( F a l s e - n e g a t i v e r e s u l t s ma y o c c u r e v e n w i t h u l t r a s o u n d ,
p r o d u c i n g l o w e r s e n s i t i v i t y (1)
8 6. %
I n) (t h i s s e r i e s , mo s t f a l s e - n e g a t i v e r e s u l t s
occurred either in prolonged torsion in which the testicles were no longer
s a l v a g e a b l e , o r i n c a s e s o f i n t e r mi t t e n t t o r s i o n . A n u l t r a s o u n d c a n b e h e l p f u l i n
d i f f e r e n t i a t i n g ma n y o t h e r ma s s e s , b u t s h o w e d a d i s a p p o i n t i n g a b i l i t y t o d i f f e r e n t i a t
ma l i g n a n t f r o m b e n i g n ma s s e s i n c h4)
i l d. rAesnp i( r a t i o n o f a s p e r ma t o c e l e u s u a l l y
r e v e a l s d e a d s p e r1)
m. (P y u r i a i s a l mo s t a l w a y s p r e s e n t i n e p i d i d y mi t i s , b u t i t h a s
390 / 652
tmdmss
10.8 - Scrotal Mass
also been found in up to 27% of patients with torsion (>5 white blood cells/highp o w e r f i e l d5)) (. S i mi l a r l y, l e u k o c y t o s i s s u g g e s t s a n i n f e c t i o u s c a u s e b u t i t h a s a l s o
b e e n f o u n d i n 3 3 % o f p a t i e n t s w i t h t5)
o r. s i o n (
I V. Diagnosis
D u e t o c o n s i d e r a b l e o v e r l a p i n t h e p r e s e n t a t i o n , l a b o r a t o r y f i n d i n g s , a n d i ma g i n g
s t u d i e s o f t h e s e c o n d i t i o n s , e s t a b l i s h i n g a d i a g n o s i s i s c h a l l e n g i n g i n s o me c a s e s .
t h e d i a g n o s i s o f t e s t i c u l a r t o r s i o n c a n n o t b e r a p i d l y a n d c o n f i d e n t l y e xc l u d e d ,
e me r g e n t r e f e r r a l o r u l t r a s o u n d i s s t r o n g l y r e c o mme n d e d . L e s s u r g e n t c o n s u l t a t i o n
r e c o mme n d e d i f t h e d i a g n o s i s i s u n c l e a r b u t t o r s i o n i s n o t s u s p e c t e d .
References
1 . L e w i s A G , B u k o w s k i T P, J a r v i s P D . E v a l u a t i o n o f a c u t e s c r o t u m i n t h e
e me r g e n c y d e p a r t meJn tP. edi at r S ur 1g 9 9 5 ; 3 0 : 2 7 7 2 8 2 .
2 . D o g r a V, B h a t t S . A c u t e p a i n f u l s cRr oadi
t u m.
ol C l i n N or t h A2 m
004;42:349
363.
3 . B l a i v a s M , B r a n n a m L . T e s t i c u l a r u l t r aEsmer
o u ngd .M ed C l i n N or t h A m
2004;22:723748.
4 . A r a g o n a F, P e s c a t o r i E , T a l e n t i E . P a i n l e s s s c r o t a l ma s s e s i n t h e p e d i a t r i c
population: prevalence and age distribution of different pathological conditions
a 1 0 - y e a r r e t r o s p e c t i v e mu l t i c e n t e rJ sUt ur ol
d y.
1996;155:14241426.
5 . K a t t a n S . S p e r ma t i c c o r d t o r s i o n i n Sacdand
u l t s .J U r ol N ephr1ol
994;28:277
279.
391 / 652
tmdmss
10.9 - Scrotal Pain
> T a b l e o f C o n t e n t s > 1 0 - R e n a l a n d U r o l o g i c P r o b l e ms > 1 0 . 9 - S c r o t a l P a i n
10.9
Scrotal Pain
G e o r g e R . Wi l s o n
I . Background
A.
S c r o t a l p a i n c a n r e s u l t f r o m p a t h o l o g y w i t h i n t h e s c r o t u m, t r a u ma t o t h e s c r o t u m,
p a t h o l o g y o f t h e s c r o t a l s a c , e xt r a s c r o t a l p a t h o l o g y, a n d r e f e r r e d p a i n f r o m i n t r a a b d o mi n a l p a t h o l o g y. I t c a n b e a c u t e , c h r o n i c , o r i n t e r mi t t e n t .
B.
S c r o t a l p a i n i s a c o mmo n p r o b l e m i n a d u l t s , mo s t o f t e n d u e t o e p i d i d y mi t i s o r
v a r i c o c e l e . W i t h t h e e xc e p t i o n o f t r a u ma , s c r o t a l p a i n i n a n a d u l t r a r e l y r e q u i r e s
e me r g e n t s u r g i c a l i n t e r v e n t i o n . O n t h e o t h e r h a n d , s c r o t a l p a i n i n c h i l d r e n a n d
a d o l e s c e n t s , a l t h o u g h l e s s c o mmo n t h a n i n a d u l t s , i s o f t e n d u e t o c a u s e s t h a t
r e q u i r e u r g e n t i n t e r v e n t i o n . T h e mo s t c o mmo n c a u s e o f s c r o t a l p a i n i n a c h i l d o r
a d o l e s c e n t t h a t r e q u i r e s t i me l y d i a g n o s i s a n d s u r g i c a l i n t e r v e n t i o n i s t e s t i c u l a r
t o r s i o n 1)( . T h e h i g h e s t p r o b a b i l i t y f o r s a v i n g a t o r s e d t e s t i c l e o c c u r s w i t h i n t h e f i r s t
6 h o u r s . A f t e r 6 h o u r s , t h e p r o b a b i l i t y o f s u c c e s s d e c l i n e s , r e a c h i n g ze r o a t
a p p r o xi ma t e l y 4 8 h o u r s . C h o i c e o f d i a g n o s t i c mo d a l i t y i n t h i s e a r l y p e r i o d c a n p l a y
a n i mp o r t a n t r o l e i n d e t e r mi n i n g w h a t i n t e r v e n t i o n i s a p p r o p r i a t e .
A. Etiology
1. I n t r a s c r o t a l p a t h o l o g y t e s t i c u l a r t o r s i o n , t o r s i o n o f t e s t i c u l a r a p p e n d a g e ,
e p i d i d y mi t i s , o r c h i t i s , v a r i c o c e l e , s p e r ma t o c e l e , p r i ma r y o r me t a s t a t i c t e s t i c u l a r
t u mo r
2. P a t h o l o g y o f t h e s c r o t a l s a c u s u a l l y c o n f i n e d t o b l u n t t r a u ma o r i n f e c t i o n
3. E xt r a s c r o t a l p a t h o l o g y d i r e c t / i n d i r e c t h e r n i a , i n c a r c e r a t e d h e r n i a , h y d r o c e l e ,
u r i n a r y t r a c t i n f e c t i o n ( U T I ) , p r o s t a t i t i s , s e xu a l l y t r a n s mi t t e d d i s e a s e ( S T D )
4. S c r o t a l t r a u ma f a l l s , a n i ma l b i t e s , s e xu a l a b u s e
5. P a i n r e f e r r e d f r o m i n t r a - a b d o mi n a l p a t h o l o g y o r s y s t e mi c d i s e a s e r e n a l c o l i c ,
H e n o c h - S c h n l e i n p u r p u r a , mo n o n u c l e o s i s , B u e r g e r ' s d i s e a s e , c o xs a c k i e B
v i r u s , p o l y a r t e r i t i s n o d2)
o s. a (
392 / 652
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10.9 - Scrotal Pain
B. Epidemiology
1. I n t r a s c r o t a l p a t h o l o g y
a. T e s t i c u l a r t o r s i o n i s h i g h e s t i n i n c i d e n c e a t 1 2 t o 1 8 y e1,3)
a r s. oI tf ias g e (
t h e mo s t c o mmo n c a u s e o f s c r o t a l p a i n i n t h e f i r s t y e a r o f l i f e ( o f t e n
mi s d i a g n o s e d a s c o l i c o r i n t r a - a b d o mi n a l d i1)
s o ar dnedr )i ,s (u n c o mmo n i n
adults.
b. T o r s i o n o f t e s t i c u l a r a p p e n d a g e i s c o mmo n a t 1 0 t o 1 5 y e a r s o f a g e . I t i s
r a r e i n n e o n a t e s a n d a d 3)
u l .t s (
c. E p i d i d y mi t i s i s t h e mo s t c o mmo n c a u s e o f a c u t e s c r o t a l p a1)
i n. i Int a d u l t s (
i s r a r e i n p r e p u b e r t a l c h i l 3)
d r.e nI f (i t o c c u r s i n p r e p u b e r t y, u r o g e n i t a l
a n o ma l y o r d y s f u n c t i o n s h o u l d b e s u s p e c t e d .
d. O r c h i t i s i s u s u a l l y d u e t o e xt e n s i o n o f e p i d i d y mi t i s . M u mp s o r c h i t i s , w h i c h
o c c u r s o n l y p o s t p u b e r t y i n a p p r o xi ma t e l y 2 0 % o f mu mp s c a s e s , i s u n i l a t e r a l
i n 7 0 % o f c a s e s . I t i s l e s s c o mmo n s i n c e t h e mu mp s v a c c i n e .
e. Va r i c o c e l e o c c u r s p r i ma r i l y i n a d u l t s a n d a d o l e s c e n t s . I t i s r a r e b u t c a n
o c c u r i n p r e p u b e r t a l c h i l d r e n ; i n s u c h c a s e s , a n i n t r a - a b d o mi n a l l e s i o n
obstructing venous drainage should be considered.
f. S p e r ma t o c e l e i s c o mmo n p o s t v a s e c t o my. N o t s e e n i n p r e p u b e r t y, i t i s v e r y
rare in adolescents.
g. P r i ma r y o r me t a s t a t i c t e s t i c u l a r t u mo r i s h i g h e s t i n i n c i d e n c e a t 1 8 t o 3 0
y e a r s o f a g e . M e t a s t a t i c t u mo r i s r a r e b u t d o e s o c c u r.
2. P a t h o l o g y o f t h e s c r o t a l s a c
a. B a c t e r i a l i n f e c t i o n s e c o n d a r y t o f u n g a l d i s e a s e o r c o n t a c t d e r ma t i t i s
b. I n c l u s i o n / s e b a c e o u s c y s t s
P. 2 3 3
3. E x t r a s c r o t a l p a t h o l o g y
a. D i r e c t / i n d i r e c t h e r n i a o r i n c a r c e r a t e d h e r n i a i s e q u a l l y p r e v a l e n t i n a d u l t s
and children.
b. H y d r o c e l e o c c u r s p r i ma r i l y i n n e o n a t e s . A l t h o u g h i t o c c u r s i n a d u l t s , i t i s n o
c o mmo n . T h e c o n d i t i o n o f t e n r e s o l v e s s p o n t a n e o u s l y.
c. U T I mu s t b e c o n s i d e r e d i n s ma l l c h i l d r e n , e s p e c i a l l y i n f a n t s . I t i s o f t e n
a s s o c i a t e d w i t h c o n g e n i t a l a n o ma l y a n d / o r u r i n e r e f l u x.
d. P r o s t a t i t i s i s p o s t p u b e r t a l . N o t s e e n i n p r e p u b e r t y, i t i s r a r e i n a d o l e s c e n t s .
e. S T D s a r e g e n e r a l l y p o s t p u b e r t a l . T h e y a r e r a r e l y p r e p u b e r t a l ; s e xu a l a b u s e
should be suspected.
4. S c r o t a l t r a u mSai g n i f i c a n t d a ma g e t o a t e s t i c l e f r o m t r a u ma , i n a n y a g e - g r o u p ,
i s r a r e . S c r o t a l p a i n d u e t o t r a u ma i s g e n e r a l l y s e l f - e v i d e n t o r s e l f - r e p o r t e d .
T r a u ma t i c d a ma g e i n p r e p u b e r t y i s r a r e d u e t o t h e s ma l l s i ze o f t h e t e s t i c l e .
W h e n i t d o e s o c c u r, s e l f - e xp e r i me n t a t i o n o r s e xu a l a b u s e i s o f t e n t h e c a u s e .
393 / 652
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10.9 - Scrotal Pain
S c r o t a l p a i n d u e t o t r a u ma u s u a l l y r e s o l v e s i n2)<. 1Phaoi nu r p(e r s i s t i n g

l o n g e r t h a n 1 h o u r s u g g e s t s t e s t i c u l a r r u p t u r e . T e s t i c u l a r r u p t u r e f r o m mi n o r
t r a u ma i s c o n s i s t e n t w i t h o c c u l t t e s t i c u l a r n e o p l a s m.
I I I . Evaluation
A. History
T h e p r i ma r y p i e c e o f h i s t o r i c a l i n f o r ma t i o n t o b e o b t a i n e d i s t h e t i me o f t h e o n s e t o
p a i n 4)
( . T h i s i s c r i t i c a l i n t e s t i c u l a r t o r s i o n , b e c a u s e i t d e t e r mi n e s h o w r a p i d l y
d i a g n o s t i c t e s t s n e e d t o b e a c c o mp l i s h e d a n d ma y d i c t a t e t h e o r d e r o f t e s t s t h a t
n e e d t o b e p e r f o r me d . O t h e r h i s t o r i c a l i n f o r ma t i o n i n c l u d e s w h e t h e r t h e p a i n i s
s e v e r e o r mi l d ; a c u t e o r c h r o n i c ; o r c o n s t a n t o r i n t e r mi t t e n t . H i s t o r y o f t r a u ma n e e d
t o b e e l i c i t e d . F e v e r, n a u s e a a n d / o r v o mi t i n g , d y s u r i a , a n d o t h e r me d i c a l c o n d i t i o n s
n e e d t o b e d e t e r mi n e d . W h e n a p p r o p r i a t e , t h e o p t i o n s o f s e xu a l e xp e r i me n t a t i o n
a n d / o r u n u s u a l s e xu a l p r a c t i c e s s h o u l d b e e xp l o r e d .
1. T e s t i c u l a r t o r s i o n u s u a l l y p r e s e n t s w i t h a n a b r u p t o n s e t o f p a i n . N a u s e a a n d
v o mi t i n g a r e c o mmo n . H i s t o r y o f p r e v i o u s i p s i l a t e r a l p a i n i s a s s o c i a t e d w i t h a
4 4 % i n c i d e n c e o f t e s t i c u l a r t o1)
r s.i oEna r( l y f e v e r i s n o t p a r t o f t h e h i s t o r y.
2. T o r s i o n o f t h e t e s t i c u l a r a p p e n d a g e h a s l e s s i n t e n s e p a i n t h a n t e s t i c u l a r t o r s i o n
P a i n ma y p e r s i s t f o r d a y s , b e g i n n i n g i n t h e l o w e r a b d o me n b e f o r e mo v i n g i n t o
t h e s c r o t u m. M a l a i s e i s c o mmo n . F e v e r i s n o t u s u a l l y p a r t o f t h e h i s t o r y.
3. E p i d i d y mi t i s c a n b e a b r u p t o r i n s i d i o u s i n o n s e t . F e v e r i s c o mmo n . Vo i d i n g
s y mp t o ms ma y o c c u r.
1. I n f a n t s a n d s ma l l c h i l d r e n s h o u l d b e o b s e r v e d p r i o r t o e xa mi n i n g t h e s c r o t u m.
T e s t i c u l a r t o r s i o n g e n e r a l l y c a u s e s r e s t l e s s n e s s , i r r i t a b i l i t y, a n d c r y i n g . A l l
n e o n a t e s a n d s ma l l c h i l d r e n w i t h a h i s t o r y o f a b d o mi n a l p a i n r e q u i r e a n
e xa mi n a t i o n o f t h e s c r o t u m.
2. S c r o t u m i s e xa mi n e d f o r e v i d e n c e o f t r a u ma , c h a n g e s i s c o l o r, d i s c o l o r a t i o n ,
u n i l a t e r a l o r b i l a t e r a l s w e l l i n g , i n f e c t i o n , a n d ma s s e s . I f p a i n i s p r e s e n t l o n g e r
t h a n 2 4 h o u r s w i t h o u t s c r o t a l c h a n g e s , t o r s i o n i s2) u. nDl i ks ec ol yl o(r a t i o n i s
u s u a l l y d u e t o t r a u ma b u t c a n b e s e e n w i t h t o r s i o n a n d e p i d i d y mi t i s . T h e
c r e ma s t e r i c r e f l e x s h o u l d b e c h e c k e d . I f p r e s e n t o n t h e p a i n f u l s i d e , t e s t i c u l a r
t o r s i o n i s r u l e d o u t . F o r t h e a b s e n c e o f a c r e ma s t e r i c r e f l e x t o b e a r e l i a b l e
i n d i c a t o r, i t s p r e s e n c e mu s t b e d e mo n s t r a t e d o n t h e n o n p a i n f u l s i d e . U n i l a t e r a
swelling without skin changes indicate hernia or hydrocele. Elevating the
scrotum increases pain with testicular torsion, and decreases pain with
e p i d i d y mi t i s ( P r e h n ' s s3)
i g.n ) (
3. T h e t e s t e s a r e e xa mi n e d f o r p r e s e n c e o r a b s e n c e o f t o r s i o n . H i g h t r a n s v e r s e l i e
suggests torsion. Testicular torsion causes early swelling. Appendiceal torsion
a n d e p i d i d y mi t i s a r e p a l p a b l e a s a ma s s o r s w e l l i n g a l o n g t h e ma r g i n o f t h e
t e s t i s . T r a n s i l l u mi n a t i o n o f t h e s c r o t u m ma y r e v e a l a b l u e d o t s i g n i n
394 / 652
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10.9 - Scrotal Pain
a p p e n d i c e a l t o r s i o2,3)
n (.
C. Testing
Ve r y f e w l a b o r a t o r y t e s t s a r e i n d i c a t e d i n t h e e v a l u a t i o n o f s c r o t a l p a i n .
1. U r i n a l y s i s i s u s u a l l y n e g a t i v e . I f i t i s p o s i t i v e , t h e d i a g n o s i s i s mo r e l i k e l y t o b e
e p i d i d y mi t i s , r a t h e r t h a n t e s t i c u l a r o r a p p e n d i c e a l t o r s i o n .
P. 2 3 4
2. W h i t e b l o o d c e l l c o u n t ma y i n d i c a t e n o n s p e c i f i c l e u k o c y t o s i s . I t i s mo r e
c o mmo n l y e l e v a t e d i n e p i d i d y mi t i s a n d ma y b e e l e v a t e d w i t h t e s t i c u l a r o r
appendiceal torsion after 24 hours.
3. S e r o l o g y a n d e r y t h r o c y t e s e d i me n t a t i o n r a t e a r e n o t h e l p f u l , a n d t h e y ma y
unnecessarily delay diagnosis.
4. I m a g i n g s t u d i e s
a. C o l o r D o p p l e r i s t h e f i r s t - l i n e mo d a l i t y f o r d i a g n o s i s . T h i s p r o v i d e s a
definitive diagnosis of testicular torsion in 91.7% of cases. A negative test
i s 9 6 . 2 % r e l i a b l e t e s t i c u l a r t o r s i o n i s n o t 5)p .r e s e n t (
b. G r a y - s c a l e D o p p l e r i s n o t r e l i a b l e i n d i a g n o s i n g t e s t i c u l a r t o r s i o n . I t ma y
g i v e f a l s e - n e g a t i v e r e s u l t s e a r l y. O n c e c h a n g e s b e c o me e v i d e n t , t h e
t e s t i c l e ma y n o l o n g e r b e s a l v a g3)
e a. b l e (
c. N u c l e a r s c a n n i n g h a s b e e n s h o w n t o b e a n e f f e c t i v e me t h o d f o r e v a l u a t i n g
a c u t e s c r o t u m. H o w e v e r, t h e p r o c e s s i s o f t e n t o o s l o w t o b e u n i v e r s a l l y
u s e d i n d e t e r mi n i n g t h e n e e d f o r s u r g i c a l i n t e r v e n t i o n i n a n a r r o w
t h e r a p e u t i c w i n d o w. T h e p r i ma r y u s e s a r e e v a l u a t i o n i n t r a u ma , w i t h
a s y mp t o ma t i c ma s s e s , a n d w h e n e l e c t i v e s u r g i c a l e xp l o r a t i o n i s
contraindicated and Doppler studies are equivocal.
d. M a g n e t i c r e s o n a n c e i ma g i n g p r o v i d e s g o o d a n a t o mi c a l d a t a b u t i s n o t
useful to evaluate for blood flow in the acute setting.
D. Genetics
T h i s p l a y s a v e r y s ma l l r o l e i n s c r o t a l p a i n .
I V. Diagnosis
Most critical is testicular torsion due to the finite survival of the testis. Other
e t i o l o g i e s ( a p p e n d i c e a l t o r s i o n , e p i d i d y mi t i s , t r a u ma , h e r n i a / h y d r o c e l e , t u mo r ) h a v e
l a r g e r w i n d o w o f o p p o r t u n i t y. A l l t e s t s s h o u l d b e d i r e c t e d t o w a r d i n i t i a l l y e xc l u d i n g
testicular torsionand as rapidly as possible.
T h e t w o p r i ma r y p i e c e s o f c l i n i c a l h i s t 1)
o r yt haer ea g( e o f t h e p a t i e n t 2)
a n tdh e(
t i me l a p s e d s i n c e t h e o n s e t o f p a i n . S e v e r e u n i l a t e r a l p a i n o f < 6 h o u r s , w o r s e n e d b
395 / 652
tmdmss
10.9 - Scrotal Pain
t h e e l e v a t i o n o f t h e s c r o t u m, i n a n a d o l e s c e n t , i s t e s t i c u l a r t o r s i o n u n t i l p r o v e n
o t h e r w i s e . E v e r y n e o n a t e w i t h a b d o mi n a l s y mp t o ms mu s t h a v e a s c r o t a l e xa mi n a t i o n
a s a p a r t o f t h e w o r k u p . C o l o r D o p p l e r u l t r a s o n o g r a p h y i s t h e d i a g n o s t i c mo d a l i t y o
c h o i c e f o r t h e e v a l u a t i o n o f a c u t e s c r o t a l p a i n . S u r g i c a l e xp l o r a t i o n mu s t a l w a y s b e
c o n s i d e r e d w h e n e v e r t h e h i s t o r y a n d / o r p h y s i c a l e xa mi n a t i o n i s c o n s i s t e n t w i t h
t e s t i c u l a r t o r s i o n , o r w h e n t h e d a t a dcoompl
e s n oett el yr u l e i t o u t . T h e o p p o r t u n i t y
to salvage the testis is in the first 6 hours.
References
1 . E s p y P G , K o o H P. T o r s i o n o f t h e t e s t i c l e . I n : G r a h a m S D , G l e n n J F, K e a n e
T E , e d sG
. l enn' s ur ol ogi c s ur ,ger
6 t yh e d . P h i l a d e l p h i a , PA : L i p p i n c o t t W i l l i a ms
and Wilkins, 2004:513517.
2 . K a s s E J , L u n d a k B . T h e a c u t e s c rPoedi
t u m.
at r C l i n N or t h A m
1997;44(5):12511266.
3. S andock D S , Herbener T E , Resnick M I. Disorders of the scrotum and its
c o n t e n t s . I n : R e s n i c k M I , O l d e r R AD,i agnos
e d s . i s of geni t our i nar y di ,s eas e
2 n d e d . N e w Yo r k : T h i e me - S t r a t t o n I n c . , 1 9 9 7 : 4 6 5 4 8 3 .
4 . G a l e j s L E . D i a g n o s i s a n d t r e a t me n t o f t h e a c u t eAsmc rFoam
t u m.P hy s i c i an
1999;59(4):817824.
5 . K a r ma zy n B , S t e i n b e r g R , K o r n r e i c h L , e t a l . C l i n i c a l a n d s o n o g r a p h i c c r i t e r i a
o f a c u t e s c r o t u m i n c h i l d r e n : a r e t r o s p e c t i v e s t u d y oP
f edi
1 7 2atbro R
y sadi
. ol
2005;35(3):302310.
396 / 652
tmdmss
10.10 - Urethral Discharge
> T a b l e o f C o n t e n t s > 1 0 - R e n a l a n d U r o l o g i c P r o b l e ms > 1 0 . 1 0 - U r e t h r a l
Discharge
10.10
Urethral Discharge
Ge orge P N Sam raj
I . Background
U r e t h r a l d i s c h a r g e ( U D ) i s a c o mmo n s y mp t o m, w i t h e t i o l o g y v a r y i n g f r o m s e xu a l l y
t r a n s mi t t e d i n f e c t i o n t o c a n c e r. U D ma y b e p r o f u s e o r s c a n t y ; c l e a r, y e l l o w i s h o r
w h i t e , b r o w n o r g r e e n ; mu c o p u r u l e n t o r s e r o u s ; o r b l o o d y, w a t e r y, o r f r a n k p u s . U D
ma y b e a n a c u t e o r c h r o n i c c o n d i t i o n , a n d p a t i e n t s ma y o r ma y n o t h a v e s y mp t o ms .
U D i s t h e p r e s e n t i n g s i g n o f ma n y d i s o r d e r s a n d c a n b e c l a s s i f i e d a s n o t e d b e l o w.
A. Sexually transmitted diseases (STDs)
1. G o n o c o c c a l ( G C ) i n f e c tGi oCn i n f e c t i o n i s mo r e c o mmo n i n me n t h a n w o me n .

T he Centers for Disease Control and Prevention reports that in 2004, 330,132
c a s e s o f ( 11 3 . 5 c a s e s p e r 1 0 0 , 0 0 0 p o p u l a t i o n i n 2 0 0 4 ) g o n o r r h e a w e r e r e p o r t e d
i n t h e U n i t e d S t a t e s . I t i s 2 0 t i me s mo r e c o mmo n i n y o u n g p a t i e n t s f r o m l a r g e
c i t i e s a n d i n A f r i c a n - A me r i c a n i n d i v1)
i d. u a l s (
2. N o n g o n o c o c c a l ( N G C ) i n f e cTt ihoi sn i s t h e mo s t c o mmo n S T D i n t h e U n i t e d
S t a t e s , w i t h 2 . 8 mi l l i o n n e w c a s e s o c c u r r i n g 1)
a n. nAusa lma
l y n( y a s 8 5 % o f
w o me n w i t h c h l a my d i a l i n f e c t i o n s a n d 4 0 % o f i n f e c t e d me n a r e a s y mp t o ma t i c .
a. C hl amy di a t r ac homat
, (i2s0 % 4 0 % o f N G C )
b. U r eapl as ma ur eal y t i ,c um
(10%20% of N G C )
c. M y c opl as ma geni t al i um
d. O t h e r o r g a n i s ms l i n k e d t o U D :
i. B a c t e r i aG ar dner el l a v agi nal i s , E s c her i c hi
, at u cbol
e ric u l o s iCs ,or y ne
b a c t e r i u m g e n i t a l i u m, B a c t e r o i d e s , my c o p l a s ma s
i i. V i r u s e sH e r p e s s i mp l e x v i r u s , a d e n o v i r u s e s , c y t o me g a l o v i r u s , h u ma n
p a p i l l o ma v i r u s , a n d o t h e r s
i i i. P r o t o z o aTr
l i c homonas v agi nal( ai bs o u t 5 mi l l i o n c a s e s o c c u r a n n u a l l y
in the United States)
i v. F u n g a lC andi das p e c i e s
397 / 652
tmdmss
B. Nonsexually transmitted diseases

1. I n f e c t i o n sC y s t i t i s , p r o s t a t i t i s
2. A n a t o m i c a n d c o n g e n i t a l a b n o r m aUl irtei tehsr a l s t r i c t u r e , P h i mo s i s
3. I a t r o g e n i cC a t h e t e r i za t i o n , i n s t r u me n t a t i o n , a n d o t h e r p r o c e d u r e s
4. C h e mi c a l i r r i t a t i o n f r o m d o u c h e s , l u b r i c a n t s , a n d o t h e r c h e mi c a l s
5. Tu m o r sM a l i g n a n t l e s i o n s a n d n e w g r o w t h s
6. F o r e i g n b o d i eCso mmo n i n c h i l d r e n a n d t e e n a g e r s
7. S u b s t a n c e a b u sCeh r o n i c u s e o f a mp h e t a mi n e s o r o t h e r s t i mu l a n t s p r o d u c e s a
s e r o u s d i s c h a r g e . C a f f e i n e a n d a l c o h o l a r e a l s o i mp l i c a t e d i n U D .
8. M i s c e l l a n e o u s f a c t o r s l i n k e d tSoeU
xuDa l p r a c t i c e s , ma s t u r b a t i o n , o r a l s e x
9. U n k n o w nN o o r g a n i s ms ma y b e f o u n d i n u p t o o n e - t h i r d o f p a t i e n t s .
I I I . Evaluation
A. History
A c o mp r e h e n s i v e me d i c a l h i s t o r y i s e s s e n t i a l f o r t h e e v a l u a t i o n o f U D . I t s h o u l d
i n c l u d e ( a ) d y s u r i a , ( b ) U D , ( c ) i t c h i n g a t t h e u r e t h r a , ( d ) h e ma t u r i a , ( e ) r e c t a l
s y mp t o ms , ( f ) c o n t a c t w i t h i n f e c t i o u s a g e n t s , ( g ) t e s t i c u l a r p a i n , ( h ) l o w b a c k p a i n
a n d c o n s t i t u t i o n a l s y mp t o ms . T h e c h a r a c t e r i s t i c o f U D i s c h a r a c t e r i ze d i n r e l a t i o n t
c o l o r ( p u r u l e n t , mu c o i d , o r b l o o d y ) , q u a n t i t y, o d o r, c o n s i s t e n c y, f r e q u e n c y, p a s t
h i s t o r y, o r a n y p r e v i o u s t r e a t me n t a n d r e l a t i o n s h i p t o u r i n a t i o n . P r o f u s e , t h i c k y e l l o
t o g r a y i s h U D 3 t o 7 d a y s a f t e r s e xu a l e xp o s u r e i s c h a r a c t e r i s t i c o f G C i n f e c t i o n .
C l e a r t o w h i t e s c a n t y, o r mu c o p u r u l e n t U D ( 2 3 % 5 5 % ) t h a t d e v e l o p s g r a d u a l l y a t
l e a s t a w e e k a f t e r e xp o s u r e a n d w a xe s a n d w a n e s i n i n t e n s i t y i s s u g g e s t i v e o f
c h l a my d i a l i n f e c t i o n . M u c o i d , s c a n t w a t e r y d i s c h a r g e t h a t d e v e l o p s
P. 2 3 6
o v e r a p e r i o d o f 2 t o 3 w e e k s i s c o mmo n w i t h N G C U D . A b l o o d y d i s c h a r g e i s
s u g g e s t i v e o f u r e t h r a l c a r c i n o ma . S e xu a l h i s t o r y s h o u l d i n c l u d e s e xu a l o r i e n t a t i o n ,
p a s t s e xu a l h i s t o r y, s e xu a l b e h a v i o r s , c o n d o m u s a g e , a n d n u mb e r o f s e xu a l p a r t n e r s
r e c e n t s e xu a l c o n t a c t s , a n d t h e o r i f i c e s u s e d f o r s e xu a l c o n t a c t s . C o n s i s t e n t u s a g e
o f c o n d o ms p r e v e n t s s e xu a l l y t r a n s mi t t e d u r e t h r i t i s . O r a l s e x i n c r e a s e s U D d u e t o
infections from oral flora.
1. A f o c u s e d p h y s i c a l e xa mi n a t i o n i n c l u d e s v i t a l s i g n s a n d u r o l o g i c a n d r e c t a l
e xa mi n a t i o n . I n me n , t h i s s h o u l d i n c l u d e e xa mi n a t i o n o f t h e p e n i s , p e r i me a t a l
r e g i o n f o r t h e e v i d e n c e o f e r y t h e ma , u r e t h r a l me a t u s , s c r o t u m, t e s t i c l e s ,
e p i d i d y mi s , p r o s t a t e , p e r i a n a l , a n d t h e i n g u i n a l r e g i o n f o r l y mp h n o d e s . S t a i n s
p r e s e n t o n t h e p a t i e n t ' s u n d e r w e a r ma y i n d i c a t e t h e c h a r a c t e r i s t i c s o f t h e
d i s c h a r g e , p a r t i c u l a r l y i n a p a t i e n t w h o h a s u r i n a t e d s h o r t l y b e f o r e e xa mi n a t i o n .
R e c e n t mi c t u r i t i o n c a n e l i mi n a t e mu c h i n f l a mma t o r y d i s c h a r g e . S o me t i me s i t ma y
398 / 652
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b e n e c e s s a r y t o e xa mi n e t h e p a t i e n t i n t h e mo r n i n g b e f o r e v o i d i n g t o e n h a n c e
t h e d i a g n o s i s . I n w o me n , a c o mp l e t e u r o g y n e c o l o g i c e xa mi n a t i o n s h o u l d b e
p e r f o r me d .
2. A c o mp l e t e e xa mi n a t i o n o f t h e a b d o me n ma y b e i n d i c a t e d t o r u l e o u t i n t r a a b d o mi n a l p a t h o l o g y s u c h a s ma s s e s , i n f l a mma t i o n , o b s t r u c t i o n , a n d d i s t e n t i o n
o f o r g a n s . A d d i t i o n a l p h y s i c a l e xa mi n a t i o n s h o u l d i n c l u d e a n e xa mi n a t i o n o f t h e
s k i n a n d o t h e r s y s t e ms a s n e e d e d . I f a G C i n f e c t i o n i s s u s p e c t e d , i t ma y b e
necessary to check the patient's joints, skin, throat, eyes, and other organs.
C. Testing
1. U D s a m p l e c o l l e c t iPorno p e r c o l l e c t i o n a n d h a n d l i n g o f U D s p e c i me n s i s
essential for diagnosis. When the discharge is not spontaneous, the urethra
s h o u l d b e g e n t l y s t r i p p e d . T h i s i s b e s t a c c o mp l i s h e d b y g r a s p i n g t h e p e n i s
f i r ml y b e t w e e n t h e t h u mb a n d f o r e f i n g e r w i t h t h e t h u mb p r e s s i n g o n t h e v e n t r a l
s u r f a c e . T h e e xa mi n e r ' s h a n d i s t h e n mo v e d d i s t a l l y, c o mp r e s s i n g t h e u r e t h r a .
T h i s ma n e u v e r ma y e xp r e s s a s ma l l a mo u n t o f d i s c h a r g e . U D f o r t e s t i n g s h o u l d
p r e f e r a b l y b e c o l l e c t e d w i t h o u t c o n t a mi n a t i o n b y t h e v a r i o u s b a c t e r i a p r e s e n t i n
t h e u r e t h r a l me a t u s . T h e u r e t h r a l me a t u s c a n b e g e n t l y s p r e a d a n d U D c o l l e c t e d
by gently inserting (24 cm into the urethra) a calcium alginate urogenital swab
( c o t t o n s w a b s a r e u n c o mf o r t a b l e d u e t o t h e l a r g e s i ze a n d ma y i n t e r f e r e w i t h t h e
culture) by rotating for 3 to 6 seconds. U D should be collected 1 to 4 hours
( p r e f e r a b l y 4 h o u r s ) a f t e r u r i n a t i o n . P h a r y n g e a l s w a b s ma y b e c o l l e c t e d w h e n
c l i n i c a l l y i n d i c a t e d . T h e s p e c i me n s h o u l d b e d i r e c t l y p l a c e d i n t o t h e c u l t u r e
me d i u m. T h e s a me s w a b ma y b e u s e d f o r a G r a2,3)
m s. t a i n (
2. G r a m ' s s t a i n a n d c u l t Tu hr ee p r e s e n c e o f p o l y mo r p h s w i t h i n t r a c e l l u l a r
d i p l o c o c c i i s d i a g n o s t i c o f G C d i s e a s e . P o l y mo r p h s w i t h o u t t h e i n t r a c e l l u l a r
d i p l o c o c c i a r e s u g g e s t i v e o f N G C d i s e a s e . F e w o r n o p o l y mo r p h s a r e
s u g g e s t i v e o f o t h e r e t i o l o g i e s . T h e G r a m s t a i n i s q u i t e a c c u r a t e f o r me n b u t i t i
n o t v e r y s e n s i t i v e f o r w o me n ( 5 0 % ) , w h e r e c u l t u r e s o f t h e t h r o a t , r e c t u m, a n d
s o me t i me s c o n j u n c t i v a e a r e r e q u i r e d t o e s t a b l i s h t h e d i a g n o s i s .
3. We t p r e p a r a t i o n o f U
T hDi s t e s t i s p e r f o r me d t o e s t a b l i s h t h e d i a g n o s i s o f
t r i c h o mo n a s , c a n d i d i a s i s , a n d s o me v i r a l a n d b a c t e r i a l i n f e c t i o n s ( e . g . , b a c t e r i a
vaginosis; clue cells, viral inclusions).
4. U r e t h r i t i s c o n f i r m a t Ti ohni s i s c o n f i r me d w i t h o n e o f t h e t h r e e c l i n i c a l
findings.
a. P r e s e n c e o f p u r u l e n t o r mu c o p u r u l e n t d i s c h a r g e
b. M o r e t h a n f i v e w h i t e b l o o d c e l l s ( W B C s ) p e r f i e l d i n t h e o i l i mme r s i o n
( 1 0 0 0 ) mi c r o s c o p y o f t h e G r a m s t a i n o f t h e d i s c h a r g e
c. P r e s e n c e o f l e u k o c y t e e s t e r a s e i n t h e f i r s t v o i d e d u r i n e o r t h e p r e s e n c e o f
> 1 0 W B C s p e r h i g h - p o w e r f i e l d ( 4 0 0 ) i n t h e s e d i me n t o f t h e s p u n u r i n e )
5. U r i n a l y s i s a n d u r i n e c u l t ua r e se s s e n t i a l f o r t h e d i a g n o s i s o f u r i n a r y
i n f e c t i o n s . C o l l e c t i o n o f a u r i n e s p e c i me n a s d e s c r i b e d 4)
b y wSi ttha me
f o uyr (
s t e r i l e c o n t a i n e r s ( b e f o r e a n d a f t e r p r o s t a t i c ma s s a g e ) i s s o me t i me s u s e f u l t o
399 / 652
tmdmss
i d e n t i f y t h e s i t e o f i n f e c t i o n i n me n . U r i n a r y l e u k o c y t e e s t e r a s e i s a u s e f u l
s c r e e n i n g t e s t f o r c h l a my d i a l a n d G C i n f e c t i o n s i n a s y mp t o ma t i c me n .
6. N u c l e i c a c i d b a s e d t e(sntusc l e i c a c i d a mp l i f i c a t i o n a s s a y, n u c l e i c a c i d
h y b r i d i za t i o n t e s t , n u c l e i c a c i d g e n e t i c t r a n s f o r ma t i o n t e s t ) a r e n o w u s e d b y
ma n y
P. 2 3 7
c e n t e r s b e c a u s e o f t h e i r s i mp l i c i t y, h i g h s e n s i t i v i t y, a n d s p e c i f i c i t y w h e n
c o mp a r e d w i t h t h e d i f f i c u l t i e s e n c o u n t e r e d w i t h t h e t r a d i t i o n a l c u l t u r e t e s t ( l o w
s e n s i t i v i t y a n d s t o r a g e p r o b l e ms ) . N u c l e i c a c i d a mp l i f i c a t i o n a s s a y s a r e mo r e
p o p u l a r a n d u s e v a r i o u s t e c h n i q u e s ( p o l y me r a s e c h a i n r e a c t i o n , l i g a s e c h a i n
r e a c t i o n , o r s t r a n d d i s p l a c e me n t a mp l i f i c a t i o n o f d e o xy r i b o n u c l e i c a c i d ) t o
i d e n t i f y a n i n f e c t i o n . T h e s e t e s t s c a n b e p e r f o r me d f r o m U D o r 2 0 t o 4 0 mL
( mo r e v o l u me c a n i n t e r f e r e w i t h t h e t e s t ) o f f i r s t v o i d e d , f i r s t c a t c h i n i t i a l s t r e a m
o f u r i n e . F e ma l e p a t i e n t s s h o u l d n o t c l e a n s e t h e l a b i a l a n d u r e t h r a l r e g i o n
b e f o r e t h e c o l l e c t i o n o f t h e f i r s t s t r e a m o f u r i n e . S o me c e n t e r s p r e f e r a u r i n e
s p e c i me n f o r i t s s i mp l i c i t y a n d i n c r e a s e d d e t e c t i o n r a t e , e s p e c i a l l y w i t h s c a n t y
U D (5) .
7. B l o o d s t u d i eAs c o mp l e t e b l o o d c o u n t , c h e mi s t r y p r o f i l e , r a p i d p l a s ma r e a g i n ,
h u ma n i mmu n o d e f i c i e n c y v i r u s , a n d i mmu n o l o g i c s t u d i e s ma y b e r e q u i r e d .
8. D i a g n o s t i c i m a g i A
n gu r e t h r o g r a m a n d p e l v i c , v a g i n a l , a n d r e c t a l u l t r a s o u n d
s t u d i e s ma y b e i n d i c a t e d i n s o me c l i n i c a l c o n d i t i o n s .
9. O t h e r p r o c e d u r eEsxa mi n a t i o n u n d e r a n e s t h e s i a f o r c h i l d r e n a n d e l d e r l y
p a t i e n t s i s s o me t i me s n e c e s s a r y i n t h e e v a l u a t i o n o f U D . A n o s c o p y i s
appropriate for patients who have anal intercourse or for those with anal and
r e c t a l s y mp t o ms . C y s t o u r e t h r o s c o p y a n d l a p a r o s c o p y ma y a l s o b e u s e f u l i n
certain conditions.
D. Diagnosis and Special Concerns

a n d C hl amy di a t r ac homat
i ni fse c t i o n s a r e r e p o r t a b l e t o S t a t e
H e a l t h D e p a r t me n t s , a n d a s p e c i f i c d i a g n o s i s i s e s s e n t i a l . U D s e c o n d a r y t o S T D
i n v o l v e s ma n y p s y c h o s o c i a l a n d me d i c o l e g a l i mp l i c a t i o n s f o r t h e p a t i e n t , t h e i r
p a r t n e r s , t h e i r f a mi l i e s , a n d s o c i e t y. S e xu a l p a r t n e r s s h o u l d b e t r a c e d , t e s t e d , a n d
t r e a t e d . I n c h i l d r e n w i t h U D , s e xu a l a b u s e s h o u l d b e s u s p e c t e d . P r e g n a n t w o me n
w i t h G C i n f e c t i o n o r c h l a my d i a ma y i n f e c t t h e i n f a n t a t b i r t h ( o p h t h a l mi a
n e o n a t o r u m) .
References
1 . S ex ual l y t r ans mi t t ed di s eas es s ur v ei l l anc e and, s2t 0at0i4s,t i R
c se p o r t s 2 0 0 4
N a t i o n a l R e p o r t C D C , h t t p : / / w w w. c d c . g o v / s t d / s t a t s / g o n o r r h e a . h t m.
2 . B e r g e r R E , L e e J C . S e xu a l l y t r a n s mi t t e d d i s e a s e : t h e c l a s s i c d i s e a s e .
C ampbel l ' s ur ol ogy
, 8 t h e d . P h i l a d e l p h i a , PA : W B S a u n d e r s , 2 0 0 2 : 6 7 1 6 7 8 .
3 . L y o n C J . U r e t h r i tCi sl i. n F am P r ac2t0 0 5 ; l 7 ( 1 ) : 3 1 4 1 .
400 / 652
tmdmss
4 . M e a r e s E M , S t a me y T A . B a c t e r i o l o g i c l o c a l i za t i o n p a t t e r n s i n b a c t e r i a l
p r o s t a t i t i s a n d u r e t h rIinv
t i ses
. t U r ol1 9 6 8 ; 5 : 4 9 2 5 1 8 .
5 . h t t p : / / w w w. c d c . g o v / S T D / L a b G u i d e l i n e s / 1 - L G . h t m. S c r e e n i n g T e s t s T o D e t e c t
C hl amy di a t r ac homat
a ni sd N ei s s er i a gonor r hoeae
Infections C D C- 2002.
401 / 652
tmdmss
11.1 - Amenorrhea
> T a b l e o f C o n t e n t s > 11 - P r o b l e ms R e l a t e d t o t h e F e ma l e R e p r o d u c t i v e S y s t e m >

11 . 1 - A me n o r r h e a
11.1
Amenorrhea
Ta n i k a L . D a y
I . Background
A me n o r r h e a i s t h e a b s e n c e o r a b n o r ma l c e s s a t i o n o f me n s e s .
E xc l u d i n g p r e g n a n c y, a me n o r r h e a i s c a u s e d b y d y s f u n c t i o n s o me w h e r e a l o n g t h e
f e ma l e r e p r o d u c t i v e p a t h w a y.
A. Etiology of primary amenorrhea

1. H y p o t h a l a m i c / p i t u i t a r y d y s f u n P
c thiyosni o l o g i c d e l a y o f p u b e r t y o r a b s e n c e
o f g o n a d o t r o p i n - r e l e a s i n g h o r mo n e ( G n R H ) s e c r e t i o n f r o m t h e h y p o t h a l a mu s
( e . g . , K a l l ma n n ' s s y n d r o me p r i ma r y a me n o r r h e a w i t h i mp a i r e d s e n s e o f s me l l )
2. O v a r i a n d y s f u n c t i O
o nv a r i a n f a i l u r e d u e t o c h r o mo s o ma l a b n o r ma l i t y ( i . e . ,
T u r n e r ' s s y n d r o me )
3. O u t f l o w t r a c t d y s f u n c tMi onl l e r i a n d y s g e n e s i s ( mi s s i n g u p p e r t w o - t h i r d s o f
v a g i n a , u t e r u s , a n d f a l l o p i a n t u b e s ) , i mp e r f o r a t e h y me n , t r a n s v e r s e v a g i n a l
s e p t u m, a n d t e s t i c u l a r f e mi n i za t i o n ( g e n o t y p i c a l l y ma l e b u t p h e n o t y p i c a l l y f e ma l
e xc e p t f o r t h e a b s e n c e o f a n o r ma l f e ma l e g e n i t a l t r a c t )
B. Etiology of secondary amenorrhea

(1,2)
1. H y p o t h a l a m i c d y s f u n c tLi o nw G n R H s e c r e t i o n d u e t o s t r e s s e s , s u c h a s
i n t e n s e e xe r c i s e , w e i g h t l o s s / l o w b o d y ma s s i n d e x ( B M I ) a n d / o r ma l n u t r i t i o n
( c o n s i d e r e a t i n g d i s o r d e r a n d / o r t h e f e ma l e a t h l e t e ) ; c h r o n i c d i s e a s e ;
a u t o i mmu n e d i s o r d e r s ( e . g . , h y p e r t h y r o i d i s m, h y p o t h y r o i d i s m, d i a b e t e s me l l i t u s ,
h y p o p a r a t h y r o i d i s m, s y s t e mi c l u p u s e r y t h e ma t o s u s ) ; d e p r e s s i o n o r o t h e r
p s y c h i a t r i c d i s o r d e r s ; p s y c h o t r o p i c me d i c a t i o n u s e ; o r r e c r e a t i o n a l d r u g a b u s e
2. P i t u i t a r y d y s f u n c t iPoint u i t a r y t u mo r s ( p r o l a c t i n o ma , a d r e n o c o r t i c o t r o p i c
h o r mo n e s e c r e t i n g a d e n o ma ( C u s h i n g ' s s y n d r o me ) o r g r o w t h h o r mo n e
s e c r e t i n g a d e n o ma ( a c r o me g a l y ) , S h e e h a n ' s s y n d r o me ( p o s t p a r t u m p i t u i t a r y
n e c r o s i s ) , p a n h y p o p i t u i t a r i s m, e mp t y s e l l a s y n d r o me , i n f i l t r a t i v e d i s e a s e o f
402 / 652
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11.1 - Amenorrhea
p i t u i t a r y ( s a r c o i d o s i s , t u b e r c u l o s i s ) , h e a d i r r a d i a t i o n , o r t r a u ma
3. O v a r i a n d y s f u n c t i o n
a. N o e v i d e n c e o f a n d r o g e n e x
c ep sr esma t u r e o v a r i a n f a i l u r e ( w o me n
y o u n g e r t h a n 4 0 y e a r s o f a g e ) , r a d i a t i o n / c h e mo t h e r a p y t r e a t me n t
b. E v i d e n c e o f a n d r o g e n e x c e s s ( h i r s u t i s m , v i r
i l ipzoaltyi coyns)t i c o v a r y
d i s e a s e , a n d r o g e n - s e c r e t i n g a d r e n a l o r o v a r i a n t u mo r s
4. U t e r i n e d y s f u n c t iAosnh e r ma n ' s s y n d r o me ( u t e r i n e a d h e s i o n s ) , e n d o me t r i a l
hyperplasia, or cancer
C. Epidemiology
T h e p r e v a l e n c e o f s e c o n d a r y a me n o r r h e 3)
a .i s 5 % (
I I I . Evaluation
(3,4)
A. History
1. M e n s t r u a l a n d r e p r o d u c t i v e h i s t o r y
1. M e n a r c h e
2. L a s t me n s t r u a l p e r i o d
3. F r e q u e n c y o f me n s e s a n d d u r a t i o n
4. H i s t o r y o f p r i o r p r e g n a n c i e s
5. H i s t o r y o f p r e g n a n c y o r d e l i v e r y
6. H i s t o r y o f s p o n t a n e o u s o r t h e r a p e u t i c a b o r t i o n s o r d i l a t a t i o n a n d c u r e t t a g e
7. H i s t o r y o f u t e r i n e s u r g e r i e s
2. S y s t e m i c p r o c e s s e s
1. We i g h t l o s s o r g a i n
2. F r e q u e n c y a n d l e v e l o f i n t e n s i t y o f e xe r c i s e
3. P s y c h o s o c i a l o r me d i c a l s t r e s s o r s / c r i s e s
P. 2 4 2
4. N i p p l e d i s c h a r g e
5. H e a d a c h e s o r d e c r e a s e d p e r i p h e r a l v i s i o n
6. H o t f l a s h e s
7. P a i n f u l i n t e r c o u r s e
8. M o o d s w i n g s
9. D e t a i l e d me d i c a l a n d p s y c h i a t r i c h i s t o r y, i n c l u d i n g d i a g n o s e s a n d
me d i c a t i o n s
1 0. D e t a i l e d s o c i a l h i s t o r y, p a r t i c u l a r l y i l l i c i t d r u g u s e
403 / 652
tmdmss
11.1 - Amenorrhea
1. V i t a l s i g n s
2. G e n e r a lA s s e s s f o r g e n e r a l h e a l t h , a f f e c t , T a n n e r s t a g i n g , a n d me a s u r e B M I .
3. S k i n I n s p e c t f o r v i r i l i za t i o n / h i r s u t i s m ( a c n e , ma l e p a t t e r n b a l d n e s s , e xc e s s i v e
h a i r o n f a c e o r c h e s t , i n c r e a s e d mu s c l e ma s s ) v e r s u s s p a r s e a xi l l a r y o r p u b i c
h a i r g r o w t h . L o o k f o r a c a n t h o s i s n i g r i c a n s o r b u f f a l o h u mp , t h i n s k i n , a n d / o r
purple striae.
4. N e c k P a l p a t e f o r t h y r o me g a l y o r t h y r o i d n o d u l e s .
5. B r e a s tI n s p e c t f o r g a l a c t o r r h e a .
6. P e l v i cI n s p e c t f o r a n a t o mi c a b n o r ma l i t i e s , c l i t o r o me g a l y, o r s i g n s o f v a g i n a l
a t r o p h y.
C. Testing
1. L a b o r a t o r y t e s t s
a. O bt ai n a pr egnanc y t es t i n al l women of r epr oduc
. t i v e age
b. I n a n y w o ma n w i t h a me n o r r h e a w h o i s n o t p r e g n a n t , o b t a i n t h e f o l l o w i n g :
i. S e r u m p r o l a c t i n
i i. T h y r o i d - s t i mu l a t i n g h o r mo n e
i i i. F o l l i c l e - s t i mu l a t i n g h o r mo n e
i v. E s t r a d i o l
c. I f t h e p a t i e n t h a s a me n o r r h e a w i t h h i r s u t i s m a n d v i r i l i za t i o n , o b t a i n t h e
following:
i. S e r u m t o t a l t e s t o s t e r o n e
i i. 1 7 - H y d r o xy p r o g e s t e r o n e l e v e l s
i i i. D e h y d r o e p i a n d r o s t e r o n e ( D H E A - S )
d. I f t h e w o ma n h a s b e e n a n o v u l a t o r y f o r mo r e t h a n a y e a r, c o n s i d e r
e n d o me t r i a l b i o p s y. C o n s i d e r h y s t e r o s c o p y i n p a t i e n t s a t r i s k f o r u t e r i n e
adhesions.
2. P r o g e s t i n c h a l l e n g e t e5)s tT h( i s t e s t i s u s e d t o d e mo n s t r a t e e s t r o g e n e f f e c t
a t t h e l e v e l o f t h e e n d o me t r i u m. M e d r o xy p r o g e s t e r o n e a c e t a t e 1 0 mg i s g i v e n
d a i l y f o r 5 t o 1 0 d a y s , a n d i f me n s e s s t a r t s w i t h i n 1 0 d a y s o f c o mp l e t i n g t h e r a p y
the test is positive and the patient is able to produce enough estrogen to cause
e n d o me t r i a l p r o l i f e r a t i o n . T h e d i s a d v a n t a g e i s a d e l a y i n d i a g n o s i s .
Ogb t a i n a ma g n e t i c r e s o n a n c e i ma g i n g ( M R I ) s c a n o f t h e
p i t u i t a r y a n d h y p o t h a l a mu s i n p a t i e n t s w i t h a s s o c i a t e d h e a d a c h e s , v i s u a l f i e l d
d e f e c t s , o r h y p e r p r o l a c t i n e mi a . C o n s i d e r a d u a l - e n e r g y x- r a y a b s o r p t i o me t r y
scan in patients at risk of osteoporosis. In patients with suspected androgens e c r e t i n g t u mo r s , o b t a i n a p e l v i c u l t r a s o u n d a n d c o n s i d e r a d r e n a l c o mp u t e d
404 / 652
tmdmss
11.1 - Amenorrhea
t o mo g r a p h y s c a n o r M R I . I n p a t i e n t s w i t h p r i ma r y a me n o r r h e a a n d a b n o r ma l i t i e s
o f p e l v i c a n a t o my, o b t a i n a p e l v i c u l t r a s o u n d .
D. Genetics
I n p a t i e n t s w i t h c l i n i c a l s i g n s o f T u r n e r ' s s y n d r o me , o b t a i n k a r y o t y p i n g . A l s o
c o n s i d e r a k a r y o t y p e i n w o me n w h o a r e y o u n g e r t h a n 3 0 y e a r s o f a g e w i t h p r e ma t u r
ovarian failure.
I V. Diagnosis
A l w a y s e xc l u d e p r e g n a n c y f i r s t .
1. P r i m a r y a m e n o r r h T
e ah i s i s d e f i n e d a s f o l l o w s :
a. F a i l u r e t o r e a c h me n a r c h e a n d p u b e r t a l mi l e s t o n e s b y 1 4 y e a r s o f a g e
b. F a i l u r e t o r e a c h me n a r c h e b y 1 6 y e a r s o f a g e i n a g i r l w h o h a s d e v e l o p e d
s e c o n d a r y s e xu a l c h a r a c t e r i s t i c s
2. S e c o n d a r y a m e n o r r hTehai s i s d e f i n e d a s t h e d i s r u p t i o n o f r e g u l a r me n s e s f o r
t h e e q u i v a l e n t o f mo r e t h a n t h r e e c y c l e s o r 6 mo n t h s o n c e t h e y h a v e b e g u n .
P. 2 4 3
3. C o m p l i c a t i o nTsh e l o w e s t r o g e n l e v e l s o f p r o l o n g e d a me n o r r h e a a r e
a s s o c i a t e d w i t h i n c r e a s e d r i s k o f o s t e o p e n i a a n d o s t5)
e o. p o r o s i s (
References
1 . L a r s e n P RWi
. l l i ams t ex t book of endoc r i nol
, 1ogy
0 t h e d . P h i l a d e l p h i a , PA :
E l s e v i e r, 2 0 0 3 .
2 . S t e n c h e v e r M DC.ompr ehens i v e G y nec ol, ogy
4th ed. St. Louis, M O: Mosby
Inc., 2001.
3 . E - medi c i ne: amenor r.hea
h t t p : / / w w w. e me d i c i n e . c o m/ me d / t o p i c 11 7 . h t m, 2 0 0 6 .
4 . U p t o dat e: et i ol ogy, di agnos i s , and t r eat ment of s ec ondar y . amenor r hea
h t t p : / / w w w. u t d o l . c o m, 2 0 0 6 .
5 . A p g a r B . D i a g n o s i s a n d ma n a g e me n t o f a me nCol ri n
r hF
e aam
. P r ac t
2002;4(3):643.
405 / 652
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11.2 - Breast Mass

11 . 2 - B r e a s t M a s s
11.2
Breast M ass
Joy ce A. Cope land
I . Background
A b r e a s t ma s s i s a c o l l e c t i o n o f t i s s u e t h a t i s n o t p a r t o f t h e n o r ma l p h y s i o l o g i c
a r c h i t e c t u r e o f t h e ma mma r y g l a n d . T h e p a l p a b l e ma s s i s t h e s u b j e c t o f t h i s c h a p t e
A. Etiology
T h e e t i o l o g y o f t h e b r e a s t ma s s ma y b e ma l i g n a n t o r b e n i g n . M a l i g n a n c y i s t h e ma j o
c o n c e r n , e s p e c i a l l y i n o l d e r w o me n . B e n i g n c h a n g e s s u c h a s a d e n o ma s o r c y s t s a r e
e xt r e me l y c o mmo n .
B. Epidemiology
O n e o f t h e t h r e e mo s t c o mmo n p r e s e n t a t i o n s f o r b r e a s t c o mp l a i n t s i s a p a l p a b l e
b r e a s t ma s s . M o s t o f t h e s e a r e b e n i g n , b u t t h i s mu s t b e t e mp e r e d w i t h t h e f a c t t h a t
b r e a s t c a n c e r i s t h e mo s t c o mmo n c a n c e r i n w o me n a n d t h e s e c o n d mo s t c o mmo n
c a u s e o f c a n c e r d e a1)t h. M
( o s t c a n c e r s a r e p r e s e n t i n w o me n o l d e r t h a n 5 0 y e a r s
o f a g e , b u t o n e - t h i r d o f t h e m a r e i n y o u n g e r2)w. o me n (
I I I . Evaluation
A. History
1. C u r r e n t m e d i c a l h i s t o r y a n d c h i e f c o m p l a i n t
a. W h e n a n d h o w w a s t h e ma s s d i s c o v e r e d ? D o e s t h e p a t i e n t p e r f o r m r e g u l a r
b r e a s t s e l f - e xa mi n a t i o n s ? W h a t , i f a n y, c h a n g e s h a v e o c c u r r e d s i n c e t h e
discovery?
b. A g e a n d m e n s t r u a l s t a tCuasn. c e r i s mo r e p r o mi n e n t i n t h e a g e - g r o u p s
o v e r 5 0 y e a r s o f a g e , a l t h o u g h 3 % ma y b e s e e n i n t h e 2 0 - t o 2 9 - y e a r a g e g r o u p . A p p r o xi ma t e l y 8 5 % o f ma s s e s i n t h e p o s t me n o p a u s a l a g e - g r o u p
p r o v e t o b e c a n c e r o3)
u s. (
c. I s t h e ma s s p a i n f u l ? I f s o , i s t h e r e a n y c y c l i c v a r i a t i o n i n t h e p a i n ? H a s
t h e r e b e e n a n y n i p p l e d i s c h a r g e ? H a s t h e p a t i e n t n o t i c e d a n y w a r mt h o r
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11.2 - Breast Mass
redness?
2. P a s t m e d i c a l h i s t o r y
a. R e p r o d u c t i v e h i s t oWr yh a t i s t h e r e p r o d u c t i v e h i s t o r y a n d c u r r e n t
me n s t r u a l s t a t u s ? H a s t h e p a t i e n t e v e r b r e a s t - f e d a n i n f a n t ? I s s h e o n
e s t r o g e n r e p l a c e me n t t h e r a p y ( E R T ) ? A w o ma n w h o b r e a s t - f e e d s f o r 2
y e a r s ma y h a v e a d e c r e a s e d r i s k o f b r e a s t c a n c e r. E R T h a s a c o n t r o v e r s i a l
r o l e i n e t i o l o g y o r a d v a n c e me n t o f b r e a s t c a n c e r. W h a t w a s h e r a g e a t t h e
birth of her first child?
b. B r e a s t h i s t o rTyh e p a t i e n t s h o u l d b e q u e s t i o n e d a b o u t a n y p r e v i o u s
e xp e r i e n c e o f a b r e a s t ma s s , b r e a s t b i o p s y o r s u r g e r y, a n d t h e c l i n i c a l
o u t c o me . H a s s h e h a d a p e r s o n a l h i s t o r y o f b r e a s t c a n c e r o r a t y p i c a l
hyperplasia on
P. 2 4 4
a previous biopsy? A prior history of breast cancer or atypical hyperplasia
o n a b i o p s y i n c r e a s e s t h e r i s k o f ma l i g n a n c y. D o e s t h e p a t i e n t h a v e a
h i s t o r y o f r a d i a t i o n e xp o s u r e o r c h e mo t h e r a p y ? H a s t h e r e b e e n a n y r e c e n t
b r e a s t t r a u ma ? W h a t i s h e r c u r r e n t l a c t a t i o n s t a t u s ?
3. F a m i l y h i s t o rIys t h e r e a f a mi l y h i s t o r y o f b r e a s t c a n c e r ? I f y e s , w h a t i s t h e
r e l a t i o n s h i p o f t h e f a mi l y me mb e r a n d a t w h a t a g e w a s t h e c a n c e r d i a g n o s e d
a n d w h a t w a s t h e r e l a t i v e ' s me n s t r u a l s t a t u s ? A mo t h e r o r s i s t e r w i t h
p r e me n o p a u s a l b r e a s t c a n c e r i n d i c a t e s t h e h i g h e s t l e v e l o f r i s k .
4. L i f e s t y l eI n q u i r e a b o u t t h e p a t i e n t ' s a l c o h o l i n t a k e a n d d i e t a r y h a b i t s . T h e r e i s
a p o s s i b l e l i n k b e t w e e n b r e a s t c a n c e r a n d a l c o h o l c o n s u mp t i o n , a h i g h - f a t d i e t ,
a n d o b e s i t y. A s k a b o u t s mo k i n g h i s t o r y a s w e l l .
1. I n s p e c t i o nI n s p e c t t h e b r e a s t s f o r s y mme t r y, c o n t o u r, s k i n r e t r a c t i o n , r a s h e s ,
peau d' or ange
, n i p p l e d i s c h a r g e , a n d e r y t h e ma o r e d e ma . D i s r u p t i o n s o f
s y mme t r y a n d c o n t o u r ma y a c c o mp a n y a b r e a s t ma s s .
2. P a l p a t i o n a n d c o m p r e s sPi oa nl p a t e b o t h b r e a s t s , i n c l u d i n g t h e n i p p l e a n d
a r e o l a r r e g i o n . P a l p a t e t h e s u p r a c l a v i c u l a r, i n f r a c l a v i c u l a r, a n d a xi l l a r y r e g i o n s
f o r a d e n o p a t h y. E v a l u a t e t h e c o n s i s t e n c y, r e g u l a r i t y, l o c a t i o n , a n d mo b i l i t y o f
t h e ma s s , a s w e l l a s t h e p r e s e n c e o f t e n d e r n e s s .
C. Testing
1. I m a g i n g s t u d i eTsh e ma mmo g r a m i s u s e d t o c h a r a c t e r i ze t h e n a t u r e o f t h e
ma s s a n d t o p r o v i d e a n a s s e s s me n t o f t h e r e ma i n d e r o f t h e b r e a s t t i s s u e a n d
the contralateral breast. It is not a diagnostic procedure. Ultrasound is used to
c h a r a c t e r i ze a ma s s a s s o l i d o r c y s t i c o r i d e n t i f y ma s s e s t h a t ma y n o t b e
i d e n t i f i e d b y ma mmo g r a p h y. I t i s h e l p f u l i n e v a l u a t i n g ma s s i n a p a t i e n t y o u n g e r
t h a n 3 5 y e a r s o f a g e a n d ma y b e u s e d a s a n a d j u n c t i n t h e p e r f o r ma n c e o f
a s p i r a t i o n o r b i o p s y f o r t h e i n d e t e r mi n a t e l e s i o n .
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11.2 - Breast Mass
2. F i n e n e e d l e a s p i r a t i o n ( F4)
N AF )N( A c a n b e u s e d t o o b t a i n t i s s u e o r f l u i d i n a
p a l p a b l e ma s s . F l u i d a s p i r a t i o n p l u s r e s o l u t i o n o f t h e ma s s s u g g e s t s a c y s t i c
o r i g i n . G r o s s l y b l o o d y f l u i d d e ma n d s a f u r t h e r e v a l u a t i o n o f t h e ma s s . A c y s t i c
ma s s i n a p o s t me n o p a u s a l w o ma n n o t r e c e i v i n g E R T r e q u i r e s a mo r e t h o r o u g h
evaluation.
R e e xa mi n e t h e b r e a s t i n 4 t o 6 w e e k s i f t h e c y s t r e s o l v e s . I f t h e f l u i d
r e a c c u mu l a t e s , r e a s p i r a t e . R e s i d u a l ma s s o r a s y mme t r y a f t e r a s p i r a t i o n r e q u i r e
ma mmo g r a p h y a n d b i o p s y. I f n o a s p i r a t e i s o b t a i n e d , p r o c e e d w i t h e xc i s i o n a l
b i o p s y.
3. F i n e n e e d l e a s p i r a t i o n b i o p s y ( F4)
N AF B
N )A( B h a s a s e n s i t i v i t y o f 0 . 6 5 t o
0 . 9 8 a n d a s p e c i f i c i t y o f 0 . 3 4 t o 1 . 0 . T h e p r o c e d u r e p r o v i d e s ma t e r i a l f o r a
c y t o l o g i c e xa mi n a t i o n . C o r r e l a t i o n w i t h i ma g i n g s t u d i e s mu s t b e c o n c o r d a n t i n
c o n c l u s i o n o r e xc i s i o n a l b i o p s y i s i n d i c a t e d . I ma g i n g g u i d a n c e i s i n d i c a t e d f o r a
n o n p a l p a b l e ma s s . A t y p i a o f a n y d e g r e e w a r r a n t s e xc i s i o n a l b i o p s y.
4. C o r e n e e d l e b i o p T
s yh i s t e c h n i q u e i n v o l v e s t h e u s e o f a l a r g e r g a u g e n e e d l e
a n d p r o v i d e s h i s t o l o g i c ma t e r i a l f o r a s s e s s me n t . I t i s u s u a l l y p e r f o r me d i n
c o n j u n c t i o n w i t h s t e r e o t a c t i c ma mmo g r a p h y o r u l t r a s o u n d g u i d a n c e . T h e r e i s a
5 0 % c o n c o r d a n c e w i t h s u r g i c a l b i o p s y a t 5 0 % o f 5)t h. e c o s t (
5. T r i p l e t e s t f o r s o l i d m 6)
a s sT h( e t r i p l e t e s t i n c l u d e s p h y s i c a l e xa mi n a t i o n ,
i ma g i n g f i n d i n g s , a n d c y t o l o g y t h r o u g h F N A B . T h i s t e c h n i q u e h a s a s e n s i t i v i t y
of 97% to 100% and a specificity of 98% to 100%. C oncordance for benign
findings allows the clinician to stop testing. Malignant cytopathology requires
e xc i s i o n a l b i o p s y. I n c o n c l u s i v e r e s u l t s w i t h o u t c o n c o r d a n c e r e q u i r e o p e n
e xc i s i o n a l b i o p s y.
6. O p e n e x c i s i o n a l b i o pAs lye s i o n t h a t i s h i g h l y s u s p i c i o u s o n c l i n i c a l
e xa mi n a t i o n a n d / o r ma mmo g r a p h y i s b e s t e v a l u a t e d w i t h o p e n b i o p s y a n d
e xc i s i o n . A t y p i c a l c e l l s o n b i o p s y a l s o r e q u i r e a mo r e d e f i n i t i v e t i s s u e d i a g n o s i s
7. E m e r g i n g t e c h n o l o M
g ya g n e t i c r e s o n a n c e i ma g i n g w i t h g a d o l i n i u m h a s a h i g h
s e n s i t i v i t y b u t i t s s p e c i f i c i t y i s 4 7 . 6 7 % . I t i s l e s s s e n s i t i v e t h a n ma mmo g r a p h y f
i n s i t ul e s i o n s . I t ma y h a v e a r o l e i n t h e e v a l u a t i o n o f w o me n w i t h s i l i c o n e
P. 2 4 5
b r e a s t i mp l a n t s , t h o s e w h o h a v e h a d b r e a s t - c o n s e r v i n g s u r g e r y, a n d t h o s e w h o
h a v e a g e n e t i c p r e d i s p o s i t i o n f o r c7)a .n c e r (
D. Genetics
A f a mi l y h i s t o r y o f b r e a s t c a n c e r, o v a r i a n c a n c e r, c o l o n c a n c e r, o r p r o s t a t e c a n c e r
s h o u l d b e e l i c i t e d . A mu l t i g e n e r a t i o n a l h i s t o r y i s p a r t i c u l a r l y u s e f u l .
R e c o mme n d a t i o n s f o r g e n e t i c t e s t i n g , a l t h o u g h n o t a p a r t o f t h e a c u t e w o r k u p o f a
b r e a s t ma s s , ma y b e c o n s i d e r e d i f a n u mb e r o f r e l a t i v e s , e s p e c i a l l y f i r s t - d e g r e e o r
mu l t i g e n e r a t i o n a l , h a v e t h e s e c a n c e r s .
I V. Diagnosis
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11.2 - Breast Mass
T h e d i f f e r e n t i a l d i a g n o s i s o f a b r e a s t ma s s i n c l u d e s c y s t , f i b r o a d e n o ma , f i b r o c y s t i c
b r e a s t c h a n g e s , g a l a c t o c e l e s , h e ma t o ma , a b s c e s s , a n d c a n c e r.
1. A g e o f p r e s e n t a t i o n i s a c r i t i c a l f e a t u r e i n t h e c l i n i c a l p r e s e n t a t i o n o f a b r e a s t
ma s s . Wo me n o l d e r t h a n 4 0 y e a r s o f a g e h a v e a h i g h e r r i s k o f b r e a s t c a n c e r.
T h i s i s p a r t i c u l a r l y t r u e i n p o s t me n o p a u s a l w o me n w i t h a n e w ma s s .
2. C y c l i c p a i n s u g g e s t s a c y s t i c o r i g i n . P e r s i s t e n t p a i n ma y r e p r e s e n t b r e a s t
c a n c e r o r a n i n f l a mma t o r y p r o c e s s . P r e g n a n c y e xp a n d s t h e l i s t o f p o s s i b l e
c a u s e s o f a ma s s t o i n c l u d e ma s t i t i s , g a l a c t o c e l e , o r a b r e a s t a b s c e s s .
3. H a r d , i mmo b i l e , i r r e g u l a r ma s s e s r a i s e t h e s u s p i c i o n o f b r e a s t c a n c e r. S mo o t h ,
c y s t i c , o r r u b b e r y ma s s e s s u g g e s t a c y s t o r f i b r o a d e n o ma . T h e s e c h a r a c t e r i s t i c
d o n o t g u a r a n t e e t h e b e n i g n n a t u r e o f t h8)
e .ma
F isbsr o(c y s t i c c h a n g e s a r e
o f t e n n o n d i s c r e t e a n d i r r e g u l a r b u t a r e a l s o mo b i l e a n d r e l a t i v e l y s o f t .
C o mp r e s s i o n o f t h e n i p p l e ma y e xp r e s s a d i sCchhaaprtgeer (11). .6
4. R e t r a c t i o n s u g g e s t s e i t h e r c h r o n i c i n f l a mma t i o n o r b r e a s t c a n c e r d u e t o t h e
a d h e r e n c e o f s k i n t o t h e ma
P eau
s s . d' or ange
is a puckering or indentation of
t h e s k i n o v e r a ma s s . T h i s f e a t u r e r a i s e s t h e s u s p i c i o n o f ma l i g n a n c y.
5. A r a s h ma y b e r e l a t e d t o P a g e t ' s d i s e a s e w i t h a r e l a t e d d u c t a l c a r c i n o ma .
References
1 . A me r i c a n C a n c e r S o c i e t y. C a n c e r f a c t s a n d f i g u r e s 2 0 0 3 . Av a i l a b l e a t :
h t t p : / / w w w. c a n c e r. o r g / d o w n l o a d s / S T T / C A F F 2 0 0 3 P W S e c u r e d . p d f , a c c e s s e d
online August 7, 2005.
2 . N a t i o n a l C a n c e r I n s t i t u t e . S E E R 1 9 7 3 2 0 0 1 p u b l i c - u s e d a t a . Av a i l a b l e a t :
h t t p : / / s e e r. c a n c e r. g o v / p u b l i c d a t a / , a c c e s s e d o n l i n e A u g u s t 7 , 2 0 0 5 .
3 . W h i t e G , G r i f f i t h C , N e n s t i e l R , e t a l . B r e a s t c a n c e r : r e d u c i n g mo r t a l i t y
t hr ough ear l y det ectC
i olni n. R ev1 9 9 6 ; 6 ( 9 ) : 7 7 7 9 , 8 3 8 4 , 1 0 0 1 0 6 .
4 . h t t p : / / w w w. me d s c a p e . c o m/ v i e w a r t i c l e / 4 4 3 3 8 1 , a c c e s s e d o n 2 0 0 6 .
5 . L i b e r ma n L , F a h s M C , D e s h a w D D , e t a l . I mp a c t o f s t e r e o t a xi c c o r e b r e a s t
b i o p s y o n c o s t o f d i a g n oRsadi
i s . ol ogy1 9 9 5 ; 1 9 5 : 6 3 3 .
6 . T h e u n i f o r m a p p r o a c h t o b r e a s t f i n e - n e e d l e a s p i r a t i o n b i o p s y. [ E d i t o r i a l
O p i n i o n ] . N a t i o n a l C a n c e r I n s t i t u t e C o n fAem
r e nJ cSe ur
. g1 9 9 7 ; 1 7 4 ( 4 ) : 3 7 1
385.
7 . K l e i n S . E v a l u a t i o n o f p a l p a b l e b r e a s t ma
A ms sFeam
s . P hy s i c i an
2005;71:17311738.
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11.2 - Breast Mass
8 . Ve n e t L , S t r a x P, Ve n t W, e t a l . A d e q u a c i e s a n d i n a d e q u a c i e s o f b r e a s t
e xa mi n a t i o n s b y p h y s i c i a n s i n ma s s s c rCe anc
e n i ner
g 1. 9 7 1 ; 2 8 : 1 5 4 6 .
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11.3 - Chronic Pelvic Pain

11 . 3 - C h r o n i c P e l v i c P a i n
11.3
Chronic Pelvic Pain
P a u l V . A i t k e n J r.
Albe rt A. Me y e r
I . Background
Chronic pelvic pain is defined as an episodic or continuous pain that persists for 6
mo n t h s o r l o n g e r a n d i s s u f f i c i e n t l y s e v e r e t o h a v e a s i g n i f i c a n t i mp a c t o n a w o ma n
l i f e s t y l e a n d d a y - t o - d a y f u n c t i o n o r r e l a t i o n s h i p s . A w o ma n h a s a n a p p r o xi ma t e 5 %
c h a n c e o f h a v i n g c h r o n i c p e l v i c p a i n i n h e r1)l i.f e t i me (
Chronic pelvic pain is less likely to be associated with an identifiable

p a t h o p h y s i o l o g i c d i s o r d e r t h a n t h a t l a s t i n g < 3 mo n t h s . E v e r y a n a t o mi c s t r u c t u r e i n
t h e a b d o me n o r p e l v i s c o u l d h a v e a r o l e i n t h e e t i o l o g y o f c h r o n i c p e l v i c p a i n . I t i s
h e l p f u l t o a t t e mp t t o c a t e g o r i ze t h e p a i n a s e i t h e r g y n e c o l o g i c o r n o n g y n e c o l o g i c a s
t h e e v a l u a t i o n p r o c e e d s t o w a r d t h e mo r e s p e c i f i c d i a g n o s i s . G o o d a n d c o n s i s t e n t
scientific evidence shows that gynecologic conditions that cause chronic pelvic pain
a r e e n d o me t r i o s i s , g y n e c o l o g i c ma l i g n a n c i e s , a n d p e l v i c i n f l a mma t o r y d i s e a s e . I t
also shows that nongynecologic conditions that cause chronic pelvic pain are
c y s t i t i s , i n f l a mma t o r y b o w e l d i s e a s e , p e l v i c f l o o r my a l g i a , a n d s o ma t i za t i o n d i s o r d e
(2) . T h e r e f o r e , a c o mp r e h e n s i v e a n d d e t a i l e d mu l t i s y s t e m e v a l u a t i o n i s w a r r a n t e d .
I I I . Evaluation
A. History
1. C o mp o n e n t s o f a n a p p r o p r i a t e me d i c a l h i s t o r y a r e a3)s : f o l l o w s (
a. O n s e t , d u r a t i o n , a n d p a t t e r n o f t h e p a i n
b. L o c a t i o n , i n t e n s i t y, c h a r a c t e r, a n d r a d i a t i o n o f t h e p a i n
c. A g g r a v a t i n g o r r e l i e v i n g f a c t o r s
d. R e v i e w o f u r i n a r y, mu s c u l o s k e l e t a l , a n d g a s t r o i n t e s t i n a l s y s t e ms
e. R e l a t i o n s h i p o f t h e p a i n t o s e xu a l a c t i v i t y o r me n s t r u a t i o n
f. F a t i g u e a n d a n o r e xi a
g. M e d i c a t i o n h i s t o r y ( e . g . , u s e o f b i r t h c o n t r o l p i l l s o r o v e r - t h e - c o u n t e r
411 / 652
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me d i c a t i o n s )
h. P a s t o b s t e t r i c , g y n e c o l o g i c , a n d g e n e r a l s u r g i c a l h i s t o r i e s
2. I t s h o u l d b e n o t e d t h a t w o me n w i t h a h i s t o r y o f p e l v i c i n f l a mma t o r y d i s e a s e a r e
f o u r t i me s mo r e l i k e l y t o d e v e l o p c h r o n i c p e l v i c p a i n . A n i n d i v i d u a l w i t h
i n t e s t i n a l , s e xu a l , u r i n a r y, mu s c u l o s k e l e t a l , a n d s y s t e mi c s y mp t o ms ma y b e
suffering from a psychiatric disorder (e.g., depression). Specific questions
s h o u l d e xp l o r e t h e p o s s i b i l i t y o f a c u r r e n t o r r e mo t e h i s t o r y o f s e xu a l a b u s e .
O f t e n i t i s p o s s i b l e t o o b t a i n t h i s i n f o r ma t i o n o n l y w h e n t h e p r o v i d e r c r e a t e s a n
a t mo s p h e r e o f mu t u a l r e s p e c t a n d t r u s t .
3. D y s p a r e u n i a i s o f t e n a f e a t u r e o f c h r o n i c p e l v i c p a i n . S u p e r f i c i a l d y s p a r e u n i a
ma y i n d i c a t e a u r e t h r i t i s , w h e r e a s d e e p d y s p a r e u n i a ma y s i g n i f y a d h e s i o n s .
C y c l i c p a i n t h a t i s r e l a t e d t o me n s t r u a t i o n u s u a l l y p o i n t s t o a g y n e c o l o g i c
p r o b l e m. P a i n r e f e r r e d t o t h e a n t e r i o r t h i g h , o r a s s o c i a t e d w i t h i r r e g u l a r u t e r i n e
b l e e d i n g , o r n e w o n s e t d y s me n o r r h e a ma y h a v e a u t e r i n e o r o v a r i a n c a u s e .
Urethral tenderness, dysuria, or bladder pain suggests interstitial cystitis or a
u r e t h r a l p r o b l eC
mh(a p t e r 1 0). .1 P a i n o n d e f e c a t i o n , me l a n a , b l o o d y s t o o l s , o r
a b d o mi n a l p a i n w i t h a l t e r n a t i n g d i a r r h e a a n d c o n s t i p a t i o n c a n p o i n t t o w a r d
p e l v i c f l o o r p r o b l e ms , i r r i t a b l e b o w e l s y n d r o me , o r i n f l a mma t o r y b o w e l d i s e a s e s
1. T h e g e n e r a l c o n d i t i o n o f t h e p a t i e n t s h o u l d b e n o t e d . D o e s t h e p a t i e n t l o o k
c h r o n i c a l l y i l l , w h i c h ma y s u g g e s t a p e l v i c l e s i o n o r a n i n f l a mma t o r y b o w e l
d i s o r d e r ? D o e s t h e p a t i e n t a p p e a r a n xi o u s , s t r e s s e d , o r i n a p p r o p r i a t e ?
a. C a n t h e p a t i e n t p o i n t t o t h e p a i n w i t h o n e f i n g e r ? I f s o , t h i s c a n i n d i c a t e
t h a t t h e p a i n ma y h a v e a d i s c r e t e s o u r c e .
TAB L E 11.3.1 Causes of Chronic Pelvic Pain

E n d o me t r i o s i s
P e l v i c i n f l a mma t o r y d i s e a s e
G y n e c o l o g i c ma l i g n a n c i e s
Cystitis
P e l v i c f l o o r my a l g i a
S o ma t i za t i o n d i s o r d e r s
Depression
H e r n i a t e d l u mb a r d i s c
L u mb a r l o r d o s i s
Spondylolisthesis
P. 2 4 7
b. A n e xa mi n a t i o n o f t h e l o w e r b a c k , s a c r a l a r e a , a n d c o c c y x, i n c l u d i n g a
412 / 652
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n e u r o l o g i c e xa mi n a t i o n o f t h e l o w e r e xt r e mi t i e s , i s n e c e s s a r y. A h e r n i a t e d
d i s c , e xa g g e r a t e d l u mb a r l o r d o s i s , a n d s p o n d y l o l i s t h e s i s c a n a l l c a u s e
pelvic pain.
c. E xa mi n e t h e a b d o me n , l o o k i n g f o r s u r g i c a l s c a r s , d i s t e n t i o n , a n d p a l p a b l e
t e n d e r n e s s , p a r t i c u l a r l y i n t h e e p i g a s t r i u m, f l a n k , b a c k , o r b l a d d e r.
2. A t h o r o u g h p e l v i c e xa mi n a t i o n i s t h e mo s t i mp o r t a n t p a r t o f t h e e v a l u a t i o n .
C. Testing(4)
I f n o o b v i o u s c a u s e i s a p p a r e n t , i t i s r e a s o n a b l e t o o b t a i n a c o mp l e t e b l o o d c o u n t ,
u r i n a l y s i s , s e d i me n t a t i o n r a t e , a n d s e r u m c h e mi s t r y p r o f i l e . A p e l v i c u l t r a s o u n d ma y
b e h e l p f u l i f p e l v i c e xa mi n a t i o n i s i n c o n c l u s i v e . L a p a r o s c o p y i s b e s t u s e d t o
d i a g n o s e a d e f i n i t e p e l v i c ma s s . L a p a r o s c o p y h a s b e e n u s e d i n t h e p a s t b u t v a r i o u s
studies have shown a 66% negative laparoscopy rate for patients with chronic pelvi
p a i n . A mu l t i d i s c i p l i n a r y a p p r o a c h u s i n g me d i c a l , p s y c h o l o g i c , e n v i r o n me n t a l , a n d
n u t r i t i o n a l d i s c i p l i n e s s h o w e d a d e c r e a s e i n p a i n a f t e r 1 y e a r.
I V. Diagnosis
C h r o n i c p e l v i c p a i n h a s a n e xt e n s i v e d i f f e r e n t i a l 1)
d i a( gs ne oeTsai sb l(e 11 . 3). .1
T h e s e c o mp l e x p r o b l e ms c a n b e a s s e s s e d u s i n g a mu l t i s y s t e m a p p r o a c h . W h e r e a s
g a s t r o i n t e s t i n a l , g y n e c o l o g i c , mu s c u l o s k e l e t a l , a n d p s y c h i a t r i c c o n d i t i o n s c a n c a u s
c h r o n i c p e l v i c p a i n , a t h o r o u g h g y n e c o l o g i c h i s t o r y a n d p e l v i c e xa mi n a t i o n a r e t h e
c o r n e r s t o n e s o f t h e d i a g n o s t i c a s s e s s me n t . A f e w l a b o r a t o r y t e s t s a r e h e l p f u l . A
pelvic ultrasound is useful when the pelvic organs cannot be adequately assessed
d u r i n g t h e p h y s i c a l e xa mi n a t i o n . A t e a m a p p r o a c h , c o o r d i n a t e d b y a t r u s t e d f a mi l y
p h y s i c i a n , c a n b r i n g mu c h r e l i e f t o p a t i e n t s w h o h a v e t h i s f r u s t r a t i n g c l i n i c a l
p r o b l e m.
References
1 . R y d e r R M . C h r o n i c p e l v i c pAami nF. am P hy s i c i an
1996;54(7):22252232.
2 . A me r i c a n C o l l e g e o f O b s t e t r i c i a n s a n d G y n e c o l o g i s t s . C h r o n i c p e l v i c p a i n .
A C O G p r a c t i c e b u l l e t i n N oO. b5s1t e t G y n e c o2l0 0 4 ; 1 0 3 ( 3 ) : 5 8 9 6 0 5 .
3. Gunter J. Chronic pelvic pain: an integrated approach to diagnosis and
t r e a t me n tO. bs t et G y nec ol S ur
2 0v 0 3 ; 5 8 ( 9 ) : 6 1 5 6 2 3 .
4 . C h a n P D , W i n k l e C R , eGdysnec
. ol ogy and obs t et r i c s , 19992000
. Laguna
H ills, C A: C urrent C linical S trategies P ublishers, 1999:2325.
413 / 652
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11.4 - Dysmenorrhea

11 . 4 - D y s me n o r r h e a
11.4
Dysmenorrhea
P a u l V . A i t k e n J r.
Albe rt A. Me y e r
I . Background
D y s me n o r r h e a c a n b e d e f i n e d a s a c o mp l a i n t o f p a i n e xp e r i e n c e d d u r i n g o r
i mme d i a t e l y b e f o r e me n s t r u a t i o n .
D y s me n o r r h e a i s t h e mo s t c o mmo n g y n e c o l o g i c p r o b l e m i n me n s t r u a t i n g w o me n , w i t
u p t o 9 0 % o f w o me n e xp e r i e n c i n g t h i s s y mp t o m a t s o me p o i n t i1)
n .t hRei isr k l i v e s (
f a c t o r s i n c l u d e y o u n g e r a g e a t me n a r c h e ; c i g a r e t t e s mo k i n g ; a n d t h e c o n c o mi t a n t
p r e s e n c e o f p r o l o n g e d , h e a v y, a n d i r r e g u l a r p e r i o d s . P r e g n a n c y h i s t o r y a n d d i e t a r y
f a c t o r s d o n o t s e e m t o c o r r e l a t e w i t h t h i s s2)y mp
. tom (
I I I . Evaluation
A. History
1. A d e t a i l e d me n s t r u a l a n d g y n e c o l o g i c h i s t o r y i s c r i t i c a l t o t h e d i a g n o s i s . G i v e n
t h e h i s t o r y, i t i s e xt r e me l y i mp o r t a n t t o d i s t i n g u i s h p r i ma r y f r o m s e c o n d a r y
d y s me n o r r h e a .
a. P r i ma r y d y s me n o r r h e a s t a r t s a t t h e o n s e t o f me n a r c h e a n d i s t h o u g h t t o b e
t h e r e s u l t o f p r o s t a g l a n d i n - 2 , w h i c h p r o d u c e s u t e r i n e i s c h e mi a . I t c a n b e
treated with antiprostaglandins and oral contraceptives.
b. S e c o n d a r y d y s me n o r r h e a s t a r t s l a t e r i n a w o ma n ' s o v u l a t o r y l i f e a n d ma y
b e c a u s e d b y e n d o me t r i o s i s o r p e l v i c p a t h o l o g y.
i. I f a b n o r ma l b l e e d i n g i s a s s o c i a t e d w i t h e i t h e r t y p e o f d y s me n o r r h e a , i t
i s i mp o r t a n t t o e l i c i t s y mp t o ms o f p r e g n a n c y, s u c h a s mi s s e d o r l a t e
me n s e s , b r e a s t t e n d e r n e s s , n a u s e a , o r u r i n a r y f rCehqaupetnecr y (
11 . 1) .
i i. I f s e v e r e p a i n d e v e l o p s d u r i n g t h e f i r s t p a r t o f t h e me n s t r u a l c y c l e ,
a s c e r t a i n t h e h i s t o r y o f s e xu a l p a r t n e r s , a b n o r ma l v a g i n a l d i s c h a r g e , o r
d y s p a r e u n i a . T h e s e s y mp t o ms c o u l d p o i n t t o w a r d p e l v i c i n f l a mma t o r y
d i s e a s e ( P I DC) h( a p t e r 11). .3
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11.4 - Dysmenorrhea
i i i. P a i n t h a t d e v e l o p s d u r i n g me n s e s , b u t n o t r e l a t e d t o p r e g n a n c y o r
i n f e c t i o n , c a n a l s o b e c a u s e d b y t u mo r. I n y o u n g e r w o me n , s e c o n d a r y
d y s me n o r r h e a t h a t i s s u f f i c i e n t l y s e v e r e t o a f f e c t d a i l y f u n c t i o n i n g o r
r e l a t i o n s h i p s s u g g e s t s e n d o me t r i o s i s . T h i s c o n d i t i o n a f f e c t s a s ma n y
a s 1 9 % o f w o me3)
n .( D e e p d y s p a r e u n i a a n d s a c r a l b a c k a c h e w i t h
me n s e s a r e c o mmo n s y mp t o ms . P r e me n s t r u a l t e n e s mu s o r d i a r r h e a
c o r r e l a t e s w i t h e n d o me t r i o s i s o f t h e r e c t o s i g mo i d a r e a , w h e r e a s c y c l i c
h e ma t u r i a o r d y s u r i a ma y i n d i c a t e b l a d d e r e n d o me t r i o s i s .
i v. I n f e r t i l i t y i s o f t e n a c o n s e q u e n c e o f e n d o me t r i o s i s .
1. A s w i t h a l l me n s t r u a l c o mp l a i n t s , a t h o r o u g h p h y s i c a l e xa mi n a t i o n i s a n e s s e n t i a
p a r t o f ma k i n g a d i a g n o s i s . T h e g e n e r a l c o n d i t i o n o f t h e p a t i e n t n e e d s t o b e
a s s e s s e d . A r e t h e v i t a l s i g n s s t a b l e o r i s t h e p a t i e n t s h o w i n g s i g n s o f s y s t e mi c
i l l n e s s s u c h a s f e v e r, w h i c h c a n i n d i c a t e p e l v i c i n f e c t i o n ? H y p o t e n s i o n a n d
p a l l o r c a n i n d i c a t e a r u p t u r e d e c t o p i c p r e g n a n c y.
2. A g e n e r a l p h y s i c a l a s s e s s me n t w i t h a t t e n t i o n t o t h e b a c k , s a c r u m, s p i n e ,
a b d o me n , a n d b l a d d e r i s i mp o r t a n t .
3. A t h o r o u g h p e l v i c e xa mi n a t i o n i s t h e k e y t o e xc l u d i n g o t h e r p o s s i b l e d i a g n o s e s .
T h e e xt e r n a l g e n i t a l i a ma y s h o w s i g n s o f c y a n o s i s , a s i s s e e n w i t h p r e g n a n c y,
o r a b n o r ma l d i s c h a r g e , a s i s s e e n w i t h i n f e c t i o n . P a l p a t e t h e v a g i n a l a r e a f o r
n o d u l e s t h a t ma y p r e s e n t o n t h e acnul
t e-rdei o r s aco r o n t h e p o s t e r i o r v a g i n a l
f o r n i x o n b i ma n u a l e xa mi n a t i o n ; t h e y c o u l d i n d i c a t e e n d o me t r i o s i s . C e r v i c a l
mo t i o n t e n d e r n e s s a n d c e r v i c a l l e u k o r r h e a ma y b e p r e s e n t i n P I D . U t e r i n e
t e n d e r n e s s i s o f t e n p r e s e n t a n d u t e r i n e d i s p l a c e me n t a n d f i xa t i o n ma y b e n o t e d
O v a r i a n e n l a r g e me n t o r a d n e xa f i xa t i o n ma y c o r r e l a t e w i t h e n d o me t r i o s i s . A n
a d n e xa l
P. 2 4 9
ma s s f r o m a n e o p l a s t i c o r i n f e c t i o u s c a u s e ma y b e f o u n d . N o d u l e s ma y a l s o b e
p a l p a t e d a l o n g t h e u t e r o s a c r a l l i g a me n t s o n r e c t o v a g i n a l e xa mi n a t i o n .
C. Testing(4)
1. A c o mp l e t e b l o o d c o u n t l o o k i n g f o r a n e mi a o r l e u k o c y t o s i s i s h e l p f u l .
2. I f a b n o r ma l b l e e d i n g i s a s s o c i a t e d w i t h t h e d y s me n o r r h e a , t h y r o i d t e s t i n g a n d a
qualitative serum pregnancy test are indicated.
3. U r i n a l y s i s s h o u l d b e o b t a i n e d t o t e s t f o r h e ma t u r i a . W i t h a n i n d i c a t i o n o f
infection, a urine culture is often helpful.
4. A p e l v i c u l t r a s o u n d ma y b e h e l p f u l i f a n y ma s s e s s e e m a p p a r e n t o n p e l v i c
5. T h e d e f i n i t i v e d i a g n o s i s o f e n d o me t r i o s i s c a n o n l y b e ma d e w i t h l a p a r o s c o p y.
415 / 652
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11.4 - Dysmenorrhea
I V. Diagnosis(1)
D i f f i c u l t me n s t r u a l p e r i o d s o c c u r a t s o me p o i n t f o r mo s t w o me n d u r i n g t h e i r
reproductive years. If it is recurrent and significantly interferes with daily activity o
r e l a t i o n s h i p s , i t w a r r a n t s t r e a t me n t . P r i ma r y d y s me n o r r h e a n o t a s s o c i a t e d w i t h
a b n o r ma l b l e e d i n g c a n o f t e n b e t r e a t e d s u c c e s s f u l l y w i t h n o n s t e r o i d a l a g e n t s o r o r a
contraceptives. If it does not respond to these agents or if it is associated with
a b n o r ma l b l e e d i n g , f u r t h e r d i a g n o s t i c t e s t i n g i s i n d i c a t e d . S e c o n d a r y d y s me n o r r h e a
e i t h e r w i t h o r w i t h o u t a b n o r ma l b l e e d i n g , ma y p o i n t t o a p e l v i c t u mo r, i n f e c t i o n , o r
p r e g n a n c y. F u r t h e r t e s t i n g i s e s s e n t i a l i n t h i s c a s e .
References
1 . J a mi e s o n D J , S t e e g e J F. T h e p r e v a l e n c e o f d y s me n o r r h e a , d y s p a r e u n i a ,
p e l v i c p a i n a n d i r r i t a b l e s y n d r o me i n p r i ma r y c a r e Opbs
r a tcet
t i cG
e sy.nec ol
1996;87:5558.
2 . P r o c t o r M L , F a r q u h a r C M . D y s me n o r r h o e a . C l i n i c a l E v i d e n c e C o n c i s e
2005;14:573576.
3 . A p g a r B S . D y s me n o r r h e a a n d d y s f u n c t i o n a l u t e r i n e Pbrli eme dCi ar
n ge.
1997;24(1):161179.
4 . C h a n P D , W i n k l e CGRy. nec ol ogy and obs t et r i c s 19992000
. Laguna Hills,
C A: C urrent C linical S trategies P ublishers, 1999:2526.
416 / 652
tmdmss
11.5 - Menorrhagia

11 . 5 - M e n o r r h a g i a
11.5
M enorrhagia
Kathry n M. Andolse k
I . Background
M e n o r r h a g i a , d e f i n e d a s h e a v y ( 8 0 mL ) b l e e d i n g f r o m t h e u t e r u s o c c u r r i n g a t
r e g u l a r ( 2 1 3 5 d a y s ) o r l a s t i n g 7 d a y s i n d u r a t i o n , i s c o mmo n . A s s e s s i n g t h e
v o l u me o f b l o o d l o s s h a s l i mi t e d u t i l i t y. I t f a i l s t o d i f f e r e n t i a t e a mo n g e t i o l o g i e s ,
p r e d i c t i r o n s t a t u s , d i r e c t a d i a g n o s t i c s t r a t e g y, o r i n d i c a t1)
e . pPr or ag cn toi sc iasl l (y,
i t i s d i f f i c u l t t o q u a n t i f y t h e a mo u n t o f b l o o d l o s s . D i a g n o s e w h e n a w o ma n
s u b j e c t i v e l y r e p o r t s t h a t me n s t r u a l b l e e d i n g i s h e a v i e r o r l o n g e r t h a n i s t y p i c a l f o r
h e r.
A. Etiology
T h e e t i o l o g y v a r i e s c o n s i d e r a b l y w i t h t h e p a t i e n t ' s a g e , h i s t o r y, a n d p h y s i c a l
f i n d i n g s . P r e g n a n c y a n d i t s c o mp l i c a t i o n s ( e . g . , s p o n t a n e o u s a b o r t i o n , e c t o p i c ,
mi s s e d a b o r t i o n , t r o p h o b l a s t i c d i s e a s e ) a mo n g w o me n i n t h e i r r e p r o d u c t i v e y e a r s
mu s t a l w a y s b e e xc l u d e d ; i f mi s s e d , i t ma y b e l i f e t h r e a t e n i n g . B l e e d i n g a s a s i d e
e f f e c t o f h o r mo n a l c o n t r a c e p t i v e s , i n t r a u t e r i n e d e v i c e s , a n d o t h e r me d i c a t i o n s i s
c o mmo n . O t h e r f r e q u e n t c a u s e s a r e a n o v u l a t i o n , u t e r i n e f i b r o i d s , p o l y p s ,
e n d o me t r i o s i s , a d e n o my o s i s , c o a g u l a t i o n d i s o r d e r s , e n d o c r i n o p a t h i e s , ma l i g n a n c y,
p e l v i c i n f l a mma t o r y d i s e a s e ( P I D ) , a n d l i v e r o r k i d n e2)y . d i s e a s e (
B. Epidemiology
M e n o r r h a g i a i s c o mmo n . I n w o me n 3 0 t o 4 9 y e a r s o f a g e , i t r e p r e s e n t s 5 % o f
g y n e c o l o g i c v i s i t s . Wo r k l o s s f r o m i n c r e a s e d b l o o d f l o w i s e s t i ma t e d a n n u a l l y t o b e
$ 1 , 6 9 2 p e r w o ma n . O n c e r e f e r r e d , w o me n h a v e a n i n c r e a s e d r i s k
P. 2 5 0
o f h y s t e r e c t o my. M e n o r r h a g i a a c c o u n t s f o r t w o - t h i r d s o f a l l h y s t e r e c t o mi e s ; n o
p a t h o l o g y i s i d e n t i f i e d i n a t l e a s t 5 0 % o f r e mo v e d u t e r i .
I I I . Evaluation
A. History
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11.5 - Menorrhagia
1. A m e n s t r u a l a n d r e p r o d u c t i v e h i s t o r y i s n e cAes ks saabroyu t p r e v i o u s
p a t t e r n s o f t h e me n s t r u a l c y c l e ; t h e f i r s t d a y o f t h e l a s t me n s t r u a l p e r i o d a n d t h
f i r s t d a y o f t h e p r e v i o u s me n s t r u a l p e r i o d ; a n d t h e r e g u l a r i t y, d u r a t i o n , a n d
f r e q u e n c y o f a l l b l e e d i n g , i n c l u d i n g a n y i n t e r me n s t r u a l f l o w. D e t e r mi n e i f p a i n i s
p r e s e n t a n d a t t e mp t t o q u a n t i f y t h e n u mb e r o f p a d s o r t a mp o n s p e r p e r i o d .
2. P r e g n a n c y s h o u l d a l w a y s b e c o n s i d e r e d a n d e A
x lcl l cuodnetdr a c e p t i v e
me t h o d s , e v e n p e r ma n e n t o n e s s u c h a s t u b a l s t e r i l i za t i o n , a r e s u b j e c t t o f a i l u r e
w o me n ma y n o t r e v e a l s e xu a l a c t i v i t y.
3. We i g h t c h a n g e , e x c e s s i v e p a t t e r n s , a n x i e t y o r s t r e s s d i s o r d e r s , a s w e l l
a s s y m p t o m s o f s y s t e m i c d i s (eea.sge. , c o a g u l o p a t h y ; t h y r o i d , r e n a l , a n d
hepatic diseases) should be evaluated.
4. M e d i c a t i o n h i s t osr hyo u l d i n c l u d e t h e u s e o f c o n t r a c e3)
p t,i v e s (
anticoagulants, selective serotonin reuptake inhibitors, corticosteroids,
a n t i p s y c h o t i c s , t a mo xi f e n , a n d h e r b a l mo d a l i t i e s s u c h a s g i n s e n g , g i n g k o , a n d
s o y.
5. P r e s e n c e o f e a s y b r u i s i n g o r a n y a b n o r m a l bsl ue cehd iansg w i t h t e e t h
b r u s h i n g o r mi n i ma l t r a u ma ma y i n d i c a t e a b l e e d i n g d i s o r d e r.
6. T h e p r e s e n c e o f m o l i m e n a l s y m p(teo.m
g .s, e d e ma , a b d o mi n a l b l o a t i n g ,
p e l v i c c r a mp i n g , a n d b r e a s t f u l l n e s s ) i s mo r e l i k e l y w i t h o v u l a t o r y c y c l e s ;
h o w e v e r, t h e s e s y mp t o ms a r e n o t r e l i a b l e e n o u g h t o b e t r u l y d i a g n o s t i c .
7. P s y c h o s o c i a l f a c t o r s s h o u l d b e c o n s iOdneer -et dh i r d o f w o me n w i t h
me n o r r h a g i a h a v e me n s t r u a l b l o o d l o s s w i t h i n a n o r ma l r a n g e . A n xi e t y,
u n e mp l o y me n t , a n d a b d o mi n a l p a i n a r e mo r e c o mmo n i n t h e s e w o me n , a n d
t h e s e f a c t o r s ma y h a v e i n f l u e n c e d t h e i r d e c i s i o n t o s e e k h e a l t h c a r e .
8. P r o b a b l e e t i o l o g i e s c a n b e c l a s s i f i e d b4)y a g e (
a. N e o n a t a l p e r i oAdl t h o u g h n o t me n o r r h a g i a i n t h i s a g e - g r o u p , v a g i n a l
b l e e d i n g ma y o c c u r d u r i n g t h e f i r s t f e w d a y s o f l i f e a s t h e i n f a n t
e xp e r i e n c e s a r a p i d d e c r e a s e i n ma t e r n a l - d e r i v e d e s t r o g e n l e v e l s . T h i s i s
g e n e r a l l y t r e a t e d w i t h r e a s s u r a n c e i f n o o t h e r s i g n s o r s y mp t o ms a r e
present.
b. N e o n a t a l p e r i o d t o m e n a rScihmi
e l a r l y, p r e p u b e r t a l b l e e d i n g i s n o t
me n o r r h a g i a b u t s h o u l d b e c a r e f u l l y e v a l u a t e d t o e xc l u d e s e xu a l a b u s e
a n d a s s a u l t , ma l i g n a n c y, s e xu a l l y t r a n s mi t t e d i n f e c t i o n s , a n d t r a u ma .
c. E a r l y m e n a r c hW
e i t h n o mo l i me n a l s y mp t o ms a n d i r r e g u l a r me n s e s ,
a n o v u l a t o r y c y c l e s a r e l i k e l y. A l mo s t a l l n o r ma l a d o l e s c e n t s e xp e r i e n c e
s o me d e g r e e o f me n s t r u a l i r r e g u l a r i t y ; h e a v y p e r i o d s s h o u l d b e e v a l u a t e d .
P r e g n a n c y s h o u l d b e e xc l u d e d i f t h e r e i s a n y q u e s t i o n o f s e xu a l a c t i v i t y.
A b n o r ma l b l e e d i n g ma y a l s o o c c u r a s a s i d e e f f e c t f o r c o n t r a c e p t i v e
h o r mo n a l me t h o d s t a k e n c o r r e c t l y o r i n c o r r e c t l y. F e v e r a n d p e l v i c p a i n c a n
i n d i c a t e P I D . E a s y b r u i s i n g o r b l e e d i n g ma y i n d i c a t e a b l e e d i n g d i s o r d e r.
W i t h n e u r o l o g i c s y mp t o ms s u c h a s b l u r r e d v i s i o n , v i s u a l f i e l d d e f e c t s ,
headache, or the presence of galactorrhea, a pituitary lesion should be
considered.
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11.5 - Menorrhagia
d. L a t e m e n a r c h e t o l a t e t h i rEt xc
i e lsu d e p r e g n a n c y a n d c o n t r a c e p t i v e r e l a t e d c a u s e s . A n o v u l a t i o n i s l e s s c o mmo n . P o l y c y s t i c o v a r i a n s y n d r o me ,
t h e f e ma l e a t h l e t e t r i a d , a n d s t r e s s - i n d u c e d c o n d i t i o n s , s u c h a s e a t i n g
d i s o r d e r s , ma y b e p r e s e n t . O t h e r g y n e c o l o g i c c o n d i t i o n s i n c l u d e
e n d o me t r i o s i s , e n d o me t r i a l h y p e r p l a s i a , e n d o me t r i a l p o l y p s , P I D , a n d
e n d o c r i n o p a t h i e s ( b o t h h y p o - a n d h y p e r t h y r o i d i s m, a s w e l l a s p i t u i t a r y a n d
h y p o t h a l a mi c c o n d i t i o n s ) .
e. L a t e t h i r t i e s a n d o l d
E excr l u d e p r e g n a n c y. I f t h e p a t i e n t i s n o t p r e g n a n t ,
a b n o r ma l b l e e d i n g i n t h i s a g e - g r o u p s h o u l d a r o u s e s u s p i c i o n o f c a n c e r u n t i l
p r o v e d o t h e r w i s e . Wo me n a r e i n c r e a s i n g l y a n o v u l a t o r y a s t h e y a p p r o a c h
t h e p e r i me n o p a u s e . I n q u i r e a b o u t me n o p a u s a l s y mp t o ms , e xo g e n o u s u s e o f
e s t r o g e n s , a n d p e r s o n a l o r f a mi l y h i s t o r y o f g y n e c o l o g i c ma l i g n a n c y o r
genetically linked cancers, such as colon and breast. Other causes include
a d e n o my o s i s .
f. P o s t m e n o p a u s a l p e r iSoede C h a p t e r 11.. 8
P. 2 5 1
9. I n > 5 0 % o f w o m e n w i t h m e n o r r h a g i a , n o e t i o l o g y; it sh ef odui angdn o s i s i s
then dysfunctional uterine bleeding.
1. A s s e s s v i t a l s i g n s a n d t h e p a t i e n t ' s g e n e r a l a p p e a r a n c e , b l o o d p r e s s u r e ,
a n d p u l s eI.f o r t h o s t a t i c c h a n g e s a r e p r e s e n t , e v a l u a t e f o r s y mp t o ms o f s h o c k .
I f p r e s e n t , t h e s e a r e u s u a l l y r e l a t e d t o p r e g n a n c y a n d ma y i n d i c a t e a r u p t u r e d
e c t o p i c p r e g n a n c y ; a l t e r n a t i v e l y, t r a u ma , s e p s i s , o r c a n c e r ma y b e p r e s e n t .
2. P a l l o rw i t h n o r ma l v i t a l s i g n s ma y b e p r e s e n t i f c h r o n i c b l o o d l o s s h a s r e s u l t e d
i n a n e mi a . A n e mi a , i f p r e s e n t , ma y b e d u e t o t h e b l o o d l o s s i t s e l f , b l o o d
d y s c r a s i a , s y s t e mi c c o n d i t i o n s , o r ma l i g n a n c y.
3. F e v e r a n d p e l v i c t e n d e r naer sess u g g e s t i v e o f a c u t e P I D .
4. P h y s i c a l e x a m i n a t i o n i n c l u d e s t h e v u l v a , c e r v i x u t e r u s , a A
nd adne x a.
p e l v i c ma s s ma y i n d i c a t e a b s c e s s , f i b r o i d , e c t o p i c p r e g n a n c y, o r ma l i g n a n c y.
E xc l u d e g e n i t a l t r a u ma .
5. S i g n s o f t h y r o i d d i s e a( es.eg . , r a p i d o r s l o w p u l s e , r e f l e x c h a n g e s , h a i r
c h a n g e s , a n d t h y r o me g a l y ) c a n b e a s s o c i a t e d w i t h me n s t r u a l a b n o r ma l i t i e s .
6. E x c e s s i v e b r u i s i cnagn i n d i c a t e n u t r i t i o n a l d e f i c i e n c y, e a t i n g d i s o r d e r, t r a u ma ,
a b u s e , me d i c a t i o n o v e r u s e , o r a b l e e d i n g d i s o r d e r.
7. J a u n d i c e a n d h e p a t o m e gma
a l y s i g n i f y a n u n d e r l y i n g b l e e d i n g d i s o r d e r.
8. O b e s i t y , h i r s u t i s m , a c n e , a n d a c a n t h o s i s n isgurgi gc ea snts p o l y c y s t i c o v a r y
disease.
9. G a l a c t o r r h ema
a y i n d i c a t e p i t u i t a r y p a t h o l o g y.
1 0. E d e m ama y s i g n i f y r e n a l d i s e a s e .
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11.5 - Menorrhagia
C. Testing
1. L a b o r a t o r y t e s t s
a. A b a s e l i n e c o m p l e t e b l o o d c o u n t a n d s e r u m p r e g n asnhcoyu l dt e bset
o b t a i n e d i n mo s t w o me n .
b. A p l a t e l e t c o u n t , b l e e d i n g t i me , a n d o t h e r t e s t s f o r b l e e d i n g d i s o r d e r s
s h o u l d b e p e r f o r me d a s i n d i c a t e d t o e xc l u d e a b l e e d i n g d i s o r d e r i f n o o t h e r
e t i o l o g y i s r e a d i l y a p p a r e n t . I n h e r i t e d b l e e d i n g d i s o r d e r s o c c u r i n a s ma n y
a s 11 % o f p a t i e n t s w i t h me n o r r h a g i a c o mp a r e d w i t h 3 % o f c o n t r o l w o me n ;
mo s t o f t h e m h a v e v o n W i l l e b r a n d ' s d i s e a s e , w h i c h i s mo r e c o mmo n i n
w h i t e w o me n . E s t a b l i s h i n g t h i s d i a g n o s i s i s i mp o r t a n t , b e c a u s e ma n y o f t h e
d i a g n o s t i c a n d t h e r a p e u t i c o p t i o n s a r e s u r g i c a l a n d ma y p r e s e n t a d d e d r i s k
if an underlying bleeding disorder is present. T he available data do not
s u p p o r t r o u t i n e l y s c r e e n i n g a l l w o me n w i t h me n5)
o r. r h a g i a (
c. S c r e e n i n g f o r s e xu a l l y t r a n s mi t t e d d i s e a s e s a n d t h y r o i d d y s f u n c t i o n s h o u l d
be considered.
d. A P a p a n i c o l a o u s me a r s h o u l d b e p e r f o r me d i f i n d i c a t e d , a l t h o u g h c e r v i c a l
dysplasia rarely causes significant degrees of vaginal bleeding.
e. R e n a l a n d l i v e r f u n c t i o n t e s t s a r e u s e f u l i f t h e s e e t i o l o g i e s a r e s u s p e c t e d .
2. D i a g n o s t i c i m a g i n g
a. A n y n o n p r e g n a n t w o ma n w i t h i r r e g u l a r b l e e d i n g a n d a p e l v i c ma s s r e q u i r e s
c o mp l e t e e v a l u a t i o n w i t h u l t r a s o u n d , c o mp u t e d t o mo g r a p h y ( C T ) s c a n , o r
l a p a r o s c o p y.
b. T r a n s v a g i n a l u l t r a s o u n d d e t e c t s l e i o my o ma , e n d o me t r i a l t h i c k e n i n g , a n d
f o c a l ma s s e s b u t ma y mi s s e n d o me t r i a l p o l y p s a n d s u b mu c o u s f i b r o i d s .
A l t h o u g h h i g h l y s e n s i t i v e f o r e n d o me t r i a l c a r c i n o ma , i t ma y mi s s 4 % mo r e
c a n c e r s t h a n a d i l a t i o n a n d c u r e t t a g e ( D & C ) . A n e n d o me t r i a l s t r i p e o f < 5
mm i s r e a s s u r i n g b u t d o e s n o t c o n c l u s i v e l y e xc l u d e c a n c e r.
c. S a l i n e - i n f u s e d s o n o g r a p h y ( s o n o h y s t e r o g r a p h y ) ( 5 1 0 mL s t e r i l e s a l i n e
i n f u s e d i n t o t h e e n d o me t r i a l c a v i t y ) w i t h u l t r a s o u n d i ma g i n g c a n b e d o n e .
T h e u t i l i t y o f t h i s p r o c e d u r e i s c o mp a r a b l e t o d i a g n o s t i c h y s t e r o s c o p y a n d
i s mo r e a c c u r a t e t h a n t r a n s v a g i n a l u l t r a s o u n d a l o n e . T h e s e n s i t i v i t y i s 9 5 %
t o 9 7 % a n d t h e s p e c i f i c i t y i s 7 0 % t o 9 8 % w h e n c o mb i n e d w i t h e n d o me t r i a l
b i o p s y. A d e c i s i o n a n a l y s i s r e c o mme n d s i t a s t h e p r o c e d u r e o f f i r s t c h o i c e
(6) .
d. M a g n e t i c r e s o n a n c e i ma g i n g ( M R I ) ma y b e u s e f u l f o r a d e n o my o s i s .
P. 2 5 2
3. E n d o m e t r i a l s a m p l iins gr e c o mme n d e d i n w o me n 3 5 y e a r s o f a g e o r t h o s e a t
i n c r e a s e d r i s k o f e n d o me t r i a l c a r c i n o ma . T h i s p r o c e d u r e i s b e s t p e r f o r me d o n
t h e f i r s t d a y o f me n s e s t o a v o i d u n e xp e c t e d p r e g n a n c y.
4. H y s t e r o s c o p y
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11.5 - Menorrhagia
I V. Diagnosis(7)
M e n o r r h a g i a p r e s e n t s mo s t f r e q u e n t l y a t t h e e xt r e me s o f t h e r e p r o d u c t i v e y e a r s ,
d u r i n g me n a r c h e a n d i n p e r i me n o p a u s e . P r e g n a n c y mu s t b e e xc l u d e d . A n y p e l v i c
ma s s mu s t b e e v a l u a t e d w i t h u l t r a s o u n d , C T s c a n , o r M R I . I f n o d i a g n o s i s c a n b e
ma d e , l a p a r o s c o p y o r h y s t e r o s c o p y w i t h s a l i n e i n f u s i o n ma y b e i n d i c a t e d .
References
1 . Wa r n e r P E , C r i t c h l e y H O , L u ms d e n M A , e t a l . M e n o r r h a g i a I I : i s t h e 8 0 mL
b l o o d l o s s c r i t e r i o n u s e f u l i n ma n a g e me n t o f c o mp l a i n t o f me
Am
n o Jr r h a g i a ?
O bs t et G y nec ol
2004;190(5):12241229.
2 . A l b e r s J R , H u l l S K , We s l e y R M . A b n o r ma l u t e r i n e Abm
l e eFdam
ing.
P hy s i c i an2 0 0 4 ; 6 9 : 1 9 1 5 1 9 2 6 .
3 . S c h r a g e r S . A b n o r ma l U t e r i n e B l e e d i n g A s s o c i a t e d w i t h h o r mo n a l
c o n t r a c e p t i oA
n .m F am P hy s i c i an
2002;65(1):20732080.
4 . F i n l e y B , H a r n i s c h D R , C o me r B ,Weomen'
t a l . s geni t our i nar y c ondi t i ons : F P
es s ent i al, sM o n o g r a p h N o , 3 1 4 A A F P H o me S t u d y, L e a w o o d , K a n : A me r i c a n
A c a d e my o f F a mi l y P h y s i c i a n s , 2 0 0 5 : 2 8 4 1 J u l y.
5 . J a me s A , M a t c h a r D B , M y e r s E R . T e s t i n g f o r v o n W i l l e b r a n d d i s e a s e i n
w o me n w i t h me n o r r h a g i a : a s y s t e ma t i c O
r ebs
v iteetw. G y nec ol
2004;104(2):381
388.
6 . D i j k h u i ze n F P H L J , M o l B W J , B o n g e r s M Y, e t a l . C o s t e f f e c t i v e n e s s o f
transvaginal sonography and saline infused sonography in the evaluation of
me n o r r h a g i aI nt
. J G y nec ol O bs t2et
003;83(1):4552.
7 . R o y S N , B h a t t a c h a r y a S . B e n e f i t s a n d r i s k s o f p h a r ma c o l o g i c a l a g e n t s u s e d
f o r t h e t r e a t me n t o f me n o r r hDargug
i a . S af2 0 0 4 ; 2 7 ( 2 ) : 7 5 9 0 .
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11.6 - Nipple Discharge in the Nonpregnant Female

11 . 6 - N i p p l e D i s c h a r g e i n t h e N o n p r e g n a n t F e ma l e
11.6
Nipple Discharge in the Nonpregnant Female
Joy ce A. Cope land
I . Background
N i p p l e d i s c h a r g e i n t h e n o n p r e g n a n t f e ma l e ma y b e o f e i t h e r p h y s i o l o g i c o r
p a t h o l o g i c o r i g i n . G a l a c t o r r h e a i s mi l k p r o d u c t i o n u n r e l a t e d t o p r e g n a n c y. A b n o r ma l
d i s c h a r g e s o t h e r t h a n mi l k ma y o c c u r o w i n g t o a p a t h o l o g i c e t i o l o g y.
A. Etiology
G a l a c t o r r h e a i s t h e r e s u l t o f t h e s t i mu l a t i o n o f t h e p i t u i t a r y g l a n d r e s u l t i n g i n t h e
r e l e a s e o f p r o l a c t i n . P r o l a c t i n i n i t i a t e s t h e p r o d u c t i o n o f mi l k i n t h e l o b u l a r a n d
ductal epithelium of the breast.
1. M e d i c a t i o n s t h a t i n h i b i t d o p a mi n e f u n c t i o n o r t h e me t a b o l i s m o f o t h e r
n e u r o t r a n s mi t t e r s a r e o f t e n a s s o c i a t e d w i t h n i p p l e d i s c h a r g e o n t h e b a s i s o f t h e
i mp a c t o f t h e s u b s t a n c e s o n p r o l a c t i n p r o d u c t i o n . T h e s e p h y s i o l o g i c a l l y b a s e d
a g e n t s a r e mo s t o f t e n a s s o c i a t e d w i t h b i l a t e r a l , mu l t i d u c t a l d i s c h a r g e . C o mmo n
offenders include the following:
a. P h e n o t h i a zi n e s , h a l o p e r i d o l , a n d o t h e r a n t i p s y c h o t i c s
b. T r i c y c l i c a n t i d e p r e s s a n t s , b e n zo d i a ze p i n e s , s e l e c t i v e s e r o t o n i n r e u p t a k e
inhibitors
c. M e t o c l o p r a mi d e , c i me t i d i n e
d. R e s e r p i n e , me t h y l d o p a , d i g i t a l i s , v e r a p a mi l
e. S t e r o i d h o r mo n e s s u c h a s o r a l c o n t r a c e p t i v e s , e s t r o g e n s , P r o g e s t i n s
f. I s o n i a zi d , d a n a zo l
P. 2 5 3
2. S i g n i f i c a n t s t r e s s ma y i n d u c e n i p p l e d i s c h a r g e b y t h e s a me me c h a n i s m a s
me d i c a t i o n .
3. N e u r o l o g i c s y mp t o ms i n a s s o c i a t i o n w i t h b i l a t e r a l d i s c h a r g e a n d a me n o r r h e a
r e q u i r e i n v e s t i g a t i o n f o r p i t u i t a r y a b n o r ma l i t i e s s u c h a s p i t u i t a r y a d e n o ma .
4. N o n p h y s i o l o g i c c a u s e s o f n i p p l e d i s c h a r g e i n v o l v e i n f l a mma t i o n o r f r i c t i o n a s
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w e l l a s ma l i g n a n c y.
B. Epidemiology
B e t w e e n 5 0 % a n d 8 0 % o f w o me n w i l l p r e s e n t w i t h a n i p p l e d i s c h a r g e a t s o me p o i n t
in their reproductive years. T his finding is associated with breast cancer in
a p p r o xi ma t e l y 5 % o f t h e s e w o1)
me
. nR i(s k s o f ma l i g n a n t e t i o l o g y i n c r e a s e w i t h a g e ,
unilateral and uniductal bloody discharges. T he risk of cancer increases after 40
years of age.
I I I . Evaluation
A. History(2)
Initial history should begin with queries regarding the nature of the discharge and
t h e c h a r a c t e r i s t i c s o f t h e p a t i e n t . H o w o l d i s t h e p a t i e n t ? W h a t w a s t h e c i r c u ms t a n c
o f d i s c o v e r y o f t h e d i s c h a r g e i n c l u d i n g s p o n t a n e i t y, a n d t h e p r e s e n c e o f p a l p a b l e
ma s s ? H o w d o e s t h e p a t i e n t d e s c r i b e t h e c h a r a c t e r i s t i c s o f t h e d i s c h a r g e ? A
t h o r o u g h h i s t o r y s h o u l d i n c l u d e p a s t b r e a s t h i s t o r y, c h e s t t r a u ma , i n f e c t i o n , a n d
me d i c a t i o n a n d s u b s t a n c e u s e . F a mi l y h i s t o r y o f b r e a s t c a n c e r i s a l s o c r i t i c a l t o r i s
a s s e s s me n t . I s t h e r e a h i s t o r y o f f r i c t i o n o f t h e n i p p l e ? D o e s t h e p a t i e n t s mo k e ?
1. R e p r o d u c t i v e h i s t oWr yh a t i s t h e p a t i e n t ' s me n s t r u a l s t a t u s ? H a s t h e r e b e e n a
r e c e n t p r e g n a n c y o r a b o r t i o n ? T h i s i n f o r ma t i o n h e l p s i d e n t i f y n o r ma l l a c t a t i o n .
I s t h e p a t i e n t u s i n g h o r mo n a l c o n t r a c e p t i o n ?
2. R e v i e w o f s y s t e mTsh e r e v i e w o f s y s t e ms s h o u l d i n c l u d e q u e r i e s a b o u t
thyroid function, renal and hepatic disease as well as adrenal or pituitary
s y mp t o ms . D o e s t h e p a t i e n t h a v e a h i s t o r y o f h e a d a c h e s , v i s u a l d i s t u r b a n c e ,
a n d a s s o c i a t e d a me n o r r h e a o r me n s t r u a l d i s t u r b a n c e ?
1. C l i n i c a l b r e a s t e x a m i n a t i o n
a. I n s p e c t i o nO b s e r v e t h e s k i n o f t h e b r e a s t f o r e r y t h e ma , c r u s t i n g , o r a r a s h
o n t h e n i p p l e o r a r e o l a r r e g i o n . D o c u me n t t h e c o l o r o f a n y d i s c h a r g e . L o o k
for evidence of nipple retraction. Identify the location of the discharge.
M a g n i f i c a t i o n a n d a d e q u a t e l i g h t i n g ma y a s s i s t i n t h e p r o c e s s o f
l o c a l i za t i o n . L o o k f o r c h e s t w a l l s c a r s , e v i d e n c e o f v i r a l i n f e c t i o n s ( e . g . ,
h e r p e s zo s t e r o r s i mp l e x) , a n d s i g n s o f e c ze ma o r i n f l a mma t i o n .
b. P a l p a t i o nF e e l t h e s k i n f o r w a r mt h . P a l p a t e b o t h b r e a s t s f o r a ma s s o r
t e n d e r n e s s . P a l p a t e r e g i o n a l l y mp h n o d e s f o r e v i d e n c e o f a d e n o p a t h y.
D o c u me n t t h e s i ze , l o c a t i o n , c o n s i s t e n c y, a n d mo b i l i t y o f a n y ma s s .
c. C o m p r e s s i o n
C o mp r e s s t h e b a s e o r t h e a r e o l a r r e g i o n o f t h e b r e a s t w i t h
t h e t h u mb a n d i n d e x f i n g e r t o a t t e mp t e xp r e s s i o n o f t h e d i s c h a r g e i n
q u e s t i o n . A w a r m c o mp r e s s p r i o r t o t h e e xa mi n a t i o n ma y a s s i s t i n t h e
identification of the discharge. Note the location of any discharge as well as
t h e c o l o r o f t h e d i s c h a r g e a n d t h e n u mb e r o f d u c t s i n v o l v e d .
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2. O t h e r e x a m i n a t i o n c o m p o n ePnatl sp a t e t h e t h y r o i d a n d l i v e r i f h i s t o r y
i n d i c a t e s r i s k s o f p a t h o l o g y i n t h e s e o r g a n s . A n e u r o l o g i c e xa mi n a t i o n , i n c l u d i n g
v i s u a l f i e l d s , s h o u l d a c c o mp a n y a n y h i s t o r y o f v i s u a l d i s t u r b a n c e o r h e a d a c h e s .
C. Testing
Clinical history suggestive of endocrine, renal, or hepatic disease should trigger th

e v a l u a t i o n o f a s s o c i a t e d l a b o r a t o r y a s s e s s me n t . S e r u m p r o l a c t i n l e v e l i s i n d i c a t e d
f o r a h i s t o r y o f h e a d a c h e , v i s u a l d i s t u r b a n c e , a n d me n s t r u a l i r r e g u l a r i t y i n t h e
presence of a nipple discharge. Pregnancy testing is also a consideration in the
w o me n o f r e p r o d u c t i v e a g e w i t h me n s t r u a l d i s t u r b a n c e .
1. T h e d i s c h a r gG
e u a i a c t e s t i n g o f t h e d i s c h a r g e s h o u l d b e p e r f o r me d i f f r o m t h e
v i s u a l e xa mi n a t i o n t h e d i s c h a r g e i s n o t o b v i o u s l y b l o o d y o r s e r o s a n g u i n e o u s i n
the unilateral, uniductal presentation. T he role of cytology is controversial, but
t h e p r e s e n c e o f b l o o d i n c r e a s e s t h e p o s i t i v e p r e d i c t i v e v a l u e o f t h i s mo d a l i t y. A
p o s i t i v e c y t o l o g y i s h i g h l y p r e d i c t i v e o f c a n c e r.
2. I m a g i n gM a mmo g r a p h y i s i n d i c a t e d i n a l l w o me n o l d e r t h a n 3 5 y e a r s o f a g e
w i t h a s p o n t a n e o u s n i p p l e d i s c h a r g e . T h e p u r p o s e o f ma mmo g r a p h y i s t o
i d e n t i f y o c c u l t d i s e a s e a n d h e l p i n t h e c h a r a c t e r i za t i o n o f a n y p a l p a b l e ma s s .
T he
P. 2 5 4
r o l e o f d u c t o s c o p y, d u c t o g r a p h y, d u c t a l l a v a g e , a n d ma g n e t i c r e s o n a n c e
i ma g i n g i s u n d e r i n v e s t i g a t i o n . M a mmo g r a p h y i n y o u n g e r w o me n i s l e s s
s e n s i t i v e . U l t r a s o u n d i s mo r e u s e f u l i n w o me n w h o a r e y o u n g e r t h a n 3 5 y e a r s o f
age.
3. P o s s i b l e s u r g e rSyu r g i c a l r e f e r r a l f o r d u c t a l e xp l o r a t i o n o r f u r t h e r e v a l u a t i o n i s
indicated for a patient with a unilateral, uniductal nipple discharge or a nipple
d i s c h a r g e i n t h e p r e s e n c e o f a ma s s , a n d f o r a n y p a t i e n t w i t h p o s i t i v e c y t o l o g y.
D. Genetics
A f a mi l y h i s t o r y o f a p a r e n t o r s i b l i n g w i t h b r e a s t c a n c e r o r a b n o r ma l B R C A 1 / B R C A
g e n e s i n c r e a s e s t h e c h a n c e t h a t a d i s c h a r g e ma y b e ma l i g n a n t . G e n e t i c t e s t i n g i s
not indicated in the evaluation of a nipple discharge.
I V. Diagnosis
T he chief consideration in the differential diagnosis is the distinction between
physiologically and pathologically based etiologies. T he differential diagnosis
i n c l u d e s p r e g n a n c y, p s e u d o d i s c h a r g e , f r i c t i o n o r ma n i p u l a t i o n , s y s t e mi c d i s e a s e ,
p i t u i t a r y d i s e a s e o r s t i mu l a t i o n , c a n c e r, d u c t a l e c t a s i a , i n t r a d u c t a l p a p i l l o ma , P a g e
d i s e a s e , e c ze ma , a n d l o c a l i n f l a mma t i o n s e c o n d a r y t o t r a u ma o r i n f e c t i o n .
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P h y s i o l o g i c d i s c h a r g e s a r e mu l t i d u c t a l , b i l a t e r a l , p a i n l e s s , a n d a s s o c i a t e d w i t h
s t i mu l a t i o n o r me d i c a t i o n . T h i s d i s c h a r g e i s u s u a l l y w h i t e , c l e a r, y e l l o w, o r g r e e n i n
c o l o r. T h e c o n s i s t e n c y i s u s u a l l y mi l k y, b e c a u s e t h i s i s t h e p h y s i o l o g i c r e s p o n s e t o
t h e s t i mu l a t i o n o f t h e p i t u i t a r y, r e s u l t i n g i n g a l a c t o r r h e a .
Pathologic discharges are usually unilateral, uniductal, and spontaneous. T he color

i s v a r i a b l e a n d b l o o d o r p u r u l e n c e ma y b e a p p a r e n t . C a n c e r i s p r e s e n t i n o n e - t h i r d
o f b l o o d y d i s c h a r g e s . T h i s i s e v e n mo r e l i k e l y i f t h e w o ma n i s o l d e r t h a n 4 0 y e a r s o
a g e a n d / o r a ma s s i s p r e s e n t . B e n i g n t u mo r s , i n f e c t i o n s , a n d s y s t e mi c d i s e a s e a r e
also pathologic causes for this type.
1. P s e u d o d i s c h a r g e ma y b e i n d i c a t e d b y t h e p r e s e n c e o f a s t a i n o n t h e b r a o r
b l o u s e , w h i c h ma y b e i n t e r p r e t e d a s a d i s c h a r g e . C o n s i d e r a t i o n s i n t h i s
c i r c u ms t a n c e i n c l u d e e c ze ma , i n f e c t i o n , t r a u ma , o r P a g e t ' s d i s e a s e .
2. M a n i p u l a t i o n o f t h e n i p p l e o r f r i c t i o n ma y b e r e s p o n s i b l e f o r a d i s c h a r g e .
Runners or joggers are candidates for friction-related nipple discharge.
3. D i s c h a r g e a s s o c i a t e d w i t h t h y r o i d d i s e a s e , c h r o n i c r e n a l f a i l u r e , a n d h e p a t i c
f a i l u r e i s s u g g e s t e d b y a p o s i t i v e r e v i e w o f t h e s y s t e ms i n t h e s e a r e a s .
4. H e a d a c h e a n d v i s u a l d i s t u r b a n c e w i t h a d i s c h a r g e s u g g e s t s t h e p o s s i b i l i t y o f
pituitary disease.
I n t r a d u c t a l p a p i l l o ma i s t h e mo s t c o mmo n c a u s e o f b e n i g n p a t h o l o g i c
discharges. It is associated with a straw-colored or clear transparent discharge
5. D u c t a l e c t a s i a i s t h e r e s u l t o f a p r o g r e s s i o n o f d u c t a l s t a g n a t i o n a n d r e s u l t a n t
i n f l a mma t o r y p r o c e s s . T h e i n c i d e n c e o f t h i s d i s o r d e r i s h i g h e r i n s mo k e r s a n d i s
mo s t p r o mi n e n t i n w o me n i n t h e 4 0 - t o 6 0 - y e a r - o l d a g e - g r o u p . I n d u r a t i o n a n d
n o n c y c l i c b u r n i n g p a i n i s c h a r a c t e r i s t i c o f t h i s d i s o r d e r.
6. P a g e t ' s d i s e a s e i n v o l v e s t h e s k i n o f t h e n i p p l e a n d a r e o l a . I t i s u s u a l l y
a s s o c i a t e d w i t h d u c t a l c a r c i n o ma . A n y a r e o l a r l e s i o n t h a t d o e s n o t r e s p o n d t o
a n t i b i o t i c s o r t o p i c a l t r e a t me n t mu s t b e b i o p s i e d t o e xc l u d e t h i s d i s o r d e r.
References
1 . S h i r l e y R . N i p p l e D i s c h a r g e . U p t o D a t e 2 0 0 5 ; Ve r s i o n 1 3 . 1 ;
w w w. u p t o d a t e . c o m.
2 . A n d o l s e k K , C o p e l a n d J . I n : T a y l o r CBondi
, e dt.i ons of t he br eas t i n f ami l y
medi c i ne: pr i nc i pl es and pr ,ac5ttihc ee d . N e w Yo r k : S p r i n g e r - Ve r l a g , 1 9 9 8 .
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11.7 - Pap Smear Abnormality

11 . 7 - P a p S me a r A b n o r ma l i t y
11.7
Pap Smear Abnormality
Fide l A. Vale a
I . Background
T h e i n c i d e n c e o f c e r v i c a l c a n c e r, o n c e t h e l e a d i n g c a u s e o f c a n c e r d e a t h s i n
w o me n , h a s s t e a d i l y d e c r e a s e d i n t h e U n i t e d S t a t e s s i n c e t h e i n c e p t i o n o f ma s s
s c r e e n i n g w i t h c e r v i c a l c y t o l o g y ( t h e P a p a n i c o l a o u [ P a p ] s me a r ) . A p p r o xi ma t e l y 5 0
mi l l i o n P a p s me a r s a r e p e r f o r me d i n t h e U n i t e d S t a t e s a n n u a l l y, a n d a p p r o xi ma t e l y
5 % r e v e a l a b n o r ma l i t i e s . A l t h o u g h mo s t o f t h e a b n o r ma l i t i e s a r e l o w g r a d e a n d w i l l
l i k e l y r e g r e s s , a f e w c a n p r o g r e s s t o c a n c e r ; t h e r e f o r e e v e n l o w - g r a d e a b n o r ma l i t i e
should be evaluated.
A. Etiology
1. T h e c e r v i x, b e c a u s e o f i t s l o c a t i o n i n t h e v a g i n a , i s v e r y s u s c e p t i b l e t o e xp o s u r
t o e n v i r o n me n t a l f a c t o r s . T h e s q u a mo c o l u mn a r j u n c t i o n , o r t r a n s f o r ma t i o n zo n e ,
is the part of the cervix with the greatest likelihood for neoplastic change
b e c a u s e i t i s t h e j u n c t i o n w h e r e t h e c e r v i c a l g l a n d u l a r ( c o l u mn a r ) e p i t h e l i u m
me e t s t h e n a t i v e s q u a mo u s e p i t h e l i u m o f t h e e c t o c e r v i x. T h e e xp o s e d c o l u mn a r
e p i t h e l i u m u n d e r g o e s me t a p l a s t i c c h a n g e a n d i s r e p l a c e d b y s q u a mo u s
e p i t h e l i u m i n t h e t r a n s f o r ma t i o n zo n e . B e c a u s e o f t h e c o n s t a n t i n f l a mma t i o n ,
d a ma g e , a n d r e p a i r t h a t o c c u r i n a r e a s o f me t a p l a s i a , i t i s p a r t i c u l a r l y
susceptible to the incorporation of viruses and the effects of other carcinogens.
2. T h e h u ma n p a p i l l o ma v i r u s ( H P V ) i s a d o u b l e - s t r a n d e d d e o xy r i b o n u c l e i c a c i d
( D N A) virus that replicates within epithelial cells at specific sites and has been
linked to cervical intraepithelial neoplasia, or dysplasia, as well as cervical
c a n c e r. E p i t h e l i a l c e l l s b e c o me i n f e c t e d w i t h H P V a t t h e b a s a l a n d p a r a b a s a l
l a y e r s . T r a u ma f r o m i n t e r c o u r s e , f o r e xa mp l e , c a n a b r a d e t h e s u r f a c e e p i t h e l i u m
a n d e xp o s e t h e b a s a l c e l l s . T h e k o i l o c y t e s ( c e l l s i n f e c t e d w i t h H P V ) t h a t
develop are present in the superficial layers of the epithelium and further traum
i n c r e a s e s s h e d d i n g o f t h e s e c e l l s a n d p r o mo t e s t r a n s mi s s i o n . A f t e r e xp o s u r e ,
t h e i n c u b a t i o n p e r i o d c a n b e a n y w h e r e f r o m 1 t o 8 mo n t h s . T h e f i r s t l i n e o f h o s t
r e s p o n s e i s t o s l o w v i r a l r e p l i c a t i o n a n d i n d u c e a c e l l - me d i a t e d r e s p o n s e t o
c o n t a i n t h e v i r u s . I n mo s t h e a l t h y i n d i v i d u a l s w i t h n o r ma l i mmu n e r e s p o n s e , t h e
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viral infection is transient with an average duration of viral shedding after a new
H P V i n f e c t i o n o f 8 mo n1,2)
t h s . ( C o n v e r s e l y, H P V c a n e v a d e t h e h o s t i mmu n e
response because it is an intracellular infection and is not readily identified as
a n a n t i g e n . T h e p h e n o t y p i c e xp r e s s i o n o f a n H P V i n f e c t i o n a l s o d e p e n d s o n t h e
v i r a l t y p e . O f t h e > 1 0 0 t y p e s o f H P V, > 3 0 i n f e c t t h e l o w e r g e n i t a l t r a c t . T h e s e
s e xu a l l y t r a n s mi t t e d t y p e s o f H P V h a v e g e n e r a l l y b e e n d i v i d e d i n t o t w o g r o u p s :
l o w o n c o g e n i c p o t e n t i a l a n d h i g h o n c o g e n i c pToatbelnet i11
a l . 7 .s1h o w s t h e
P. 2 5 6
breakdown of the various types. T he low oncogenic potential viral types are
a s s o c i a t e d w i t h c o n d y l o ma a n d l o w - g r a d e c e r v i c a l a b n o r ma l i t i e s , w h e r e a s t h e
high oncogenic potential viral types are associated high-grade cervical dysplasi
a n d c a n c e r. H P V i s a n e c e s s a r y b u t i n s u f f i c i e n t p r e c u r s o r o f s q u a mo u s c e l l
c a r c i n o ma o f t h e c e r v i x. H o s t f a c t o r s s u c h a s a g e , n u t r i t i o n a l s t a t u s , i mmu n e
f u n c t i o n , s mo k i n g , a n d p o s s i b l y s i l e n t g e n e t i c p o l y mo r p h i s ms mo d u l a t e t h e
incorporation of viral D N A into host cervical cells.
TAB L E 11.7.1 Hum an Papillom a Virus Types

According to the Oncogenic Potential
Oncoge nic
pote ntial
Virus ty pe
Low risk
6 , 11 , 4 0 , 4 2 4 4 , 5 3 , 5 4 , 6 1 , 7 2 , 7 3 , 8 1
High risk
16, 18, 31, 33, 35, 39, 45, 51, 52, 56, 58, 59, 68,
82
TAB L E 11.7.2 Natural History of Cervical

Dysplasia
Progre ssion Progre ssion
Re gre ssionPe rsiste nce to se v e re
to cance r
(%)
(%)
dy splasia (% )
(%)
Mild
57
dysplasia
32
11
Moderate 43
dysplasia
35
22
Severe
~56
12
32
427 / 652
tmdmss
dysplasia
B. Epidemiology
1. H P V i s o n e o f t h e mo s t c o mmo n s e xu a l l y t r a n s mi t t e d i n f e c t i o n s i n t h e U n i t e d
S t a t e s , w i t h a n e s t i ma t e d p r e v a l e n c e o f 1 5 % , l e a d i n g t o a p p r o xi ma t e l y 2 t o 5
mi l l i o n a b n o r ma l P a p s me a r s i n t h e U n i t e d S t a t e s a n n u a l l y. U s i n g mo d e r n H P V
d e t e c t i o n me t h o d s , a p p r o xi ma t e l y 9 5 % t o 1 0 0 % o f s q u a mo u s c e l l c e r v i c a l
cancers and 75% to 95% of high-grade cervical intraepithelial neoplasia lesions
have detectable H P V D N A. In the U nited S tates, peak incidence and prevalence
o f H P V i n f e c t i o n o c c u r a mo n g w o me n y o u n g e r t h a n 2 5 y e a r s o f a g e , b u t mo s t
i n f e c t i o n s i n y o u n g e r w o me n a r e t r a n s i e n t . I n o n e s t u d y, 2 5 % o f c o l l e g e
f r e s h me n w e r e a l r e a d y e xp o s e d t o H P V. O f t h e f r e s h me n w h o w e r e n e g a t i v e
w h e n t h e y e n r o l l e d , 4 2 % h a d e v i d e n c e o f e xp o s u r e o v e r t h e n e xt 3 y e a r s ,
l e a d i n g a p r e v a l e n c e o f >2)
6 0. %I n( f e c t i o n s w i t h H P V i n o l d e r w o me n a r e mu c h
less prevalent but carry a higher risk of progressing to cervical neoplasia.
A l t h o u g h t h e p r e v a l e n c e o f H P V i n f e c t i o n i s h i g h e r a mo n g i mmu n o c o mp r o mi s e d
w o me n s u c h a s t h o s e w i t h h u ma n i mmu n o d e f i c i e n c y v i r u s , t h e s p e e d o f
progression to cervical cancer is not increased. Natural history studies confirm
t h a t , i n mo s t c a s e s , t h e c o u r s e o f i n f e c t i o n a n d c e r v i c a l a b n o r ma l i t i e s t h a t
p r o g r e s s d o s o i n a n o r d e r l y f a s h i o n f r o m l e s s s e v e r e t o mo r e s e v e r e l e s i o n s ,
ma k i n g i t a n i d e a l c o n d i t i o n f o r s c r e3)e.n Ti nhgi s( i s d e mo n s t r a t eTda ibnl e
11 . 7 . 2.
2. R i s k f a c t o r s f o r c e r v i c a l n e o p l a s i a i n c l u d e n o t o n l y H P V b u t a l s o e a r l y o n s e t o f
i n t e r c o u r s e , mu l t i p l e s e xu a l p a r t n e r s , y o u n g a g e , ma l e f a c t o r s ( h i g h - r i s k
p a r t n e r ) , l o w s o c i o e c o n o mi c s t a t u s , r a c e , h i s t o r y o f o t h e r s e xu a l l y t r a n s mi t t e d
d i s e a s e s , c o mp r o mi s e d i mmu n i t y, c i g a r e t t e s mo k i n g , o r a l c o n t r a c e p t i v e u s e , a n d
e v e n h i s t o r y o f d i e t h y l s t i l b e s t r o l e xp o s u r e .
3. T h e A me r i c a n C a n c e r S o c i e t y e s t i ma t e s t h a t t h e r e w i l l b e a p p r o xi ma t e l y 1 0 , 3 7 0
new cases of invasive cervical cancer and 3,710 deaths in the United States in
2 0 0 5 4)
( . I t i s n o w t h e 1 3 t h mo s t c o mmo n c a n c e r i n w o me n i n t h e U n i t e d S t a t e s .
P a p s me a r s c o n t i n u e t o b e o n e o f t h e f e w i n t e r v e n t i o n s a w a r d e d a n A
r e c o mme n d a t i o n f r o m t h e U . S . P r e v e n t i v e S e r v i c e s T a5)s.k F o r c e (
I I I . Evaluation
A. History
M o s t p a t i e n t s w i t h c e r v i c a l d y s p l a s i a a r e a s y mp t o ma t i c . T h e y c a n a l s o p r e s e n t w i t h
e v i d e n c e o f e xt e r n a l c o n d y l o ma , v a g i n a l d i s c h a r g e , o r e v e n v a g i n a l b l e e d i n g . F o r
mo s t , t h e i n i t i a l d i s c o v e r y o f c e r v i c a l n e o p l a s i a i s u s u a l l y a s a n a b n o r ma l i t y i n t h e
P a p s me a r. T h e g y n e c o l o g i c h i s t o r y, w h i c h i s e s s e n t i a l i n d e t e r mi n i n g a p a t i e n t ' s
risk, should be shared with the cytopathologist.
P. 2 5 7
428 / 652
tmdmss
1. O n p h y s i c a l e xa mi n a t i o n , t h e c e r v i x u s u a l l y a p p e a r s n o r ma l t o t h e n a k e d e y e .
G r o s s c e r v i c a l l e s i o n s s h o u l d n o t b e e v a l u a t e d w i t h a P a p s me a r ; i n s t e a d , t h e y
should be biopsied to confirm the diagnosis.
2. T h e r e a r e t w o g e n e r a l t e c h n i q u e s t o o b t a i n a c y t o l o g i c s p e c i me n f r o m t h e
c e r v i x. T h e t r a d i t i o n a l me t h o d u s e s a s p a t u l a t o g e n t l y s c r a p e t h e e c t o c e r v i x a n
t h e t r a n s f o r ma t i o n zo n e a n d a c y t o b r u s h , o r a s i mi l a r a p p a r a t u s , t o s a mp l e t h e
e n d o c e r v i x. B o t h t h e s e s p e c i me n s a r e s me a r e d e v e n l y o n a g l a s s s l i d e a n d
f i xe d i mme d i a t e l y. I t i s i mp o r t a n t t o n o t a l l o w t h e s l i d e s t o d r y b e f o r e f i xa t i o n . A
mo r e c o n t e mp o r a r y me t h o d i s t o u s e a l i q u i d - b a s e d s y s t e m. T h e c e r v i x a n d
e n d o c e r v i x a r e s a mp l e d w i t h a p l a s t i c b r o o m o r a s i mi l a r a p p a r a t u s a n d t h e
s p e c i me n i s s u s p e n d e d i n a b o t t l e c o n t a i n i n g t h e f i xa t i v e a n d t r a n s p o r t e d a s a
suspension. Both are acceptable, although the latter allows for additional H P V
D N A testing.
3. T h e B e t h e s d a S y s t e m w a s d e v e l o p e d i n 1 9 8 8 a n d r e v i s e d i n 1 9 9 1 a n d a g a i n i n
2 0 0 1 b y t h e N a t i o n a l C a n c e r I n s t i t u t e t o p r o v i d e u n i f o r m t e r mi n o l o g y f o r c e r v i c a
c y t o p a t h o l o g y r e p o r t i n g T( saebel e 11 . 7). .3 I n a l l r e p o r t s , s p e c i f i c me n t i o n
s h o u l d b e ma d e a b o u t t h e s p e c i me n t y p e , a d e q u a c y o f t h e s p e c i me n , a g e n e r a l
c a t e g o r i za t i o n o f t h e r e s u l t s , h o w t h e s p e c i me n w a s e v a l u a t e d , i f a n y o t h e r
a n c i l l a r y t e s t i n g w a s p e r f o r me d , a s w e l l a s a f i n a l i n t e r p r e t a t i o n o f f i n d i n g s a n d
r e c o mme n d a t i o n s . T h e e v a l u a t i o n o f e a c h a b n o r ma l i t y s h o u l d b e i n d i v i d u a l i ze d
o n t h e b a s i s o f ma n y f a c t o r s i n c l u d i n g a g e , me d i c a l h i s t o r y, r e p r o d u c t i v e p l a n s ,
and the presence of risk factors.
4. T h e A me r i c a n C a n c e r S o c i e t y g u i d e l i n e s f o r c e r v i c a l c a n c e r s c r e e n i n g w e r e l a s t
u p d a t e d i n 2 0 06)
2 .( T h e y r e f l e c t t h e c u r r e n t u n d e r s t a n d i n g o f t h e
p a t h o p h y s i o l o g y a n d e p i d e mi o l o g y o f c e r v i c a l n e o p l a s i a . T h e y r e c o mme n d t h a t
c e r v i c a l c a n c e r s c r e e n i n g s h o u l d c o mme n c e a p p r o xi ma t e l y 3 y e a r s a f t e r t h e
onset of vaginal intercourse, but no later than 21 years of age. Screening
s h o u l d b e p e r f o r me d a n n u a l l y u n t i l 3 0 y e a r s o f a g e , w i t h c o n v e n t i o n a l c y t o l o g i c
s me a r s o r e v e r y 2 y e a r s u s i n g l i q u i d - b a s e d c y t o l o g y. A f t e r 3 0 y e a r s o f a g e ,
w o me n w h o h a v e h a d t h r e e c o n s e c u t i v e , t e c h n i c a l l y s a t i s f a c t o r y n o r ma l r e s u l t s
ma y u n d e r g o s c r e e n i n g e v e r y 2 t o 3 y e a r s u s i n g e i t h e r t e c h n o l o g y o r c a n
u n d e r g o t e s t i n g f o r h i g h - r i s k H P V D N A , i n a d d i t i o n t o c y t o l o g y, e v e r y 3 y e a r s .
T h e A me r i c a n C a n c e r S o c i e t y d i d n o t r e c o mme n d s c r e e n i n g w o me n o l d e r t h a n
7 0 y e a r s o f a g e w i t h t h r e e c o n s e c u t i v e n o r ma l s me a r s a n d n o h i s t o r y o f
a b n o r ma l P a p s me a r s o r w o me n w h o h a v e h a d t o t a l h y s t e r e c t o mi e s .
C. Testing
T h e e v a l u a t i o n o f a p a t i e n t w i t h a n a b n o r ma l P a p s me a r b e g i n s w i t h t h e
u n d e r s t a n d i n g t h a t a P a p s me a r i s a s c r e e n i n g t e s t , n o t a d i a g n o s t i c t e s t . T h e
c o r r e l a t i o n b e t w e e n P a p s me a r a n d h i s t o l o g i c d i a g n o s i s i s v a r i a b l e . I n o n e s e r i e s ,
t h e mo s t c o mmo n P a p s me a r r e p o r t p r i o r t o a h i s t o l o g i c d i a g n o s i s o f s e v e r e
d y s p l a s i a w a s a t y p i c a l s q u a mo u s c e l l s o f u n d e t e r mi n e d s i g n i f i c a n7)c .e ( A S C U S ) (
S i mi l a r l y, i n a l a r g e n a t i o n a l t r i a l , < 3 0 % o f p a t i e n t s w i t h a n A S C U S P a p s me a r w e r e
429 / 652
tmdmss
f o u n d t o h a v e p a t h o l o8)g .y T( h e s e e xa mp l e s r e v e a l t h e i mp o r t a n c e o f e s t a b l i s h i n g a
h i s t o l o g i c d i a g n o s i s . H o w e v e r, t h e e v a l u a t i o n o f a n a b n o r ma l P a p s me a r ma y
c o mme n c e w i t h s i mp l y r e p e a t i n g t h e t e s t , d e p e n d i n g o n t h e d e g r e e o f a b n o r ma l i t y
and the patient's age.
1. Ve r y mi n i ma l a b n o r ma l i t i e s s u c h a s A S C U S i n y o u n g e r p a t i e n t s ( y o u n g e r t h a n
3 0 y e a r s o f a g e ) c a n b e f o l l o w e d s i mp l y b y r e p e a t i n g ( o r c o n f i r mi n g ) t h e P a p
s me a r b e f o r e f u r t h e r t e s t i n g . H P V D N A t e s t i n g ma y b e u s e d t o h e l p t r i a g e t h e s e
p a t i e n t s ; u n f o r t u n a t e l y, b e c a u s e o f t h e h i g h p r e v a l e n c e o f H P V i n f e c t i o n s i n t h i s
y o u n g a g e - g r o u p , t h e u s e f u l n e s s o f H P V D N A t y p i n g i n t h i s s e t t i n g i s l i mi t e d .
H o w e v e r, i n o l d e r p a t i e n t s ( o l d e r t h a n 3 0 y e a r s o f a g e ) , i t i s a v e r y h e l p f u l t e s t
to help triage patients according to the presence or absence of high-risk H P V
P. 2 5 8
P. 2 5 9
D N A 8( ) . I n a l a n d ma r k t r i a l , t h e A S C U S / l o w - g r a d e s q u a mo u s i n t r a e p i t h e l i a l
l e s i o n ( L G S I L ) T r i a g e S t u d y d e mo n s t r a t e d t h a t H P V D N A t y p i n g f o r t h e h i g h risk strains was useful in the triage of patients older than 30 years of age with
a n A S C U S P a p s me a r. P a t i e n t s o l d e r t h a n 3 0 y e a r s o f a g e w i t h h i g h - r i s k H P V
D N A s h o u l d p r o c e e d w i t h d i a g n o s t i c t e s t s , w h e r e a s t h e s i mi l a r p a t i e n t w i t h n o
h i g h - r i s k H P V D N A c a n s i mp l y b e o b s e r v e d a n d r e s c r e e n e d a t a l a t e r d a t e .
S creening for the low-risk strains of H P V has no usefulness in this setting. In
a d d i t i o n , p a t i e n t s w i t h P a p s me a r s s h o w i n g l o w - g r a d e c h a n g e s ( L G S I L s ) o r
worse did not benefit at all from H P V typing and should proceed with diagnostic
tests.
TAB L E 11.7.3 Bethesda 2001 Term inology for the

Reporting of Cervical Cytopathology
Spe cim e n ty pe
I ndi c at e c onv ent i onal s mear ( P ap s mear ) v s . l i qui d bas ed v s . ot her
Spe cim e n ade quacy
S a t i s f a c t o r y f o r e v a l u a des
t i o nc (r i be pr es enc e or abs enc e of
endoc er v i c al / t r ans f or mat i on zone c omponent and any ot her qual i t y
i ndi c at or s , e. g. , par t i al l y obs c ur i ng bl ood, i nf l ammat
) i on, et c .
U n s a t i s f a c t o r y f o r e v a l u a t i osnpec
i (f y r eas )on
S p e c i me n r e j e c t e d / n o t p r o c e s spec
e d i (f y r eas )on
S p e c i me n p r o c e s s e d a n d e xa mi n e d , b u t u n s a t i s f a c t o r y f o r
e v a l u a t i o n o f e p i t h e l i a l a b n o r ma l i t y b e c sapec
u s ei foyf r(eas )on
G e n e r a l c a t e g o r i z a t (iopt
o n i onal
)
N e g a t i v e f o r i n t r a e p i t h e l i a l l e s i o n o r ma l i g n a n c y
E p i t h e l i a l c e l l a b n o r ma l i t y : s e e i n t e r p r e t a t i os pec
n / r ei sf yu l t (
s quamous or gl andul ar as appr)opr i at e
O t h e r : s e e i n t e r p r e t a t i o n / r ee.s g.
u l ,t endomet
(
r i al c el l s i n a woman
o v e r 40 y of age
)
Autom ate d re v ie w
430 / 652
tmdmss
I f c as e ex ami ned by aut omat ed dev i c e, s pec i f y dev i c e and r es ul t

Ancillary te sting
P r ov i de a br i ef des c r i pt i on of t he t es t met hods and r epor t t he r es ul t s o
t hat i t i s eas i l y under s t ood by t he c l i ni c i an
I nte rpre tation/re sult
Ne gativ e for intrae pithe lial le sion or m alignancy
( when t her e i s no c el l ul ar ev i denc e of neopl as i a, r ec or d i n t he G ener al
C at egor i zat i on s ec t i on and/ or i n t he I nt er pr et at i on/ R es ul t s ec t i on of t he
r epor t , whet her t her e ar e or gani s ms or ot her non- neopl as t i c f i ndi ngs )
O r g a n i s ms
Tr i c homonas v agi nal i s
F u n g a l o r g a n i s ms mo r p h o l o g i c a l l y c o n s i s tCe andi
n t w idas
t h pec i es
Shift in flora suggestive of bacterial vaginosis
B a c t e r i a mo r p h o l o g i c a l l y c o n s i s t e A
n tc twi nomy
i t h c es s pec i es
C e l l u l a r c h a n g e s c o n s i s t e n t w i t h h e r p e s s i mp l e x v i r u s
O t h e r n o n - n e o p l a s t i c f i n d opt
i n g si onal
(
t o r epor t ; l i s t not i nc) l us i v e
Reactive cellular changes associated with radiation, intrauterine
contraceptive device
G l a n d u l a r c e l l s s t a t u s p o s t h y s t e r e c t o my
Atrophy
Other
E n d o me t r i a l c e l il ns a
( womano v e r40 y of age) ( S pec i f y i f
negat i v e f or s quamous i nt r aepi t hel i al) l es i on
Epithe lial ce ll abnorm alitie s
Squam ous ce ll
A t y p i c a l s q u a mo u s c e l l s o f u n d e t e r mi n e d s i g n i f i c a n c e c a n n o t
e xc l u d e H G S I L ( A S C - H )
L o w - g r a d e s q u a mo u s i n t r a e p i t h e l i a l l e s i o n e n c o mp a s s i n g : H P V / mi l d
dysplasia/C IN 1
H G S I L e n c o mp a s s i n g : mo d e r a t e a n d s e v e r e d y s p l a s i a , c a r c i n o ma i n
s i t u / C I N 2 a n d C I N 3 w i t h f e a t u r e s s u s p i c i o u s f o ri fi n v a s i o n (
i nv as i on i s s us pec) t ed
S q u a mo u s c e l l c a r c i n o ma
Glandular cell
Atypical
E n d o c e r v i c a l c e l l s ( Nor
O Ss pec i f y i n c omment
) s
E n d o me t r i a l c e l l s ( Nor
) s
G l a n d u l a r c e l l s ( N or
) s
Atypical
Endocervical cells, favor neoplastic
Glandular cells, favor neoplastic
E n d o c e r v i c a l a d e n o c a r c i ni onma
si tu
A d e n o c a r c i n o ma
Endocervical
E n d o me t r i a l
E xt r a u t e r i n e
431 / 652
tmdmss
NOS
O t h e r m a l i g n a n t n e o p l a(ssm
pec
s i f )y
E d u c a t i o n a l n o t e s a n d s u g g e s( opt
t i o ni onal
s )
Suggestions should be concise and consistent with clinical follow-up
g u i d e l i n e s p u b l i s h e d b y p r o f e s s i o n a l o r g a n i za t i o n s ( r e f e r e n c e s t o
r e l e v a n t p u b l i c a t i o n s ma y b e i n c l u d e d )
H G S I L , h i g h - g r a d e s q u a mo u s i n t r a e p i t h e l i a l l e s i o n ; C I N , c e r v i c a l
intraepithelial neoplasia; N O S, not otherwise specified, AS C-H, atypical
s q u a mo u s c e l l s - c a n n o t e xc l u d e h i g h g r a d e l e s i o n .
2. A l t h o u g h p o s t me n o p a u s a l p a t i e n t s w i t h A S C U S P a p s me a r s w e r e n o t
s p e c i f i c a l l y e v a l u a t e d , t h e y ma y b e t r e a t e d w i t h e s t r o g e n v a g i n a l c r e a m i f t h e
c h a n g e s a r e b e l i e v e d t o b e c a u s e d b y a t r o p h y. A f t e r a s h o r t c o u r s e o f t r e a t me n t
t h e P a p s me a r s h o u l d b e r e p e a t e d a n d a n y s u b s e q u e n t a b n o r ma l i t y, e v e n i f
mi n i ma l , s h o u l d b e e v a l u a t e d f u r t h e r w i t h d i a g n o s t i c t e s t s .
3. T h e r e i s n o c u r r e n t r o l e f o r t e s t i n g f o r l o w - r i s k H P V D N A , r e g a r d l e s s o f t h e
patient's age.
I V. Diagnosis
T h e d i a g n o s i s o f c e r v i c a l d y s p l a s i a , o r c a n c e r, s h o u l d b e ma d e o n l y h i s t o l o g i c a l l y.
C o l p o s c o p y u s e s ma g n i f i c a t i o n t o i d e n t i f y t h e a b n o r ma l a r e a s o f t h e c e r v i x. A f t e r
e xc e s s mu c u s h a s b e e n g e n t l y w i p e d a w a y a n d a P a p s me a r p e r f o r me d , t h e c e r v i x,
p a r t i c u l a r l y t h e t r a n s f o r ma t i o n zo n e , i s mo i s t e n e d w i t h a s o l u t i o n o f 3 % t o 5 % a c e t i
a c i d , a l l o w i n g f o r e a s i e r v i s u a l i za t i o n o f t h e a b n o r ma l a r e a s ( p u n c t a t i o n , w h i t e
e p i t h e l i u m, mo s a i c i s m, a n d a t y p i c a l v e s s e l s ) a n d d i r e c t i n g t h e c l i n i c i a n t o t h e
s p e c i f i c a r e a s t h a t s h o u l d b e b i o p s i e d . T h e e xa mi n a t i o n i s c o n s i d e r e d s a t i s f a c t o r y
o n l y i f t h e e n t i r e t r a n s f o r ma t i o n zo n e c a n b e v i s u a l i ze d c o l p o s c o p i c a l l y a n d t h e mo s
a b n o r ma l a r e a s c a n b e b i o p s i e d . A n e n d o c e r v i c a l c u r e t t a g e i s f r e q u e n t l y p e r f o r me d
in a blind fashion to evaluate the cells in the endocervical canal that cannot be see
r e a d i l y. I f c o l p o s c o p y i s i n a d e q u a t e , t h e r e i s a n a b n o r ma l i t y i n t h e e n d o c e r v i c a l
c a n a l , o r t h e r e i s a s u s p i c i o n o f mi c r o i n v a s i v e c a n c e r, f u r t h e r d i a g n o s t i c t e s t i n g
s h o u l d i n c l u d e e i t h e r a c o n i za t i o n o f t h e c e r v i x o r a l o o p e l e c t r o s u r g i c a l e xc i s i o n
p r o c e d u r e ( L E E P ) t o c o mp l e t e t h e w o r k u p . B e c a u s e o f t h e l e s s - t h a n - p e r f e c t
c o r r e l a t i o n b e t w e e n P a p t e s t r e s u l t s a n d h i s t o l o g i c d i a g n o s i s , i t i s i mp e r a t i v e t h a t a
d i a g n o s i s b e e s t a b l i s h e d h i s t o l o g i c a l l y a n d n o t me r e l y b y t h e P a p t e s t . T h e
differential diagnosis includes the following:
A. Ascus
A l t h o u g h t h i s ma y b e t h e l o w e s t g r a d e o f a b n o r ma l i t y, a p p r o xi ma t e l y 3 0 % o f p a t i e n t
w i t h a n A S C U S P a p s me a r
P. 2 6 0
h a v e d y s p l a s i a o r s i g n i f i c a n t p a t h o l o g y. T h e p a t i e n t y o u n g e r t h a n 3 0 y e a r s o f a g e
c a n s i mp l y b e f o l l o w e d w i t h a r e p e a t P a p s me a r i n 4 t o 6 mo n t h s o r p r o c e e d t o
diagnostic colposcopy and biopsies, depending on the clinical situation. T he patien
432 / 652
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older than 30 years of age can be triaged according to the high-risk H P V D N A

s t a t u s . I f p r e s e n t , s h e s h o u l d u n d e r g o c o l p o s c o p y. I f h i g h - r i s k H P V D N A i s n o t
p r e s e n t , s h e c a n b e f o l l o w e d w i t h a r e p e a t P a p t e s t i n 1 y e a r.
B.
A t y p i c a l s q u a mo u s c e l l s ( c a n n o t e xc l u d e h i g h - g r a d e s q u a mo u s i n t r a e p i t h e l i a l l e s i o n
[AS C-H]).
T his is a new category in the Bethesda reporting system of 2001. Because 30% to
40% of patients with AS C-H have high-grade dysplasia and a very high prevalence
o f h i g h - r i s k H P V D N A ( a p p r o a c h i n g 8 5 % ) , mo r e s c r e e n i n g t e s t s a r e n o t
r e c o mme n d e d . T h e y s h o u l d a l l h a v e c o l p o s c o p i c e v a l u a t i o n a n d b i o p s i e s .
C.
Atypical glandular cells ( AG C ) (previously called atypical glandular cells of uncerta

significance [AG U S]).
T h i s r e s u l t r e q u i r e s f u r t h e r e v a l u a t i o n o n t h e b a s i s o f t h e i mp r e s s i o n o f t h e
c y t o p a t h o l o g i s t . I n t h e c u r r e n t B e t h e s d a s y s t e m, t h e r e p o r t s h o u l d i n c l u d e a
c o mme n t r e g a r d i n g t h e p r o b a b l e e t i o l o g y o f t h e A G C . B e c a u s e t h e mo s t c o mmo n
a b n o r ma l i t y i n a p a t i e n t w i t h a n A G C P a p s me a r i s s t i l l s q u a mo u s d y s p l a s i a , a
c o l p o s c o p y, i n c l u d i n g a n e n d o c e r v i c a l c u r e t t a g e , s h o u l d b e p e r f o r me d . D e p e n d i n g o
t h e c e l l ' s o r i g i n , s y mp t o ms , a n d p a t i e n t a g e , t h e w o r k u p ma y i n c l u d e a c o n i za t i o n o f
t h e c e r v i x a n d / o r e n d o me t r i a l b i o p s y, h y s t e r o s c o p y, o r d i l a t i o n a n d c u r e t t a g e . A n
A G C P a p s me a r s h o u l d n e v e r b e f o l l o w e d o r r e p e a t e d , b e c a u s e t h e r e i s a 3 0 % t o
60% yield of significant pathology in this patient population. It should never be
c o n f u s e d w i t h A S C U S , a n d H P V t y p i n g i s n o t r e c o mme n d e d .
D. Lgsil
D e p e n d i n g o n t h e p a t i e n t ' s a g e , t h i s c a n b e ma n a g e d c o n s e r v a t i v e l y w i t h r e p e a t P a
t e s t i n g o r mo r e t h o r o u g h l y w i t h c o l p o s c o p y a n d b i o p s i e s . T h e r e i s n o r o l e f o r H P V
D N A testing, because the yield for high-risk H P V D N A is so great that it is not a
useful screening tool in this setting. Between 15% and 30% of patients with L G S IL
actually have a high-grade lesion.
E. High-grade squamous epithelial lesion (H GSI L)
P a t i e n t s w i t h H G S I L s s h o u l d a l w a y s b e w o r k e d u p f u r t h e r w i t h v e r y r a r e e xc e p t i o n s
Colposcopy and biopsies are appropriate, but several patients undergo either
d i a g n o s t i c o r t h e r a p e u t i c c o n i za t i o n o f t h e c e r v i x o r L E E P. H P V t y p i n g i s n o t
appropriate, because the risk of high-grade dysplasia approaches 75%.
F. Suspicion for invasion
A l t h o u g h t h i s a b n o r ma l i t y i s f r e q u e n t l y e v a l u a t e d b y c o l p o s c o p y a n d b i o p s y, ma n y
patients undergo a cervical cone biopsy to confirm the diagnosis. If a gross lesion i
p r e s e n t , i t s h o u l d b e b i o p s i e d t o c o n f i r m t h e d i a g n o s i s , a n d a c o n i za t i o n i s n o t
r e c o mme n d e d . O n t h e o t h e r h a n d , t h e d i a g n o s i s o f mi c r o i n v a s i v e c e r v i c a l c a n c e r c a
b e ma d e o n l y o n t h e b a s i s o f a c o n i za t i o n s p e c i me n , b e c a u s e b i o p s i e s a r e j u s t n o t
s u f f i c i e n t . P a t i e n t s s h o u l d b e ma n a g e d b y c l i n i c i a n s w h o a r e t h o r o u g h l y f a mi l i a r w i t
433 / 652
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t h e t r e a t me n t a n d ma n a g e me n t o f c e r v i c a l c a n c e r.
References
1 . T y r i n g S K . H u ma n p a p i l l o ma i n f e c t i o n s : e p i d e mi o l o g y, p a t h o g e n e s i s , a n d h o s t
i mmu n e r e s p o n sJe .A m A c ad D er mat2ol0 0 0 ; 4 3 : S 1 8 S 2 6 .
2 . H o G Y, B i e r ma n R , B e a r d s l e y L , e t a l . N a t u r a l h i s t o r y o f c e r v i c o v a g i n a l
p a p i l l o ma v i r u s i n f e c t i o n i n y o u n g wNo me
E ngl
n . J M ed1 9 9 8 ; 3 3 8 : 4 2 3 4 2 8 .
3 . s t o r A J I. nt J G y nec ol P at hol
1993;12:186.
4 . J e ma l A , M u r r a y T , Wa r d E , e t a l . C a n c e r s t a t i s t C
i cAs , C2anc
0 0 5er
. J Cl i n
2005;55:1030.
5 . U . S . P r e v e n t i v e S e r v i c e s T a s k FSocrrceeni
e . ng f or c er v i c al c anc er :
r ec ommendat i ons and r at i onal
. h t t pe : / / w w w. a h r q . g o v / c l i n i c / u s p s t f / u s p s c e r v. h t m,
accessed on January 2003.
6 . S a s l o w D , R u n o w i c z C D , S o l o mo n D , e t a l . A me r i c a n C a n c e r S o c i e t y
g u i d e l i n e s f o r t h e e a r l y d e t e c t i o n o f c e r v i c a l n e o p l a s i a Ca nAdCcanc
a n cer
e r.J
Cl i n2002; 52: 342362.
7. K inney W K , Manos M M, H urley L B , et al. Where's the high-grade cervical
n e o p l a s i a ? t h e i mp o r t a n c e o f mi n i ma l l y a b n o r ma l P a p a n i c o l a o u d i a g n o s e s .
O bs t et G y nec ol
1998;91:973976.
8 . S o l o mo n D , S c h i f f ma n M , T a r o n e R . C o mp a r i s o n o f t h r e e ma n a g e me n t
s t r a t e g i e s f o r p a t i e n t s w i t h a t y p i c a l s q u a mo u s c e l l s o f u n d e t e r mi n e d s i g n i f i c a n c e :
b a s e l i n e r e s u l t s f r o m a r a n d o mi zeJd N
t rat
i al . C anc er I ns
2 0t 0 1 ; 9 3 : 2 9 3 2 9 9 .
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11.8 - Postmenopausal Bleeding

11 . 8 - P o s t me n o p a u s a l B l e e d i n g
11.8
Postmenopausal Bleeding
Victoria S. Kaprie lian
I . Background
P o s t me n o p a u s a l b l e e d i n g i s d e f i n e d a s v a g i n a l b l e e d i n g t h a t o c c u r s i n a w o ma n w h
h a s h a d n o me n s e s f o r a y e a r o r mo r e .
A. Etiology
A n y v a g i n a l b l e e d i n g i n a p o s t me n o p a u s a l w o ma n n o t o n h o r mo n e r e p l a c e me n t
t h e r a p y ( H R T ) r e q u i r e s a d i a g n o s i s , b e c a u s e ma l i g n a n t c a u s e s a r e f o u n d i n 1 0 % t o
2 0 % o f c a s e s . Wo me n o n c y c l i c H R T a r e e xp e c t e d t o h a v e u t e r i n e b l e e d i n g ;
b l e e d i n g a t u n e xp e c t e d t i me s o r i n e xc e s s i v e a mo u n t s r e q u i r e s i n v e s t i g a t i o n .
B. Epidemiology
A b n o r ma l v a g i n a l b l e e d i n g i s a c o mmo n o u t p a t i e n t p r o b l e m, o c c u r r i n g i n 1 0 % o f
w o me n o l d e r t h a n 5 5 y e a r s o f1)a gaen d( a c c o u n t i n g f o r 7 0 % o f g y n e c o l o g i c v i s i t s
d u r i n g t h e p e r i me n o p a u s a l a n d p o s t me n o p a u s 2)
al.years (
I I I . Evaluation
A. History
1. P a t t e r n o f b l e e d i A
n lgt h o u g h t h e a mo u n t o f b l e e d i n g i s n o t h e l p f u l i n
i d e n t i f y i n g ma l i g n a n c y, i t s h o u l d b e a s s e s s e d t o d e t e r mi n e t h e l i k e l i h o o d o f
s i g n i f i c a n t a n e mi a o r h y p o v o l e mi a t h a t ma y r e q u i r e i n t e r v e n t i o n . T i mi n g o f t h e
b l e e d i n g ma y s u g g e s t i t s c a u s e . S p e c i f i c r e l a t i o n s h i p t o me d i c a t i o n c o u r s e s o r
cycles suggests drug-induced bleeding. Postcoital bleeding suggests an
a t r o p h i c c a u s e o r c e r v i c a l p o l y p . A s s o c i a t i o n w i t h b o w e l mo v e me n t s o r u r i n a t i o n
suggests a nongenital source.
2. C u r r e n t m e d i c a t i oAnnsy h o r mo n a l t h e r a p y, i n c l u d i n g e s t r o g e n , p r o g e s t e r o n e ,
t a mo xi f e n , t h y r o i d r e p l a c e me n t , o r c o r t i c o s t e r o i d s , s h o u l d b e q u a n t i f i e d a n d
recorded.
a. A c y c l i c b l e e d i n g i s c o mmo n i n t h e f i r s t 3 t o 4 mo n t h s o n c o n t i n u o u s
e s t r o g e n p r o g e s t i n t h e r a p y a n d u s u a l l y d o e s n o t i n d i c a t e p a t h o l o g y.
435 / 652
tmdmss
B l e e d i n g t h a t i s e xc e s s i v e , p e r s i s t s a f t e r mo n t h s o f t h e r a p y, o r o c c u r s a f t e r
a me n o r r h e a h a s b e e n e s t a b l i s h e d o n t h e s e r e g i me n s s h o u l d b e e v a l u a t e d .
b. T h e r a t e o f e n d o me t r i a l c a n c e r i n w o me n t a k i n g t a mo xi f e n o r u n o p p o s e d
e s t r o g e n i s s i x t o s e v e n t i me s t h e r a t e f o r u n t r e a t e d w o me n . T h e f r e q u e n c y
o f e n d o me t r i a l p o l y p s i s a l s o i n c r e a s e d .
c. E xo g e n o u s c o r t i c o s t e r o i d s a n d i n c o r r e c t d o s a g e o f t h y r o i d r e p l a c e me n t c a n
l e a d t o me n s t r u a l i r r e g u l a r i t i e s a n d p o s t me n o p a u s a l b l e e d i n g .
3. P a s t m e d i c a l h i s t oNr uy l l i p a r i t y, e a r l y me n a r c h e , l a t e me n o p a u s e , a n d h i s t o r y
o f c h r o n i c a n o v u l a t i o n a r e r i s k f a c t o r s f o r e n d o me t r i a l h y p e r p l a s i a a n d
c a r c i n o ma . O b e s i t y, h y p e r t e n s i o n , d i a b e t e s , a n d l i v e r d i s e a s e a r e c o mmo n l y
a s s o c i a t e d w i t h e s t r o g e n e xc e s s a n d c a n a l s o i n c r e a s e r i s k . P a s t u s e o f o r a l
contraceptives is associated with decreased risk.
4. F a m i l y h i s t o rAy s t r o n g f a mi l y h i s t o r y o f e n d o me t r i a l , b r e a s t , o r c o l o n c a n c e r i s
a r i s k f a c t o r f o r e n d o me t r i a l c a n c e r.
1. V i t a l s i g n B
s lood pressure and pulse can indicate the degree and acuity of
b l o o d l o s s ; o r t h o s t a t i c c h a n g e s c a n b e e v i d e n c e o f s i g n i f i c a n t v o l u me d e p l e t i o n
F e v e r s u g g e s t s i n f e c t i o n a s a p o t e n t i a lCchaaupst e r ( 2 ). .6
2. A b d o m e nT e n d e r n e s s o r g u a r d i n g s u g g e s t s a n i n f e c t i o u s o r i n f l a mma t o r y
c a u s e . P a l p a t i o n f o r s u p r a p u b i c ma s s e s i s n e c e s s a r y a s p a r t o f t h e e v a l u a t i o n
f o r ma l i g n a n t c a u s e s .
3. P e l v i sI t i s n e c e s s a r y t o e xa mi n e t h e e xt e r n a l g e n i t a l i a , v a g i n a , a n d c e r v i x f o r
lesions or lacerations that could be the source of bleeding. T he uterus and
o v a r i e s mu s t b e p a l p a t e d t o a s s e s s f o r e n l a r g e me n t , ma s s e s , a n d t e n d e r n e s s .
P. 2 6 2
4. R e c t u mR e c t a l e xa mi n a t i o n a n d a n o s c o p y ma y b e w a r r a n t e d t o r u l e o u t
h e mo r r h o i d s o r o t h e r i n t e s t i n a l s o u r c e s o f bCl he ae pd ti ne gr 9( . 11
).
C. Testing
1. O f f i c e l a b o r a t o r y t e s tUi nr ign a l y s i s , s t o o l g u a i a c t e s t i n g , o r b o t h c a n b e u s e f u l
t o l o o k f o r n o n g e n i t a l s o u r c e s o f b l o o d . A c o mp l e t e b l o o d c o u n t ma y b e h e l p f u l
in assessing the degree of blood loss and likelihood of infection. Testing for
g o n o r r h e a a n d c h l a my d i a ma y b e w a r r a n t e d w h e n t e n d e r n e s s o r f e v e r i s
present.
2. P a p a n i c o l a o u ( P a p ) s m M
e a rn y s o u r c e s r e c o mme n d a P a p s me a r a s p a r t o f
t h e e v a l u a t i o n , a l t h o u g h i t s d i a g n o s t i c y i e l d i n t h e s e c a s e s i s l o w. C e r v i c a l
lesions or friability raise the possibility of a cervical bleeding source.
E n d o me t r i a l c e l l s f o u n d o n t h e P a p s me a r o f a p o s t me n o p a u s a l w o ma n n o t
r e c e i v i n g H R T w a r r a n t s f u r t h e r e v a l u a t i o n o f t h e e n d o me t r i u m.
3. B i o p s y
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a. Vi s i b l e l e s i o n s o f t h e v u l v a , v a g i n a , o r c e r v i x s h o u l d b e s e n t f o r b i o p s y.
b. I n t h e a b s e n c e o f a c l e a r n o n u t e r i n e s o u r c e o f b l e e d i n g , e n d o me t r i a l b i o p s y
i s u s u a l l y r e c o mme n d e d . O f f i c e - b a s e d e n d o me t r i a l b i o p s y i s l e s s i n v a s i v e
a n d mo r e c o s t - e f f e c t i v e t h a n d i l a t i o n a n d c u r e t t a g e ( D & C ) , w i t h
c o mp a r a b l e s e n s i t i v i t y a n d s p e c 3)
i f i.c iTt yh e( r e i s e xc e l l e n t c o r r e l a t i o n
b e t w e e n t h e h i s t o p a t h o l o g y o f s p e c i me n s t a k e n b y o f f i c e b i o p s y a n d D & C
(4) . B l i n d s a mp l i n g o f e i t h e r t y p e i s mo s t e f f e c t i v e w h e n p a t h o l o g y i s g l o b a l ,
rather than focal.
c. I f b l e e d i n g c o n t i n u e s a f t e r n o r ma l b i o p s y, f u r t h e r a s s e s s me n t i s n e c e s s a r y.
T h i s ma y b e d o n e b y r e p e a t b i o p s y, D & C , a n d / o r i ma g i n g .
S ge v e r a l me t h o d s a r e a v a i l a b l e ; a g r e e me n t i s l a c k i n g w i t h
r e g a r d t o t h e s p e c i f i c s o f w h e n e a c h me t h o d s h o u l d1,5)
b e. u s e d (
a. T r a n s v a g i n a l u l t r a s o u n d ( T V U S ) i s g a i n i n g p o p u l a r i t y a s a n a l t e r n a t i v e o r
a d j u n c t t o e n d o me t r i a l b i o p s y. A c l e a r l y i d e n t i f i a b l e e n d o me t r i a l s t r i p e < 4 o r
5 mm i n t h i c k n e s s i s h i g h l y u n l i k e l y t o c o n t a i n h y p e r p l a s i a o r c a r c i n o ma ,
a n d b i o p s y ma y n o t b e n e c e s3)
s a. r T
y V( U S i s b e t t e r t o l e r a t e d t h a n
e n d o me t r i a l b i o p s y, w i t h s i mi l a r d e t e c t i o n r a t e s f o r e n d o me t r i a l
a b n o r ma l i t i e 1,4)
s ( . F l u i d i n t h e e n d o me t r i a l c a v i t y h a s b e e n a s s o c i a t e d w i t h
c a r c i n o ma , a n d i t s p r e s e n c e w a r r a n t s f u r t h e r i n v e s t i g a t i o n . T V U S s h o u l d
n o t b e u s e d i n p l a c e o f b i o p s y i n w o me n t a k i n g t a mo xi f e n , b e c a u s e t h e d r u g
i s k n o w n t o c a u s e mi s l e a d i n g u l t r a s o u n d f i n d i n g s .
b. S a l i n e i n f u s i o n s o n o h y s t e r o g r a p h y ( S I S ; u l t r a s o u n d e v a l u a t i o n a f t e r t h e
i n s t i l l a t i o n o f f l u i d i n t o t h e e n d o me t r i a l c a v i t y ) a l l o w s t h e a r c h i t e c t u r a l
e v a l u a t i o n o f t h e u t e r i n e c a v i t y t o d e t e c t s ma l l l e s i o n s t h a t ma y b e mi s s e d
b y e n d o me t r i a l b i o p s y o r T V U S . T h e d i s a d v a n t a g e o f t h i s me t h o d i s t h a t n o
tissue is obtained, so if a lesion is found, hysteroscopy is then necessary
f o r d i r e c t e d b i o p s y.
c. H y s t e r o s c o p y i s b e c o mi n g t h e g o l d s t a n d a r d a g a i n s t w h i c h o t h e r me t h o d s
o f e n d o me t r i a l a s s e s s me n t a r e c o mp a r e d . T h i s p r o v i d e s d i r e c t v i s u a l i za t i o n
o f t h e e n d o me t r i a l c a v i t y, a l l o w i n g t a r g e t e d b i o p s y o r e xc i s i o n o f l e s i o n s .
H o w e v e r, i t i s mo r e c o s t l y a n d r e q u i r e s mo r e s p e c i a l e xp e r t i s e t h a n mo s t
o t h e r mo d a l i t i e s . B e c a u s e l e s i o n s a r e o c c a s i o n a l l y mi s s e d e v e n w i t h t h i s
me t h o d , s o me r e c o mme n d p e r f o r mi n g D & C a l o n g w i t h h y s4)t e. r o s c o p y (
d. M a g n e t i c r e s o n a n c e i ma g i n g i s o c c a s i o n a l l y h e l p f u l i n d e t e r mi n i n g t h e
presence of fibroids when sonography is not definitive.
e. P a l p a b l e a d n e xa l a b n o r ma l i t i e s s h o u l d b e e v a l u a t e d b y u l t r a s o u n d o r o t h e r
i ma g i n g a s a p p r o p r i a t e .
D. Genetics
G e n e t i c t e s t i n g i s n o t h e l p f u l i n t h e e v a l u a t i o n o f p o s t me n o p a u s a l b l e e d i n g . Wo me n
w i t h a f a mi l y h i s t o r y o f g y n e c o l o g i c ma l i g n a n c y a r e a t h i g h r i s k a n d s h o u l d b e
e v a l u a t e d t h o r o u g h l y. C o n s i d e r a t i o n o f t h e p o s s i b i l i t y o f i n h e r i t e d c o a g u l a t i o n
d e f i c i e n c y ma y b e w a r r a n t e d i n c a s e s o f u n u s u a l l y h e a v y b l e e d i n g .
437 / 652
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I V. Diagnosis
C a u s e s o f p o s t me n o p a u s a l b l e e d i n g h a v e b e e n r e p o r t e d a s a t r o p h y ( 5 9 % ) , p o l y p
( 1 2 % ) , e n d o me t r i a l c a n c e r ( 1 0 % ) , e n d o me t r i a l h y p e r p l a s i a ( 9 . 8 % ) , h o r mo n a l e f f e c t
( 7 % ) , c e r v i c a l c a n c e r ( < 1 % ) , a n d o t h e3)r . ( 2 % ) (
P. 2 6 3
I n i t i a l c l i n i c a l e v a l u a t i o n ma y i d e n t i f y a n o n u t e r i n e s o u r c e . P o s t c o i t a l s p o t t i n g i n
conjunction with vaginal atrophy or cervical friability suggests cervical or vaginal
mu c o s a l b l e e d i n g . I f n o o t h e r s o u r c e i s i d e n t i f i e d , t h e k e y t o d i a g n o s i s i s i ma g i n g
a n d t i s s u e s a mp l i n g o f t h e e n d o me t r i u m. A t h i n e n d o me t r i a l s t r i p e i n a w o ma n i n a
l o w - r i s k c a t e g o r y s u g g e s t s e n d o me t r i a l a t r o p h y. I f n e i t h e r b i o p s y n o r T V U S p r o v i d e
s u f f i c i e n t i n f o r ma t i o n , S I S , a n d / o r h y s t e r o s c o p y w i t h d i r e c t e d b i o p s y ma y b e u s e d .
& C s h o u l d b e r e s e r v e d f o r c a s e s i n w h i c h o t h e r me t h o d s a r e u n s u c c e s s f u l o r
unavailable.
References
1 . G o l d s t e i n R B , B r e e R L , B e n s o n C B , e t a l . E v a l u a t i o n o f t h e w o ma n w i t h
p o s t me n o p a u s a l b l e e d i n g : s o c i e t y o f r a d i o l o g i s t s i n u l t r a s o u n d - s p o n s o r e d
c o n s e n s u s c o n f e r e n c e s t a t e me
J Unlt t. r as ound M ed
2001;20:10251036.
2 . C l a r k T J , M a n n C H , S h a h N , e t a l . A c c u r a c y o f o u t p a t i e n t e n d o me t r i a l b i o p s y
i n t h e d i a g n o s i s o f e n d o me t r i a l c a n c e r : a s y s t e ma t i c q u a n t i t B
a tJi O
v eGr e v i e w.
2002;109:313321.
3 . G o o d ma n AE. v al uat i on and management of ut er i ne bl eedi ng i n
pos t menopaus al women
. U p T o D a t e o n l i n e ( w w w. u p t o d a t e . c o m) , a c c e s s e d J u l y
2005, last update October 2004.
4 . F e l d ma n SD. i agnos t i c ev al uat i on of t he endomet r i um i n women wi t h
abnor mal ut er i ne bl eedi
. Ung
p T o D a t e o n l i n e ( w w w. u p t o d a t e . c o m) , a c c e s s e d J u l y
2005, last update January 2005.
5 . C l a r k T J , Vo i t D , G u p t a J K , e t a l . A c c u r a c y o f h y s t e r o s c o p y i n t h e d i a g n o s i s o f
e n d o me t r i a l c a n c e r a n d h y p e r p l a s i a : a s y s t e ma t i c q u a n t i t aJt A
i vM
e Ar e v i e w.
2002;288(13): 16101621.
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11.9 - Vaginal Discharge

11 . 9 - Va g i n a l D i s c h a r g e
11.9
Vaginal Discharge
Sandra M. Carr-Johnson
I . Background
Va g i n a l d i s c h a r g e c a n b e p h y s i o l o g i c o r p a t h o l o g i c . W h e n p a t h o l o g i c , mu l t i p l e
s o u r c e s 1,2,3)
(
r e p o r t t h a t 9 0 % o f a f f e c t e d w o me n h a v e b a c t e r i a l v a g i n o s i s ( B V ) ,
v u l v o v a g i n a l c a n d i d i a s i s ( V V C ) , o r t r i c h o mo n i a s i s .
B V i s t y p i c a l l y c a u s e d b y n u me r o u s a n a e r o b i c o r g a n i s ms f o u n d i n n o r ma l v a g i n a l
f l o r a s u c h aGs ar dner el l a v agi nal i s , M obi l unc
s p eus
c i e sP, r ev ot el l, aa n d
M y c opl as ma homi (4)
ni s a n d c a n o c c u r i n u p t o 3 3 % o f w o me n . I n n o n r e s i s t a n t
c a s e s , V V C i s c a u s e dCbandi
y da al bi c ans
and accounts for 40% of infections.
Tr i c homonas v agi nal
, ai sp r o t o zo a n p a r a s i t e , i s f o u n d i n w o me n a n d me n a t
g e n i t o u r i n a r y s i t e s . I t i s r e s p o n s i b l e f o r t r i c h o mo n i a s i s , w h i c h c a u s e s 1 0 % t o 2 5 % o
v a g i n a l i n f e c t i o n4)s. (A l t h o u g h mo s t p a t i e n t s h a v e B V, VT.
V Cv,agi
o r nal,i st h e
o t h e r 1 0 % ma y h a v e p e l v i c i n f l a mma t o r y d i s e a s e , s e xu a l l y t r a n s mi t t e d d i s e a s e ,
n o n i n f e c t i o u s c a u s e s s u c h a s a t r o p h y a n d a l l e r g i e s , c h e mi c2)a ,l i r r i t a t i o n (
n e o p l a s i a , a n d f o r e i g n ma t e r i a l s s u c h a s r e t a i n e d5)t aamp
n do innst r(a u t e r i n e
devices.
I I I . Evaluation
A. History
I n p r e p a r a t i o n f o r t h e c l i n i c a l e n c o u n t e r, t h e c l i n i c i a n s h o u l d c o n s i d e r t h e f o l l o w i n g
1. K e y p o i n t s
a. A g e o f t h e p a t i e n t
b. M e d i c a l , s e xu a l , a n d s u r g i c a l h i s t o r y
c. C o n c e r n f o r p r e g n a n c y
d. M a r i t a l s t a t u s
P. 2 6 4
e. R e c e n t t r e a t me n t s
f. C u r r e n t h e a l t h a n d s mo k i n g s t a t u s
439 / 652
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2. C o n s i d e r a t i o n s
a. T h e y o u n g e r p a t i e n t i s mo r e l i k e l y t o h a v e p h y s i o l o g i c d i s c h a r g e t h a t s h e i s
n o t f a mi l i a r w i t h y e t b e c a u s e s h e i s l e a r n i n g a b o u t h e r b o d y a n d h e r
me n s t r u a l c y c l e .
b. T h e a d o l e s c e n t a n d y o u n g a d u l t ma y b e a t i n c r e a s e d r i s k o f s e xu a l l y
t r a n s mi t t e d i n f e c t i o n s ( S T I s ) .
c. T h e mo r e ma t u r e p a t i e n t ma y h a v e v a g i n a l a t r o p h y. N e o p l a s i a ma y b e a
c o n c e r n i f a s y mp t o m, p a r t i c u l a r l y p r u r i t u s , i s r e c u r r e n t .
d. P a t i e n t s w h o h a v e h a d r e c e n t g y n e c o l o g i c s u r g e r y o r i n s t r u me n t a t i o n a r e a t
increased risk of infection that can be evidenced by vaginal discharge.
e. M a r i t a l s t a t u s a n d s e xu a l h i s t o r y a r e h e l p f u l i n t h e a s s e s s me n t o f S T I r i s k .
f. R e c e n t t r e a t me n t p r o v i d e s a d i r e c t i o n r e g a r d i n g t h e t r e a t me n t o f r e c u r r e n t
or resistant infection.
g. S mo k i n g i n c r e a s e s t h e r i s k o f t r i c h o mo1)n i a ns di s B( V5)( .
h. T h e p a t i e n t ' s h e a l t h s t a t u s i s s i g n i f i c a n t b e c a u s e f r e q u e n t d i s c h a r g e ma y
s u g g e s t c h r o n i c i l l n e s s s u c h a s d i a b e t e s o r h u ma n i mmu n o d e f i c i e n c y v i r u s
( H IV) or be due to frequent antibiotics and long-term steroids and other
conditions.
3. D i s c h a r g e c h a r a c t e r i s tI ti ci s i mp o r t a n t t o k n o w t h e c h a r a c t e r i s t i c s o f
physiologic and pathologic discharge.
a. P h y s i o l o g i c d i s c h a r g e i s o f t e n c l e a r a n d t h i n a n d c h a n g e s w i t h t h e
me n s t r u a l c y c l e .
b. P a t h o l o g i c d i s c h a r g e i s o f t e n d i f f u s e , h a s c o l o r, a n d ma y h a v e a n o d o r.
i. T h e d i s c h a r g e o f B V a h o mo g e n o u s w h i t e , n o n i n f l a mma t o r y d i s c h a r g e
t h a t s mo o t h l y c o a t s t h e v a g i n a l w a l l s . T h e d i s c h a r g e c a n b e f i s h y o r
mu s t y b e f o r e o r a f t e r t h e a d d i t i o n o f K O H ( a p o s i t i v e w h i f f t e s t ) ; s e e
s e c t i o nI I I . C(3) .
i i. T h e d i s c h a r g e o f V V C i s o f t e n t h i c k , w h i t e , a n d o d o r f r e e , a n d i t c a n b e
a s s o c i a t e d w i t h v a g i n a l p r u r i t u s a n d e r y t h e ma .
i i i. T h e d i s c h a r g e o f t r i c h o mo n i a s i s ma y b e d i f f u s e , ma l o d o r o u s , a n d
y e l l o w - g r e e n , w i t h v u l v a r i r r i t3)
a t. i o n (
4. M e n s e s
a. T h e l a s t me n s t r u a l p e r i o d a s s e s s e s me n s t r u a l h o r mo n a l f l u c t u a t i o n s ,
p r e g n a n c y, a n d me n o p a u s a l s t a t e s .
b. T h e c a u s e o f v a g i n a l d i s c h a r g e i n p r e g n a n c y i s i mp o r t a n t t o k n o w, b e c a u s e
B V, V V C , a n d t r i c h o mo n i a s i s c a n b e a s s o c i a t e d w i t h a d v e r s e p r e g n a n c y
o u t c o me s1,3)
( .
T hs e s e c a n i n c l u d e i t c h i n g , s o r e n e s s , d y s u r i a , o r
p o s t c o i t a l b l e e d i n g , l o w e r a b d o mi n a l p a i n , p e l v i c p a i n , a n d d y s p a r e u n i a .
6. R i s k f a c t o r s f o r S TTIhse s e c a n i n c l u d e a g e < 2 5 y e a r s , mu l t i p l e a n d f r e q u e n t
440 / 652
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partners, previous S T Is, unprotected intercourse, and partners with high-risk

s e xu a l b e h a v i o5)r .(
W h i l e e xa mi n i n g t h e f e ma l e p a t i e n t , c a r e mu s t b e g i v e n t o h e r c o mf o r t , p r i v a c y, a n d
t h e s e n s i t i v e n a t u r e o f t h e l i t h o t o my p o s i t i o n . T h e e n t i r e p h y s i c a l e xa mi n a t i o n s h o u
b e c o n s i d e r e d i f t h e p a t i e n t a p p e a r s a c u t e l y i l l , h a s s i g n s o f s y s t e mi c i l l n e s s ,
i n c l u d i n g a b d o mi n a l p a i n , r a s h , f e v e r, o r o t h e r c o mp l a i n t s . A t t e n t i o n s h o u l d b e g i v e
t o a s s o c i a t e d s y mp t o ms e l i c i t e d d u r i n g t h e h i s t o r y a s w e l l a s t h e h i g h l i g h t e d
c o mp l a i n t o f v a g i n a l d i s c h a r g e a n d i t s c h a r a c t e r i s t i c s . A s y s t e ma t i c g e n i t o u r i n a r y
e xa mi n a t i o n w i t h d e t a i l e d d o c u me n t a t i o n i s t h e s t a n d a r d o f c a r e .
C. Testing
R e f e r t oT a b l e 11 . 9.. 1
I V. Diagnosis
C o mmo n l y, s y mp t o ms r e s o l v e a f t e r t r e a t me n t f o r mo s t p a t i e n t s w h o p r e s e n t w i t h t h e
c o mp l a i n t o f v a g i n a l d i s c h a r g e . H o w e v e r, r e c u r r e n t v a g i n i t i s , p a r t i c u l a r l y w i t h B V a
V V C , i s n o t u n c o mmo n . R e c u r r e n t B V c a n b e d u e t o t r e a t me n t f a i l u r e , p e r s i s t e n t o r
reintroduction of foreign bodies, or reintroduction of infection. Recurrent VVC can b
c a u s e d b y n oC
n . al bi c ans
infection, which is increasing with
P. 2 6 5
t h e f r e q u e n t u s e o f o v e r - t h e - c o u n t e r a n t i f u n g a l me d i c a t i o n s . R e c u r r e n t
t r i c h o mo n i a s i s i s l i k e l y t h e r e s u l t o f t r e a t me n t f a i l u r e o r r e i n t r o d u c t i o n o f i n f e c t i o n .
A l s o , i t i s h e l p f u l t o c o n s i d e r t h a t n o n i n f e c t i o u s e t i o l o g i e s a s w e l l a s l e s s c o mmo n
i n f e c t i o u s e t i o l o g i e s ma y b e p r e s e n t .
TAB L E 11.9.1 Differential Diagnosis and Clinical

Manifestations in Vaginal Discharge
Bacte rial
v aginosis
Vulv ov aginal
candidiasis
Trichom oniasis
Discharge
characteristics
H o mo g e n o u s ,
T hick, white,
white,
cottage cheese
n o n i n f l a mma t o r y l i k e
Ye l l o w - g r e e n
copious,
w a t e r y,
pooling, frothy
(4)
Odor
Fishy before
and after K O H
+ whiff test
Malodorous
(10%)
Physical
D i s c h a r g e c o a t s E xc o r i a t i o n e d e ma , S t r a w b e r r y
Not typical
441 / 652
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e xa mi n a t i o n
vaginal wall
e r y t h e ma
Precipitating
factors
Changes to
vaginal flora,
p r e g n a n c y,
c o n d o ms ,
douching, new
or frequent
partners (1)
Antibiotics,
S e xu a l l y
s y s t e mi c i l l n e s s , t r a n s mi t t e d
me d i c a t i o n ,
infection
i mmu n o c o mp r o mi s e
pH
>4.5
N o r ma l l y < 4 . 5
M i c r o s c o p y / G r a m A ms e l ' s
stain
diagnostic
c r i t e r iaa
cervix (2% of
patients) (4)
>4.5 in 90%
B udding yeast or 60%70%

hyphae after 10% sensitive
KOH
C u l t u r e mo s t
sensitive (3)
Presence of
clue cells highly
suggestive (1)
Moving
flagellae
Associated
s y mp t o ms
May be
a s y mp t o ma t i c
P r u r i t u s , v a g i n a l Vu l v a r i r r i t a t i o n
irritation, dysuria and dysuria,
(1)
lower
a b d o mi n a l
p a i n ; ma y b e
(4)
a T h i n h o mo g e n e o u s d i s c h a r g e , p o s i t i v e w h i f f t e s t , c l u e c e l l s , v a g i n a l p H
> 4.5three out of four is highly suggestive of B V (1).
References
1 . E g a n M E , L i p s k y M S , D i a g n o s i s o f v aAg m
i n i Ft i am
s . P hy s i c i an
2 0 0 0 ; 6 2 ( 5 ) : 1 0 9 5 11 0 4 .
2 . M i l l e r K E , R u i z D E , G r a v e s J C . U p d a t e o n t h e p r e v e n t i o n a n d t r e a t me n t o f
442 / 652
tmdmss
s e xu a l l y t r a n s mi t t e d d i s e aAsm
e sF. am P hy s i c i an
2003;67(9):19151922.
3 . h t t p : / / w w w. c d c . g o v / mmw r / p r e v i e w / mmw r h t ml / r r 5 1 0 6 a 1 . h t m, M a y 2 0 0 6 .
4 . w w w. i n f o p o e ms . c o m, M a y 2 0 0 6 .
5 . M i t c h e l l H . Va g i n a l d i s c h a r g e - c a u s e s , d i a g n o s i s a n d Bt rMe Ja t me n t .
2004;328:13061308.
443 / 652
tmdmss
12.1 - Arthralgia
> T a b l e o f C o n t e n t s > 1 2 - M u s c u l o s k e l e t a l P r o b l e ms > 1 2 . 1 - A r t h r a l g i a
12.1
Arthralgia
I m an S. Al-Jabi
I . Background
A r t h r a l g i a me a n s j o i n t p a i n . J o i n t p a i n ma y a r i s e f r o m t h e j o i n t s o r p e r i a r t i c u l a r
s t r u c t u r e s s u c h a s b o n e s , mu s c l e s , l i g a me n t s , o r n e r v e s .
T h e mo s t c o mmo n c a u s e o f a r t h r a l g i a i s me c h a n i c a l j o i n t d y s f u n c t i o n o f t e n r e l a t e d
t o t r a u ma r a t h e r t h a n i n f l a mma t o r y o r r e f e r r e d p a i n . T w e n t y p e r c e n t o f a l l p r i ma r y
c a r e c o n s u l t a t i o n s w o r l d w i d e a r e f o r a c h e s a n d p a i n s t h a t s e e m r e l a t e d t o mu s c l e s
a n d j o i n t s . T h e s e a r e mo s t c o mmo n i n t h e a d u l t p o p u l a t i o n . I n t h e e l d e r l y, t h e mo s t
c o mmo n p r o b l e m i s o s t e o a r t h r i t i s ; i n mi d d l e a g e , i n f l a mma t o r y c o n d i t i o n s
p r e d o mi n a t e ; a n d i n t h e y o u n g , s y s t e mi c c o n d i t i o n s a r e 6)
mo. r e l i k e l y (
I I I . Evaluation
A l t h o u g h t h e c a u s e s o f a r t h r a l g i a a r e n u me r o u s , a d e t a i l e d me d i c a l h i s t o r y, t h o r o u g
p h y s i c a l e xa mi n a t i o n , a n d j u d i c i o u s u s e o f l a b o r a t o r y t e s t i n g a n d r a d i o l o g i c i ma g i n g
u s u a l l y a l l o w a d i a g n o s i s t o b e 4)
ma. dTeh e( h i s t o r y h e l p s d e t e r mi n e w h e t h e r t h e
p r o b l e m i s me c h a n i c a l o r i n f l a mma t o r y, t h e e xa mi n a t i o n h e l p s d e f i n e w h e t h e r t h e
p a i n i s a r t i c u l a r, p e r i a r t i c u l a r, o r r e f e r r e d .
A. History
Factors to consider include the following:
1. P a t i e n t a g e : d i a g n o s t i c p r o b a b i l i t i e s d i f f e r w i t h e a c h a g e - g r o u p . I n f e c t i o u s
c a u s e s t e n d t o b e l e s s a g e d e p e n7)d e n t (
2. H i s t o r y o f t r a u ma
3. L o c a t i o n o f t h e p a i n a n d j o i n t s w e l l i n g
4. P a s t me d i c a l h i s t o r y
5. F a mi l y h i s t o r y ( s y s t e mi c l u p u s e r y t h e ma t o s u s [ S L E ] , r h e u ma t o i d a r t h r i t i s [ R A ] ,
ankylosing spondylosis, osteoarthritis)
6. N u mb e r o f j o i n t s i n v o l v e d ( i f t h r e e o r mo r e , c o n s i d e r R A )
7. L i mi t a t i o n o f t h e mo v e me n t o f t h e j o i n t
444 / 652
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12.1 - Arthralgia
8. S t i f f n e s s , w e a k n e s s , d i u r n a l v a r i a t i o n , o r e f f e c t o f e xe r c i s e
9. C h r o n o l o g y o f s y mp t o ms : d u r a t i o n , a c u i t y o f o n s e t , p a i n c o n t i n u o u s
( i n f l a mma t o r y ) o r i n t e r mi t t e n t ( me c h a n i c a l )
1 0. S y s t e mi c s y mp t o ms s u c h a s f e v e r, f a t i g u e , r a s h , o r w e i g h t l o s s
11. I mmu n i za t i o n o r r e c e n t t r a v e l
1 2. M e d i c a t i o n
1 3. R e v i e w o f s y s t e ms
a. S k i n : ma l a r r a s h ( S L E ) , G o t t r o n ' s p a p u l e s ( d e r ma t o my o s i t i s ) , p s o r i a t i c r a s h
o r n a i l s , mo u t h u l c e r s ( B e h e t ' s d i s e a s e )
b. C a r d i o v a s c u l a r : i n f e c t i o u s e n d o c a r d i t i s , r h e u ma t i c f e v e r
c. G a s t r o i n t e s t i n a l : i n f l a mma t o r y b o w e l d i s e a s e , H e n o c h - S c h n l e i n p u r p u r a
d. G e n i t o u r i n a r y : d y s u r i a , d i s c h a r g e
e. H e ma t o l o g i c : h e mo g l o b i n o p a t h i e s
f. N e u r o l o g i c / p s y c h i a t r i c
1. G e n e r a l a p p e a r a n c e : c o n j u n c t i v i t i s , s k i n l e s i o n s
2. Vi t a l s i g n s : w e i g h t l o s s , f e v e r
3. O t h e r s i g n s , s u c h a s l y mp h a d e n o p a t h y o r h e a r t mu r mu r
4. J o i n t e xa mi n a t i o n : s w e l l i n g , w a r mt h , t e n d e r n e s s , d e f o r mi t y, r a n g e o f mo v e me n t ,
mu s c l e w a s t i n g
C. Testing
L a b o r a t o r y t e s t i n g i s h e l p f u l i n d i a g n o s i n g i n f l a mma t o r y p r o c e s s e s . H o w e v e r, i t i s
mo s t o f t e n u s e f u l i n c o n f i r mi n g o r r e f u t i n g a d i a g n o s i s ma d e o n c l i n i c a l g r o u n d s .
P. 2 7 0
TAB L E 12.1.1 Differential Diagnosis of Arthralgia

(4)
Childhood
Adole sce nce
Middle age
Nonaccidental SportsOsteoarthritis
injury
related pain
Growing
pains
R h e u ma t o i d
arthritis
Elde rly
Osteoarthritis
R h e u ma t o i d a r t h r i t i s o r P o l y my a l g i a
o t h e r c o n n e c t i v e t i s s u e r h e u ma t i c a
d i s e a s e ( e . g . , s y s t e mi c
445 / 652
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12.1 - Arthralgia
l u p u s e r y t h e ma t o s u s ,
s y s t e mi c s c l e r o s i s ,
spondyloarthropathies)
Acute
r h e u ma t i c
fever
Vi r a l ( e . g . ,
rubella)
R e a c t i v e a r t h r i t i s ( e . g . ,P a r a n e o p l a s t i c
e n t e r o p a t h i c a r t h r i t i s ) s y n d r o me
Juvenile
r h e u ma t o i d
arthritis
Sarcoidosis
Hypothyroidism
P aget's
disease
HenochSchnlein
purpura
Gout
Metabolic
bone disease
Vi r a l a r t h r i t i s
S y p h i l i s , g o n o c o c c a l i n f e c t i o n , h u ma n
i mmu n o d e f i c i e n c y v i r a l i n f e c t i o n
L e u k e mi a
Brucellosis
Medication
side effects
Depression
1. L a b o r a t o r y t e s t1,5)
s (
a. C o mp l e t e b l o o d c o u n t , e r y t h r o c y t e s e d i me n t a t i o n r a t e , a n d C - r e a c t i v e
p r o t e i n a r e f r e q u e n t l y a b n o r ma l i n i n f l a mma t o r y c o n d i t i o n s .
b. A n t i n u c l e a r a n t i b o d y i s p o s i t i v e i n 9 0 % o f S L E c a s e s .
c. R h e u ma t o i d f a c t o r i s p o s i t i v e i n mo s t c a s e s o f r h e u ma t o i d a r t h r i t i s a s w e l l
a s i n ma n y a u t o i mmu n e d i s e a s e s a n d c h r o n i c i n f e c t i o n s .
d. H L A - B 2 7 h a s a h i g h p r e v a l e n c e i n s p o n d y l o a r t h r i t i d e s ( a n k y l o s i n g
spondylosis, R eiter's disease, enteropathic arthritis).
e. U r i n a l y s i s ma y s h o w p r o t e i n u r i a o r h e ma t u r i a i f t h e r e i s r e n a l i n v o l v e me n t i n
connective tissue disease.
f. L y me s e r o l o g y
g. L i v e r f u n c t i o n t e s t i n g
2. I m a g i n g 2)(
a. P l a i n r a d i o g r a p h ma y h e l p i n mo n o a r t i c u l a r j o i n t p a i n .
b. C o mp u t e d t o mo g r a p h y a n d ma g n e t i c r e s o n a n c e i ma g i n g ma y b e u s e f u l f o r
j o i n t d e r a n g e me n t s .
c. S y n o v i a l f l u i d a s p i r a t i o n h e l p s d i s t i n g u i s h i n f l a mma t o r y f r o m i n f e c t i v e a n d
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12.1 - Arthralgia
crystal arthropathies. A bloody joint aspirate without elevated white blood

c o u n t mo s t l i k e l y i n d i c a t e s t r a u ma .
I V. Diagnosis
T h e d i f f e r e n t i a l d i a g n o s i s i s p r e s eTnat ebdl ei n1 2 . 1.. 1
References
1 . M o r r i s D . O s t e o a r t h rP
i t ri si m
. c ar e U pdat
. 2e0 0 5 ; 3 ( 1 ) : 2 0 2 6 .
2 . H a m R J , S l o a n e P D , Wa r s h a w G A P
, rei dmar
s . y c ar e ger i at r i c s , a c as ebas ed appr oac
, h4 t h e d . M i s s o u r i , M O : H a r c o u r t H e a l t h S c i e n c e s , 2 0 0 2 .
3 . W i l k i n g A P.
A n appr oac h t o t he c hi l d wi t h j oi .nt2 2pai
Mna r c h 2 0 0 5 .
h t t p : / / w w w. u t d o l . c o m/ a p p l i c a t i o n / t o p i c / t o p i c O u t l i n e . a s p ?
file=pedirheu/12386&type= P
P. 2 7 1
4 . D i e p p e PA , K l i p p e l J H , P
e dr ac
s . t i c al r heumat ol.ogy
L o n d o n : T i me s M i r r o r
International, 1995.
5 . B r i t t e n N , C u l p e p p e r L , G a s s D , e t aVol
l . , ume
e d s . 2: c l i ni c al management
f r om O x f or d t ex t book of pr i mar y medi ,c al
3 r dc ar
e de. N e w Yo r k , N Y : O xf o r d
6 . K h o t A , P o l me a r A , ePdrsac
. t i c al gener al pr ac t i c e, gui del i nes f or ef f ec t i v e
c l i ni c al management
, 4th ed. Edinburgh: Elsevier Science, 2003.
7 . C o u ma s J M , H o w a r d B A , J a c o b s o n E W. D i a g n o s t i c i ma g i n g o f r h e u ma t o l o g i c
d i s o r d e r s . I n : N o b l e J ,Tex
e dt. book of pr i mar y c ar e medi
, c3irne
d ed. Missouri,
M O: Harcourt Health Sciences, 2001.
447 / 652
tmdmss
12.2 - Calf Pain
> T a b l e o f C o n t e n t s > 1 2 - M u s c u l o s k e l e t a l P r o b l e ms > 1 2 . 2 - C a l f P a i n
12.2
Calf Pain
Ste phe n J. Hartsock
Re be cca L. Spaulding
I . Background
Calf pain is caused by an array of causes that range from benign to life threatening
T h e p h y s i c i a n mu s t b e a b l e t o d e t e r mi n e q u i c k l y w h i c h p a t i e n t s r e q u i r e a d e t a i l e d
w o r k u p . D e e p v e n o u s t h r o mb o s i s ( D V T ) , c o mp a r t me n t s y n d r o me , a n d c e l l u l i t i s a r e
t h e mo s t s e r i o u s c o n d i t i o n s r e q u i r i n g p r o mp t r e c o g n i t i o n a n d ma n a g e me n t .
A n u mb e r o f c o n d i t i o n s c a n r e s u l t i n c a l f pTaai nb l (es e1 e2 . 2). .1 M u s c u l o s k e l e t a l

c a u s e s a c c o u n t f o r 4 0 % o f c a l f1)p.a P
i na i( n c a n b e r e f e r r e d f r o m t h e b a c k , h i p s , o r
o t h e r a r e a s i n t h e l o w e r l i mb . D V T i s o n e o f t h e mo s t s e r i o u s c a u s e s o f c a l f p a i n .
R i s k f a c t o r s f o r D V T i n c l u d e e l e me n t s o f Vi r c h o w ' s t r i a d ( v e n o u s w a l l d a ma g e ,
s t a s i s , h y p e r c o a g u l a b i l i t y ) ; t h e r e f o r e i mmo b i l i za t i o n , p r e g n a n c y, a n d r e c e n t s u r g e r y
a r e c l a s s i c a n t e c e d e n t s . C o mp a r t me n t s y n d r o me u s u a l l y r e s u l t s f r o m s w e l l i n g ,
t y p i c a l l y f r o m t r a u ma , a n d c a n a f f e c t a n y o f t h e f o u r f a s c i a l c o mp a r t me n t s o f t h e
a n k l e , r e s u l t i n g i n i n c r e a s e d i n t r a c o mp a r t me n t a l p r e s s u r e , i s c h e mi a , a n d i r r e v e r s i b l
l o s s o f n e u r o mu s c u l a r f u n c t i o n i f n o t p r o mp t l y r e c o g n i ze d a n d t r e a t e d .
R h a b d o my o l y s i s c a n b e t r i g g e r e d b y t r a u ma , i s c h e mi a , d r u g s , o r i n f e c t i o n .
P e r i p h e r a l n e u r o p a t h y c a n b e c a u s e d b y n e r v e e n t r a p me n t o r me d i c a l c a u s e s s u c h
a s d i a b e t e s , v i t a mi1n2 B
a n d f o l a t e d e f i c i e n c i e s , t h y r o i d d i s e a s e , a l c o h o l i s m, h u ma n
i mmu n o d e f i c i e n c y v i r u s , o r s y p h i l i s .
I I I . Evaluation
A. History
T h e h i s t o r y i s c r i t i c a l i n n a r r o w i n g t h e d i f f e r e n t i a l d i a g n o s i s . P e r t i n e n t i n f o r ma t i o n
i n c l u d e s t h e e xa c t l o c a t i o n o f t h e p a i n , a s w e l l a s q u a l i t y, s e v e r i t y, d u r a t i o n , a n d
a g g r a v a t i n g o r a l l e v i a t i n g f a c t o r s . O t h e r s y mp t o ms i n c l u d e s w e l l i n g , c o l o r c h a n g e s ,
w a r mt h , n u mb n e s s , w e a k n e s s , a n d f e v e r o r c h i l l s . R e l a t e d t r a u ma o r e xe r c i s e mu s t
b e n o t e d , a s w e l l a s a r e c e n t h i s t o r y o f i mmo b i l i za t i o n . C u r r e n t me d i c a t i o n s ,
i n c l u d i n g h o r mo n e s , s t a t i n s , o r d r u g s t h a t a f f e c t e l e c t r o l y t e s s u c h a s d i u r e t i c s ,
bisphosphonates, and alcohol should be recorded.
448 / 652
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12.2 - Calf Pain
T h e e xa mi n a t i o n b e g i n s b y a s s e s s i n g v i t a l s i g n s , s p e c i f i c a l l y b l o o d p r e s s u r e ,
t e mp e r a t u r e , a n d h e a r t r a t e . T h e l o w e r e xt r e mi t i e s s h o u l d b e e xa mi n e d f o r s w e l l i n g ,
c o l o r c h a n g e s , w o u n d s , h a i r a n d n a i l p a t t e r n s , a n d s y mme t r y. T h e c a l f
c i r c u mf e r e n c e s ma y b e me a s u r e d . P a l p a t i o n e v a l u a t e s w a r mt h , t e n d e r n e s s , e d e ma ,
a n d b o n e o r mu s c l e d e f e c t s . R a n g e o f mo t i o n o f t h e k n e e s , a n k l e s , a n d t o e s i s
noted. Suspicion that the pain is referred should result in evaluation of the back an
h i p s . Va s c u l a r e xa mi n a t i o n i n c l u d e s e v a l u a t i o n f o r v e n o u s i n s u f f i c i e n c y, t e n d e r
varicosities, or venous cords, arterial pulses, and capillary refill. Neurologic
e xa mi n a t i o n i n c l u d e s l o w e r e xt r e mi t y e v a l u a t i o n o f mo t o r a n d s e n s o r y f u n c t i o n , a s
w e l l a s r e f l e xe s . A b i l i t y t o b e a r w e i g h t a n d g a i t i s o b s e r v e d . S p e c i a l t e s t s f o r D V T
e xi s t , i n c l u d i n g H o ma n ' s s i g n ( p a i n o n p a s s i v e d o r s i f l e xi o n o f t h e f o o t ) . H o w e v e r, t h
p o s i t i v e p r e d i c t i v e v a l u e o f p h y s i c a l e xa mi n a t i o n f i n d i n g s i s o n l y 5 5 % . T h e r e f o r e ,
suspicion of the presence of D VT should lead to radiologic evaluation to confirm
P. 2 7 2
t h e p r e s e n c e o r a b s e n c e o f t h r o mb o s i s . T h o mp s o n ' s t e s t ( s q u e e zi n g o f t h e p r o xi ma l
g a s t r o c n e mi u s a n d s o l e u s t e n d o n s , o b s e r v i n g f o r a n k l e p l a n t a r f l e xi o n ) e v a l u a t e s
possible rupture of the Achilles tendon.
TAB L E 12.2.1 Differential Diagnosis of Calf Calf

pain
Va s c u l a r
Ve n o u s
D e e p v e n o u s t h r o mb o s i s
S u p e r f i c i a l t h r o mb o p h l e b i t i s
Va r i c o s e v e i n
Arterial
Claudication
I s c h e mi a / e mb o l u s
Neurologic
Referred pain
Hip
Back
P e r i p h e r a l n e u r o p a t h y o r n e r v e e n t r a p me n t
R e s t l e s s l e g s y n d r o me
Musculoskeletal
C a l f mu s c l e s t r a i n
Calf tendon rupture
B aker's cyst
D e l a y e d o n s e t mu s c l e s o r e n e s s
C o mp a r t me n t s y n d r o me
M u s c l e c r a mp s / my a l g i a
E l e c t r o l y t e i mb a l a n c e
R h a b d o my o l y s i s
T r a u ma
449 / 652
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12.2 - Calf Pain
Fracture
B r u i s e / h e ma t o ma
Infectious
C. Testing
D u p l e x u l t r a s o u n d i s u s e d t o d i a g n o s e s u p e r f i c i a l t h r o mb o s i s a n d D V T ( s e n s i t i v i t y
8 9 % a n d s p e c i f i c i t y 9 42)%. ) C( o n t r a s t v e n o g r a p h y i s t h e g o l d s t a n d a r d ( s e n s i t i v i t y
9 5 % a n d s p e c i f i c i t y 9 7 % ) b u t i s mo r e i n2)v .a sTi hv e (a n k l e - b r a c h i a l i n d e x
( s e n s i t i v i t y 9 5 % , s p e c i f i c i t y 3)
99%
w i)t h( d u p l e x u l t r a s o u n d i s u s e d t o d i a g n o s e
p e r i p h e r a l a r t e r i a l d i s e a s e ( PA D ) . A n g i o g r a p h y i s c o n s i d e r e d t h e g o l d s t a n d a r d .
Plain radiographs are unnecessary unless there is suspicion of fracture, foreign
b o d y, o r ma l i g n a n c y. M a g n e t i c r e s o n a n c e i ma g i n g c a n b e u s e d f o r t h e d i a g n o s i s o f
mu s c l e a n d s o f t t i s s u e i n j u r i e s . U l t r a s o u n d c a n e v a l u a t e mu s c l e t e a r s a n d
t e n d i n o p a t h y. C o mp a r t me n t p r e s s u r e t e s t i n g i s p e r f o r me d t o c o n f i r m t h e d i a g n o s i s o
c o mp a r t me n t s y n d r o me . E l e v a t e d l e v e l s o f c r e a t i n e p h o s p h o k i n a s e a n d
t r a n s a mi n a s e s ma y b e s e e n i n p a t i e n t s w i t h mu s c l e i n j u r y.
I V. Diagnosis
P e r t i n e n t e xa mi n a t i o n f i n d i n g s f o r D V T i n c l u d e s w e l l i n g , w a r mt h , t e n d e r n e s s , a n d
d i s c o l o r a t i o n . PA D t y p i c a l l y p r e s e n t s w i t h i n t e r mi t t e n t c l a u d i c a t i o n t h a t r e s o l v e s
r a p i d l y u p o n r e s t . R e s t l e s s l e g s y n d r o me p r e s e n t s w i t h a n u n c o mf o r t a b l e s e n s a t i o n ,
a s s o c i a t e d w i t h a n u n c o n t r o l l a b l e u r g e t o mo v e t h e l e g s , u s u a l l y a t n i g h t . S u d d e n
c a l f p a i n i n a n a c t i v e p a t i e n t o f t e n r e p r e s e n t s a mu s c l e s t r a i n o r t e n d o n r u p t u r e . T h
P. 2 7 3
g a s t r o c n e mi u s i s t h e mo s t c o mmo n l y i n j u r e d mu s c l e . R u p t u r e o f t h e A c h i l l e s t e n d o n
l e a d s t o i n a b i l i t y t o a c t i v e l y p l a n t a r f l e x t h e a n k l e a n d a p o s i t i v e T h o mp s o n ' s t e s t . A
r u p t u r e d B a k e r ' s c y s t ma y c a u s e s w e l l i n g a n d d i s c o l o r a t i o n t h a t a d v a n c e s d i s t a l l y
i n t o t h e c a l f . E xe r t i o n a l , o r c h r o n i c , c o mp a r t me n t s y n d r o me u s u a l l y o c c u r s w i t h
e xe r c i s e . T h e p a t i e n t h a s p a i n o r n u mb n e s s t h a t r e s o l v e s a f t e r c e s s a t i o n o f e xe r c i s
T h e a n t e r i o r c o mp a r t me n t i s i n v o l v e d 7 0 % o f 4)
t h.e I nt i me
a c u(t e c o mp a r t me n t
s y n d r o me , i n c r e a s e d p a i n o n p a s s i v e s t r e t c h i n g o f t h e l o n g mu s c l e s p a s s i n g t h r o u g h
a c o mp a r t me n t i s a n i mp o r t a n t s i g n . S e v e r e p a i n , p a l l o r, a n d p a r a l y s i s a r e s i g n s o f
a d v a n c e d i s c h e mi c c o mp a r t me n t s y n d r o me . D e l a y e d o n s e t mu s c l e s o r e n e s s ( D O M S )
s t a r t s a p p r o xi ma t e l y 1 2 h o u r s a f t e r e xe r c i s e a n d l a s t s 2 t o 3 d a y s . M u s c l e c r a mp s
a n d s o r e n e s s ma y a l s o r e s u l t f r o m d e h y d r a t i o n o r r h a b d o my o l y s i s . C e l l u l i t i s c a u s i n g
r e d n e s s , p a i n , w a r mt h , a n d s w e l l i n g i s u s u a l l y d u e t o a n o b v i o u s l o c a l a r e a o f s k i n
disruption on calf or foot, but occasionally the infection originates in an area such
t h e i n t e r d i g i t a l w e b s p a c e a n d ma y b e mi s s e d u n l e s s s p e c i f i c a l l y s o u g h t f o r.
References
1 . K a h n S R . T h e c l i n i c a l d i a g n o s i s o f d e e p v e i n t h r o mb o s i s : i n t e g r a t i n g
i n c i d e n c e , r i s k f a c t o r s , a n d s y mp t o ms a nAd r cs h
i g nI nt
s . er n M ed
1998;158(21):23152323.
2 . L i n e B R . P a t h o p h y s i o l o g y a n d d i a g n o s i s o f d e e p v e i n tS
h remi
o mbn oNs iuc
s .l
450 / 652
tmdmss
12.2 - Calf Pain
M ed 2 0 0 1 ; 3 1 ( 2 ) : 9 0 1 0 1 .
3 . C o me r o t a A J . T h e c a s e f o r e a r l y d e t e c t i o n a n d i n t e g r a t e d i n t e r v e n t i o n i n
p a t i e n t s w i t h p e r i p h e r a l a r t e r y d i s e a s e a n d i n t e r mi t t e n t cJl aEundov
d i c a tas
i o cn .
T her 2 0 0 3 ; 1 0 : 6 0 1 6 1 3 .
4 . K o r k o l a M . Av a n d o l a A . E xe r c i s e i n d u c e d l P
e ghypsa S
i npor
. t s med
2001;29(6):3550.
451 / 652
tmdmss
12.3 - Hip Pain
> T a b l e o f C o n t e n t s > 1 2 - M u s c u l o s k e l e t a l P r o b l e ms > 1 2 . 3 - H i p P a i n
12.3
Hip Pain
Sam ue l B. Adkins I I I
I . Background
T h e h i p j o i n t i s t h e mo s t i n a c c e s s i b l e j o i n t i n t h e a n y o f t h e l i mb s . I t i s l o c a t e d d e e
w i t h i n t h e l a r g e mu s c l e s o f t h e h i p , p e l v i s , a n d t h i g h . I t a l s o h a s mo r e b o n y s u p p o r t
than any other joint.
T h e d i f f e r e n t i a l d i a g n o s i s f o r t h e p a t i e n t w i t h h i p p a t hT oa lbol ge y1(2s. e3)e. 1i s
significantly influenced by age. T he vascular supply to the hip is subject to
d i s r u p t i o n t h a t c a n l e a d t o a v a s c u l a r n e c r o s i s o f t h e f e mo r a l h e a d . T h i s i s
p a r t i c u l a r l y i mp o r t a n t i n c h i l d r e n a n d a d o l e s c e n t s , i n w h o m t h e p r e s e n c e o f a c t i v e
growth centers results in diagnoses specific to people in those age-groups. Both
g e n e t i c a n d e n v i r o n me n t a l f a c t o r s c a n a l s o l e a d t o d e g e n e r a t i v e c h a n g e s i n t h e h i p
I I I . Evaluation
A. History
P a t i e n t s ma y p r e s e n t w i t h a c o mp l a i n t o f p a i n , s n a p p i n g ( l a t e r a l s n a p p i n g s u g g e s t s
i l i o t i b i a l b a n d t i g h t n e s s , me d i a l s n a p p i n g s u g g e s t s i l i o p s o a s t i g h t n e s s ) , o r l i mp . T h
history should include specifics about the onset, location, duration, and severity of
s y mp t o ms . Q u e s t i o n i n g t h e p a t i e n t a b o u t a s s o c i a t e d g a s t r o i n t e s t i n a l s y mp t o ms
( c h a n g e i n s t o o l f r e q u e n c y, r e c t a l b l e e d i n g , a b d o mi n a l p a i n ) , g e n i t o u r i n a r y s y mp t o m
( d y s u r i a , h e ma t u r i a , me n s t r u a l i r r e g u l a r i t i e s ) , o r s y s t e mi c s y mp t o ms ( n i g h t s w e a t s ,
w e i g h t l o s s , f e v e r ) ma y a l e r t t h e p h y s i c i a n t o a l t e r n a t i v e d i a g n o s e s . S t e r o i d u s e ,
s i c k l e c e l l d i s e a s e , a n d h u ma n i mmu n o d e f i c i e n c y v i r u s i n f e c t i o n a r e a l l r i s k f a c t o r s
f o r a v a s c u l a r n e c r o s i s o f t h e f e mo r a l h e a d . T h e me n s t r u a l h i s t o r y a n d p o s s i b l y t h e
d i e t a r y h i s t o r y i n y o u n g w o me n ma y i n d i c a t e a n i n c r e a s e d l i k e l i h o o d o f o s t e o p e n i a o
stress fracture. A careful account of the patient's current and past occupational,
d o me s t i c , a n d r e c r e a t i o n a l a c t i v i t i e s i s h e l p f u l b o t h d i a g n o s t i c a l l y a n d i n p l a n n i n g f
t r e a t me n t , r e h a b i l i t a t i o n , a n d r e t u r n t o n o r ma l a c t i v i t i e s .
P. 2 7 4
TAB L E 12.3.1 Differential Diagnosis of Hip pain

452 / 652
tmdmss
12.3 - Hip Pain
Diagnosis
Patie nt age
S eptic arthritis Infants and

toddlers
Sugge stiv e findings

P s e u d o p a r a l y s i s , i r r i t a b i l i t y, l i mp
Transient
synovitis
Child,
P a i n , l i mp , l i mi t e d R O M
preadolescent
L egg-C alv'Perthes
disease
P r e a d o l e s c e n t L i mp a n d l i mi t e d R O M
S lipped capital Adolescent

f e mo r a l
epiphysis
O b e s i t y, ma l e s , p a i n , l i mi t e d i n t e r n a l
rotation
Av u l s i o n
fracture
Sudden onset, pop heard or felt with

i n j u r y, p a i n a t i n s e r t i o n s i t e
Yo u n g a d u l t
F e mo r a l n e c k Yo u n g a d u l t
stress fracture
Insidious onset, associated with

increased activity or disordered eating,
me n s t r u a l i r r e g u l a r i t i e s , p a i n f u l R O M
Osteoid
o s t e o ma
Yo u n g a d u l t
Va g u e p a i n , n o c t u r n a l p a i n , d e c r e a s e d
ROM
Iliotibial band
s y n d r o me
Yo u n g a d u l t
Lateral thigh/leg pain/snapping, positive

O ber's test
Trochanteric
bursitis
Adult
L a t e r a l t h i g h p a i n l o c a l i ze d o v e r g r e a t e r
trochanter
Av a s c u l a r
Adult
necrosis of the
f e mo r a l h e a d
Dull pain with weight bearing,

decreased R O M
Iliopsoas
bursitis
Adult
Medial thigh pain/snapping, pain with

standing
Meralgia
paresthetica
Adult
A n t e r i o r / l a t e r a l t h i g h n u mb n e s s , o b e s i t y,
c o mp r e s s i v e c l o t h i n g , n o w e a k n e s s
Degenerative
joint disease
Adult
Progressive pain, decreased internal

rotation early in the disease, pain with
weight bearing
453 / 652
tmdmss
12.3 - Hip Pain
R O M , r a n g e o f mo t i o n .
A l t h o u g h t h e h i p j o i n t i s o b s c u r e d b y b o n e a n d s o f t t i s s u e s , p h y s i c a l e xa mi n a t i o n o f
t h e h i p i s n o t d i f f i c u l t . T h e e xa mi n a t i o n s h o u l d b e c a r r i e d o u t i n a me t h o d i c a l f a s h i o
a n d t h e p a t i e n t s h o u l d b e a p p r o p r i a t e l y u n d r e s s e d t o o b t a i n ma xi ma l i n f o r ma t i o n .
1. W i t h t h e p a t i e n t s i t t i n g o n t h e e xa mi n a t i o n t a b l e ( h i p s f l e xe d a t 9 0 ) , t e s t t h e
i n t e r n a l a n d e xt e r n a l r o t a t i o n o f t h e h i p . A l s o c h e c k f o r h i p f l e xo r s t r e n g t h .
2. W i t h t h e p a t i e n t l y i n g s u p i n e , t e s t a b d u c t i o n . I n t h i s p o s i t i o n , p a l p a t i o n o f t h e
g r o i n ma y l o c a l i ze t h e a n t e r i o r h i p p a i n . E xt e n s i o n i s t e s t e d w i t h t h e p a t i e n t l y i n
o n t h e c o n t r a l a t e r a l s i d e . O b e r ' s t e s t f o r i l i o t i b i a l b a n d f l e xi b i l i t y a n d p a l p a t i o n
t h e g r e a t e r t r o c h a n t e r a r e a i s a l s o p e r f o r me d w i t h t h e p a t i e n t i n1)t h. i s p o s i t i o n (
3. H a v e t h e p a t i e n t s t a n d f r o m a s i t t i n g p o s i t i o n , i f a b l e . O b s e r v e f o r s i g n s o f
d i s c o mf o r t o r d y s f u n c t i o n . I n s p e c t t h e p a t i e n t ' s s t a n c e . I s t h e p a t i e n t a b l e t o
b e a r w e i g h t w i t h o u t p a i n ? H o w s t a b l e i s t h e s t a n c e ? A r e t h e l o w e r e xt r e mi t i e s
s y mme t r i c a l ( mu s c l e s a n d a l i g n me n t ) ? N e xt w a t c h t h e p a t i e n t w a l k ( s l o w l y f i r s t
t h e n b r i s k l y i f a b l e ) . I s t h e g a i t s y mme t r i c a l ? I s t h e g a i t p h a s e s h o r t e n e d ? T h i s
ma y i n d i c a t e j o i n t p a t h o l o g y. I s t h e r e a l u r c h i n g ( t h e p a t i e n t l i mi t i n g t i me s p e n t
P. 2 7 5
o n t h e a f f e c t e d h i p a n d t h e l i mi t e d r a n g e o f mo t i o n s u g g e s t h i p j o i n t p a t h o l o g y )
or a Trendelenburg (weak abductors causing the pelvis to tilt) gait? Does the
p a t i e n t c i r c u md u c t t h e h i p ( s e e n w i t h r e s t r i c t e d mo t i o n ) ?
4. E xa mi n a t i o n o f t h e k n e e ( e s p e c i a l l y i n c h i l d r e n ) , l u mb a r s p i n e , a n d s a c r o i l i a c
joints should also be considered.
C. Testing
1. P l a i n x- r a y s ( a n t e r o p o s t e r i o r p e l v i s , a n t e r o p o s t e r i o r a n d f r o g - l e g o r l a t e r a l
v i e w s ) a r e i n d i c a t e d f o r p a t i e n t s w i t h g r o i n o r a n t e r i o r t h i g h p a i n , w h o a r e mo r e
l i k e l y t o h a v e h i p j o i n t p a t h o l o g y, a s w e l l a s f o r c a s e s i n w h i c h t h e r e i s s u s p i c i o
o f s i g n i f i c a n t p a t h o l o g y, i n a b i l i t y t o b e a r w e i g h t , u n c l e a r d i a g n o s i s , o r l a c k o f
r e s p o n s e t o t h e r a p y.
2. M a g n e t i c r e s o n a n c e i ma g i n g ( M R I ) o r c o mp u t e d t o mo g r a p h y i s h e l p f u l i f t h e
diagnosis is unclear or a fracture is still suspected after plain radiographs, if a
loose body or labral injury is suspected, and in the evaluation of cystic or lytic
lesions.
3. U l t r a s o u n d c a n b e h e l p f u l i n d e t e c t i n g s ma l l j o i n t e f f u s i o n s w h e n t h e s u s p i c i o n
of transient synovitis or septic arthritis is high. Bone scanning can also be
useful in diagnosing early Legg-Calv-Perthes disease or aseptic necrosis,
a l t h o u g h g a d o l i n i u m- c o n t r a s t M R I ma y b e p r e f e r a b l e i f p r a c t i c a l a n d a v a i l a b l e .
4. C o mp l e t e b l o o d c o u n t , e r y t h r o c y t e s e d i me n t a t i o n r a t e , a n d C - r e a c t i v e p r o t e i n
454 / 652
tmdmss
12.3 - Hip Pain
l e v e l s c a n b e h e l p f u l i n e v a l u a t i n g p a t i e n t s w i t h s u s p e c t e d i n f l a mma t o r y
disorders.
5. O c c a s i o n a l l y, j o i n t a s p i r a t i o n i s n e e d e d t o ma k e a d i a g n o s i s . F l u i d s h o u l d b e
sent for cell count, gram stain, culture, crystals, and other studies as
appropriate.
I V. Diagnosis
T a b l e 1 2 . 3 .l1i s t s t h e d i f f e r e n t i a l d i a g n o s i s f o r c o mmo n h i p c o n d i t i o n s .
References
1 . H o p p e n f e l d S t a n l ePy.
hy s i c al ex ami nat i on of t he s pi ne and ex t.r emi
N e wt i es
Yo r k , N Y : A p p l e t o n - C e n t u r y - C r o f t s , 1 9 7 6 : 1 3 3 1 6 9 .
455 / 652
tmdmss
12.4 - Knee Pain
> T a b l e o f C o n t e n t s > 1 2 - M u s c u l o s k e l e t a l P r o b l e ms > 1 2 . 4 - K n e e P a i n
12.4
Knee Pain
Chris Madde n
I . Background
K n e e p a i n i s o n e o f t h e mo s t f r e q u e n t mu s c u l o s k e l e t a l c o mp l a i n t s i n p r i ma r y c a r e . A
c a r e f u l h i s t o r y, f o c u s e d p h y s i c a l e xa mi n a t i o n , a n d o c c a s i o n a l d i a g n o s t i c s t u d i e s
a l l o w f o r a c c u r a t e d i a g n o s i s o f mo s t k n e e p r o b l e ms .
A. Etiology
C a u s e s o f k n e e p a i n ma y i n c l u d e o v e r u s e a n d t r a u ma t i c i n j u r i e s , d e g e n e r a t i v e a n d
i n f l a mma t o r y a r t h r i t i s , i n f e c t i o n , o s t e o n e c r o s i s a n d o s t e o c h o n d r i t i s , r e f e r r e d p a i n
f r o m t h e h i p a n d b a c k , a n d o t h e r mi s c e l l a n e o u s d1,2)
i s o. r d e r s (
B. Epidemiology
1. K n e e p a i n i n c h i l d h o o d , e s p e c i a l l y e a r l y, i s u s u a l l y c a u s e d b y mi n o r t r a u ma .
M a j o r t r a u ma ma y r e s u l t i n g r o w t h p l a t e o r o t h e r f r a c t u r e s , w h e r e a s
i n f l a mma t o r y a r t h r i t i s ( e . g . , j u v e n i l e r h e u ma t o i d a r t h r i t i s ) a n d i n f e c t i o n a r e l e s s
f r e q u e n t c a u s e s . A c t i v e c h i l d r e n , e s p e c i a l l y a s t h e y ma t u r e i n t o p r e a d o l e s c e n c e
a n d b e c o me mo r e i n v o l v e d i n d e ma n d i n g s p o r t s , ma y e xp e r i e n c e o v e r u s e
injuries. Apophysitises such as tibial apophysitis ( O sgood- S chlatter disease) ar
s t r e s s i n j u r i e s t o g r o w t h p l a t e s t h a t a r e o f t e n mi s t a k e n f o r i n f l a mma t o r y i n j u r i e s
a n d t r e a t e d w i t h a n t i - i n f l a mma t o r y me d i c a t i o n s i n s t e a d o f a p p r o p r i a t e r e s t .
2. O v e r u s e a n d t r a u ma t i c i n j u r i e s t o l i g a me n t s , t e n d o n s , a n d c a r t i l a g e ma y o c c u r i
p e o p l e o f a n y a g e a n d a r e c o mmo n i n a c t i v e a d o l e s c e n t s a n d a d u l t s . I t i s
P. 2 7 6
a c o mmo n mi s c o n c e p t i o n t h a t ma t u r i n g y o u n g s t e r s a l mo s t a l w a y s s u s t a i n a n
a v u l s i o n f r a c t u r e o f b o n e o r a g r o w t h p l a t e r a t h e r t h a n r u p t u r e a l i g a me n t
b e c a u s e t h e l i g a me n t i s s t r o n g e r. Yo u n g a t h l e t e s c a n r u p t u r e l i g a me n t s i n p a r
b e c a u s e t h e ma t u r i n g g r o w t h p l a t e i s v u l n e r a b l e t o i n j u r y, t h o u g h r u p t u r e i s l e s s
c o mmo n i n t h e m t h a n i n a d u l t s .
TAB L E 12.4.1 Differential Diagnosis of Knee

Pain
456 / 652
tmdmss
Pain
12.4 - Knee Pain
Overuse
P a t e l l o f e mo r a l p a i n , p a t e l l a r t e n d o p a t h y, q u a d r i c e p s
t e n d i n o p a t h y, i l i o t i b i a l b a n d s y n d r o me , a p o p h y s i t i s
( O s g o o d - S c h l a t t e r mo s t c o mmo n ) , p e s a n s e r i n e
bursitis, synovial plica, bipartite patella
T r a u ma t i c
A n t e r i o r c r u c i a t e l i g a me n t r u p t u r e , c o l l a t e r a l l i g a me n t
s p r a i n ( me d i a l a n d l a t e r a l c o l l a t e r a l ) , f r a c t u r e ( b o n y
a n d / o r c h o n d r a l ) , me n i s c a l t e a r, p a t e l l a r s u b l u xa t i o n o r
dislocation, prepatellar bursitis, posterior cruciate
l i g a me n t r u p t u r e , q u a d r i c e p s t e n d o n r u p t u r e , p a t e l l a r
tendon rupture
Arthritic
O s t e o a r t h r i t i s , i n f l a mma t o r y ( r h e u ma t o i d , g o u t ,
pseudogout, other)
Infectious
Septic joint
Referred
Back or hip
Miscellaneous Osteonecrosis, osteochondritis desiccans, Baker

( p o p l i t e a l ) c y s t , t u mo r, d e e p v e n o u s t h r o mb o s i s
( p r e s e n t i n g a s p o p l i t e a l p a i n ) , b e n i g n o r ma l i g n a n t
t u mo r, p i g me n t e d v i l l o n o d u l a r s y n o v i t i s
O t h e r s y s t e mi c c a u s e ( c o mp l e x r e g i o n a l p a i n
s y n d r o me , f i b r o my a l g i a )
3. A d u l t s a n d s e n i o r s f r e q u e n t l y e xp e r i e n c e p a i n f r o m k n e e o s t e o a r t h r i t i s . L e s s
f r e q u e n t l y, t h e c a u s e ma y b e o t h e r d e g e n e r a t i v e i n t r a - a r t i c u l a r p a t h o l o g y s u c h
a s me n i s c a l t e a r s o r c h o n d r a l l e s i o n s . I n f l a mma t o r y a r t h r i t i s , i n c l u d i n g g o u t a n d
p s e u d o g o u t , a r e mo r e c o mmo n i n a d u l t s t h a n i n y o u n g e r p a t i e n t s .
4. A v a r i e t y o f o t h e r mi s c e l l a n e o u s c o n d i t i o n s ma y c a u s e k n e e p a i n i n p e o p l e o f a l
a g e s ( s e eT a b l e 1 2 . 4). ,1 a n d t h e s e s h o u l d b e c o n s i d e r e d i f p a i n p a t t e r n i s
a t y p i c a l , d i f f u s e , o c c u r s a t n i g h t , o r p e r s i s t s d e s p i t e t r e a t me n t .
I I I . Evaluation
Attention to patient age, injury specifics, and pain characteristics and pattern help
g u i d e a d e t a i l e d a n d c a r e f u l k n e e p a i n 3)
h i.s t o r y (
A. History
1. I f t h e r e i s a h i s t o r y o f i n j u r y, d e t e r mi n i n g t h e me c h a n i s m o f i n j u r y i s t h e f i r s t t a s
i n o b t a i n i n g a h i s t o r y. I d e n t i f i c a t i o n o f k n e e p o s i t i o n a n d f o r c e s i n v o l v e d h e l p s
457 / 652
tmdmss
12.4 - Knee Pain
f o c u s t h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n . I f a p a t i e n t d o e s n o t r e c a l l t h e
me c h a n i s m, o r t h e r e w a s n o o b v i o u s i n j u r y, t h i s i s a l s o v a l u a b l e i n f o r ma t i o n t h a
narrows the differential diagnosis.
2. M o s t k n e e c o n d i t i o n s b e c o me a p p a r e n t a f t e r t a k i n g a h i s t o r y t h a t f o c u s e s o n
p a i n c h a r a c t e r i s t i c s . T h e mn e mo n i c C h L O R I D E ma y h e l p e l i c i t a c o mp r e h e n s i v e
h i s t o r y.
a. C h : p a i nc h a r a c t e rt h a t i s d e s c r i b e d a s b u r n i n g o r a c h i n g f r e q u e n t l y
r e p r e s e n t s p a t e l l o f e mo r a l p a i n o r o s t e o a r t h r i t i s . T r a u ma t i c l i g a me n t o u s p a i n
ma y b e s h a r p i n i t i a l l y a n d w i t h s t r e s s b u t b e a c h i n g a t r e s t . D r a ma t i c o r
i n t e n s e p a i n a n d n i g h t p a i n ma y r e p r e s e n t a s y s t e mi c p r o c e s s . S t i f f n e s s ,
e s p e c i a l l y a f t e r r e s t , o c c u r s w i t h p a t e l l o f e mo r a l p a i n a n d o s t e o a r t h r i t i s .
b. L : p a i nl o c a t i o nma y b e d e mo n s t r a t e d b y h a v i n g t h e p a t i e n t p o i n t t o t h e
mo s t p a i n f u l a r e a . R e l a t i v e l y l o c a l i ze d p a i n i mme d i a t e l y n a r r o w s t h e
d i f f e r e n t i a l d i a g n o s i s t o i n c l u d e r e g i o n a l s t r u c t u r e s . G e n e r a l i ze d p a i n ma y
present a challenge, but having the patient trace the pain pattern using
h i s o r h e r f i n g e r s ma y n a r r o w c o n s i d e r a t i o n s . C i r c u l a r mo t i o n s a t t h e
a n t e r i o r k n e e o f t e n r e p r e s e n t s p a t e l l o f e mo r a l p r o b l e ms , w h e r e a s h o r i zo n t a l
mo t i o n s a l o n g t h e me d i a l o r l a t e r a l j o i n t l i n e s a r e c o mmo n w i t h me n i s c a l
tears and
P. 2 7 7
o s t e o a r t h r i t i s . S w e e p s o v e r s p e c i f i c l i g a me n t o r t e n d o n a r e a s ma y i n d i c a t e
pathology involving these structures.
c. O : p a i no n s e .t D e t e r mi n e i f t h e p a i n b e g a n a c u t e l y o r i n s i d i o u s l y. A c u t e
o n s e t i mp l i e s t i s s u e t e a r i n g , b r u i s i n g , o r f r a c t u r e . I n s i d i o u s o n s e t i mp l i e s
t i s s u e i n f l a mma t i o n o r s t r e s s i n j u r y, o f t e n f r o m o v e r u s e .
d. R : p a i nr a d i a t i o nc a n g i v e c l u e s t o i n v o l v e d s t r u c t u r e s . P a t e l l o f e mo r a l p a i n
ma y r a d i a t e a l o n g me d i a l o r l a t e r a l p a t e l l a r r e t i n a c u l u m o r r e t r o p a t e l l a r
a r e a . H a ms t r i n g t e n d o n i t i s ma y r a d i a t e i n t o d i s t a l p o s t e r i o r t h i g h . M e n i s c a l
p a t h o l o g y c a n r a d i a t e p a i n a l mo s t a n y w h e r e i n t h e k n e e , b u t f r e q u e n t l y
r a d i a t e s p o s t e r i o r l y o r t o t h e j o i n t l i n e . D i s t a l o r p r o xi ma l p a i n s h o u l d r a i s e
the suspicion for nonknee pathology such as referred pain from the back or
hip.
e. I : p a i ni n t e n s i t yma y p r o v i d e c l u e s t o d i a g n o s i s . S e v e r e p a i n o c c u r s w i t h
l i g a me n t o r t e n d o n r u p t u r e , f r a c t u r e s , a n d g o u t . L o w t o mo d e r a t e i n t e n s i t y
p a i n ma y b e a s s o c i a t e d w i t h a n o v e r u s e i n j u r y.
f. D : p a i nd u r a t i o ni s a s s e s s e d a l o n g s i d e p a i n o n s e t . S h o r t e r d u r a t i o n p a i n
c o r r e l a t e s mo s t f r e q u e n t l y w i t h r e c e n t t r a u ma t i c o r i n f l a mma t o r y i n j u r y,
w h e r e a s me d i u m a n d l o n g d u r a t i o n p a i n i s o b s e r v e d w i t h o v e r u s e i n j u r i e s
s u c h a s t e n d i n o p a t h y. P r o l o n g e d p a i n , p a r t i c u l a r l y i f i t i s n o t r e s p o n d i n g t o
t r e a t me n t , s h o u l d p r o mp t a n a g g r e s s i v e s e a r c h f o r l e s s c o mmo n s e r i o u s
c a u s e s o f k n e e p aTi na b( l e 1 2 . 4). .1
g. E : p a i ne x a c e r b a t i o nasn d r e mi t t i n g f e a t u r e s a r e i mp o r t a n t . A n t e r i o r k n e e
p a i n a n d / o r s t i f f n e s s a f t e r s i t t i n g w i t h k n e e s f l e xe d i s f r e q u e n t w i t h
p a t e l l o f e mo r a l p a i n a n d i s r e f e r r e d t o a s t h e mo v i e g o e r s s i g n . S q u a t t i n g
458 / 652
tmdmss
12.4 - Knee Pain
a n d k n e e f l e xi o n ma y e xa c e r b a t e p a t e l l o f e mo r a l p a i n b u t c a n a l s o c a u s e
me d i a l o r l a t e r a l j o i n t l i n e p a i n t h a t ma y r e p r e s e n t me n i s c a l o r o t h e r i n t r a a r t i c u l a r p a t h o l o g y. C u t t i n g , t w i s t i n g , a n d p i v o t i n g w o r s e n s c o l l a t e r a l
l i g a me n t p a i n a n d ma y c a u s e a s e n s e o f i n s t a b i l i t y o r s l i d i n g w i t h c r u c i a t e
l i g a me n t i n j u r y. J u mp i n g a n d r u n n i n g w o r s e n s p a i n w i t h p a t e l l o f e mo r a l ,
patellar tendon, pes anserine bursa, and other tendon or overuse injuries.
Stairs and uneven surfaces or hills can worsen iliotibial band and
p a t e l l o f e mo r a l p a i n , w h i c h f r e q u e n t l y o c c u r t o g e t h e r. P a i n w i t h o s t e o a r t h r i t i s
ma y w o r s e n a f t e r p e r i o d s o f r e s t a n d a t t h e e n d o f t h e d a y b u t c a n e i t h e r
i mp r o v e o r w o r s e n w i t h a c t i v i t y, d e p e n d i n g o n t h e s e v e r i t y. N i g h t p a i n ma y
i n d i c a t e t u mo r o r c o mp l e x r e g i o n a l p a i n s y n d r o me . I f p a i n a n d s t i f f n e s s a r e
mo s t s e v e r e u p o n r i s i n g i n t h e mo r n i n g , i n f l a mma t o r y a r t h r i t i s s h o u l d b e
considered.
3. A s s o c i a t e d s y mp t o ms . A n i mp o r t a n t f e a t u r e o f k n e e p a i n i s t h e p r e s e n c e o f
me c h a n i c a l s y mp t o ms , w h i c h i n c l u d e c a t c h i n g a n d l o c k i n g . M e c h a n i c a l
s y mp t o ms o f t e n r e p r e s e n t a n u n s t a b l e s t r u c t u r a l l e s i o n s u c h a s d i s p l a c e d
me n i s c a l t e a r s o r i n t r a - a r t i c u l a r, c h o n d r a l , o r b o n y f r a c t u r e s a n d l o o s e b o d i e s
t h a t ma y ma n d a t e e a r l y o r t h o p a e d i c r e f e r r a l . A s e n s a t i o n o f g i v i n g w a y i s mo s
f r e q u e n t l y a r e s u l t o f r e f l e x i n h i b i t i o n d u e t o p a t e l l o f e mo r a l p a i n . H o w e v e r,
g i v i n g w a y ma y a l s o r e p r e s e n t u n d e r l y i n g k n e e i n s t a b i l i t y s u c h a s t h a t o c c u r r i n g
w i t h a n a n t e r i o r, o r l e s s f r e q u e n t l y, a p o s t e r i o r c r u c i a t e l i g a me n t t e a r. N o t e o t h e
p e r t i n e n t d i s t a l o r p r o xi ma l e xt r e mi t y o r b a c k s y mp t o ms a n d o t h e r s y s t e mi c o r
c o n s t i t u t i o n a l s y mp t o ms .
4. P a s t me d i c a l a n d f a mi l y h i s t o r y. I n q u i r e a b o u t p r i o r i n j u r y, i n s t a b i l i t y, a n d
n o n t r a u ma t i c k n e e p a i n . I s t h e p a t i e n t o n a n y me d i c a t i o n s t h a t ma y mo d i f y k n e e
p a i n ? A n y p r i o r k n e e o p e r a t i o n s ? E xp l o r e a f a mi l y h i s t o r y o f k n e e p a i n , w h i c h
ma y r e p r e s e n t p a t e l l o f e mo r a l p a i n o r o s t e o a r t h r i t i s .
1. O b s e r v e t h e g a i t . E xa mi n e b o t h t h e a f f e c t e d a n d n o n a f f e c t e d k n e e s t o e s t a b l i s h
a n o r ma l b a s e l i n e . F o c u s o n s t r u c t u r e s h i g h l i g h t e d i n t h e h i s t o r y. I n s p e c t f o r
a s y mme t r y, e xt r a - a r t i c u l a r s w e l l i n g , i n t r a - a r t i c u l a r s w e l l i n g ( e f f u s i o n ) , a n d
a n t a l g i c g a i t . M o s t a c u t e e f f u s i o n s a r e h e ma r t h r o s e s a n d r e p r e s e n t a n t e r i o r
c r u c i a t e l i g a me n t ( A C L ) t e a r s . P a l p a t e i n j u r e d s t r u c t u r e s a n d n o t e t h e
t e n d e r n e s s , t h i c k e n i n g , a n d d e f e c t s t o l o c a l i ze d i a g n o s t i c c o n s i d e r a t i o n s .
A s s e s s t h e p a s s i v e a n d a c t i v e r a n g e s o f mo t i o n a n d mu s c l e s t r e n g t h . S i g n i f i c a n
l o s s o f mo t i o n o c c u r s
P. 2 7 8
w i t h i n t r a - a r t i c u l a r s t r u c t u r a l l e s i o n s , a n d r e f u s a l t o mo v e t h e k n e e ma y b e d u e
to severe pain associated with a septic joint.
2. S p e c i f i c t e s t i n g i s h e l p f u l w i t h a v a r i e t y o f i n j u r i e s a n d d y s f u n c t i o n s . T h e
p a t e l l o f e mo r a l c o mp r e s s i o n a n d s h r u g t e s t s i n v o l v e c o mp r e s s i n g t h e p a t e l l a i n t o
t h e t r o c h l e a r g r o o v e o f t h e f e mu r. T h i s ma n e u v e r e l i c i t s p a i n w i t h p a t e l l o f e mo r a
p r o b l e ms . T h e L a c h ma n t e s t i n v o l v e s a n a n t e r i o r l y d i r e c t e d f o r c e a p p l i e d t o t h e
p r o xi ma l t i b i a w h i l e s t a b i l i zi n g t h e f e mu r a t 2 0 o f k n e e f l e xi o n , a n d i t r e v e a l s
459 / 652
tmdmss
12.4 - Knee Pain
i n c r e a s e d a n t e r i o r t r a n s l a t i o n o f t h e t i b i a o n t h e f e mu r a n d a s o f t o r a b s e n t e n d
p o i n t w i t h A C L t e a r s . T h e M c M u r r a y t e s t i n v o l v e s a c i r c u md u c t i o n ma n e u v e r o f
t h e f l e xe d k n e e . A p o s i t i v e t e s t e l i c i t s j o i n t l i n e p a i n a n d / o r p o p p i n g w i t h
me n i s c a l a n d s o me t i me s w i t h a r t i c u l a r c a r t i l a g e p a t h o l o g y. T h e v a r u s a n d v a l g u
s t r e s s t e s t s a t 3 0 c a u s e j o i n t l i n e o p e n i n g w i t h l a t e r a l a n d me d i a l c o l l a t e r a l
l i g a me n t i n j u r i e s , r e s p e c t i v e l y. T h e s e t e s t s p e r f o r me d a t 0 ma y i n d i c a t e d a ma g
t o t h e p o s t e r i o r c r u c i a t e l i g a me n t . T h e p o s t e r i o r d r a w e r t e s t i s p e r f o r me d b y
a p p l y i n g a p o s t e r i o r f o r c e t o t h e p r o xi ma l t i b i a w i t h t h e k n e e f l e xe d a t 9 0 a n d
t h e h i p f l e xe d a t 4 5 , a n d i t r e v e a l s i n c r e a s e d p o s t e r i o r t i b i a l t r a n s l a t i o n w i t h
p o s t e r i o r c r u c i a t e l i g a me n t i n j u r i e s .
C. Testing
1. T h e a p p l i c a t i o n o f c l i n i c a l d e c i s i o n r u l e s ma y h e l p p r e v e n t t h e o v e r u s e o f p l a i n
r a d i o g r a p h y t o d e t e c t f r a c t u r e s . T h e P i t t s b u r g h K n e e R u l e ma y b e u s e d i n
adults and children older than 6 years of age, whereas the Ottawa Rule is
d i s c o u r a g e d i n p e d i a t r i c p o p u l a4)
t i .o nTsh e( P i t t s b u r g h R u l e r e c o mme n d s a
r a d i o g r a p h f o r a n y o n e w i t h a f a l l o r b l u n t t r a u ma , a n y o n e y o u n g e r t h a n 1 2 y e a r s
of age or older than 50 years of age, and anyone unable to take four weightb e a r i n g s t e p s i n t h e e me r g e n c y d e p a r t me n t a f t e r i n j u r y ( w i t h a c u t e e v a l u a t i o n ) .
O n e s t u d y c l a i ms t h a t t h e r u l e i s 9 9 % s e n s i t i v e a n d 6 05)
%. sApsei dc ief i fcr o( m
the decision rules, plain radiography should always be considered with an acute
knee effusion to assess for intra-articular fracture.
2. M a g n e t i c r e s o n a n c e i ma g i n g ( M R I ) i s t h e b e s t i ma g i n g mo d a l i t y f o r a s s e s s i n g
ma j o r l i g a me n t a n d t e n d o n i n j u r i e s a n d i s mo s t f r e q u e n t l y u s e d t o c o n f i r m A C L
r u p t u r e . I t i s u s u a l l y i n d i c a t e d w i t h a n a c u t e k n e e e f f u s i o n a n d f o r me c h a n i c a l
s y mp t o ms n o t a d e q u a t e l y e xp l a i n e d b y p l a i n r a d i o g r a p h y. C h r o n i c p a i n a n d
chronic effusion are less frequent indications for M R I. Other potentially helpful
d i a g n o s t i c s t h a t ma y b e a p p l i e d o n a n i n d i v i d u a l i ze d b a s i s i n c l u d e c o mp u t e d
t o mo g r a p h y, a r t h r o g r a p h y, b o n e s c a n , u l t r a s o u n d , a n d a r t h r o c e n t e s i s .
I V. Diagnosis
A c c u r a t e d i a g n o s i s o f k n e e p a t h o l o g y r e l i e s o n t h e a b i l i t y o f t h e e xa mi n e r t o l o c a l i z
t h e i n j u r y u s i n g t h e h i s t o r y o f t h e i n j u r y me c h a n i s m a n d p a i n c h a r a c t e r i s t i c s t h a t
g u i d e a f o c u s e d k n e e e xa mi n a t i o n t o a d d r e s s t h e a f f e c t e d s t r u c t u r e s u s i n g s p e c i f i c
t e s t i n g . A n t e r i o r k n e e p a i n i s u s u a l l y p a t e l l o f e mo r a l , w h i c h i s b y f a r t h e mo s t c o mm
k n e e c o mp l a i n t i n t h e p r i ma r y c a r e p h y s i c i a n ' s o f f i c e . A n a c u t e t r a u ma t i c e f f u s i o n i s
a n A C L i n j u r y u n t i l p r o v e d o t h e r w i s e ; j o i n t l i n e p a i n o f t e n r e p r e s e n t s me n i s c a l
p a t h o l o g y ; a n d p a i n o f i n s i d i o u s o n s e t o c c u r r i n g a l o n g t e n d o n s i mp l i e s t e n d i n o p a t h y
P r o l o n g e d o r a t y p i c a l p a i n w a r r a n t s i mme d i a t e a n d a g g r e s s i v e e v a l u a t i o n t o r u l e o u
s e r i o u s p a t h o l o g y.
References
1 . Wa l s h W, Va n i c e k J . K n e e i n j u r i e s . I n : M e l l i o n M , Wa l s h W, M a d d e n C , e t a l . ,
e d s . T he t eam phy s i c i an' s handbook
, 3 r d e d . P h i l a d e l p h i a , PA : E l s e v i e r S c i e n c e ,
460 / 652
tmdmss
12.4 - Knee Pain
2002:490509.
2 . C a l mb a c h W, H u t c h e n s M . E v a l u a t i o n o f p a t i e n t s p r e s e n t i n g w i t h k n e e p a i n :
P a r t I . h i s t o r y, p h y s i c a l e xa mi n a t i o n , r a d i o g r a p h s , a n d l a b oAr m
a t oFr am
y tests.
P hy s i c i an2 0 0 3 ; 6 8 : 9 0 7 9 1 2 .
3 . C a l mb a c h M , H u t c h e n s M . E v a l u a t i o n o f p a t i e n t s p r e s e n t i n g w i t h k n e e p a i n :
P a r t I I . d i f f e r e n t i a l d i a g nAom
s i sF. am P hy s i c i an
2003;68:917922.
4 . E b e l l M . A t o o l f o r e v a l u a t i n g p a t i e n t s w i t h k nAe m
e iFn am
j u r y.P hy s i c i an
2 0 0 5 ; 1 2 : 11 6 9 11 7 2 .
5 . S e a b e r g D , Ye a l y D , L u k e n s T , e t a l . M u l t i c e n t e r c o mp a r i s o n o f t w o c l i n i c a l
d e c i s i o n r u l e s f o r t h e u s e o f r a d i o g r a p h y i n a c u t e , h i g h - rAi snn
k iEn mer
j u r i egs .
M ed 1 9 9 8 ; 3 2 : 8 1 3 .
461 / 652
tmdmss
12.5 - Low Back Pain
> T a b l e o f C o n t e n t s > 1 2 - M u s c u l o s k e l e t a l P r o b l e ms > 1 2 . 5 - L o w B a c k P a i n
12.5
Low Back Pain
Ronald McCoy
I . Background
L o w b a c k p a i n i s c o mmo n i n d e v e l o p e d c o u n t r i e s , a f f e c t i n g a p p r o xi ma t e l y 7 0 % o f t h
a d u l t p o p u l a t i o1)
n (a t s o me s t a g e d u r i n g t h e i r l i f e .
T h e c a u s e o f p a i n i s n o n s p e c i f i c i n a p p r o xi ma t e l y 9 5 % o f p e o p l e p r e s e n t i n g w i t h
acute low back pain; serious conditions are rare. T he condition is generally selfl i mi t e d 2)( b u t d i a g n o s i s mu s t e xc l u d e r a r e r b u t p o t e n t i a l l y s e r i o u s , l i f e - t h r e a t e n i n g
c a u s e s ( s eTea b l e 1 2 . 5). .1
I I I . Evaluation
A. History
T he clinician needs to be alert to the presence of indications of potentially serious
l o w b a c k c o n d i t i o n s , o f t e n c a l l e d r e d f l aTgas b l e( s 1e 2e . 5). .2
1. P a i n c h a r a c t e r i s t iAcsss e s s t h e n a t u r e o f t h e p a i n , a l o n g w i t h t h e p o s i t i o n ,
o n s e t , a n d d u r a t i o n o f t h e s y mp t o m. I s t h e r e a n y r a d i a t i n g p a i n , l e g w e a k n e s s ,
o r p a r e s t h e s i a ? D o e s t h e p a i n l i mi t t h e p a t i e n t p h y s i c a l l y o r s o c i a l l y ? I s t h e r e a
h i s t o r y o f p r e v i o u s b a c k p r o b l e ms o r b a c k s u r g e r y ?
2. R e v i e w o f s y s t e mL so o k f o r a n y r e d f l a g i n d i c a t o r s o f s e r i o u s Tdai bs el ea s e (
1 2 . 5 . 2). G a s t r o i n t e s t i n a l a n d g e n i t o u r i n a r y s y mp t o ms a r e p a r t i c u l a r l y i mp o r t a n t ,
especially incontinence.
TAB L E 12.5.1 causes of Low Back Pain

Com m on cause s
Muscle and soft tissue strain
Degenerative disease, including osteoarthritis and spondylosis
Ve r t e b r a l d y s f u n c t i o n , i n c l u d i n g f a c e t j o i n t a n d l u mb a r d i s c i n v o l v e me n t
L u mb a r o r s a c r a l n e r v e r o o t c o mp r e s s i o n : d i s c h e r n i a t i o n , c a u d a e q u i n a
s y n d r o me , s c i a t i c a , s p i n a l s t e n o s i s
462 / 652
tmdmss
Ve r t e b r a l f r a c t u r e o r s u b l u xa t i o n
I n f l a mma t o r y c o n d i t i o n s
R h e u ma t o l o g i c c o n d i t i o n s ( e . g . , r h e u ma t o i d a r t h r i t i s , a n k y l o s i n g
spondylitis)
Sacroiliac joint sprain or degenerative disease
Othe r le ss com m on cause s that m ay also be life thre ate ning
I n f e c t i o n : o s t e o my e l i t i s , d i s c i t i s , e p i d u r a l a b s c e s s
H e ma t o l o g i c
M u l t i p l e my e l o ma , my e l o d y s p l a s i a
C a n c e r ( p r i ma r y o r me t a s t a t i c )
B e n i g n t u mo r s
Aortic aneurysm
Retroperitoneal pathology
Pyelonephritis, renal calculus, cancer
A b d o mi n a l p a t h o l o g y
Perforated viscus, pancreatitis
P. 2 8 0
TAB L E 12.5.2 Red Flags T hat I ndicate

Potentially Serious Lower Back Conditions
Age > 50 y
H i s t o r y o f ma l i g n a n c y
T e mp e r a t u r e > 3 7 . 8 C
Constant pain
We i g h t l o s s
H i s t o r y o f t r a u ma ( ma y b e mi n o r i n p a t i e n t s w i t h o s t e o p o r o s i s )
Features of spondyloarthropathy
Neurologic signs
Alcohol or drug disorder
Recent invasive urologic procedures
S u d d e n o n s e t o f s h a r p b a c k p a i n w i t h u n e v e n p u l s e s ( a b d o mi n a l
a n e u r y s m)
History of anticoagulant use
History of corticosteroid use
P a i n n o t i mp r o v e d a f t e r 1 mo
S i g n s o f c a u d a e q u i n a s y n d r o me
saddle anaesthesia
recent onset of bladder dysfunction
severe or progressive neurologic deficit
A d a p t e d f r o m M u r t a g hG ener
J . al pr ac t i. cAe u s t r a l i a : M c G r a w - H i l l , 2 0 0 3 .
463 / 652
tmdmss
3. P s y c h o s o c i a l i n f o r m a tLi o no k f o r a n y r e d f l a g i n d i c a t o r s o f s e r i o u s d i s e a s e
(T a b l e 1 2 . 5). .2 H a v e t h e r e b e e n a n y r e c e n t e v e n t s o r a c t i v i t i e s t h a t ma y b e
associated with the pain? If work related, assess workplace activities. Assess
u r i n a r y a n d s e xu a l f u n c t i o n , w h i c h c a n b e a f f e c t e d b y n e u r o l o g i c c o mp r o mi s e .
P s y c h i a t r i c a n d p s y c h o s o c i a l a s s e s s me n t ma y s u g g e s t t h e p r e s e n c e o f
p s y c h o g e n i c b a c k p a i n , b u t d e p r e s s i o n ma y a l s o o c c u r a s a c o n s e q u e n c e o f
c h r o n i c b a c k p a i n . D i s r u p t e d s l e e p p a t t e r n s a r e c o mmo n i n b o t h d e p r e s s i o n a n d
b a c k p a i n . P a t i e n t s s e e k i n g d r u g s o f d e p e n d e n c e ma y p r e s e n t w i t h b a c k p a i n ,
a n d a d d i c t i o n ma y h a v e r e s u l t e d f r o m p r e v i o u s b a c k p a i n t r e a t me n t . A l e g a l
h i s t o r y ma y c o mp l i c a t e t h e d i a g n o s i s a n d ma n a g e me n t o f b a c k p a i n , a n d t h e
physician should ask whether there are any legal or insurance issues under
consideration.
E xa mi n a t i o n a i ms t o i d e n t i f y t h e l o c a t i o n , l e v e l , a n d c a u s e o f d i s c o mf o r t , i n p a r t b y
r e p r o d u c i n g t h e p a i n . F o r t h i s r e a s o n , t h e mo s t p a i n f u l p a r t s o f t h e e xa mi n a t i o n a r e
l e f t t o t h e e n d o f t h e a s s e s s me n t .
1. G e n e r a lI n i t i a l i mp r e s s i o n s o f h o w t h e p a t i e n t mo v e s ma y g i v e i mp o r t a n t
d i a g n o s t i c c l u e s . P a t i e n t s w i t h d i s c l e s i o n s ma y p r e f e r t o s t a n d . G a i t c a n b e
o b s e r v e d a s t h e p a t i e n t mo v e s a b o u t t h e c o n s u l t i n g r o o m, a n d l e v e l o f
functionality and disability can be observed when the patient sits in chairs and
c l i mb s o n t o t h e e xa mi n a t i o n t a b l e . T h e p a t i e n t ' s c l o t h e s s h o u l d b e r e mo v e d t o a
mi n i mu m t o a l l o w c l o s e e xa mi n a t i o n o f t h e b a c k a n d i n s p e c t i o n o f t h e g a i t .
A b d o mi n a l e xa mi n a t i o n s h o u l d f o c u s o n p o s s i b l e c a u s e s o fT ab ba lcek p a i n (
1 2 . 5 . 1). S y s t e mi c e xa mi n a t i o n , e s p e c i a l l y n e u r o l o g i c e xa mi n a t i o n , i s a l s o
i mp o r t a n t t o e xc l u d e o t h e r s e r i o u s c a u s e s o f b a c k p a i n .
2. M u s c u l o s k e l e t a l
a. I n s p e c t i o nI n s p e c t t h e c o n t o u r a n d s h a p e o f t h e b a c k , l o o k i n g f o r s c o l i o s i s ,
l o r d o s i s , s p a s m, a n d mu s c l e w a s t i n g . A s s e s s t h e r a n g e o f mo t i o n o f t h e
s p i n e a n d l o w e r e xt r e mi t i e s t h r o u g h e xt e n s i o n , l a t e r a l f l e xi o n , a n d f l e xi o n .
Perform the straight leg raising (S L R) test passively with the patient supine
Note the
P. 2 8 1
angle of leg elevation precipitating pain. A positive test for sciatica is
buttock pain radiating to the posterior thigh and perhaps to the lower leg
a n d f o o t . T h e S L R t e s t i s u s u a l l y n e g a t i v e i n s p i n a3)
l s. t e n o s i s (
TAB L E 12.5.3 Neurologic Findings Seen with

Disc Herniation
Disc
pain/num bne ss
Functional
Motor w e akne ss m ane uv e r
Reflex
464 / 652
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L 3 - 4 / a n t e r o me d i a l Q u a d r i c e p s
thigh and knee
Deep knee
bends
Patellar
L4-5/lateral leg,
first three toes
D o r s i f l e xi o n o f
foot or great toe
Heel
walking
Achilles
L5-S1/posterior
leg, lateral heel
P l a n t a r f l e xi o n o f T o e w a l k i n g
foot or great toe
F rom D avis S . L ow back pain. In: Taylor M

R us
B , ceuld os
. k el et al
pr obl ems
. T h e 1 0 mi n u t e d i a g n o s i s ma n u a l . P h i l a d e l p h i a , PA :
L i p p i n c o t t W i l l i a ms & W i l k i n s , 2 0 0 0 .
b. P a l p a t i o nP a l p a t i o n a n d p e r c u s s i o n o f t h e s p i n e a n d u p p e r p e l v i s h e l p
i d e n t i f y a r e a s o f l o c a l i ze d t e n d e r n e s s , a s s e e n i n my o f a s c i a l c o n d i t i o n s ,
f r a c t u r e , me t a s t a t i c d i s e a s e , a n d s o me i n f l a mma t o r y c o n d i t i o n s . I n c l u d e a n
e xa mi n a t i o n o f t h e h i p a n d s a c r o i l i a c j o i n t .
3. N e u r o l o g i cN e u r o l o g i c e xa mi n a t i o n i s e s p e c i a l l y i mp o r t a n t i n t h e p r e s e n c e o f
paresthesia, weakness, and radiating pain. Assess strength by having the
patient walk on his or her heels (L 5), walk on his or her toes (S1), and testing
f o r s p e c i f i c n e r v e r o o t mo t o r, s e n s o r y, a n d r e f l e x f u n c t i o n f o r e a c h l u mb a r l e v e l .
T h e l o w e r e xt r e mi t y e xa mi n a t i o n i n c l u d e s mo t o r s t r e n g t h , d e e p t e n d o n r e f l e xe s ,
s e n s a t i o n , p r o p r i o c e p t i o n , a n d c e r t a i n f u n c t i o n a l ma n eTuavbel res 1(2s .e5e). .3
R o mb e r g a n d B a b i n s k i r e f l e xe s s h o u l d a l s o b e a s s e s s e d . R e c t a l e xa mi n a t i o n
s h o u l d a s s e s s s p h i n c t e r t o n e , w h i c h c a n b e c o mp r o mi s e d i n s a c r a l r o o t
d y s f u n c t i o n . I n t h e p r i ma r y c a r e s e t t i n g , mo s t c l i n i c a l l y s i g n i f i c a n t d i s c
h e r n i a t i o n s a r e d e t e c t e d b y t h e f o l l o w i n g l i mi t e d e xa mi n a t i o n : d o r s i f l e xi o n o f t h e
g r e a t t o e a n d a n k l e , A c h i l l e s r e f l e x, l i g h t t o u c h s e n s a t i o n o f t h e me d i a l ( L 4 ) ,
d o r s a l ( L 5 ) , a n d l a t e r a l ( S 1 ) a s p e c t o f t h e f o o t , a n d t h1,3)
e S .L R t e s t (
C. Testing
1. C l i n i c a l l a b o r a t o r y t e s t s
a. T e s t i n g i s i n f l u e n c e d b y t h e d i f f e r e n t i a l d i a g n o s i s a f t e r t h e h i s t o r y a n d
p h y s i c a l e xa mi n a t i o n . I n t h e p r e s e n c e o f r e d f l a g i n d i c a t o r s , t e s t s ma y
i n c l u d e u r i n a r y e xa mi n a t i o n , c o mp l e t e b l o o d c o u n t , e r y t h r o c y t e
s e d i me n t a t i o n r a t e , e l e c t r o l y t e s , i n c l u d i n g s e r u m c a l c i u m, s e r u m a l k a l i n e
phosphatase, or prostate-specific antigen. Pain suspected to be caused by
a r e d f l a g c o n d i t i o n ma y r e q u i r e o t h e r u r g e n t t e s t s .
b. S p e c i f i c t e s t s f o r i n f l a mma t o r y c o n d i t i o n s ( s u c h a s r h e u ma t o i d a r t h r i t i s o r
a n k y l o s i n g s p o n d y l i t i s ) o r i n f e c t i o n s ma y a l s o b e n e e d e d i f i n d i c a t e d f r o m
h i s t o r y a n d e xa mi n a t i o n .
I ng l o w - r i s k p a t i e n t s , d i a g n o s t i c i ma g i n g i s u n l i k e l y t o b e
h e l p f u l . A p o s t e r o a n t e r i o r a n d l a t e r a l r a d i o g r a p h o f t h e l u mb o s a c r a l s p i n e ma y
465 / 652
tmdmss
b e u s e d t o d e l i n e a t e b o n y s t r u c t u r e a n d a l i g n me n t b u t d o e s n o t p r o v i d e
d i a g n o s t i c i n f o r ma t i o n r e g a r d i n g ma n y s e r i o u s c a u s e s o f b a c k p a i n . P a t i e n t s
w i t h a p o t e n t i a l r e d f l a g c o n d i t i o n , s u c h a s s p i n a l t r a u ma , o r s u s p e c t e d c a u d a
e q u i n a s y n d r o me ma y r e q u i r e c o mp u t e d t o mo g r a p h y ( C T ) s c a n n i n g a n d / o r
ma g n e t i c r e s o n a n c e i ma g i n g ( M R I ) . M R I i s u s u a l l y t h e p r e f e r r e d mo d a l i t y i f
a v a i l a b l e a n d t h e p a t i e n t i s s t a b l e . A b o n e s c a n ma y b e u s e d w h e n t u mo r,
i n f e c t i o n , o r o c c u l t f r a c t u r e i s s u s p e c t e d . E l e c t r o my o g r a p h y ma y b e u s e f u l t o
a s s e s s f o r n e r v e r o o t d y s f u n c t i o n w h e n s y mp t o ms a r e q u e s t i o n a b l e .
M y e l o g r a p h y w i t h o r w i t h o u t C T s c a n ma y
P. 2 8 2
b e o b t a i n e d i n p r e o p e r a t i v e p l a 1)
n n. i nPge r( s i s t e n t , c h r o n i c p a i n ma y r e q u i r e
f u r t h e r d i a g n o s t i c i ma g i n g .
I V. Diagnosis
A.
T h e mo s t c o mmo n c a u s e o f l o w b a c k p a i n i n p r i ma r y c a r e i s my o f a s c i a l d y s f u n c t i o n
w i t h s u b s e q u e n t mu s c l e s p a s m. T h e p h y s i c a l e xa mi n a t i o n r e v e a l s l i mi t a t i o n o f
mo t i o n o f t h e a f f e c t e d a r e a , w i t h t e n d e r n e s s a n d i n c r e a s e d t o n e i n t h e a f f e c t e d
mu s c l e g r o u p s . S p o n d y l o l i s t h e s i s t y p i c a l l y p r e s e n t s i n a d o l e s c e n c e , p a r t i c u l a r l y i n
a t h l e t e s . L o w b a c k p a i n , l o s s o f l u mb a r l o r d o s i s , a n d a p a l p a b l e s t e p o f f a r e
c l a s s i c f i n d i n g s . L u mb a r d e g e n e r a t i v e d i s c d i s e a s e ma y p r e s e n t i n o l d e r a g e - g r o u p s
w i t h l o c a l i ze d o r r a d i c u l a r p a i n d u e t o n e r v e r o o t c o mp r e s s i o n . P a i n r a d i a t i n g b e l o w
t h e k n e e i s mo r e l i k e l y t o b e a t r u e r a d i c u l o p a t h y.
B.
A c h i n g , t h r o b b i n g p a i n o f i n s i d i o u s o n s e t t h a t i s w o r s e i n t h e mo r n i n g a n d i s
u n r e l i e v e d b y r e s t a n d w o r s e a t n i g h t s u g g e s t s a n i n f l a mma t o r y o r i g i n o f t h e p a i n . A
d e e p , d u l l p a i n , w i t h i n t e r mi t t e n t s t i f f n e s s r e l i e v e d b y r e s t a n d w o r s e a f t e r a c t i v i t y
the end of the day associated with a precipitating event or a previous history of bac
p a i n s u g g e s t s me c h a n i c a l o r i g i n . I n c r e a s e d p a i n f r o m s t a n d i n g o r w a l k i n g s u g g e s t s
spinal stenosis, whereas pain on sitting is often due to disc disease. Pain and
s t i f f n e s s i n t h e mo r n i n g s u g g e s t s i n f l a mma t o r y d i s e a s e , w h e r e a s c o n t i n u o u s p a i n i s
mo r e s u g g e s t i v e o f n e o p l a s m o r i n f e c t i o n . A mo r e c o mp l i c a t e d mi xe d p a t t e r n i s s e e n
i n t h e c o mmo n s i t u a t i o n i n w h i c h me c h a n i c a l d i s e a s e c o e xi s t s w i t h i n f l a mma t o r y
disease.
References
1 . D e y o R A , R a i n v i l l e J , K e n t D L . W h a t c a n t h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n
t e l l u s a b o u t l o w b a c k pJaAi nM? A 1 9 9 2 ; 2 6 8 : 7 6 0 7 6 5 .
2 . A u s t r a l i a n A c u t e M u s c u l o s k e l e t a l P a i n G u i d e l i n eEsv iGdenc
r o u pe. bas ed
management of ac ut e mus c ul os k el et.alB rpai
i s bna n e : A u s t r a l i a n A c a d e mi c
Press, 2003.
3 . D a v i s S . L o w b a c k p a i n . I n : T a y l o r RMBus
, ec dul. os k el et al pr obl ems . T he 10
466 / 652
tmdmss
mi nut e di agnos i s manual

. P h i l a d e l p h i a , PA : L i p p i n c o t t W i l l i a ms & W i l k i n s , 2 0 0 0 .
467 / 652
tmdmss
12.6 - Monoarticular Joint Pain
> T a b l e o f C o n t e n t s > 1 2 - M u s c u l o s k e l e t a l P r o b l e ms > 1 2 . 6 - M o n o a r t i c u l a r J o i n t
Pain
12.6
M onoarticular Joint Pain
Trish P alm e r
I . Background
P a i n i n a s i n g l e j o i n t i s a c o mmo n p r e s e n t i n g c o mp l a i n t .
A. Etiology
M o n o a r t i c u l a r j o i n t p a i n i s a s i g n t h a t c a n h a v e ma n y c a u s e s , i n c l u d i n g , t r a u ma t i c ,
i n f e c t i o u s , c r y s t a l - i n d u c e d , d e g e n e r a t i v e , ma l i g n a n t , a n d r h e u ma t i c c o n d i t i o n s .
T h e s e p r o c e s s e s ma y a l s o i n v o l v e c a r t i l a g e , b o n y s t r u c t u r e s , s u r r o u n d i n g b u r s a e , o r
l i g a me n t s o r t e n d o n s , r e s u l t i n g i n p a i n .
B. Epidemiology
E a c h y e a r, t h e r e a r e mo r e t h a n 3 1 5 mi l l i o n o f f i c e v i s i t s f o r mu s c u l o s k e l e t a l
c o mp l a i n t s1)(. T h i s a c c o u n t s f o r > 1 0 % o f a l l o u t p a t i e n t v i s i t s i n g e n e r a l me d i c a l
p r a c t i c e2)( . I n 2 0 0 1 , a t e l e p h o n e s u r v e y r e v e a l e d a r t h r i t i s a n d c h r o n i c j o i n t
s y mp t o ms i n o n e t h i r d o f a l l a3)
d u. l t s (
I I I . Evaluation
A. History
A k e y p o i n t i s w h e t h e r t h e p a i n s t e ms f r o m t r a u ma . A h i s t o r y o f h i g h i mp a c t o r a n
inability to bear weight indicates the possibility of a fracture, dislocation, or soft
t i s s u e d a ma g e . U n u s u a l o r e xc e p t i o n a l p h y s i c a l d e ma n d ma y i n d i c a t e a s t r e s s
f r a c t u r e4)( . A t r a u ma t i c j o i n t p a i n s h o u l d p r o mp t c o n s i d e r a t i o n o f d i a g n o s e s s u c h a s
r h e u ma t i c d i s e a s e , d e g e n e r a t i v e d i s e a s e , c r y s t a l - i n d u c e d a r t h r o p a t h y, i n f e c t i o n , a n d
ma l i g n a n c y. I n f o r ma t i o n a b o u t f a mi l y h i s t o r y o f j o i n t d i s e a s e a n d r h e u ma t i c
c o n d i t i o n s s h o u l d b e a s s e s5)s e. dWe
( ight loss, fevers, chills, night sweats,
P. 2 8 3
u n r e l e n t i n g o r n o c t u r n a l p a i n , a n d s i g n i f i c a n t d i s a b i l i t y ma y i n d i c a t e i n f e c t i o n o r
ma l i g n a n c y.
TAB L E 12.6.1 Diagnoses Consistent with Findings From

Synovial Fluid Analysis
White
P o l y m o r p h o -G l u c o s e
nucle ar
(%
Cry stals
unde r
468 / 652
tmdmss

Condition
N o r ma l
blood
ne utrophils se rum
Appe arance ce lls/m m
(%)
level)
polarize d
light
Clear
<200
<25
95100
None
N o n i n f l a mma t o r y C l e a r
(e.g.,
degenerative
joint disease)
<400
<25
95100
None
Acute gout
Turbid
2,000
5,000
>75
80100
Negative
birefringence;
needlelike
crystals
Pseudogout
Turbid
5,000
50,000
>75
80
1,000
Positive
birefringence;
r h o mb o i d
crystals
Septic arthritis
Purulent/turbid >50,000
>75
<50
None
I n f l a mma t o r y
(e.g.,
r h e u ma t o i d
arthritis)
Turbid
5075
~75
None
5,000
50,000
A d a p t e d f r o m R o b e r t s J R , H e d g e s J RC,l ienid cs al
. pr oc edur es i n emer genc y medi
, c i ne
3 r d e d . P h i l a d e l p h i a , PA : W B S a u n d e r s , 1 9 9 8 .
1. E xa mi n a t i o n o f t h e e n t i r e p a t i e n t i s i mp e r a t i v e . D i s t r i b u t i o n o f p a i n o r s w e l l i n g
c a n g i v e a d d i t i o n a l c l u e s r e g a r d i n g t h e d i a g n o s i s . R h e u ma t o i d a r t h r i t i s ( R A )
o f t e n i n v o l v e s t h e p r o xi ma l i n t e r p h a l a n g e a l , me t a c a r p o p h a l a n g e a l , a n d
me t a t a r s o p h a l a n g e a l j o i n t s , w h e r e a s i n v o l v e me n t o f t h e h i p , k n e e , d i s t a l
i n t e r p h a l a n g e a l j o i n t s , o r c a r p o me t a c a r p a l j o i n t o f t h e t h u mb s u g g e s t s
o s t e o a r t h r i t i s . E l e me n t s o f p h y s i c a l e xa mi n a t i o n s h o u l d i n c l u d e i n s p e c t i o n ,
p a l p a t i o n , r a n g e o f mo t i o n ( R O M ) , a n d s p e c i a l t e s t s .
2. M a r k e d s w e l l i n g o r e c c h y mo s i s , l a xi t y, g r o s s d e f o r mi t y, a n d t e n d o n o r mu s c l e
d y s f u n c t i o n ma y i n d i c a t e f r a c t u r e o r s o f t t i s s u e i n j u r y. C r e p i t u s i n d i c a t e s a
d e r a n g e me n t o f b o n e , c a r t i l a g e , o r me
6) .n iSs ec in s( o r y c h a n g e s i n d i c a t e
p o s s i b l e n e u r o l o g i c o r v a s c u l a r p a t h o l o g y. I n c r e a s e d R O M ma y i n d i c a t e a n
u n s t a b l e j o i n t , w h e r e a s d e c r e a s e d R O M ma y r e p r e s e n t e f f u s i o n , c a p s u l e
f i b r o s i s , o r b o n y a b n o r ma l i t y.
3. B r u i s e s i n c o n s i s t e n t w i t h t h e p a t i e n t ' s e xp l a n a t i o n ma y i n d i c a t e u n d e t e c t e d
abuse.
469 / 652
tmdmss
C. Testing
B l o o d t e s t i n g ( e . g . , s e d i me n t a t i o n r a t e , C - r e a c t i v e p r o t e i n , r h e u ma t o i d f a c t o r,
a n t i n u c l e a r a n t i b o d y, u r i c a c i d ) i s u s e f u l o n l y i f t h e r e i s a h i g h s u s p i c i o n o f a s p e c i f i c
d i a g n o s i s . T h e s e t e s t s h a v e a h i g h s e n s i t i v i t y b u t l o w 7,8)
s p e. cFi foi cr i et yxa( mp l e ,
r h e u ma t o i d f a c t o r i s n e g a t i v e i n a s ma n y a s 3 0 % o f c a s e s o f R A b u t ma y b e p o s i t i v e
i n n o r ma l a g i n g i n d i v i d u a l s a s w e l l a s i n c e r t a i n d i s e9)
a s. e s t a t e s (
1. A r t h r o c e n t e s i s i s u r g e n t l y i n d i c a t e d w h e n t h e r e i s a w a r m, r e d j o i n t w i t h
e f f u s i o n , e s p e c i a l l y w h e n t h e r e i s n o h i s t o r y o1f0)t .r aAubma
s e n( c e o f f e v e r
d o e s n o t r e l i a b l y e xc l u d e t h e p r e s e n c e o f a s e p t i c j o i n t a n d s h o u l d n o t i n f l u e n c e
t h i s d e c i s i o 11)
n (. Synovial fluid should be sent for the 3 Cs : cell count,
c u l t u r e ( G r a m' s s t a i n ) , a n d c r yTsat ba l es . 1 2 . 6 .r1e v i e w s t h e d i a g n o s e s
consistent with findings on synovial fluid analysis.
2. X - r a y s s h o u l d b e o b t a i n e d w h e n t h e r e i s c h r o n i c p a i n , s u s p e c t e d a r t h r i t i s , b o n y
t e n d e r n e s s , i n a b i l i t y t o b e a r w e i g h t , g r o s s d e f o r mi t y, a n d s k e l e t a l i mma t u r i t y.
M a g n e t i c r e s o n a n c e i ma g i n g ( M R I ) i s a l s o a c h o i c e t o f u r t h e r e v a l u a t e t h e
P. 2 8 4
p r o b l e m a n d l i mi t e xp o s u r e t o r a d i a t i o n . F o r c l i n i c a l q u e s t i o n s d e a l i n g p r i ma r i l y
w i t h s o f t t i s s u e p r o b l e ms , M R I i s t h e mo d a l i t y o f c h o i c e e xc e p t i n p a t i e n t s w i t h
i n d w e l l i n g me t a l l i c d e v i c e s . C o mp u t e d t o mo g r a p h y ( C T ) s c a n i s t h e mo d a l i t y o f
c h o i c e i n p a t i e n t s w h o h a v e i n d w e l l i n g me t a l f o r w h o m p l a i n r a d i o g r a p h s a r e n o t
diagnostic.
3. I n j e c t i o n o f i o d i n a t e d c o n t r a s t c a n b e p e r f o r me d i n c o n j u n c t i o n w i t h C T ( C T
a r t h r o g r a p h y ) t o p r o v i d e i n f o r ma t i o n a b o u t h y a l i n e c a r t i l a g e , l i g a me n t s , a n d t h e
j o i n t c a p s u l e . M R I w i t h g a d o l i n i u m i n j e c t i o n ( M R I a r t h r o g r a p h y ) c a n v i s u a l i ze
i n t r a - a r t i c u l a r s t r u c t u r e s . T h i s mo d a l i t y ma y a l s o d e t e c t e a r l y i n f l a mma t o r y
a r t h r i t i s1 2)
( .
D. Genetics
P r e ma t u r e d e v e l o p me n t o f O A i s r e l a t e d t o a d e f e c t i n t h e t y p e I I p r o c o l l a g e n g e n e
[ C O L 2 A 1 ] a n d i s o f d o mi n a n t i n h e r i t a n c e . G e n e t i c s t u d i e s s u p p o r t a s i g n i f i c a n t
g e n e t i c c o n t r i b u t i o n t o 1O3)A. (
1. R A i s g e n e t i c a l l y c o mp l e x. P a t i e n t s w i t h R A h a v e a n i n c r e a s e d i n c i d e n c e o f
H L A - D R 4 a n d H L A - D R 1 a n t i g e n s c o mp a r e d t o t h e g e n e r a l p o p u l a t i o n .
TAB L E 12.6.2 Differential Diagnosis of Joint

Pain
Traum a
I nfe ction
Othe r
Sprain
Gonococcal
Reflex
s y mp a t h e t i c
dystrophy
Strain
Nongonococcal: viral,
S jgren's
my c o b a c t e r i a l , o r f u n g a l s y n d r o me
470 / 652
tmdmss

Fracture
L y me d i s e a s e
P o l y my o s i t i s
Dislocation
Subacute bacterial
endocarditis
T e a r o f l i g a me n t ,
Secondary to enteric and Sarcoidosis
t e n d o n , o r me n i s c u s u r o g e n i t a l i n f e c t i o n s
Tendinitis
E r y t h e ma
nodosum
Sickle cell
disease
Aseptic necrosis
C harcot's
disease
Drug reaction
Hypothyroidism
Irritable bowel
s y n d r o me
Osteochondritis
dissecans
Cry stal-induce d
arthropathy
De ge ne rativ e joint
dise ase
Malignant
Gout
Osteoarthritis
T u mo r
Pseudogout
Metastases
L e u k e mi a
Rhe um atic
R h e u ma t o i d a r t h r i t i s
R e i t e r ' s s y n d r o me
Psoriatic arthritis
L u p u s e r y t h e ma t o s u s
Ankylosing spondylitis
P. 2 8 5
2. C l a s s i c f a mi l i a l g o u t ma y b e a mo n o me r i c d o mi n a n t l y i n h e r i t e d d i s o r d e r. C a l c i u m
p y r o p h o s p h a t e d e h y d r a t e d e p o s i t i o n d i s e a s e o c c u r s i n a h e r e d i t a r y f o r m.
I V. Diagnosis
T a b l e 1 2 . 6 .s2h o w s t h e d i f f e r e n t i a l d i a g n o s i s f o r mo n o a r t i c u l a r j o i n t p a i n .
471 / 652
tmdmss
1. O s t e o a r t h r i t i s i s u s u a l l y s e e n i n o l d e r p a t i e n t s , p r e s e n t i n g s y mme t r i c a l l y i n l a r g e
w e i g h t - b e a r i n g j o i n t s . I t ma y ma n i f e s t i n i t i a l l y o r d u r i n g f l a r e s a s mo n o a r t i c u l a r
joint pain, usually worse at the end of the day or after prolonged weight-bearing.
T h e r e ma y b e s o me s w e l l i n g , u s u a l l y w i t h o u t e r y t h e ma o r mu c h w a r mt h .
2. R A o f t e n b e g i n s w i t h s y s t e mi c s y mp t o ms . I n i t i a l l y, i t ma y a f f e c t o n l y o n e j o i n t b u t
c o mmo n l y p r e s e n t s a s s w e l l i n g , r e d n e s s , a n d w a r mt h o f t h e s ma l l j o i n t s i n t h e
h a n d a n d w r i s t . M o r n i n g s t i f f n e s s i s c o mmo n .
3. G o u t y a r t h r i t i s o c c u r s s u d d e n l y, o f t e n d u r i n g t h e n i g h t . T h e p a i n i s t y p i c a l l y
d e s c r i b e d a s e xc r u c i a t i n g , w i t h t h e p a t i e n t u n a b l e t o e v e n h a v e a s h e e t t o u c h i n g
t h e a f f e c t e d j o i n t . C o mmo n l y, t h e g r e a t t o e i s w a r m, s w o l l e n , a n d r e d .
4. A s e p t i c j o i n t ma y h a v e a n e n t r y p o i n t f o r t h e b a c t e r i a t h a t i s o b v i o u s , l i k e a n
a b r a s i o n , a n d i s s w o l l e n , w a r m a n d r e d . L o w - g r a d e f e v e r ma y a c c o mp a n y j o i n t
p a i n , b u t l a c k o f f e v e r s h o u l d n o t p r e c l u d e j o i n t a s p i r a t i o n . C h i l d r e n ma y p r e s e n t
w i t h n o n s p e c i f i c s y mp t o ms s u c h a s i r r i t a b i l i t y, f e v e r, c r y i n g w i t h mo v e me n t o f t h e
hip (as with diaper changes), or refusal to bear weight.
References
1. F auci A, B raunwald E , Isselbacher K , et H
a lar
. , rei sdon'
s . s pr i nc i pl es of
i nt er nal medi c .i ne
1 4 t h e d . N e w Yo r k : M c G r a w - H i l l , 1 9 9 8 .
2 . I s s e l b a c h e r K , M a r t i n J , B r a u n w a l d E , e t Ha ar
l . , r iesdon'
s . s pr i nc i pl es of
i nt er nal medi c .i ne
1 3 t h e d . N e w Yo r k : M c G r a w - H i l l , 1 9 9 4 .
3. Centers for Disease Control and Prevention. Prevalence of self-reported
a r t h r i t i s o r c h r o n i c j o i n t s y mp t o ms a mo n g a d u l t s U n i t e d S M
t aor
t ebs , 2 0 0 1 .
M or t al W k l y R ep
2002;51:948950.
4. R eeder M T, D ick B H , Atkins J K , et al. S tress fractures. C urrent concepts of
d i a g n o s i s a n d t r e a t meSnpor
t . t s M ed1 9 9 6 ; 2 2 ( 3 ) : 1 9 8 2 1 2 .
5 . L i t t ma n K . A r a t i o n a l a p p r o a c h t o t h e d i a g n o s i s oAf m
a r tFham
r i t i sP. hy s i c i an
1996;53(4):12951310.
6 . R i c h i e A M , F r a n c i s M L . D i a g n o s t i c a p p r o a c h t o p o l y a r t i c u l aAr mj o i n t p a i n .
2 0 0 3 ; 6 8 ( 6 ) : 11 5 1 11 6 0 .
7 . B a r t h W F. O f f i c e e v a l u a t i o n o f t h e p a t i e n t w i t h mu s c u l o s k e l e t aAl m
c o mp l a i n t s .
J M ed; 1 9 9 7 ( 1 0 2 1 A ) : 3 S 1 0 S .
8 . F r e e d J F, N i e s K M , B o y e r R S , e t a l . A c u t e mo n o a r t i c u l a r a r t h r i t i s : a d i a g n o s t i c
a p p r o a c hJ. A M A 1 9 8 0 ; 2 4 3 ( 2 2 ) : 2 3 1 4 2 3 1 6 .
9 . K l i p p e l J , We y a n d C , Wo r t ma n n R , e t aPl .r,i mer
e d s .on t he r heumat i c
di s eas es
. 11 t h e d . A t l a n t a , G A : A r t h r i t i s F o u n d a t i o n , 1 9 9 7 .
1 0 . T i l l S H , S n a i t h M L . A s s e s s me n t , i n v e s t i g a t i o n , a n d ma n a g e me n t o f a c u t e
mo n o a r t h r i t i Js . A c c i d E mer g M ed
1999;16(5):355361.
472 / 652
tmdmss
11 . L e a r c h T J . I ma g i n g o f i n f e c t i o u s a rSt hemi
r i t ins .M us c ul os k el et R adi ol
2003;7(2):137142.
1 2 . M c G o n a g l e D , C o n a g h a n P G , Wa k e f i e l d R , e t a l . I ma g i n g t h e j o i n t s i n e a r l y
r h e u ma t o i d a r t h r i B
t i es
s . t P r ac t R es C l i n R heumat
2 0 0ol1 ; 1 5 ( 1 ) : 9 1 1 0 4 .
1 3 . F e l s o n D T , C o u r o p mi t r e e N N , C h a i s s o n C E , e t a l . E v i d e n c e f o r a M e n d e l i a n
g e n e i n a s e g r e g a t i o n a n a l y s i s o f g e n e r a l i ze d r a d i o g r a p h i c o s t e o a r t h r i t i s : t h e
F r a mi n g h a m S t u dAy.r t hr i t i s R heum
1998;41(6):10641071.
473 / 652
tmdmss
12.7 - Neck Pain
> T a b l e o f C o n t e n t s > 1 2 - M u s c u l o s k e l e t a l P r o b l e ms > 1 2 . 7 - N e c k P a i n
12.7
Neck Pain
Jame s R. Barre tt
Brian Cole m an
I . Background
Neck pain is defined as pain occurring anywhere between the base of the skull and
t h o r a x. T h i s p a i n c a n o c c u r a n t e r i o r l y, p o s t e r i o r l y, o r l a t e r a l l y. T h e c e r v i c a l s p i n e i
o n e o f t h e mo s t mo b i l e a n d c o mp l e x j o i n t s i n t h e b o d y. C o n s i d e r i n g t h a t , o n a v e r a g e
t h e n e c k mo v e s o v e r 6 0 0 t i me s a n1)h, oiut r i s( n o w o n d e r t h a t i t i s a c o mmo n
source of pain. Most episodes of neck pain are short lived and resolve
s p o n t a n e o u s l y.
A. Etiology
1. T r a u ma , j o b - r e l a t e d c a u s e s ( e . g . , ma n u a l l a b o r, d r i v i n g f o r l o n g p e r i o d s , h e a d
f o r w a r d p o s i t i o n i n g w h i l e t y p i n g ) , s mo k i n g , a n d a p r e v i o u s h i s t o r y o f l o w b a c k
pain are considered risk factors for neck pain. Often, no specific cause can be
elicited.
2. S y mp t o ms d u e t o c e r v i c a l p a t h o l o g y c a n b e r e f e r r e d t o o t h e r a r e a s o f t h e b o d y,
mo s t c o mmo n l y t h e u p p e r b a c k , c h e s t , a n d a r ms . L i k e w i s e , p a i n f r o m s h o u l d e r
or chest pathology can be referred to the neck.
B. Epidemiology
N e c k p a i n i s e xt r e me l y c o mmo n i n t h e g e n e r a l p o p u l a t i o n w i t h a 4 0 % t o 7 0 % l i f e t i me
prevalence. Cervical arthritis is seen in 80% of individuals over 50 years of age an
c e r v i c a l r a d i c u l o p a t h y, i n 8 3 . 2 / 1 0 0 , 0 0 0 o f t h e g e n e r a l p o p u l a t i o n w i t h mo s t o f t h e m
t h e C 6 - 7 d i s t r i b u t i2)
o n. (
TAB L E 12.7.1 Special Physical Exam ination Tests

of Cervical Spine
Te s t
Ev aluation
for
How pe rform e d
Positiv e
te st
474 / 652
tmdmss
12.7 - Neck Pain
S purling's
test
Nerve root
H e a d i n mi l d e xt e n s i o n a n d P a i n i n
c o mp r e s s i o n f l e xi o n t o w a r d s i d e o f r a d i c u dl aerr ma t o ma l
(disc
s y mp t o ms
pattern on
herniation)
affected
side
Distraction Nerve root

H e a d l i f t e d a xi a l l y w i t h o n e P a i n r e l i e f
test
c o mp r e s s i o n h a n d u n d e r c h i n a n d o t h e r w i t h l i f t i n g
(disc
hand around occiput
head
herniation)
Adson's
T horacic
ma n e u v e r s o u t l e t
s y n d r o me
E xt e n d p a t i e n t ' s s y mp t o ma t i cL o s s o f
s h o u l d e r w h i l e p a t i e n t r o t a t eps u l s e i n
neck toward affected side. affected
P u l s e i s c h e c k e d d u r i n g d e e pe xt r e mi t y
inspiration
L h e r mi t t e ' s S p i n a l c a n a l P a t i e n t s i t t i n g w i t h l e g s
Shock like
sign
narrowing
e xt e n d e d ; a s k p a t i e n t t o f l e x s e n s a t i o n
(spinal
neck forward
into lower
stenosis),
back and/or
mu l t i p l e
e xt r e mi t i e s
sclerosis
P. 2 8 7
TAB L E 12.7.2 Differential Diagnosis of Neck Pain

Musculoske le tal Ne urologic
I nfe ctious Ne oplastic
Cervical strain T horacic

or sprain
outlet
s y n d r o me
Diskitis
Spinal
cord
t u mo r
Disc herniation Peripheral

neuropathy
O s t e o my e l i t i s P r i ma r y
neck
neoplasm
Degenerative
disc disease
Myelopathy
Meningitis
arthritis
R a d i c u l o p a t h y C e r v i c a l l y mp h a d e n i t i s
Re fe rre d
Rotator cuff
tendinopathy
Myocardial
i s c h e mi a
M a l i g n a n t P n e u mo n i a
neoplasm
475 / 652
tmdmss
12.7 - Neck Pain
( r h e u ma t o i d ,
ankylosing
spondylitis)
Cervical fracture
Cervical instability
Cervical stenosis
Whiplash
Diffuse idiopathic skeletal hyperostosis
Torticollis
I I I . Evaluation
A. History
C h a r a c t e r i zi n g t h e l o c a t i o n , q u a l i t y, i n t e n s i t y, r a d i a t i o n , d u r a t i o n , a n d a s s o c i a t e d
s y mp t o ms h e l p s d e t e r mi n e t h e l i k e l y c a u s e o f n e c k p a i n .
1. K e y a s s o c i a t e d n e c k c o mp l a i n t s a r e r a d i c u l a r s y mp t o ms , s u c h a s p a r e s t h e s i a s ,
s e n s o r y l o s s , mu s c l e w e a k n e s s , w h i c h c a n i n d i c a t e n e r v e r o o t c o mp r e s s i o n ;
l o w e r e xt r e mi t y s y mp t o ms , s u c h a s l o w e r e xt r e mi t y p a r e s t h e s i a s , b o w e l / b l a d d e r
d y s f u n c t i o n , w h i c h c a n i n d i c a t e c a u d a e q u i n a s y n d r o me ; a n d f e v e r, w e i g h t l o s s ,
a n d o t h e r j o i n t i n v o l v e me n t t h a t c a n i n d i c a t e i n f l a mma t o r y a r t h r i t i s , i n f e c t i o n , o r
n e o p l a s m.
2. U n u s u a l s y mp t o ms c a n b e r e l a t e d t o n e c k p a t h o l o g y a n d s h o u l d n o t b e
d i s c o u n t e d . S y mp a t h e t i c n e r v o u s s y s t e m a c t i v a t i o n c a n c a u s e e y e p a i n ,
increased tearing, and blurry vision. Irritation of the C3-5 nerve root (due to
p h r e n i c n e r v e i n v o l v e me n t ) c a n c a u s e r e s p i r a t o r y s y mp t o ms s u c h a s s h o r t n e s s
o f b r e a t h3)( .
E xa mi n a t i o n s h o u l d i n c l u d e i n s p e c t i o n , p a l p a t i o n f o r t e n d e r n e s s i n b o t h mi d l i n e a n d
p a r a s p i n o u s a r e a s , r a n g e o f mo t i o n , n e u r o v a s c u l a r e xa mi n a t i o n , a n d s p e c i a l t e s t s .
I n s p e c t i o n o f t h e n e c k s h o u l d e v a l u a t e f o r l o s s o f n o r ma l l o r d o s i s , r a s h , o r o t h e r
a b n o r ma l i t i e s . A c t i v e r a n g e o f mo t i o n i s o b s e r v e d . R e d u c t i o n o f a c t i v e r a n g e o f
mo t i o n ma y b e a s s o c i a t e d w i t h u n d e r l y i n g b o n y p a t h o l o g y s u c h a s a r t h r i t i s o r b e d u
t o mu s c l e s p a s m. L o s s o f p a s s i v e r a n g e o f mo t i o n i s u s u a l l y d u e t o u n d e r l y i n g b o n y
p a t h o l o g y. N e u r o v a s c u l a r e xa mi n a t i o n i n c l u d e s t h e e v a l u a t i o n o f mo t o r s t r e n g t h ,
476 / 652
tmdmss
12.7 - Neck Pain
s e n s a t i o n , a n d d e e p t e n d o n r e f l e xe s . S p e c i a l t e s t s f o r c e r v i c a l p a t h o l o g y a r e
r e v i e w e d iTna b l e 1 2 . 7.. 1
C. Testing
L aboratory tests are rarely needed in patients with neck pain. Suspicion of a
ma l i g n a n c y o r i n f l a mma t o r y a r t h r i t i s , s u c h a s r h e u ma t o i d a r t h r i t i s o r a n k y l o s i n g
s p o n d y l i t i s ma y p r o mp t t e s t s s u c h a s r h e u ma t o i d f a c t o r, s e d i me n t a t i o n r a t e , c o mp l e
blood count, and/or H L A B27.
P. 2 8 8
P. 2 8 9
TAB L E 12.7.3 Most Com m on Causes of Neck Pain
Etiology
Ty p i c a l
history
Spondylosis Dull neck

ache
Older agegroup
Occipital
headache
radicular
s y mp t o ms
Cervical
disc
herniation
Key
phy sical
e x am ination
findings
Tender to
palpation
mi d l i n e
Decreased
active and
passive
ROMs
Ke y lab findings
R a d i o g r a p h ( x- r a y ) s h o w s
degenerative changes that
can include narrowing of
disc space, sclerosis of
p o s t e r i o r e l e me n t s , a n d
osteophytes
Sharp neck
pain
Decreased
M R I shows disc protrusion
a c t i v e R O M o r e xt r u s i o n i n t o s p i n a l
canal
Burning or
tingling in
upper
e xt r e mi t i e s
Reduced
deep tendon
r e f l e xe s
Pain with
n e c k mo t i o n
Decreased
strength in
upper
e xt r e mi t i e s
Upper
Positive
477 / 652
tmdmss
12.7 - Neck Pain
e xt r e mi t y
weakness
Cervical
I n t e r mi t t e n t
strain/sprain dull neck
pain
S purling's
test
N o r ma l
ROM
Loss of
Occupational lordosis
related
(postural)
T r a u ma
history
( mo t o r
vehicle
accident,
fall)
Palpable
tightness,
ropiness
Muscle
spasm
Occasional
acute
e d e ma
X - r a y i s n o r ma l o r s h o w s
loss of lordosis
C o n s i d e r c o mp u t e d
t o mo g r a p h y t o r u l e o u t b o n y
i n j u r y i n t r a u ma
Muscle
s p a s ms
F i b r o my a l g i a D i f f u s e a xi a l N o r ma l
skeletal pain passive
ROM
Sleep
disturbance
No laboratory test to
confirm
Trigger
points
Fatigue
I n f l a mma t o r y D u l l a c h e
arthritis
such as R A
or AS
Morning
stiffness >1
h
Decreased
active and
passive
ROMs
R A : i n c r e a s e d r h e u ma t o i d
factor and erythrocyte
s e d i me n t a t i o n r a t e
Other joint AS: positive H L A B27

i n f l a mma t i o n
O t h e r j o i n t i n v o l v e me n t
478 / 652
tmdmss
12.7 - Neck Pain
Referred
pain
S y mp t o ms
from other
sites (e.g.,
chest pain,
shoulder
pain)
N o r ma l
R O M of
neck
X-ray of other sites

e l e c t r o c a r d i o g r a m, M R I
potentially helpful
P h y s i c a l e xa mi n a t i o n f i n d i n g s a t o t h e r s i t e s
(e.g., shoulder strength loss, chest rales)
R O M , r a n g e o f mo t i o n ; M R I , ma g n e t i c r e s o n a n c e i ma g i n g ; R A , r h e u ma t o i d
arthritis; AS, ankylosing spondylitis.
1. R a d i o g r a p h s a r e f r e q u e n t l y h e l p f u l i n c a s e s o f t r a u ma , r a d i c u l a r s y mp t o ms , o r
p r o l o n g e d ( 3 6 w e e k s ) s y mp t o ms . T y p i c a l v i e w s i n c l u d e a n t e r o p o s t e r i o r,
o d o n t o i d ( o p e n - mo u t h v i e w ) , a n d l a t e r a l r a d i o g r a p h s o f t h e c e r v i c a l s p i n e . A l l
s e v e n c e r v i c a l v e r t e b r a e s h o u l d b e v i s u a l i ze d i n t h e l a t e r a l r a d i o g r a p h . O b l i q u e
v i e w s a s s i s t i n t h e e v a l u a t i o n o f t h e n e u r a l f o r a mi n a a n d p o s t e r i o r e l e me n t s .
F l e xi o n a n d e xt e n s i o n v i e w s a r e o b t a i n e d w h e n i n s t a b i l i t y o f t h e c e r v i c a l s p i n e
i s s u s p e c t e d . I t i s i mp o r t a n t t o r e me mb e r t h a t r a d i o g r a p h i c a b n o r ma l i t i e s a r e
c o mmo n i n t h e g e n e r a l p o p u l a t i o n a n d ma y n o t b e t h e c a u s e o f t h e s y mp t o ms .
2. C o mp u t e r i ze d t o mo g r a p h y i s b e s t u s e d f o r i d e n t i f y i n g b o n y p a t h o l o g y s u c h a s
s u b t l e f r a c t u r e s , w h e r e a s ma g n e t i c r e s o n a n c e i ma g i n g i s i d e a l f o r s o f t t i s s u e
p a t h o l o g y, s u c h a s d i s c d i s e a s e a n d s p i n a l s t e n o s i s . T h e u s e o f d i s c o g r a p h y t o
l o c a l i ze t h e e xa c t d i s c c a u s i n g p a i n i s c o n t r1)o .v eNr es ri av le ( c o n d u c t i o n
v e l o c i t y a n d e l e c t r o my o g r a m ma y b e h e l p f u l i n d i f f e r e n t i a t i n g t h e n e r v e r o o t f r o m
p e r i p h e r a l n e r v e p a t h o l o g y.
D. Genetics
C e r v i c a l d e g e n e r a t i v e d i s c d i s e a s e ma y h a v e a g e n e t i c c4)o.mp
G oe n e nt itc (
p r e d i s p o s i t i o n t o i n f l a mma t o r y d i s o r d e r s s u c h a s r h e u ma t o i d a r t h r i t i s , a n k y l o s i n g
s p o n d y l i t i s , a n d o s t e o a r t h r i t i s i s w e l l e s t a1)b.l i s h e d (
I V. Diagnosis
S e e T a b l e 1 2 . 7.. 2
S e e T a b l e 1 2 . 7.. 3
References
1. Nankano K K . Neck pain. In: Harris E , B udd R, F irestein G, et al., eds.
K el l ey ' s t ex t book of r heumat,ol7ogy
t h e d . P h i l a d e l p h i a , PA : E l s e v i e r S a u n d e r s ,
2005:537554.
479 / 652
tmdmss
12.7 - Neck Pain
2 . D e v e r e a u x M W. N e c k p P
a irni m
. C ar e C l i n O f f i c e P2r ac
0 0t4 ; 3 1 : 1 9 3 1 .
3 . B l a n d J H . D i s o r d e r s o f t h e c e r v i c a l s p i n e . I n : N oTex
b l et book
J , e d .of
pr i mar y c ar e medi c, i 3ner d e d . S t L o u i s , M O : M o s b y, 2 0 0 1 : 11 2 5 11 3 7 .
4 . M a c G r e g o r A J , A n d r e w T , S a mb r o o k P N , e t a l . S t r u c t u r a l , p s y c h o l o g i c a l , a n d
g e n e t i c i n f l u e n c e s o n l o w b a c k a n d n e c k p a i n : a s t u d y o f a d u l t f e ma l e t w i n s .
A r t hr i t i s R heum
2004;51:160167.
480 / 652
tmdmss
12.8 - Polymyalgia
> T a b l e o f C o n t e n t s > 1 2 - M u s c u l o s k e l e t a l P r o b l e ms > 1 2 . 8 - P o l y my a l g i a
12.8
Polymyalgia
Martina Ke lly
Jose ph A. Moran
I . Background
M u l t i p l epol
( y) mu s c l e a c h emy
s ( al gi )a a r e a c o mmo n p r e s e n t a t i o n i n p r i ma r y c a r e
with a broad differential diagnosis. T he challenge for the physician lies in
d i s t i n g u i s h i n g b e n i g n a n d s e l f - l i mi t i n g c a u s e s , s u c h a s a b e n i g n v i r a l i n f e c t i o n , f r o m
mo r e s e r i o u s i l l n e s s e s . I n mo s t c a s e s , d i a g n o s i s r e ma i n s p r i ma r i l y a c l i n i c a l o n e .
T h e mo s t c o mmo n c a u s e s a r e p o l y my a l g i a r h e u ma t i c a a n d i n f l a mma t o r y c o n d i t i o n s
( e . g . , p o l y my o s i t i s / d e r ma t o my o s i t i s a n d f i b r oTmy
a bal leg i1a2) . 8 .g1i v e s a s u mma r y
o f h o w t h e s e ma y b e d i s t i n g u i s h e d . T h e e t i o l o g y a n d e p i d e mi o l o g y a r e d e p e n d e n t o n
t h e c a u s e . I n ma n y c a s e s , t h e p r e c i s e e t i o l o g y i s n o t k n o w n .
I I I . Evaluation
T h e k e y t o t h e e v a l u a t i o n o f t h i s c o n d i t i o n l i e s i n a c a r e f u l h i s t o r y a n d e xa mi n a t i o n
(4) . F r e q u e n t l y, l a b o r a t o r y i n v e s t i g a t i o n s a r e i n c o n c l u s i v e , a n d t h e r e i s a d a n g e r t h a t
p a t i e n t s ma y b e o v e r i n v e s t i g a t e d .
TAB L E 12.8.1 Differential Diagnosis of Polym yalgia

Poly m y algia
rhe um atica(2)
I nflam m atory conditions(1)

(e .g.,
F i b r o m y a l g i ap o l y m y o s i t i s / d e r m a t o m y o s i t i s )
E p i d e mi o l o g y ( 4 ) M o r e c o mmo n i n M i d d l e a g e
t h e e l d e r l y, r a r e l y ( u s u a l l y < 5 0
<50 y
y)
A f f e c t s w o me n
mo r e t h a n me n
700/100,000 >50
y
Pathophysiology Large vessel
4060 y
Affects
2 : 1 w o me n : me n 1 / 1 0 0 , 0 0 0
w o me n mo r e
t h a n me n 2 %
8% adults
(where
studied)
Unclear
I n f l a mma t i o n o f s t r i a t e d mu s c l e
481 / 652
tmdmss
12.8 - Polymyalgia
vasculitis
S e n s i t i za t i o n I f i t i n v o l v e s t h e s k i n i t i s
of central
d e r ma t o my o s i t i s
nervous
system
History
Sudden onset
S y s t e mi c :
Insidious
fatigue, sleep
disturbance,
headaches,
frequently
mu l t i p l e
s y mp t o ms
S y s t e mi c : f e v e r, W i d e s p r e a d
ma l a i s e , a n o r e xi a , p a i n
weight loss,
depression
S y s t e mi c : f e v e r, ma l a i s e , w e i g h t
loss in acute phase
S p e c i f i c : p r o xi ma l
s y mme t r i c a l
shoulder hip
girdle pain and
s t i f f n e s,sw o r s e
i n t h e mo r n i n g o r
after rest
S p e c i f i c : p r o xi ma l p a i n ( 5 0 % )
and weakness (worse after use)
D ysphagia (50%)
Respiratory
mu s c l e s
Physical
P r o xi ma l
tenderness
Te n d e r
trigge r
points
P r o xi ma l t e n d e r n e s s
N O w e akne ss
No weakness Prox im al w e akne ss
Muscle atrophy
Rash
( d e r ma t o my o s i t i s ) ,
G ottron's papules,
heliotrophic rash
on eyelids
Laboratory tests Elevated E S R
No specific
> 5 0 mm/ h ( r a r e l y t e s t
n o r ma l )
N o r mo c h r o mi c
N o r ma l E S R
E S R, creatine phosphokinase
E l e c t r o my o g r a p h y
482 / 652
tmdmss
12.8 - Polymyalgia
n o r mo c y t i c
a n e mi a
Muscle biopsy
T r e a t me n t
R a p i d r e s p o n s e t o N o r e s p o n s e A d mi s s i o n , s t e r o i d s , c y t o t o xi c s
oral prednisone to steroids
( 6 0 8 0 mg / d )
Multifaceted Splinting of joints and gradual
i n d i v i d u a l i ze d r e h a b i l i t a t i o n
p r o g r a mme
Associations
T e mp o r a l a r t e r i t i s I r r i t a b l e
(15% patients)
bowel
s y n d r o me
Myofascial
pain
s y n d r o me
S y s t e mi c l u p u s e r y t h e ma t o s u s ,
r h e u ma t o i d a r t h r i t i s , s y s t e mi c
sclerosis
M a l i g n a n c y ( l u n g , o v a r y, b r e a s t ,
s t o ma c h )
C h r o n i c f a t i g u e s y n d r o me
E S R , e r y t h r o c y t e s e d i me n t a t i o n r a t e .
P. 2 9 1
A. History
1. A s k a b o u t o n s e t ( a c u t e o r i n s i d i o u s ) a n d t h e mu s c l e s a f f e c t e d ( d i f f u s e , p r o xi ma l
mu s c l e s o f s h o u l d e r / h i p g i r d l e ) . T o a s s e s s p r o xi ma l mu s c l e w e a k n e s s , a s k a b o u t
difficulty going up stairs, getting up from chairs, and raising hands above the
head.
2. E n q u i r e a b o u t t h e p r e s e n c e o f s y s t e mi c s y mp t o ms ( f e v e r, w e i g h t l o s s , f a t i g u e ) .
I s t h e r e j o i n t i n v o l v e me n t ( a n d w h i c h o n e s ) ?
3. I s t h e r e a s i g n i f i c a n t p a s t me d i c a l h i s t o r y ? A s k a b o u t me d i c a t i o n a n d f a mi l y
h i s t o r y. I n t h e s o c i a l h i s t o r y, i t i s i mp o r t a n t t o e n q u i r e a b o u t o c c u p a t i o n a n d
sources of stress.
P a y p a r t i c u l a r a t t e n t i o n t o t h e mu s c u l o s k e l e t a l s y s t e m. I n s p e c t f o r mu s c l e a t r o p h y
a n d p a l p a t e f o r mu s c l e t e n d e r n e s s ( i n c l u d i n g t h e s c a l p a n d t e mp o r a l a r t e r y ) . T h e
p r e s e n c e o r a b s e n c e o f mu s c l e w e a k n e s s i s t h e k e y c l i n i c a l f i n d i n g . D i s t i n g u i s h
b e t w e e n s o f t t i s s u e p a i n ( my a l g i a ) a n d j o i n t p a i n ( a r t h r a l g i a ) . I n t h e l a t t e r, s y n o v i t i s
is a frequent finding. Is a rash present?
C. Testing
F r e q u e n t l y, l a b o r a t o r y t e s t s f a i l t o e l u c i d a t e t h e c a u s e . B a s e l i n e i n v e s t i g a t i o n s ma y
i n c l u d e a c o mp l e t e b l o o d c o u n t , e r y t h r o c y t e s e d i me n t a t i o n r a t e , C - r e a c t i v e p r o t e i n ,
483 / 652
tmdmss
12.8 - Polymyalgia
glucose, liver function tests, creatine phosphokinase, thyroid function tests, and a
c h e s t r a d i o g r a p h . I f mu s c l e w e a k n e s s i s p r e s e n t , c o n s i d e r a n e l e c t r o my o g r a m a n d / o r
mu s c l e b i o p s y. C o n s i d e r a t i o n ma y b e g i v e n t o o b t a i n i n g s e r o l o g i e s f o r i n f e c t i o u s
diseases, depending on risk factors.
P. 2 9 2
I V. Diagnosis
T h e d i f f e r e n t i a l d i a g n o s i s i s w i d e r aTnagbil neg1(2 . 8). .1 A p a r t f r o m t h e
a f o r e me n t i o n e d , t h e f o l l o w i n g s h o u l d b e c o n s i d e r e d . M a j o r r h e u ma t o l o g i c d i s o r d e r s
( r h e u ma t o i d a r t h r i t i s , s y s t e mi c l u p u s e r y t h e ma t o s u s ) c a n b e d i s t i n g u i s h e d b y t h e
p r e s e n c e o f j o i n t p a i n . O t h e r p o s s i b i l i t i e s i n c l u d e u n d e r l y i n g ma l i g n a n c y, s e r i o u s
i n f e c t i o n s , e n d o c r i n e d i s o r d e r s ( e . g . , h y p e r t h y r o i d i s m) , a n d d e p r e s s i o n .
References
1 . S a l v a r a n i C , C a n t i n i F, B o i a r d i L , e t a l . P o l y my a l g i a rBhes
e utma
P rt ac
i c at .
R es C l i n R heumat2ol0 0 4 ; 1 8 ( 5 ) : 7 0 5 7 2 2 .
2 . M e a s e P. F i b r o my a l g i a s y n d r o me : r e v i e w o f c l i n i c a l p r e s e n t a t i o n ,
p a t h o g e n e s i s , o u t c o me me a s u r e s , a n d t r eJa tRme
heumat
nt.
ol S uppl
2005;75:6
21.
3 . L a w r e n c e R C , H e l mi c k C G , A r n e t t F C , e t a l . E s t i ma t e s o f t h e p r e v a l e n c e o f
a r t h r i t i s a n d s e l e c t e d mu s c u l o s k e l e t a l d i s o r d e r s i n t h e U nAi tret hr
d iSt it sa t e s .
R heum1 9 9 8 ; 4 1 : 7 7 8 .
4 . N i r ma l a n a n t h a n N , H o l t o n J L , H a n n a M G . I s i t r e a l l y my o s i t i s ? A c o n s i d e r a t i o n
o f t h e d i f f e r e n t i a l d i a g nCour
s i rs O pi n R heumat2ol0 0 4 ; 1 6 ( 6 ) : 6 8 4 6 9 1 .
484 / 652
tmdmss
12.9 - Shoulder Pain
> T a b l e o f C o n t e n t s > 1 2 - M u s c u l o s k e l e t a l P r o b l e ms > 1 2 . 9 - S h o u l d e r P a i n
12.9
Shoulder Pain
Kaly anakrishnan Ram akrishnan
Andre w D. Jone s
I . Background
S i x t o t w e n t y - f i v e p e r c e n t o f t h e g e n e r a l p o p u l a t i o n r e p o r t s h1)o.u l d e r p a i n (
Significant disability including inability to carry out day-to-day household activities
ma y r e s u l t1)(. M o s t p a t i e n t s ( 8 5 % ) w i t h s h o u l d e r p a i n h a v e a d i s o r d e r i n t r i n s i c t o
t h e s h o u l d e r.
A. T h e s h o u l d e r j o i n t ' s p r i n c i p a l a r t i c u l a t i o n , t h e g l e n o h u me r a l j o i n t , i s s ma l l a n d
p r o v i d e s o n l y a l i mi t e d d e g r e e o f b o n y s t a b i l i t y. T h e r e f o r e , ma n y c a u s e s o f
shoulder pain are related to stress on its soft tissue support structures,
p r i n c i p a l l y t h e r o t a t o r c u f f . T h i s mu s c u l o t e n d i n o u s c u f f h e l p s s t a b i l i ze t h e
s h o u l d e r j o i n t . T h e s u b a c r o mi a l b u r s a p r o t e c t s t h e s e t e n d o n s f r o m c o mp r e s s i o n
b e t w e e n t h e a c r o mi o n a n d h u me r a l h e a d . R e p e t i t i v e o v e r h e a d a c t i v i t i e s , a c u t e
t r a u ma , o r i n s t a b i l i t y ma y r e s u l t i n i mp i n g e me n t o f t h e r o t a t o r c u f f t e n d o n a n d
s u b a c r o mi a l b u r s a b e t w e e n h u me r u s a n d a c r o mi o n , r e s u l t i n g i n p a i n a n d
i n f l a mma t i o n .
B. I n y o u n g e r p a t i e n t s , e s p e c i a l l y i n t h o s e i n v o l v e d i n s p o r t s r e q u i r i n g o v e r h e a d
mo t i o n s ( b a s e b a l l , t e n n i s , f o o t b a l l , o r s w i mmi n g ) , p a i n a n d i n s t a b i l i t y a r e o f t e n
d u e t o r e p e t i t i v e mi c r o t r a u ma i n v o l v i n g t h e l a b r o c a p s u l a r2)s. t r u c t u r e s (
P s y c h o s o c i a l f a c t o r s ma y c o n t r i b u t e t o p e r s i s t e n c e o f p a i n l o n g a f t e r t h e i n j u r y
o r i n f l a mma t i o n h a s r e s o l v e d . W i t h a d v a n c i n g a g e , f o c a l d e g e n e r a t i o n a n d
i s c h e mi a o f t h e r o t a t o r c u f f ma y o c c u r.
C. O t h e r s i t e s o f s i g n i f i c a n t s h o u l d e r p a i n d u e t o t r a u ma o r o v e r u s e i n c l u d e t h e
b i c e p s t e n d o n , a c r o mi o c l a v i c u l a r ( A C ) j o i n t , b r a c h i a l p l e xu s . P a i n ma y a l s o b e
r e f e r r e d t o s o u r c e s i n t h e n e c k , t h o r a x, a n d avbi de
d o me
i nfnr) a.(
I I I . Evaluation
A. History
H i s t o r y s h o u l d i n c l u d e p a i n o n s e t , t y p e , l o c a t i o n , s e v e r i t y, e f f e c t o n a c t i v i t i e s o r
w o r k , a n y t h e r a p y, h i s t o r y o f i n j u r y, a n d a s s o c i a t e d s y mp t o ms . A n a t o mi c l o c a l i za t i o n
485 / 652
tmdmss
o f t h e p a i n i s i mp o r t a n t . I n s p e c t i o n s h o u l d f o c u s o n t h e c r a n i u m, c e r v i c a l s p i n e , a n d
t h e e n t i r e u p p e r l i mb , i n c l u d i n g t h e d e s i g n a t e d p a i n f u l s i t e .
T h e s h o u l d e r i s e xa mi n e d f o r a t r o p h y, l o s s o f mu s c l e t o n e , f a s c i c u l a t i o n s , r e f l e x, a n
s e n s o r y c h a n g e s . T h e u p p e r l i mb i s t h e n i n s p e c t e d f o r s w e l l i n g , s k i n c h a n g e s , a n d
a b n o r ma l p o s t u r e . A c t i v e r a n g e o f mo t i o n ( f l e xi o n , e xt e n s i o n , a d d u c t i o n , a b d u c t i o n ,
me d i a l a n d l a t e r a l r o t a t i o n ) i s o b s e r v e d , a s i s t h e
P. 2 9 3
s c a p u l a r mo t i o n f r o m t h e r e a r. G l e n o h u me r a l mo t i o n i s a s s e s s e d b y s t a b i l i zi n g t h e
s c a p u l a w i t h t h e e xa mi n e r ' s h a n d . T h e s t e r n o c l a v i c u l a r a n d A C j o i n t s , c o r a c o i d
p r o c e s s , s p i n e o f s c a p u l a a n d c l a v i c l e , a c r o mi o n , r o t a t o r c u f f , a n d o t h e r s h o u l d e r
j o i n t mu s c l e s a r e a l l p a l p a t e d . S p e c i a l t e s t s p e r f o r me d t o d e t e c t c l i n i c a l e n t i t i e s
s p e c i f i c t o t h e s h o u l d e r a r e d e s c r Ti baebdl ei n1 2 . 9.. 1T h e N e e r i mp i n g e me n t s i g n i s
mo s t r e l i a b l e i n d i a g n o s i n g i mp i n g 4)
e me
. nt (
TAB L E 12.9.1 Special Tests to Confirm Shoulder

Pathology
Te s t
Mane uv e r
Pathology
Neer test
E xa mi n e r s t a b i l i ze s p a t i e n t s
S u b a c r o mi a l
( i mp i n g e me n t a c r o mi o n w i t h o n e h a n d , p e r f o r ms
i n f l a mma t i o n ,
sign)
ma xi mu m p a s s i v e s h o u l d e r
i mp i n g e me n t
abduction and internal rotation
w i t h t h e o t h e r.
H awkin's
i mp i n g e me n t
sign
F o r c e d i n t e r n a l r o t a t i o n w i t h t h eI mp i n g e me n t
s h o u l d e r f l e xe d f o r w a r d t o 9 0
J obe's test
( e mp t y c a n
test)
D e l t o i d a s s e s s e d w i t h a r m a t 9 0S u p r a s p i n a t u s
abduction and neutral rotation. weakness due to
S h o u l d e r t h e n i n t e r n a l l y r o t a t e di n j u r y, p a i n d u e t o
a n d a n g l e d f o r w a r d 3 0 ; t h u mb s r o t a t o r c u f f p a t h o l o g y
p o i n t i n g t o w a r d t h e f l o o r.
D o w n w a r d p r e s s u r e o f e xa mi n e r ' s
hand resisted.
Cross-body
adduction
test
S h o u l d e r f l e xe d t o 9 0 a n d
Rotator
a d d u c t e d a c r o s s t h e p a t i e n t ' s b oc du yf f / a c r o mi o c l a v i c u l a r
pathology
Lift-off test
P atient's arm behind back, with S ubscapularis

v o l a r s u r f a c e o f t h e h a n d r e s t i n mu
g scle
486 / 652
tmdmss
o n t h e s a c r u m. We a k n e s s i n l i f t iwnega k n e s s / i n j u r y
the hand away from the spine
Ye r g a s o n t e s t S u p i n a t i o n o f t h e p r o n a t e d
Biceps
forearm against resistance with tendonitis/tear
t h e e l b o w f l e xe d a t 9 0
S peed's test
E xt e n s i o n a n d s u p i n a t i o n o f t h e B i c e p s
e l b o w a s t h e e xa mi n e r r e s i s t s t e n d o n i t i s / t e a r
f l e xi o n o f t h e h u me r u s
A p p r e h e n s i o n A b d u c t i o n t o 9 0 a n d e xt e r n a l P o s i t i v e a n t e r i o r
test
r o t a t i o n o f t h e s h o u l d e r w i t h t h ei n s t a b i l i t y
e xa mi n e r ' s h a n d a p p l y i n g f o r w a r d
pressure to the scapula. T he
patient resists further shoulder
e xt e n s i o n
D r o p a r m t e s t T h e a r m i s p a s s i v e l y a b d u c t e d tRo o t a t o r c u f f t e a r o r
9 0 , a n d t h e s u p p o r t i s s u d d e n l ya xi l l a r y n e r v e i n j u r y
discontinued. A positive test is
reported if the arm drops abruptly
1. P a t h o l o g y i n v o l v i n g t h e g r e a t e r t u b e r o s i t y o r s u b a c r o mi a l s t r u c t u r e s o f t e n
results in pain on abduction. A painful arc (pain between 30 and 90 of
a b d u c t i o n ) s u g g e s t s i mp i n g e me n t o f t h e g r e a t e r t u b e r o s i t y u n d e r t h e a c r o mi o n
or supraspinatus tendonitis.
2. I n r o t a t o r c u f f t e a r s , a c t i v e a b d u c t i o n i s o f t e n l i mi t e d , a n d t h e r e i s w e a k n e s s
with abduction against resistance.
P. 2 9 4
3. A C j o i n t p a t h o l o g y i s i n d i c a t e d b y p a i n o n t o p o f t h e s h o u l d e r, w h i c h ma y r e f e r
t o t h e n e c k a n d j a w, a n d l o c a l j o i n t t e n d e r n e s s e xa g g e r a t e d b y a d d u c t i o n o f t h e
a r m.
4. C r e p i t u s o v e r t h e s h o u l d e r w h i l e r o t a t i n g t h e h u me r u s ma y i n d i c a t e s e v e r e
r o t a t o r c u f f d i s e a s e w i t h s e c o n d a r y g l e n o h u me r a l o s t e o a r t h r i t i s .
C. Testing
1. L a b o r a t o r y t e sAt sc o mp l e t e b l o o d c o u n t a n d a n a l y s i s o f s y n o v i a l o r b u r s a l
aspirate for cell count, Gram stain, and culture are obtained in suspected septic
a r t h r i t i s o r b u r s i t i s . I n s u s p e c t e d i n f l a mma t o r y a r t h r i t i s , a n e r y t h r o c y t e
s e d i me n t a t i o n r a t e o r C - r e a c t i v e p r o t e i n , r h e u ma t o i d f a c t o r, a n d a n t i n u c l e a r
a n t i b o d y t e s t s ma y b e h e l p f u l , a n d t h e j o i n t a s p i r a t e ma y b e e xa mi n e d f o r
crystals.
487 / 652
tmdmss
2. I m a g i n g s t u d i eSst a n d a r d a n t e r o p o s t e r i o r ( A P ) a n d l a t e r a l x- r a y s o f t h e
s h o u l d e r d e t e c t mo s t b o n e a n d j o i n t p a t h o l o g y. A xi l l a r y o r s p e c i a l Y v i e w s a r e
needed in suspected posterior dislocation; AP views with the arm in internal and
e xt e r n a l r o t a t i o n a r e u s e f u l f o l l o w i n g 5)
t r .a uVima
e w (s w i t h a n d w i t h o u t w e i g h t s
b e t t e r a s s e s s A C j o i n t s e p a r a t i o n . A r t h r o g r a p h y ma y b e u s e f u l i n p l a n n i n g
s u r g e r y f o r r o t a t o r c u f f t e a r s . U l t r a s o u n d i s a n o n i n v a s i v e , r e l a t i v e l y i n e xp e n s i v
mo d a l i t y ; i t d i a g n o s e s mo s t f u l l - t h i c k n e s s r o t a t o r c u f f t e a r s ( s e n s i t i v i t y 8 7 % ,
specificity 96%
2,6)
) ( . M a g n e t i c r e s o n a n c e i ma g i n g a l s o h a s h i g h s e n s i t i v i t y
( 8 0 % 1 0 0 % ) a n d s p e c i f i c i t y ( 8 8 % 9 4 % ) i n r o t a t o r c u2,6)
f f i n. j M
u rai eg sn e( t i c
r e s o n a n c e a r t h r o g r a p h y ( s e n s i t i v i t y 9 5 % , s p e c i f i c i t y 9 3 % i n c o mp l e t e r o t a t o r c u f
t e a r s ) ma y a l s o b e u s e d .
I V. Evaluation
T h e d i f f e r e n t i a l d i a g n o s i s i n c l u d e s mu s c u l o s k e l e t a l i n j u r y, t r a u ma , i n f e c t i o n , a n d
i n f l a mma t o r y c o n d i t i o n s . R e f e r r e d s o u r c e s s h o u l d a l s o b e c o n s i d e r e d a n d i n c l u d e
c e r v i c a l d i s c d i s e a s e , my o c a r d i a l i s c h e mi a , r e f l e x s y mp a t h e t i c d y s t r o p h y,
d i a p h r a g ma t i c i r r i t a t i o n a n d i n t r a t h o r a c i c t u mo r s , t h o r a c i c o u t l e t s y n d r o me , a n d
gallbladder disease.
1. I m p i n g e m e n t / s u b a c r o m i a l b u r s i t i s / r o t a t o r c u f f t e n d oPnai it ni si/st e a r
typically severe at night and worse with overhead activities. Rotator cuff tears
s h o u l d b e d i s t i n g u i s h e d f r o m i mp i n g e me n t / b u r s i t i s , b e c a u s e s u b s e q u e n t
e v a l u a t i o n a n d t r e a t me n t ma y b e d i f f e r e n t . P a i n e l i c i t e d b y t h e N e e r t e s t
c o n f i r ms i mp i n g e me n t ; i t r e s o l v e s o n s u b a c r o mi a l i n j e c t i o n o f 1 0 mL o f l i d o c a i n e
R o t a t o r c u f f t e n d o n i t i s i s s e e n mo r e o f t e n i n mi d d l e - a g e d o r o l d e r p a t i e n t s a n d
o f t e n c a u s e s l o s s o f a c t i v e r a n g e o f mo t i o n mo s t p r o n o u n c e d i n a b d u c t i o n ,
f o r w a r d f l e xi o n , a n d i n t e r n a l r o 4)
t a.t i T
o nh e( N e e r i mp i n g e me n t a n d J o b e ' s
t e s t s a r e p o s i t i v e , a n d t h e r e ma y b e w e a k n e s s t o r e s i s t e d a b d u c t i o n d u e t o p a i n
P a i n a n d w e a k n e s s a r e r e v e r s e d w i t h s u b a c r o mi a l l i d o c a i n e i n j e c t i o n . R o t a t o r
c u f f t e a r s ma y p r e s e n t w i t h s e v e r e p a i n w o r s e a t n i g h t , w e a k n e s s , a n d
d e c r e a s e d a c t i v e s h o u l d e r mo t i o n ; p a s s i v e r a n g e o f mo t i o n ma y b e n o r ma l . T h e
N e e r t e s t i s u s u a l l y p o s i t i v e ; s u b a c r o mi a l l i d o c a i n e i n j e c t i o n ma y r e s u l t i n p a i n
relief, but weakness to resisted abduction persists.
2. S h o u l d e r s t i f f n e s s ( f r o ze n s h o u l d e r, a d h e s i v e c a p s u l i t i s ) i s o f t e n s e e n i n o l d e r
p a t i e n t s a n d i n p a t i e n t s w i t h d i a4)
b eft oelsl o(w i n g u p p e r l i mb i mmo b i l i za t i o n ,
c a u s i n g a c h i n g p a i n a n d r e d u c t i o n s i n b o t h a c t i v e a n d p a s s i v e r a n g e s o f mo t i o n
3. S h o u l d e r d i s l o c a t i o n , i n s t a bMi ol ist ty d i s l o c a t i o n s a r e a n t e r i o r ( s u b c o r a c o i d )
and follow a fall on the outstretched hand or forced abduction/rotation injuries.
L a b r a l a n d r o t a t o r c u f f t e a r s ma y c o e xi s t . T h e a r m i s f i xe d i n a b d u c t i o n / e xt e r n a l
rotation, the shoulder is flattened, and there is a subcoracoid bulge.
R a d i o g r a p h s a r e c o n f i r ma t o r y a n d ma y r e v e a l a s s o c i a t e d f r a c t u r e s . P o s t e r i o r
dislocations follow forced adduction internal rotation, as occurring during a
488 / 652
tmdmss
s e i zu r e . I n f e r i o r d i s l o c a t i o n s f o l l o w mu s c l e a t r o p h y a s s o c i a t e d w i t h n e u r o l o g i c
disorders. Shoulder instability follows lesser grades of injury associated with
s p o r t i n g a c t i v i t i e s . A p o s i t i v e a p p r e h e n s i o n s i g n i s c o n f i r ma t o r y. R e c u r r e n t
i n s t a b i l i t y i s s e e n mo r e o f t e n i n y o u n g e r 4)
p a. t i e n t s (
4. G l e n o h u me r a l a r t h r i t i s a l s o p r o d u c e s s h o u l d e r p a i n t h a t w o r s e n s a t n i g h t , j o i n t
c r e p i t u s , a n d d e c r e a s e d a c t i v e a n d p a s s i v e r a n g e s o f mo t i o n . X - r a y s ma y s h o w
features of arthritis such as subchondral sclerosis, bone cysts, and osteophytes
P. 2 9 5
5. A C s e p a r a t i o n r e s u l t s f r o m a f a l l o n t o t h e a c r o mi o n , s e p a r a t i n g i t f r o m i t s d i s t a l
c l a v i c u l a r a r t i c u l a t i o n . T h e A C a n d c o r a c o c l a v i c u l a r ( c o n o i d a n d t r a p e zo i d )
l i g a me n t s a r e s t r e t c h e d o r t o r n . I n t y p e I s e p a r a t i o n , t h e r e i s l i g a me n t s p r a i n ,
j o i n t t e n d e r n e s s , a n d n o r ma l x- r a y s . I n t y p e I I , t h e r e i s A C l i g a me n t t e a r a n d
p r o mi n e n c e o f t h e d i s t a l c l a v i c l e , w h i c h i s h i g h e r t h a n t h e a c r o mi o n o n x- r a y. I n
t y p e I I I s e p a r a t i o n , c o mp l e t e l i g a me n t d i s r u p t i o n o c c u r s a n d t h e d i s t a l c l a v i c l e i
p r o mi n e n t a n d t e n d e r a n d i s s e e n a b o v e i t s a c r o mi a l a r t i c u l a t3)
i o.n o n x- r a y (
6. A C o s t e o a r t h r i t i s f o l l o w s a n A C s e p a r a t i o n o r e xc e s s i v e w e i g h t t r a i n i n g a n d i s
i n d i c a t e d b y p a i n l o c a l i ze d a t t h e t o p o f t h e s h o u l d e r, w o r s e n s w i t h a c t i v i t y a n d
i s e xa c e r b a t e d b y a d d u c t i o n . A C j o i n t p a l p a t i o n c a u s e s p a i n . R a d i o g r a p h s s h o w
osteoarthritis of the joint.
7. B i c e p s t e n d o n i t i s o r t e n d o n r u p t u r e t y p i c a l l y f o l l o w s r e p e t i t i v e mo t i o n o r t r a u ma
R u p t u r e p r e s e n t s w i t h p a i n a n d a n a b n o r ma l b u l g e i n t h e a n t e r i o r a r m a n d
tendonitis with shoulder pain and tenderness to palpation of the bicipital groove
8. P o l y my a l g i a r h e u ma t i c a i s s e e n i n o l d e r p a t i e n t s , w h o ma y a l s o h a v e p a i n a n d
s t i f f n e s s i n t h e c o n t r a l a t e r a l s h o u l d e r o r o t h e r j o i n t s . T e mp o r a l a r t e r i t i s ma y a l s
present with shoulder pain in association with headache and visual changes.
References
1 . Va n d e r w i n t D AW M , T h o ma s E , P o p e D P, e t a l . O c c u p a t i o n a l r i s k f a c t o r s f o r
s h o u l d e r p a i n : a s y s t e ma t i c r eOvci ec w.
up E nv i r on M ed
2000;57:433442.
2 . O xn e r K G . M a g n e t i c r e s o n a n c e i ma g i n g o f t h e mu s c u l o s k e l e t a l s y s t e m: P a r t 6 .
T h e s h o u l d eCr.l i n O r t hop
1997;334:354373.
4 . M c M a h o n P J , S a l l i s R E . T h e p a i n f u l s h o u l d e r. Z e r o i n g i n o n t h e mo s t c o mmo n
c a u s e s .P os t gr ad M ed
1999;106:3649.
5 . S t e v e n s o n H J , T r o j i a n T . E v a l u a t i o n o f s h o u l Jd eFr am
p a iPn r. ac t
2 0 0 2 ; 5 1 : 6 0 5 6 11 .
6 . D i n n e s J , L o v e ma n E , M c I n t y r e L , Wa u g h N . T h e e f f e c t i v e n e s s o f d i a g n o s t i c
t e s t s f o r t h e a s s e s s me n t o f s h o u l d e r p a i n d u e t o s o f t t i s s u e d i s o r d e r s : a
s y s t e ma t i c r e v i eHw.eal t h Tec hnol A s s es
2 0s0 3 ; 7 ( 2 9 ) ,
h t t p : / / w w w. c i n a h l . c o m/ c e xp r e s s / h t a / s u mm/ s u mm7 2 9 . p d f .
489 / 652
tmdmss
3 . S t e i n f e l d R , Va l e n t e R M , S t u a r t M J . A c o mmo n s e n s e a p p r o a c h t o s h o u l d e r
p r o b l e msM
. ay o C l i n P r oc
1999;74:785794.
490 / 652
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13.1 - Alopecia
> T a b l e o f C o n t e n t s > 1 3 - D e r ma t o l o g i c P r o b l e ms > 1 3 . 1 - A l o p e c i a
13.1
Alopecia
Kare n Hughe s
I . Background
A l o p e c i a , o r h a i r l o s s , i s a c o mmo n p r o b l e m. I t s h o u l d b e c o n s i d e r e d a s i g n o f i l l n e s
a n d n o t a d i a g n o s i s . H a i r l o s s c a n b e d i v i d e d i n t o t w o p a t t e r n s : g e n e r a l i ze d a n d
l o c a l i ze d .
A. Etiology
H a i r f o l l i c l e s h a v e t h r e e p h a s e s o f g r o w t h : a n a g e n , c a t a g e n , a n d t e l o g e n . N o r ma l l y,
hairs are shed at the end of telogen phase and the cycle restarts. Alopecia disorder
can be divided into two categories on the basis of whether cyclic hair growth is
a b n o r ma l o r t h e h a i r f o l l i c l e i s d a ma
1) . gGe ed n(e r a l i ze d h a i r l o s s h a s n o r ma l h a i r
f o l l i c l e s . L o c a l i ze d h a i r l o s s c a n h a v e e i t h e r n o r ma l o r a b n o r ma l h a i r f o l l i c l e s .
G e n e r a l i z e d a l o p e ci ni ac l u d e s t e l o g e n e f f l u v i u m, l o o s e a n a g e n s y n d r o me , f e ma l e
pattern androgenic alopecia, and postpartum alopecia. It can be induced by
c h e mo t h e r a p y, r a d i a t i o n t h e r a p y, a n a l g e s i c s , a n t i c o a g u l a n t s , a n t i e p i l e p t i c s ,
psychotropics, selective serotonin reuptake inhibitors, oral contraceptives and
e s t r o g e n s , p r o t o n - p u mp i n h i b i t o r s , c a r d i o v a s c u l a r d r u g s , i mmu n o s u p p r e s s a n t s , a s
w e l l a s p h y s i c a l a n d / o r me n t a l s t r e1)s .sLo or sc a( l i z e d a l o p e c i a w i t h n o r m a l h a i r
f o l l i c l e si n c l u d e s a n d r o g e n i c a l o p e c i a , a l o p e c i a a r e a t a , a n d t r a c t i o n a l o p e c i a .
L o c a l i z e d a l o p e c i a w i t h a b n o r m a l h a i r f ionl cl il cu ldeess a p l a s i a c u t i s c o n g e n i t a l ,
i n f e c t i o n s ( e . g . , k e r i o n ) , a u t o i mmu n e ( e . g . , d i s c o i d l u p u s ) , t r a u ma ( e . g . , t h e r ma l
b u r n s ) , s k i n c a r c i n o ma s , me t a s t a t i c a d e n o c a r c i n o ma , l y mp h o ma , a n d c i c a t r i c i a l
p e mp h i g u s .
B. Epidemiology
A l o p e c i a i s a c o mmo n p r o b l e m a f f e c t i n g b o t h g e n d e r s , a l l r a c e s , a n d a l l a g e s . A
v a r i e t y o f i l l n e s s e s r e s u l t i n a l o p e c i a , e a c h w i t h i t s o w n d i s t i n c t a g e , s e x, a n d r a c e
c h a r a c t e r i s t i c s a n d p a t t e r n s o f mo r b i d i t y a n d mo r t a l i t y.
I I I . Evaluation
A. History
T h e i mp o r t a n t q u e s t i o n s t o a s k a r e a s f o l l o w s : I s t h e h a i r l o s s g e n e r a l i ze d o r l o c a l ?
491 / 652
tmdmss
13.1 - Alopecia
Wa s t h e l o s s g r a d u a l o v e r t i me o r s u d d e n ? We r e a n y n e w me d i c a t i o n s , p r e s c r i p t i o n ,
o r o v e r - t h e - c o u n t e r d r u g s t a k e n ? We r e a n y n e w h a i r g r o o mi n g p r o d u c t s , p r o c e d u r e s
o r t r e a t me n t s u s e d ? We r e a n y f a mi l y me mb e r s k n o w n t o h a v e h a d s i mi l a r p a t t e r n s o
h a i r l o s s ? We r e a n y p r e c e d i n g p s y c h o s o c i a l o r p h y s i c a l s t r e s s o r s p r e s e n t ?
G e n e r a l i z e d h a i r l ocsasn b e s e e n i n t h e s e c o n d i t i o n s . A c u t e t e l o g e n e f f l u v i u m i s
u s u a l l y d e s c r i b e d a s f i n d i n g h a n d f u l s o f l o s t h a i r s i n t h e s h o w e r, i n t h e h a i r b r u s h
o n t h e p i l l o w o r b a t h r o o m f l o o r, w h i c h c a u s e s c o n s i d e r a b l e e mo t i o n a l d i s t r e s s .
T e l o g e n e f f l u v i u m c a n u n ma s k l o c a l i ze d a l o p e c i a t h a t h a s b e e n h i d d e n b y t h e
p r e v i o u s l y t h i c k e r h a i r g r o1)w. t hB o( t h ma l e a n d f e ma
a nl d
e r o g e n i c a l o p e cairae
i n s i d i o u s a t o n s e t . M o s t o t h e r e t i o l o g i e s a r e mo r e s u d d e n a t o n s e t . H i s t o r y o r
p r e s e n c e o f k n o w n i r o n d e f i c i e n c y, t h y r o i d p r o b l e ms , s p r u e , o r ma l n u t r i t i o n ma y l e a
t o t h e e t i o l o gSy.t r es s or sl e a d i n g t o t e l o g e n e f f l u v i u m i n c l u d e a s e v e r e a c u t e o r
c h r o n i c i l l n e s s , ma j o r s u r g e r y, a n o r e xi a ( ma l n u t r i t i o n ) , c r a s h d i e t i n g , e xc e s s i v e
e xe r c i s i n g l e a d i n g t o a me n o r r h e a , a n d s e v e r e p s y c h o l o g i c s t r e s s . A n y n e w
me d i c a t i o n s o r h a i r t r e a t me n t s t h a t a r e t e mp o r a l l y r e l a t e d t o t h e h a i r l o s s n e e d t o b
c o n s i d e r e d a s p o s s i b l e c a u s e s . U s e o f c h e mi c a l o r t h e r ma l h a i r t r e a t me n t s s u c h a s
h a i r s t r a i g h t e n i n g , c u r l i n g , o r p e r ma n e n t w a v i n g c a n l e a d t o c u mu l a t i v e d a ma g e . H a
weaving or braiding that is too tight can lead to traction alopecia.
T h e t e xt u r e , l e n g t h , a n d t h i c k n e s s o f i n d i v i d u a l h a i r s ma y s u g g e s t t h e c a u s e o f h a i r
l o s s . S h o r t e r, f i n e h a i r s ma y b e f o u n d i n a r e a s o f t h i n n i n g i n a n d r o g e n i c a l o p e c i a .
E xc l a ma t i o n p o i n t h a i r s w h i c h h a v e a d i s t a l
P. 3 0 0
b r o k e n s h a f t a n d a p r o xi ma l c l u b - s h a p e d h a i r r o o t , a r e s e e n a t t h e p e r i p h e r y o f h a i r
l o s s i n a l o p e c i a a r e a2)t .a S( h o r t b r o k e n h a i r s i n t h e a r e a o f l o s s o c c u r w i t h
t r i c h o t i l l o ma n i a a n d t i n e a c a p i t i s . L o n g e y e l a s h e s a n d s t r a i g h t e n i n g o f s c a l p h a i r c a
b e s e e n i n h u ma n i mmu n o d e f i c i e n c y v i r u s ( H I V ) i n f e c t i o n . A l l a r e a s o f t h e b o d y mu s
b e c a r e f u l l y e xa mi n e d f o r h a i r g r o w t h p a t t e r n s a n d c h a n g e s . T r i c h o t i l l o ma n i a c a n b e
s e e n i n t h e s c a l p , e y e b r o w a r e a s , a n d e v e n e y e l a s h e s . P a t t e r n s o f h a i r l o s s i n ma l e
p a t t e r n a n d r o g e n i c a l o p e c i a t y p i c a l l y r a n g e f r o m b i t e mp o r a l r e c e s s i o n , t o f r o n t a l a n
v e r t e x t h i n n i n g , a n d t o l o s s o f a l l h a i r e xc e p t f o r o c c i p i t a l a n d t e mp o r a l f r i n g e s .
F e ma l e p a t t e r n a l o p e c i a i s s e e n a s d i f f u s e t h i n n i n g t h a t i s mo r e p r o mi n e n t i n t h e
f r o n t a l o r p a r i e t a l a r e a s , w i t h s p a r i n g o f t h e f r o n2)t a. l I nf r iwnogme
e (n , w a t c h f o r
s i g n s o f v i r i l i za t i o n t h a t c a n b e a s s o c i a t e d w i t h a n d r o g e n i c a l o p e c i a . Vi r i l i za t i o n c a
also cause acne, hypertrichosis in other areas, deepening of the voice, and
c l i t o r o me g a l y. R a s h e s o r o t h e r c h a n g e s i n t h e s k i n e i t h e r a t t h e a r e a o f h a i r l o s s o r
e l s e w h e r e ma y s u g g e s t v a r i o u s c a u s e s . S c a l i n g a n d f l a k i n g s u g g e s t t i n e a , p s o r i a s i s
o r d r y i n g o f t h e s k i n a s a r e s u l t o f h e a t o r c h e mi c a l s . S c a r r i n g o f t h e a r e a s o f h a i r
l o s s s u g g e s t t r a u ma , i n f e c t i o n , o r d i s c o i d l u p u s . A mo t h e a t e n p a t t e r n o f h a i r l o s s
on either scalp or face should suggest syphilis, sarcoid, or discoid lupus. Telogen
e f f l u v i u m c a n b e d e t e c t e d b y t h e p u l l t e s t , w h i c h i s p e r f o r me d b y g r a s p i n g
a p p r o xi ma t e l y 6 0 h a i r s b e t w e e n t h u mb a n d f i n g e r s a n d p u l l i n g g e n t l y b u t f i r ml y.
N o r ma l s h e d d i n g s h o u l d y i e l d s i x o r f e w e r h a i r s . H a i r s h o u l d n o t h a v e b e e n
s h a mp o o e d w i t h i n 2 4 h o u r s o f t h i 1)
s .t e s t (
C. Testing
492 / 652
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13.1 - Alopecia
1. L a b o r a t o r y t e s t s s h o u l d b e o r d e r e d o n t h e b a s i s o f c l i n i c a l f i n d i n g s . A t y p i c a l
a n d r o g e n i c a l o p e c i a p a t t e r n o f h a i r l o s s w i t h n o r ma l s k i n i n me n a n d w o me n
w i t h o u t e v i d e n c e o f v i r i l i za t i o n r e q u i r e s n o f u r t h e r t e s t i n g . Wo me n w h o s h o w
e v i d e n c e o f v i r i l i za t i o n s h o u l d h a v e a f r e e t e s t o s t e r o n e , t o t a l t e s t o s t e r o n e ,
prolactin, and dehydroepiandrosterone sulfate ( D H E A- S ) levels drawn first. If
t h e s e a r e a b n o r ma l , t h e n w o r k u p s h o u l d c o n t i n u e . P a t i e n t s w i t h n o n a n d r o g e n i c
p a t t e r n s o f a l o p e c i a s h o u l d h a v e t h y r o i d f u n c t i o n t e s t s , a c o mp l e t e b l o o d c o u n t ,
ferritin level, and an antinuclear antibody done. T he need for syphilis serology
s h o u l d a l s o b e c o n s i d e r e d o n t h e b a s i s o f h i s t o r y a n d e xa mi n a t i o n . I f t h e s e t e s t
a r e a l l n o r ma l , o t h e r n u t r i t i o n a l d e f i c i e n c i e s s u c h a s zi n c d e f i c i e n c y s h o u l d b e
c o n s i d e r e d1)(. B a c t e r i a l a n d f u n g a l c u l t u r e s o f a n y d r a i n a g e s h o u l d b e
o b t a i n e d . P o t a s s i u m h y d r o xi d e mi c r o s c o p i c e v a l u a t i o n o f s k i n s c r a p i n g s f o r
f u n g a l e l e me n t s a n d / o r f u n g a l c u l t u r e s o f s c a l i n g a r e a s c a n c o n f i r m t h e
diagnosis of fungal infection.
2. S c a l p b i o p s y i s o f t e n h e l p f u l e s p e c i a l l y w h e r e s c a r r i n g i s a s s o c i a t e d w i t h h a i r
loss; biopsy can distinguish between sarcoid, discoid lupus, and lichen
p l a n o p i l a r i s . T h e b i o p s y s h o u l d b e t a k e n w i t h a 4 t o 5 mm p u n c h o f t h e a c t i v e
a r e a o f a l e s i o n . A r e a s o f i n f i l t r a t i o n ma y b e s c l e r o d e r ma o r a l o c a l o r me t a s t a t i
c a r c i n o ma . F o l l i c u l a r s t r u c t u r e a n d n u mb e r a s w e l l a s t h e s t a g e s o f h a i r g r o w t h
can be evaluated.
D. Genetics
T h e h a i r f o l l i c l e i s t h e mo s t c o mp l e x o r g a n o f t h e e p i d e r ma l s t r u c t u r e s . T h e r e a r e
mo r e t h a n 3 0 0 g e n e t i c a b n o r ma l i t i e s t h a t a f 3)
f e .c t h a i r (
I V. Diagnosis
T h e k e y c o n s i d e r a t i o n i n d i a g n o s i s i s w h e t h e r t h e h a i r l o s s i s l o c a l i ze d o r
g e n e r a l i ze d . S u b s e q u e n t e xa mi n a t i o n s a s n o t e d e a r l i e r a l l o w a d e f i n i t i v e d i a g n o s i s .
Most cases of alopecia are caused by either androgenic alopecia or telogen

e f f l u v i u m. T h e s e e t i o l o g i e s d o n o t r e q u i r e t r e a t me n t . E a r l y d i a g n o s i s a n d
i n t e r v e n t i o n c a n b e c r i t i c a l i n t h e r e ma i n i n g c a s e s i f c a u s e d b y t h y r o i d d i s o r d e r,
c a r c i n o ma , me t a s t a t i c a d e n o c a r c i n o ma , me l a n o ma , s y p h i l i s , o r H I V. P e r ma n e n t h a i r
l o s s ma y b e p r e v e n t e d o r l i mi t e d b y e a r l y i n s t i t u t i o n o f t h e r a p y w h e n a l o p e c i a i s
c a u s e d b y e xc e s s i v e t r a c t i o n , i n f e c t i o n , o r o t h e r i n f i l t r a t i v e o r s c a r r i n g p r o c e s s e s .
the alopecia is caused by a drug, hair loss can be reversible if the drug is stopped
e a r l y i n t h e p r o c e s s . T r e a t me n t , i f d e s i r e d , f o r a n d r o g e n i c a l o p e c i a w o r k s b e s t i f
started early on in hair loss. Resolution of the
P. 3 0 1
stressful event that precipitates acute telogen effluvium is often difficult and, in so
c a s e s , c a n o n l y b e ma n a g e d b y t i me . R e a s s u r a n c e a n d s u p p o r t a r e t h e b e s t t h e r a p y
References
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13.1 - Alopecia
1 . T h i e d k e C . A l o p e c i a i n w o me
A mn F
. am P hy s i c i an
2003;67(5):10171018.
2 . B e r t o l i n o A . A l o p e c i a a r ePaos
t a t. gr ad M ed
2000;107(7):8190.
3. Irvine A, C hristiano A. H air on a gene string: recent advances in understanding
t h e mo l e c u l a r g e n e t i c s o f h a i rClloi ns sE. x p D er mat2ol0 0 1 ; 2 6 : 5 9 7 1 .
494 / 652
tmdmss
13.2 - Erythema Multiforme
> T a b l e o f C o n t e n t s > 1 3 - D e r ma t o l o g i c P r o b l e ms > 1 3 . 2 - E r y t h e ma M u l t i f o r me
13.2
Erythema M ultiforme
R a y T. P e r r i n e
I . Background
E r y t h e ma mu l t i f o r me ( E M ) d e s c r i b e s a s y n d r o me o f s y mp t o ms . I t i s a h y p e r s e n s i t i v i
r e a c t i o n t r i g g e r e d b y v a r i o u s s t i mu l i o r b y r e a c t i v a t i o n o f a l a t e n t i n f e c t i o n . E M mi n
is an eruption that is confined to the skin as typical acral target lesions with or
w i t h o u t mu c o s a l i n v o l v e me n t . E M ma j o r i s a mu c h mo r e s e r i o u s i l l n e s s a n d i n c l u d e s
t h e s y n d r o me s o f e r y t h e ma mu l t i f o r me ma j u s ( E M M ) , S t e v e n s - J o h n s o n s y n d r o me
( S J S ) , a n d t o xi c e p i d e r ma l n e c r o l y s i s ( T E N ) . M a n y n o w b e l i e v e t h a t S J S a n d T E N
are a single entity distinguished only by the severity of the illness. Although E M M i
a s e v e r e f o r m, i t i s c o n s i d e r e d t o b e a s e p a r a t e e n t i t y f r o m S J S / T E N i n t h a t i t d i f f e
i n d e mo g r a p h i c s a n d c a u1)
s e. s (
A. Etiology
E M mi n o r, E M M , S J S , a n d T E N a r e b e l i e v e d t o b e h y p e r s e n s i t i v i t y r e a c t i o n s
t r i g g e r e d b y v a r i o u s s t i mu l i o r b y r e a c t i v a t i o n o f a l a t e n t i n f e c t i o n . R e c e n t h e r p e t i c
i n f e c t i o n s a r e t h e mo s t c o mmo n t r i g g e r s ; r e c e n t w o r k i mp l i c a t e s t r a n s p o r t o f v i r a l
p a r t i c l e s t o t h e s k i n b y C D 3 4 2)
c e. l O
l sxi( c a m n o n s t e r o i d a l a n t i - i n f l a mma t o r y d r u g s ,
a l l o p u r i n o l , p h e n o b a r b i t a l , p h e n y t o i n , a n d s u l f o n a mi d e s a r e d r u g s t h a t a r e h i g h l y
s u s p e c t i n l e a d i n g t o mo r e s e v e r e d i s1)e .a sDersu g( - r e l a t e d E M a p p e a r s t o i n v o k e
a t u mo r n e c r o s i s f a c t o r me c h a n i s m, w h e r e a s h e r p e s s i mp l e x v i r u s ( H S V ) r e l a t e d
i l l n e s s d o e s n o3)t . (
B. Epidemiology
E M M i n o r, E M M , S J S , a n d T E N o c c u r w o r l d w i d e , i n i n d i v i d u a l s o f a n y a g e ,
a l t h o u g h mo s t p a t i e n t s a r e y o u n g e r t h a n 4 0 y e a r s o f a g e . D r u g - r e l a t e d i l l n e s s t e n d s
t o b e mo r e s e v e r e a n d mo r e o f t e n a s s o c i a t e d w1)
i t .h STJESN/ T(E N a r e
a c c o mp a n i e d b y h i g h e r mo r b i d i t y a n d mo r t a l i t y r a t e s .
I I I . Evaluation
A. History
T h e p r i ma r y f o c u s o f t h e h i s t o r y i s o n w h e t h e r t h e r e w e r e a n y p r o d r o ma l s y mp t o ms .
E M mi n o r i s o f t e n a s y mp t o ma t i c . S o me p a t i e n t s ma y e xp e r i e n c e i t c h i n g o r
495 / 652
tmdmss
t e n d e r n e s s . T h e c o n d i t i o n i s o f s u d d e n o n s e t a n d g e n e r a l l y d o e s n o t c a u s e s y s t e mi
s y mp t o ms . I n E M M / S J S / T E N , t w o - t h i r d s o f p a t i e n t s p r e s e n t w i t h o u t e xp e r i e n c i n g a
p r o d r o me . I n t h e o n e - t h i r d o f c a s e s i n w h i c h p r o d r o ma l s y mp t o ms o c c u r, t h e s e
u s u a l l y a p p e a r i n t h e f o r m o f a n u p p e r r e s p i r a t o r y i l l n e s s w i t h f e v e r, ma l a i s e , a n d
my a l g i a s . A n o t h e r f o c u s i s o n r e c e n t e xp o s u r e s . H a s t h e p a t i e n t b e e n e xp o s e d t o
a n y d r u g s 1 t o 3 w e e k s p r i o r t o o n s e t ? T h e s e mo s t o f t e n i n c l u d e s u l f o n a mi d e s ,
p e n i c i l l i n , a n t i c o n v u l s a n t s , a n d n o n s t e r o i d a l a n t i - i n f l a mma t o r y d r u g s . H a s t h e p a t i e
b e e n i l l r e c e n t l y ? M o s t c a s e s o f E M mi n o r, p a r t i c u l a r l y t h o s e w i t h r e c u r r e n c e , a r e
a s s o c i a t e d w i t h H S V i n f e cM
t i yo cnopl
. as map n e u mo n i a , t u b e r c u l o s i s ( T B ) , h e mo l y t i c s t r e p t o c o c c u s , s t a p h y l o c o c c a l i n f e c t i o n s , r a d i a t i o n t h e r a p y, c o l l a g e n
v a s c u l a r d i s e a s e s , p r e g n a n c y, c a r c i n o ma s , a n d t h e b a c i l l u s C a l me t t e - G u r i n v a c c i n
h a v e a l s o b e e n i mp l i c a t e d .
P. 3 0 2
C h a r a c t e r i s t i c s o f t h e r a s h a r e i mp o r t a n t t o d e t e r mi n e t h e d i s e a s e . T h e c l a s s i c
a p p e a r a n c e o f E M , t h e t a r g e t l e s i o n , i s a d u s k y, r e d d i s h , c e n t r a l p a p u l e s u r r o u n d e d
by a red ring. T he lesions blanch partially with pressure. No scaling is seen. E M
mi n o r ma n i f e s t s a s t a r g e t o r i r i s l e s i o n s t h a t o f t e n a p p e a r a t t h e e xt r e mi t i e s ( e . g . ,
p a l ms , s o l e s ) a n d s o me t i me s o n t h e f a c e o r t r u n k . T h e d i s t r i b u t i o n i s s y mme t r i c . T h
l e s i o n s d e v e l o p o v e r 1 0 d a y s o r mo r e a n d r e s o l v e o n t h e i r o w n , u s u a l l y i n 1 t o 6
w e e k s . R e c u r r e n c e s a r e c o mmo n a n d ma y c o n t i n u e f o r y e a r s . L e s i o n s t h a t a r e
i r r e g u l a r, o r o c c u r i n l a r g e e r y t h e ma t o u s p a t c h e s , b l i s t e r, o r b u l l a e w i t h s l o u g h i n g i
l a r g e s h e e t s a r e h i g h l y s u g g e s t i v e o f S J S a n d t h e mo r e s e v e r e T E N . S J S / T E N
l e s i o n s a r e e r y t h e ma t o u s o r p u r p u r i c ma c u l e s w i t h i r r e g u l a r s h a p e a n d s i ze . B l i s t e r
o f t e n o c c u r o n a l l o r p a r t o f t h e ma c u l e . L e s i o n s a r e w i d e s p r e a d . I n T E N , c o n f l u e n t
b l i s t e r s r e s u l t i n t h e d e t a c h me n t o f t h e e p i d e r mi s a n d e r o s i o n s o n 1 0 % t o mo r e t h a n
3 0 % o f t h e b o d y s u r f a c e a r e a . L a r g e e r y t h e ma t o u s a r e a s w i t h o u t d i s c r e t e l e s i o n s
c a n b e p r e s e n t . S y s t e mi c s i g n s o f h i g h f e v e r, i n v o l v e me n t o f t h e e y e s w i t h c o r n e a l
u l c e r a t i o n , p u l mo n a r y f i n d i n g s , w i d e s p r e a d c u t a n e o u s i n v o l v e me n t , o r p n e u mo n i a a r
p r e s e n t i n E M ma j o r.
C. Testing
P u n c h b i o p s y ma y b e u s e f u l i n t h e c a s e o f a t y p i c a l l e s i o n s . T y p i c a l f i n d i n g s i n c l u d e
v a c u o l i za t i o n o f t h e b a s a l c e l l l a y e r, w i t h l y mp h o c y t e s a l o n g t h e d e r ma l e p i d e r ma l
j u n c t i o n4)( . N e c r o s i s o f i n d i v i d u a l k e r a t i n o c y t e s i s s e e n i n t h e e p i d e r mi s , w h e r e a s
p e r i v a s c u l a r l y mp h o c y t i c i n f i l t r a t e s ma y b e p r e s e n t i n t h e d e r mi s . I f u n d e r l y i n g
i n f e c t i o n i s s u s p e c t e d , o t h e r l a b o r a t o r y t e s t s ma y b e i n d i c a t e d , i n c l u d i n g a c o mp l e t
b l o o d c o u n t , t h r o a t c u l t u r e , a n t i s t r e p t o l y s i n - O t i t e r, a n d a s l i d e t e s t f o r i n f e c t i o u s
mo n o n u c l e o s i s , a n d h e p a t i t i s s c r e e n ma y b e i n d i c a t e d . A c h e s t x- r a y ma y b e n e e d e d
i f M y c opl as ma pneumoni
, hae
i s t o p l a s mo s i s , c o c c i d i o my c o s i s , o r T B i s s u s p e c t e d .
S k i n t e s t s o r s e r u m c o mp l e me n t f i xa t i o n t i t e r s f o r i n f e c t i o u s a g e n t s ma y b e n e e d e d .
D. Genetics
T h e r e i s n o w e l l - d e f i n e d g e n e t i c b a s i s t o E M mi n o r, S J S , o r T E N . T h e t r i g g e r i n g
a g e n t i n mo s t c a s e s a p p e a r s t o b e i n f e c t i o u s o r d r u g - r e l a t e d e xp o s u r e .
496 / 652
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I V. Diagnosis
A s y s t e mi c , f i xe d , d i s c r e t e , r o u n d , e r y t h e ma t o u s r a s h , w h i c h l a s t s 1 t o 6 w e e k s f r o m
o n s e t t o h e a l i n g a n d i s s e l f - l i mi t e d , a c u t e , o r e p i s o d i c , s a t i s f i e s t h e c l i n i c a l c r i t e r i a
f o r E M mi n o r. B i o p s y ma y b e n e e d e d f o r c o n f i r ma t i o n . A s y s t e mi c , e r y t h e ma t o u s
rash, with irregular and target lesions, blisters, sloughing when the patient is
s y s t e mi c a l l y i l l i s mu c h mo r e i n d i c a t i v e o f S J S / T E N .
D e t e r mi n i n g w h i c h s u b t y p e o f E M i s p r e s e n t h e l p s d i c t a t e t r e a t me n t a n d a n t i c i p a t e
p r o g n o s i s . E M mi n o r i s a l i mi t e d i l l n e s s w i t h l i t t l e mo r b i d i t y a n d mo r t a l i t y, a l t h o u g h
r e c u r r e n c e s a r e c o mmo n . S J S / T E N a r e o f t e n l i f e - t h r e a t e n i n g i l l n e s s e s w i t h
c o n s i d e r a b l e mo r b i d i t y.
References
1. Auquier- D unant A, M ockenhaupt M , N alda L , et al. C orrelations between
c l i n i c a l p a t t e r n s a n d c a u s e s o f e r y t h e ma mu l t i f o r me ma j u s , S t e v e n s - J o h n s o n
s y n d r o me , a n d t o xi c e p i d e r ma l n e c r o l y s i s : r e s u l t s o f a n i n t e r n a t i o n a l p r o s p e c t i v e
s t u d y.A r c h D er mat ol
2002;138:10191024.
2 . O n o F, S h a r ma B , S mi t h C , e t a l . C D 3 4 + C e l l s i n t h e p e r i p h e r a l b l o o d
t r a n s p o r t h e r p e s s i mp l e x v i r u s D N A f r a g me n t s t o t h e s k i n o f p a t i e n t s w i t h
e r y t h e ma mu l t i f o r me ( H A EJ MI nv
) . es t D er mat2ol0 0 5 ; 1 2 4 ( 6 ) : 1 2 1 5 1 2 2 4 .
3 . K o k u b a H , A u r e l i a n L , B u r n e t t J . H e r p e s s i mp l e x v i r u s a s s o c i a t e d e r y t h e ma
mu l t i f o r me ( H A E M ) i s me c h a n i s t i c a l l y d i s t i n c t f r o m d r u g - i n d u c e d e r y t h e ma
mu l t i f o r me : i n t e r f e r o n - i s e xp r e s s e d i n H A E M l e s i o n s a n d t u mo r n e c r o s i s f a c t o r i n d r u g - i n d u c e d e r y t h e ma mu l t i f o r me lJe sI inv
o nes
s . t D er mat1ol9 9 9 ; 11 3 ( 5 ) : 8 0 8
815.
4 . K a t t a R . T a k i n g a i m a t e r y t h e ma mu l tPi fos
o r tme
gr ad
. M ed
2000;107(1):87.
497 / 652
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13.3 - Maculopapular Rash
> T a b l e o f C o n t e n t s > 1 3 - D e r ma t o l o g i c P r o b l e ms > 1 3 . 3 - M a c u l o p a p u l a r R a s h
13.3
M aculopapular Rash
Jam e s R. Lundy
I . Background
M a c u l o p a p u l a r d e s c r i b e s a r a s h t h a t c o n t a i n s b o t h ma c u l e s a n d p a p u l e s . A
ma c u l o p a p u l a r r e a c t i o n i s u s u a l l y a l a r g e a r e a t h a t i s r e d a n d h a s s ma l l , c o n f l u e n t
b u mp s .
A. Etiology
W h e n f e v e r i s p r e s e n t , t h e r a s h i s u s u a l l y d u e t o i n f e c t i o n . R a s h e s n o t a c c o mp a n i e
b y f e v e r r e g u l a r l y r e s u l t f r o m a l l e r g i c r e a c t i o n s . R a r e l y, a ma c u l o p a p u l a r r a s h i s a
s y s t e mi c s i g n o f a n u n d e r l y i n g ma l i g n a n c y. S e r i o u s i n f e c t i o u s i l l n e s s e s , s u c h a s
me n i n g o c o c c e mi a , d i s s e mi n a t e d g o n o r r h e a , a n d R o c k y M o u n t a i n s p o t t e d f e v e r
( R M S F ) c a n i n i t i a l l y p r e s e n t w i t h a c u t e o n s e t o f ma c u l o p a p u l a r r a s h a n d f e v e r.
A n a p h y l a xi s o c c a s i o n a l l y p r e s e n t s e a r l y i n t h e c o u r s e w i t h a ma c u l o p a p u l a r r a s h ,
w i t h p a l ma r o r p h a r y n g e a l i t c h i n g .
B. Epidemiology
M a c u l o p a p u l a r r a s h i s t h e r e s u l t o f v a r i e d d i s e a s e s . T h e p r e v a l e n c e , mo r b i d i t y, a n d
mo r t a l i t y i n v a r i o u s a g e s , s e xe s , a n d r a c e p o p u l a t i o n s r e f l e c t t h e u n d e r l y i n g d i s e a s
I I I . Evaluation
A. History
Pertinent history of present illness includes where the rash started; how far it has
s p r e a d ; w h a t a s s o c i a t e d s y mp t o ms , s u c h a s f e v e r, i t c h i n g , b u r n i n g o r p a i n ,
a c c o mp a n y t h e l e s i o n s ; a n d i t s p r e v i o u s o c c u r r e n c e . I f i t h a s a p p e a r e d b e f o r e , i t
h e l p s d e t e r mi n e w h a t t r e a t me n t w a s u s e d a n d t h e p a t i e n t ' s r e s p o n s e a s w e l l a s t h e
e xi s t e n c e o f a n y s i c k c o n t a c t s . O t h e r q u e s t i o n s o f i mp o r t a n c e i n c l u d e t h e f o l l o w i n g :
d o e s t h e p a t i e n t h a v e a n y c h r o n i c me d i c a l c o n d i t i o n s ; w h a t a r e t h e p a t i e n t ' s c u r r e n
me d i c a t i o n s , i n c l u d i n g o v e r - t h e - c o u n t e r a n d h e r b a l t h e r a p i e s a n d h a v e a n y o f t h e s e
drugs been started recently; and does the patient have any known allergies. T he
s o c i a l h i s t o r y i n c l u d i n g o c c u p a t i o n , h o b b i e s , n e w s e xu a l p a r t n e r s , a n d t r a v e l ma y
also be significant.
498 / 652
tmdmss
A g e n e r a l p h y s i c a l e xa mi n a t i o n s h o u l d b e c o n d u c t e d . E xa mi n a t i o n s h o u l d f o c u s o n
the distribution of the rash. Consider centrally and peripherally distributed eruption
s e p a r a t e l y1)(. A c e n t r a l r a s h ( f a c e a n d t h e t r u n k ) i s c h a r a c t e r i s t i c o f v i r a l i l l n e s s e s
i n c l u d i n g me a s l e s a n d r u b e l l a . A p e r i p h e r a l r a s h s t a r t s o n t h e e xt r e mi t i e s . T h e r a s h
o f me n i n g o c o c c e mi a o f t e n s t a r t s p e r i p h e r a l l y, p r e s e n t i n g a s a ma c u l e w i t h c e n t r a l
petechiae, which then progresses to a nodule as the rash then appears and spreads
w i d e l y. A ma c u l o p a p u l a r r a s h o c c u r r i n g o n t h e p a l ms s h o u l d p r o mp t c o n c e r n a b o u t
s y p h i l i s , R M S F, o r d i s s e mi n a t e d g o n o r r h e a . T h e l e s i o n s o f g o n o r r h e a a r e u s u a l l y
a c r a l ( a f f e c t i n g l i mb s a n d d i g i t s ) a n d q u i c k l y b e c o me p u s t u l a r.
1. H e a d , e y e s , e a r s , n o s e , a n d t h r o a t ( H E E N T ) e x a m
T hi ne ast ci oa nl p i s a
c o mmo n l o c a t i o n f o r t i c k s , w h i c h l e n d s s u p p o r t t o t h e d i a g n o s i s o f R M S F.
M u c o u s me mb r a n e s w e l l i n g ma y i n d i c a t e e a r l y a n a p h y l a xi s . K o p l i k ' s s p o t s i n t h e
o r o p h a r y n x a r e p a t h o g n o mo n i c f o r me a s l e s .
2. L u n g e x a m i n a t i oWnh e e zi n g o n e xa mi n a t i o n c a n i n d i c a t e a n a p h y l a xi s .
3. G e n i t o u r i n a r y e x a m i n a tPi ou nr u l e n t d i s c h a r g e o r e v i d e n c e o f p e l v i c
i n f l a mma t o r y d i s e a s e i n d i c a t e s g o n o r r h e a . A c h a n c r e i n d i c a t e s p r i ma r y s y p h i l i s ,
a l t h o u g h p a l ma r l e s i o n s ( s e c o n d a r y s y p h i l i s ) o f t e n o c c u r w e l l a f t e r t h e h e a l i n g o
the initial chancre.
4. E x t r e m i t i e s e x a m i n a t E
i ovni d e n c e o f j o i n t s w e l l i n g i n d i c a t e s
me n i n g o c o c c e mi a , g o n o c o c c e mi a , o r r h e u ma t o l o g i c c o n d i t i o n s .
5. N e u r o l o g i c e x a m i n a t iEovni d e n c e o f me n i n g i t i s i n d i c a t e s me n i n g o c o c c e mi a o r
R M S F.
P. 3 0 4
C. Testing
T h e c o mp l e t e b l o o d c o u n t ( C B C ) i s v a l u a b l e . A n e l e v a t e d w h i t e b l o o d c e l l c o u n t w i t
a l e f t s h i f t ma y i n d i c a t e a b a c t e r i a l i n f e c t i o n ; l y mp h o c y t o s i s ma y i n d i c a t e a v i r a l
i n f e c t i o n ; e o s i n o p h i l s a r e s o me t i me s i n c r e a s e d w i t h a l l e r g i c r e a c t i o n s ; a n d r a r e l y,
my e l o g e n o u s l e u k e mi a s c a n p r e s e n t w i t h r a s h a n d a b n o r ma l i t i e s o n C B C . O t h e r
t e s t i n g s h o u l d b e p e r f o r me d o n t h e b a s i s o f t h e mo s t l i k e l y c a u s e s o f t h e r a s h .
C o n s i d e r t h e r a p i d p l a s ma r e a g i n t e s t a n d g o n o c o c c e mi a i n s e xu a l l y a c t i v e p a t i e n t s
C o n s i d e r a s me a r a n d c u l t u r e o f a n y p u s t u l e s , e s p e c i a l l y i f me n i n g o c o c c e mi a o r
g o n o c o c c e mi a i s s u s p e c t e d . C e r e b r o s p i n a l f l u i d e xa mi n a t i o n i s u s e f u l i f
me n i n g o c o c c e mi a i s s u s p e c t e d ; i t i s u s u a l l y n e g a t i v e i n R M S F. C o n s i d e r a n
e r y t h r o c y t e s e d i me n t a t i o n r a t e i f t h e r e i s j o i n t i n v o l v e me n t .
D. Genetics
M aculopapular rash is the result of varied diseases. Any genetic basis is reflective
the underlying disease. Most underlying causes are infectious and lack a genetic
basis.
I V. Diagnosis
499 / 652
tmdmss
M a c u l o p a p u l a r e r u p t i o n s a r e mo s t f r e q u e n t l y s e e n i n v i r a l i l l n e s s e s a n d i mmu n e me d i a t e d s y n d r o me s . H o w e v e r, t h e d i f f e r e n t i a l d i a g n o s i s i n c l u d e s b a c t e r i a ,
s p i r o c h e t e s , r i c k e t t s i a , a n d r h e u ma t o l o g i c d i s e a s e s .
1. V i r a l e x a n t h e mTsh e s e v i r a l e t i o l o g i e s o f r a s h e s i n c l u d e r u b e o l a , r u b e l l a , F i f t h
d i s e a s e , a n d r o s e o l a a mo n g o t2)
h e. rTs h(e r a s h o f r u b e o l a ( me a s l e s ) b e g i n s
a r o u n d t h e f o u r t h f e b r i l e d a y, w i t h d i s c r e t e l e s i o n s t h a t b e c o me c o n f l u e n t a s
t h e y s p r e a d f r o m t h e h a i r l i n e d o w n w a r d , s p a r i n g t h e p a l ms a n d s o l e s . T h e
e xa n t h e m t y p i c a l l y l a s t s 4 t o 6 d a y s , w i t h l e s i o n s f a d i n g g r a d u a l l y i n t h e o r d e r o
a p p e a r a n c e , l e a v i n g a r e s i d u a l y e l l o w - t a n c o l o r a t i o n o r f a i n t d e s q u a ma t i o n ,
a c c o mp a n i e d b y t h e p r e s e n c e o f K o p l i k ' s s p o t s i n t h e o r a l mu c o s a . R u b e l l a
( G e r ma n me a s l e s ) i s s i mi l a r t o r u b e o l a b u t i s l e s s s e v e r e a n d o f s h o r t e r
d u r a t i o n . H o w e v e r, r u b e l l a c a n c a u s e s e v e r e b i r t h d e f e c t s i f a n e xp e c t a n t
mo t h e r b e c o me s i n f e c t e d . R o s e o l a h a s a p r o d r o me o f a s y mp t o ma t i c f e v e r
lasting 3 to 4 days. Within 2 to 3 days following defervescence, a diffuse rash,
s p a r i n g t h e f a c e a n d h a n d s , a p p e a r s w h i c h r e s o l v e s s p o n t a n e o u s l y. H u ma n
herpes virus 6 is the causative agent.
2. A l l e r g i c e r u p t i o A
n sd u l l r a s h t h a t i s o f t e n a c c o mp a n i e d b y c e n t r a l v e s i c l e s o r
b u l l a e ; t h i s a p p e a r s c o mmo n l y o n t h e h a n d s , i n c l u d i n g p a l ms , a s w e l l a s s o l e s ,
a r ms , k n e e s , a n d g e n i t a l s i n a s y mme t r i c d i s t r i b u t i o n . T h e r a s h , w h i c h i s
intensely pruritic, usually appears within the first week after the offending drug
is started and typically resolves within days after the drug is discontinued.
3. B a c t e r i a l i n f e c t i To hn i s d i a g n o s i s i s u s u a l l y s e r i o u s , w i t h me n i n g o c o c c u s a n d
g o n o c o c c u s b e i n g t h e mo s t c o mmo n i n t h e U n i t e d S t a t e s . C o n s i d e r t h i s
d i a g n o s i s i n a c u t e l y i l l p a t i e n t s w i t h h i g h f e v e r, t a c h y p n e a , t a c h y c a r d i a ,
h y p o t e n s i o n , l e u k o c y t o s i s , a n d me n i n g e a l s i g n s . S e c o n d a r y s y p h i l i s c a n b e
d i f f u s e , w i t h l o c a l i ze d e r u p t i o n s o c c u r r i n g o n t h e h e a d , n e c k , p a l ms , a n d s o l e s .
T h e l e s i o n s a r e t y p i c a l l y b r o w n i s h - r e d o r p i n k ma c u l e s a n d p a p u l e s , b u t t h e y
ma y b e p a p u l o s q u a mo u s , p u s t u l a r, o r a c n e i f o r m. T h e e r u p t i o n u s u a l l y o c c u r s 2
t o 6 mo n t h s a f t e r t h e p r i ma r y i n f e c t i o n a n d 2 t o 1 0 w e e k s a f t e r t h e p r i ma r y
c h a n c r e 3)( . A d d i t i o n a l s i g n s a n d s y mp t o ms i n c l u d e f e v e r, l y mp h a d e n o p a t h y, a n d
s p l e n o me g a l y. R e c u r r e n t e p i s o d e s w i t h s y mp t o m- f r e e p e r i o d s i n b e t w e e n ma y
o c c u r.
4. R o c k y M o u n t a i n s p o t t e d f eTvheer r a s h b e g i n s a s p i n k i s h - r e d ma c u l o p a p u l e s
on the wrists and ankles but spreads toward the trunk and progresses to
p e t e c h i a e . I n v o l v e me n t o f t h e p a l ms a n d s o l e s o c c u r s l a t e i n d i s e a s e . T h e o n s e
i s t y p i c a l l y a b r u p t w i t h f e v e r, s e v e r e h e a d a c h e , my a l g i a s , b r a d y c a r d i a , a n d
l e u k o p e n i a . T h e r a s h a p p e a r s a r o u n d t h e f o u r t h d a y Roi fc ki let
l ntessi a
s.
r i c k et t s i iis t h e c a u s a t i v e a g e n t .
5. K a w a s a k i ' s d i s e a K
s ea w a s a k i ' s d i s e a s e ma n i f e s t s a s a ma c u l o p a p u l a r t o
s c a r l a t i n i f o r m r a s h w i t h mu c o u s me mb r a n e i n v o l v e me n t . I t i s a c c o mp a n i e d b y
h i g h f e v e r l a s t i n g 5 d a y s o r mo r e , c o n j u n c t i v i t i s , l y mp h a d e n o p a t h y,
d e s q u a ma t i o n o f t h e h a n d s , a n d / o r s t r a w b e r r y t o n g u e . I t u s u a l l y o c c u r s i n
younger children. T he etiology is unknown.
500 / 652
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T hese vary according to the underlying disease process.
P. 3 0 5
References
1 . B o l o g n i a J , B r a v e r ma n I . S k i n ma n i f e s t a t i o n s o f i n t e r n a l d i s e a s e . I n :
B r a u n w a l d E , eHd ar
. r i s on' s pr i nc i pl es of i nt er nal medi
. 1 5ctihnee d . N e w Yo r k ,
N Y: M c G raw- H ill, 2001.
2 . M c K i n n o n H , H o w a r d T . E v a l u a t i n g t h e f e b r i l e p a t i e n t wAim
t h Faam
rash.
P hy s i c i an2 0 0 0 ; 6 2 ( 4 ) : 8 0 4 8 1 6 .
3 . P i c k e r i n g L , e d . A A P - C o mmi t t e e - o n - I n f e c t i o u s -RDed
i s ebook
a s e . : r epor t of
t he c ommi t t ee on i nf ec t i ous di. s2eas
5 t he e d . E l k G r o v e , I L : A me r i c a n A c a d e my
of Pediatrics, 2000.
501 / 652
tmdmss
13.4 - Pigmentation Disorders
> T a b l e o f C o n t e n t s > 1 3 - D e r ma t o l o g i c P r o b l e ms > 1 3 . 4 - P i g me n t a t i o n D i s o r d e r s
13.4
Pigmentation Disorders
Kare n Hughe s
I . Background
P a t i e n t s o f t e n p r e s e n t c o mp l a i n i n g o f p a t c h e s o f s k i n t h a t a r e l i g h t e r o r d a r k e r.
A. Etiology
D i s o r d e r s o f h y p e r p i g me n t a t i o n i n c l u d e p i t y r i a s i s cv af
e r s i au
c o l ol ai
r,ma
t cules,
me l a s maac
, ant hos i s ni gr i c,ans
solar lentigines, drug eruptions,
p h y t o p h o t o d e r ma t i t i s , a n d p o s t i n f l a mma t o r y h y p e r p i g me n t a t i o n . D i s o r d e r s o f
h y p o p i g me n t a t i o n i n c l u d e v i t i l i g o , p i t y r i a s i s a l b a , a s h l e a f ma c u l e s , h a l o n e v u s ,
i d i o p a t h i c g u t t a t e h y p o me l a n o s i s , a n d p o s t i n f l a mma t o r y h y p o p i g me n t a t i o n .
B. Epidemiology
P r e v a l e n c e a s w e l l a s mo r b i d i t y a n d mo r t a l i t y r a t e s v a r y w i t h t h e s p e c i f i c d i s e a s e .
P i g me n t a t i o n d i s o r d e r s a f f e c t a l l r a c e s , a l l a g e s , a n d b o t h g e n d e r s .
I I I . Evaluation
A. History
T h e f i r s t s t e p i n d i a g n o s i n g a p i g me n t a t i o n d i s o r d e r i s t o c l a s s i f y t h e c o mp l a i n t a s
h y p e r p i g me n t a t i o n o r h y p o p i g me n t1)a .t iToanb (l e 1 3 . 4 .p1r o v i d e s o t h e r n e c e s s a r y
h i s t o r y, i n c l u d i n g o n s e t , e xa c e r b a t i n g f a c t o r s , r e l i e v i n g f a c t o r s , a n d a s s o c i a t e d
s y mp t o ms .
1. H y p e r p i g m e n t e d d i s o r d ec af
r s. au l aima
t c u l e s a r e 0 . 2 t o 1 0 c m, u n i f o r m,
w e l l - d e ma r c a t e d b r o w n a r e a s f o u n d o n s u n - p r o t e c t e d s i t e s o f t h e t r u n k a n d
e xt r e mi t i e s . M e l a s ma a p p e a r s o n t h e ma l a r e mi n e n c e a n d o t h e r s u n - e xp o s e d
a r e a s .A c ant hos i s ni gr i c ans
appears as dirty or unwashed skin and as
t h i c k e n e d p l a q u e s i n a s y mme t r i c p a t t e r n i n t h e a xi l l a e , n e c k , a n d f o l d s o f t h e
b r e a s t a n d g r o i n . P i t y r i a s i s v e r s i c o l o r b e g i n s a s a r e d d i s h ma c u l e , g e n e r a l l y o n
the upper back, appearing darker than the surrounding skin during winter and
l i g h t e r t h a n t a n n e d s k i n d u r i n g s u mme r. M i n o c y c l i n e - i n d u c e d h y p e r p i g me n t a t i o n
o c c u r s i n o l d s c a r s , o n t h e l o w e r e xt r e mi t i e s a n d f o r e a r ms , o r d i f f u s e l y i n s u n e xp o s e d a r e a s . F i xe d - d r u g e r u p t i o n s o c c u r i n t h e s a me p l a c e w i t h e a c h
e xp o s u r e : t h e y a r e o f t e n v e s i c o b u l l o u s , r e s o l v i n g a s a h y p e r p i g me n t e d p a t c h
(1) . P h y t o p h o t o d e r ma t i t i s i n i t i a l l y r e s e mb l e s s u n b u r n , f o l l o w e d b y p r o l o n g e d
h y p e r p i g me n t a t i o n . P o s t i n f l a mma t o r y h y p e r p i g me n t a t i o n i s g e n e r a l l y l i g h t b r o w n
to black discoloration.
2. H y p o p i g m e n t e d d i s o r d eVir st i.l i g o a p p e a r s a s d e p i g me n t e d ma c u l e s w i t h
s c a l l o p e d e d g e s , i n i t i a l l y o n t h e f a c e , h a n d s , w r i s t s , a xi l l a e , u mb i l i c u s , a n d
g e n i t a l i a . T h e ma c u l e s t h e n c o a l e s c e w i t h t i me i n t o l a r g e r d e p i g me n t e d a r e a s .
502 / 652
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P i t y r i a s i s a l b a a p p e a r s a s s ma l l ma c u l e s t h a t d o n o t t a n , a r e p a l e p i n k t o l i g h t
brown with irregular borders and a powdery scale located on the face, and are
s e e n mo r e o f t e n i n d a r k l y p i g me n t e d s k i n t y p e s . A s h l e a f ma c u l e s a r e
h y p o p i g me n t e d l e s i o n s a p p e a r i n g w i t h o n e e n d r o u n d e d a n d t h e o t h e r p o i n t e d
( l a n c e o v a t e ) r e s e mb l i n g a n a s h t r e e l e a f ; i t s s i ze a n d s h a p e r e ma i n s t a b l e w i t h
a g e 1( ) . T h e y c a n o c c u r i n n o r ma l c h i l d r e n b u t i f a c c o mp a n i e d b y a c n e l i k e
lesions, they are suspects for
P. 3 0 6
P. 3 0 7
t u b e r o u s s c l e r o s i s . A h a l o n e v u s i s a n a r e a o f d e p i g me n t a t i o n s u r r o u n d i n g a
t y p i c a l p i g me n t e d n e v u s . G u t t a t e h y p o me l a n o s i s i s s e e n a s s ma l l 5 - mm o r l e s s
p o r c e l a i n - w h i t e ma c u 2)
l e .s (
TAB L E 13.4.1 Factors Used to Differentiate between

Hyperpigm entation and Hypopigm entation
Onse t
Ex ace rbating
factors
Re lie v ing
factors
Associate d
sy m ptom s
Hy popigm e nte d disorde rs
C af au l ai t s pot s
Birth or early
childhood
None
M e l a s ma
Onset of liver
dysfunction,
p r e g n a n c y,
phenytoin use,
oral
contraceptive
use
Wo r s e n i n g l i v e r M a y i mp r o v e
disease or
w i t h r e mo v a l
ongoing
of offending
e xp o s u r e
agents, but
rarely
disappears
entirely
A c ant hos i s ni gr i c ansI n c r e a s e d

weight, insulin
use
Regress over
t i me
None
Usually
none
We i g h t g a i n o r I mp r o v e s w i t h D i a b e t e s the use of
weight loss
related
insulin,
a n d r e mo v a l o f s y mp t o ms
nicotinic acid, offending
glucocorticoids,agents
or estrogens
Halo nevus
Severe sun
Ongoing sun
e xp o s u r e i n a
e xp o s u r e
youth, especially
in T urner's
s y n d r o me
Tends to
Usually
disappear with none
t i me
Solar lentigines
Older age with

earlier age sun
e xp o s u r e
Ongoing sun
e xp o s u r e
None
Usually
none
Ongoing
e xp o s u r e t o
h u mi d i t y a n d
heat
L ower
h u mi d i t y,
t r e a t me n t
Occasionally
mi l d p r u r i t u s
P i t y r i a s i s v e r s i c o l o r E xp o s u r e t o
h u mi d i t y a n d
heat
503 / 652
tmdmss

Drug-induced
h y p e r p i g me n t a t i o n
D r u g e xp o s u r e ,
especially to
mi n o c y c l i n e o r
zi d o v u d i n e
R e e xp o s u r e t o O c c a s i o n a l l y
causative
fades with
agent
r e mo v a l o f
offending
agents.
Minocyclineinduced
changes are
often
p e r ma n e n t
Usually
none
F i xe d - d r u g e r u p t i o n D r u g e xp o s u r e , R e e xp o s u r e t o
( p h e n o l p h t h a l e i n ,c a u s a t i v e
salicylates,
agent
tetracyclines,
and
s u l f o n a mi d e s )
M a y f a d e w i t h S o me t i me s
r e mo v a l o f
painful
offending
agents, but
o f t e n r e ma i n s
P h y t o p h o t o d e r ma t i t i sE xp o s u r e t o
Ongoing
topical agents
e xp o s u r e t o
containing
topical agents
f u r o c o u ma r i n s
containing
( o i l o f b e r g a mo t , f u r o c o u ma r i n s
p s o r a l e n s , l i me s ) ( o i l o f
b e r g a mo t ,
psoralens,
l i me s )
T o p i c a l o r o r a l S o me t i me s
steroids,
painful
a n t i h i s t a mi n e s .
I n f l a mma t i o n
With
Relief of
Pityriasis alba
Yo u n g c h i l d r e n D r y i n g a g e n t s , M a y f a d e w i t h O c c a s i o n a l l y
(especially those sunlight
mo i s t u r i ze r s , i t c h y o r
w i t h e c ze ma )
tends to
burning
disappear at
puberty
A s h l e a f ma c u l e
Childhood
None
Vi t i l i g o
1030 years of
age
Stress, illness, Progressive illness

personal
crises, skin
t r a u ma
Guttate
h y p o me l a n o s i s
Middle age and

older
None
(idiopathic
Ongoing
None
None
(idiopathic
Pain from
If underlying
tuberous
sclerosis:
me n t a l
retardation,
s e i zu r e s ,
and
a d e n o ma
sebaceum
None
504 / 652
tmdmss

lesions)
lesions)
3. O p h t h a l mo l o g i c e xa mi n a t i o n i s i mp o r t a n t f o r p a t i e n t s w i t h v i t i l i g o , b e c a u s e
p i g me n t a t i o n a b n o r ma l i t i e s o f t h e c h o r o i d a n d r e t i n a c a n l e a d t o p o o r v i s u a l
a c u i t y o r b l i n d n e s s . L u n g e xa mi n a t i o n o f c h i l d r e n w i t h p i t y r i a s i s a l b a i s u s e f u l ,
b e c a u s e a t o p y a n d a s t h ma o f t e n c o e xi s t .
4. N e u r o l o g i c e xa mi n a t i o n i s n e c e s s a r y i n i n d i v i d u a l s a u l a i t ma
c afcu lau
es with
l ai t ma c u l e s o r a s h l e a f s p o t s . A c o mp l e t e p h y s i c a l e xa mi n a t i o n , s e a r c h i n g f o r
u n d e r l y i n g ma l i g n a n c y, i s n e c e s s a r y i n p a t i e n t s w i t h r a pac
i dant
o nhos
s e t i so f
ni gr i c ans
, especially if it occurs without weight loss or in the absence of
diabetes.
C. Testing
1. H y p e r p i g m e n t e d d i s o r dSe kr si n s c r a p i n g o f p i t y r i a s i s v e r s i c o l o r ma y r e v e a l
t h e c h a r a c t e r i s t i c s p a g h e t t i a n d me a t b a l l s a pof
p eM
a ral
a nas
c es ezi a f ur;f ur
Wo o d ' s l i g h t i l l u mi n a t i o n o f t e n r e v e a l s a y e l l o w - g o l d l u mi
1) n. eMs oc reen c e (
t h a n s i xc af au l aima
t c u l e s l a r g e r t h a n 0 . 5 c m i n l e n g t h s h o u l d p r o mp t a n
e v a l u a t i o n f o r n e u r o f i b r o ma t1)o .s iEs v(a l u a t e t h e p a t i e n t w i t h u n e xp l a i n e d
ac ant hos i s ni gr i c,ans
d i a b e t e s , f o r u n d e r l y i n g t u mo r, e s p e c i a l l y g a s t r o i n t e s t i n a l
a d e n o c a r c i n o ma s .
2. H y p o p i g m e n t e d d i s o r d Vi
e rtsi l i g o c a n b e a s s o c i a t e d w i t h h y p e r t h y r o i d i s m o r
h y p o t h y r o i d i s m, d i a b e t e s , p e r n i c i o u s a n e mi a , a n d o t h e r e n d o c r i 2)
n e. d i s o r d e r s (
H a v e p a t i e n t s w i t h a s h l e a f ma c u l e s s c r e e n e d f o r t u b e r o u s s c l e r o s i s .
D. Genetics
E xa mp l e s o f t h e ma n y k n o w n g e n e t i c d e f e c t s c a u s i n g h y p o me l a n o s i s i n c l u d e
p i e b a l d i s m, v i t i l i g o , t u b e r o u s s c l e r o s i s c o mp l e x, p i g me n t a r y mo s a i c i s m
( h y p o me l a n o s i s o f I t o ) . S o me d e f e c t s r e s u l t i n g i n h y p e r me l a ncoafs
i s au
include
l ai t ma c u l e s a n d n e u r o f i b r o ma t o s i s . P i g me n t a r y d e f e c t s a s s o c i a t e d w i t h l e n t i g i n e s
i n c l u d e P e u t z- T o u r a i n e - J e g h e r s s y n d r o me a n d 3)
f r .e c k l e s (
I V. Diagnosis
P a t i e n t s w i t h p i g me n t a r y d i s o r d e r s a r e i n i t i a l l y b e s t c l a s s i f i e d i n t o d i s o r d e r s o f
e xc e s s i v e p i g me n t a t i o n o r h y p o p i g me n t a t i o n . H i s t o r y i s u s e f u l
P. 3 0 8
i n e l i c i t i n g i n c i t i n g f a c t o r s , e s p e c i a l l y d r u g - o r i n f l a mma t i o n - i n d u c e d c h a n g e s .
Characteristic appearances of the lesions further define the illness. Biopsy of
l e s i o n s i s g e n e r a l l y n o t n e c e s s a r y, a l t h o u g h s e l e c t e d l e s i o n s ma y r e q u i r e f u r t h e r
testing for underlying diseases.
T hese vary according to the illness.
References
1 . K i m N , P a n d y a A . P i g me n t a r y d i s eMaed
s e sC. l i n N or t h A1m9 9 8 ; 8 2 ( 5 ) : 11 8 5
1207.
2 . H a c k e r S . C o mmo n d i s o r d e r s o f p i g me n t a t i o n : w h e n a r e mo r e t h a n c o s me t i c
c o v e r - u p s r e q u i r ePdos
? t gr ad M ed
1996;99(6):177186.
505 / 652
tmdmss
3 . P a s s e r o n T , M a n t o u x F, O r t o n n e H - P. G e n e t i c d i s o r d e r s o f p iCglme
i n ntation.
D er mat ol2 0 0 5 ; 2 3 ( 1 ) : 5 6 6 7 .
506 / 652
tmdmss
13.5 - Pruritus
> T a b l e o f C o n t e n t s > 1 3 - D e r ma t o l o g i c P r o b l e ms > 1 3 . 5 - P r u r i t u s
13.5
Pruritus
Jam e s R. Lundy
I . Background
Pruritus, a sense of the need to scratch, is an unpleasant cutaneous sensation that

h a s n u me r o u s c a u s e s . T h e i t c h c a n b e e i t h e r l o c a l o r g e n e r a l i ze d a n d mi l d o r
s u f f i c i e n t l y a n n o y i n g t o i mp a i r s l e e p .
A. Etiology
Pruritus can be classified into one of four categories on the basis of the origin of th
i t c h : p r u r i t o c e p t i v e , n e u r o p a t h i c , n e u r o g e n i c , o r p s 1)
y c.h o g e n i c (
1. P r u r i t o c e p t i v e i t c h o r i g i n a t e s i n t h e s k i n a n d i s c a u s e d b y i n f l a mma t i o n , d r y n e s s
o r o t h e r s k i n d a ma g e . E xa mp l e s i n c l u d e a q u a g e n i c p r u r i t u s , b u l l o u s p e mp h i g o i d
c o n t a c t d e r ma t i t i s , d e r ma t i t i s h e r p e t i f o r mi s , f i b e r g l a s s d e r ma t i t i s , i n s e c t b i t e s ,
mi l i a r i a ( p r i c k l y h e a t ) , p e d i c u l o s i s ( l i c e ) , s c a b i e s , u r t i c a r i a ( h i v e s ) , xe r o s i s ( d r y
s k i n ) , d r u g r e a c t i o n s , f o l l i c u l i t i s , f u n g a l i n f e c t i o n s , l i c h e n p l a n u s , l i c h e n s i mp l e x
c h r o n i c u s , my c o s i s f u n g o i d e s , p e mp h i g u s f o l i a c e u s , p i t y r i a s i s r o s e a , p r u r i t i c
u r t i c a r i a l p a p u l e s , a n d p l a q u e s o f p r e g n a n c y, p s o r i a s i s , a n d s u n b u r n .
2. N e u r o p a t h i c i t c h o r i g i n a t e s i n t h e a f f e r e n t p a t h w a y t h r o u g h a s p e c i f i c d i s e a s e
me c h a n i s m. E xa mp l e s i n c l u d e p o s t h e r p e t i c n e u r a l g i a , mu l t i p l e s c l e r o s i s , a n d
s o me b r a i n t u mo r s .
3. N e u r o g e n i c i t c h o r i g i n a t e s c e n t r a l l y w i t h o u t a n y i n d i c a t i o n o f a s p e c i f i c n e u r a l
l e s i o n . E xa mp l e s i n c l u d e b i l i a r y d i s e a s e c a u s e d b y d r u g s , p r e g n a n c y, o r
c i r r h o s i s ; c h r o n i c r e n a l f a i l u r e ; h y p e r t h y r o i d d i s e a s e ; l y mp h o r e t i c u l a r d i s o r d e r s
( H o d g k i n ' s a n d n o n - H o d g k i n ' s l y mp h o ma ) ; a n d v i s c e r a l ma
2) .l i g n a n c y (
4. P s y c h o g e n i c i t c h h a s n o o r i g i n . T h i s c a n r a n g e f r o m mi l d c a s e s l i k e t h e
itchiness we all seem to have while discussing a patient with scabies to part of
p s y c h i a t r i c d i s o r d e r w h e r e t h e p a t i e n t ' s a r ms a r e s c a r r e d b y c o n s t a n t c l a w i n g a t
t h e s k i n3)( .
B. Epidemiology
P r u r i t u s i s a r a t h e r c o mmo n c o mp l a i n t , a n d i t s e e ms u n l i k e l y t h a t a n y i n d i v i d u a l w i t
507 / 652
tmdmss
13.5 - Pruritus
intact cognition will not have at least one brief episode of a sensation of needing to
s c r a t c h a n i t c h d u r i n g a n y g i v e n y e a r.
I I I . Evaluation
A. History
1. H i s t o r y o f t h e p r e s e n t i l l nDeest es r mi n e t h e l o c a t i o n a n d d u r a t i o n o f t h e
p r u r i t u s . F o r e xa mp l e , s c a b i e s i n v o l v e s t h e i n t e r d i g i t a l w e b s , v o l a r w r i s t s , a n d
g e n i t a l i a , w h e r e a s a t o p i c d e r ma t i t i s o c c u r s i n t h e a n t e c u b i t a l o r p o p l i t e a l f o s s a e
Pityriasis rosea typically has a herald patch on the trunk. Fungal infections
t e n d t o o c c u r i n w a r m, d a r k , mo i s t b o d y s u r f a c e s ( e . g . , g e n i t a l i a , f e e t , a n d
inguinal folds).
P. 3 0 9
2. E x a c e r b a t i n g a n d a l l e v i a t i n g f aScyt omp
r st o ms d u r i n g o r i mme d i a t e l y
following bathing are distinctive of aquatic pruritus.
3. T i m e o f y e aOr n s e t o r w o r s e n i n g o f t h e i t c h i n g i n w i n t e r w o u l d s u g g e s t xe r o s i s .
A r e t h e r e o t h e r i n d i v i d u a l s o r p e t s w i t h s i mi l a r s y mp t o ms ?
4. O t h e r h i s t o rAyr e t h e r e c h r o n i c me d i c a l p r o b l e ms s u c h a s d i a b e t e s me l l i t u s ,
chronic renal failure, or hepatic disorders? Is the patient taking current
me d i c a t i o n s a s w e l l a s o v e r - t h e - c o u n t e r d r u g s a n d h e r b a l s ?
5. S o c i a l h i s t o rW
y hat are the patient's occupation, travel, and bathing habits?
H a s t h e r e b e e n e xp o s u r e t o c h e mi c a l s , n e w s o a p s , o r d e t e r g e n t s t h a t c a n c a u s e
a l l e r g i c o r i r r i t a n t d e r ma t i t i s ?
6. F a m i l y h i s t o rIys t h e r e a f a mi l y h i s t o r y o f a t o p y ( a l l e r g i c r h i n i t i s , a s t h ma ,
a n d / o r a t o p i c d e r ma t i t i s ) o r c a n c e r ?
7. M e n s t r u a l h i s t o R
r ye me mb e r t o a s k a b o u t p o t e n t i a l p r e g n a n c y.
1. H e a d , e y e s , e a r s , n o s e , a n d t h r o a t ( H E E N T ) e x a m
L oi onka tfioorns c l e r a l
i c t e r u s a s a s i g n o f h e p a t i c d i s e a s e . E v a l u a t e mu c o u s me mb r a n e s f o r s i g n s o f
allergies.
2. L u n g e x a m i n a t i oLni s t e n f o r s t r i d o r i n a c u t e p r e s e n t a t i o n s , w h i c h i s a n
o mi n o u s s i g n o f a n a p h y l a xi s .
3. G a s t r o i n t e s t i n a l e x a m i n aPt iaol np a t e f o r o r g a n o me g a l y a n d p a r t i c u l a r l y
h e p a t o me g a l y.
4. L y m p h n o d e C
s h e c k f o r l y mp h a d e n o p a t h y o f t h e c e r v i c a l , a xi l l a r y, a n d i n g u i n a l
r e g i o n s . T h e d i f f e r e n t i a l d i a g n o s i s o f l y mp h a d e n o p a t h y i n c l u d e s my c o s i s
f u n g o i d e s , l y mp h o ma , a n d c h r o n i c i r r i t a t i o n .
5. M e n t a l s t a t u s / p s y c h i a t r i c e x a m i nI sa t ti hoenr e a n y i n d i c a t i o n o f a n xi e t y, d r u g
withdrawal, or psychosis?
6. S k i n e x a m i n a t i oPne r f o r m a t h o r o u g h a s s e s s me n t o f t h e u n d r e s s e d p a t i e n t
508 / 652
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13.5 - Pruritus
with good lighting. Notice skin areas not easily observed or reached by the
p a t i e n t . P a y a t t e n t i o n t o n e w, u n s c r a t c h e d l e s i o n s , b e c a u s e c h r o n i c a l l y
e xc o r i a t e d s k i n o w i n g t o a n y c a u s e h a s c o mp a r a b l e s e c o n d a r y c h a n g e s . A r e
t h e r e d e r ma t o g r a p h i s m a n d w h e a l s t h a t c h a r a c t e r i s t i c a l l y i n d i c a t e u r t i c a r i a
( h i v e s ) ? F l a t - t o p p e d p o l y g o n a l p a p u l e s w i t h s u b t l e w h i t e l i n e s ( W i c k h a m' s
s t r i a e ) a r e d i s t i n c t i v e o f l i c h e n p l a n u s . S i l v e r p l a q u e s o n a n e r y t h e ma t o u s b a s e
with a positive Auspitz sign (punctate bleeding of the scale after blunt scraping)
are typical of psoriasis. T he application of tangential pressure on superficial,
c r u s t i n g l e s i o n s r e s u l t i n g i n d i s l o d g i n g t h e e p i d e r mi s ( N i k o l s k y ' s s i g n ) i n d i c a t e s
p e mp h i g u s f o l i a c e u s . P u s t u l a r l e s i o n s a b o v e h a i r f o l l i c l e s a r e a s i g n o f
folliculitis.
C. Testing
I f t h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n d o n o t r e v e a l t h e d i a g n o s i s , i t ma y b e
s e n s i b l e t o t r y a n e mp i r i c c o u r s e o f t h e r a p y b e f o r e p r o c e e d i n g w i t h t e s t s .
1. S k i n s c r a p i n gU.s i n g a s c a l p e l , c e l l s a r e s c r a p e d a w a y f r o m a r e c e n t l e s i o n
o n t o a s l i d e . T h e s l i d e c a n t h e n b e t r e a t e d w i t h p o t a s s i u m h y d r o xi d e f o r c e l l u l a r
d i s r u p t i o n s o t h a t h y p h a e a n d b u d d i n g y e a s t s ma y e me r g e i n c a s e s o f f u n g a l
infection.
2. S k i n p u n c h b i o p s i e s o f t e n y i e l d u s e f u l i n f o r ma t i o n .
3. B l o o d s t u d i e sI f. a s y s t e mi c d i s o r d e r i s s u s p e c t e d , i n c l u d e t h e f o l l o w i n g i n t h e
e v a l u a t i o n : a c o mp l e t e b l o o d c o u n t w i t h d i f f e r e n t i a l ; c o mp l e t e me t a b o l i c p a n e l ;
t h y r o i d - s t i mu l a t i n g h o r mo n e ; h u ma n i mmu n o d e f i c i e n c y s c r e e n ; a n d s e r o l o g i c t e s
for syphilis.
4. O t h e r s t u d i e s m a y b e w a r r a nI ft et hd e. h i s t o r y a n d p h y s i c a l e xa mi n a t i o n i mp l y
o t h e r s y s t e mi c d i a g n o s e s , a d d i t i o n a l r e c o mme n d e d t e s t s t o c o n s i d e r i n c l u d e
c h e s t x- r a y, f e c a l o c c u l t b l o o d t e s t , u r i n a l y s i s , s e r u m i r o n s t u d i e s , s t o o l f o r o v a
and parasites, serum glucose, and serum electrophoresis. Additional diagnostic
t e s t s f o r s y s t e mi c d i s o r d e r s c a n b e c o n s i d e r e d t o r u l e o u t t h e mo r e o b s c u r e
d i a g n o s e s l i s t e d i n t h e p r e c e d i n g t e xt .
D. Genetics
A f a mi l y h i s t o r y o f a t o p y i s u s e f u l f o r d i a g n o s i s , e s p e c i a l l y i n c h i l d r e n . T h e s p e c t r u
of diseases that can cause pruritus is broad, and genetic testing should be pursued
only once an illness with a strong genetic basis is strongly suspected.
P. 3 1 0
I V. Diagnosis
T h e p o t e n t i a l c a u s e s o f p r u r i t u s a r e n u me r o u s . T h e l i s t i n g i n c l u d e d i n t h e s e c t i o n o
e t i o l o g y r e p r e s e n t s t h e mo r e f r e q u e n t a n d c o mmo n c a u s e s . U n f o r t u n a t e l y, s o me t i me
n o c a u s e c a n b e f o u n d . O f t e n , a t r i a l o f t r e a t me n t i s n e e d e d o n t h e b a s i s o f t h e mo s
l i k e l y c a u s e a n d d i a g n o s i s i s h o n e d o n t h e r e s p o n s e t o t r e a t me n t .
509 / 652
tmdmss
13.5 - Pruritus
In addition to itching, secondary changes of the skin are often induced by the
p a t i e n t ' s s c r a t c h i n g . S c a r r i n g i s c o mmo n i n s e v e r e c a s e s .
References
1 . Vo n d e r P o o l V. P r u r i t u s . I n : T a y l o r TRhe
, e10d . mi nut e di agnos i s manual
.
P h i l a d e l p h i a , PA : L i p p i n c o t t W i l l i a ms & W i l k i n s , 2 0 0 0 .
2 . C h a r l e s w o r t h E , B e l t r a n i V. P r u r i t i c d e r ma t o s e s : o v e r v i e w o f e t i o l o g y a n d
t h e r a p y.A m J M ed2 0 0 2 ; 11 3 ( 9 A ) : 2 5 s 3 3 s .
3 . Yo s i p o v i t c h G , G r e a v e s M , S c h me l z ML .anc
I t cet
h 2. 0 0 3 ; 3 6 1 : 6 9 0 6 9 4 .
510 / 652
tmdmss
13.6 - Rash Accompanied by Fever
> T a b l e o f C o n t e n t s > 1 3 - D e r ma t o l o g i c P r o b l e ms > 1 3 . 6 - R a s h A c c o mp a n i e d b y
Fever
13.6
Rash Accompanied by Fever
Michae l L. O'De ll
I . Background
F e v e r w i t h a n a c c o mp a n y i n g r a s h r e p r e s e n t s a d i a g n o s t i c c h a l l e n g e f o r e v e n t h e
mo s t e xp e r i e n c e d c l i n i c i a n b e c a u s e t h i s c o mb i n a t i o n o f s i g n s ma y r e p r e s e n t e i t h e r a
trivial or a life-threatening illness.
A u s e f u l w a y o f a p p r o a c h i n g t h e d i f f e r e n t i a l d i a g n o s i s i s t o d i s t i n g u i s h a mo n g t h e
v a r i o u s e n t i t i e s t h a t c a u s e f e v e r a n d i l l n e s s b y t h e t y p e s o f r a s h t h e y c o mmo n l y
c a u s e . A l t h o u g h v a r i o u s f e b r i l e d i s e a s e s ma y p r e s e n t b y mo r e t h a n o n e t y p e o f r a s h
this grouping allows the clinician to look at fewer causes rather than the entire
s p e c t r u m o f p o s s i b l e c a u1)
s e. s (
A. P e t e c h i a l r a s h e s a r e c o mmo n l y a s s o c i a t e d w i t h t h e f o l l o w i n g :
1. T r e a t a b l e i n f e c t i o n s , i n c l u d i n g e n d o c a r d i t i s , me n i n g o c o c c e mi a ,
g o n o c o c c e mi a , s e p t i c e mi a f r o m a n y b a c t e r i a , r i c k e t t s i o s i s ( e s p e c i a l l y R o c k y
M o u n t a i n s p o t t e d f e v e r [ R M2)
SF])(
2. I n f e c t i o u s c a u s e s n o t s u b j e c t t o a c u t e t r e a t me n t , i n c l u d i n g e n t e r o v i r u s ,
d e n g u e f e v e r, h e p a t i t i s B v i r u s , r u b e l l a , a n d E p s t e i n - B a r r v i r u s ( E B V )
3. N o n i n f e c t i o u s c a u s e s , i n c l u d i n g u r t i c a r i a , t h r o mb o c y t o p e n i a , s c u r v y,
H e n o c h - S c h n l e i n d i s e a s e , h y p e r s e n s i t i v i t y v a s c u l i t i s , a c u t e r h e u ma t i c
f e v e r, a n d s y s t e mi c l u p u s e r y t h e ma t o s u s ( S L E )
B. M a c u l o p a p u l a r r a s h e s a r e c o mmo n l y a s s o c i a t e d w i t h t h e f o l l o w i n g :
1. T r e a t a b l e i n f e c t i o n s , i n c l u d i n g t y p h o i d , s e c o n d a r y s y p h i l i s ,
me n i n g o c o c c e mi a , g o n o c o c c e mi a , my c o p l a s ma , L y me d i s e a s e , p s i t t a c o s i s ,
rickettsiosis (especially R M S F)
p a r v o v i r u s B - 1 9 , h u ma n h e r p e s v i r u s 6 , r u b e o l a , r u b e l l a , a d e n o v i r u s , E B V,
p r i ma r y h u ma n i mmu n o d e f i c i e n c y v i r u s ( H I V ) 1
3. N o n i n f e c t i o u s c a u s e s , i n c l u d i n g a l l e r g y, e r y t h e ma mu l t i f o r me , S L E ,
d e r ma t o my o s i t i s , s e r u m s i c k n e s s , a n d j u v e n i l e r h e u ma t o i d a r t h r i t i s
511 / 652
tmdmss
C. Ve s i c o b u l l o u s r a s h e s a r e c o mmo n l y a s s o c i a t e d w i t h t h e f o l l o w i n g :
1. T r e a t a b l e i n f e c t i o n s , i n c l u d i n g s t a p h y l o c o c c a l l a r g e v e s i c l e i mp e t i g o a n d
t o xi c s h o c k s y n d r o me , g o n o c o c c e mi a , r i c k e t t s i a l p o x, v a r i c e l l a - zo s t e r, h e r p e s
s i mp l e x v i r u Vi
s , br i o v ul ni f i c sus
epsis, and folliculitis
P. 3 11
p a r v o v i r u s B - 1 9 , a n d H I V ( a l t h o u g h n o n e o f t h e s e t h r e e c o mmo n l y p r e s e n t
i n t h i s ma n n e r )
3. N o n i n f e c t i o u s c a u s e s , i n c l u d i n g e c ze ma v a c c i n a t u m a n d e r y t h e ma
mu l t i f o r me b u l l o s u m
D. D i f f u s e e r y t h e ma t o u s r a s h e s a r e c o mmo n l y a s s o c i a t e d w i t h t h e f o l l o w i n g :
1. T r e a t a b l e i n f e c t i o n s , i n c l u d i n g s t r e p t o c o c c a l s c a r l e t f e v e r, t o xi c s h o c k
s y n d r o me , e h r l i c h i o 3)
s i s, S( t r ept oc oc c us v i r i dans
( c h e mo t h e r a p y p a t i e n t s ) ,
C or y nebac t er i um haemol y t ipchum
aryngitis, and K awasaki's disease
2. I n f e c t i o u s c a u s e s n o t s u b j e c t t o a c u t e t r e a t me n t , i n c l u d i n g e n t e r o v i r a l
infections
3. N o n i n f e c t i o u s c a u s e s o f e r y t h e ma a r e o n l y r a r e l y a s s o c i a t e d w i t h f e v e r
E. U r t i c a r i a r a s h e s a r e c o mmo n l y a s s o c i a t e d w i t h t h e f o l l o w i n g :
1. T r e a t a b l e i n f e c t i o n s , i n c l u d i n g my c o p l a s ma a n d L y me d i s e a s e
2. I n f e c t i o u s c a u s e s n o t s u b j e c t t o a c u t e t r e a t me n t , i n c l u d i n g e n t e r o v i r a l
i n f e c t i o n s , a d e n o v i r a l i n f e c t i o n s , E B V, H I V, a n d h e p a t i t i s
3. N o n i n f e c t i o u s c a u s e s o f u r t i c a r i a o n l y r a r e l y a s s o c i a t e d w i t h f e v e r
I I I . Evaluation
A. History
H i s t o r y i s q u i t e i mp o r t a n t a n d s h o u l d i n c l u d e s t a n d a r d i t e ms , s u c h a s o n s e t ,
d u r a t i o n , a g g r a v a t i n g f a c t o r s , r e l i e v i n g f a c t o r s , a n d a s s o c i a t e d s y mp t o ms .
A d d i t i o n a l l y, o t h e r f a c t o r s s h o u l d b e c o n s i d e r e d , s u c h a s t h e f o l l o w i n g :
1. E x p o s u r e h i s t o rAyr.e a n y o t h e r f a mi l y me mb e r s o r c l o s e c o n t a c t s i l l ? I s t h e r e
a h i s t o r y o f e xp o s u r e t o b r a c k i s h w a t e r, mo s q u i t o e s , o r f o r e i g n t r a v e l ?
2. A r e t h e r e a n y u n d e r l y i n g i l l n e s s e s o r i s t h e r e a s i g n i f i c a n t c h a n c e o f
i mmu n o l o g i c c o mp r o mi s e , s u c h a s u n d i a g n o s e d H I V i n f e c t i o n ?
T h e l e s i o n s a n d t h e i r d i s t r i b u t i o n s h o u l d b e c a r e f u l l y e xa mi n e d . T h e r a s h s h o u l d b e
c l a s s i f i e d a s p e t e c h i a l , ma c u l o p a p u l a r, v e s i c o b u l l o u s , e r y t h e ma t o u s , o r u r t i c a r i a l .
T he distribution of the rash should be noted. For instance, rubella and rubeola
generally begin on the face and spread to the trunk, whereas R M S F petechiae tend
to occur on the ankles and wrists first.
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1. A g e n e r a l p h y s i c a l e xa mi n a t i o n s h o u l d b e c o n d u c t e d . A r e a s o f p a r t i c u l a r
concern are as follows:
a. H e a d , e y e s , e a r s , n o s e , a n d t h r o a t ( H E E N T ) e x a m
K oi np lai tki'os ns p o t s
a r e p a t h o g n o mo n i c f o r r u b e o l a . T h e d i s c o v e r y o f a t i c k l e n d s s u p p o r t t o t h e
d i a g n o s i s o f R M S F. S i n u s i t i s ma y r e p r e s e n t a s o u r c e f o r me n i n g o c o c c e mi a .
P h a r y n g i t i s i n a y o u n g a d u l t w i t h d i f f u s e e r y t h e ma ma yC .b e d u e t o
haemol y t i c um
. T h e p r e s e n c e o f mu c o u s me mb r a n e s w e l l i n g ma y i n d i c a t e
e a r l y a n a p h y l a xi s .
b. L u n g e x a m i n a t i oWnh e e zi n g o n e xa mi n a t i o n , e s p e c i a l l y i n a p a t i e n t w h o
h a s r e c e n t l y r e c e i v e d me d i c a t i o n s o r c o n t r a s t d y e , c a n i n d i c a t e a n a p h y l a xi s .
E v i d e n c e o f p n e u mo n i a i s c o n s i s t e n t w i t h p s i t t a c o s i s a n d my c o p l a s ma .
c. C a r d i a c e x a m i n a t i C
o na r d i o v a s c u l a r c o l l a p s e i s a s s o c i a t e d w i t h
me n i n g o c o c c e mi a a n d o t h e r s e p s i s . A n e w mu r mu r ma y i n d i c a t e s u b a c u t e
bacterial endocarditis in a patient with subungual or scleral petechiae.
d. G e n i t a l e x a m i n a t i P
o un r u l e n t u r e t h r a l d r a i n a g e o r e v i d e n c e o f p e l v i c
i n f l a mma t o r y d i s e a s e s u p p o r t s t h e c o n s i d e r a t i o n o f g o n o r r h e a . A c h a n c r e
s u p p o r t s a d i a g n o s i s o f s y p h i l i s , a l t h o u g h p a l ma r l e s i o n s o f t e n o c c u r w e l l
after the healing of the initial chancre.
e. J o i n t e x a m i n a t i o n a n d e x t r e mAi t pi eest e c h i a l r a s h n e a r t h e a n k l e s a n d
w r i s t i s s u g g e s t i v e o f R M S F. E v i d e n c e o f j o i n t s w e l l i n g s u p p o r t s a
d i a g n o s i s o f me n i n g o c o c c e mi a o r g o n o c o c c e mi a . A ma c u l o p a p u l a r r a s h ma y
b e s e e n i n j u v e n i l e r h e u ma t o i d a r t h r i t i s a n d o t h e r r h e u ma t o l o g i c a l
conditions as well.
f. N e u r o l o g i c e x a m i n a t iEovni d e n c e o f me n i n g i t i s s u p p o r t s a d i a g n o s i s o f
me n i n g o c o c c e mi a . P a t i e n t s w i t h R M S F ma y a l s o h a v e me n i n g e a l s i g n s .
C. Testing
Testing should be directed by the suspected illnesses, with life-threatening illnesse

b e i n g t e s t e d f o r u p o n r e a s o n a b l e s u s p i c i o n . A c o mp l e t e b l o o d c o u n t i s g e n e r a l l y
useful, although life-threatening sepsis often presents without significant elevation
the white blood cell count. In general, a blood culture should be obtained in all
patients with petechial rashes and in those with signs of cardiovascular collapse.
P. 3 1 2
I V. Diagnosis
O n t h e b a s i s o f t h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n , t h e l i k e l i h o o d o f v a r i o u s
i l l n e s s e s c a n b e a s s e s s e d . P a t i e n t s w h o a p p e a r t o xi c s h o u l d b e t r e a t e d a s s e p t i c
u n t i l i n i t i a l l a b o r a t o r y t e s t s a n d c u l t u r e r e s u l t s c a n b e4)e. v a l u a t e d (
References
1 . S c h l o s s b e r g D . F e v e r a n d rI anfsec
h . t D i s C l i n N or t h 1A9m9 6 ; 1 0 ( 1 ) : 1 0 1 11 0 .
2. D rolet B A, B aselga E , E sterly N B . P ainful, purpuric plaques in a child with
513 / 652
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f e v e r.A r c h D er mat ol
1997;133(12):15001501.
3 . A m J M ed
. F e v e r, n a u s e a , a n d r a s h i n a 3 7 - y e a r - o l d ma n [ c l i n i c a l c o n f e r e n c e ] .
A m J M ed1 9 9 8 ; 1 0 4 ( 6 ) : 5 9 6 6 0 1 .
4 . D e l l i n g e r R P. C u r r e n t t h e r a p y f o r sI nf
e pec
s i ts .D i s C l i n N or t h A m
1999;13(2):495509.
514 / 652
tmdmss
13.7 - Urticaria
> T a b l e o f C o n t e n t s > 1 3 - D e r ma t o l o g i c P r o b l e ms > 1 3 . 7 - U r t i c a r i a
13.7
Urticaria
R a y T. P e r r i n e
I . Background
U r t i c a r i a i s d e f i n e d b y t h e a p p e a r a n c e o f w h e a l , a l e s i o n t h a t i s g e n e r a l l y c i r c u l a r,
ma d e u p o f r e d d i s h l e s i o n s , u s u a l l y s u r r o u n d e d b y r e d n e s s ( f l a r e ) , s p o n g y t o t h e
t o u c h , a n d o f t e n c h a n g e s i n a p p e a r a n c e r a p i d l y. L e s i o n s a r e o f t e n c o mp l e t e l y g o n e
w i t h i n mi n u t e s t o h o u r s , b u t o t h e r l e s i o n s a p p e a r i n o t h e r l o c a t i o n s o v e r t h a t s a me
t i me f r a me . T h e s e a r e g e n e r a l l y i n t e n s e l y p r u r i t i c .
A. Etiology
U r t i c a r i a c a n b e c l a s s i f i e d a s i mmu n o l o g i c , n o n i mmu n o l o g i c , o r i d i o p a t h i c .
I mmunol ogi c ur t i c ari ni cal u d e s i mmu n o g l o b u l i n E ( I g E ) - a n d c o mp l e me n t - me d i a t e d
h y p e r s e n s i t i v i t y a n d p h y s i c a l a n d c o n t a c t uNroni
t i c ammunol
ria.
ogi c ur t i c ar i a
i n c l u d e s ma s t c e l l r e l e a s e , p h y s i c a l a g e n t s , a n d c o n t a c t u r t i c a r i a . A ma j o r a d v a n c e
t h e u n d e r s t a n d i n g o f c h r o n i c u r t i c a r i a h a s b e e n t h e d e mo n s t r a t i o n o f c i r c u l a t i n g I g G
autoantibodies directed against the -subunit of the high-affinity Ig E receptor or an
I g E a u t o a n t i b o d i e s i n a s u b s e t o f p a t i e n t s . A p p r o xi ma t e l y 4 0 % t o 5 0 % o f p a t i e n t s
w i t h c h r o n i c u r t i c a r i a a r e n o w c o n s i d e r e d t o h a v e c h r o n i c a u t o i mmu n e u r t i c a r i a , w i t
t h e r e ma i n i n g h a v i n g i d i o p a t h i c c h r o n i c u1)r t.i c a r i a (
B. Epidemiology
U r t i c a r i a i s a c o mmo n i l l n e s s , a n d 1 5 % t o 2 0 % o f t h e p o p u l a t i o n i s e s t i ma t e d t o h a v
o n e e p i s o d e o f h i v e s d u r i n g t h e i r l i f e t i me . U r t i c a r i a o c c u r s mo s t f r e q u e n t l y b e t w e e n
2 0 a n d 4 0 y e a r s o f a g e , a n d i t i s mo r e c o mmo n i n w o me n t h a n me n . T h e r e a r e t w o
p r i ma r y f o r ms o f u r t i c a r i a : a c u t e a n d c h r o n i c . A c u t e u r t i c a r i a l e s i o n s l a s t < 6 w e e k s .
Chronic urticaria lasts >6 weeks. Only 50% of the cases of chronic urticaria are
l i k e l y t o r e mi t w i t h i n 1 y e a r, a n d u p t o 4 0 % o f t h e c a s e s t h a t l a s t > 6 mo n t h s a r e l i k e
t o p e r s i s t f o r 1 0 y e a r s o r mo r e .
I I I . Evaluation
A. History
1. F o o d o r d r u g e xp o s u r e s a r e c o mmo n c a u s e s o f u r t i c a r i a . C e r t a i n s y s t e mi c
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13.7 - Urticaria
d i s e a s e s c a n c a u s e u r t i c a r i a , i n c l u d i n g b u t n o t l i mi t e d t o c o n n e c t i v e t i s s u e
diseases, endocrine disorders, and neoplastic diseases. Insect stings and bites
a r e a n o t h e r c o mmo n c a u s e o f u r t i c a r i a . D o e s a n y t h i n g s p e c i f i c t r i g g e r t h e r a s h ?
I s i t l o c a l i ze d o r s y s t e mi c ? D o e s i t r e s p o n d t o a n y t r e a t me n t ?
2. S y mp t o m c h r o n o l o g y i s a l s o i mp o r t a n t . W h e n d o e s u r t i c a r i a o c c u r ? H o w l o n g
d o e s i t l a s t ? I s i t i n a s s o c i a t i o n w i t h p h y s i c a l t r a u ma ? H a s t h e p a t i e n t b e e n o n
a n y me d i c a t i o n t h a t h a s h e l p e d r e l i e v e s y mp t o ms ( e . g . , a n t i h i s t a mi n e s ) ?
A c o mp l e t e p h y s i c a l e xa mi n a t i o n i s r e q u i r e d t o r u l e o u t i n f e c t i o n o r o t h e r s y s t e mi c
diseases. T he cardinal features of urticaria that distinguish
P. 3 1 3
i t f r o m a n y o t h e r t y p e o f i n f l a mma t o r y e r u p t i o n a r e t h e r e p e a t e d o c c u r r e n c e o f s h o r t
l i v e d c u t a n e o u s w h e a l s a n d t h e a c c o mp a n y i n g r e d n e s s a n d i t c h i n g . W h e a l s a r e
l e s i o n s r a n g i n g f r o m a f e w mi l l i me t e r s t o s e v e r a l c e n t i me t e r s i n d i a me t e r, a l t h o u g h
t h e y r u n t o g e t h e r a n d b e c o me c o n f l u e n t , mu c h l a r g e r p l a q u e s ma y o c c u r. I f
a n g i o e d e ma i s p r e s e n t , i t c a n l a s t f o r s e v e r a l d a y s . T h e s k i n r e t u r n s t o n o r ma l o n c e
the wheal has disappeared.
C. Testing
1. B l o o d s t u d i e s a r e g e n e r a l l y n o t h e l p f u l i n c o n f i r mi n g t h e d i a g n o s i s . S t u d i e s
s h o u l d b e p e r f o r me d w h e n t h e r e i s c o n c e r n a b o u t t h e d i a g n o s i s o r a t y p i c a l
f e a t u r e s . M e a s u r e me n t o f c o mp l e t e b l o o d c o u n t , e r y t h r o c y t e s e d i me n t a t i o n r a t e ,
s e r u m c h e mi s t r y v a l u e s , c o mp l e me n t C 3 a n d C 4 , a n d t h y r o t r o p i n l e v e l s h o u l d b e
c o n s i d e r e d t o e xc l u d e s y s t e mi c d i s e a s e . I n s o me c a s e s , t e s t s f o r a n t i n u c l e a r
a n t i b o d i e s a n d h e p a t i t i s a r e a l s o n e c 2)
e s. s a r y (
2. S p e c i f i c a l l e r g y o r p r o v o c a t i v e t e s t s ( e . g . , a d d i t i v e c h a l l e n g e , e xe r c i s e ,
p r e s s u r e , c o l d ) ma y b e r e q u i r e d t o f u r t h e r c l a r i f y t h e d i a g n o s i s . S k i n t e s t s f o r
food and latex sensitivities are often indicated, especially in chronic urticaria.
3. P u n c h b i o p s y o f a n u r t i c a r i a l l e s i o n o f t e n p r o v i d e s u s e f u l i n f o r ma t i o n .
4. D i a g n o s t i c i ma g i n g s t u d i e s , s u c h a s c h e s t x- r a y, c o mp u t e d t o mo g r a p h y, s i n u s ,
a n d d e n t a l f i l ms ma y h e l p r u l e o u t u n d e r l y i n g c a n c e r a n d i n f e c t i o n .
D. Genetics
I t i s u s e f u l t o a s k w h e t h e r t h e r e a r e a n y me mb e r s o f t h e f a mi l y w h o s u f f e r f r o m a
c o n n e c t i v e t i s s u e d i s o r d e r ? T h e r e i s a ma r k e d i n c r e a s e i n t h e i n c i d e n c e o f c h r o n i c
u r t i c a r i a i n f i r s t - d e g r e e r e l a3)
t i .v eDso ( a n y c o mp l e me n t d i s o r d e r s o c c u r i n t h e
f a mi l y, s u c h a s h e r e d i t a r y a n g i o e d e ma ? I s t h e r e a f a mi l y h i s t o r y o f a t o p y ?
I V. Diagnosis
T he lesions of urticaria are generally distinctive. T he differential diagnosis usually

f o c u s e s o n u n d e r l y i n g c a u s e s . T h e s e i n c l u d e c a n c e r, e n d o c r i n e d i s o r d e r s ,
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13.7 - Urticaria
c o n n e c t i v e t i s s u e d i s e a s e s , a n d i n f e c t i o n s . T h e mo s t s i g n i f i c a n t f a c t o r s i n
d i a g n o s i n g a c u t e u r t i c a r i a a r e t h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n . F a c t s mu s t b e
obtained regarding food or drug ingestion, insect stings, current infections, or
p h y s i c a l t r i g g e r s s u c h a s c o l d , h e a t , o r e xe r c i s e . A t p r e s e n t , d e mo n s t r a t i o n o f
circulating Ig G autoantibodies is directed against the -subunit of the high-affinity
IgE receptor or anti-IgE autoantibodies in a subset of patients; the testing lacks
a p p l i c a b i l i t y c l i n i c a l l y. A p p r o xi ma t e l y 3 0 % t o 4 0 % o f p a t i e n t s w i t h c h r o n i c u r t i c a r i a
h a v e t h e a n t i - F c e R I a a u t o a n t i b o d i e s a n d 1 0 % h a v e a n t i - I g E a u t4)
o a. n t i b o d i e s (
Although the lesions of urticaria are distinctive, the various underlying conditions
t h a t t r i g g e r t h e s k i n ' s r e s p o n s e a r e n u me r o u s a n d v a r i e d i n t h e i r c l i n i c a l
ma n i f e s t a t i o n s . Vi r t u a l l y a l l p a t i e n t s w i t h u r t i c a r i a c o mp l a i n o f p r u r i t u s i n a d d i t i o n t
the rash.
References
1 . K a p l a n A P. C h r o n i c u r t i c a r i a : p a t h o g e n e s i s a n d tJr eAaltlme
er gy
nt.Cl i n
I mmunol2 0 0 4 ; 11 4 ( 3 ) : 4 6 5 4 7 4 .
2 . K a p l a n A P. C h r o n i c u r t i c a r i a a n d a n g i oNe dEengl
ma . J M ed2 0 0 2 ; 3 4 6 ( 3 ) : 1 7 5
179.
3 . R i b o l d i P, A s e r o R , T e d e s c h i A , e t a l . C h r o n i c u r t i c a r i a : n e w i mmu n o l o g i c
a s p e c t s I. s r M ed A s s oc 2J0 0 2 ; 4 ( 11 s u p p l ) : 8 7 2 8 7 3 .
4 . G r e a v e s M . C h r o n i c u r t i c Ja rAi al l. er gy C l i n I mmunol
2000;105(4):664672.
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13.8 - Vesicular and Bullous Eruptions
> T a b l e o f C o n t e n t s > 1 3 - D e r ma t o l o g i c P r o b l e ms > 1 3 . 8 - Ve s i c u l a r a n d B u l l o u s
Eruptions
13.8
Vesicular and Bullous Eruptions
Michae l L. O'De ll
I . Background
Ve s i c u l a r a n d b u l l o u s e r u p t i o n s ( V B L s ) a r e b l i s t e r i n g i l l n e s s e s .
A. Etiology
V B L s o c c u r a s a r e s u l t o f b a c t e r i a , v i r u s e s , s y s t e mi c i l l n e s s e s ( e . g . , a l l e r g i c
v a s c u l i t i s ) , o r i n j u r y ( e s p e c i a l l y s u n o r h e a t e xp o s u r e ) . S p e c i a l p r e c i p i t a t o r s c a n l e
t o V B L . S u n l i g h t c a n p r e c i p i t a t e r e c u r r e n t h e r p e s s i mp l e x ( H S ) ( a s c a n w i n d ,
me n s e s , d r y s k i n , s mo k i n g , d r i n k i n g a l c o h o l , l a c k o f s l e e p , a n d f e v e r ) . M a n y d r u g s
a l c o h o l c a n t r i g g e r p o r p h y r i a c u t a n e a t a r d1)
a .( PDCr uTg) (i n g e s t i o n c a n a l s o
p r e c i p i t a t e a l l e r g i c v a s c u l i t i s , S t e v e n s - J o h n s o n s y n d r o me ( S J S ) / t o xi c e p i d e r ma l
n e c r o l y s i s ( T E N ) , a n d P C T . C o n t a c t w i t h s k i n p r e c i p i t a t e s d e r ma
R hus
t i t ,i s d u e t o
n i c k e l , a n d p e r f u me .
B. Epidemiology
1. I n p a t i e n t s o f c e r t a i n a g e s , p a r t i c u l a r d i a g n o s e s s h o u l d b e c o n s i d e r e d :
a. N e w b o r n sE p i d e r mo l y s i s b u l l o s a , p e mp h i g u s n e o n a t o r u m, a n d s y p h i l i t i c
p e mp h i g u s
b. C h i l d r e nVa r i c e l l a ( i f u n i mmu n i ze d ) , p r i ma r y H S , h a n d , f o o t , a n d mo u t h
( H F M ) d i s e a s e , a n d b u l l o u s i mp e t i g o ( B I )
c. A d u l t sR e c u r r e n t H S , P C T , p e mp h i g u s v u l g a r i s ( P V ) , d y s h i d r o t i c e c ze ma
( D E ) , d e r ma t i t i s h e r p e t i f o r mi s ( D H ) , l i n e a r i mmu n o g l o b u l i n A ( L I g A ) d i s e a s e
d. E l d e r l yB u l l o u s p e mp h i g o i d ( B P ) a n d h e r p e s zo s t e r ( H Z )
2. I n p a t i e n t s o f a n y a g e , a l l e r g i c c o n t a c t d e r ma t i t i s , a l l e r g i c v a s c u l i t i s , S J S / T E N ,
insect bites, and second-degree burns should be considered.
3. T h e r e i s s e a s o n a l v a r i a t i o n a s w e l l .
a. F a l l a n d w i n t eVa
r r i c e l l a , H F M , a n d p r i ma r y H S a r e o f t e n s e e n i n
e p i d e mi c s a f t e r g a t h e r i n g s o f c h i l d r e n , s u c h a s r e t u r n t o s c h o o l .
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b. S p r i n g a n d s u m m B
e rI ( d u e t o s t a p h y l o c o c c a l i n f e c t i o n ) a n d D E
( i n c r e a s e d s w e a t i n g o f h a n d s a n d f e e t ) . C o n t a c t d e r maRt ihus
tis due to
species parallels yard work.
I I I . Evaluation
A. History
1. P a i n o r p r u r i t uI tsc h i n g i s c o mmo n , e s p e c i a l l y i n c o n t a c t d e r ma t i t i s . P a i n i s
c o mmo n b e f o r e , d u r i n g , a n d a f t e r t h e e r u p t i o n o f H Z .
2. O n s e t a n d d u r a t i o
Mna n y V B L s o c c u r a c u t e l y w i t h o u t p r e c e d i n g e p i s o d e s :
S J S / T E N , v a r i c e l l a , B I , H Z , a l l e r g i c v a s c u l i t i s , H F M , a n d P V. S o me d i s e a s e s
a r e c h r o n i c w i t h e xa c e r b a t i o n s , s u c h a s D H , D E , B P, e p i d e r mo l y s i s b u l l o s a , a n d
P C T . A c u t e c o n t a c t d e r ma t i t i s a n d H S o f t e n r e c u r i n e p i s o d e s . D H i s mo r e
c o mmo n i n p a t i e n t s w i t h g l u t e n - s e n s i t i v e e n t e2)r o. p a t h y (
1. A p p e a r a n c eI n p a t i e n t s w h o l o o k s i c k , t o xi c , o r i l l , c o n s i d e r p o s s i b l e d i a g n o s e s
o f S J S / T E N , P V, o r p r i ma r y H S ( p a r t i c u l a r l y i n t o d d l e r s ) .
2. P r e s e n c e o f f e v P
e ra t i e n t s w i t h v a r i c e l l a a n d H F M ma y h a v e l o w - g r a d e f e v e r.
P a t i e n t s w i t h S J S / T E N o r p r i ma r y H S ma y h a v e p r e c e d i n g a n d / o r c o n c u r r e n t
f e v e r.
3. P r e s e n c e o f o r a l l e s i oOnr sa l l e s i o n s t e n d t o s i g n i f y mo r e s e r i o u s
c o n s e q u e n c e s a n d mo r e s i g n i f i c a n t i l l n e s s , s u c h a s S J S / T E N , P V, T E N ,
v a r i c e l l a , a n d H F M . T h e s k i n l e s i o n s o f P V ma y a p p e a r mo n t h s a f t e r t h e o n s e t
o f o r a l l e s i o n2)
s .(
4. C h a r a c t e r i s t i c s o f t h e l e s Ve
i o ns si c l e s a r e < 1 c m; b u l l a e a r e > 1 c m. T h e f l u i d
i n t h e l e s i o n ma y b e c l e a r, p u r u l e n t , o r h e mo r r h a g i c . R u p t u r e o f V B L s ma y l e a d
t o e r o s i o n s , u l c e r s , a n d / o r c r u s t s . Ve s i c u l a r d i s e a s e s ma y r e p r e s e n t H S ,
P. 3 1 5
v a r i c e l l a , H Z , c o n t a c t d e r ma t i t i s , D E , h e mo r r h a g i c v a s c u l i t i s , H F M , K a p o s i ' s
v a r o c e l l i f o r m e r u p t i o n ( K V E ) , a n d D H . B u l l o u s d i s e a s e s a r e g e n e r a l l y P V, B P,
B I , P C T , S J S / T E N , T E N , a n d e p i d e r mo l y s i s b u l l o s a . T h e r e i s c o n s i d e r a b l e
o v e r l a p i n l e s i o n s i ze i n s o me d i s e a s e s , b u t t h e f i n d i n g o f l a r g e r b u l l a e t e n d s t o
b e c o n f i n e d t o t h e g r o u p l i s t e d a s b u l l o u s d i s e a s e s . S o me l e s i o n s a r e
distinctive. T he bullae of B I are thin, fragile, short lived, and easily ruptured,
leaving a thin, varnishlike often honey-colored crust with occasionally a delicate
r e mn a n t o f t h e b l i s t e r r o o f a t i t s r i m. C o n t a c t d e r ma t i t i s l e s i o n s a r e o f t e n
e xc o r i a t e d b y t h e t i me t h e p a t i e n t p r e s e n t s b e c a u s e o f i n t e n s e p r u r i t u s . Va r i c e l l
i s c h a r a c t e r i ze d b y l e s i o n s i n v a r i o u s s t a g e s t h e n e w e s t o n e s v e s i c u l a r ( d e w
d r o p o n a r o s e p e t a l ) , t h e o l d e r o n e s b e c o mi n g p u r u l e n t , t h e n c r u s t i n g o v e r.
T he bullae of B P are large and tense. Bullae of P V are flaccid and easily
ruptured, leaving large denuded, bleeding, and weeping erosions. T he lesions
o f P C T , D H , a n d a l l e r g i c v a s c u l i t i s ma y b e h e mo r r h a g i c a n d s e c o n d a r i l y
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c r u s t e d . U mb i l i c a t i o n i s g e n e r a l l y c h a r a c t e r i s t i c o f a v i r a l e t i o l o g y H S , H Z ,
varicella, and K VE. Chronic urticarial lesions progressing to bullae often signal
B P. I n T E N , l i g h t p r e s s u r e o n n o r ma l a p p e a r i n g s k i n ma y c a u s e i t t o w r i n k l e ,
s l i d e l a t e r a l l y, a n d s e p a r a t e f r o m t h e d e r mi s ( N i k o l s k y ' s s i g n ) .
5. L o c a t i o n a n d d i s t r i b u t i o n o f t h e l e s i o n s
a. L o c a l i z e dL. e s i o n s i n s u n - e xp o s e d a r e a s a r e o f t e n d u e t o c o n t a c t
d e r ma t i t i s , i n s e c t b i t e s , o r P C T . C o n t a c t d e r ma t i t i s i s l o c a l i ze d t o t h e a r e a
of contact. Allergic vasculitis lesions are dependent. H F M has hand, foot,
a n d mo u t h l e s i o n s . D H o c c u r s p r i ma r i l y o n t h e s h o u l d e r s , b u t t o c k s , e l b o w s ,
a n d p o s t e r i o r u p p e r b a c k . H Z f o l l o w s a s e n s o r y ( c u t a n e o u s ) d e r ma t o me
w h i c h d o e s n o t c r o s s t h e mi d l i n e . D E i n v o l v e s t h e l a t e r a l a s p e c t s o f t h e
f i n g e r s , p a l ms , a n d s o l e s . K V E o c c u r s a t s i t e s o f p r e e xi s t i n g d e r ma t i t i s ,
e s p e c i a l l y a r e a s o f a t o p i c d e r ma t i t i s . L I g A d i s e a s e c o mmo n l y o c c u r s i n t h e
g e n i t a l a r e a , w i t h u p t o 5 0 % o f c a s e s a l s o h a v i n g o r2)
a l. l e s i o n s (
b. G e n e r a l i z e d
( s o me d i s e a s e s b e g i n i n o n e a r e a a n d t h e n b e c o me
g e n e r a l i ze d ) . Va r i c e l l a b e g i n s o n t h e t r u n k o r h e a d , a n d s u c c e s s i v e c r o p s
e r u p t mo r e d i s t a l l y. S J S / T E N o f t e n b e g i n s i n t h e o r a l c a v i t y, g r o i n , o r a xi l l a
P V ma y a l s o b e g i n i n t h e o r a l c a v i t y. B P o c c u r s mo s t l y i n t h e f l e xo r
s u r f a c e s , a xi l l a , a n d g r o i n b u t c a n b e g e n e r a l i ze d .
C. Testing
1. T h e T z a n c k s m e ma
a r y b e u s e d t o d i a g n o s e v i r a l d e r ma t o s e s . T h e s me a r ma y
be obtained by the following procedure: unroof an early intact vesicle, one
w i t h o u t i n f e c t i o n o r t r a u ma . S c r a p e t h e b a s e o f t h e l e s i o n s l i g h t l y w i t h a s c a l p e
S me a r t h e ma t e r i a l o n t h e s c a l p e l b l a d e o n t o a c l e a n g l a s s s l i d e . A i r - d r y t h e
s me a r a n d f i x a n d f o l l o w b y s t a i n w i t h Wr i g h t o r G i e ms a . T h e t e s t i s p o s i t i v e i f
mu l t i n u c l e a t e d g i a n t c e l l s a r e n3)o.t e d (
2. B i o p s yo f t h e e d g e o f t h e b l i s t e r a n d s u b s e q u e n t i mmu n o f l u o r e s c e n t s t a i n i n g i s
h e l p f u l f o r d i a g n o s i n g P V, B P, a n d S J S
4)/ .T E N (
D. Genetics
S o me b u l l o u s o r v e s i c u l a r i l l n e s s e s h a v e a g e n e t i c b a s i s , e s p e c i a l l y P C T ( a u t o s o ma
d o mi n a n t i l l n e s s , g e n e r a l l y w i t h i n c o mp l e t e p e n e t r a n c e ) .
I V. Diagnosis
Diagnosis is reached considering the age of the patient, the location of the lesions,
w h e t h e r t h e l e s i o n s a r e b u l l o u s o r v e s i c u l a r, a n d w h e t h e r t h e p a t i e n t a p p e a r s t o xi c .
T hese vary widely according to the underlying disease or agent.
References
520 / 652
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References
1 . R o b s o n K J , P i e t t e W W. C u t a n e o u s ma n i f e s t a t i o n s o f s y s t e miM
c ed
diseases.
C l i n N or t h A m
1998;82(6):13591379, vivii.
2 . B i c k l e K M , R o a r k T R , H s u S H . A u t o i mmu n e b u l l o u s d e r ma t o s eAs m
: a r e v i e w.
2002;65(9):18611870.
3 . B r o d e l l R T , H e l ms S E , D e v i n e M . O f f i c e d e r ma t o l o g i c t e s t i n g : t h e T za n c k
p r e p a r a t i o nA. m F am P hy s i c i an
1991;44(3):857860.
4 . G e l l i s S E . B u l l o u s d i s e a s e s o f c h i lD
d heromat
o d . ol C l i1n9 8 6 ; 4 ( 1 ) : 8 9 9 8 .
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14.1 - Diabetes Mellitus
> T a b l e o f C o n t e n t s > 1 4 - E n d o c r i n e a n d M e t a b o l i c P r o b l e ms > 1 4 . 1 - D i a b e t e s
Mellitus
14.1
Diabetes M ellitus
J. Ste v e n Crame r
Andre a Many on
I . Background
D i a b e t e s me l l i t u s ( D M ) i s a g r o u p o f me t a b o l i c d i s e a s e s ( t y p e 1 , t y p e 2 , g e s t a t i o n a
a n d o t h e r s ) . I t i s c h a r a c t e r i ze d b y h y p e r g l y c e mi a r e s u l t i n g f r o m d e f e c t s i n i n s u l i n
s e c r e t i o n ( t y p e 1 ) , i n s u l i n a c t i o n ( t y p e 2 ) ,1)o. r b o t h (
A. Etiology
1. Ty p e 1 d i a b e t e s ( i m m u n e - m e d i a t e d i n s u l i n d e fTi cyipeen c1 yD) M i s
t h o u g h t t o b e c a u s e d b y a g r a d u a l a u t o i mmu n e d e s t r u c t i o n o f p a n c r e a t i c c e l l s
leading to deficiency in the production of insulin. Autoantibodies to islet cells,
i n s u l i n , g l u t a mi c a c i d d e c a r b o xy l a s e , a n d t y r o s i n e p h o s p h a t a s e s a r e s e e n i n
85% to 90% of these patients.
2. Ty p e 2 d i a b e t e s ( i n s u l i n r e s i s t T
a nh ce eh) a l l ma r k o f t y p e 2 D M i s i n s u l i n
r e s i s t a n c e w i t h a r e l a t i v e , r a t h e r t h a n a b s o l u t e , i n s u l i n d e f i c i e n c y. R i s k f a c t o r s
for type 2 D M include obesity (especially with a central distribution), lack of
p h y s i c a l a c t i v i t y, i n c r e a s i n g a g e , a f a mi l y h i s t o r y o f t y p e 2 D M , a n d p r i o r
gestational diabetes.
B. Epidemiology
A p p r o xi ma t e l y 1 8 . 2 mi l l i o n p e o p l e i n t h e U n i t e d S t a t e s h a v e D M a n d o f t h e s e , 5 . 2
mi l l i o n a r e u n d i a g n o s2)e .d T( h e l i f e t i me r i s k o f d e v e l o p i n g d i a b e t e s f o r t h o s e b o r n
i n 2 0 0 0 i s e s t i ma t e d t o b e 3 2 . 8 % f o r me n a n d 3 8 . 5 % f3)
o r. w
T yo pme
e n1 (D M
accounts for 5% to 10% of patients with diabetes and its prevalence in people
y o u n g e r t h a n 2 0 y e a r s o f a g e i s a p p r o xi ma t e l y 1 i n 4 0 0 . T y p e 1 D M h a s n o s e a s o n a
variation and gender differences are not clinically significant. Type 2 D M accounts
f o r 9 0 % t o 9 5 % o f a l l t h o s e w i t h d i a b e t e s . I t s p r e v a l e n c e v a r i e s a mo n g d i f f e r e n t
r a c i a l a n d e t h n i c g r o u p s ( A f r i c a n - A me r i c a n : 11 . 4 % , L a t i n o : 8 . 2 % , a n d N a t i v e
A me r i c a n : 1 4 . 9 %2)) . ( T h e i n c i d e n c e o f t y p e 2 D M i n c h i l d r e n a n d a d o l e s c e n t s
appears to be increasing. In one study of adolescents, the incidence rose from
522 / 652
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0 . 7 / 1 0 0 , 0 0 0 p e r y e a r i n 1 9 8 2 t o 7 . 2 / 1 0 0 , 0 0 0 p e r y e a4)r . i n 1 9 9 4 (
I I I . Evaluation
A. History
T h e i n i t i a l p r e s e n t a t i o n o f D M c a n v a r y. B o t h t y p e 1 a n d t y p e 2 c a n p r e s e n t w i t h t h
c l a s s i c s y mp t o ms o f p o l y u r i a , p o l y d i p s i a , a n d u n e xp l a i n e d w e i g h t l o s s .
1. Ty p e 1 d i a b e t ePsa t i e n t s w i t h t y p e 1 D M u s u a l l y p r e s e n t a s c h i l d r e n o r y o u n g
a d u l t s , b u t t h e d i s e a s e c a n o c c u r a t a n y a g e . T h e e me r g e n c e o f s y mp t o ms
varies, depending on the rate of cell destruction. Serum ketoacidosis is often
t h e f i r s t ma n i f e s t a t i o n o f t h e d i s e a s e i n y o u n g e r p a t i e n t s i n w h o m c e l l
d e s t r u c t i o n i s mo r e r a p i d a n d ma y p r e s e n t w i t h l i f e - t h r e a t e n i n g k e t o a c i d o s i s a s
t h e f i r s t ma n i f e s t a t i o n o f t h e d i s e a s e . M o d e s t e l e v a t i o n s i n f a s t i n g
h y p e r g l y c e mi a c a n r a p i d l y c h a n g e t o s e v e r e h y p e r g l y c e mi a o r k e t o a c i d o s i s i n
t h e f a c e o f i n f e c t i o n o r o t h e r s t r e s s . E n u r e s i s ma y b e a c l u e f o r p o l y u r i a i n a
c h i l d w h o w a s p r e v i o u s l y t o i l e t t r a i n e d . L e t h a r g y, w e a k n e s s , a n d w e i g h t l o s s a r e
o t h e r c o mmo n f e a t u r e s .
2. Ty p e 2 d i a b e t ePsa t i e n t s w i t h t y p e 2 D M t r a d i t i o n a l l y p r e s e n t a f t e r 4 0 y e a r s o f
a g e . T h e d i a g n o s i s i s o f t e n ma d e i n a n a s y mp t o ma t i c p a t i e n t a s a r e s u l t o f
r o u t i n e b l o o d t e s t s t h a t r e v e a l a n e l e v a t i o n o f p l a s ma g l u c o s e . O t h e r p a t i e n t s
ma y p r e s e n t w i t h s y mp t o ms o f h y p e r g l y c e mi a s u c h a s e xt r e me h u n g e r, f a t i g u e ,
a n d i r r i t a b i l i t y. T h e p a t i e n t ma y h a v e a h i s t o r y o f r e c u r r e n t s k i n i n f e c t i o n s o r
p e r s i s t e n t mo n i l i a l v u l v o v a g i n i t i s . M i n o r s k i n i n f e c t i o n s a r e o f t e n s l o w t o h e a l .
O t h e r c o mmo n s y mp t o ms i n c l u d e a l t e r e d s e n s a t i o n i n t h e e xt r e mi t i e s , n o c t u r i a ,
er ect i l e dysf unct i on, and vi sual di st urC
b ahnacpet es r (s 4,. 65 . 1, 1 0 . 3, a n d1 0 . 5) .
P. 3 2 0
T h e u s e o f g l u c o c o r t i c o i d s , - a d r e n e r g i c a g o n i s t s , o r t h i a zi d e d i u r e t i c s c a n
p r e c i p i t a t e s y mp t o ms a n d u n ma s k l a t e n t t y p e 2 D M .
P a t i e n t s o f t e n p r e s e n t w i t h s i mi l a r p h y s i c a l f i n d i n g s i n b o t h t y p e 1 a n d t y p e 2 D M ,
o w i n g t o h y p e r g l y c e mi a . I n t y p e 1 D M , t h e y o u n g c h i l d ma y f a i l t o g r o w o r g a i n
w e i g h t . O t h e r s ma y a p p e a r i l l a n d l e t h a r g i c w i t h s i g n s o f d e h y d r a t i o n ( t a c h y c a r d i a ,
h y p o t e n s i o n , a n d d r y mu c o u s me mb r a n e s w i t h r e d u c e d s k i n t u r g o r ) . I n k e t o s i s , a n
a c e t o n e o r f r u i t y o d o r ma y b e n o t e d o n t h e p a t i e n t ' s b r e a t h . T h e p a t i e n t w i t h t y p e 2
D M t e n d s t o b e o b e s e ( e s p e c i a l l y c e n t r a l o b e s i t y ) a n d ma y a p p e a r f a t i g u e d a n d
f l u s h e d w i t h mu s c l e w e a k n e s s o r b l u r r y v i s i o n . M o n i l i a l i n f e c t i o n s ma y b e f o u n d i n
t h e v a g i n a a n d i n t e r t r i g i n o u s r e g i o n s . T h e n e u r o l o g i c e xa mi n a t i o n i n p r e v i o u s l y
u n d i a g n o s e d a d v a n c e d c a s e s ma y r e v e a l e xt r e mi t i e s w i t h a l t e r e d s e n s a t i o n o r f r a n k
diabetic foot ulcers.
C. Testing
A c a s u a l p l a s ma b l o o d g l u c o s e l e v e l i s o b t a i n e d a t a n y t i me o f t h e d a y w i t h o u t
r e g a r d t o t h e t i me o f t h e l a s t me a l a n d a f a s t i n g l e v e l i s o b t a i n e d a f t e r a f a s t o f a
l e a s t 8 h o u r s . T h e A me r i c a n D i a b e t e s A s s o c i a t i o n ( A D A ) d i a g n o s t i c c r i t e r i a f o r
523 / 652
tmdmss
d i a b e t e s a r e e i t h e r s y mp t o ms o f d i a b e t e s a n d a c a s u a l p l a s ma g l u c o s e 2 0 0 mg / d L
( 11 . 1 mmo l / L ) o r a f a s t i n g p l a s ma g l u c o s e 1 2 6 mg / d L ( 7 . 0 mmo l / L ) , o r a p l a s ma
g l u c o s e 2 0 0 mg / d L ( 11 . 1 mmo l / L ) 2 h o u r s a f t e r a n o r a l g l u c o s e l o a d ( 7 5 g ) a s
d e s c r i b e d b y t h e Wo r l d H e a l t h O r g a n i5)
za. t Ii fo nt h(e o n l y c r i t e r i o n i s
h y p e r g l y c e mi a , c o n f i r ma t i o n s h o u l d b e ma d e b y r e p e a t t e s t i n g o n a d i f f e r e n t d a y.
T h e A D A a l s o r e c o mme n d s s c r e e n i n g i n d i v i d u a l s o l d e r t h a n 4 5 y e a r s o f a g e w i t h a
b o d y ma s s i n d e x 2 5 k2go/ m
r t h o s e w i t h r i s k f a c t o r s s u c h a s i n a c t i v i t y, a f a mi l y
h i s t o r y, a p e r s o n a l h i s t o r y o f g e s t a t i o n a l d i a b e t e s , o r h y1)p.e r t e n s i o n (
D. Genetics
T y p e 1 D M h a s s t r o n g h u ma n l e u k o c y t e a n t i g e n ( H L A ) a s s o c i a t i o n s a n d l i n k a g e s t o
t h e D Q A a n dD Q B g e n e s a n d i s i n f l u e n c e d bD
y Rt hBeg e n e s 1)( . T y p e 2 D M i s
a s s o c i a t e d w i t h a f a mi l y h i s t o r y o f t h e d i s e a s e a n d i s a s s o c i a t e d w i t h a h i g h r i s k f o
g e s t a t i o n a l d i a b e t e s i n w o me n . T h e s e p r e d i s p o s i t i o n s h a v e n o t y e t b e e n l i n k e d w i t h
any specific genetic pattern.
I V. Diagnosis
A. Clinical manifestations
T h e p r e s e n c e o f p o l y u r i a , p o l y d i p s i a , w e i g h t l o s s , a n d h y p e r g l y c e mi a w i t h k e t o s i s i
sufficient to establish the diagnosis of type 1 D M. If the diagnosis of type 1 D M is
n o t c l e a r, a l o w o r a b s e n t C - p e p t i d e l e v e l ma y h e l p c o n f i r m t h e d i a g n o s i s . T h e k e y
t h e d i a g n o s i s o f t y p e 2 D M i s t h e d e t e c t i o n o f h y p e r g l y c e mi a i n t h e f a c e o f t h e
classic risk factors.
B. Differential diagnosis
Assigning a type of diabetes to an individual begins with a clear understanding of
t h e e p i d e mi o l o g y a n d a n a w a r e n e s s o f t h e p a t i e n t ' s c i r c u ms t a n c e s . C l i n i c i a n s mu s t
first account for the age of the patient, body habitus, risks associated with past
me d i c a l h i s t o r y ( a s w i t h g e s t a t i o n a l d i a b e t e s ) , a n d f a mi l y h i s t o r y.
1. Ty p e 1 d i a b e t eNso t a l l c h i l d r e n w i t h h y p e r g l y c e mi a h a v e d i a b e t e s . S o me
c h i l d r e n w i t h a s e v e r e i l l n e s s ( e . g . , s e v e r e d e h y d r a t i o n f r o m d i a r r h e a o r a s t h ma
t r e a t e d w i t h c o r t i c o s t e r o i d s ) ma y h a v e e l e v a t e d s e r u m g l u c o s e a n d k e t o s i s . A n
e l e v a t e d H bI C
A c a n p r o v i d e a s t r o n g c i r c u ms t a n t i a l c a s e f o r t h e d i a g n o s i s o f
D M b u t i s n o t r e c o mme n d e d a s a s c r e e n i n g t e s t b e c a u s e o f t h e a s s o c i a t e d
costs.
2. Ty p e 2 d i a b e t eTsh e d i a g n o s i s c a n b e ma d e a c c o r d i n g t o t h e A D A c r i t e r i a .
P r e d i a b e t i c p a t i e n t s h a v e i mp a i r e d g l u c o s e t o l e r a n c e w i t h a f a s t i n g p l a s ma
g l u c o s e o f 1 0 0 t o 1 2 5 mg / d L ( 5 . 6 6 . 9 mmo
1) . Ll i/ fLe)s( t y l e mo d i f i c a t i o n f o r t h i s
g r o u p i s p a r a mo u n t a n d i n c l u d e s e xe r c i s e a n d a s u g g e s t e d w e i g h t l o s s o f 5 % t o
10%.
References
1 . A me r i c a n D i a b e t e s A s s o c i a t i o n P o s i t i o n S t a t e me n t . D i a g n o s i s a n d
524 / 652
tmdmss
c l a s s i f i c a t i o n o f d i a b e t e s meDl l i abet
t u s . es C ar2e0 0 5 ; 2 8 ( S u p p l 1 ) : S 3 7 S 4 2 .
2 . C e n t e r s f o r D i s e a s e C o n t r o l a n d P r e vNe at
n t i onal
o n . di abet es f ac t s heet :
gener al i nf or mat i on and nat i onal es t i mat es on di abet es i n t he ,U ni t ed S t at es
2 0 0 0 . C D C [ I n t e r n e t ] . Av a i l a b l e a t : w w w. c d c . g o v / d i a b e t e s / p u b s / n d f s . p d f ,
accessed on October 12, 2003.
P. 3 2 1
3 . Ve n k a t N a r a y a n K M . L i f e t i me r i s k f o r d i a b e t e s me l l i t u s i n t h e U n i t e d S t a t e s .
JAM A2003;290:18841890.
4 . P i n h a s - H a mi e l O , D o l a n L M , D a n i e l s S R , e t a l . I n c r e a s e d i n c i d e n c e o f n o n i n s u l i n - d e p e n d e n t d i a b e t e s me l l i t u s a mo n g a d oJl ePs cedi
e nat
t s r.1 9 9 6 ; 1 2 8 : 6 0 8
615.
5 . Wo r l d H e a l t h O r g a n i za tLi oabor
n . at or y di agnos i s and moni t or i ng of di
. abet es
Wo r l d H e a l t h O r g a n i za t i o n . Av a i l a b l e a t :
http://whqlibdoc.who.int/hq/2002/9241590483.pdf, accessed on J une 1, 2005
525 / 652
tmdmss
14.2 - Gynecomastia
> T a b l e o f C o n t e n t s > 1 4 - E n d o c r i n e a n d M e t a b o l i c P r o b l e ms > 1 4 . 2 G y n e c o ma s t i a
14.2
Gynecomastia
Charle s M. Kodne r
I . Background
G y n e c o ma s t i a i s t h e p a l p a b l e e n l a r g e me n t o f t h e b r e a s t t i s s u e i n me n . T h e r e a r e
t h r e e p e a k a g e - g r o u p s f o r p r e s e n t a t i o n , c o r r e s p o n d i n g t o t h e c o mmo n p h y s i o l o g i c
c a u s e s o f b r e a s t e n l a r g e me n t . A f t e r t h e n e o n a t a l p e r i o d , t h e mo s t c o mmo n c a u s e s o
g y n e c o ma s t i a a r e i d i o p a t h i c ( 2 5 % ) , p u b e r t a l f a c t o r s ( 2 5 % ) , me d i c a t i o n s ( 1 0 % 2 0 % )
c i r r h o s i s o r ma l n u t r i t i o n ( 8 % ) , o r p r i ma r y h y p o g o n a1)
d i. s m ( 8 % ) (
G y n e c o ma s t i a i s c a u s e d b y a r e l a t i v e i n c r e a s e o f e s t r o g e n o v e r a n d r o g e n , u s u a l l y
f r o m t h e a r o ma t i za t i o n o f t e s t o s t e r o n e a n d a n d r o s t e n e d i o n e t o e s t r a d i o l .
A. Etiology
M o s t c a s e s o f g y n e c o ma s t i a r e p r e s e n t a t r a n s i e n t , p h y s i o l o g i c i mb a l a n c e b e t w e e n
circulating estrogens and androgens.
1. N o r ma l p h y s i o l o g i c c o n d i t i o n s a r e a s s o c i a t e d w i t h t r a n s i e n t g y n e c o ma s t i a . I n
the neonatal period, transplacental estrogen causes transient breast tissue
e n l a r g e me n t i n mo s t n e w b o r n s ; t h i s ma y b e a s s o c i a t e d w i t h n i p p l e d i s c h a r g e
a n d t y p i c a l l y r e s o l v e s o v e r 3 t o 4 w e e k s . N o a d d i t i o n a l e v a l u a t i o n i s n e c e s s a r y.
D u r i n g p u b e r t y, h o r mo n a l c h a n g e s a n d b r e a s t t i s s u e p r o l i f e r a t i o n c a u s e
t r a n s i e n t g y n e c o ma s t i a i n a d o l e s c e n t b o y s ; t h i s ma y b e a s y mme t r i c a n d t e n d e r.
T h e c o n d i t i o n u s u a l l y r e s o l v e s w i t h i n 1 y e a r, b u t ma y p e r s i s t f o r u p t o 2 y e a r s .
O l d e r me n ( 5 0 8 0 y e a r s o f a g e ) ma y h a v e p a l p a b l e b r e a s t t i s s u e e n l a r g e me n t
that is often physiologic due to a relative increase in body fat and increased
a r o ma t i za t i o n o f e s t r o g e n p r e c u r s o r s , b u t me d i c a t i o n e f f e c t s o r me d i c a l
d i s o r d e r s s h o u l d b e c o n s i d e2)r .e d (
2. M e d i c a t i o n s ma y c a u s e g y n e c o ma s t i a . E xa mp l e s i n c l u d e a l c o h o l , ma r i j u a n a ,
e s t r o g e n s , d i g i t o xi n , c i me t i d i n e , s p i r o n o l a c t o n e , a n a b o l i c s t e r o i d s , f i n a s t e r i d e ,
k e t o c o n a zo l e , a n d a n t i a n d r o g1)e.n P
s r(o s t a t e c a n c e r ma y b e a s s o c i a t e d w i t h
g y n e c o ma s t i a , b e c a u s e t h e t r e a t me n t w i t h o r c h i e c t o my, e s t r o g e n s , o r
a n t i a n d r o g e n s ma y c a u s e g y n e c o ma s t i a a s a s i d e e f f e c t .
3. M e d i c a l d i s o r d e r s ( r e n a l f a i l u r e , l i v e r d i s e a s e , o r s t a r v a t i o n / ma l n u t r i t i o n ) ma y b
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14.2 - Gynecomastia
a s s o c i a t e d w i t h g y n e c o ma s t i a . S o me c a n c e r s ( l u n g , l i v e r, a n d k i d n e y ) ma y
p r o d u c e e c t o p i c h u ma n c h o r i o n i c g o n a d o t r o p i n ( H C G ) , w h i c h s t i mu l a t e s
a r o ma t a s e a c t i v i t y, c a u s i n g g y n e c o ma s t i a .
4. E n d o c r i n e d i s o r d e r s ( h y p e r t h y r o i d i s m a n d p r i ma r y t e s t i c u l a r f a i l u r e ) ma y a l s o b e
a s s o c i a t e d w i t h g y n e c o ma s t i a .
B. Epidemiology
T h e o v e r a l l p r e v a l e n c e o f p a l p a b l e g y n e c o ma s t i a i s a p p r o xi ma t e l y 3 6 % a n d ma y b e
u p t o 4 8 % i n b o y s 1 0 t o 1 5 y e a r s o f a g e o r 5 7 % i n me n o l d e r t h a n 4 4 y e a r s o f a g e
(3) . O b e s i t y i s a r i s k f a c t o r f o r g y n e c o ma s t i a , w i t h u p t o 8 0 % o f t h o s e w i t h a b o d y
ma s s i n d e x o v e r 2 5 k2ga/ m
ffected.
I I I . Evaluation
G y n e c o ma s t i a t h a t i s p e r s i s t e n t o r s y mp t o ma t i c o r p r e s e n t s o u t s i d e t h e e xp e c t e d a g
r a n g e s ma y r e p r e s e n t a p a t h o l o g i c p r o c e s s r e q u i r i n g d i a g n o s t i c e v a l u a t i o n .
A. History
T h e h i s t o r y s h o u l d b e d i r e c t e d t o w a r d i d e n t i f y i n g s y mp t o ms s u g g e s t i v e o f b r e a s t
cancer or an underlying endocrine disorder and any specific causative factors
P. 3 2 2
( a s d e s c r i b e d i n t h e p r e c e d i n g t e xt ) . P e r s i s t e n t o r r a p i d b r e a s t t i s s u e e n l a r g e me n t
ma y n e c e s s i t a t e f u r t h e r e v a l u a t i o n i f n o d i a g n o s i s i s a p p a r e n t . M i l d p a i n o r
tenderness by itself does not indicate a concerning underlying cause.
A f o c u s e d p h y s i c a l e xa mi n a t i o n s h o u l d e xc l u d e c a n c e r o f t h e b r e a s t o r t e s t e s , a s
w e l l a s s o me e n d o c r i n e d i s o r d e r s . T h e p h y s i c a l e xa mi n a t i o n s h o u l d a l s o d i f f e r e n t i a
t r u e g y n e c o ma s t i a f r o m p s e u d o g y n e c o ma s t i a . T h e b r e a s t i s g r a s p e d b e t w e e n t h u mb
a n d f o r e f i n g e r a n d t h e d i g i t s a r e mo v e d t o w a r d t h e n i p p l e ; a f i r m, r u b b e r y, mo b i l e ,
d i s k - s h a p e d ma s s o f t i s s u e b e n e a t h t h e n i p p l e i n d i c a t e s t r u e b r e a s t t i s s u e
e n l a r g e me n t , r a t h e r t h a n s o f t e r, l e s s - d e f i n e d b r e a s t e n l a r g e me n t d u e t o a d i p o s e
t i s s u e d e p o s i t i o1)n . ( T h e t e s t i c l e s s h o u l d b e e xa mi n e d c a r e f u l l y. C o n g e n i t a l
a n o r c h i a i s a r a r e c a u s e o f g y n e c o ma s t i a . S ma l l b i l a t e r a l t e s t e s s u g g e s t g o n a d a l
f a i l u r e . T e s t i c u l a r a t r o p h y ma y b e d u e t o mu mp s , l e p r o s y, o r o t h e r g r a n u l o ma t o u s
d i s o r d e r s2)( . A s y mme t r y o r a p a l p a b l e ma s s s u g g e s t s t e s t i c u l a r c a n c e r.
C. Testing
I f p a t h o l o g y i s s u s p e c t e d , a d d i t i o n a l t e s t i n g ma y b e n e e d e d . T e s t s o f l i v e r, k i d n e y,
and thyroid function should be assessed if clinically indicated. If an underlying
e n d o c r i n e d i s o r d e r i s s u s p e c t e d , s e r u m H C G , t e s t o s t e r o n e , e s t r a d i o l , a n d l u t e i n i zi n
h o r mo n e ( L H ) s h o u l d b e c h e c1)k .e dA (h i g h L H a n d a n o r ma l - t o - l o w t e s t o s t e r o n e
i n d i c a t e t e s t i c u l a r i n s u f f i c i e n c y. E l e v a t e d e s t r a d i o l , w i t h s ma l l f i r m t e s t e s , b e h a v i o r
a b n o r ma l i t i e s o r me n t a l r e t a r d a t i o n , a n d a n a r m s p a n g r e a t e r t h a n t h e h e i g h t s h o u l d
p r o mp t a d i a g n o s t i c c h r o mo s o me a n a l y s i s f o r p o s s i b l e K l i n e f e l t e r ' s s y n d r o me . A h i g
L H a n d h i g h t e s t o s t e r o n e s u g g e s t a n a n d r o g e n r e s i s t a n c e s y n d r o me . A h i g h H C G
527 / 652
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14.2 - Gynecomastia
l e v e l ma y i n d i c a t e a n H C G - s e c r e t i n g t u mo r o f t h e l u n g , s t o ma c h , l i v e r, o r k i d n e y, o r
a t e s t i c u l a r o r e xt r a g o n a d a l g e r m c e l 2)
l t.u A
mo
n re (l e v a t e d H C G s h o u l d p r o mp t a
s e a r c h f o r o n e o f t h e s e c a n c e r s , b y c h e s t x- r a y, a b d o mi n a l c o mp u t e d t o mo g r a p h y,
a n d d e t a i l e d p h y s i c a l e xa mi n a t i o n . M a mmo g r a p h y h a s r e c e n t l y b e e n f o u n d t o b e
a c c u r a t e i n d i s t i n g u i s h i n g b e t w e e n ma l i g n a n t a n d b e n i g n ma l e b r e a s t d i s e a s e a n d
ma y b e u s e f u l i n l i mi t i n g t h e n e e d f o r b i o p s y i f p h y s i c a l e xa mi n a t i o n a n d
ma mmo g r a p h y s u g g e s t b e n i g n d i s4)
e a. sFei n( e n e e d l e a s p i r a t i o n o f a ma s s ma y b e
c o n s i d e r e d t o d i a g n o s e b r e a s t c a n c e r.
D. Genetics
K l i n e f e l t e r ' s s y n d r o me i n c r e a s e s t h e r i s k o f b r e a s t c a n c e r, b u t o t h e r c a u s e s o f
g y n e c o ma s t i a a r e n o t a s s o c i a t e d w i t h a n i n c r e a s e d r i s k o f b r1)
e a. s t c a n c e r (
I V. Diagnosis
I f t r u e g y n e c o ma s t i a a p p e a r s i n t h e e xp e c t e d a g e r a n g e s , i t i s l i k e l y t o b e
physiologic, and only reassurance and observation over 1 to 2 years are necessary
b e f o r e s p e c i f i c t r e a t me n t i s i n i t i a t e d . T h e me d i c a l h i s t o r y a t t h e t i me o f p r e s e n t a t i o
s h o u l d s c r e e n f o r me d i c a l o r e n d o c r i n e d i s o r d e r s t h a t s u g g e s t a d i a g n o s i s a n d
i d e n t i f y me d i c a t i o n s t h a t ma y b e t h e c a u s e o f g y n e c o ma s t i a . T h e p h y s i c a l
e xa mi n a t i o n f o c u s e s o n r u l i n g o u t b r e a s t c a n c e r a n d t e s t i c u l a r e xa mi n a t i o n , a n d
screening for endocrine disorders. If these steps are unrevealing, no related
d i a g n o s i s i s s u g g e s t e d , a n d t h e p a t i e n t i s o t h e r w i s e a s y mp t o ma t i c , r e a s s u r e a n d
o b s e r v e f o r 1 t o 2 y e a r s , e xp e c t i n g t h a t t h e c o n d i t i o n w i l l r e s o l v e s p o n t a n e o u s l y.
P s e u d o g y n e c o ma s t i a c a n b e d e t e c t e d b y t h e t y p i c a l f i n d i n g s o n p h y s i c a l e xa mi n a t i o
o r t h e p r e s e n c e o f o b e s i t y, w h i c h i s u s u a l l y a d e q u a t e t o i d e n t i f y t r u e b r e a s t t i s s u e
e n l a r g e me n t a g a i n s t e n l a r g e me n t o f t h e ma l e b r e a s t s d u e t o a d i p o s e t i s s u e i n o b e s e
me n .
B r e a s t c a n c e r p r e s e n t s a s a u n i l a t e r a l , e c c e n t r i c ma s s t h a t i s h a r d o r f i r m, f i xe d t o
u n d e r l y i n g t i s s u e , o r a s s o c i a t e d w i t h o v e r l y i n g s k i n d i mp l i n g , n i p p l e d i s c h a r g e o r
r e t r a c t i o n , o r a xi l l a r y l y mp h a d e n o p a t h y. A b i o p s y i s i n d i c a t e d .
G y n e c o ma s t i a i s b i l a t e r a l i n mo r e t h a n 5 0 % o f a f f e c t e d me n , b u t i t ma y b e u n i l a t e r a
o r s y mme t r i c . I t p r e s e n t s a s a f i r m, mo b i l e , o r r u b b e r y ma s s t h a t ma y b e s l i g h t l y
t e n d e r a n d f o r ms a s y mme t r i c a l mo u n d a r o u n d t h e n i p p l e . M o s t p a t i e n t s w i t h n o r ma l
p h y s i o l o g i c g y n e c o ma s t i a c a n b e r e a d i l y i d e n t i f i e d , a n d n o f u r t h e r e v a l u a t i o n i s
required. If the condition does not resolve with observation, if it is progressive or o
r a p i d o n s e t , o r i f h i s t o r y a n d e xa mi n a t i o n s u g g e s t a g e n e r a l me d i c a l d i s o r d e r, t h e n
an underlying pathologic process should be sought.
P. 3 2 3
References
1 . B r a u n s t e i n G D . G y n e c o ma N
s t iEa ngl
.
J M ed1 9 9 3 ; 3 2 8 : 4 9 0 4 9 5 .
528 / 652
tmdmss
14.2 - Gynecomastia
2 . F r a n t z A G , W i l s o n J D . D i s o r d e r s o f b r e a s t s i n me n . I n : W i l s o n J D , F o s t e r D W,
e d s . Wi l l i ams t ex t book of endoc r i nol
, 9ogy
t h e d . P h i l a d e l p h i a , PA : W B
S a u n d e r s C o mp a n y, 1 9 9 8 : 8 8 5 9 0 0 .
3 . W i s e G J . M a l e b r e a s t d i s eJa sAem. C ol l S ur2g0 0 5 ; 2 0 0 : 2 5 5 2 6 9 .
4 . E v a n s G F F. T h e d i a g n o s t i c a c c u r a c y o f ma mmo g r a p h y i n t h e e v a l u a t i o n o f
ma l e b r e a s t d i s e a A
s em. J S ur g2 0 0 1 ; 1 8 1 : 9 6 1 0 0 .
529 / 652
tmdmss
14.3 - Hirsutism
> T a b l e o f C o n t e n t s > 1 4 - E n d o c r i n e a n d M e t a b o l i c P r o b l e ms > 1 4 . 3 - H i r s u t i s m
14.3
Hirsutism
Richard D. Blonde ll
Vinod R. Pate l
I . Background
H i r s u t i s m i s e xc e s s i v e ma l e - p a t t e r n h a i r g r o w t h i n w o me n o f r e p r o d u c t i v e a g e .
P a t h o l o g i c c a u s e s o f h i r s u t i s m ma y b e a s s o c i vait rei dl i zat
w i t ihon
( t h e d e v e l o p me n t
o f o t h e r s e c o n d a r y ma l e s e x c h a r a c t e r i sdef
t i cemi
s ) oni
r zat i on
(the loss of
s e c o n d a r y f e ma l e s e x c h a r a c t e r i s t i c s ) .
A. Etiology
H i r s u t i s m ma y b e i d i o p a t h i c o r d u e t o e xo g e n o u s a n d r o g e n s , i n c r e a s e d p r o d u c t i o n o
androgens by the ovaries or adrenal glands, or increased hair follicle sensitivity to
a n d r o g e n s . C o mmo n c a u s e s a r e s u mma rTi ze
a bdl ei n1 4 . 3.. 1
1. C o m m o n c a u s eTsh e mo s t c o mmo n c a u s e s o f h i r s u t i s m a r e p o l y c y s t i c o v a r y
s y n d r o me ( P C O S ) a n d i d i o p a t h i c 1)
f o .r ms
L e s( s c o mmo n c a u s e s i n c l u d e
hyperandrogenic insulin-resistant acanthosis nigricans (H AIR AN) and various
f o r ms o f 2 1 - h y d r o xy l a s e d e f i c i e n t a d r e n a l h y p e r p l a s i a .
2. O t h e r c a u s eAsn d r o g e n - s e c r e t i n g t u mo r s o f t h e o v a r i e s ( S e r t o l i - L e y d i g c e l l
t u mo r s , g r a n u l o s a - t h e c a c e l l t u mo r s , a n d h i l u s - c e l l t u mo r s ) , a d r e n a l g l a n d s , o r
l u n g s a r e n o t c o mmo n , e v e n a mo n g r e f e r r a l p o p1)u .l aHt i ior ns us t (i s m ma y b e
o b s e r v e d a mo n g p a t i e n t s w h o h a v e h y p o t h y r o i d i s m, h y p e r p r o l a c t i n e mi a , o r
C u s h i n g ' s s y n d r o me . M i l d h i r s u t i s m ma y b e c o n s t i t u t i o n a l ( a s a v a r i a n t o f n o r ma
me n o p a u s e , p r e g n a n c y, o r o b e s i t y ) .
B. Epidemiology
T h e p r e v a l e n c e o f h i r s u t i s m d e p e n d s o n t h e me t h o d s u s e d t o d e f i n e i t a n d t h e
p o p u l a t i o n s t u d i e d , b u t i t i s e s t i ma t e d t o a f f e c t b e t w e e n 4 % a n d 8 % o f w o me n i n
t h e i r c h i l d b e a r i n g y e 2,3)
a r s . ( A p p r o xi ma t e l y 5 0 % o f w o me n w i t h mi n i ma l u n w a n t e d
h a i r g r o w t h h a v e a n a n d r o g e n e xc e s s d i2)
s o. r d e r (
I I I . Evaluation
530 / 652
tmdmss
14.3 - Hirsutism
A. History
T h e h i s t o r y o f h i r s u t i s m ( a g e a t o n s e t , r a t e o f p r o g r e s s i o n ) a n d t h e me n s t r u a l h i s t o
( a g e a t me n a r c h e , r e g u l a r i t y o f me n s t r u a l c y c l e s ) ma y p r o v i d e i mp o r t a n t d i a g n o s t i c
c l u e s . I n f e r t i l i t y a n d w e i g h t g a i n s u g g e s t P C O S . C e r t a i n me d i c a t i o n s s u c h a s
d a n a zo l a n d o r a l c o n t r a c e p t i v e s t h a t c o n t a i n a n d r o g e n i c p r o g e s t i n s ( e . g . ,
l e v o n o r g e s t r e l ) c a n c a u s e h i r s u t i s m. H i g h l y c o mp e t i t i v e w o me n a t h l e t e s ma y a b u s e
a n a b o l i c s t e r o i d s . S o me f o r ms o f h i r s u t i s m a r e f a mi l i a l .
D u r i n g e xa mi n a t i o n , t h e h e i g h t a n d w e i g h t s h o u l d b e d o c u me n t e d a n d t h e b o d y ma s
index calculated. T he distribution of hair growth on nine areas of the body (upper li
c h i n , c h e s t , a b d o me n , s u p e r i o r p u b i c t r i a n g l e , u p p e r a r ms , t h i g h s , u p p e r b a c k , a n d
b u t t o c k s ) a r e u s e d t o q u a n t i f y h i r s3)u.t iAs nmd (r o g e n e xc e s s ma y c a u s e a c n e . S i g n s
o f v i r i l i za t i o n ( ma l e - p a t t e r n b a l d i n g , d e e p e n i n g o f t h e v o i c e , i n c r e a s e d mu s c l e ma s s
a n d c l i t o r o me g a l y ) o r d e f e mi n i za t i o n ( l o s s o f b r e a s t t i s s u e , v a g i n a l a t r o p h y ) s h o u l d
b e n o t e d . S o me d i a g n o s e s ma y b e s u g g e s t e d b y o t h e r p h y s i c a l f i n d i n g s : C u s h i n g ' s
s y n d r o me ( t r u n c a l o b e s i t y, b u f f a l o
P. 3 2 4
h u mp ) , h y p e r p r o l a c t i n e mi a ( g a l a c t o r r h e a ) , H A I R A N ( v e l v e t y h y p e r p i g me n t a t i o n o f
t h e a xi l l a , g r o i n , n e c k , o r u mb i l i c u s ) , a n d o v a r i a n t u mo r ( p e l v i c ma s s ) .
TAB L E 14.3.1 T he Com m on Causes of Hirsutism

Diagnosis
F r e q u e n cay
Clinical
(%)
m anife stations Laboratory te sts
Polycystic ovarian 82
s y n d r o me
A b n o r ma l
me n s e s ,
i n f e r t i l i t y,
o b e s i t y,
diabetes
Elevated androgens,
ultrasound showing
mu l t i p l e o v a r i a n
cysts
Idiopathic
hirsutism
12
N o r ma l
me n s e s a n d
n o r ma l
e xa mi n a t i o n
N o r ma l s e r u m
progesterone in
luteal phase (2024
d)
N o r ma l
androgen levels
6.8
Elevated
androgen levels
4.7
Hyperandrogenic 3
F a mi l y h i s t o r y E l e v a t e d i n s u l i n
531 / 652
tmdmss
14.3 - Hirsutism
insulin-resistant
acanthosis
nigricans
of diabetes,
levels
o b e s i t y, b r o w n
velvety skin
patches
Late-onset 212
h y d r o xy l a s e
deficient
nonclassic adrenal
hyperplasia
F a mi l y h i s t o r y, E l e v a t e d 1 7 v i r i l i za t i o n ,
h y d r o xy p r o g e s t e r o n e
d e f e mi n i za t i o n
Congenital 211
h y d r o xy l a s e
deficient adrenal
hyperplasia
Congenital
v i r i l i za t i o n
E levated 17 h y d r o xy p r o g e s t e r o n e
Androgens e c r e t i n g t u mo r
<1
Rapid-onset
h i r s u t i s m,
v i r i l i za t i o n
D i a g n o s t i c i ma g e s
d e mo n s t r a t i n g a
t u mo r
All other causes
<2
S e e t e xt
S e e t e xt
aF r e q u e n c y d a t a f r o m A zzi z R , S a n c h e z E S , K n o c h e n h a u e r C , e t a l .
A n d r o g e n e xc e s s i n w o me n : e xp e r i e n c e w i t h o v e r 1 , 0 0 0 c o n s e c u t i v e p a t i e n t s .
J C l i n E ndoc r i nol M et2ab
004;89:453462.
C. Testing
I f t h e p a t i e n t ' s h i s t o r y a n d p h y s i c a l e xa mi n a t i o n a r e u n r e ma r k a b l e , s e r u m l e v e l s o f
total testosterone and dehydroepiandrosterone sulfate ( D H E A- S ) can be obtained to
e xc l u d e a n a n d r o g e n - p r o d u c i n g t u mo r. E l e v a t e d l e v e l s o f a n d r o g e n s o v e r t w i c e t h e
n o r ma l v a l u e s s h o u l d p r o mp t a n e v a l u a t i o n f o r a n o v a r i a n o r a d r e n a l t u mo r. H i g h resolution pelvic ultrasonography with a transvaginal probe can identify ovarian
f o l l i c l e s a n d c y s t s a s s ma l l a s 3 t o 5 mm i n d i a me t e r. B a s a l b o d y t e mp e r a t u r e c h a r t s
a n d s e r u m p r o g e s t e r o n e l e v e l s i n t h e l u t e a l p h a s e ( 2 0 2 4 d a y s ) o f t h e me n s t r u a l
c y c l e c a n b e u s e d t o d o c u me n t t h e n o r ma l o v a r i a n f u n c t i o n o f w o me n w h o a r e
t h o u g h t t o h a v e i d i o p a t h i c h i r s u t i s m. A c l i n i c a l s u s p i c i o n o f h y p o t h y r o i d i s m,
h y p e r p r o l a c t i n e mi a , o r C u s h i n g ' s d i s e a s e r e q u i r e s c o n f i r ma t o r y t e s t i n g . R e f e r r a l a n
f u r t h e r d i a g n o s t i c t e s t i n g ma y b e w a r r a n t e d f o r p a t i e n t s w i t h e a r l y o n s e t , s e v e r e , o r
r a p i d l y p r o g r e s s i v e h i r s u t i s m.
P. 3 2 5
D. Genetics
A f a mi l i a l p a t t e r n c a n b e a s s o c i a t e d w i t h i d i o p a t h i c h i r s u t i s m, P C O S , H A I R A N , a n d
late-onset nonclassic congenital adrenal hyperplasia, which is a disorder particular
a s s o c i a t e d w i t h A s h k e n a zi J e w i s h w o me n .
532 / 652
tmdmss
14.3 - Hirsutism
I V. Diagnosis
M o s t h i r s u t e w o me n h a v e e i t h e r P C O S o r i d i o p a t h i c h i r s u t i s m. T h e c h a l l e n g e i s t o
i d e n t i f y t h e s ma l l n u mb e r o f w o me n w h o h a v e s o me o t h e r c a u s e .
1. H i r s u t i s mP C O S a n d i d i o p a t h i c h i r s u t i s m a r e d i a g n o s e s o f e xc l u s i o n . P C O S
can be diagnosed if two of the following are present: oligo-ovulation or
a n o v u l a t i o n ( u s u a l l y ma n i f e s t e d a s o l i g o me n o r r h e a o r a me n o r r h e a ) , e l e v a t e d
l e v e l s o f a n d r o g e n s o r s i g n s o f a n d r o g e n e xc e s s ( h y p e r a n d r o g e n i s m) , a n d
p o l y c y s t i c o v a r i e s a s d e f i n e d b y u l t r a s o n o4)g.r aTphheyr e( f o r e , p o l y c y s t i c
ovaries are not always present in P C O S and their presence alone does not
establish the diagnosis.
2. H y p e r t r i c h o s iTsh i s c o n d i t i o n , w h i c h ma y b e f a mi l i a l , i s d e s c r i b e d a s e xc e s s i v e
g r o w t h o f a n d r o g e n - i n d e p e n d e n t5)h. aIitr i (s u s u a l l y v e l l u s a n d p r o mi n e n t i n
n o n s e xu a l a r e a s . C a u s e s i n c l u d e h y p o t h y r o i d i s m, a n o r e xi a n e r v o s a ,
ma l n u t r i t i o n , p o r p h y r i a , d e r ma t o my o s i t i s , a n d me d i c a t i o n s ( p h e n y t o i n ,
p e n i c i l l a mi n e , d i a zo xi d e , mi n o xi d i l , o r c y c l o s p o r i n e ) .
S e v e r a l c l i n i c a l f i n d i n g s s u g g e s t o n e o f t h e r a r e a n d mo r e s e r i o u s c a u s e s o f
h i r s u t i s m: ( 1 ) a b r u p t o n s e t , s h o r t d u r a t i o n ( t y p i c a l l y < 1 y e a r ) , o r p r o g r e s s i v e
w o r s e n i n g o f h i r s u t i s m; ( 2 ) o n s e t i n t h e t h i r d d e c a d e o f l i f e o r l a t e r, r a t h e r t h a n n e a
p u b e r t y ; ( 3 ) s y mp t o ms o r s i g n s o f v i r i l i za t i o n o r d e f e mi n i za t i o n ; ( 4 ) mo r e s e v e r e
h i r s u t i s m ( o v a r i a n h y p e r t h e c o s i s ) ; ( 5 ) s y mp t o ms a n d s i g n s o f c o r t i s o l e xc e s s , s u c h
a s o b e s i t y, h y p e r t e n s i o n , s t r i a e , t h a t s u g g e s t t h e p r e s e n c e o f C u s h i n g ' s s y n d r o me ;
a n d ( 6 ) mo d e r a t e l y e l e v a t e d ( o r h i g h e r ) s e r u m a n d r o g e n c o n c e n t r a t i o n s . S e r u m
t e s t o s t e r o n e v a l u e s a b o v e 1 5 0 n g / d L ( 5 . 2 n mo l / L ) , s e r u m- f r e e t e s t o s t e r o n e v a l u e s
a b o v e 2 n g / d L ( 0 . 0 7 n mo l / L ) , o r D H E A - S v a l u e s g r e a t e r t h a n 7 0 0 g / d L ( 1 3 . 6
mo l / L ) i n y o u n g w o me n r a i s e t h e p o s s i b i l i t y o f a n a n d r o g e n - s e c r e t i n g t u mo r.
References
1 . A zzi z R , S a n c h e z L A , K n o c h e n h a u e r E S , e t a l . A n d r o g e n e xc e s s i n w o me n :
e xp e r i e n c e w i t h o v e r 1 0 0 0 c o n s e c u t i v e pJa tCi el innt sE. ndoc r i nol M et ab
2004;89:453462.
2 . S o u t e r I , S a n c h e z L A , P e r e z M , e t a l . T h e p r e v a l e n c e o f a n d r o g e n e xc e s s
a mo n g p a t i e n t s w i t h mi n i ma l u n w a n t e d h a i rAgmr oJw tOhbs
. t et G y nec ol
2004;191:19141920.
3 . A zzi z R . T h e e v a l u a t i o n a n d ma n a g e me n t o f hO
i r bs
s ut et
i s m.
G y nec ol
2003;101:9951007.
4 . E h r ma n n D A . P o l y c y s t i c o v a r y s y n dNr oEme
ngl. J M ed2 0 0 5 ; 3 5 2 : 1 2 2 3 1 2 3 6 .
533 / 652
tmdmss
14.3 - Hirsutism
5 . We n d e l i n D S , P o p e D N , M a l l o r y S B . H y p e r t rJi cAhm
o sAi sc.ad D er mat ol
2003;48:161179.
534 / 652
tmdmss
14.4 - Hypothyroidism
> T a b l e o f C o n t e n t s > 1 4 - E n d o c r i n e a n d M e t a b o l i c P r o b l e ms > 1 4 . 4 Hypothyroidism
14.4
Hypothyroidism
R i c h a r d W. P r e t o r i u s
S t e p h e n F. W h e e l e r
I . Background
H y p o t h y r o i d i s m i s t h e c l i n i c a l s y n d r o me r e s u l t i n g f r o m t h y r o i d h o r mo n e d e f i c i e n c y.
T h y r o t r o p i n - r e l e a s i n g h o r mo n e , s e c r e t e d b y t h e h y p o t h a l a mu s , s t i mu l a t e s t h e
a n t e r i o r p i t u i t a r y t o p r o d u c e t h e t h y r o i d - s t i mu l a t i n g h o r mo n e ( T S H ) , w h i c h me d i a t e s
t h e p r o d u c t i o n o f t h y r o xi 4n)e. ( T
A. Etiology
P r i mar y hy pot hy r oi dii s mt h e r e s u l t o f t h y r o i d g l a n d f 1)

a i .l uTr eh e( mo s t c o mmo n
c a u s e i s a u t o i mmu n e t h y r o i d i t i s , o r H a s h i mo t o ' s d i s e a s e , w h i c h
P. 3 2 6
r e s u l t s f r o m t h e g r a d u a l d e s t r u c t i o n o f t h e t h y r o i d b y a b n o r ma l T c e l l s . I a t r o g e n i c
h y p o t h y r o i d i s m f r o m e i t h e r r a d i o a c t i v e i o d i n e o r s u r g e r y i s t h e n e xt mo s t c o mmo n
cause. Goitrous hypothyroidism from iodine deficiency is rare in the United States
s i n c e t h e i n t r o d u c t i o n o f i o d i zeSd ec
s aondar
lt.
yo r t er t i ar y hy pot hy r oi dii s m
c a u s e d b y p i t u i t a r y o r h y p o t h a l a mi c d i s e a s e , r e s p e c t i v e l y. T h e mo s t c o mmo n c a u s e
o f s e c o n d a r y h y p o t h y r o i d i s m i s a p i t u i t a r y t u mo r ; o t h e r c o mmo n c a u s e s i n c l u d e
p i t u i t a r y s u r g e r y, c r a n i a l r a d i a t i o n t h e r a p y, p o s t p a r t u m h e mo r r h a g e ( S h e e h a n ' s
s y n d r o me ) , h e a d t r a u ma , g r a n u l o ma t o u s d i s e a s e s , me t a s t a t i c d i s e a s e ( b r e a s t , l u n g ,
colon, and prostate), and infectious diseases (tuberculosis and others). Multiple
e n d o c r i n e e n d - o r g a n f a i l u r e c a u s e d b y t h e a u t o i mmu n e d e s t r u c t i o n o f e n d o c r i n e
g l a n d s ( S c h mi d t ' s s y n d r o me ) i s a r a r e c a u s e o f p r i ma r y h y p o t h y r o i d i s m t h a t mi mi c s
secondary disease.
B. Epidemiology
O v e r t h y p o t h y r o i d i s m i s f o u n d i n 0 . 5 % o f mi d d l e - a g e d w o me n b e t w e e n 4 0 a n d 6 0
y e a r s o f a g e , i n c r e a s i n g t o 2 % i n w o me n o l d e r t h a n 7 0 y e2)a.r sI t oifs asgl ieg h( t l y
l e s s c o mmo n a mo n g me n , H i s p a n i c s , a n d b l a c k s .
I I I . Evaluation
535 / 652
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A. History
S y mp t o ms g e n e r a l l y c o r r e s p o n d d i r e c t l y t o t h e d u r a t i o n a n d s e v e r i t y o f d i s e a s e .
1. T h e p r o b a b i l i t y o f t h y r o i d d i s e a s e i s d i r e c t l y r e l a t e d t o t h e n u mb e r o f t y p i c a l
s y mp t o ms ma n i f e s t e d b y t h e p a t i e n t , i n c l u d i n g w e a k n e s s , l e t h a r g y, f a t i g u e , s k i n
c h a n g e s ( d r y, c o a r s e , c o l d , y e l l o w ) , c o a r s e n e s s o r l o s s o f h a i r, c o l d i n t o l e r a n c e
w e i g h t g a i n , c o n s t i p a t i o n , me mo r y o r c o n c e n t r a t i o n i mp a i r me n t , d e p r e s s i o n ,
h o a r s e n e s s , g o i t e r, me n s t r u a l a b n o r ma l i t i e s ( mo s t c o mmo n l y me n o r r h a g i a ) , a n d
fluid infiltration of tissues (eyelids, face, periphery). Secondary or tertiary
h y p o t h y r o i d i s m i s s u g g e s t e d b y l o s s o f a xi l l a r y o r p u b i c h a i r, h e a d a c h e s , v i s u a l
f i e l d d e f e c t s , a me n o r r h e a , g a l a c t o r r h e a , a n d s y mp t o ms o f p o s t u r a l h y p o t e n s i o n .
2. P a t i e n t s w i t h a n i n c r e a s e d r i s k i n c l u d e w o me n 4 t o 8 w e e k s p o s t p a r t u m, w o me n
o l d e r t h a n 5 0 y e a r s o f a g e , p a t i e n t s w i t h i mmu n o l o g i c a l l y me d i a t e d d i s e a s e s
( d i a b e t e s me l l i t u s t y p e 1 , p e r n i c i o u s a n e mi a , v i t i l i g o , A d d i s o n ' s d i s e a s e , a n d
r h e u ma t o i d a r t h r i t i s ) , a n d p e r s o n s w i t h a f a mi l y h i s t o r y o f t h y r o i d d i s e a s e .
1. O b s e r v a t i o A
n w e l c o mi n g h a n d s h a k e ma y r e v e a l c o l d s k i n a n d f u r t h e r
o b s e r v a t i o n s u n c o v e r a l t e r e d a f f e c t , h o a r s e n e s s , f a c i a l o r e y e l i d e d e ma , h a i r
l o s s ( s c a l p a n d e y e b r o w s ) , a n d p h y s i c a l o r me n t a l s l o w i n g .
2. G e n e r a l e x a m i n a t i Vi
o nt a l s i g n a b n o r ma l i t i e s c o mmo n l y i n c l u d e w e i g h t g a i n ,
d i a s t o l i c h y p e r t e n s i o n , a n d b r a d y c a r d i a . A s y s t e ma t i c h e a d - t o - t o e e xa mi n a t i o n i s
h e l p f u l , b e c a u s e a l l ma j o r o r g a n s y s t e ms a r e a f f e c t e d b y t h y r o i d h o r mo n e
d e f i c i e n c y. T h e h e a r t ma y b e e n l a r g e d o w i n g t o e i t h e r d i l a t i o n o r p e r i c a r d i a l
e f f u s i o n , w h i c h ma y b e i n d i c a t e d b y a c a r d i a c r u b o r d i s t a n t h e a r t s o u n d s .
A d y n a mi c i l e u s c a n c a u s e c o n s t i p a t i o n a n d a b d o mi n a l d i s t e n t i o n a n d , r a r e l y, c a n
r e s u l t i n me g a c o l o n o r i n t e s t i n a l o b s t r u c t i o n . E v e n c a r p a l t u n n e l s y n d r o me c a n
o c c u r f r o m t h e g e n e r a l i ze d e d e ma o w i n g t o t i s s u e g l y c o s a mi n o g l y c a n
a c c u mu l a t i o n a n d r e d u c e d l y mp h a t i c c l e a r a n c e o f i n t e r s t i t i a l p r o t e i n s .
H y p o r e f l e xi a ma y b e p r e s e n t i n a d d i t i o n t o a p r o l o n g e d r e l a xa t i o n p h a s e o f t h e
d e e p t e n d o n r e f l e xe s , c r e a t i n g t h e c h a r a c t e r i s t i c h u n g - u p r e f l e x. S e c o n d a r y o r
tertiary disease is suggested by orthostatic hypotension, visual field defects,
and galactorrhea.
3. T h y r o i d e x a m i n a t i To hn e t h y r o i d i s p a l p a t e d b y u s i n g t h e f i n g e r s o r t h u mb s
while standing in front of or behind the patient. If felt between the cricoid
c a r t i l a g e a n d t h e s u p r a s t e r n a l n o t c h , t h e t h y r o i d i s t h mu s c a n b e u s e d t o h e l p
l o c a t e t h e g l a n d . T h e l o c a t i o n , s i ze , c o n s i s t e n c y, mo b i l i t y, a n d t e n d e r n e s s o f
any nodules should be noted. Having the patient swallow during both inspection
a n d p a l p a t i o n c a u s e s t h e t h y r o i d t o mo v e a n d a i d s i n d e v e l o p i n g a t h r e e d i me n s i o n a l i mp r e s s i o n o f g l a n d ' s s h a p e a n d s i ze .
C. Testing
T h e mo s t u s e f u l t e s t f o r t h e d i a g n o s i s o f t h y r o i d d i s e a s e i s a n e l e v a t e d T S H o f > 1 0
mI U / L , w h i c h h a s a h i g h s e n s i t i v i t y ( 9 8 % ) a n d s p e c i f i c i t y ( 9 2 % ) i n a r e f e r r a l o f f i c e .
536 / 652
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I t s p o s i t i v e p r e d i c t i v e v a l u e , h o w e v e r, i s l o w w h e n u s e d f o r s c r e e n i n g p r i ma r y c a r e
p o p u l a t i o n s . A f r e4e, T
o r o c c a s i o n a l l y t h e mu c h mo r e e xp e n s i v3e, df roeees Tn o t
need to be ordered routinely but can confirm the presence of hypothyroidism
P. 3 2 7
when the T S H is elevated or in the rare situation when hypothyroidism is suspected
i n t h e p r e s e n c e o f a n o r ma l T S H . A l t h o u g h a n t i b o d i e s t o t h y r o i d p e r o xi d a s e a r e
e l e v a t e d i n 9 5 % o f p a t i e n t s w i t h H a s h i mo t o ' s t h y r o i d i t i s , d e t e r mi n i n g t h e i r p r e s e n c e
i s u s u a l l y n o t n e c e s s a r y i n t h e e v a l u a t i o n o f p a t i e n t s w i t h h y p o t h y r o i d i s m, b e c a u s e
ma n a g e me n t i s u n c h a n g3)e .d S( i mi l a r l y, b e c a u s e r a d i o a c t i v e i o d i n e u p t a k e i s
typically low in hypothyroidism of any cause, radionuclide scans are also not
n o r ma l l y h e l p f u l . A l t h o u g h t h e T S H i s c h a r a c t e r i s t i c a l l y e l e v a t e d i n p r i ma r y
h y p o t h y r o i d i s m, p i t f a l l s c a n o c c u r. S t a r v a t i o n , c o r t i c o s t e r o i d a d mi n i s t r a t i o n , a n d u s
o f d o p a mi n e c a n l o w e r T S H , e v e n i n p a t i e n t s w i t h h y p o t h y r o i d i s m. I n s o me
h o s p i t a l i ze d p a t i e n t s w i t h s e v e r e n o n t h y r o i d a l i l l n e s s , l o w p e r i p h e r a l t h y r o i d h o r mo n
l e v e l s ma y s u g g e s t h y p o t h y r o i d i s m, a l t h o u g h t h e T S H i s u s u a l l y n o r ma l i n t h i s
setting. T he U . S . P reventive S ervices Task F orce finds the evidence for either
b e n e f i t o r h a r m i n s u f f i c i e n t t o r e c o mme n d f o r o r a g a i n s t r o u t i n e s c r e e n i n g f o r t h y r o i
d i s e a s e i n a s y mp t o ma t i c a d4)u .l t s (
D. Genetics
T h e mo l e c u l a r d e f e c t s h a v e b e e n i d e n t i f i e d i n o n l y a f e w c a s e s o f h u ma n
hypothyroidism
5) .( H y p o t h y r o i d i s m r e ma i n s p r i ma r i l y a n a c q u i r e d d i s e a s e
associated with aging.
I V. Diagnosis
B e c a u s e t h e s y mp t o ms o f h y p o t h y r o i d i s m a r e n o n s p e c i f i c , i t s d i f f e r e n t i a l d i a g n o s i s
l a r g e a n d i n c l u d e s d e p r e s s i o n , e mo t i o n a l o r p h y s i c a l s t r e s s , c h r o n i c i n f e c t i o n s ,
a u t o i mmu n e d i s o r d e r s , a n e mi a , c a r d i o v a s c u l a r d i s e a s e , o c c u l t ma l i g n a n c i e s , a d v e r s
r e a c t i o n s t o me d i c a t i o n , a n d o t h e r e n d o c r i n e d i s o r d e r s s u c h a s d i a b e t e s . B e c a u s e o
t h e mu l t i s y s t e m i n v o l v e me n t o f h y p o t h y r o i d i s m, t h e c l i n i c i a n mi g h t f o c u s a t t e n t i o n o
c h a n g e s , s u c h a s c o n s t i p a t i o n , i n o n e o r g a n s y s t e m, t h e g a s t r o i n t e s t i n a l s y s t e m,
w h i c h c a n d i v e r t a t t e n t i o n t o t h e p r e s e n c e o f s i g n s a n d s y mp t o ms o f h y p o t h y r o i d i s m
i n o t h e r o r g a n s y s t e ms .
M o s t p a t i e n t s h a v e o n l y mi l d o r mo d e r a t e d i s e a s e a t t h e t i me o f d i a g n o s i s .
S ubc l i ni c al hy pot hy r oi dio cs cmu r s w h e n t h e T S H i s mi l d l y e l e v a t e d ( 4 . 5 1 0 . 0
mI U / L ) a n d t h e r e a r e n o a s s o c i a t e d s i g n s o r s y mp t o ms . I f n o t t r e a t e d t o p r e v e n t
p r o g r e s s i o n , t h e s e p a t i e n t s c a n b e mo n i t o r e d e v e r y 2 t o 5 y e a r s t o d e t e c t t h e c a s e s
t h a t w i l l p r o g r e s s t o o v e r t h y p o t h y r o i d i s m a t a r a t e o f u p t o 2)
5%
. Ppr iemar
r y eya r (
hy pot hy r oi di scma n b e d i a g n o s e d b y t h e t y p i c a l f i n d i n g s o n h i s t o r y a n d p h y s i c a l
e xa mi n a t i o n , c o u p l e d w i t h a n e l e v a t e d T S H a n d a 4l .o S
wec
f r ondar
ee T y
hy pot hy r oi di ssmh o u l d b e s u s p e c t e d w h e n b o t h t h e T S H a n d t4h ea r fer el oe w.
T
M y x edema c oma
i s a l i f e - t h r e a t e n i n g c o mp l i c a t i o n o f l o n g - s t a n d i n g h y p o t h y r o i d i s m,
w h i c h i s r a r e i n w a r m c l i ma t e s a n d u n c o mmo n i n c o l d c l i ma t e s . T h e s e p a t i e n t s ma y
537 / 652
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p r e s e n t w i t h s t u p o r, a r e f l e xi a , r e s p i r a t o r y d e p r e s s i o n , h y p e r c a p n i a , a n d p r o f o u n d
h y p o t h e r mi a t h a t ma y g o u n d e t e c t e d w i t h s t a n d a r d me d i c a l t h e r mo me t e r s . P r o mp t
d i a g n o s i s , h o s p i t a l i za t i o n , a n d r a p i d t r e a t me n t a r e r e q u i r e d t o p r e v e n t d e a t h . F a c t o
t h a t p r e d i s p o s e t o my xe d e ma c o ma i n c l u d e i n f e c t i o n , t r a u ma , c o l d e xp o s u r e , a n d
central nervous system depressants.
References
1 . H o l l o w e l l J G , S t a c h l i n g N W, F l a n d e r s W D , e t a l . S e r u m T S H , T 4 , a n d t h y r o i d
antibodies in the United States population (1988 to 1994): National Health and
N u t r i t i o n E xa mi n a t i o n S u r v e y ( H H A N EJ SC Il Ii In) .E ndoc r i nol M et ab
2002;87:489499.
2 . H e l f a n d M , R e d f e r n C C . S c r e e n i n g f o r t h y r o i d Adnn
i s eIants eer. n M ed
1998;129:144158.
3 . L e G r y s VA , H a r t ma n n K , Wa l s h J F. T h e c l i n i c a l c o n s e q u e n c e s a n d d i a g n o s i s
o f h y p o t h y r o i d i sCm.l i n L ab S c2i 0 0 4 ; 1 7 ( 4 ) : 2 0 3 2 0 8 .
4 . A g e n c y f o r H e a l t h c a r e R e s e a r c h a n d Q u a l i t y. U . S . P r e v e n t i v e S e r v i c e s T a s k
F o r c e .R ec ommendat i on s t at ement : s c r eeni ng f or t hy r oi d di s eas e ( J anuar y
2004). Av a i l a b l e a t : h t t p : / / w w w. a h r q . g o v / c l i n i c / 3 r d u s p s t f / t h y r o i d / t h y r r s . h t m,
accessed on July 7, 2005.
5 . M o r e n o J C , d e Vi j l d e r J J , Vu l s ma T , e t a l . G e n e t i c b a s i s o f h y p o t h y r o i d i s m:
r e c e n t a d v a n c e s , g a p s a n d s t r a t e g i e s f o r f u t u r e Tr
r eends
s e a r cEhndoc
.
r i nol
M et ab2 0 0 3 ; 1 4 ( 7 ) : 3 1 8 3 2 6 .
538 / 652
tmdmss
14.5 - Polydipsia
> T a b l e o f C o n t e n t s > 1 4 - E n d o c r i n e a n d M e t a b o l i c P r o b l e ms > 1 4 . 5 - P o l y d i p s i a
14.5
Polydipsia
S o r a y a P. N a s r a t y
I . Background
P o l y d i p s i a i s e xc e s s i v e d r i n k i n g o f w a t e r o w i n g t o a b n o r ma l t h i r s t t h a t c a n b e
a t t r i b u t e d t o me d i c a l o r p s y c h o g e n i c c a u s e s .
A. Epidemiology
P o l y d i p s i a i s a c o mmo n s y mp t o m a mo n g p a t i e n t s w i t h d i a b e t e s me l l i t u s ( D M ) a n d
p r o mi n e n t i n p a t i e n t s w i t h d i a b e t e s i n s i p i d u s ( D I ) . P o l y d i p s i a h a s a p r e v a l e n c e o f 3
t o 3 9 % a mo n g c h r o n i c p s y c h i a t r i c i n p a1)t .i e n t s (
B. Etiology
I n i t i a l l y, t h e p h y s i c i a n s h o u l d t r y t o c l a s s i f y a n d i d e n t i f y t h e e t i o l o g y o f p o l y d i p s i a ,
w h i c h i s u s u a l l y a c c o mp a n i e d b y p o l y u r i a .
1. P o o r l y r e s o r b e d s o l u G
t el us c o s e , ma n n i t o l , o r s o r b i t o l c a n c a u s e a n o s mo t i c
diuresis. D M should be suspected in any patient with polydipsia and polyuria of
recent onset.
2. P r i m a r y p o l y d i p sTi ah i s ma y b e c a u s e d b y p s y c h o t i c d e l u s i o n s , a h y p e r a c t i v i t y
o f h y p o t h a l a mi c t h i r s t c e n t e r s , o r a d r y mo n t h d u e t o t h e a n t i c h o l i n e r g i c e f f e c t s
o f me d i c a t i o n s .
3. D i a b e t e s i n s i p i d u s (TDhI i) s ma y b e d u e t o e i t h e r a c e n t r a l ( n e u r o g e n i c D I ) o r
r e n a l ( n e p h r o g e n i c D I ) c a u s e . C e n t r a l ( c o mp l e t e o r p a r t i a l ) D I i s c a u s e d b y a
d e f e c t i n t h e s e c r e t i o n o f a n t i d i u r e t i c h o r mo n e ( A D H ) b y t h e p i t u i t a r y g l a n d .
Neurogenic D I can be idiopathic, genetic, or secondary to intracranial pathology
s u c h a s a b r a i n t u mo r, h e a d t r a u ma , t o xi c b r a i n i n j u r y, me t a s t a t i c c a n c e r,
g r a n u l o ma t o u s d i s e a s e ( t u b e r c u l o s i s , s a r c o i d o s i s ) , o r f r o m a c o mp l i c a t i o n o f a
n e u r o s u r g i c a l p r o c e d u r e . Va s o p r e s s i n a s e - i n d u c e d D I o c c u r s i n t h e l a s t t r i me s t e
o f p r e g n a n c y a n d i s o f t e n a s s o c i a t e d w i t h p r e - e c l a mp s i a .
Nephrogenic D I can be caused by the nephrons not responding to AD H from an
i n h e r i t e d d e f e c t , c a n r e s u l t f r o m a n a c q u i r e d p r o b l e m s e c o n d a r y t o me d i c a t i o n s
( l i t h i u m, me t h o xy f l u r a n e , d e me c l o c y c l i n e ) , o r c a n r e s u l t f r o m s y s t e mi c d i s e a s e
( h y p o k a l e mi a , h y p e r c a l c e mi a ) .
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14.5 - Polydipsia
4. I a t r o g e n i c p o l y d i p sT ihai s c a n o c c u r b e c a u s e o f p a t i e n t s mi s i n t e r p r e t i n g
p h y s i c i a n ' s i n s t r u c t i o n s t o d r i n k p l e n t y o2)
f .w a t e r (
I I I . Evaluation
A. History
I n e l i c i t i n g t h e h i s t o r y, t h e c l i n i c i a n s h o u l d t a k e n o t e o f n e u r o l o g i c s y mp t o ms
( p r o b l e ms w i t h v i s u a l f i e l d s , h e a d a c h e s , n u mb n e s s ) ; a p r i o r h i s t o r y o f c a n c e r,
p a r t i c u l a r l y me t a s t a t i c b r a i n c a n c e r ; a h i s t o r y o f t r a u ma ; n e u r o s u r g e r y ; a n d i n f e c t i o
s u c h a s e n c e p h a l i t i s . T h e p a t i e n t ' s p s y c h i a t r i c h i s t o r y ma y a l s o b e r e l e v a n t .
P olydipsia usually starts abruptly in central D I, and patients often have a preferenc
f o r i c e c o l d w a t 3)
e r. (
A g o o d g e n e r a l p h y s i c a l e xa mi n a t i o n , i n c l u d i n g v i t a l s i g n s , i s h e l p f u l i n ma k i n g t h e
d i a g n o s i s , b u t t h e e mp h a s i s i s o n t h e n e u r o l o g i c e xa mi n a t i o n ( i . e . , v i s u a l f i e l d s ,
c r a n i a l n e r v e d e f i c i t s , o c u l o mo t o r p a l s i e s , a n d r e f l e xe s ) . S i g n s o f r e c e n t w e i g h t l o s
or the presence of peripheral neuropathy suggest the diagnosis of D M.
C. Testing
1. L a b o r a t o r y t e sAt su r i n a l y s i s n e e d s t o b e p e r f o r me d t o c h e c k f o r g l u c o s u r i a o f
D M o r t h e l o w s p e c i f i c g r a v i t y a s s o c i a t e d w i t h D I . A c h e mi s t r y p a n e l i s h e l p f u l i
checking for elevated serum glucose levels of D M or an elevated creatinine
seen with renal disease and nephrogenic D I. A calcium level could be useful if
h y p e r c a l c e mi a i s s u s p e c t e d . S e r u m a n d u r i n e o s mo l a l i t y a r e u s e f u l i n
d i f f e r e n t i a t i n g b e t w e e n D I , w h i c h p r e s e n t s w i t h i n c r e a s e d s e r u m o s mo l a l i t y a n d
a n i n a p p r o p r i a t e l y l o w u r i n e o s mo l a l i t y ( s p e c i f i c g r a v i t y < 1 . 0 0 5 ) , a n d
P. 3 2 9
e xc e s s i v e w a t e r i n t a k e , w h i c h p r e s e n t s w i t h l o w o r n o r ma l s e r u m o s mo l a l i t y a n d
a n a p p r o p r i a t e l y l o w u r i n e o s mo l a l i t y. N o r ma l s e r u m v a l u e s a r e b e t w e e n 2 8 5
a n d 2 9 5 mO s m/ L .
2. I m a g i n gM a g n e t i c r e s o n a n c e i ma g i n g ( M R I ) o f t h e h e a d ma y b e i n d i c a t e d t o
e xc l u d e p i t u i t a r y o r h y p o t h a l a mi c t u mo r s . I n D I a s s o c i a t e d w i t h p i t u i t a r y d i s e a s e
M R I i s q u i t e s p e c i f i c , b e c a u s e t h e n o r ma l b r i g h t s p o t o f a f u n c t i o n i n g p i t u i t a r y
g l a n d i s a b s e n3)t .(
3. Wa t e r d e p r i v a t i o n t eT shti s t e s t ma y b e u s e f u l i n t h e d i a g n o s i s o f D I a n d t o
d i f f e r e n t i a t e b e t w e e n n e u r o g e n i c a n d n e p h r o g e n i c D I b y d e t e r mi n i n g t h e e f f e c t s
o f w a t e r d e p r i v a t i o n ( mi l d d e h y d r a t i o n ) o n A D H s e c r e t i o n b y me a s u r i n g s e r u m,
u r i n e o s mo l a l i t y, u r i n e s p e c i f i c g r a v i t y, a n d s e r u m s o d i u m i n a c o n t r o l l e d
e n v i r o n me n 3)
t (. T h i s t e s t n e e d s t o b e c a r e f u l l y s u p e r v i s e d b y s o me o n e a b l e t o
t r e a t s e v e r e h y p e r t o n i c d e h y d r a t i o n , i f n e c e s s a r y. P a t i e n t s w i t h mi l d p o l y d i p s i a
a r e p l a c e d o n f l u i d r e s t r i c t i o n s t a r t i n g a t mi d n i g h t p r i o r t o t e s t i n g b u t i n t h o s e
w i t h s e v e r e p o l y d i p s i a f l u i d s a r e r e s t r i c t e d o n l y d u r i n g t h e d a y. B a s e l i n e b o d y
w e i g h t , p l a s ma o s mo l a r i t y, s e r u m s o d i u m, a n d u r i n e o s mo l a r i t y a r e d e t e r mi n e d .
U r i n e o s mo l a r i t y a n d w e i g h t a r e a s s e s s e d o n a n h o u r l y b a s i s . A d e q u a t e
540 / 652
tmdmss
14.5 - Polydipsia
d e h y d r a t i o n i s n o t e d b y a d e c r e a s e i n b o d y w e i g h t b y 5 % a n d s e r u m o s mo l a r i t y
> 2 7 5 mO s m/ L . A n o r ma l r e s p o n s e w o u l d s h o w n o r ma l p l a s ma o s mo l a r i t y a n d
sodium concentration with decreased urine output and increasing urine
o s mo l a r i t y t o > 8 0 0 mO s m/ L ( i . e . , t w o t o f o u r t i me s g r e a t e r t h a n t h e p l a s ma ) . I n
contrast to healthy patients, patients with D I cannot concentrate their urine in
r e s p o n s e t o d e h y d r a t i o n . P a t i e n t s w i t h c e n t r a l D I r e s p o n d t o d e s mo p r e s s i n ( a
s y n t h e t i c a n a l o g o f v a s o p r e s s i n ) a d mi n i s t e r e d i n t r a n a s a l l y, w h e r e a s p a t i e n t s
w i t h n e p h r o g e n i c D I d o 4)
n o. tP(a t i e n t s d o n o t f a l l i n t o d e f i n i t e c a t e g o r i e s
s o me t i me s ( e . g . , p a r t i a l c e n t r a l D I ) . T h e d i r e c t f o r m o f t e s t i n g w h e r e A D H l e v e l s
a r e me a s u r e d a f t e r i n f u s i n g h y p e r t o n i c s a l i n e i s r a r e l y p e r f o r me d .
D. Genetics
A n i n h e r i t e d a u t o s o ma l l y d o mi n a n t f o r m o f n e u r o g e n i c D I i s c a u s e d b y mu t a t i o n s i n
t h e AV P neur ophy s i n gIeI n e AV
( P - N P I Ig e n e ) , a n d h e r e d i t a r y n e p h r o g e n i c D I c a n
b e c a u s e d b y V 2 r e c e p t o r p r o b l e ms ( X - l i n k e d mo d e o f i n h e r i t a n c e o r a d e f e c t i n t h e
A D H - s e n s i t i v e a q u a p o r i n - 2 w a t e r c h a3)n .n e l s ) (
I V. Diagnosis
O f t e n , i mp o r t a n t c l u e s a b o u t t h e c a u s e o f p o l y d i p s i a c a n b e o b t a i n e d w i t h a d i r e c t e
c l i n i c a l h i s t o r y w i t h p a r t i c u l a r a t t e n t i o n t o t h e o n s e t o f s y mp t o ms , t h e p r e s e n c e o f
n o c t u r i a , a n d t h e me d i c a t i o n h i s t o r y. T h e v a l u e o f t h e p h y s i c a l e xa mi n a t i o n i s l i mi t e
u n l e s s t h e r e a r e s i g n s o f d e f e c t s d u e t o a p i t u i t a r y t u mo r ( e . g . , p r o g r e s s i v e
h e a d a c h e s , v i s u a l f i e l d d e f e c t s ) o r e n d o c r i n o l o g i c s y mp t o ms ( e . g . , a me n o r r h e a ,
g a l a c t o r r h e a , a c r o me g a l y, C u s h i n g ' s s y n d r o me ) . T h e d i a g n o s i s i s o f t e n ma d e w i t h
r o u t i n e l a b o r a t o r y t e s t s . S o me t i me s , a w a t e r d e p r i v a t i o n t e s t n e e d s t o b e p e r f o r me d
t o ma k e t h e d i a g n o s i s , b u t t h i s t e s t s h o u l d b e p e r f o r me d i n a h o s p i t a l s e t t i n g w i t h t
p a t i e n t mo n i t o r e d c l o s e l y f o r d e h y d r a t i o n .
T h i r s t a s s o c i a t e d w i t h p o l y u r i a i s t h e c h i e f c o mp l a i n t i n p a t i e n t s w i t h D M , D I , a n d
p s y c h o g e n i c p o l y d i p s i a . N o c t u r i a o c c u r s mo r e f r e q u e n t l y w i t h D M a n d D I t h a n w i t h
p s y c h o g e n i c p o l y d i p s i a . P a t i e n t s w i t h p s y c h o g e n i c p o l y d i p s i a ma y h a v e d e l u s i o n s
l e a d i n g t o i n c r e a s e d f l u i d i n t a k e o f u p t o 21)0 . L / d a y (
Acknowledgm ent
T h e a u t h o r t h a n k s L a r a O . F a k u n l e , M D , f o r h e r h e l p i n r e v i e w i n g t h i s c h a p t e r.
References
1. G reendyke R M , B ernhardt AJ , Tasbas H E , et al. P olydipsia in chronic
psychiatric patients: therapeutic trials of clonidine and enalapril.
N eur ops y c hophar mac ol ogy
1998;18:272281.
P. 3 3 0
2. O lapade- O laopa E O , M orley R N , Ahiaku E K , et al. Iatrogenic polydipsia: a
541 / 652
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14.5 - Polydipsia
r a r e c a u s e o f w a t e r i n t o xi c a t i o n i n uBr ro lJo gUy.r ol1 9 9 7 ; 7 9 : 4 8 8 .

3. Robertson G L . Disorders of the neurohypophysis. In: K asper D L , F auci AS ,
L o n g o D L , e dH
s .ar r i s on' s pr i nc i pl es of i nt er nal medi
, 1 6ctihnee d . N e w Yo r k :
McGraw-Hill, 1998:20982103.
4 . A d a m P. E v a l u a t i o n a n d ma n a g e me n t o f d i a b e t e s iAnm
s i pFi am
d u sP
. hy s i c i an
1997;55:21462153.
542 / 652
tmdmss
14.6 - Thyroid Enlargement, Goiter
> T a b l e o f C o n t e n t s > 1 4 - E n d o c r i n e a n d M e t a b o l i c P r o b l e ms > 1 4 . 6 - T h y r o i d
E n l a r g e me n t / G o i t e r
14.6
Thyroid Enlargement/Goiter
Je ri R. Re id
I . Background
G o i t e r, a n e n l a r g e d t h y r o i d g l a n d , i s t h e mo s t c o mmo n t h y r o i d a b n o r ma l i t y. G o i t e r i s
t e r me dendemi ci f i t o c c u r s i n mo r e t h a n 1 0 % o f a p o p u l a t i o n . E n d e mi c g o i t e r mo s t
c o mmo n l y r e s u l t s f r o m d i e t a r y i o d i n e d e f i c i e n c y a n d i s e xt r e me l y r a r e i n t h e U n i t e d
S t a t e s .S por adi cg o i t e r a r i s e s i n n o n e n d e mi c a r e a s a n d f r o m v a r i o u1)s . c a u s e s (
S i mpl eg o i t e r d e s c r i b e s a d i f f u s e l y e n l a r g e d t h y r oM
i dulgtlianodul
n d . arg o i t e r i s a n
e n l a r g e d g l a n d w i t h mu l t i p l e a r e a s o f n o d u l a r i t y. T h e f u n c t i o n a l s t a t u s o f a g o i t e r i s
u s u a l l y n o r manont
l ( ox i)c b u t c a n b e h y p o t h y r o i d o r h y p e r t thox
y r ioc)i .d T( h e me a n
weight of the thyroid gland in iodine-sufficient populations is 10 g, with the upper
l i mi t o f t h e n o r ma l b e i n g 2)
2 0. g (
A. Etiology
A n y p r o c e s s t h a t i mp e d e s t h y r o i d h o r mo n e s y n t h e s i s o r r e l e a s e c a n c a u s e g o i t e r. B
f a r t h e mo s t i mp o r t a n t r i s k f a c t o r f o r t h e d e v e l o p me n t o f g o i t e r i s i o d i n e d e f i c i e n c y.
G o i t r o g e n s , s u b s t a n c e s t h a t i n t e r f e r e w i t h t h y r o i d h o r mo n e p r o d u c t i o n a n d a c t i o n ,
c a n c a u s e s p o r a d i c g o i t e r. T h i s c a t e g o r y i n c l u d e s c e r t a i n d r u g s ( t h i o a mi d e
d e r i v a t i v e s , l i t h i u m, i o d i d e s , a mi o d a r o n e , a n d o t h e r s ) a n d f o o d s ( r u t a b a g a s ,
c a b b a g e , t u r n i p s , s o y b e a n s , k e l p , a n d 1)
o t. hCe ir gs a) (r e t t e s mo k i n g h a s b e e n l i n k e d
t o t h e d e v e l o p me n t o f g o i t e r i n i o d i n e - d e f i c i e n t a r e a s a n d i s b e l i e v e d t o i n t e r f e r e w
iodine uptake of the thyroid gland. Pregnancy-induced goiter is related to the
e xa c e r b a t i o n o f t h e e xi s t i n g i o d i n e d e f i c i e n c y a n d p r o l i f e r a t i v e e f f e c t o f e s t r o g e n o n
t h e t h y r o i d g l a n d . H o w e v e r, t h e u s e o f o r a l c o n t r a c e p t i v e s h a s b e e n f o u n d t o b e
a s s o c i a t e d w i t h r e d u c e d i n c i d e n c e o f 3)g.o i t e r (
B. Epidemiology
T h e p r e v a l e n c e o f g o i t e r i n t h e U n i t e d S t a t e s i s e s t i ma t e d a t 4 % t o 7 % b u t v a r i e s
w i d e l y, d e p e n d i n g o n t h e r e g i o n a l i o d i n e i n t a k e . O n e a u t o p s y s t u d y o f t h y r o i d g l a n d
t h a t w e r e t h o u g h t t o c o n t a i n n o p a t h o l o g y d e mo n s t r a t e d a 3 8 % i n c i d e n c e o f
mu l t i n o d u l a r g o i t2)
e r. (G o i t e r p r e v a l e n c e i n c r e a s e s w i t h a g e a n d i s 5 t o 1 0 t i me s
mo r e c o mmo n i n w o me n t h a n 1)
me. n (
543 / 652
tmdmss
I I I . Evaluation
A. History
A l t h o u g h s i mp l e g o i t e r s a r e u s u a l l y e u t h y r o i d , t y p i c a l s y mp t o ms o f h y p o t h y r o i d i s m o
t h y r o t o xi c o s i s s h o u l d b e s o u g h t . G e n e r a l i ze d t h y r o i d p a i n s u g g e s t s s u b a c u t e
t h y r o i d i t i s , w h e r e a s s u d d e n l o c a l i ze d p a i n a n d s w e l l i n g a r e c o n s i s t e n t w i t h
h e mo r r h a g e i n t o a n o d4)
u l .e A( f a mi l y h i s t o r y o f g o i t e r a n d a p e r s o n a l h i s t o r y o f
r e s i d i n g i n a n e n d e mi c g o i t e r a r e a o r i n g e s t i n g g o i t r o g e n s ma y b e s i g n i f i c a n t .
P h y s i c a l s i g n s c o n s i s t e n t w i t h h y p o t h y r o i d i s m o r t h y r o t o xi c o s i s ma y b e p r e s e n t .
P e mb e r t o n ' s s i g n c a n b e i n d u c e d b y h a v i n g t h e p a t i e n t r a i s e b o t h a r ms a b o v e t h e
h e a d f o r o n e mi n u t e . T h e p a t i e n t d e v e l o p s f a c i a l a n d n e c k p l e t h o r a i f v e n o u s o u t f l o
is obstructed by the thyroid gland. Inspect the neck below the thyroid cartilage from
t h e f r o n t , u s i n g c r o s s l i g h t i n g t o a c c e n t u a t e s h a d o w s a n d ma s s e s . F u l l e xt e n s i o n o f
the neck enhances the visibility of the gland. Inspection from the side with
me a s u r e me n t o f a n y p r o mi n e n c e o f t h e n o r ma l l y s mo o t h a n d
P. 3 3 1
straight contour between the cricoid cartilage and the suprasternal notch is useful.
P a l p i t a t i o n i s p e r f o r me d u s i n g t h e t e c h n i q u e w i t h w h i c h t h e e xa mi n e r i s mo s t
e xp e r i e n c e d a n d s k i l l e d . T h e t h y r o i d i s p a l p a t e d b y u s i n g t h e f i n g e r s o r t h u mb s w h i
standing in front of or behind the patient. If felt between the cricoid cartilage and t
s u p r a s t e r n a l n o t c h , t h e t h y r o i d i s t h mu s c a n b e u s e d t o h e l p l o c a t e t h e g l a n d .
P a l p a t i o n o f t h e l o b e s c a n b e i mp r o v e d b y r e l a xa t i o n o f t h e s t e r n o c l e i d o ma s t o i d ; f o
e xa mp l e , t h e l e f t l o b e c a n b e d e f i n e d b e t t e r b y h a v i n g t h e p a t i e n t s l i g h t l y f l e x a n d
r o t a t e t h e n e c k t o t h e l e f t . O t h e r u s e f u l ma n e u v e r s i n c l u d e me a s u r i n g t h e
c i r c u mf e r e n c e o f t h e n e c k o r t h e d i me n s i o n s o f e a c h l o b e . T h e l o c a t i o n , s i ze ,
c o n s i s t e n c y, mo b i l i t y, a n d t e n d e r n e s s o f a n y n o d u l e s s h o u l d b e n o t e d . H a v i n g t h e
p a t i e n t s w a l l o w d u r i n g b o t h i n s p e c t i o n a n d p a l p a t i o n c a u s e s t h e t h y r o i d t o mo v e a n
a i d s i n d e v e l o p i n g a t h r e e - d i me n s i o n a l i mp r e s s i o n o f t h e g l a n d ' s s h a p e a n d s i ze .
T h i s ma n e u v e r c a n a l s o ma k e a l o w - p l a c e d g l a n d a c c e s s i b l e . T h e p a t i e n t s h o u l d b e
p l a c e d i n t h e s u p i n e p o s i t i o n t o d e t e r mi n e t h e i n f e r i o r e xt e n t 2,5)
o f t. h e g l a n d (
C. Testing
1. L a b o r a t o r y t e s Tt sh e h i g h l y s e n s i t i v e t h y r o i d - s t i mu l a t i n g h o r mo n e ( T S H ) a s s a y
is the single best test to evaluate the thyroid status. An elevated T S H is highly
s u g g e s t i v e o f h y p o t h y r o i d i s m. I f T S H i s s u p p r e s s e d , a n e l e v a t e d f r e e t h y r o xi n e
i n d e x ( F T I ) o r f r e e t h y r o xi n4)e me
( f Ta s u r e d d i r e c t l y, c o n f i r ms t h y r o t o xi c o s i s . I n
a p a t i e n t w i t h a s u p p r e s s e d T S H a n d a n o r ma l 4F, Ts Ie rour mf T
t r i i o d o t h y r o n i n e3)( Ts h o u l d b e me a s u r e d t o a s s e s s f o r p o3s s i b l e T
t h y r o t o xi c o s i s . A n t i t h y r o i d a n t i b o d i e s a r e u s u a l l y l o w o r a b s e n t b u t t h e p r e s e n c e
of elevated levels can help predict those patients at a higher risk of developing
t h y r o i d i t i s , h y p o t h y r o i d i s m, o r G r a v e s ' d i s e a s e .
2. I m a g i n gN u c l e a r s c a n s a n d u l t r a s o u n d s t u d i e s a r e n o t w a r r a n t e d i n t h e r o u t i n e
e v a l u a t i o n o f s i mp l e o r mu l t i n o d u l a r g o i t e r. U l t r a s o n o g r a p h y ma y b e h e l p f u l i n
544 / 652
tmdmss
p a t i e n t s w i t h e q u i v o c a l f i n d i n g s o n p a l p a t i o n . S y mp t o ms s u g g e s t i v e o f
s u b s t e r n a l me c h a n i c a l p r e s s u r e r e q u i r e e v a l u a t i o n , u s u a l l y b y c o mp u t e d
t o mo g r a p h y o r ma g n e t i c r e s o n a n c e i ma
4) .g i n g (
3. O t h e r t e s t F
s i n e n e e d l e b i o p s y s h o u l d b e p e r f o r me d i n c a s e s o f a s o l i t a r y o r
d o mi n a n t n o d u l e f o u n d b y p a l p a t i o n . P u l mo n a r y f u n c t i o n t e s t s a r e w a r r a n t e d
w i t h e v i d e n c e o f i n s p i r a t o r y i mp a i r me n t . B a r i u m s w a l l o w i s i n d i c a t e d t o e v a l u a t e
g o i t e r - a s s o c i a t e d d y s p h a4)g .i a (
D. Genetics
C u r r e n t l y, n o g e n e t i c ma r k e r s a r e u s e d i n e v a l u a t i n g g o i t e r. F a mi l y a n d t w i n s t u d i e s
h a v e d e mo n s t r a t e d t h a t g e n e t i c f a c t o r s p l a y a r o l e i n t h e d e v e l o p me n t o f g o i t e r, b u t
a t p r e s e n t , t h e r e s u l t s c a n n o t b e e xt r a p o l a t e d t o t h e g e n e r a5)
l p. o p u l a t i o n (
I V. Diagnosis
A c y s t i c h y g r o ma o r a t h y r o g l o s s a l d u c t c y s t , w h i c h ma y t r a n s i l l u mi n a t e , a n d
l y mp h a d e n o p a t h y c a n b e c o n f u s e d w i t h a g o i t e r. P r i ma r y t h y r o i d c a n c e r s ,
l y mp h o ma s , o r me t a s t a t i c c a n c e r s ma y p r e s e n t a s a f i r m ma s s i n t h e n e c k . P a t i e n t s
w i t h t h y r o i d i t i s ( H a s h i mo t o ' s , s u b a c u t e , o r s i l e n t ) c a n p r e s e n t w i t h a n e n l a r g e d
t h y r o i d g l a n d . A n a s y mp t o ma t i c p a t i e n t w i t h a s i mp l e o r mu l t i n o d u l a r g o i t e r
a s s o c i a t e d w i t h a n o r ma l me t a b o l i c s t a t e d o e s n o t n e c e s s a r i l y r e q u i r e f u r t h e r
d i a g n o s t i c s t u d i e s o r t r e a t me n t . P e r i o d i c a s s e s s me n t , a t l e a s t a n n u a l l y, t o e v a l u a t e
g r o w t h , f u n c t i o n , a n d s y mp t o ms i s w a r r a n t e d . I n t w o s t u d i e s , i t w a s s h o w n t h a t 1 0 %
o f p a t i e n t s w i t h n o d u l a r g o i t e r d e v e l o p e d t h y r o t o xi c o s i s d u r i n g a 7 - t o 1 0 - y e a r f o l l o
u p p e r i o d1)( .
I n s i mp l e g o i t e r, p a t i e n t s a r e a s y mp t o ma t i c o r, i f t h e g l a n d i s s u f f i c i e n t l y e n l a r g e d ,
p r e s e n t w i t h s y mp t o ms c a u s e d b y me c h a n i c a l p r e s s u r e . S u b s t e r n a l g o i t e r s a r e
f r e q u e n t l y r e s p o n s i b l e f o r t r a c h e a l p r e s s u r e s y mp t o ms , i n c l u d i n g d y s p n e a a n d
i n s p i r a t o r y s t r i d o r. T h e y c a n a l s o o b s t r u c t t h e l a r g e c e r v i c a l v e i n s a t t h e t h o r a c i c
i n l e t , c a u s i n g s u f f u s i o n o f t h e f a c e , g i d d i n e s s , a n d s y n c o p e ( P e mb e r t o n ' s s i g n ) .
E s o p h a g e a l c o mp r e s s i o n c a n l e a d t o d y s p h a g i a . H o a r s e n e s s c a u s e d b y c o mp r e s s i o n
o f o r t r a c t i o n o n t h e r e c u r r e n t l a r y n g e a l n e r v e i s r a r e i n s i mp l e g o i t e r a n d s u g g e s t s
ma l i g n a n c y. G o i t e r w i t h c o mp r e s s i v e o r c o s me t i c s y mp t o ms u s u a l l y r e q u i r e s s u r g i c a
consultation but referral for radioiodine therapy could be considered especially in
o l d e r p a t i e n t s . T h e n a t u r a l h i s t o r y o f s i mp l e g o i t e r ma y
P. 3 3 2
i n c l u d e s p o n t a n e o u s r e s o l u t i o n o r n o c l i n i c a l c h a n g e b u t ma y a l s o i n v o l v e g r a d u a l
t h y r o i d g r o w t h , n o d u l e f o r ma t i o n , a n d t h e d e v e l o p me n t o f f u n c t i o n a l a u t o n o my. T h e
u s e o f t h y r o i d h o r mo n e s u p p r e s s i o n i n g o i t e r i s c o n t r o v e r s i a l . N o d u l e s w i t h i n a
mu l t i n o d u l a r g o i t e r ma y b e c o me t o xi c i n t h e p r e s e n c e o f a s u p p l e me n t a l t h y r o i d
h o r mo n e 4,5)
( .
References
545 / 652
tmdmss
1 . H e r mu s A R , H u y ma n s D A . P a t h o g e n e s i s o f n o n t o xi c d i f f u s e a n d n o d u l a r
g o i t e r. I n : B r a v e r ma n L E , U t i g e r R DWer
, e ner
d s . and I nbar ' s t he t hy, r9oit hd
e d . P h i l a d e l p h i a . PA : L i p p i n c o t t W i l l i a ms & W i l k i n s , 2 0 0 5 : 8 7 3 8 7 8 .
2 . D a y T A , C h u A , H o a n g K G . M u l t i n o d u l a rOgt ol
o i tar
e r.
y ngol C l i n N or t h A m
2003;36:3554.
3 . K n u d s e n N , L a u r b e r g P, P e r r i l d H , e t a l . R i s k f a c t o r s f o r g o i t e r a n d t h y r o i d
n o d u l e s T. hy r oi d2 0 0 2 ; 1 2 : 8 7 9 8 8 8 .
4 . H e r mu s A R , H u y ma n s D A . C l i n i c a l ma n i f e s t a t i o n s a n d t r e a t me n t o f n o n t o xi c
d i f f u s e a n d n o d u l a r g o i t e r. I n : B r a v e r ma n L E , U t i g eWer
r R Dner
, e dand
s.
I nbar ' s T he T hy r,oi9dt h e d . P h i l a d e l p h i a , PA : L i p p i n c o t t W i l l i a ms & W i l k i n s ,
2005:879885.
5 . H e g e d u s L , B o n n e ma S J , B e n n e d b a e k F N . M a n a g e me n t o f s i mp l e n o d u l a r
g o i t e r : c u r r e n t s t a t u s a n d f u t u r e p e r s pEe ndoc
c t i v e rs .R ev2 0 0 3 ; 2 4 : 1 0 2 1 3 2 .
546 / 652
tmdmss
14.7 - Thyroid Nodule
> T a b l e o f C o n t e n t s > 1 4 - E n d o c r i n e a n d M e t a b o l i c P r o b l e ms > 1 4 . 7 - T h y r o i d
Nodule
14.7
Thyroid Nodule
Je ri R. Re id
I . Background
A t h y r o i d n o d u l e i s a p a l p a b l e s w e l l i n g i n a n o t h e r w i s e n o r ma l t h y r o i d g l a n d . T h e
t y p e s o f t h y r o i d n o d u l e s a r e s u mma rTi ze
a bdl ei n1 4 . 7.. 1T h e mo s t c o mmo n n o d u l e
i s t h e c o l l o i d n o d u l e , w h i c h i s n o t a s s o c i a t e d w i t h a n i n c r e a s e d r i s k o f c a n c e r.
A. Etiology
E xp o s u r e t o i o n i zi n g r a d i a t i o n o r e xt e r n a l b e a m r a d i a t i o n t h e r a p y ( e s p e c i a l l y b e f o r e
2 0 y e a r s o f a g e ) i n c r e a s e s t h e i n c i d e n c e o f b o t h b e n i g n a n d ma l i g n a n t
P. 3 3 3
t h y r o i d n o d u l e s a t a r a t e o f 2 % a n n u a l l y a n d p e a k s 1 5 t o 2 0 y e a r s a1)
f t .e r e xp o s u r e (
N o d u l e s a r e o b s e r v e d i n 2 5 % o f p a t i e n t s w i t h G r a v e s ' d i s e a s e , a n d a p p r o xi ma t e l y
1 5 % o f t h e s e a r e ma l i g n2)
a n. tI o( d i n e d e f i c i e n c y r e s u l t s i n i n c r e a s e d t h y r o i d s t i mu l a t i n g h o r mo n e ( T S H ) l e v e l s , i n c r e a s e d t h y r o i d c e l l r e p l i c a t i o n , a n d a n
i n c r e a s e d i n c i d e n c e o f n o d2)
u l .e s (
TAB L E 14.7.1 types of T hyroid Nodules

Ade nom a
M a c r o f o l l i c u l a r a d e n o ma ( s i mp l e c o l l o i d )
M i c r o f o l l i c u l a r a d e n o ma ( f e t a l )
E mb r y o n a l a d e n o ma ( t r a b e c u l a r )
H c e l l a d e n o ma ( o xy p h i l i c , o n c o c y t i c )
A t y p i c a l a d e n o ma
A d e n o ma w i t h p a p i l l a e
S i g n e t - r i n g a d e n o ma
Carcinom a
P apillary (75%)
F ollicular (10%)
547 / 652
tmdmss
M edullary (5%10%)
Anaplastic (5%)
T h y r o i d l y mp h o ma ( 5 % )
Cy st
S i mp l e c y s t
C y s t i c / s o l i d t u mo r s ( h e mo r r h a g i c , n e c r o t i c )
Colloid nodule
D o mi n a n t n o d u l e i n a mu l t i n o d u l a r g o i t e r
Othe r
I n f l a mma t o r y d i s o r d e r s
Subacute thyroiditis
C h r o n i c l y mp h o c y t i c t h y r o i d i t i s
G r a n u l o ma t o u s d i s e a s e
D e v e l o p me n t a l a b n o r ma l i t i e s
D e r mo i d
Rare unilateral lobe agenesis
R e p r o d u c e d w i t h p e r mi s s i o n f r o m We l k e r M J , O r l o v D . T h y r oAi m
d nodules.
2003;67:55966
B. Epidemiology
Palpable nodules are present in 4% to 7% of adults. Nodules found incidentally on

d i a g n o s t i c i ma g i n g a r e e s t i ma t e d t o o c c u r a s o f t e n a s 1 9 % t o 6 7 % . A p p r o xi ma t e l y 5 %
o f a l l n o d u l e s a r e c a r c i n o ma s , r e g a r d l e s s o f h o w t h e y a r e 1)
d i. s B
c oevnei gr en d (
t h y r o i d n o d u l e s a r e p r e s e n t f o u r t o f i v e t i me s mo r e o f t e n i n w o me n b u t a r e mo r e
l i k e l y t o b e c a n c e r o u s i n 3)
me. nA g( e y o u n g e r t h a n 2 0 y e a r s o r o l d e r t h a n 6 5 y e a r s ,
ma l e g e n d e r, e xp o s u r e t o r a d i a t i o n , a n d p r e v i o u s h i s t o r y o f t h y r o i d c a n c e r a r e r i s k
f a c t o r s f o r t h y r o i d c a n c e r.
I I I . Evaluation
T he evaluation of nodular thyroid disease focuses on the functional status of the
g l a n d a n d d e t e c t i o n o f t h y r o i d c a n c e r. H y p o t h y r o i d i s m o r h y p e r t h y r o i d i s m ma y b e
s u g g e s t e d b y t h e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n .
A. History
R a p i d g r o w t h o f a n o d u l e o r s y mp t o ms o f l o c a l i n v a s i o n ( h o a r s e n e s s , n e c k p a i n ,
d y s p h a g i a , s t r i d o r, o r d y s p n e a ) i n c r e a s e s t h e s u s p i c i o n o f c a n c e r. S u d d e n o n s e t o f
l o c a l i ze d s w e l l i n g , p a i n , o r t e n d e r n e s s s u g g e s t s h e mo r r h a g e i n t o a p r e e xi s t i n g
nodule or cyst.
T he neck is inspected below the thyroid cartilage from the front and side, using
c r o s s l i g h t i n g t o a c c e n t u a t e s h a d o w s a n d ma s s e s . F u l l e xt e n s i o n o f t h e n e c k
enhances the visibility of the gland. T he patient is approached from either the front
o r b e h i n d d u r i n g p a l p a t i o n w h i c h i s a c c o mp l i s h e d u s i n g t h e f i n g e r s o r t h u mb s .
548 / 652
tmdmss
Having the patient swallow during both inspection and palpation causes the thyroid
t o mo v e a n d a i d s i n d e v e l o p i n g a t h r e e - d i me n s i o n a l i mp r e s s i o n o f t h e g l a n d . T h e
l o c a t i o n , s i ze , c o n s i s t e n c y, mo b i l i t y, a n d t e n d e r n e s s o f a l l n o d u l e s s h o u l d b e
d o c u me n t e d . A n o d u l e t h a t i s h a r d , i r r e g u l a r, n o n t e n d e r, > 4 c m, f i xe d t o s u r r o u n d i n g
s t r u c t u r e s o r t h a t i s a s s o c i a t e d w i t h l o c a l l y mp h a d e n o p a t h y s u g g e s t1)
s .ma l i g n a n c y (
C. Testing
1. L a b o r a t o r y t e s S
t se r u m T S H , p e r f o r me d b y a s e n s i t i v e me t h o d , s h o u l d b e
assessed in every patient. It is the best screening test for both hypothyroidism
( e l e v a t e d T S HC;h a p t e r 1 4). 4a n d t h y r o t o xi c o s i s ( s u p p r e s s e dCThSa pHt;e r
1 4 . 8) . A f a mi l y h i s t o r y o f me d u l l a r y t h y r o i d c a n c e r o r mu l t i p l e e n d o c r i n e
n e o p l a s i a t y p e I I w a r r a n t s a b a s a l s e r u m c a4)l c. i t o n i n (
2. F i n e n e e d l e b i o p Isny e u t h y r o i d p a t i e n t s w i t h a n o d u l e , a f i n e n e e d l e b i o p s y
( F N B ) s h o u l d b e p e r f o r me d . F N B h a s d e mo n s t r a t e d a s e n s i t i v i t y o f 6 8 % t o 9 8 %
a n d s p e c i f i c i t y o f 7 2 % t o 11)0 .0 %
T h( e u s e o f F N B h a s d e c r e a s e d t h e n u mb e r
of surgical procedures for thyroid nodules by 50% and increased the rate of
d e t e c t i n g c a r c i n o ma a f t e r s u r g e r y b4,5)
y 5.0 % (
3. I m a g i n gD i a g n o s t i c i ma g i n g c a n n o t r e l i a b l y d i f f e r e n t i a t e b e n i g n f r o m ma l i g n a n t
n o d u l e s ; h o w e v e r, i n c i d e n t a l l y d i s c o v e r e d n o d u l e s s ma l l e r t h a n 1 c m a r e u s u a l l y
n o t b i o p s i e d i f t h e r e a r e n o ma j o r r i s k f a c t o r s f o r c a n c e r. U l t r a s o n o g r a p h y c a n
be useful when findings on palpation are inconclusive regarding the presence of
a s i n g l e n o d u l e o r a d o mi n a n t o n e i n a mu l t i n o d u l a r g l a n d . I t c a n d i s t i n g u i s h
b e t w e e n s o l i d a n d c y s t i c l e s i o n s w i t h a c c u r a c y, e v a l u a t e n o d u l e s i ze , a n d
facilitate F N B. Ultrasound-guided F N B decreases the incidence of
i n d e t e r mi n a t e s p e c i me n s f r o m 1 5 % 1)t o. S
4%
o me
( authors have suggested
u l t r a s o u n d t o b e p e r f o r me d b y a n o p e r a t o r a n d t h a t c o u l d p r o c e e d w i t h a b i o p s y
i f i n d i c a t e d2) (. A n1 2 3I r a d i o n u c l i d e s c a n s h o u l d o n l y b e p e r f o r me d i n t h e i n i t i a l
e v a l u a t i o n o f t h y r o i d n o d u l e s i f t h e T S H l e v e l i n d i c a t e s h y p e r t h y r o i d i s m. A
hyperfunctioning nodule with suppressed uptake in the rest of the gland
s u g g e s t s a t o xi c n o d u l e a n d r e d u c e s t h e r i s k o f c a n c5)e.r Ht oo w< e1 v%e(r,
s u c h l e s i o n s c o n s t i t u t e < 1 0 % o f a l l n4)
o d. u l e s (
D. Genetics
M o s t a u t o n o mo u s l y f u n c t i o n i n g n o d u l e s a r e c a u s e d b y a mu t a t i o n i n t h e T S H
r e c e p t o r. O t h e r mu t a t i o n s t h a t ma y e xp l a i n t h e c a u s e s o f p a p i l l a r y a n d f o l l i c u l a r
c a r c i n o ma s h a v e b e e n i d e n t 2)
i f i.e A
d p(p r o xi ma t e l y 4 % t o 8 % o f c a s e s o f p a p i l l a r y
c a n c e r a r e f a mi l i a l , a n d a h i g h i n c i d e n c e h a s b e e n r e p o r t e d i n p a t i e n t s w i t h
a d e n o ma t o u s p o l y p o s i s c o l i ( G a r d n e r ' s s y n d r o me ) a n d mu l t i p l e h a ma r t o ma
P. 3 3 4
s y n d r o me ( C o w d e n d i s e a s e ) . M e d u l l a r y c a n c e r, u s u a l l y a s p a r t o f mu l t i p l e e n d o c r i n
n e o p l a s i a t y p e I I , o f t e n o c c u r s i n a h e r e d i t a r 3)
y .p a t t e r n (
I V. Diagnosis
549 / 652
tmdmss
E v e n i n e xp e r i e n c e d h a n d s , u p t o 1 5 % o f b i o p s i e s a r e u n s a t i s f a c t o r y a n d n e e d t o b e
r e p e a t e d , p r e f e r a b l y u s i n g u l t r a s o u n d g u5)i d. aInf ct eh e( r e s u l t s o f F N B
d e mo n s t r a t e a ma l i g n a n c y o r a r e s u s p i c i o u s , i n d e t e r mi n a t e , o r r e p e a t e d l y
u n s a t i s f a c t o r y, t h e n a n i mme d i a t e s u r g i c a l r e f e r r a l i s 3)w.a S
r roame
n t et di me
( s,
radionuclide scanning is used to evaluate a suspicious biopsy result, and
h y p e r f u n c t i o n i n g n o d u l e s a r e o b s e r v e d r a t h e r t h a n5)r .e mo
I f tvheed b(i o p s y i s
c l e a r l y b e n i g n , a n e xa mi n a t i o n o f t h e n e c k , a n u l t r a s o u n d , a n d a T S H me a s u r e me n t
s h o u l d b e p e r f o r me d e v e r y 6 t o 1 2 mo
4) .n t h s (
S u b a c u t e t h y r o i d i t i s i s s u g g e s t e d b y f e v e r, a p r e c e d i n g v i r a l i l l n e s s , o r a g r a d u a l
o n s e t o f s w e l l i n g , p a i n , a n d t e n d e r n e s s . T y p i c a l s y mp t o ms o f h y p o t h y r o i d i s m
s u g g e s t H a s h i mo t o ' s t h y r o i d i t i s , w h e r e a s t h y r o t o xi c o s i s s u g g e s t s t o xi c a d e n o ma o r
t o xi c mu l t i n o d u l a r g o 3)
i t e. r T( h e p r e s e n c e o f h y p e r t h y r o i d i s m o r h y p o t h y r o i d i s m
l o w e r s t h e s u s p i c i o n o f c a n c e r b u t d o e s n o t e xc l u d e i t . A n y n o d u l e t h a t e n l a r g e s o r
b e c o me s c l i n i c a l l y s u s p i c i o u s s h o u l d b e r e b i o p s i e d .
References
1 . We l k e r M J , O r l o v D . T h y r o i d n o dAuml eFs am
.
P hy s i c i an
2003;67:559566.
2 . We i s s R E , L a d o - A b e a l J . T h y r o i d n o d u l e s : d i a g n o s i s a nCdurt rh eOr pi
a pny.
O nc ol 2 0 0 2 ; 1 4 : 4 6 5 2 .
3 . K a p l a n M M . C l i n i c a l e v a l u a t i o n a n d ma n a g e me n t o f s o l i t a r y t h y r o i d n o d u l e s .
I n : B r a v e r ma n L E , U t i g e r R D ,Wer
e d sner
. and I nbar ' s T he T hy, r9oit hd e d .
P h i l a d e l p h i a , PA : L i p p i n c o t t W i l l i a ms & W i l k i n s , 2 0 0 5 : 9 9 6 1 0 1 0 .
4 . H e g e d u s L . T h e t h y r o i d n o dNu E
l engl
.
J M ed2 0 0 4 ; 3 5 1 : 1 7 6 4 1 7 7 1 .
5 . Wa l s h R M , Wa t k i n s o n J C , F r a n k l y n J . T h e ma n a g e me n t o f t h e s o l i t a r y t h y r o i d
n o d u l e : a r e v i eCw.l i n O t ol ar y ngol
1999;24:388397.
550 / 652
tmdmss
14.8 - Hyperthyroidism, Thyrotoxicosis
> T a b l e o f C o n t e n t s > 1 4 - E n d o c r i n e a n d M e t a b o l i c P r o b l e ms > 1 4 . 8 H y p e r t h y r o i d i s m/ T h y r o t o xi c o s i s
14.8
Hyperthyroidism/Thyrotoxicosis
Barbara A. Maje roni
I . Background
H y p e r t h y r o i d i s m i s e xc e s s i v e t h y r o i d h o r mo n e s e c r e t i o n . T h y r o t o xi c o s i s i s t h e
c l i n i c a l s y n d r o me o f i n c r e a s e d me t a b o l i s m c a u s e d b y e xc e s s t h y r o i d h o r mo n e
a c t i v i t y, r e g a r d l e s s o f t h e s o u r c e .
C a u s e s o f t h y r o t o xi c o s i s a r e s u mma rTi ze
a bdl ei n1 4 . 8.. 1
A. Etiology
1. G r a v e s ' d i s e a,s ae n a u t o i mmu n e d i s o r d e r a n d t h e mo s t c o mmo n c a u s e o f
h y p e r t h y r o i d i s m, r e s u l t s w h e n t h y r o t r o p i n r e c e p t o r a n t i b o d i e s s t i mu l a t e t h y r o i d
g r o w t h , l e a d i n g t o t h e s y n t h e s i s a n d r e l e a s e o f t h e t h y r o i d h o r mo n e .
2. To x i c a d e n o m a a n d t o x i c m u l t i n o d u l a r agr eo i ct eh ar r a c t e r i ze d b y t h e
d e v e l o p me n t o f a r e a s o f f o c a l o r d i f f u s e h y p e r p l a s i a o f t h y r o i d f o l l i c u l a r c e l l s ,
w h i c h f u n c t i o n w i t h o u t r e g u l a t i o n b y t h y r o i d - s t i mu l a t i n g h o r mo n e ( T S H ) . I n
t r o p h o b l a s t i c t u mo r s , T S H r e c e p t o r s ma y b e s t i mu l a t e d b y t h e o v e r p r o d u c t i o n o f
t h e h u ma n c h o r i o n i c g o n a d o t r o p i n ( H C G ) o r o t h e r p l a c e n t a l p r o t e i n s .
3. T h y r o i d i t i rse s u l t s i n t h e r e l e a s e o f p r e f o r me d t h y r o i d h o r mo n e f r o m t h e t h y r o i d
g l a n d . I t i s o f v i r a l o r p o s t v i r a l o r i g i n ( s u b a c u t e g r a n u l o ma t o u s t h y r o i d i t i s ) o r
f r o m a n a u t o i mmu n e p r o c e s s ( s u b a c u t e l y mp h o c y t i c t h y r o i d i t i s ) . S u b a c u t e
P. 3 3 5
t h y r o i d i t i s c a n a l s o b e c a u s e d b y c h e mi c a l t o xi c i t y f r o m d r u g s s u c h a s
a mi o d a r o n e , b y r a d i a t i o n , o r b y d r u g s t h a t i n t e r f e r e w i t h t h e i mmu n e s y s t e m,
such as interferon alfa.
TAB L E 14.8.1 Causes of T hyrotoxicosis and

Associated Radioactive I odine Uptake Findings
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Cause
Radioactiv e iodine uptake
G r a v e s ' d i s e a s e ( mo s t c o mmoH
n )o mo g e n e o u s i n c r e a s e
T o xi c mu l t i n o d u l a r g o i t e r
Increased heterogeneous pattern
T o xi c a d e n o ma
One area increased, rest of the gland

suppressed
E xo g e n o u s
Low or absent uptake
I a t r o g e n i c ( o v e r t r e a t me n t )
Factitious (patient taking
e xc e s s )
T hyroiditis
Acute suppurative (rare)
N o r ma l
S u b a c u t e ( d e Q u e r v aai n ' s ) L o w ( t r a n s i e n t )
S i l e n t t h y r o i dai t i s
Low (transient)
E xc e s s i o d i n e
Low or absent
T h y r o i d c a r c i n o ma
Va r i a b l e
F u n c t i o n i n g b o n e me t a s t a s e s A b s e n t
T S H - s e c r e t i n g p i t u i t a r y t u mo rI n c r e a s e d
S t r u ma o v a r i i
Reduced in thyroid (increased over

pelvis)
A c t i v a t i n g mu t a t i o n o f T S H
receptor
Increased
T h y r o i d h o r mo n e r e s i s t a n c e I n c r e a s e d
s y n d r o me
aT r a n s i e n t , f o l l o w e d b y h y p o t h y r o i d i s m, t h e n r e t u r n t o n o r ma l .
T S H , t h y r o i d - s t i mu l a t i n g h o r mo n e .
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B. Epidemiology
H y p e r t h y r o i d i s m a f f e c t s u p t o 2 % o f w o me n a n d 0 . 2 % o f me n . T h e p r e v a l e n c e
i n c r e a s e s w i t h a g e a n d i s h i g h e s t i n p a t i e n t s o l d e r t h a n 8 0 y1)e .a rGs r ao vf easg' e (
d i s e a s e a c c o u n t s f o r 6 0 % t o 8 0 % o f c a s e s o f t h y r o t o xi c o s i s , o c c u r s t y p i c a l l y i n
patients between 20 and 50 years of age, and is less prevalent in areas of low
i o d i n e i n t a k 2)
e .( T o xi c n o d u l a r g o i t e r i s t h e n e xt mo s t c o mmo n c a u s e o f
t h y r o t o xi c o s i s ( 1 0 % 4 0 % ) a n d i s t h e mo s t c o mmo n c a u s e o f t h y r o t o xi c o s i s i n
patients older than 40 years of age. T hyroiditis accounts for 5% to 20% of the case
T h e o t h e r c a u s e s o f t h y r o t o xi c o s i s a r e mu c h l e s s c o mmo n .
I I I . Evaluation
A. History
S y mp t o ms v a r y a n d a r e n o n s p e c i f i c . E l d e r l y p a t i e n t s ma y p r e s e n t w i t h f e w e r t y p i c a l
s y mp t o ms t h a n y o u n g e r p a t i e n t s . M o r e t h a n 5 0 % o f p a t i e n t s e xh i b i t s o me o f t h e
c o mmo n s y mp t o ms , w h i c h i n c l u d e n e r v o u s n e s s , i n c r e a s e d s w e a t i n g , h e a t
intolerance, palpitations, dyspnea, fatigue, weight loss, diarrhea, polyuria,
o l i g o me n o r r h e a , l o s s o f l i b i d o , a n d e y e c o mp l a i n t s .
Vi t a l s i g n s ma y r e v e a l w e i g h t l o s s , t a c h y c a r d i a , a n d s y s t o l i c h y p e r t e n s i o n w i t h a
w i d e n e d p u l s e p r e s s u r e . P a t i e n t s ma y a p p e a r n e r v o u s o r r e s t l e s s . S i n u s t a c h y c a r d i
i s t h e mo s t f r e q u e n t a r r h y t h mi a . A t r i a l f i b r i l l a t i o n o c c u r s i n 5 % t o 1 5 % a n d ma y b e
t h e p r e s e n t i n g p r o b l e m. T h e r i s k o f a t r i a l f i b r i l l a t i o n o r f l u t t e r i s h i g h e r i n me n t h a n
w o me n ( 1 . 8 1 ) a n d i n c r e a s e s w i t h3) .a gDee s( p i t e c o mp l a i n t s o f e xe r t i o n a l
d y s p n e a , t h e l u n g e xa mi n a t i o n i s u s u a l l y n o r ma l u n l e s s t h e r e i s c o n c o mi t a n t
c o n g e s t i v e h e a r t f a i l u r e . T h e s k i n ma y b e w a r m a n d mo i s t w i t h p a l ma r e r y t h e ma .
P r e t i b i a l my xe d e ma , p a i n l e s s r a i s e d s w e l l i n g o f s u b c u t a n e o u s t i s s u e s , mo s t o f t e n
found on the anterior lower leg or dorsal foot of patients with Graves' disease,
c a u s e s apeau d' or ange
t e xt u r e , w h i c h c a n b e p r u r i t i c a n d
P. 3 3 6
h y p e r p i g me n t e d . A f i n e t r e mo r i s mo s t e v i d e n t i n t h e f i n g e r t i p s w h e n t h e h a n d s a r e
e xt e n d e d . H y p o k a l e mi c p e r i o d i c p a r a l y s i s i s mo s t c o mmo n l y s e e n i n A s i a n me n . T h e
neck is inspected below the thyroid cartilage from the front and side. During
palpation, the patient is approached from behind using the fingers. Making the
p a t i e n t s w a l l o w d u r i n g t h e e xa mi n a t i o n c a u s e s t h e t h y r o i d t o mo v e a n d a i d s i n
d i s t i n g u i s h i n g i t f r o m e xc e s s f a t t y t i s s u e o r n e c k mu s c l e . A u s c u l t a t i o n o f a b r u i t o v e
t h e g l a n d c o r r e l a t e s w i t h i n c r e a s e d v a s c u l a r i t y.
C. Testing
M e a s u r i n g s e r u m t h y r o t r o p i n ( T S H ) i s t h e mo s t s e n s i t i v e t e s t f o r s c r e e n i n g f o r
h y p e r t h y r o i d i s m. A n o r ma l r e s u l t v i r t u a l l y e xc l u d e s h y p e r t h y r o i d i s m e xc e p t i n t h e r a
instances where it is due to thyrotropin hypersecretion. An undetectable level of
T S H i s t h e h a l l ma r k o f h y p e r t h y r o i d i s m, w h i c h i s c o n f i r me d b y a n e l e v a t e d f r e e
t h y r o xi n e (4T) . I f t h e f r e e4 Ti s n o r ma l a n d s u s p i c i o n i s h i g h , a s e r u m f r e e
t r i i o d o t h y r o n i n e3)( Ts h o u l d a l s o b e me a s u r e d t o r u l e3 toouxit cTo s i s . D r u g s s u c h
a s g l u c o c o r t i c o i d s , l e v o d o p a , a n d d o p a mi n e c a n c a u s e a l o w T S H i n p a t i e n t s w h o
553 / 652
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a r e e u t h y r o i d . A l o w T S H w i t h a n o r ma4l af rnede T3T ma y i n d i c a t e s u b c l i n i c a l

h y p e r t h y r o i d i s m. A t h y r o i d s c a n w i t h r a d i o a c t i v e i o d i n e u p t a k e c a n h e l p d i f f e r e n t i a t e
t h e c a u s e s o f t h y r o t o xi c o s i s a s i n d i cTaat be lde i 1n 4 . 8(4)
. 1 . U l t r a s o n i c e xa mi n a t i o n
of the thyroid is useful to detect the presence of nodules or cysts.
D. Genetics
A c o mb i n a t i o n o f g e n e t i c f a c t o r s , i n c l u d i n g H L A - D R a n d C T L A - 4 p o l y mo r p h i s ms ,
a n d e n v i r o n me n t a l f a c t o r s c o n t r i b u t e t o G r a v e s ' d i s e a s e . A c t i v a t i n g mu t a t i o n s o f G s
p r o t e i n s h a v e b e e n i d e n t i f i e d i n ma n y t o xi c a d e n o ma s . A c t i v a t i n g s o ma t i c mu t a t i o n
o f t h e g e n e s f o r T S H r e c e p t o r h a v e b e e n i d e n t i f i e d i n b o t h t o xi c a d e n o ma s a n d t o xi
mu l t i n o d u l a r g o i t e r s .
I V. Diagnosis
T h y r o t o xi c o s i s mu s t b e d i f f e r e n t i a t e d f r o m o t h e r c a u s e s o f u n e xp l a i n e d w e i g h t l o s s ,
i n c l u d i n g ma l i g n a n c i e s , p s y c h i a t r i c d i s o r d e r s , a l c o h o l o r d r u g a b u s e , d i a b e t e s , o c c u
infections, gastrointestinal disorders, and chronic renal, hepatic, cardiac, or
p u l mo n a r y d i s e a s e .
T h e s i g n s a n d s y mp t o ms a r e e xt r e me l y v a r i a b l e .
1. G r a v e s ' d i s e a si se s u g g e s t e d b y a c h a r a c t e r i s t i c s t a r e w i t h w i d e n e d p a l p e b r a l
f i s s u r e s , l i d l a g , a n d v i s u a l i za t i o n o f t h e s c l e r a o n a l l s i d e s o f t h e i r i s . T y p i c a l l y
t h e t h y r o i d i s i n c r e a s e d i n s i ze a n d i s s mo o t h a n d n o n t e n d e r. A b r u i t i s p r e s e n t
in 50% of patients.
2. To x i c n o d u l a r g o i tper er s e n t s w i t h a g l a n d t h a t i s t y p i c a l l y e n l a r g e d a n d
n o n t e n d e r w i t h mu l t i p l e n o d u l e s . A s i n g l e t o xi c n o d u l e i s mo r e c o mmo n i n
younger people.
3. T h y r o i d i t ,i sw h i c h u s u a l l y r e f e r s t o s u b a c u t e g r a n u l o ma t o u s t h y r o i d i t i s ( d e
Q uervain's thyroiditis), presents with an enlarged, tender thyroid. In subacute
l y mp h o c y t i c t h y r o i d i t i s ( p a i n l e s s o r s i l e n t t h y r o i d i t i s ) , t h e g l a n d i s e n l a r g e d b u t
n o n t e n d e r.
References
1 . F l y n n R W V, M a c D o n a l d T M , M o r r i s A D , e t a l . T h e t h y r o i d e p i d e mi o l o g y, a u d i t ,
a n d r e s e a r c h s t u d y : t h y r o i d d y s f u n c t i o n i n t h e g e n e r a l Jp oCpl ui nl a t i o n .
E ndoc r i nol M et ab
2004;89:38793884.
2 . C o o p e r D S . H y p e r t h y r o i dLi sanc
m. et2 0 0 3 ; 3 6 2 : 4 5 9 4 6 8 .
3 . F r o s t L , Ve s t e r g a a r d P, M o s e k i l d e L . H y p e r t h y r o i d i s m a n d t h e r i s k o f a t r i a l
f i b r i l l a t i o n o r f l u t t e r : a p o p u l a t i o n b a s eAdr csht uIdnty.er n M ed
2004;164:1675
554 / 652
tmdmss
1678.
4 . I n t e n zo C M , d e P a p p A E , J a b b o u r S , e t a l . S c i n t i g r a p h i c ma n i f e s t a t i o n s o f
t h y r o t o xi c o s iRs .adi ogr aphi c2s0 0 3 ; 2 3 : 8 5 7 8 6 9 .
555 / 652
tmdmss
15.1 - Lymphadenopathy, Generalized
> T a b l e o f C o n t e n t s > 1 5 - Va s c u l a r a n d L y mp h a t i c S y s t e m P r o b l e ms > 1 5 . 1 L y mp h a d e n o p a t h y, G e n e r a l i ze d
15.1
Lymphadenopathy, Generalized
Kristy D. Edw ards
I . Background
L y mp h a d e n o p a t h y, e n l a r g e d , t e n d e r, o r i n f l a me d l y mp h n o d e s , i s a c o mmo n
p r e s e n t i n g c o mp l a i n t . I n p r i ma r y c a r e , p a t i e n t s p r e s e n t i n g w i t h n o n s p e c i f i c
l y mp h a d e n o p a t h y a r e ma i n l y y o u n g e r t h a n 4 0 y e a r s o f a g e , a n d o n e - f o u r t h o f t h e
p a t i e n t s h a v e g e n e r a l i ze d l y mp h a d e n o p a t h y. G e n e r a l i ze d l y mp h a d e n o p a t h y i s
d i a g n o s e d w h e n a b n o r ma l l y mp h n o d e s a r e i d e n t i f i e d i n t w o o r mo r e n o n c o n t i g u o u s
a r e a s ( e . g . , n e c k a n d g r o i n ) . G e n e r a l i ze d l y mp h a d e n o p a t h y s h o u l d p r o mp t f u r t h e r
i n v e s t i g a t i o n o f s y s t e mi c d i s e a s e b y t h e p h y s i c i a n .
I n f e c t i o u s , a u t o i mmu n e , g r a n u l o ma t o u s , ma l i g n a n t d i s e a s e s , o r me d i c a t i o n r e a c t i o n s
c a n c a u s e g e n e r a l i ze d l y mp h a d e n o p a t h y. T h e o v e r a l l r i s k o f c a n c e r i n p a t i e n t s w i t h
g e n e r a l i ze d l y mp h a d e n o p a t h y i s l o w ; h o w e v e r, t h e r i s k o f ma l i g n a n c y i n c r e a s e s w i t h
age. T here is a 4% cancer risk in those patients older than 40 years of age with
g e n e r a l i ze d l y mp h a d e n o p a1)t h. yA ( g o o d c o mp l e t e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n
c a n o f t e n l e a d t o a d i a g n o s i s o f t h e c a u s e o f l y mp h a d e n o p a t h y.
I I I . Evaluation
A. History
T h e h i s t o r y s h o u l d f o c u s o n t h e c o mmo n c a u s e s o f g e n e r a l i ze d l y mp h - a d e n o p a t h y.
1. H i s t o r y o f p r e s e n t i l l nsehsosu l d f o c u s o n t h e d u r a t i o n , l o c a t i o n , q u a l i t y, a n d
c o n t e xt o f t h e l y mp h a d e n o p a t h y. E n l a r g e d , t e n d e r l y mp h n o d e s p r e s e n t f o r < 2
w e e k s a r e o f t e n d u e t o i n f e c t i o u s c a u s e s . L y mp h a d e n o p a t h y p r e s e n t f o r mo r e
t h a n a y e a r i s u s u a l l y f r o m n o n s p e c i f i c c a u s e s . A s s o c i a t e d s i g n s a n d s y mp t o ms
s u c h a s r a s h , f e v e r, n i g h t s w e a t s , w e i g h t l o s s , s o r e t h r o a t , a n d a r t h r a l g i a s ma y
h e l p i d e n t i f y a s p e c i f i c c a u s e o f t h e g e n e r a l i ze d l y mp h a2)
d e. n o p a t h y (
2. P a s t m e d i c a l h i s t osrhyo u l d f o c u s o n k n o w n i l l n e s s a n d me d i c a t i o n u s a g e .
C o mmo n c h r o n i c i l l n e s s e s ( e . g . , l u p u s e r y t h e ma t o s u s , r h e u ma t o i d a r t h r i t i s , a n d
h u ma n i mmu n o d e f i c i e n c y v i r u s [ H I V ] ) c a n a l s o c a u s e g e n e r a l i ze d
l y mp h a d e n o p a t h y. D r u g r e a c t i o n s r e s u l t i n g i n l y mp h a d e n o p a t h y c a n o c c u r w i t h
c e r t a i n a n t i b i o t i c s a n d s e i zu r e a n d h y p e r t e n s i o n me1)d.i c a t i o n s (
556 / 652
tmdmss
3. S o c i a l h i s t o rma
y y identify risk factors for hepatitis B, secondary syphilis, and
e a r l y H I V. A l l o f t h e s e d i s e a s e s c a n p r e s e n t w i t h g e n e r a l i ze d l y mp h a d e n o p a t h y.
4. F a m i l y h i s t o ri ys i mp o r t a n t t o i d e n t i f y i l l n e s s w i t h a g e n e t i c p r e d i s p o s i t i o n s u c h
a s l i p i d s t o r a g e d i s e a s e s a n d i mmu n o l o g i c d i s e a s e s ( e . g . , N i e ma n n - P i c k
d i s e a s e , r h e u ma t o i d a r t h r i t i s ) . A n y k n o w n e xp o s u r e s t o f a mi l y me mb e r s w i t h
i n f e c t i o u s d i s e a s e s ( e . g . , t u b e r c u l o s i s , i n f e c t i o u s mo n o n u c l e o s i s , o r h e p a t i t i s B
c a n a l s o y i e l d i mp o r t a n t i n f o r ma t i o n w h e n t r y i n g t o i d e n t i f y a c a u s a t i v e e t i o l o g y
o f l y mp h a d e n o p a t h y.
5. R e v i e w o f s y s t e ms hs o u l d f o c u s o n c o n s t i t u t i o n a l s y mp t o ms s u c h a s w e i g h t
l o s s , f a t i g u e , n i g h t s w e a t s , ma l a i s e , a r t h r a l g i a s , n a u s e a , a n d v o mi t i n g .
1. G e n e r a lA c o mp r e h e n s i v e p h y s i c a l e xa mi n a t i o n s h o u l d b e p e r f o r me d o n a l l
p a t i e n t s w i t h g e n e r a l i ze d l y mp h a d e n o p a t h y. I t s h o u l d f o c u s o n i d e n t i f y i n g
s y s t e mi c d i s e a s e s . Vi t a l s i g n s a r e i mp o r t a n t , b e c a u s e f e v e r ma y s u g g e s t
i n f e c t i o u s e t i o l o g y a n d w e i g h t l o s s ma y s u g g e s t s y s t e mi c d i s e a s e . S k i n r a s h e s
o r l e s i o n s , mu c o u s me mb r a n e u l c e r s , a n d i n f l a mma t o r y a r t h r i t i s a r e i mp o r t a n t
p h y s i c a l f i n d i n g s i n e s t a b l i s h i n g a d i f f e r e n t i a l d i a g n o s i s f o r t h e a d e n o p a t h y. A n
a b d o mi n a l e xa mi n a t i o n f o r s p l e n o me g a l y c a n y i e l d u s e f u l i n f o r ma t i o n . P a t i e n t s
w i t h g e n e r a l i ze d l y mp h a d e n o p a t h y a n d s p l e n o me g a l y i mp l i e s s y s t e mi c i l l n e s s
( e . g . , i n f e c t i o u s mo n o n u c l e o s i s , l y mp h o ma , l e u k e mi a , l u p u s , s a r c o i d o s i s ,
P. 3 4 0
t o xo p l a s mo s i s , o r c a t - s c r a t c h d i s e a s e ) a n d v i r t u a l l y e xc l u d e s n o n h e ma t o l o g i c
me t a s t a t i c d i s e a s2,1)
e (.
2. N o d a l e x a m i n a t i oI nn g e n e r a l i ze d l y mp h a d e n o p a t h y, l y mp h n o d e s i ze , l o c a t i o n ,
and consistency can help in establishing a diagnosis.
a. S i z e L y mp h n o d e s > 1 . 5 c m i n d i a me t e r h a v e a 3 8 % r i s k o f c a n c e r
i n v o l v e me n t a n d r e q u i r e f u r t h e r w2)
o r.k Lu ypmp
( h n o d e s 1 c m i n d i a me t e r
a n d s ma l l e r c a n b e n o r ma l a n d c a n u s u a l l y b e o b s e r v e d .
b. L o c a t i o nT h e a n a t o mi c l o c a t i o n o f l y mp h a d e n o p a t h y i s s o me t i me s h e l p f u l
i n e s t a b l i s h i n g a d i a g n o s i s . H o w e v e r, w i t h g e n e r a l i ze d l y mp h a d e n o p a t h y,
t h e a n a t o mi c l o c a t i o n i s l e s s h e l p f u l . A n t e r i o r c e r v i c a l , s u b ma n d i b u l a r, a n d
i n g u i n a l n o d e s ma y n o r ma l l y b e p a l p a b l e . H o w e v e r, l y mp h n o d e s p a l p a t e d
i n c e r t a i n a r e a s a r e a l w a y s a l a r mi n g . F o r e xa mp l e , s u p r a c l a v i c u l a r
l y mp h a d e n o p a t h y h a s a 9 0 % r i s k o f c a n c e r i n p a t i e n t s o l d e r t h a n 4 0 y e a r s
of age.
c. C o n s i s t e n c yR o c k h a r d n o d e s p a r t i c u l a r l y i n o l d e r p a t i e n t s a r e w o r r y i n g
f o r me t a s t a t i c d i s e a2)
s e. F( i r m r u b b e r y n o d e s a r e f o u n d w i t h l y mp h o ma s .
S o f t t e n d e r n o d e s t e n d t o o c c u r w i t h i n f e c t i o u s c a u s e s ; h o w e v e r, t h i s
should not be considered diagnostic. Pain is not a reliable indicator of the
c a u s e o f l y mp h a d e n o p a t h y.
557 / 652
tmdmss
C. Testing
1. L a b o r a t o r y t e s Lt sa b o r a t o r y t e s t i n g i n p a t i e n t s w i t h g e n e r a l i ze d l y mp h a d e n o pathy should be purposeful and specific. Tests should be directed by the

p a t i e n t ' s s i g n s a n d s y mp t o ms o f a n u n d e r l y i n g d i s e a s3)
e .pAr occoemp
s s l (e t e
b l o o d c o u n t ( C B C ) w i t h p e r i p h e r a l s me a r i s a l mo s t a l w a y 4)
s .i n d i c a t e d (
E l e v a t e d n e u t r o p h i l s s u g g e s t p y o g e n i c e t i o l o g i e s , l y mp h o c y t o s i s s u g g e s t s v i r a l
i n f e c t i o n , a n d p a n c y t o p e n i a s u g g e s t s l e u k e mi a o r H I V i n f e c t i o n . A n e r y t h r o c y t e
s e d i me n t a t i o n r a t e i s n o n s p e c i f i c , b u t i f i t i s p e r s i s t e n t l y e l e v a t e d , f u r t h e r
investigation is indicated. Disease-specific serologic tests, including antibody
t e s t i n g f o r E p s t e i n - B a r r v i r u s , c y t o me g a l o v i r u s , H I V, r u b e l l a v i r u s , s y p h i l i s
( F TA- AB S ), and others, are useful. Antibody testing can be diagnostic and can
d i f f e r e n t i a t e b e t w e e n a c u t e a n d c h r o n i c i l l n e s s . C h e s t x- r a y s a r e r a r e l y p o s i t i v e
b u t s h o u l d b e o r d e r e d t o l o o k f o r me d i a s t i n a l l y mp h n o d e i n v o l v e me n t i n s a r c o i d
d i s e a s e , me t a s t a t i c d i s e a s e , l y mp h o ma s , o r g r a n u l o ma t o u s d i s e a s e . P u r i f i e d
p r o t e i n d e r i v a t i v e ( P P D ) t e s t i n g i s u s e d t o i d e n t i f y t u b e r c u2)l i.n d i s e a s e (
2. L y m p h n o d e b i o p sI fy t h e l a b o r a t o r y t e s t i n g i s n o n d i a g n o s t i c , t h e n l y mp h n o d e
b i o p s y ma y b e i n d i c a t e d . T h e l a r g e s t a n d mo s t p a t h o l o g i c n o d e s h o u l d b e
r e mo v e d . A xi l l a r y a n d i n g u i n a l n o d e s s h o u l d b e a v o i d e d , b e c a u s e t h e y o f t e n
reveal only reactive hyperplasia. Biopsy should be avoided in cases of
s u s p e c t e d i n f e c t i o u s mo n o n u c l e o s i s a n d d r u g r e a c t i o n b e c a u s e t h e h i s t o l o g i c
p i c t u r e i s e a s i l y c o n f u s e d w i t h ma l i g n a n t l y 2)
mp. hEoxp
mae r(i e n c e d
h e ma t o l o g i s t s o r h e ma t o p a t h o l o g i s t s s h o u l d h a n d l e a l l s p e c i me n s . T h e v a l u e o f
f i n e n e e d l e a s p i r a t i o n i s c o n t r o v e r s i a l , w i t h r e a s o n a b l e a r g u me n t s b o t h f o r a n d
a g a i n s t t h i s p r o c e d u5)r e. (
I V. Diagnosis
T h e t h o r o u g h h i s t o r y a n d e xa mi n a t i o n s h o u l d e s t a b l i s h a d i f f e r e n t i a l d i a g n o s i s
i n c l u d i n g i n f e c t i o u s , a u t o i mmu n e , g r a n u l o ma t o u s , a n d ma l i g n a n t e t i o l o g i e s .
I n v e s t i g a t i o n s h o u l d b e l i mi t e d t o s p e c i f i c d i s e a s e s b e c a u s e g e n e r a l i ze d
l y mp h a d e n o p a t h y i s o f t e n a s i g n o f a s p e c i f i c s y s t e mi c i l l n e s s . I n t h e e v e n t t h a t t h e
c a u s e i s u n c l e a r, i n f e c t i o u s e t i o l o g i e s mu s t b e c o n s i d e r e d a n d a C B C a n d
mo n o n u c l e o s i s s p o t o r d e r e d . I f t h e s e a r e n e g a t i v e , t h e n i mmu n o l o g i c a n d
g r a n u l o ma t o u s e t i o l o g i e s a r e c o n s i d e r e d w i t h s e r o l o g i c t e s t i n g , a c h e s t x- r a y, a n d
P P D . A l y mp h n o d e b i o p s y mu s t b e c o n s i d e r e d i n t h o s e c a s e s w h e r e t h e n o d e i s
r o c k h a r d o r l a r g e r t h a n 1 . 5 c m 1 . 5 c m1)i n. Bs i ozep s(y s h o u l d b e a v o i d e d i n
those cases in which viral causes are clinically suggested.
References
1 . F e r r e r R . L y mp h a d e n o p a t h y : d i f f e r e n t i a l d i a g n o s i s a n d Ae m
v a lFuam
ation.
P hy s i c i an1 9 9 8 ; 5 8 : 1 3 1 3 1 3 2 0 .
2 . P a n g a l i s G A , Va s s i l a l o p o u l o s T P, B o u s s i o t i s VA , e t a l . C l i n i c a l a p p r o a c h t o
l y mp h a d e n o p a t hSy.emi n O nc ol1 9 9 3 ; 2 0 : 5 7 0 5 8 2 .
P. 3 4 1
558 / 652
tmdmss
3 . W i l l i a ms o n H A . L y mp h a d e n o p a t h y i n a f a mi l y Jp rFaam
c t i cP
e .r ac t
1985;20:449452.
4 . S i mo n H B . E v a l u a t i o n o f l y mp h a d e n o p a t h y. I n : G o r o l l A H , M a y L A , M u l l e y A G ,
e d s . P r i mar y c ar e medi c i ne: of f i c e ev al uat i on and management of t he adul t
pat i ent, 3 r d e d . P h i l a d e l p h i a , PA : J B L i p p i n c o t t , 1 9 9 5 : 5 4 5 8 .
5 . H e n r y P, L o n g o EDnl
. ar gement of l y mph nodes and s. pl
H ar
een
r i s on' s on
l i ne 1 9 9 9 ; 6 1 . w w w. h a r r i s o n s o n l i n e . c o m/
559 / 652
tmdmss
15.2 - Lymphadenopathy, Localized
> T a b l e o f C o n t e n t s > 1 5 - Va s c u l a r a n d L y mp h a t i c S y s t e m P r o b l e ms > 1 5 . 2 L y mp h a d e n o p a t h y, L o c a l i ze d
15.2
Lymphadenopathy, Localized
Kristy D. Edw ards
I . Background
L y mp h n o d e s a r e n o r ma l l y p a l p a t e d i n c h i l d r e n a n d ma y b e n o r ma l l y f e l t i n t h e n e c k
a xi l l a , a n d i n g u i n a l r e g i o n s o f a d u l t s . T h r e e - f o u r t h s o f p r i ma r y c a r e p a t i e n t s
p r e s e n t i n g w i t h u n e xp l a i n e d l y mp h n o d e e n l a r g e me n t h a v e r e g i o n a l
l y mp h a d e n o p a t h y. L o c a l i ze d o r r e g i o n a l l y mp h a d e n o p a t h y o c c u r s w h e n e n l a r g e d
l y mp h n o d e s a r e i d e n t i f i e d i n o n e a n a t o mi c l o c a t i o n . T h e mo s t c o mmo n a n a t o mi c
l o c a t i o n s f o r l o c a l i ze d l y mp h a d e n o p a t h y a r e t h e h e a d a n d n e c k ( 5 5 % ) a n d i n g u i n a l
( 1 4 % ) r e g i o n s . O n c e l y mp h a d e n o p a t h y i s i d e n t i f i e d , a c o mp l e t e l y mp h n o d e
e xa mi n a t i o n s h o u l d b e p e r f o r me d t o r u l e o u t g e n e r a l i ze d l y mp h a d e n o p a t h y. A
t h o r o u g h h i s t o r y a n d p h y s i c a l e xa mi n a t i o n i n c l u d i n g r e g i o n s d r a i n e d b y t h e l y mp h
n o d e s s h o u l d b e p e r f o r me
1) .d (
T h e c a u s e o f l o c a l i ze d l y mp h a d e n o p a t h y c a n b e s e p a r a t e d b y t h e a g e o f t h e p a t i e n t
a n d i t s l o c a t i o n . R e a c t i v e h y p e r p l a s i a a n d b e n i g n e t i o l o g i e s ma k e u p 8 0 % o f t h e
c a u s e s o f l y mp h a d e n o p a t h y i n c h i l d r e n a n d a d u l t s y o u n g e r t h a n 3 0 y e a r s o f a g e .
Older patients, especially those older than 40 years of age, are at increased risk of
ma l i g n a n c y2)(. I n l o c a l i ze d l y mp h a d e n o p a t h y, t h e l o c a t i o n c a n a i d i n d e t e r mi n i n g a
c a u s a t i v e e t i o l o g y. K n o w l e d g e o f t h e p a t t e r n s o f l y mp h a t i c d r a i n a g e a n d r e g i o n s p e c i f i c c o n d i t i o n s a r e e s s e n t i a l i n t h e i n v e s t i g a t i o n o f l o c a l i ze d l y mp h a d e n o p a t h y
(3) . T h e c o n t e xt i n w h i c h l y mp h a d e n o p a t h y o c c u r s i s v e r y i mp o r t a n t i n e s t a b l i s h i n g
differential diagnosis. A detailed history including present illness, review of system
p a s t me d i c a l h i s t o r y, s o c i a l h i s t o r y, a n d t h o r o u g h p h y s i c a l e xa mi n a t i o n o f t h e r e g i o n
s h o u l d b e p e r f o r me d .
I I I . Evaluation
A. History
I t i s i mp o r t a n t t o e l i c i t a d e t a i l e d h i s t o r y. L y mp h a d e n o p a t h y t h a t i s p r e s e n t f o r mo n t
t o y e a r s s u g g e s t s u n d e r l y i n g ma l i g n a n c y o r s y s t e mi c d i s e a s e , w h e r e a s
l y mp h a d e n o p a t h y t h a t i s p r e s e n t f o r a f e w w e e k s i s u s u a l l y d u e t o i n f e c t i o u s
e t i o l o g i e s . H i s t o r y o f e xp o s u r e t o a c a t s c r a t c h o r s e xu a l l y t r a n s mi t t e d d i s e a s e c a n
e xp l a i n l y mp h a d e n o p a t h y i n t h e a xi l l a o r i n g u i n a l r e g i o n s . H i s t o r y o f r e c e n t c o l d
560 / 652
tmdmss
s y mp t o ms o r l o c a l s i g n s o f r e d n e s s , s w e l l i n g , o r d i s c h a r g e ma y s u g g e s t i n f e c t i o n ,
w h e r e a s n o n s p e c i f i c s i g n s o f f e v e r, c h i l l s , o r n i g h t s w e a t s ma y s u g g e s t s y s t e mi c
illness.
T h e e xa mi n a t i o n o f l y mp h n o d e s s h o u l d i n c l u d e s i ze , l o c a t i o n , p a i n , c o n s i s t e n c y, a n
w h e t h e r ma t t i n g i s p r e s1)
e n. t (
1. S i z e A s w i t h g e n e r a l i ze d l y mp h a d e n o p a t h y, l y mp h n o d e s > 1 c m i n d i a me t e r
should arouse suspicion.
2. L o c a t i o nT h e l o c a t i o n o f t h e a b n o r ma l l y mp h n o d e h e l p s f o c u s t h e e xa mi n a t i o n .
A t h o r o u g h e xa mi n a t i o n o f t h e a n a t o mi c r e g i o n d r a i n e d b y t h e a f f e c t e d l y mp h
n o d e i s o f t h e h i g h e s t y i e l d f o r d i a g n o s i s . C e r v i c a l l y mp h n o d e s d r a i n t h e
o r o p h a r y n x, t o n g u e , a n d e a r s . C e r v i c a l l y mp h a d e n o p a t h y w i t h o u t a k n o w n
s o u r c e s h o u l d b e t r e a t e d wi t h a n t i b i o t i c c o vS
e rt aaphy
g e flooc
r oc c us aur eus
a n d g r o u p A h e mo l y t i c s t r e p t o c o c c i . H o w e v e r, b i l a t e r a l c e r v i c a l
l y mp h a d e n o p a t h y i s o f t e n c a u s e d b y v i r a l o r s t r e p t o c o c c a l 4)
p h. a r y n g i t i s (
L y mp h a d e n o p a t h y i n t h e n e c k p o s t e r i o r t o t h e s t e r n o c l e i d o ma s t o i d mu s c l e i s a
mo r e o mi n o u s f i n d i n g a n d
P. 3 4 2
w a r r a n t s f u r t h e r e v a l u a 3)
t i o. nP(a l p a t e d s u p r a c l a v i c u l a r l y mp h n o d e s ( S C L N s )
a r e a l s o w o r r i s o me . L e f t S C L N s d r a i n i n t r a - a b d o mi n a l r e g i o n s a n d r i g h t S C L N s
d r a i n t h e l u n g s , me d i a s t i n u m, a n d t h e e s o p h a g u s . I n g u i n a l l y mp h n o d e s
h e i g h t e n t h e c o n c e r n f o r v e n e r e a l d i s e a s e o r l o w e r e xt r e mi t y i n f e c t i o n . A xi l l a r y
l y mp h n o d e s s u g g e s t b r e a s t p a t h o l o g y o r u p p e r e xt r e mi t y i n f e c t i o n . O t h e r a r e a s
o f l y mp h a d e n o p a t h y, s u c h a s a b d o mi n a l o r me d i a s t i n a l l y mp h n o d e s , ma y n o t b e
p a l p a b l e b u t ma y b e i d e n t i f i e d w i t h r a d i o l o g i c s t u d i e s .
3. P a i n P a i n i s o f t e n a s s o c i a t e d w i t h l y mp h a d e n i t i s , t e n d e r, w a r m, s o f t , e n l a r g e d
l y mp h n o d e s . T h i s i s u s u a l l y a r e s u l t o f p y o g e n i c2)i n. f e c t i o n (
4. C o n s i s t e n c yC o n s i s t e n c y o f l y mp h n o d e s i s d i f f i c u l t t o a s s e s s . T r a d i t i o n a l l y,
f i r m a n d h a r d n o d e s a r e a s s o c i a t e d w i t h ma l i g n a n c y a n d r u b b e r y n o d e s s u g g e s t
l y mp h o ma . M a t t e d o r f i xe d n o d e s a r e p a r t i c u l a r l y w o r r i s o me f o r me t a s t a t i c
d i s e a s e 5)( .
C. Testing
L a b o r a t o r y t e s t i n g i s o f t e n p e r f o r me d t o u n c o v e r p a t h o l o g y i n v o l v i n g t h e r e g i o n o f
t h e b o d y d r a i n e d b y t h e a f f e c t e d l y mp h n o d e s . F o r e xa mp l e , a mo n o s p o t f o r E p s t e i n
B a r r v i r u s o r t h r o a t c u l t u r e f o r s t r e p t o c o c c a l p h a r y n g i t i s ma y b e o b t a i n e d i n a p a t i e
w i t h c e r v i c a l l y mp h a d e n o p a t h y. A ma mmo g r a m ma y b e o r d e r e d i n o l d e r f e ma l e s w i t h
a xi l l a r y l y mp h a d e n o p a t h y. S c r e e n i n g f o r s e xu a l l y t r a n s mi t t e d d i s e a s e s s h o u l d b e
p e r f o r me d i n p a t i e n t s w i t h p e r s i s t e n t i n g u i n a l l y mp h a d e n o p a t h y. I f t h e i n i t i a l
e v a l u a t i o n f o r l o c a l i ze d l y mp h a d e n o p a t h y d o e s n o t r e v e a l a d i a g n o s i s , i t i s u s u a l l y
acceptable to observe the patient for 2 to 4 weeks rather than perform unnecessary
t e s t s 6)
( . A n y t e s t i n g p e r f o r me d s h o u l d b e s p e c i f i c t o i d e n t i f y r e g i o n a l p a t h o l o g y. T h
t e s t o f c h o i c e i s a n e xc i s i o n a l b i o p s y o f t h e n o d e o r n o d e s i n v o l v e d w h e n t h e
561 / 652
tmdmss
d i a g n o s i s i s u n k n o w n a n d a s e r i o u s c o n d i t i o n i s s u s p e c t e d . U s u a l l y, t h e b i o p s y s i t e
i s d e t e r mi n e d b y l o c a t i o n a n d s i ze . L a r g e n o d e s t h a t h a v e r e c e n t l y e n l a r g e d a r e
p r e f e r r e d f o r b i o p5)
s y. (
I V. Diagnosis
L o c a l i ze d l y mp h a d e n o p a t h y i s a c o mmo n p r e s e n t i n g c o mp l a i n t i n c l i n i c a l p r a c t i c e .
U s u a l l y, a t h o r o u g h h i s t o r y a n d p h y s i c a l e xa mi n a t i o n l e a d t o a d i a g n o s i s . I f t h e
d i a g n o s i s i s n o t r e a d i l y i d e n t i f i a b l e , t h e n d i s e a s e - s p e c i f i c t e s t s ma y b e h e l p f u l .
B i o p s y i s a l a s t r e s o r t i f s e r i o u s d i s e a s e i s s u s p e c t e d . Wa t c h f u l w a i t i n g i s a c c e p t a b
and preferred as long as a serious condition is not suspected.
References
1 . F e r r e r R . L y mp h a d e n o p a t h y : d i f f e r e n t i a l d i a g n o s i s a n d Ae m
v a lFuam
ation.
P hy s i c i an1 9 9 8 ; 5 8 : 1 3 1 3 1 3 2 0 .
2 . S i mo n H B . E v a l u a t i o n o f l y mp h a d e n o p a t h y. I n : G o r o l l A H , M a y L A , M u l l e y A G ,
e d s . P r i mar y c ar e medi c i ne: of f i c e ev al uat i on and management of t he adul t
pat i ent, 3 r d e d . P h i l a d e l p h i a , PA : J B L i p p i n c o t t , 1 9 9 5 : 5 4 5 8 .
3 . S i l l s R , J o r g e n s e nLySmphadenopat
.
.hy
e M e d i c i n e f r o m We b M D . Av a i l a b l e
a t : h t t p : / / w w w. e me d i c i n e . c o m/ p e d / t o p i c 1 3 3 3 . h t m. L a s t u p d a t e d A p r i l 11 , 2 0 0 5 .
4 . L e u n g A K , R o b s o n W L . C h i l d h o o d c e r v i c a l l y mp h a d eJ nPoedi
p a t at
h y.r
H eal t h C ar 2e 0 0 4 ; 1 8 ( 1 ) : 3 7 .
5 . P a n g a l i s G A , Va s s i l a l o p o u l o s T P, B o u s s i o t i s VA , e t a l . C l i n i c a l a p p r o a c h t o
l y mp h a d e n o p a t hSy.emi n O nc ol1 9 9 3 ; 2 0 : 5 7 0 5 8 2 .
6 . W i l l i a ms o n H A . L y mp h a d e n o p a t h y i n a f a mi l y Jp rFaam
c t i cP
e .r ac t
1985;20:449452.
562 / 652
tmdmss
15.3 - Petechiae and Purpura
> T a b l e o f C o n t e n t s > 1 5 - Va s c u l a r a n d L y mp h a t i c S y s t e m P r o b l e ms > 1 5 . 3 Petechiae and Purpura
15.3
Petechiae and Purpura
John L. Sm ith
I . Background
Purpura are discolorations in the skin as a consequence of red blood cells
e xt r a v a s a t i n g i n t o t h e s k i n o r t h e mu c o u s me mb r a n e s . A p e t e c h i a e i s a p u r p u r a t h a t
i s < 2 mm i n d i a me t e r, a n d a n e c c h y mo s i s i s a p u r p u r a t h a t i s > 1 c m i n d i a me t e r.
P e t e c h i a e mo s t o f t e n r e s u l t f r o m a p l a t e l e t d i s o r d e r e i t h e r t o o f e w ( u s u a l l y
< 5 0 , 0 0 0 / L ) o r a b n o r ma l l y f u n c t i o n i n g p l a t e l e t s . L o c a l i ze d i n c r e a s e s i n i n t r a v a s c u l a
p r e s s u r e o r c a p i l l a r i t i s ma y a l s o b e r e s p o n s i b l e . E c c h y mo s e s a r e u s u a l l y d u e t o a
disorder in the coagulation cascade. Disorders of the vascular system as well as
c o n n e c t i v e t i s s u e d i s e a s e c a n a l s o o c c a s i o n a l l y r e s u l t 1)
i n. p u r p u r a (
I I I . Evaluation
A. History
1. A t i me s e q u e n c e a n d p a s t h i s t o r y o f p u r p u r a a s w e l l a s a n y i n d i c a t i o n s o f
a b n o r ma l b l e e d i n g a r e i mp o r t a n t , b e c a u s e t h e c a u s e o f p u r p u r a c a n b e e i t h e r
c o n g e n i t a l o r a c q u i r e d . A r e c e n t v i r a l o r b a c t e r i a l i n f e c t i o n ma y a f f e c t p l a t e l e t s
o r t h e v e s s e l i n t e g r i t y. E s t a b l i s h a h i s t o r y o f e a s y o r p r o l o n g e d b l e e d i n g o r
b r u i s i n g , o r me n o r r h a g i a i n w o me n . v o n W i l l e b r a n d ' s d i s e a s e i s t h e mo s t
c o mmo n i n h e r i t e d d i s o r d e r o f h e mo s t a s i s a n d o c c u r s i n u p t o 1 % o f t h e
p o p u l a t i o n . T h e d i s e a s e i s s y mp t o ma t i c i n u p t o 1 0 %2) o. fTtehne st oe 2( 0 % o f
e a r l y - o n s e t me n o r r h a g i a ma y b e a s s o c i a t e d w i t h a n i n h e r i t e d b l e e d i n g d i s o r d e r.
2. M e d i c a t i o n s ma y d e c r e a s e t h e p l a t e l e t p r o d u c t i o n o r i n c r e a s e t h e i r d e s t r u c t i o n .
A s s o c i a t e d me d i c a t i o n s a r e ma n y, b u t a c e t y l s a l i c y l i c a c i d , n o n s t e r o i d a l
a n t i i n f l a mma t o r y d r u g s , s u l f a , h e p a r i n , a n d , c l a s s i c a l l y, q u i n i d i n e a n d q u i n i n e
a r e f r e q u e n t l y n o t e d . A f a mi l y h i s t o r y o f i n h e r i t e d b l e e d i n g t e n d e n c i e s o r
i n d i c a t o r s o f l i v e r d i s e a s e ma y b e c l u e s t o a c o a g u l a t i o n d i s o r d e r.
I n i t i a l l y, d e t e r mi n i n g t h a t t h e p a t i e n t i s s t a b l e a n d c h e c k i n g v i t a l s i g n s i s i mp e r a t i v e
563 / 652
tmdmss
b e c a u s e l i f e - t h r e a t e n i n g c a u s e s o f p u r p u r a s u c h a s d i s s e mi n a t e d i n t r a v a s c u l a r
c o a g u l a t i o n ( D I C ) , R o c k y M o u n t a i n s p o t t e d f e v e r, me n i n g o c o c c e mi a , s e p s i s f r o m
S t aphy l oc oc c us aur,eus
a n d t h r o mb o t i c t h r o mb o c y t o p e n i c p u r p u r a ( T T P ) ma y b e
p r e s e n t . A t t e n t i o n s h o u l d t h e n b e d i r e c t e d a t t h e p u r p u r i c l e s i o n s t h e ms e l v e s a n d
a l s o a t t h e l o c a t i o n o f t h e l e s i o n s . P a l p a b l e p e t e c h i a e a r e s e e n w i t h v a r i o u s f o r ms
v a s c u l i t i s . P u r p u r a d o n o t b l a n c h l i k e i n t r a v a s c u l a r b l o o d s e e n i n a n g i o ma s ,
t e l a n g i e c t a s i a s , a n d h y p e r e mi a . P u r p u r a i s o l a t e d t o e y e l i d s ma y b e s e c o n d a r y t o
c o u g h i n g , v o mi t i n g , o r s t r a i n i n g a s i n c h i l d b i r t h o r w e i g h t l i f t i n g . P u r p u r a l i mi t e d t o
f o r e a r ms a r e mo s t l i k e l y s e c o n d a r y t o p o o r s t r o ma l s u p p o r t a s i s f r e q u e n t l y s e e n i n
s e n i l e p u r p u r a i n t h e o l d e r p o p u l a t i o n o r t h o s e w i t h mu c h p r e v i o u s s u n e xp o s u r e .
F a c i a l o r p e r i o r b i t a l p u r p u r a ma y b e s e c o n d a r y t o a c r y o g l o b u l i n e mi a , o r a my l o i d o s i
C. Testing
1. I n i t i a l l a b o r a t o r y t e s t s s h o u l d i n c l u d e a c o mp l e t e b l o o d c o u n t , p l a t e l e t c o u n t ,
p e r i p h e r a l s me a r, p r o t h r o mb i n t i me ( P T ) , a c t i v a t e d p a r t i a l t h r o mb o p l a s t i n t i me
( A P T T ) , a n d p o s s i b l y a b l e e d i n g t i me o r o t h e r e v a l u a t i o n o f p l a t e l e t f u n c t i o n
such as a P FA 100 analysis.
2. I f t h e l e s i o n s a r e p a l p a b l e , a n d v a s c u l i t i s i s a c o n s i d e r a t i o n , a s e d i me n t a t i o n
r a t e o r C - r e a c t i v e p r o t e i n d e t e r mi n a t i o n s h o u l d b e o b t a i n e d .
3. I n v a s c u l i t i s , a s k i n b i o p s y ma y n e e d t o b e o b t a i n e d b e c a u s e t h e l a b o r a t o r y
findings are often nonspecific.
4. U r i n a l y s i s a n d s e r u m c r e a t i n i n e s c r e e n mu s t b e p e r f o r me d f o r a n y r e n a l
i n v o l v e me n t a n d l i v e r f u n c t i o n t e s t s f o r l i v e r a b n o r ma l i t i e s .
P. 3 4 4
D. Genetics
T h e mu l t i t u d e o f d i s o r d e r s c a u s i n g p u r p u r a ma y b e a c q u i r e d o r c o n g e n i t a l .
Antibodies to factors in the coagulation cascade, as well as infectious diseases, an
me d i c a t i o n s a r e e xa mp l e s o f a c q u i r e d a b n o r ma l i t i e s . H e r e d i t a r y d i s o r d e r s a r e
p r e s e n t w i t h v o n W i l l e b r a n d ' s d i s e a s e a n d h e mo p h i l i a .
I V. Diagnosis
T h e c a u s e s o f p u r p u r i c l e s i o n s a r e n u me r o u s a n d h a v e c l i n i c a l i mp l i c a t i o n s , w h i c h
ma y p o t e n t i a l l y b e l e t h a l . A t h o r o u g h h i s t o r y a n d p h y s i c a l e xa mi n a t i o n a l o n g w i t h
s o me b a s i c l a b o r a t o r y s t u d i e s a n d o c c a s i o n a l s k i n b i o p s y a r e a l l t h a t a r e f r e q u e n t l y
needed to establish a likely diagnosis.
1. I n p a t i e n t s w i t h i s o l a t e d t h r o mb o c y t o p e n i a a n d p r o l o n g e d b l e e d i n g t i me ,
i d i o p a t h i c t h r o mb o c y t o p e n i c p u r p u r a i s t h e p r o b a b l e d i a g n o s i s a f t e r r u l i n g o u t
d r u g - i n d u c e d t h r o mb o c y t o p e n i a , h u ma n i mmu n o d e f i c i e n c y v i r u s i n f e c t i o n , a n d
p r e g n a n c y - i n d u c e d t h r o mb o c y t o p e n i a .
2. A n i s o l a t e d i n c r e a s e d A P T T i s s e e n i n d e f i c i e n c i e s o r i n h i b i t o r s o f f a c t o r s V I I I ,
564 / 652
tmdmss
I X , a n d X I . v o n W i l l e b r a n d ' s d i s e a s e ma y h a v e a n i n c r e a s e d b l e e d i n g t i me w i t h
a n i n c r e a s e d A P T3)
T . ( H e p a r i n a d mi n i s t r a t i o n i s i n c l u d e d i n t h e d i f f e r e n t i a l
diagnosis.
3. A n i s o l a t e d P T e l e v a t i o n i s s e e n i n l i v e r d i s e a s e , v i t a mi n K d e f i c i e n c y, w a r f a r i n
( C o u ma d i n ) a d mi n i s t r a t i o n , a n d a f a c t o r V I I d e f i c i e n c y o r i n h i b i t o r.
4. W h e n t h e P T a n d A P T T a r e b o t h e l e v a t e d , c o n s i d e r D I C a n d l i v e r f a i l u r e a l o n g
with the various deficiencies in the coagulation cascade.
5. I n n e w b o r n s w i t h p u r p u r a , e v a l u a t i o n f o r s e p s i s , s e r o l o g i e s f o r t h e T O R C H
( t o xo p l a s mo s i s , o t h e r i n f e c t i o n s , r u b e l l a , c y t o me g a l o v i r u s i n f e c t i o n , a n d h e r p e s
s i mp l e x s y n d r o me ) , a n d c o a g u l a t i o n f a c t o r s a r e r e c o mme n d e d . P u r p u r a
f u l mi n a n s a n d l e u k e mi a a r e a l s o i n c l u d e d i n t h e d i f f e r e n t i a4)l .d i a g n o s i s (
1. I n a d d i t i o n t o t h o s e ma n i f e s t a t i o n s n o t e d a b o v e , c e r t a i n c o n s t e l l a t i o n s o f c l i n i c a
a n d l a b o r a t o r y f i n d i n g s s h o u l d b e me n t i o n e d . T T P a n d h e mo l y t i c u r e mi c
s y n d r o me ( H U S ) a r e s e e n i n ma n y c l i n i c a l s i t u a t i o n s , i n c l u d i n g p r e g n a n c y,
c a n c e r, i n f e c t i o n s , a n d c h e mo t h e r a p y. T h e s i g n s i n c l u d e t h e p e n t a d o f f e v e r,
t h r o mb o c y t o p e n i a , mi c r o a n g i o p a t h i c h e mo l y t i c a n e mi a , h e mo r r h a g e ( i n c l u d i n g
p u r p u r a ) , a n d n e u r o l o g i c a b n o r ma l i t i e s . B e c a u s e o f s e r i o u s c o n s e q u e n c e s ,
d i a g n o s i s s h o u l d b e c o n s i d e r e d i f t h r o mb o c y t o p e n i a a n d f r a g me n t e d r e d b l o o d
c e l l s a r e s e e n o n t h e p e r i p h e r a l s me a r. T T P - H U S h a s a n o r ma l P T , A P T T , a n d
d - d i me r a s o p p o s e d t o D I C .
2. W i t h r e g a r d t o c o a g u l a t i o n f a c t o r a b n o r ma l i t i e s , h e mo p h i l i a A a n d B c a n c a u s e
i n c r e a s e d b r u i s i n g a n d e c c h y mo s e s b u t n o t n e a r l y a s f r e q u e n t l y a s v o n
W i l l e b r a n d ' s d i s e a s e . P a t i e n t s w i t h mi l d c a s e s o f v o n W i l l e b r a n d ' s d i s e a s e ma y
h a v e a n o r ma l b l e e d i n g t i me . B e c a u s e t h i s d i s e a s e i s c a u s e d b y a g l y c o p r o t e i n
that helps protect factor VIII from breakdown and interferes with platelet
a g g r e g a t i o n , t h e A P T T i s s o me t i me s e l e v a t e d . W i t h t h e s u d d e n o n s e t o f l a r g e
e c c h y mo s e s a n d h e ma t o ma s i n a n a d u l t w i t h n o r ma l p l a t e l e t s , a n a c q u i r e d f a c t o
VIII deficiency (autoantibody) should be investigated in cases of a prolonged P T
and AP T T.
3. Va s c u l i t i s c a u s i n g p a l p a b l e p u r p u r a i n c h i l d r e n i s mo s t c o mmo n w i t h H e n o c h Schnlein purpuranlein purpura.
References
1 . B u r n s T, B r e a t h n a c h S , C o x N , e t aR
l . ,ook
e d'ss. t ex t book of der mat ol
, ogy
7 t h e d . C h a p t e r 4 8 . O xf o r d : B l a c k w e l l P u b l i s h i n g , 2 0 0 4 .
2 . E w e n s t e i n B . v o n W i l l e b r a n d ' s d i sAennu
a s e .R ev M ed
1997;48:525542.
3 . L o s e r h J M . S c r e e n i n g a n d d i a g n o s i s o f c o a g u l a t i o n Ad m
i s oJr dOebs
r s .t et
G y nec ol1 9 9 6 ; 1 7 5 : 7 7 8 7 8 3 .
565 / 652
tmdmss
4 . B a s e l g a E , D r o l e t B A , E s t e r l y N B . P u r p u r a i n i n f a n t s a nJdAcmh i A
l dcrad
en.
D er mat ol1 9 9 7 ; 3 7 : 6 7 3 7 0 5 .
566 / 652
tmdmss
15.4 - Splenomegaly
> T a b l e o f C o n t e n t s > 1 5 - Va s c u l a r a n d L y mp h a t i c S y s t e m P r o b l e ms > 1 5 . 4 S p l e n o me g a l y
15.4
Splenomegaly
Kim be rly J. Jarzy nka
I . Background
A. Definition
S p l e n o me g a l y r e f e r s t o t h e e n l a r g e me n t o f t h e s p l e e n t o a c r a n i o c a u d a l
me a s u r e me n t o f 1 3 c m o r mo r e , o r t o a w e i g h t o f > 4 0 0 t o 5 0 0 g . W h e n t h e s p l e e n
r e a c h e s > 2 0 c m i n l e n g t h o r 1 , 0 0 0 g , i t i smas
t e rsme
i v ed s pl enomegal
(1,2,3,4)
y
.
B. Anatomy and physiology

N o r ma l l y, t h e s p l e e n i s a r e t i c u l o e n d o t h e l i a l o r g a n l o c a t e d i n t h e l e f t u p p e r q u a d r a n t
o f t h e a b d o me n a t t h e l e v e l o f t h e 8 t h t o 11 t h r i b s . I t l i e s a d j a c e n t t o t h e d i a p h r a g m,
s t o ma c h , s p l e n i c f l e xu r e o f t h e c o l o n , l e f t k i d n e y, a n d t a i l o f t h e p a n c r e a s . I t w e i g h s
a n a v e r a g e o f 1 5 0 g , me a s u r e s a p p r o xi ma t e l y 11 c m i n g r e a t e s t d i a me t e r, a n d i s
p a l p a b l e i n o n l y 2 % t o 5 % o f t h e p o p u1,2,3,4)
l a t i o n . (E s s e n t i a l f u n c t i o n s o f t h e
s p l e e n i n c l u d e t h e c l e a r a n c e o f s e n e s c e n t r e d b l o o d c e l l s , mi c r o - o r g a n i s ms , a n d
o t h e r p a r t i c u l a t e ma t t e r f r o m c i r c u l a t i o n ( r e d p u l p ) ; g e n e r a t i o n o f a c e l l u l a r a n d
h u mo r a l i mmu n e r e s p o n s e ( w h i t e p u l p ) ; e xt r a me d u l l a r y h e ma t o p o i e s i s ; a n d p l a t e l e t
s e q u e s t r a t i o 1,2,3,4)
n (
.
A. Etiology
T h e ma n y d i v e r s e c a u s e s o f s p l e n o me g a l y c a n b e g r o u p e d i n t o t h e f o l l o w i n g
c a t e g o r i e s : s t r u c t u r a l , i n f l a mma t o r y, h y p e r p l a s t i c , c o n g e s t i v e , i n f i l t r a t i v e , a n d
i n f e c t i o u sS. t r uc t ur al abnor mal i tci aes
u s i n g s p l e n o me g a l y i n c l u d e h e ma n g i o ma s ,
h a ma r t o ma s , c y s t s , a n d h e ma t oI nf
mal ammat
s.
or y s pl enomegal
i s cy a u s e d b y
r e t i c u l o e n d o t h e l i a l c e l l p r o l i f e r a t i o n a n d l y mp h o i d h y p e r p l a s i a f r o m a n i n c r e a s e i n t h e
a n t i g e n c l e a r a n c e a n d a n t i b o d y p r o dHu yc per
t i o npl
. as t i c s pl enomegal
, o t hy e r w i s e
k n o w n a swor k hy per t r ophy
, r e s u l t s f r o m t h e n o r ma l s e q u e s t r a t i o n o f i n c r e a s e d
a mo u n t s o f a b n o r ma l b l o o d c e l l s o r e xt r a me d u l l a r y h e ma t o p o i e s i s . I n c r e a s e d v e n o u s
p r e s s u r e r e s u l t sc onges
in
t i v e s pl enomegal
. M ya c r o p h a g e s c a n b e c o me e n g o r g e d
w i t h i n d i g e s t i b l e ma t e r i a l o r t u mo r, ci nf
a ui lstirnat
g i v e s pl enomegal
. A bys c e s s
f o r ma t i o n w i t h i n t h e s p l e e n , mo s t o f t e n d u e t o f i l t e r e d e n c a p s u l a t e d o r g a n i s ms ,
c a u s e si nf ec t i ous s pl enomegal
(3,4)y .
B. Epidemiology
T h e r e i s n o r a c e , a g e , o r s e x p r e d i l e c t i o n f o r s p l e n o me g a l y. E p i d e mi o l o g i c d a t a i s
d e p e n d e n t o n t h e p r i ma r y e t i o4)l o. g y (
I I I . Evaluation
A. History
A t h o r o u g h p a s t me d i c a l , f a mi l y, a n d s o c i a l h i s t o r y i n c l u d i n g a h i s t o r y o f r e c e n t t r a v e l
c a n o f t e n r e v e a l a p o s s i b l e c a u s e o f s p l e n o me g a l y. S p l e n o me g a l y i t s e l f i s o f t e n
a s y mp t o ma t i c . H o w e v e r, s y mp t o ms o f v a g u e , c o l i c k y l e f t u p p e r q u a d r a n t a b d o mi n a l
p a i n a n d f u l l n e s s , e a r l y s a t i e t y, a n d p a i n w h i l e l y i n g o n t h e s i d e ma y b e p r e s e n t
(3,4) . A c u t e p l e u r i t i c l e f t u p p e r q u a d r a n t p a i n c a n s u g g e s t p e r i s p l e n i t i s , s p l e n i c
a b s c e s s , o r i n f a r c t i4)
o n. O
( t h e r s y mp t o ms r e l a t e d t o t h e p r i ma r y i l l n e s s c a u s i n g
s p l e n o me g a l y ma y b e p r e s e n t . F o r e xa mp l e , a f e b r i l e i l l n e s s c a n s u g g e s t a n
i n f e c t i o u s c a u s e , w h e r e a s w e i g h t l o s s a n d c o n s t i t u t i o n a l s y mp t o ms ma y b e r e l a t e d t o
567 / 652
tmdmss
15.4 - Splenomegaly
a n e o p l a s t i c e t i o l o g y. A h i s t o r y o f c h r o n i c l i v e r d i s e a s e , a l c o h o l i s m, c o n g e s t i v e h e a r t
f a i l u r e , o r p a n c r e a t i t i s c a n b e a s s o c i a t e d w i t h c o n g e s t i v e s p l e n o me g a l y. H y p e r p l a s t i c
s p l e n o me g a l y i s o f t e n a s s o c i a t e d w i t h s y mp t o ms o f c y t o p e n i a i n c l u d i n g p a l l o r,
d y s p n e a , e a s y b r u i s i n g , o r p e t e c h i a4)l .r a s h (
1. E xa mi n a t i o n o f t h e s p l e e n i s p e r f o r me d w i t h t h e p a t i e n t s u p i n e , l y i n g a t a s l i g h t
incline, and/or in the right lateral decubitus position with the knees, hips, and
n e c k f l e xe d a n d t h e a r ms d o w n a t t h e s i d e s . F r o m t h e p a t i e n t ' s r i g h t s i d e , l i g h t l y
p a l p a t e u n d e r t h e l e f t c o s t a l ma r g i n w i t h t h e r i g h t h a n d w h i l e l i f t i n g t h e l e f t
costovertebral angle with the left hand. During deep inspiration, palpate gently
inward toward the descending spleen. It is often necessary to also palpate from
P. 3 4 6
P. 3 4 7
t h e l e f t l o w e r q u a d r a n t u p t o w a r d t h e c o s t a l ma r g i n , a s w e l l a s i n t h e mi d l i n e , t o
i d e n t i f y t h e l o w e r p o l e a n d me d i a l b o r d e r o f a s e v e r e l y e n l a r g e d s p l e e n
(1,2,5,6) .
TAB L E 15.4.1 Differential Diagnosis of Splenom egaly

Structural and
infe ctious
H e ma n g i o ma s ,
h a ma r t o ma s ,
cysts,
h e ma t o ma s
abscesses
I nflam m atory
Hy pe rplastic
Conge stiv e
I nfiltrativ e
Vi r a l h e p a t i t i s ,
S i c k l e c e l l a n e mi a , H e p a t i c , p o r t a l , H o d g k i n ' s d i s e a s e ,
infectious
t h a l a s s e mi a ma j o r, a n d s p l e n i c v e i n l y mp h o ma s , l e u k e mi a s ,
mo n o n u c l e o s i s ,
ovalocytosis,
obstruction;
my e l o p r o l i f e r a t i v e
c y t o me g a l o v i r u s , s p h e r o c y t o s i s , o t h e rc o n g e s t i v e
s y n d r o me s ,
A I D S , t u b e r c u l o s i s ,h e mo g l o b i n o p a t h i e s ,h e a r t f a i l u r e ,
a n g i o s a r c o ma s ,
s u b a c u t e b a c t e r i a l p a r o xy s ma l
cirrhosis,
me t a s t a t i c t u mo r s ,
endocarditis,
nocturnal
splenic artery
h y p e r l i p i d e mi a s ,
bacterial
h e mo g l o b i n u r i a , a n da n e u r y s m,
a my l o i d o s i s , G a u c h e r ' s
s e p t i c e mi a ,
n u t r i t i o n a l a n e mi a s . h e p a t i c
d i s e a s e , N i e ma n n - P i c k
c o n g e n i t a l s y p h i l i s ,E xt r a me d u l l a r y
s c h i s t o s o mi a s i s , d i s e a s e , T a n g i e r
ma l a r i a ,
h e ma t o p o i e s i s c a n p o r t a l
disease, histiocytosis X,
h i s t o p l a s mo s i s ,
be seen in
h y p e r t e n s i o n o f mu c o p o l y s a c c h a r i d o s e s ,
l e i s h ma n i a s i s ,
l e u k e mi a s ,
any etiology
eosinophilic
t r y p a n o s o mi a s i s , my e l o f i b r o s i s ,
g r a n u l o ma s . B e r y l l i o s i s
ehrlichiosis.
G aucher's disease,
R h e u ma t o i d
ma r r o w i n f i l t r a t i o n
arthritis (F elty's
b y t u mo r s , a n d
s y n d r o me ) , l u p u s , ma r r o w d a ma g e
other collagen
vascular diseases,
a u t o i mmu n e
h e mo l y t i c a n e mi a s ,
t h r o mb o c y t o p e n i a s ,
and neutropenias,
serum sickness,
drug reactions,
sarcoidosis,
a n g i o i mmu n o b l a s t i c
l y mp h a d e n o p a t h y,
t h y r o t o xi c o s i s , a n d
interleukin-2
t h e r a p y.
A I D S , a c q u i r e d i mmu n o d e f i c i e n c y s y n d r o me .
2. T h e a b i l i t y t o p a l p a t e t h e s p l e e n u s u a l l y s u g g e s t s s p l e n o me g a l y, a l t h o u g h 2 % t o
568 / 652
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15.4 - Splenomegaly
5 % o f n o r ma l s p l e e n s a r e p a l p1,2,3,4)
able ( .
3. D u l l n e s s t o p e r c u s s i o n i n t h e l o w e s t i n t e r c o s t a l s p a c e i n t h e a n t e r i o r a xi l l a r y l i n e
o n f u l l i n s p i r a t i o n a l s o s u g g e s t s s p l e n o me g a l y ( C a s t e l1)
l ' s. F
me
i ntdhiondg)s( o f
s p l e n o me g a l y o n p h y s i c a l e xa mi n a t i o n a r e r e c o r d e d i n c e n t i me t e r s b e l o w t h e l e f t
c o s t a l ma r g i n a t a p o i n t s p e c i f i e d b y t h e e xa mi n e r ( i . e . i n t h e mi d c l a v i c u l a r l i n e ) .
C. Testing(1,2,4)
1. C o mp l e t e b l o o d c o u n t w i t h d i f f e r e n t i a l a n d p l a t e l e t c o u n t
2. P e r i p h e r a l s me a r
3. L i v e r f u n c t i o n t e s t s
4. U r i n a l y s i s
5. H u ma n i mmu n o d e f i c i e n c y v i r u s t e s t i n g ( s h o u l d b e c o n s i d e r e d )
6. A p p r o p r i a t e t e s t s f o r p r i ma r y d i s e a s e
7. T i s s u e p a t h o l o g y
D. I maging studies
(1,2,4)
1. U l t r a s o u n d f o r i n i t i a l e v a l u a t i o n
2. C o mp u t e d t o mo g r a p h y ( C T ) f o r p r e o p e r a t i v e e v a l u a t i o n . C T i s b e t t e r f o r
d e t e c t i n g l e s i o n s , ma s s e s , o r i n f l a mma t i o n
3. N u c l e a r s c a n s f o r d e t e r mi n i n g s i ze a n d f u n c t i o n . T h e s e s c a n s a r e u s e d t o d e t e c t
a space-occupying lesion and accessory spleen
4. S p l e n o p o r t o g r a p h y f o r d e t e r mi n i n g p o r t a l v e i n p a t e n c y a n d c o l l a t e r a l c i r c u l a t i o n
5. A n g i o g r a p h y f o r d e t e c t i n g s p l e n i c t u mo r s a n d c i r c u l a t i o n a b n o r ma l i t i e s
E. Genetics
S p l e n o me g a l y i t s e l f h a s n o g e n e t i c l i n k . H o w e v e r, ma n y o f t h e p r i ma r y d i s e a s e s t h a t
c a u s e s p l e n o me g a l y d o ( e . g . , h e r e d i t a r y s p h e r o1,2,4)
c y t o .s i s ) (
I V. Diagnosis
T h e d i f f e r e n t i a l d i a g n o s i s o f s p l e n o me g a l y i s p r eTsaebnl et e 1d 5i .n4.. 1
H y p e r s p l e n i s m i s a n i n c r e a s e i n n o r ma l s p l e n i c f u n c t i o n a s s o c i a t e d w i t h
s p l e n o me g a l y. I t r e s u l t s i n v a r y i n g d e g r e e s o f a n e mi a , l e u c o p e n i a , a n d / o r
t h r o mb o c y t o p e n i a o w i n g t o t h e i n c r e a s e d d e s t r u c t i o n a n d s e q u e s t r a t i o n o f c e l l s . T h e
b o n e ma r r o w i s e i t h e r n o r ma l o r h y p e r p l a s t i c , a n d i mp r o v e me n t i n c e l l c o u n t s i s s e e n
p o s t s p l e n e c t o my
1,2,3,4)
(
. O t h e r c l i n i c a l ma n i f e s t a t i o n s d e p e n d o n t h e p r i ma r y
e t i o l o g y.
References
1 . K a s p e r D L , B r a u n w a l d E , F a u c i A S , e t aHl .ar
, reidsson'
. s pr i nc i pl es of
i nt er nal medi c ,i ne
1 6 t h e d . Av a i l a b l e a t :
h t t p : / / w w w. a c c e s s me d i c i n e . c o m/ r e s o u r c e T O C . a s p x? r e s o u r c e I D = 4 . M c G r a w - H i l l ' s
Access M edicine, 20042005.
2 . L a n d a w S A . A p p r o a c h t o t h e p a t i e n t w i t h s p l e n o me g a l y. U p T o D a t e O n l i n e
1 3 . 2 . M a y 2 , 2 0 0 5 . Av a i l a b l e a t : h t t p : / / w w w. u t d o l . c o m/ u t d / c o n t e n t / t o p i c . d o ?
t o p i c K e y = r e d _ c e l l / 3 0 0 4 2 & t y p e = A & s e l e c t e d T i t l e = 1 ~ 11 6 .
3 . Wa y LW, D o h e r t y GCMur
. r ent s ur gi c al di agnos i s and t r eat ment
, 11
onl
t hi ne
ed. S an F rancisco: C alifornia M edical Association, 20042005.
569 / 652
tmdmss
15.4 - Splenomegaly
4 . K a p l a n L J , C o f f ma nSDpl. enomegal. ye M e d i c i n e f r o m We b M D . Av a i l a b l e a t :
h t t p : / / w w w. e me d i c i n e . c o m/ me d / t o p i c 2 1 5 6 . h t m. L a s t u p d a t e d : O c t o b e r 5 , 2 0 0 4 .
5 . B r o t t mi l l e r W G , e t a l . , M
e dos
s .by ' s gui de t o phy s i c al ex ami,nat
2 nidone d . S t
L o u i s , M O : M o s b y Ye a r B o o k , I n c , 1 9 9 1 .
6 . Ya n g J C , R i c k ma n L S , B o s s e r S K . T h e c l i n i c a l d i a g n o s i s o f s p l e n o me g a l y.
Wes t J M ed1 9 9 1 ; 1 5 5 : 4 7 5 2 .
570 / 652
tmdmss
16.1 - Anemia
> T a b l e o f C o n t e n t s > 1 6 - L a b o r a t o r y A b n o r ma l i t i e s : H e ma t o l o g y a n d U r i n e
D e t e r mi n a t i o n s > 1 6 . 1 - A n e mi a
16.1
Anemia
Katrina Carte r
I . Background
A n e mi a , s i mp l y p u t , i s t o o f e w r e d b l o o d c e l l s ( R B C s ) . I n me n , i t i s u s u a l l y d e f i n e d
a s h e mo g l o b i n o f < 1 3 . 5 g / d L o r a h e ma t o c r i t o f < 4 1 . 0 % , a n d i n w o me n , a s
h e mo g l o b i n < 1 2 . 0 g / d L o r a h e ma t o c r i t o f < 3 6 . 0 % . I t i s i mp o r t a n t t o r e me mb e r t h a t
a n e mi a i s o n l y a s y mp t o m o f a d i s e a s e , n o t t h e d i s e a s e i t s e l f . W h e n e v e r a n e mi a i s
f o u n d , t h e c a u s e mu s t b e s o u g h t .
T h e c a u s e s f o r a n e mi a c a n b e b r o k e n d o w n i n t o t h r e e ma i n c a t e g o r i e s :
A. Decreased production
A n e mi a r e s u l t s w h e n t h e r a t e o f R B C p r o d u c t i o n i s l e s s t h a n t h e r a t e o f R B C
d e s t r u c t i o n . D e c r e a s e d p r o d u c t i o n ma y b e d u e t o ma n y c a u s e s , i n c l u d i n g l a c k o f
n u t r i e n t s . D e c r e a s e d i n t a k e o r ma l a b s o r p t i o n o f n u t r i e n t s s u c h a s 1i r2o, n , v i t a mi n B
o r f o l a t e ma y c a u s e d e c r e a s e d R B C p r o d u c t i o n . A b o n e ma r r o w d i s o r d e r s u c h a s
a p l a s t i c a n e mi a , my e l o d y s p l a s i a , o r t u mo r i n f i l t r a t i o n ma y d e c r e a s e R B C p r o d u c t i o n
a s w e l l . P a t i e n t s w h o a r e u n d e r g o i n g c h e mo t h e r a p y o r r a d i a t i o n ma y h a v e b o n e
ma r r o w s u p p r e s s i o n .
B. I ncreased RBC destruction
T h e n o r ma l R B C l i f e s p a n i s 1 2 0 d a y s ; c e r t a i n s i t u a t i o n s ma y d e c r e a s e t h e s u r v i v a l
o f R B C s . H e mo l y t i c a n e mi a i s t h e r e s u l t o f i n c r e a s e d R B C d e s t r u c t i o n . T h e r e a r e
h e r e d i t a r y c a u s e s o f h e mo l y t i c a n e mi a , i n c l u d i n g h e r e d i t a r y s p h e r o c y t o s i s , s i c k l e c e l l a n e mi a , a n d t h a l a s s e mi a . A c q u i r e d h e mo l y t i c a n e mi a s i n c l u d e a u t o i mmu n e ,
t h r o mb o t i c t h r o mb o c y t o p e n i c p u r p u r a a n d h e mo l y t i c u r e mi c s y n d r o me .
C. Blood loss
B l o o d l o s s i s b y f a r t h e mo s t c o mmo n c a u s e o f a n e mi a . S o me t i me s t h e s o u r c e o f
b l e e d i n g ma y b e o b v i o u s , s u c h a s i n a t r a u ma , o r i t ma y b e o c c u l t s u c h a s i n a
g a s t r o i n t e s t i n a l b l e e d . I n w o me n , me n s t r u a l b l e e d i n g s h o u l d a l w a y s b e c o n s i d e r e d .
I I I . Evaluation
571 / 652
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A. History
A t h o r o u g h h i s t o r y s h o u l d i n c l u d e q u e s t i o n s r e g a r d i n g s y mp t o ms s u c h a s f a t i g u e ,
l i g h t - h e a d e d n e s s , f e v e r, w e i g h t l o s s , a n d n i g h t s w e a t s . A g y n e c o l o g i c h i s t o r y s h o u l
b e t a k e n i n w o me n . P a t i e n t s s h o u l d b e a s k e d w h e t h e r t h e y h a v e e v e r b e e n a n e mi c
b e f o r e o r h a v e a f a mi l y h i s t o r y o f a n e mi a o r b l e e d i n g d i s o r d e r s . A n u t r i t i o n a l h i s t o r y
s h o u l d a l s o b e o b t a i n e d t o e v a l u a t e f o r p o s s i b l e ma l n u t r i t i o n ; t h i s i s e s p e c i a l l y
i mp o r t a n t i n e l d e r l y a n d a l c o h o l i c p a t i e n t s . C o n c o mi t a n t c o n d i t i o n s t h a t ma y a l s o
c o n t r i b u t e t o t h e d e v e l o p me n t o f a n e mi a i n c l u d e r e n a l f a i l u r e , c a n c e r t r e a t me n t , a n d
i mmu n o s u p p r e s s i o n . A t h o r o u g h me d i c a t i o n h i s t o r y s h o u l d a l s o b e t a k e n ; a n y h e r b a l
s u p p l e me n t s t a k e n b y t h e p a t i e n t s h o u l d b e n o t e d .
P e r t i n e n t f i n d i n g s i n c l u d e p a l l o r o f t h e s k i n a n d c o n j u n c t i v a e . E xa mi n a t i o n o f t h e
p a t i e n t i n n a t u r a l l i g h t ma y r e v e a l j a u n d i c e . P e t e c h i a e ma y i n d i c a t e a p l a t e l e t
a b n o r ma l i t y a s w e l l . C a r d i o v a s c u l a r e xa mi n a t i o n ma y r e v e a l a s y s t o l i c f l o w mu r mu r,
t a c h y c a r d i a , o r s t r o n g p e r i p h e r a l p u l s e s . A n a b d o mi n a l e xa mi n a t i o n ma y b e
s i g n i f i c a n t f o r s p l e n o me g a l y, w h i c h ma y s u g g e s t a l y mp h o p r o l i f e r a t i v e d i s o r d e r.
C. Testing
F i n d i n g t h e e t i o l o g y o f a n e mi a r e q u i r e s a f e w b a s i c l a b o r a t o r y v a l u e s . F i r s t , a
c o mp l e t e b l o o d c o u n t ( C B C ) i s e s s e n t i a l . T h i s n o t o n l y g i v e s t h e v a l u e o f
h e mo g l o b i n a n d h e ma t o c r i t b u t a l s o g i v e s t h e w h i t e b l o o d c e l l c o u n t a n d p l a t e l e t
c o u n t . T h e s e a r e i mp o r t a n t i n t h e e v a l u a t i o n o f p a n c y t o p e n i a . T h e C B C s h o u l d a l s o
i n c l u d e t h e me a n c o r p u s c u l a r v o l u me ( M C V ) , w h i c h i n d i c a t e s t h e a v e r a g e s i ze o f t h
R B C s a n d h e l p s w i t h d i a g n o s i s . A r e t i c u l o c y t e c o u n t i s a l s o h e l p f u l i n d e t e r mi n i n g
w h e t h e r t h e b o n e ma r r o w i s r e s p o n d i n g a p p r o p r i a t e l y t o t h e l e v e l o f a n e mi a .
A d d i t i o n a l t e s t i n g ma y i n c l u d e i r o n s t u d i e s : f e r r i t i n , t o t a l i r o n - b i n d i n g
P. 3 5 2
c a p a c i t y ( T I B C ) , a n d p e r c e n t i r o n s a t u r a t i o n . Vi t a mi n d e f i c i e n c i e s ma y b e e v a l u a t e d
b y v i t a mi n 1B2 a n d f o l a t e l e v e l s . I f a h e r e d i t a r y d i s o r d e r i s s u s p e c t e d , h e mo g l o b i n
e l e c t r o p h o r e s i s ma y b e r e q u i r e d . A p e r i p h e r a l b l o o d s me a r i s a l s o h e l p f u l i n ma n y
cases.
TAB L E 16.1.1 Etiology of Anem ia Based on Mean

Corpuscular Volum e
Me an Corpuscular
Volum e (f L )
<80
P ossible etiology Iron deficiency

T h a l a s s e mi a
Myelodysplastic
s y n d r o me
Sideroblastic
a n e mi a
80100
>100
Acute
Vi t a mi n 1B2
h e mo r r h a g e
deficiency
Chronic renal
Folate
insufficiency
deficiency
Chronic disease Sickle-cell
Iron deficiency disease
572 / 652
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16.1 - Anemia
Chronic disease (early)
Reticulocytosis
Liver disease
Endocrine
dysfunction
Alcohol abuse
I V. Diagnosis
A.
T h e s i mp l e s t w a y t o d e t e r mi n e t h e d i a g n o s i s i n a n e mi a i s t o f o l l o w a t h r e e - s t e p
approach.
P. 3 5 3
F I G U R E 1 6 . 1 .M
1 a c r o c y t i c a n e mi a .
573 / 652
tmdmss
16.1 - Anemia
F I G U R E 1 6 . 1 .M
2 i c r o c y t i c a n e mi a .
1. S t e p o n eC a t e g o r i ze t h e a n e mi a a s mi c r o c y t i c , n o r mo c y t i c , o r ma c r o c y t i c o n t h e
b a s i s o f t h e M C V. STeaeb l e 1 6 . 1.. 1
2. S t e p t w oD e t e r mi n e w h e t h e r p a n c y t o p e n i a i s p r e s e n t . I f t h e r e i s a l s o a
d e c r e a s e i n t h e n u mb e r o f w h i t e b l o o d c e l l s a n d p l a t e l e t s , t h i s i n d i c a t e s a
d e p r e s s i o n o f a l l c e l l l i n e s p r o d u c e d b y b o n e ma r r o w. I f p a n c y t o p e n i a i s f o u n d ,
b o n e ma r r o w e xa mi n a t i o n i s a l mo s t a l w a y s n e c e s s a r y.
3. S t e p t h r e eD e t e r mi n e a c a u s e f o r t h e a n e mi a b y e v a l u a t i n g t h e r e t i c u l o c y t e
c o u n t . T h i s v a l u e h e l p s d e t e r mi n e w h e t h e r t h e b o n e ma r r o w r e s p o n s e t o t h e
a n e mi a i s a p p r o p r i a t e .
B. Cause of anemia
1. M i c r o c y t i c a n e m i (F
a si g u r e 1 6 . 1). 2
a. A n e m i a o f c h r o n i c d i s e D
a seec r e a s e d i r o n a n d d e c r e a s e d T I B C ;
increased ferritin
b. S i d e r o b l a s t i c a n e mI ni ac r e a s e d i r o n a n d n o r ma l T I B C ; i n c r e a s e d f e r r i t i n .
P e r i p h e r a l s me a r s h o w s b a s o p h i l i c s t i p p l i n g a n d r i n g e d s i d e r o b l a s t s .
c. I r o n d e f i c i e n c y a n e m
D ieac r e a s e d i r o n a n d i n c r e a s e d T I B C . F e r r i t i n < 1 2
g / L i s v e r y s u g g e s t i v e o f i r o n d e f i c i e n c y.
d. T h a l a s s e m i Ve
a r y l o w M C V ( u s u a l l y < 7 0 f L ) ; n o r ma l i r o n s t u d i e s . T h e
p e r i p h e r a l s me a r ma y r e v e a l b a s o p h i l i c s t i p p l i n g . H e mo g l o b i n
electrophoresis is needed for diagnosis.
2. N o r m o c y t i c a n e m i a s
a. H e m o r r h a g eL o o k f o r s o u r c e o f b l o o d l o s s , p e r f o r m a H e mo c c u l t ' s s t o o l
574 / 652
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16.1 - Anemia
b. G l u c o s e - 6 - p h o s p h a t e d e f i c i e n c y
c. A u t o i m m u n e h e m o l y t i c a n ePmoisai t i v e C o o mb s
d. M e m b r a n o p a t h i eHse r e d i t a r y s p h e r o c y t o s i s w i t h s p l e n o me g a l y o n p h y s i c a l
e xa mi n a t i o n
P. 3 5 4
3. M a c r o c y t i c a n e m i (F
a si g u r e 1 6 . 1). 1
a. V i t a m i n 1B2 o r f o l a t e d e f i c i e nLcoyw s e r u m v i t a mi 1n2Ba n d f o l a t e
l e v e l s . T h e p e r i p h e r a l s me a r r e v e a l s h y p e r s e g me n t e d n e u t r o p h i l s . Vi t a mi n
B 1 2 d e f i c i e n c y ma y a l s o h a v e n e u r o l o g i c f i n d i n g s .
b. L i v e r d i s e a sEe l e v a t e d l i v e r f u n c t i o n t e s t s , a s p a r t a t e a mi n o t r a n s f e r a s e ,
a n d a l a n i n e a mi n o t r a n s f e r a s e . T h e p e r i p h e r a l s me a r ma y r e v e a l t a r g e t a n d
spur cells.
Suggested Reading
F a r l e y P. I r o n d e f i c i e n c y a n e mi a : h o w t o d i a g n o s e a nAdnemi
c o r rae c t .
1990;87:89101.
S c h r i e r S L . A p p r o a c h t o t h e a d u l t p a t i e n t w i t h a n e mi a U p t o D a t e P a t i e n t
I n f o r ma t i o n . Av a i l a b l e a t : h t t p : / / p a t i e n t s . u p d a t e . c o m/ t o p i c . a s p ?
f i l e = r e d _ c e l l / 2 9 5 0 & t i t l e = A n e mi a . L a s t u p d a t e d J u n e 2 0 0 6 .
B r a u n w a l d E , H a r r i s o n T R , A r mi t a g e J OP, r ienc
t ai pl
l . es of i nt er nal medi, c i ne
15th ed.349352.
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16.2 - Eosinophilia
D e t e r mi n a t i o n s > 1 6 . 2 - E o s i n o p h i l i a
16.2
Eosinophilia
Tina M . F lore s
I . Background
E o s i n o p h i l i a , a n a c c u mu l a t i o n o f e o s i n o p h i l s i n t h e p e r i p h e r a l b l o o d a t l e v e l s g r e a t
t h a n t h e n o r ma l r a n g e , c a n o c c u r i n a mu l t i t u d e o f d i s e a s e s . E o s i n o p h i l i a c a n b e
c l a s s i f i e d a s mi l d ( 3 5 1 1 , 5 0 0 c e3l)l ,s /mo
mmd e r a t e ( 1 , 5 0 1 5 , 0 0 0 c e l3l )s,/ mm
or
s e v e r e ( g r e a t e r t h a n 5 , 0 0 0 c e3l l)s(1)
/ mm
. T h e r e i s l i t t l e c o n c e r n a b o u t mi l d
eosinophilia if an identifiable cause is found and the eosinophilia resolves with
t r e a t me n t . M o d e r a t e t o s e v e r e e o s i n o p h i l i a s h o u l d b e e v a l u a t e d c a r e f u l l y, b e c a u s e
c a n l e a d t o e o s i n o p h i l i c i n f i l t r a t i o n o f t h e e y e s , h e a r t , l i v e r, l u n g s , g a s t r o i n t e s t i n a l
t r a c t , o r c e n t r a l n e r v o u s s y s t e m, r e s u l t i n g i n p e r ma n e n t e n d - o r2)
g a. n d a ma g e (
A. Etiology
T h e c a u s e s o f e o s i n o p h i l i a c a n b e b r o k e n d o w n i n t o t w o c a t e g o r i e s : p r i ma r y a n d
s e c o n d a r y. P r i ma r y c a u s e s i n c l u d e c l o n a l d i s o r d e r s ( my e l o i d c a n c e r s ) a n d t h e
i d i o p a t h i c h y p e r e o s i n o p h i l i c s y n d r o me . S e c o n d a r y c a u s e s i n c l u d e p a r a s i t i c
i n f e c t i o n s , a l l e r g i c d i s e a s e s , d r u g r e a c t i o n s , a u t o i mmu n e d i s o r d e r s , n o n my e l o i d
c a n c e r s , a n d a d r e n a l i n s u f f i c i3)
e n. c y (
B. Epidemiology
I n t h e U n i t e s S t a t e s , a l l e r g i c d i s e a s e s c o n t r i b u t e t o mo s t c a s e s o f e o s i n o p h i l i a . I n
d e v e l o p i n g c o u n t r i e s , t h e mo s t c o mmo n c a u s e i s s t i l l p1)a.r a s i t o s i s (
I I I . Evaluation
A. History
A n y p a t i e n t w i t h e o s i n o p h i l i a w a r r a n t s a t h o r o u g h t r a v e l , me d i c a t i o n , a n d f a mi l y
h i s t o r i e s , a n d a r e v i e w o f s y s t e ms .
A c o mp l e t e p h y s i c a l e xa mi n a t i o n s h o u l d a l s o b e p e r f o r me d .
576 / 652
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16.2 - Eosinophilia
C. Testing
A c o mp l e t e b l o o d c o u n t w i t h d i f f e r e n t i a l s h o u l d b e p e r f o r me d t o d e t e r mi n e t h e d e g r e
of eosinophilia. All further testing should be guided by clues given in the history an
p h y s i c a l e xa mi n a t i o n ; i t ma y i n c l u d e t h e f o l l o w i n g :
1. S t o o l s t u d i e s f o r o v a a n d p a r a s i t e s ( t h r e e s aI fmt ph leesse) .a r e n e g a t i v e ,
b u t p a r a s i t i c i n f e c t i o n i s s t r o n g l y s u s p e c t e d , c o n s i d e r s ma l l b o w e l b i o p s y o r
mu s c l e b i o p s y i n c a s e s o f t r i c h i n o s i s .
2. U r i n a l y s i s w i t h e xa mi n a t i o n f o r s c h i s t o s o me e g g s
3. S e r o l o g y f o r p a r a s i t e s
4. S p u t u m e v a l u a t i o n f o r e o s i n o p h i l s
5. B l o o d c u l t u r e s t o e v a l u a t e f o r b a c t e r i a l i n f e c t i o n
6. C h e s t x- r a y w i t h o r w i t h o u t c h e s t c o mp u t e d t o mo g r a p h y s c a n t o e v a l u a t e f o r
p u l mo n a r y i n v o l v e me n t
P. 3 5 5
7. 1 2 - l e a d e l e c t r o c a r d i o g r a m a n d e c h o c a r d i o g r a m t o e v a l u a t e f o r c a r d i a c
i n v o l v e me n t
8. L i v e r f u n c t i o n p a n e l a n d r e n a l p r o f i l e t o e v a l u a t e f o r e n d - o r g a n d a ma g e
9. B o n e ma r r o w b i o p s y t o e v a l u a t e f o r c l o n a l d i s o r d e r s
D. Genetics
A l l e r g i c d i s e a s e s s u c h a s a s t h ma a n d s o me ma l i g n a n c i e s
h e r e d i t a r y c o mp o n e n t a s w e l l a s a n e n v i r o n me n t a l c a u s e .
t u mo r s a r e i n h e r i t e d . S t u d i e s h a v e s h o w n t h e h e r i t a b i l i t y
b e t w e e n 3 6 % a n d 7 24)%. (R e s e a r c h i n t h i s a r e a c o n t i n u e s t o
t h e e xa c t me c h a n i s m o f t r a n s mi s s i o n o f a s t h ma .
appear to have a strong

Ten percent of solid
c o mp o n e n t o f a s t h ma t o b e
a t t e mp t a t d e t e r mi n i n g
I V. Diagnosis
I t i s i mp o r t a n t t o c o n s i d e r t h e d i f f e r e n t i a l d i a g n o s i s o f e o s i n o p h i l i a , b e c a u s e t h e
p r o g n o s i s a n d t r e a t me n t v a r y g r e a t l y, d e p e n d i n g o n t h e c a u s e . I f t h e d i a g n o s i s o f
e o s i n o p h i l i a s t i l l r e ma i n s e l u s i v e a f t e r a n e xt e n s i v e w o r k u p , c o n s i d e r r e f e r r a l t o
i n f e c t i o u s d i s e a s e o r h e ma t o l o g y.
A. Primary eosinophilia
1. C l o n a l d i s e a sTeh i s i n c l u d e s e o s i n o p h i l i c l e u k e mi a s a n d my e l o i d d i s o r d e r s .
C l i n i c a l ma n i f e s t a t i o n s o f l e u k e mi a i n c l u d e w e i g h t l o s s , f e v e r, l y mp h a d e n o p a t h y
night sweats, recurrent infections, and easy bruising.
2. H y p e r e o s i n o p h i l i c s y n d r oPm
a tei e n t s t y p i c a l l y p r e s e n t w i t h a mo d e r a t e t o
s e v e r e e o s i n o p h i l i a . S y mp t o ms mu s t b e p r e s e n t f o r mo r e t h a n 6 mo n t h s w i t h
e n d - o r g a n d a ma g e a n d n o i d e n t i f i a b l e c a u s e o f t h e e o s i n o p h i l i a . M e n a r e mo r e
t h a n t w o t i me s mo r e l i k e l y t h a n w o me n t o b e d i a g n o s e d w i t h t2)
h i. s d i s e a s e (
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16.2 - Eosinophilia
B. Secondary eosinophilia
1. A l l e r g i c d i s e a s eT sh e s e i n c l u d e a s t h ma , a t o p i c d e r ma t i t i s , a n d a l l e r g i c r h i n i t i s .
P a t i e n t s w i t h a s t h ma ma y c o mp l a i n o f d y s p n e a , w h e e zi n g , o r c o u g h . S n e e zi n g ,
r h i n o r r h e a , a n d n a s a l i t c h i n g a r e t h e h a l l ma r k o f a l l e r g i c r h i n i t i s . P a t i e n t s w i t h
a t o p i c d e r ma t i t i s ma y c o mp l a i n o f r a s h , d r y s k i n , a n d i t c h i n g .
2. D r u g r e a c t i oCn e r t a i n me d i c a t i o n s s u c h a s s u l f o n a mi d e s , p h e n y t o i n , g o l d
c o mp o u n d s , a s p i r i n , a l l o p u r i n o l , a n d s o me t o xi n s ma y l e a d t o e o s i n o p h i l i a .
3. T i s s u e - i n v a s i v e p a r a s i t i c i n f e cTt ihoensse i n c l u d e s c h i s t o s o mi a s i s , v i s c e r a l
t o xo c a r i a s i s , s t r o n g y l o i d i a s i s , f i l a r i a s i s , a n c y l o s t o mi a s i s , f a s c i o l i a s i s ,
t r i c h i n e l l o s i s , a n d p a r a g o n i mi5)a.s iSsy (mp t o ms v a r y d e p e n d i n g o n t h e i n v a s i o n
s i t e a n d ma y i n c l u d e a b d o mi n a l p a i n , d i a r r h e a o r c o n s t i p a t i o n , f e v e r, r a s h ,
mu s c l e a c h e s , d y s u r i a , a n d l y mp h a d e n o p a t h y.
4. B a c t e r i a l / v i r a l i n f e c t iTo hniss i s a l e s s c o mmo n c a u s e o f e o s i n o p h i l i a ,

a l t h o u g hB or r el i ai n f e c t i o n s a n d h u ma n i mmu n o d e f i c i e n c y v i r u s ( H I V ) h a v e b e e n
i mp l i c a t e d . H I V c a n ma n i f e s t w i t h w e i g h t l o s s , f e v e r, n i g h t s w e a t s , a n d r e c u r r e n
infections.
5. A u t o i m m u n e d i s o r d eTrhsi s i n c l u d e s We g e n e r ' s g r a n u l o ma t o s i s , K i mu r a ' s
d i s e a s e , C h u r g - S t r a u s s s y n d r o me , s c l e r o d e r ma , s a r c o i d o s i s , p o l y a r t e r i t i s
n o d o s u m, a n d i n f l a mma t o r y b o w e l d i s e a s e . T h e s y mp t o ms a r e d e p e n d e n t o n t h e
organ involved in the disease process.
6. C a n c e rT h i s i n c l u d e s H o d g k i n ' s l y mp h o ma a n d me t a s t a t i c c a n c e r s ( l u n g ,
c e r v i c a l , b r e a s t , l i v e r, p a n c r e a s , a n d k i d n e y ) . T h e p a t i e n t ma y e xh i b i t f e v e r,
weight loss, and night sweats.
7. A d r e n a l i n s u f f i c i e nTchyi s c a n l e a d t o h y p e r p i g me n t a t i o n o f t h e s k i n , f a t i g u e ,
w e i g h t l o s s , w e a k n e s s , a n d a b d o mi n a l p a i n .
References
1 . R o t h e n b e r g M E . E o s i n o p hNi l iEa ngl
.
J M ed1 9 9 8 ; 3 8 : 1 5 9 2 1 5 9 9 .
2. S utton S , Assa'ad A, R othenberg M . Anti- IL -5 and hypereosinophilic
s y n d r o me sC. l i n I mmunol
2 0 0 5 ; 11 5 : 5 1 6 0 .
3. Tefferi A. B lood eosinophilia: a new paradigm in disease classification,
d i a g n o s i s , a n d t r e a t me
Mnay
t . o C l i n P r oc
2005;80:7583.
4 . B l u me n t h a l M . T h e i mmu n o p a t h o l o g y a n d g e n e t i c s oMf iann
s t hMma
ed.
2004;87:5356.
5. G rathwol K , L eB run C , Tenglin R . E osinophilia of P
t hos
e tbgr
l oad
o d .M ed
1995;97:169172.
578 / 652
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16.3 - Neutropenia
D e t e r mi n a t i o n s > 1 6 . 3 - N e u t r o p e n i a
16.3
Neutropenia
Sara Gray bill
I . Background
Neutropenia is defined as an absolute neutrophil count (AN C) of <1,500/L. T he
AN C is calculated using the total white blood cell count and the fraction of
p o l y mo r p h o n u c l e a r ( P M N ) c e l l s a n d b a n d f o r ms f r o m t h e d i f f e r e n t i a l . T h e f o r mu l a
f o l l o w s 1)( :
AN C = W B C (cells/L) percent (P M Ns + bands) 100
A. Etiology
T he three basic processes leading to neutropenia include (a) decreased production
(b) enhanced peripheral destruction, and (c) pooling of neutrophils in the vascular
e n d o t h e l i u m o r t i s s2)
u e. (
B. Epidemiology
B l a c k s a n d p e o p l e f r o m t h e M i d d l e E a s t ma y n o r ma l l y h a v e A N C s a s l o w a s
1 , 5 0 0 / L y e t h a v e n o p r o b l e ms w i t h r e c u r r e n t i n f e c t i o n s .
I I I . Evaluation
A. History
R e c e n t , r e c u r r e n t , o r f r e q u e n t i n f e c t i o n s p o i n t t o i mmu n e d y s f u n c t i o n . H i s t o r y o f
c u r r e n t a n d p r i o r me d i c a t i o n s s h o u l d b e t a k e n . S e v e r a l i l l n e s s e s a l s o
P. 3 5 7
h a v e n e u t r o p e n i a a s a r e l a t e d f e a t u r e o r a s a c o n s e q u e n c e o f t h e i r t r e a t me n t . A
h i s t o r y o f h e a v y a l c o h o l u s e a n d p o s s i b l e e xp o s u r e t o o c c u p a t i o n a l t o xi n s s h o u l d b e
e l i c i t e d . F i n a l l y, f a mi l y h i s t o r y ma y p o i n t t o r a r e h e r e d i t a r y c o n d i t i o n s .
TAB L E 16.3.1 Differential Diagnosis of Neutropenia

(1)
579 / 652
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16.3 - Neutropenia
Acquired
Infection
C o l l a g e n v a s c u l a r d i s e a s e s ( e . g . , F e l t y ' s s y n d r o me , s y s t e mi c l u p u s
e r y t h e ma t o s u s )
C o mp l e me n t a c t i v a t i o n ( e . g . , h e mo d i a l y s i s , a c u t e r e s p i r a t o r y d i s t r e s s
s y n d r o me )
D r u g - i n d u c e d ( e . g . , s u l f o n a mi d e s , c h l o r p r o ma zi n e , c l o za p i n e , d i u r e t i c s ,
gold)
T o xi n s ( e . g . , b e n ze n e )
A u t o i mmu n e
Transfusion reaction
Pure white cell aplasia
Hypersplenism
N u t r i t i o n a l d e f i c i e n c y ( e . g . , a l c o h o l i s m, 1v2i/tfaomi
l a tne Bd e f i c i e n c y )
D i s e a s e s a f f e c t i n g b o n e ma r r o w ( e . g . , l e u k e mi a , c h e mo t h e r a p y, a p l a s t i c
a n e mi a , t u mo r r e p l a c e me n t )
Congenital
Cyclic neutropenia
C h d i a k - H i g a s h i s y n d r o me
S e v e r e c o n g e n i t a l n e u t r o p e n i a ( K o s t ma n n ' s s y n d r o me )
Severe infantile agranulocytosis
R e v i s e d f r o m B a e h n e rOR
v er
. v i ew of neut r openi
. I na: R o s e B , e d .
U p T o D a t e . We l l e s l e y, M A : U p T o D a t e , 2 0 0 5 .
S p l e n o me g a l y ma y b e p r e s e n t . T h e o r a l c a v i t y s h o u l d a l s o b e e xp l o r e d a s g i n g i v i t i s
s t o ma t i t i s , a n d a b s c e s s e s a r e o f t e n t h e f i r s t p r e s e n t i n g s e q u e l a e o f mi l d
neutropenia.
C. Testing
A ma n u a l d i f f e r e n t i a l a n d p e r i p h e r a l b l o o d s me a r c a n c o n f i r m t h e d i a g n o s i s o f
n e u t r o p e n i a . I f p a n c y t o p e n i a e xi s t s , a b o n e ma r r o w b i o p s y i s r e q u i r e d . I f t h e
n e u t r o p e n i a i s mi l d ( A N C > 1 , 0 0 0 / L ) , a n d t h e p a t i e n t h a s n o i n f e c t i o n s , A N C
me a s u r e me n t s t h r e e t i me s p e r w e e k ma y b e d o n e t o s e e w h e t h e r t h e c o n d i t i o n
r e s o l v e s . I f c o u n t s n o r ma l i ze , s u r v e i l l a n c e f o r t h e n e xt y e a r s h o u l d i n c l u d e a
c o mp l e t e b l o o d c o u n t a t t h e f i r s t s i g n o f i n f e c t i o n t o l o o k f o r r e c u r r e n c e . I f
neutropenia fails to resolve after 8 weeks, recurrent infections develop, or lower
AN C (< 1,000/L ) counts are noted, further workup is needed. Additional laboratory
t e s t s ma y i n c l u d e b o n e ma r r o w b i o p s y ( e v e n i f n o t p a n c y t o p e n i c ) , a n t i n u c l e a r
a n t i b o d i e s , c o mp l e me n t l e v e l s , r h e u ma t o i d f a c t o r, a n t i n e u t r o p h i l a n t i b o d i e s ,
i mmu n o g l o b u l i n s , h u ma n i mmu n o d e f i c i e n c y v i r u s s e r o l o g1y,2 vaint da mi
f onl aBt e
l e v e l s , a n d b o n e ma r r o w c u l1)
t u. r e (
D. Genetics
580 / 652
tmdmss
16.3 - Neutropenia
H e r e d i t a r y n e u t r o p e n i a i s r a r e . T w o ma i n f o r ms i n c l u d e c y c l i c n e u t r o p e n i a a n d
s e v e r e c o n g e n i t a l n e u t r o p e n i a ( K o s t ma n n ' s s yEn dL rAo2me
g e)n. e mu t a t i o n s h a v e
b e e n l i n k e d t o b o t h d i s o r d3)e.r s (
I V. Diagnosis
A p r a c t i c a l a p p r o a c h t o t h e d i f f e r e n t i a l d i a g n o s i s o f n e u t r o p e nTi a bi lse l i s t e d i n
1 6 . 4 . 1.
T h e h a l l ma r k o f n e u t r o p e n i a i s r e c u r r e n t i n f e c t i o n s . H o w e v e r, o n e xa mi n a t i o n o f t h e
p a t i e n t w i t h s e v e r e n e u t r o p e n i a , t h e c l a s s i c s i g n s o f i n f e c t i o n ma y b e a b s e n t . T h e
i n f l a mma t o r y r e s p o n s e i s b l u n t e d , w h i c h ma y c a u s e d i mi n i s h e d f e v e r, p e r i t o n e a l
s i g n s , a n d r a d i o l o g i c f i n d i n g s . A s a r e s u l t , t h e p a t i e n t ' s i n f e c t i o n ma y g o u n d e t e c t e
u n t i l l a t e i n t h e c o u r s e . I n f e c t i o n s i n p a t i e n t s a l s o p r o g r e s s mo r e r a p i d l y a n d a
p a t i e n t w i t h n e u t r o p e n i a mu s t b e t r e a t e d mo r e a g g r e s s i v e l y t h a n a p a t i e n t w i t h o u t i t
References
1 . B a e h n e r RO. v er v i ew of neut r openi
, Uap T o D a t e P a t i e n t I n f o r ma t i o n 1 4 . 2 .
Av a i l a b l e a t : h t t p : / / p a t i e n t s . u p d a t e . c o m/ t o p i c . a s p . ?
file=whitecel/5073&title= Neutropenia. L ast updated J une 2006.
2 . H o l l a n d S M , G a l l i n J I . D i s o r d e r s o f g r a n u l o c y t e s a n d mo n o c y t e s . I n : K a s p e r
D , H a r r i s o n T R , A r mi t a g e J O , e t a lH. ,arerdi s on'
. s pr i nc i pl es of i nt er nal
medi c i ne
, 1 6 t h e d . N e w Yo r k , N Y : M c G r a w - H i l l , 2 0 0 5 .
3 . B e r l i n e r N , H o r w i t z M , L o u g h r aCnongeni
T P, t al and ac qui r ed neut r openi
.
a
A me r i c a n S o c i e t y o f H e ma t o l o g y E d u c a t i o n P r o g r a m, 2 0 0 4 : 6 3 7 9 . Av a i l a b l e a t :
h t t p : / / w w w. a s h e d u c a t i o n b o o k . o r g / c g i / c o n t e n t / f u l l / 2 0 0 4 / 1 / 6 3 .
581 / 652
tmdmss
16.4 - Polycythemia
D e t e r mi n a t i o n s > 1 6 . 4 - P o l y c y t h e mi a
16.4
Polycythemia
Katrina Carte r
I . Background
P o l y c y t h e mi a o c c u r s w h e n t h e s e r u m h e ma t o c r i t i s v e r y h i g h . T h i s i s g e n e r a l l y
g r e a t e r t h a n 4 8 % i n w o me n a n d 5 2 % i n me n . A h e mo g l o b i n > 1 6 . 5 g / d L i n w o me n a n d
1 8 . 5 g / d L i n me n i s a l s o c o n s i d e r e d p o l y c y t h e mi c . T h i s e l e v a t i o n i n h e mo g l o b i n o r
h e ma t o c r i t ma y b e r e l a t i v e o r a b s o l u t e . R e l a t i v e p o l y c y t h e mi a o c c u r s
P. 3 5 8
w h e n t h e r e i s n o t r u e i n c r e a s e i n t h e r e d b l o o d c e l l ( R B C ) ma s s b u t r a t h e r a
c o n t r a c t i o n o f t h e p l a s ma v o l u me . A b s o l u t e p o l y c y t h e mi a o c c u r s w h e n t h e r e i s a t r u
i n c r e a s e i n t h e R B C ma s s d u e t o e i t h e r p r i ma r y o r s e c o n d3)
a r.y c a u s e s (
A. P r i ma r y p o l y c y t h e mi a r e s u l t s f r o m a n a c q u i r e d o r i n h e r i t e d a b n o r ma l i t y o f R B C
p r e c u r s o r s . S i mp l y p u t , t h e R B C s u n d e r g o c l o n a l e xp a n s i o n . A l t h o u g h t h e R B C
i s t h e mo s t p r o mi n e n t f e a t u r e , t h e w h i t e b l o o d c e l l s a n d p l a t e l e t s mu l t i p l y a s
w e l l . T h e i n c r e a s e i n c e l l ma s s r e s u l t s i n h y p e r v i s c o s i t y o f t h e b l o o d . T h i s
c a u s e s mo s t o f t h e s y mp t o ms e xp e r i e n c e d b y t h e p a t i e n t s u c h a s h e a d a c h e ,
d i zzi n e s s , p r u r i t u s , a n d s t 1)
r o.k e (
B. S e c o n d a r y p o l y c y t h e mi a o c c u r s i n r e s p o n s e t o i n c r e a s e d e r y t h r o p o i e t i n ( E P O ) .
E P O i s p r o d u c e d i n t h e k i d n e y s i n r e s p o n s e t o h y p o xi a . T h i s i s a me c h a n i s m t o
i n c r e a s e t h e o xy g e n - c a r r y i n g c a p a c i t y o f t h e c i r c u l a t i o n . I n r e s p o n s e t o a
h y p o xi c i n s u l t , E P O i s r e l e a s e d , b u t i t ma y b e r e l e a s e d f o r o t h e r r e a s o n s a s
w e l l . I n a p p r o p r i a t e r e l e a s e o f E P O ma y o c c u r w i t h r e n a l t u mo r s o r h e p a t o ma s .
I I I . Evaluation
A. History
T h e mo s t c o mmo n c a u s e o f p o l y c y t h e mi a i s r e l a t e d t o h y p o xi a , t h e r e f o r e a t h o r o u g h
evaluation of the respiratory status should be sought. Patients should be asked if
t h e y h a v e a h i s t o r y o f a s t h ma o r c h r o n i c o b s t r u c t i v e p u l mo n a r y d i s e a s e ( C O P D ) .
A d d i t i o n a l s y mp t o ms s u c h a s s h o r t n e s s o f b r e a t h , d y s p n e a o n e xe r t i o n , o r c y a n o s i s
s h o u l d b e a s c e r t a i n e d . A t h o r o u g h s mo k i n g h i s t o r y s h o u l d b e e l i c i t e d , i n c l u d i n g h o w
582 / 652
tmdmss
16.4 - Polycythemia
ma n y c i g a r e t t e s p a t i e n t s s mo k e d a i l y a n d h o w ma n y y e a r s t h e y h a v e s mo k e d , a n d i f
t h e y a r e n o l o n g e r s mo k i n g , h o w ma n y y e a r s a g o d i d t h e y s t o p . M a n y j o b s
p r e d i s p o s e p a t i e n t s t o e n v i r o n me n t a l e xp o s u r e s . P a t i e n t s s h o u l d b e a s k e d a b o u t
t h e i r o c c u p a t i o n a n d i f t h e y a r e e xp o s e d t o s u b s t a n c e s s u c h a s c a r b o n mo n o xi d e a t
work. Particular persons who are at high risk are industrial
P. 3 5 9
w o r k e r s a n d i n d i v i d u a l s w h o w o r k i n p o o r l y v e n t i l a t e d a r e a s . P a t i e n t s ma y a l s o
r e p o r t i n c r e a s e d i t c h i n g a l l o v e r t h e i r b o d y, p a r t i c u l a r l y a f t e1,2)
r s .h o w e r i n g (
F I G U R E 1 6 . 4 . 1D.i a g n o s t i c e v a l u a t i o n o f p o l y c y t h e mi a . E P O , e r y t h r o p o i e t i n .
T h e p h y s i c a l e xa mi n a t i o n i n p a t i e n t s w i t h p o l y c y t h e mi a ma y b e n o t a b l e f o r c y a n o s i s
o f l i p s , e a r l o b e s , a n d e xt r e mi t i e s . C l u b b i n g ma y a l s o b e e v i d e n t . A t h o r o u g h
a b d o mi n a l e xa mi n a t i o n f o r h e p a t o s p l e n o me g a l y a n d a h e a r t e xa mi n a t i o n e v a l u a t i n g
f o r mu r mu r s o r b r u i t s s h o u l d b e p e r f o r me d .
C. Testing
T h e mo s t i mp o r t a n t l a b o r a t o r y t e s t s a r e h e mo g l o b i n , h e ma t o c r i t , a n d R B C c o u n t .
T hese values should be adjusted for the sex and age of the patient. One should als
obtain a white blood cell count and platelet count. A urinalysis should be obtained,
l o o k i n g f o r h e ma t u r i a . L i v e r f u n c t i o n t e s t s s h o u l d a l s o b e o b t a i n e d . I f
c a r d i o p u l mo n a r y d i s e a s e i s s u s p e c t e d , a c h e s t x- r a y ma y b e h e l p f u l t o a s s e s s t h e
patient for C O P D or congestive heart failure. If the patient has significant
e xp o s u r e s , a s e r u m c a r b o xy h e mo g l o b i n s h o u l d b e o b t a i n e d .
I V. Diagnosis
W h e n e v e r a l a b o r a t o r y v a l u e c o me s b a c k g r o s s l y e l e v a t e d , i t s h o u l d b e r e p e a t e d .
583 / 652
tmdmss
16.4 - Polycythemia
O n c e a n i n c r e a s e i n h e mo g l o b i n a n d h e ma t o c r i t i s c o n f i r me d , a n e v a l u a t i o n o f
p o s s i b l e c a u s e s mu s t b e g i n ( s e e F i g u r e 1 6 . 5 . 1 ) .
A. I f t h e p a t i e n t h a s o c c u p a t i o n a l e xp o s u r e s a n d t h e c a r b o xy h e mo g l o b i n i s
e l e v a t e d , a t r i a l o f s t o p p i n g t h e e xp o s u r e s s h o u l d b e d o n e . R e s o l u t i o n o f
a b n o r ma l b l o o d v a l u e s s h o u l d o c c u r w i t h i n 3 mo n t h s .
B. I f t h e p a t i e n t h a s n o e xp o s u r e , a n d t h e c a r b o xy h e mo g l o b i n i s w i t h i n n o r ma l
l i mi t s , a s e r u m E P O l e v e l s h o u l d b e o b t a i n e d . I f i t i s l o w, t h e d i a g n o s i s i s
p o l y c y t h e mi a v e r a , o r p r i ma r y p o l y c y t h e mi a . I f E P O l e v e l s a r e h i g h , i t i s
i n d i c a t i v e o f e i t h e r a h y p o xi c r e s p o n s e ( a p p r o p r i a t e r e s p o n s e ) o r o v e r p r o d u c t i o n
b y a t u mo r ( i n a p p r o p r i a t e ) . D e p e n d i n g o n t h e c i r c u ms t a n c e s , f u r t h e r e v a l u a t i o n
ma y n o t b e n e c e s s a r y. F o r e xa mp l e , i f t h e E P O l e v e l i s mi l d l y e l e v a t e d , a n d t h e
p a t i e n t h a s C O P D , t h e n t h e r e i s a l o w s u s p i c i o n o f a t u mo r. I f , o n t h e o t h e r
h a n d , t h e p a t i e n t h a s n o r i s k f a c t o r s f o r c a r d i o p u l mo n a r y d i s e a s e a n d a h i g h l y
e l e v a t e d E P O , e v a l u a t i o n f o r a t u mo r s h o u l d b e d o n e . A b d o mi n a l u l t r a s o u n d o r
a b d o mi n a l c o mp u t e d t o mo g r a p h y s c a n i s r e c o mme n d e d i n p a t i e n t s w i t h a h i g h
s u s p i c i o n f o r a n E P O - s e c r e t i n g t u mo r. E P O - s e c r e t i n g t u mo r s a r e u s u a l l y
h e p a t o c e l l u l a r c a r c i n o ma , p h e o c h r o mo c y t o ma , h e ma n g i o b l a s t o ma , u t e r i n e
my o ma t a , a n d , mo s t c o mmo n l y, r e n a l c e l l c a 1,3)
r c i n.o ma (
References
1 . H a r r i s o n T R , e t Pa rl .i nc i pl es of i nt er nal medi. c1i 5ne
th ed. McGraw-Hill
Professional. 2001:353354.
2 . T e f f e r i AD. i agnos t i c appr oac h t o t he pat i ent wi t h pol y. cU
y tphemi
t o Daa t e
2006.
3 . R a k e l R Tex
. t book of f ami l y pr ac, t 6i ct eh e d . P h i l a d e l p h i a , PA : W B S a u n d e r s .
2002:12621266.
584 / 652
tmdmss
16.5 - Thrombocytopenia
D e t e r mi n a t i o n s > 1 6 . 5 - T h r o mb o c y t o p e n i a
16.5
Thrombocytopenia
Te r e s a S t u m p
I . Background
T h r o mb o c y t o p e n i a o c c u r s w h e n t h e r e i s a n a b n o r ma l d e c r e a s e i n t h e n u mb e r o f
platelets. T his condition is encountered when the platelet count is <100,000 L . A
u n i q u e r e l a t i o n e xi s t s b e t w e e n p l a t e l e t c o u n t a n d b l e e d i n g t e n d e n c i e s . F o r e xa mp l e
p a t i e n t s w i t h p l a t e l e t c o u n t s o f > 5 0 , 0 0 0 L a r e g e n e r a l l y a s y mp t o ma t i c a n d a r e
d i a g n o s e d i n c i d e n t a l l y, w h e r e a s p a t i e n t s w h o s e p l a t e l e t c o u n t s a r e < 1 0 , 0 0 0 L a r e
a t r i s k o f s p o n t a n e o u s b l e e1,2,3)
d i n g .(
A. Etiology
T h r o mb o c y t o p e n i a o c c u r s t h r o u g h o n e o f t h e f o l l o w i n g me1,2,3)
c h a n: i s ms (
i n a d e q u a t e n u mb e r o f p l a t e l e t s p r o d u c e d b y t h e b o n e ma r r o w, i n c r e a s e d d e s t r u c t i o n
o f p l a t e l e t s , e n t r a p me n t i n t h e s p l e e n , o r a d i l u t i o n e f f e c t .
P. 3 6 0
1. A u t o i mmu n e t h r o mb o c y t o p e n i c c o n d i t i o n s a r i s e t h r o u g h e xp l i c i t a u t o a n t i b o d i e s ,
r e s u l t i n g i n e i t h e r i n c r e a s e d d e s t r u c t i o n o f p l a t e l e t s o r i mp a i r me n t o f s p e c i f i c
p r o t e i n s . T h i s l e a d s t o p l a t e l e t a g g r e g a t i o n a n d t h r o mb u 4)
s .f o r ma t i o n (
2. D r u g - i n d u c e d t h r o mb o c y t o p e n i a ma y b e c a u s e d b y a p l e t h o r a o f me d i c a t i o n s .
T h e mo s t f r e q u e n t c u l p r i t , e s p e c i a l l y i n h o s p i t a l i ze d p a t i e n t s , i s h e p a r i n .
A l t h o u g h d r u g - i n d u c e d t h r o mb o c y t o p e n i a i s a l s o a n a n t i b o d y - me d i a t e d
s y n d r o me , i t i s t r a n s i e n t , t y p i c a l l y r e s o l v i n g w h e n t h e o f f e n d i n g a g e n t i s
d i s c o n t i n u e d1,2,4)
( .
B. Epidemiology
I mmu n e t h r o mb o c y t o p e n i c p u r p u r a ( I T P ) i s a r e l a t i v e l y c o mmo n a u t o i mmu n e

t h r o mb o c y t o p e n i c d i s e a s e t h a t o c c u r s i n t h e a c u t e a n d c h r o n i c s e t t i n g w i t h a
prevalence of one in ten thousand persons. Acute IT P is seen solely in children 2 to
9 y e a r s o f a g e w i t h a p e a k i n c i d e n c e a t 3 t o 5 y e a r s o f a g e . I t a f f e c t s me n a n d
w o me n e q u a l l y. A c u t e I T P t y p i c a l l y f o l l o w s a n a c u t e v i r a l s y n d r o me a n d h a s a s e l f l i mi t i n g c o u r s e . I n c o n t r a s t , c h r o n i c I T P h a s a f e ma l e p r e d o mi n a n c e o f 3 : 1 , a f f e c t s
585 / 652
tmdmss
a d u l t s 2 0 t o 5 0 y e a r s o f a g e , a n d r a r e l y r e s u l t s i n s p o n t a n e o u1,2,4)
s r e mi
. ssion (
I I I . Evaluation
A. History
A me t i c u l o u s h i s t o r y mu s t b e o b t a i n e d w i t h p a r t i c u l a r f o c u s o n t h e f o l l o w i n g :
1. A t h o r o u g h r e v i e w o f a l l me d i c a t i o n s c u r r e n t l y b e i n g t a k e n a n d t h e d a t e
prescribed
2. M e n s t r u a l a n d p r e g n a n c y h i s t o r y
3. T h e p r e s e n c e o f t h e f o l l o w i n g : e p i s t a xi s a n d b l e e d i n g g u ms ; r e d d i s h o r p u r p l i s h
d i s c o l o r a t i o n o f t h e s k i n ; e xc e s s i v e b r u i s i n g ; a n d h e ma t u r i a , me l e n a ,
h e ma t o c h e zi a , a n d b r i g h t r e d b l o o d f r o m t h e r e c t u m
4. A l c o h o l u s e
A t h o r o u g h p h y s i c a l e xa mi n a t i o n i s w a r r a n t e d w i t h s p e c i a l e mp h a s i s o n t h e f o l l o w i n
1. S k i n e xa mi n a t i o n l o o k i n g f o r p e t e c h i a e , p u r p u r a , a n d e c c h y mo s e s
2. A b d o mi n a l e xa mi n a t i o n t o a s s e s s f o r s p l e n o me g a l y a n d h e p a t o me g a l y
3. N e u r o l o g i c e xa mi n a t i o n t o a s c e r t a i n i f h e mo r r h a g e h a s o c c u r r e d , a l t h o u g h r a r e
C. Testing
A c o mp l e t e b l o o d c o u n t w i t h d i f f e r e n t i a l a n d p e r i p h e r a l s me a r i s e s s e n t i a l f o r
e v a l u a t i n g t h r o mb o c y t o p e n i a . I ma g i n g s t u d i e s a r e r a r e l y i n d i c a t e d . B o n e ma r r o w
b i o p s y i s b e n e f i c i a l i n p a t i e n t s w i t h s p l e n o me g a l y a n d i n t h o s e w h o f o l l o w a n
uncharacteristic course.
I V. Diagnosis(1,2,3,4,5)
D e t e r mi n i n g t h e e t i o l o g y o f t h r o mb o c y t o p e n i a ma y s e e m d a u n t i n g i n i t i a l l y. A
c o mp r e h e n s i v e h i s t o r y a n d p h y s i c a l e xa mi n a t i o n a l o n g w i t h a f e w l a b o r a t o r y t e s t s
often leads to the correct diagnosis.
A. I n a d e q u a t e n u mb e r o f p l a t e l e t s p r o d u c e d b y t h e b o n e ma r r o w. D i a g n o s e s
include the following:
1. L e u k e mi a
2. L y mp h o ma
3. A p l a s t i c a n d me g a l o b l a s t i c a n e mi a
4. H e a v y a l c o h o l u s e
B. I n c r e a s e d d e s t r u c t i o n o f p l a t e l e t s . D i a g n o s e s i n c l u d e t h e f o l l o w i n g :
1. I mmu n e t h r o mb o c y t o p e n i a p u r p u r a ( d i a g n o s i s o f e xc l u s i o n )
586 / 652
tmdmss
2. H u ma n i mmu n o d e f i c i e n c y v i r u s / a c q u i r e d i mmu n o d e f i c i e n c y s y n d r o me
3. M e d i c a t i o n s ( e . g . , h e p a r i n , q u i n i n e , r i f a mp i n , g o l d s a l t s )
4. D i s s e mi n a t e d i n t r a v a s c u l a r c o a g u l a t i o n , H E L L P ( h e mo l y s i s , e l e v a t e d l i v e r
e n zy me s , l o w p l a t e l e t c o u n t ) s y n d r o me
5. T h r o mb o t i c t h r o mb o c y t o p e n i c p u r p u r a
6. H e mo l y t i c - u r e mi c s y n d r o me
C. E n t r a p me n t i n t h e s p l e e n . D i a g n o s e s i n c l u d e t h e f o l l o w i n g :
1. C i r r h o s i s
2. M y e l o f i b r o s i s
3. G a u c h e r ' s d i s e a s e
D. D i l u t i o n e f f e c t : l a r g e b l o o d r e p l a c e me n t w i t h t o o f e w p l a t e l e t s
E. L a b o r a t o r y e r r o r : c l o t t e d s p e c i me n , w r o n g p a t i e n t , o r t e c h n i c a l / e q u i p me n t e r r o r s
P. 3 6 1
References
1 . G e o r g e J NE. v al uat i on and management of t hr omboc y t openi a by pr i mar y
c ar e phy s i c i ans
. w w w. u p t o d a t e . c o m, 2 0 0 4 .
2. T hiagarajan P
P.l at el et di s or der
. wsw w. e me d i c i n e . c o m/ me d / t o p i c 9 8 7 . h t m. L a s t
u p d a t e d S e p t e mb e r 2 0 0 4 .
3 . T hr omboc y t openi a: bl eedi ng and c l ot t i ng di. sTor
h eder
Mse r c k M a n u a l o f
M e d i c a l I n f o r ma t i o n . Av a i l a b l e a t :
h t t p : / / w w w. me r c k . c o m/ mmh e / s e c 1 4 / c h 1 7 3 / c h 1 7 3 d . h t ml . L a s t u p d a t e d F e b r u a r y
2003.
4 . K r a v i t z M S , S h o e n f e l d Y. T h r o mb o c y t o p e n i c c o n d i t i o n s - a u t o i mmu n i t y a n d
h y p e r c o a g u l a b i l i t y : c o mmo n a l i t i e s a n d d i f f e r e n c e s i n I T P, T T P, H I T , a n d A P S .
A m J H emat ol2 0 0 5 ; 8 0 : 2 3 2 2 4 2 .
5 . Ve s e l y S K , P e r d u e J J , R i zv i M A , e t a l . M a n a g e me n t o f a d u l t p a t i e n t s w i t h
p e r s i s t e n t i d i o p a t h i c t h r o mb o c y t o p e n i c p u r p u r a f o l l o w i n g s p l e n e c t o my : a
s y s t e ma t i c r e v i eAw.
nn I nt er n M ed
2 0 0 4 ; 1 4 0 : 11 2 1 2 1 .
587 / 652
tmdmss
16.6 - Erythrocyte Sedimentation Rateand C-Reactive Protein
D e t e r mi n a t i o n s > 1 6 . 6 - E r y t h r o c y t e S e d i me n t a t i o n R a t e a n d C - R e a c t i v e P r o t e i n
16.6
Erythrocyte Sedimentation Rateand CReactive Protein
Elisabe th L. Backe r
I . Background
T h e e r y t h r o c y t e s e d i me n t a t i o n r a t e ( E S R ) a n d C - r e a c t i v e p r o t e i n ( C R P ) a r e
c u r r e n t l y t h e mo s t w i d e l y u s e d i n d i c a t o r s o f t h e a c u t e p h a s e p r o t e i n r e s p o n s e , u s e d
t o d e t e c t i l l n e s s e s a s s o c i a t e d w i t h a c u t e a n d c h r o n i c i n f e c t i o n , i n f l a mma t i o n , t i s s u e
d e s t r u c t i o n , a n d a d v a n c e d n e o p l a s m. T h e C R P i s a mo r e s e n s i t i v e a n d r a p i d l y
r e s p o n d i n g i n d i c a t o r t h a n t h e E S R , o f t e n s h o w i n g a n e a r l i e r a n d mo r e i n t e n s e
i n c r e a s e t h a n t h e E S R i n a n a c u t e i n f l a mma t o r y p r o c e s s . W i t h r e c o v e r y, t h e
d i s a p p e a r a n c e o f t h e C R P p r e c e d e s t h e n o r ma l i za t i o n o1)
f .t h e E S R (
T h e a c u t e p h a s e r e s p o n s e i s a ma j o r p a t h o p h y s i o l o g i c p h e n o me n o n t h a t
a c c o mp a n i e s i n f l a mma t i o n a n d o t h e r d i s2)
o r. dFeor sc u(s o n t h i s p h e n o me n o n f i r s t
occurred with the discovery of elevated serum concentrations of C R P during the
a c u t e p h a s e o f p n e u mo c o c c a l p n e u mo
3) . nTi ah e( i n i t i a l c o n c e p t o f a n E S R d a t e s
b a c k t o 1 9 1 8 . T h e We s t e r g r e n me t h o d i s s t i l l c o n s i d e r e d t h e g o l d s t a n d a r d f o r
me a s u r i n g t h e E S4)
R. (
I I I . Evaluation
A. Physical examination
A c u t e p h a s e r e a c t a n t me a s u r e me n t s a r e u s e f u l i n c o n j u n c t i o n w i t h a t h o r o u g h
p h y s i c a l e xa mi n a t i o n . B e c a u s e t h e E S R a n d C R P l e v e l s a r e i n f l u e n c e d b y mu l t i p l e
factors, the results should be interpreted in the light of the clinical findings.
B. Testing
1. T h e E S R , w h i c h me a s u r e s t h e d i s t a n c e i n mi l l i me t e r s t h a t e r y t h r o c y t e s f a l l
d u r i n g 1 h o u r, i s a s i mp l e b u t n o n s p e c i f i c l a b o r a t o r y t e s t o r d e r e d f r e q u e n t l y i n
c l i n i c a l p r a c t i c e . T h e C R P i s a n o n s 3)
p e caicf ui ct e( p h a s e r e a c t a n t p r o t e i n
u s e d t o d i a g n o s e i n f e c t i o u s a n d i n f l a mma t o r y d i s o r d e r s ; i t a l s o s e r v e s a s a
c a r d i o v a s c u l a r d i s e a s e ( C V D ) ma
5)r.k eTrh e( E S R h a s t h e a d v a n t a g e s o f
f a mi l i a r i t y, s i mp l i c i t y, a n d e xt e n s i v e l i t e r a t u r e c o mp i l e d o v e r ma
3)n. y d e c a d e s (
588 / 652
tmdmss
T h e C R P i s s t a n d a r d i ze d , i n e xp e n s i v e , a n d w i d e l y a v a i l a b l e .
2. A l t h o u g h e l e v a t i o n s i n mu l t i p l e c o mp o n e n t s o f t h e a c u t e p h a s e r e s p o n s e
c o mmo n l y o c c u r t o g e t h e r, n o t a l l h a p p e n u n i f o r ml y i n a l l p a t i e n t s , a n d
d i s c r e p a n c i e s b e t w e e n E S R a n d C R P a r e f o u n d f a i r l y 2)
f r e(qeu. egn. ,t l ay n(
e l e v a t e d E S R t o g e t h e r w i t h a n o r ma l C R P ma y r e f l e c t a f a l s e - p o s i t i v e v a l u e f o r
t h e E S R ) . C u r r e n t l y, t h e o p t i ma l u s e o f t h e a c u t e p h a s e r e a c t a n t s ma y b e t o
o b t a i n s e v e r a l me a s u r e me n t s a n d i n t e r p r e t t h e r e s u l t s i n t h e l i g h t o f t h e c l i n i c a l
c o n t e xt 3,5)
( .
P. 3 6 2
3. C R P l e v e l s c a n b e a f f e c t e d b y l i f e s t y l e c h o i c e s , c o n c u r r e n t d i s e a s e ,
p h a r ma c o t h e r a p y, a g e , g e n d e r ( f e ma l e ) , a n d p o s s i b l y e t h n i c i t y ( e . g . , A f r i c a n
A me r i c a n s ) .
a. F a c t o r s k n o w n t o i n c r e a s e C R P v a l u e s i n c l u d e s mo k i n g , e l e v a t e d b o d y
ma s s i n d e x, e l e v a t e d b l o o d p r e s s u r e , d y s l i p i d e mi a , me t a b o l i c s y n d r o me ,
t y p e 2 d i a b e t e s , h o r mo n e u s e , c h r o n i c i n f e c t i o n s ( b r o n c h i t i s ) , a n d c h r o n i c
i n f l a mma t i o n ( r h e u ma t o i d a r t h5)r .i t i s ) (
b. F a c t o r s k n o w n t o d e c r e a s e C R P v a l u e s i n c l u d e mo d e r a t e a l c o h o l
c o n s u mp t i o n , p h y s i c a l e xe r c i s e , w e i g h t l o s s , a n d me d i c a t i o n s ( s t a t i n s ,
f i b r a t e s , t h i a zo l i d i n e d i o n e s , a n t i - i n f l a mma t o r y a g e n t s , s a l i c y l a t e s , a n d
s t e r o i d s 5)
)(.
4. E S R l e v e l s c a n b e a f f e c t e d b y me n s t r u a t i o n a n d p r e g n a n c y, h e ma t o l o g i c
d i s o r d e r s , me d i c a t i o n s , g e n d e r, a g e , e t h n i c i t y, a n1,3)
d o.b eCsoi nt yd i(t i o n s w i t h
a n E S R o f > 1 0 0 mm/ h o u r i n c l u d e a b s c e s s f o r ma t i o n , s u b a c u t e b a c t e r i a l
e n d o c a r d i t i s , o s t e o my e l i t i s , t e mp o r a l a r t e r i t i s , c o l l a g e n v a s c u l a r d i s e a s e ,
mu l t i p l e my e l o ma , l e u k e mi a / l y mp h o ma , n e o p l a s ms , a n d d r u g h y p e r s e n s i t i v i t y
reactions.
a. F a c t o r s k n o w n t o i n c r e a s e E S R v a l u e s i n c l u d e c h r o n i c r e n a l f a i l u r e
( n e p h r i t i s , n e p h r o s i s ) , ma c r o g l o b u l i n e mi a , h y p e r f i b r i n o g e n e mi a , i r o n / v i t a mi n
B 1 2 d e f i c i e n c y a n e mi a s , me d i c a t i o n s ( d e xt r a n , h e p a r i n , me t h y l d o p a , o r a l
c o n t r a c e p t i v e s , p e n i c i l l a mi n e , p r o c a i n a mi d e , t h e o p h y l l i n e , v i t a mi n A ) , f e ma l e
g e n d e r, a d v a n c e d a g e , A f r i c a n - A me r i c a n e t h n i c i t y, a n d h y p1)
e r. l i p i d e mi a (
b. F a c t o r s k n o w n t o d e c r e a s e E S R l e v e l s i n c l u d e s i c k l e - c e l l a n e mi a ,
s p h e r o c y t o s i s , h y p o f i b r i n o g e n e mi a , p o l y c y t h e mi a v e r a , me d i c a t i o n s ( a s p i r i n ,
c o r t i s o n e , q u i n i n e ) , a n d c h r o n i c h e a r t 1)f a. i l u r e (
I V. Diagnosis
A. D e s p i t e t h e l a c k o f d i a g n o s t i c s p e c i f i c i t y, me a s u r i n g a c u t e p h a s e p r o t e i n l e v e l s
i s u s e f u l i n d i f f e r e n t i a t i n g b e t w e e n i n f l a mma t o r y a n d n o n i n f l a mma t o r y c o n d i t i o n s
and in evaluating the response to and the need for therapeutic interventions. In
general, the E S R increases as the disease worsens and decreases as it
i mp r o v e s . T h e C R P ma y b e u s e f u l w h e n t h e E S R i s e q u i v o c a l o r i n c o n s i s t e n t
w i t h t h e c l i n i c a l i mp r e s s 1)
i o .n sR e( s u l t s s h o u l d b e e xp r e s s e d a s t h e a v e r a g e o f
t w o t e s t s p e r f o r me d 2 w e e k s a p a r t . P a t i e n t s w i t h l e v e l s > 1 0 mg / L s h o u l d b e
589 / 652
tmdmss
e xa mi n e d f o r s o u r c e s o f i n f l a mma t i o n b e f o r e r e p e a t i n5)g . t A
h ec uttees tp h( a s e
r e a c t a n t s s h o u l d n o t b e o r d e r e d f o r s c r e e n i n g p u r p o s e s i n a s y mp t o ma t i c
p a t i e n t s1)( .
1. N o r ma l E S R r a t e s f o r me n b e t w e e n 2 0 a n d 6 5 y e a r s o f a g e c a n b e
e mp i r i c a l l y c a l c u l a t e d a s a g e / 2 ; f o r w o me n i t w o u l d b e ( a g e1) p. l u s 1 0 ) / 2 (
M o s t h e a l t h y s u b j e c t s h a v e a C R P l e v e l o f < 3 mg / L . L e v e l s o f 3 t o 1 0 mg / L
ma y i n d i c a t e mi n o r d e g r e e s o f i n f l a mma t i o n o r o t h e r i n f l u e n c e s . L e v e l s > 1 0
mg / L s u g g e s t s i g n i f i c a n t i n f l a mma
3) .t i o n (
2. C R P t e s t i n g i s a l s o r e c o mme n d e d a s a n a d j u n c t t o t r a d i t i o n a l r i s k f a c t o r
a s s e s s me n t i n C V D . I t h a s b e e n f o u n d t o b e t h e s t r o n g e s t ma r k e r o f f u t u r e
C V D , de nov oa t h e r o s c l e r o s i s , a n d p l a q u e r u p t u r e . A d d i t i o n a l l y, i t h a s
independent prognostic value for future strokes and peripheral vascular
d i s e a s e 5)( . T h e r e l a t i v e r i s k c a t e g o r y b a s e d o n C R P l e v e l s i s s h o w n i n
T a b l e 1 6 . 3.. 1
P. 3 6 3
TAB L E 16.6.1 Risk of cardiovascular disease

based on C-reactive protein level
Risk
Low
Av e r a g e
High
C-re activ e prote in le v e l (m g/L)

<1
1.03.0
>3.0
B. S p e c i f i c a p p l i c a t i o n s o f a c u t e p h a s e r e a c t a n t me a s u r e me n t s i n c l u d e d i s e a s e
p r o c e s s e s s u c h a s C r o h n ' s d i s e a s e , r h e u ma t o i d a r t h r i t i s ( C R P s u p e r i o r t o
E S R ) , p o l y my a l g i a r h e u ma t i c a , a n d g i a n t c e l l a r t e r i t i s ( E S R o f t e n > 1 0 0
mm/ h o u r, b u t C R P ma y b e mo r e s e n s i t i v e f o r d i s e a s e d e t e c t i o n ) , a n d t h e
n o n i n v a s i v e p r o g n o s t i c a s s e s s me n t i n p a t i e n t s w i t h ma
3) l. i gSnyasnt ec mi
y (c
l u p u s e r y t h e ma t o s u s r e p r e s e n t s a n e xc e p t i o n i n t h a t C R P l e v e l s a r e o f t e n n o t
e l e v a t e d , e xc e p t d u r i n g b a c t e r i a l i n f e3)c .t i o n s (
References
1 . P a g a n a K D , P a g a n a TMJos
. by ' s manual of di agnos t i c and l abor at or
. y t es t s
S t . L o u i s , M O : M o s b y, 1 9 9 8 .
2 . K u s h n e r I . T h e p h e n o me n o n o f t h e a c u t e p h a s e rAe nn
s p oNn sYe .A c ad S c i
1982;389:3948.
590 / 652
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3 . w w w. U p T o D a t e . c o m, a c c e s s e d o n J u n e 3 0 , 2 0 0 5 .
4 . B e d e l l S E , B u s h B T . E r y t h r o c y t e s e d i me n t a t i o n r a t e . F r o m f o l k l o r e t o f a c t s .
A m J M ed1 9 8 5 ; 7 8 ( 6 P t 1 ) : 1 0 0 1 1 0 0 9 .
5 . B r u n t o n S . T h e v a l u e o f C - r e a c t i v e p r o t e i n i n t h e c l i n i c a l a s s e s s me n t o f
c a r d i o v a s c u l a r d i s e a s e Fr iemal
s k . e P at i ent
2 0 0 5 ; 3 0 : 11 1 6 .
591 / 652
tmdmss
16.7 - Proteinuria
D e t e r mi n a t i o n s > 1 6 . 7 - P r o t e i n u r i a
16.7
Proteinuria
Carol A. LaCroix
I . Background
I t i s n o r ma l f o r a n a d u l t t o e xc r e t e 8 0 t o 1 5 0 mg o f p r o t e i n i n t h e u r i n e p e r d a y.
U s u a l l y, t h e e xc r e t e d p r o t e i n c o n s i s t s o f 4 0 % a l b u mi n , 4 0 % u r o mo d u l i n ( a l s o k n o w n
a s Tamm- H or s f al l muc opr otf ei
r onm t h e l o o p o f H e n l e ) , a n d 2 0 % s ma l l - mo l e c u l a r w e i g h t g l o b u l i n1,2)
s ( . M i c r o a l b u mi n u r i a r e f e r s t o a n e xc r e t i o n r a t e o f 3 0 t o 3 0 0 mg
o f a l b u mi n p e r d a y, w h e r e a s t h e n e p h r o t i c r a n g e i s mo r e t h a n 3 , 0 0 3)
0 .mg p e r d a y (
A. Pathophysiology
P r o t e i n u r i a ma y b e t r a n s i e n t o r p e r s i s t e n t . T r a n s i e n t p r o t e i n u r i a c a n o c c u r w i t h
e xe r c i s e , c o l d e xp o s u r e , f e v e r, a n d c o n g e s t i v e h e a r t f a i l u r e . P e r s i s t e n t p r o t e i n u r i a
d i a g n o s e d w h e n a v a l u e o f > 3 0 0 mg / d L h a s b e e n d o c u me n t e d i n t h r e e u r i n e
s p e c i me n s . T h e r e a r e t h r e e t y p e s o f p r o t e i n u r i a : g l o me r u l a r, t u b u l a r, a n d o v e r f l o w.
G l o me r u l a r p r o t e i n u r i a i n v o l v e s i n c r e a s e d p e r me a b i l i t y t o p l a s ma p r o t e i n s i n t h e
g l o me r u l i a n d ma y r a n g e f r o m t h e mi n i ma l t o t h e n e p h r o t i c r a n g e . T u b u l a r p r o t e i n u r
o c c u r s w h e n t h e p r o xi ma l t u b u l e i s u n a b l e t o r e a b s o r b p r o t e i n s . O v e r f l o w p r o t e i n u r i
r e s u l t s f r o m a n o v e r p r o d u c t i o n o f i mmu n o g l o b u l i n s , p a r t i c u l a r l y i n mu l t i p l e my e l o ma
(1) .
I I . Evaluation
A. History
Proteinuria is usually identified on a urinalysis obtained for a routine physical

e xa mi n a t i o n . S o me t i me s t h e p a t i e n t r e p o r t s f o a my u r i n e , w h i c h i s d u e t o a n a l t e r a t i o
of the surface tension by the proteins.
C h e c k v i t a l s i g n s , e s p e c i a l l y b l o o d p r e s s u r e . P e r f o r m a f u n d u s c o p i c e xa mi n a t i o n ,
c h e c k i n g f o r d i a b e t i c r e t i n o p a t h y o r v a s c u l a r c h a n g e s f r o m h y p e r t e n s i o n . E d e ma o f
t h e l e g s o r f a c e ma y b e d u e t o h y p o a l b u mi n e mi a . C h e c k t h e a b d o me n f o r ma s s e s
(such as polycystic kidneys) and renal artery bruits. Look for evidence of
r h e u ma t o l o g i c j o i n t c h a n g e s .
C. Testing
592 / 652
tmdmss
16.7 - Proteinuria
1. A d i p s t i c k t e s t o f t h e u r i n e p r e f e r e n t i a l l y i d e n t i f i e s a l b u mi n . I t c a n b e f a l s e l y
negative when the urine is dilute (specific gravity <1.015) and when the protein
a r e o f l o w mo l e c u l a r w e i g h t . T h e d i p s t i c k c a n b e f a l s e l y p o s i t i v e w i t h a l k a l i n e
u r i n e , g r o s s h e ma t u r i a , p u s , s e me n , v a g i n a l s e c r e t i o n s , a n d t h e p r e s e n c e o f
penicillin
P. 3 6 4
a n d s u l f o n a mi d e s . T h e p r o t e i n u r i a i s g r a d e d a s 3 0 mg / d L ( 1 + ) , 1 0 0 mg / d L ( 2 + ) ,
3 0 0 mg / d L ( 3 + ) , a n d 1 , 0 0 0 mg / d L ( 4 + ) .
2. A f t e r p e r s i s t e n t p r o t e i n u r i a h a s b e e n c o n f i r me d , a q u a n t i t a t i v e me a s u r e me n t
should be obtained. T he gold standard has been the 24-hour urine collection.
Another option is the urine protein-to-creatinine ratio ( U P r/ C r), which can be
d e t e r mi n e d o n a r a n d o m u r i n e s p e c i me n . A r a t i o < 0 . 2 i s n o r ma l , w h e r e a s a r a t i o
mo r e t h a n 3 . 0 i s i n t h e n e p h r o t i c r a n g e . T h e f l a g f o r mi c r o a l b u mi n u r i a h a s b e e n
s e t a t 3 0 mg / 2 4 h o u4)r s. (
3. T h e n e xt s t e p i s t o l o o k f o r a n u n d e r l y i n g c a u s e t h a t c a n b e t r e a t e d . T h e
d i f f e r e n t i a l d i a g n o s i s i n c l u d e s d i a b e t e s me l l i t u s , g l o me r u l o n e p h r i t i d e s w i t h l o w
c o mp l e me n t l e v e l s , s t r e p t o c o c c a l g l o me r u l o n e p h r i t i s , s y s t e mi c l u p u s , mu l t i p l e
my e l o ma , a n d s a r c o i d o s i s . H e ma t u r i a ma y i n d i c a t e i n f e c t i o n , s t o n e s , o r n e p h r i t i
(4,5) .
4. A n y a d u l t o r c h i l d w i t h p r o t e i n u r i a o r h e ma t u r i a w i t h o u t a c l e a r d i a g n o s i s s h o u l d
h a v e a n e p h r o l o g y c o n s u l t . T h i s i s e s p e c i a l l y t r u e i f t h e p r o t e i n u r i a i s > 2 g / d a y,
b e c a u s e t h e p e r s o n mo s t l i k e l y h a s s o me t y p e o f g l o me r u l a r d i s e a s e . M o s t o f
t h e s e p a t i e n t s w i l l n e e d a r e n a l b i o p s y. O n e e xc e p t i o n i s p a t i e n t s w i t h
p o s t i n f e c t i o u s g l o me r u l o n e p h r i t i s , w h i c h i s u s u a l l y s 4)
e l .f - l i mi t i n g (
I I I . Diagnosis
A. T r a n s i e n t p r o t e i n u r i a d o e s n o t r e q u i r e f u r t h e r e v a l u a t i o n o r mo n i t o r i n g .
O r t h o s t a t i c p r o t e i n u r i a a c c o u n t s f o r u p t o 6 0 % o f a s y mp t o ma t i c p r o t e i n u r i a i n
individuals 6 to 30 years of age. T his condition appears to be benign, although
y e a r l y f o l l o w - u p i s r e c o mme n d e d .
B. D e h y d r a t i o n , f e v e r, i n t e n s e p h y s i c a l a c t i v i t y, e mo t i o n a l s t r e s s , a n d s e i zu r e s c a n
c a u s e b e n i g n p r o t e i n u r i a . S e c o n d a r y g l o me r u l o n e p h r o p a t h y o c c u r s w i t h
d i a b e t e s me l l i t u s , l u p u s , a my l o i d o s i s , p r e e c l a mp s i a , r e c e n t s t r e p t o c o c c a l
i n f e c t i o n s , e n d o c a r d i t i s , h u ma n i mmu n o d e f i c i e n c y v i r u s , a n d h e p a t i t i s B a n d C .
T u b u l a r n e p h r o p a t h y ma y b e d u e t o h y p e r t e n s i o n , s i c k l e - c e l l d i s e a s e , o r u r a t e
stones.
C. G o l d , p e n i c i l l a mi n e , l i t h i u m, a n d h e r o i n c a n c a u s e g l o me r u l o n e p h r o p a t h y.
N o n s t e r o i d a l a n t i - i n f l a mma t o r y d r u g s a n d h e a v y me t a l s c a n d a ma g e b o t h t h e
g l o me r u l i a n d t h e t u b u l e s . P a t i e n t s w i t h g l o me r u l o n e p h r o p a t h y s h o u l d b e c l o s e l y
mo n i t o r e d w i t h r e g a r d t o p r o t e i n u r i a a n d h y p e r l i p i d e mi a .
D. A h i s t o r y o f p o l y c y s t i c k i d n e y d i s e a s e , h y p e r t e n s i o n , d i a b e t e s , o r a u t o i mmu n e
d i s e a s e s u c h a s l u p u s ma y p o i n t t o t h e d i 5)
a g. n o s i s (
593 / 652
tmdmss
16.7 - Proteinuria
References
1 . D e v u y u s t O , D a h a n K , P i r s o n Y. T a mm- H o r s f a l l p r o t e i n o r N
u rephr
o mool
dulin.
D i al Tr ans pl ant
2005;20(7):12901294.
2 . L o g h ma n - A d h a m M . E v a l u a t i n g p r o t e i n u r i a i n Ac hmi l dF ram
e n . P hy s i c i an
1 9 9 8 ; 5 8 ( 5 ) : 11 4 5 11 5 8 .
3 . M o l i t c h M R , D e F r a zo R A , F r a n z M J , e t a l . N e p h r o p a t h y iD
n i dabet
i a b ees
tes.
C ar e 2 0 0 4 ; 2 7 ( s u p p l 1 ) : S 7 9 8 3 . Av a i l a b l e a t :
http://care.diabetesjounals.org/cgi/content/full/27/suppl_1/s79#B IB L.
4 . H a s s a n A . P r o t e i n u rPi os
a . t gr ad M ed
1997;101(4):173180.
5 . C a r r o l l M F, T e mt e J L . P r o t e i n u r i a i n Aam
d uFl tam
s . P hy s i c i an
2000;62(2):13331342.
594 / 652
tmdmss
17.1 - Alkaline Phosphatase, Elevated
> T a b l e o f C o n t e n t s > 1 7 - L a b o r a t o r y A b n o r ma l i t i e s : B l o o d C h e mi s t r y a n d
I mmu n o l o g y > 1 7 . 1 - A l k a l i n e P h o s p h a t a s e , E l e v a t e d
17.1
Alkaline Phosphatase, Elevated
Jose ph B. Straton
P e t e r F. C r o n h o l m
I . Background
S e r u m a l k a l i n e p h o s p h a t a s e ( A L P ) a r i s e s p r i ma r i l y f r o m t h e l i v e r a n d b o n e a l t h o u g h
s ma l l a mo u n t s a r e d e r i v e d f r o m t h e i n t e s t i n e s a n d t h e v a s c u l a r e n d o t h e l i u m. T h e
n o r ma l r a n g e o f s e r u m A L P v a r i e s b y a g e a n d c l i n i c a l h i s t o r y. T h e n o r ma l r a n g e s f o r
a d o l e s c e n t s , a d u l t s o l d e r t h a n 6 0 y e a r s , a n d p r e g n a n t w o me n , a r e h i g h e r t h a n f o r
n o n p r e g n a n t w o me n y o u n g e r t h a n 6 0 y e a r s . N o r ma l r a n g e s a r e i n f a n t : 5 0 1 6 5 U / L ,
child: 20150 U/L, adult: 2070 U/L, adult older than 60 years: 3075 U/L.
Serum AL P should be ordered only if bone or liver disease is suspected. AL P results
s h o u l d b e c o mp a r e d w i t h a p p r o p r i a t e n o r ma l r a n g e s o n t h e b a s i s o f t h e a g e a n d
c l i n i c a l h i s t o r y. A b n o r ma l r e s u l t s s h o u l d b e r e p e a t e d , b e c a u s e s p u r i o u s e l e v a t i o n s
c a n b e c a u s e d b y a r e c e n t a l b u mi n i n f u s i o n , t h e u s e o f a n a n t i c o a g u l a n t t u b e f o r t h e
b l o o d c o l l e c t i o n , o r b y s e r u m s a mp l e s l e f t s t a n d i n g a t r o o m t e mp e r a t u r e f o r
prolonged periods.
A. T o i d e n t i f y t h e s o u r c e o f A L P e l e v a t i o n s , o n e c a n u s e A L P i s o e n zy me t e s t i n g .
A l t e r n a t i v e l y, o n e c a n u s e t h e r e s u l t s o f - g l u t a my l t r a n s f e r a s e ( G G T ) o r 5 nucleotidase testing to identify the source. In liver disorders, G G T or 5n u c l e o t i d a s e a r e u s u a l l y e l e v a t e d w i t h A L P. A L P e l e v a t i o n i n t h e s e t t i n g o f
n o r ma l G G T o r 5 - n u c l e o t i d a s e s u g g e s t s a b o n e s o u r c e o f A L P.
B. U n c o mmo n c a u s e s o f A L P e l e v a t i o n i n c l u d e h y p e r t h y r o i d i s m, v i t a mi n D
d e f i c i e n c y, h e p a t i c i n f i l t r a t i o n ( d u e t o l y mp h o p r o l i f e r a t i v e d i s o r d e r s ,
g r a n u l o ma t o u s d i s e a s e , o r p r i ma r y a n d s e c o n d a r y ma l i g n a n c i e s o f t h e l i v e r ) ,
i n t e s t i n a l c o n d i t i o n s s u c h a s b o w e l o b s t r u c t i o n , a n d i n f a r c t i o n . A d d i t i o n a l l y,
i n f a r c t i o n o f a n y s o l i d o r g a n ma y c a u s e A L P e l e v a t i o n o w i n g t o t h e p r e s e n c e o f
A L P i n t h e v a s c u l a r e n d o t h e l i u m.
I I I . Evaluation
F u r t h e r t e s t i n g s h o u l d b e p e r f o r me d o n t h e b a s i s o f t h e r e s u l t s o f a c a r e f u l h i s t o r y
a n d p h y s i c a l e xa mi n a t i o n a n d s h o u l d b e t a r g e t e d t o s p e c i f i c c l i n i c a l s u s p i c i o n s
(T a b l e 1 7 . 1). .1
I V. Diagnosis
S e e T a b l e 1 7 . 1.. 1
P. 3 6 8
P. 3 6 9
P. 3 7 0
TAB L E 17.1.1 Evaluation of the Patient with Elevated Alkaline

Phosphatase
595 / 652
tmdmss

History
Te s t i n g
Diagnosis
A. Bone disorde rs (1)

P aget's disease
2 4 - h u r i n e h y d r o xy p r o l i n e
Serum phosphate
S e r u m c a l c i u m ( n o r ma l )
X-ray involved bone(s)
Bone scan
A s y mp t o ma t i c o r
bone pain
Male
Hearing/vision
p r o b l e ms
Headaches
Pain increased
with walking
(tibia involved)
Frontal bossing
Dilated superficial
vessels
Saber tibia
Deafness
Congestive heart
failure
F e ma l e
>60 y
Diffuse aches
and pains
Va g u e
a b d o mi n a l p a i n
Depressive
s y mp t o ms
Renal calculi
N e c k ma s s ( r a r e )
Muscle weakness
No clinical evidence of
ma l i g n a n c y
P a r a t h y r o i d h o r mo n e
Serum calcium
U r i n a r y c a l c i u m/ b l o o d
Serum phosphate
Serum chloride
>50 y
U n e xp l a i n e d
weight loss
S mo k e r
Cough
H e mo p t y s i s
Shortness of
breath
Decreased air entry

( w h e e ze )
Pleural effusion
Dull to percussion
H o r n e r ' s s y n d r o me
C h e s t x- r a y
Chest C T scan
Bone scan
Serum calcium
Lung cancer
F e ma l e
>50 y
F a mi l y h i s t o r y
B r e a s t ma s s
B r e a s t ma s s
A xi l l a r y / s u p r a c l a v i c u l a r
nodes
L i v e r e n l a r g e me n t
M a mmo g r a m
Bone scan
Serum calcium
Breast cancer
F e ma l e
>45 y
A b d o mi n a l
bloating
U n e xp l a i n e d
weight loss
O v a r i a n ma s s
Ascites
Pelvic ultrasound
Pelvic C T scan
C A-125
C T scan
Serum calcium
Ovarian cancer
Male
>50 y
H e ma t u r i a
F l a n k / a b d o mi n a l
pain
U n i l a t e r a l f l a n k ma s s
Conjunctival pallor
Urinalysis
A b d o mi n a l C T s c a n
Serum calcium
Renal cell
c a r c i n o ma
Hyperparathyroidism
596 / 652
tmdmss

Male
>50 y
Urinary
c o mp l a i n t s
P r o s t a t e ma s s o n
r e c t a l e xa mi n a t i o n o r
diffusely enlarged hard
prostate
Serum calcium
Prostate-specific antigen
Ultrasound-guided
prostate biopsy
Bone scan
Prostate cancer
1030 y
Male
Pain near joint
Ve r y h i g h A L P
Mass near joint

T e n d e r o v e r ma s s
X - r a y a r e a ( mi xe d
sclerotic/lytic lesion of
bone)
Magnetic resonance
i ma g i n g o f a f f e c t e d r e g i o n
Bone scan
Biopsy
O s t e o s a r c o ma
R e c e n t t r a u ma
Bone pain in area
X - r a y ( c a l l o u s f o r ma t i o n )
Healing fracture
B. Biliary /liv e r dise ase (2)
A L P 2 t i me s
n o r ma l
Alcohol
use/abuse
(chronic)
of liver disease
R i s k y s e xu a l
practices
Blood
transfusions
Intravenous
drug use
Obesity
Fatigue
We i g h t l o s s
Spider nevi
Leukonychia
D upuytren's
contracture
Tender R U Q
L i v e r ma y b e e n l a r g e d
Jaundice
S e r u m A S T / A LT l e v e l s
in early disease
late disease
Bilirubin (elevated)
H epatitis screen ( A, B , C )
Coagulation screen
Liver biopsy
Cirrhosis
Hepatitis
Fatty liver
F e ma l e
>40 y
Obese
of gallstones
Pain after
me a l s / e p i s o d i c
Bloating/gas
R U Q a b d o mi n a l
tenderness + Murphy's
sign (only in acute
cholecystitis)
Jaundice
A S T / A LT ma y b e e l e v a t e d
Bilirubin elevated
Ultrasound of gallbladder
Gallstones
Biliary colic
Acute
cholecystitis
A L P 5 t i me s
n o r ma l
1025 y old
Fever
Sore
throat/fatigue
History of
contact with
Fever
Tender R U Q
S p l e n o me g a l y
( ma i n l y c e r v i c a l )
Monospot test
A S T / A LT
C o mp l e t e b l o o d c o u n t
T o xo p l a s ma t i t e r s
Infectious
mo n o - n u c l e o s i s
T o xo p l a s mo s i s
C y t o me g a l o v i r a l
infection
597 / 652
tmdmss

infected
friend/relative
A L P 1 0 t i me s
n o r ma l
We i g h t l o s s
A n o r e xi a
Back or R U Q
pain
Jaundice
Palpable gallbladder
Cachectic
E p i g a s t r i c ma s s
Jaundice
A b d o mi n a l C T s c a n
Elevated bilirubin
C T scan or ultrasoundguided biopsy
Pancreatic
c a r c i n o ma
Gallbladder
c a r c i n o ma
AL P 1020
t i me s n o r ma l
C h l o r p r o p a mi d e
use
Antineoplastic
agents
I mmu n e
mo d u l a t o r s
F r e q u e n t l y n o r ma l
R U Q tenderness
A S T / A LT
Liver biopsy if problem
persists (3)
Drug-induced
elevation of
AL P
A L P 5 2 0 t i me s
n o r ma l
F e ma l e
3560 years of
age (90%)
Frequently
Itching
( p a l ms / s o l e s
first)
Fatigue
Bone pain
Steatorrhea
Jaundice
E xc o r i a t i o n s
Jaundice
S k i n p i g me n t a t i o n
R U Q tenderness
X a n t h e l a s ma
Liver/spleen enlarged
A n t i mi t o c h o n d r i a l
antibodies
Liver biopsy
Elevated bilirubin (late)
E l e v a t e d l i v e r e n zy me s
(late)
Cholesterol elevated
P r i ma r y b i l i a r y
cirrhosis
AL P >520
n o r ma l
Male
3060 y of age
R U Q pain
Jaundice
Pruritus
bowel disease
Fatigue
R U Q tenderness
Jaundice
Endoscopic retrograde
cholangiopancreatography
Sclerosing
cholangitis
( p r i ma r y o r
secondary)
C T , c o mp u t e d t o mo g r a p h y ; A L P, a l k a l i n e p h o s p h a t a s e ; A S T , a s p a r t a t e a mi n o t r a n s f e r a s e ; A LT , a l a n i n e
a mi n o t r a n s f e r a s e ; R U Q , r i g h t u p p e r q u a d r a n t .
References
1 . T a y l o r A K , L u e k e n S A , L i b a n a t i C , e t a l . B i o c h e mi c a l ma r k e r s o f b o n e t u r n o v e r
f o r t h e c l i n i c a l a s s e s s me n t o f b o n e me t aCbl oi nl i sRm.
heum D 1i s9 9 4 ; 2 0 : 5 8 9
598 / 652
tmdmss
607.
2 . P r a t t D S , K a p l a n M M . E v a l u a t i o n o f a b n o r ma l l i v e r - e n zy me r e s u l t s i n
a s y mp t o ma t i c p a t i e nNt sE. ngl J M ed2 0 0 0 ; 3 4 2 : 1 2 6 6 .
3 . S o r b i D , M c G i l l D B , T h i s t l e J L , e t a l . A n a s s e s s me n t o f t h e r o l e o f l i v e r
b i o p s i e s i n a s y mp t o ma t i c p a t i e n t s w i t h c h r o n i c l i v e r t e s t a bAnmo rJma l i t i e s .
G as t r oent er ol
2000;95:3206.
599 / 652
tmdmss
17.2 - Aminotransferase Levels, Elevated
I mmu n o l o g y > 1 7 . 2 - A mi n o t r a n s f e r a s e L e v e l s , E l e v a t e d
17.2
Aminotransferase Levels, Elevated
Jose ph B. Straton
I . Background
L i v e r f u n c t i o n t e s t s ( L F T s ) a r e a mi s n o me r i n t h a t h e p a t o c e l l u l a r i n j u r y a n d
c h o l e s t a s i s a r e a c t u a l l y t h e c o n d i t i o n s b e i n g me a s u r e d r a t h e r t h a n i t s f u n c t i o n .
T h e s e t e s t s i n c l u d e a s p a r t a t e a mi n o t r a n s f e r a s e ( A S T ) ( o r s e r u m g l u t a mi c o xa l o a c e t i c t r a n s a mi n a s e [ S G O T ] ) , a l a n i n e a mi n o t r a n s f e r a s e ( A LT ) ( o r s e r u m
g l u t a mi c - p y r u n i c t r a n s a mi n a s e [ S G P T ] ) , a l k a l i n e p h o s p h a t a s e , a n d s e r u m b i l i r u b i n .
E v a l u a t i o n o f a b n o r ma l i t i e s s h o u l d i n c l u d e a n a s s e s s me n t o f me d i c a t i o n u s e
( i n c l u d i n g o v e r - t h e - c o u n t e r a n d h e r b a l me d i c a t i o n s ) , t r a v e l , a l c o h o l c o n s u mp t i o n a n d
r e c r e a t i o n a l d r u g u s e , s e xu a l b e h a v i o r, s i g n s a n d s y mp t o ms o f h e p a t i c i n j u r y, a n d a
t h o r o u g h p h y s i c a l e xa mi n a t i o n . A LT a n d A S T ma y a l s o b e e l e v a t e d i n my o c a r d i a l
i n f a r c t i o n s , h e mo l y t i c a n e mi a , a f t e r t r a u ma , a n d i n t r a mu s c u l a r i n j e c t i o n s .
I I I . Evaluation
A t h o r o u g h c l i n i c a l h i s t o r y a n d e xa mi n a t i o n a r e e s s e n t i a l i n g u i d i n g t h e t e s t i n g a n d
i n t e r p r e t i n g t h e r e s u l t s , b e c a u s e p a t t e r n s o f a b n o r ma l i t i e s i n l i v e r t e s t i n g a r e b e t t e r
p r e d i c t o r s o f c l i n i c a l d i s e a s e t h a n a n y s i n g l e c o mp o n e n t . F o r f u r t h e r d e t a i l s , s e e
T a b l e 1 7 . 2.. 1
A. Diagnosis
S e e T a b l e 1 7 . 2.. 1
P. 3 7 2
P. 3 7 3
P. 3 7 4
TAB L E 17.2.1 Evaluation of the Patient with Elevated Am inotransferase

History
A LT a n d A S T
can be
ma r k e d l y
elevated with
ratio usually
less than one
Alkaline
phosphatase
and serum
b i l i r u b i n ma y
be elevated or
n o r ma l
Patient often
Jaundice
Clay-colored stools
Dark urine
Left upper quadrant
tenderness
H e p a t o me g a l y
Urticaria
Maculopapular skin
eruptions
Isolated joint
swelling, redness, or
tenderness
Te s t i n g
Serial testing of
a mi n o t r a n s f e r a s e s
CBC
Hepatitis A IgM
Hepatitis B surface
antigen, IgM anti-hepatitis
B surface antigen
a n t i b o d y, a n d I g M a n t i
hepatitis B core antibody
IgG antihepatitis C
antibody
Epstein-Barr virus or
c y t o me g a l o v i r u s t i t e r s
Diagnosis
Acute viral hepatitis
600 / 652
tmdmss

o r e xp e r i e n c e s
transient
flulike illness
Antecedent
illness of
several days to
weeks with
nausea,
v o mi t i n g ,
a n o r e xi a ,
ma l a i s e ,
diarrhea,
arthralgias, or
low-grade
fever
Recent
shellfish
ingestion
R i s k y s e xu a l
practices
Past or
present
intravenous
drug use
History of
blood
transfusions or
tattoos
A LT a n d A S T
can (1) be
ma r k e d l y
elevated with
ratio usually
less than one
E xc e s s i v e
s o mn o l e n c e
Obtundation
History of
rectal or upper
G I bleeding
Rapidly
progressive
course in 65%
95% of
patients
S y mp t o ms o f
sepsis with or
without
mu l t i o r g a n
failure
Prior hepatitis
History of
aspirin
ingestion in
children
younger than
17 y with
i n f l u e n za o r
Liver biopsy if diagnosis

c a n n o t b e d e t e r mi n e d o r
t o g u i d e t r e a t me n t f o r
hepatitis C
L i v e r ma y b e
r e d u c e d i n s i ze
Ascites
Refractory
hypotension
Petechia
Bleeding from
mu c o u s me mb r a n e s
E d e ma
P r o t h r o mb i n t i me
(profoundly prolonged)
Low blood glucose
Low total serum protein
a n d s e r u m a l b u mi n
CBC
S e r u m a mmo n i a ( ma y b e
severely elevated)
F u l mi n a n t h e p a t i t i s
Acute hepatic failure
associated with
R e y e ' s s y n d r o me
1% of the elderly or
i mmu n o c o mp r o mi s e d
patients with
hepatitis
601 / 652
tmdmss

chickenpox
T o xi c d o s e s o f
a c e t a mi n o p h e n
Alcoholic hepatitis
A S T : A LT > 2 : 1
(2)
Chronic or
acute alcohol
ingestion
Yo u n g e r a g e
drinker
History of
pancreatitis or
erosive
gastritis
Cirrhotic liver
disease
A n o r e xi a
Nausea
Vo mi t i n g
A b d o mi n a l
pain
Jaundice
Fever
We i g h t l o s s
H e p a t o me g a l y w i t h
mi l d t e n d e r n e s s
Advanced disease
can be
c h a r a c t e r i ze d b y
s p i d e r a n g i o ma s ,
a s c i t e s , p a l ma r
e r y t h e ma , c a p u t
me d u s a e ,
g y n e c o ma s t i a ,
p a r o t i d e n l a r g e me n t ,
and testicular
atrophy
A LT a n d A S T
are variably
elevated
Often
Previous
episode of
acute hepatitis
Coagulopathy
May be no findings
(3)
T hin
Jaundiced
S ma l l , n o d u l a r l i v e r
Alkaline
phosphatase
e l e v a t e d mo r e
t h a n t h e A LT
or AS T
Elevated
serum bilirubin
R U Q pain
Gallstones
Middle aged
Overweight
F e ma l e
Jaundice
I n t e r mi t t e n t f e v e r
Rigors
R U Q tenderness
rebound
Can present
as
hepatocellular
i n j u r y,
obstructive or
We i g h t l o s s
Ascites
G I ma l i g n a n c i e s c a n
be left
supraclavicular
- G l u t a my l t r a n s f e r a s e
C B C ( p o s s i b l e a n e mi a )
Chronic hepatitis
Hepatitis B surface
antigen, IgM anti
hepatitis B surface
a n t i g e n a n t i - b o d y, a n d
IgM/G antihepatitis B
core and envelope
antibody
IgG antihepatitis C
antibody
A l b u mi n ( o f t e n l o w )
P r o t h r o mb i n t i me ( ma y b e
elevated)
Liver biopsy (4)
Biliary tract obstruction

CBC
with or without an
R U Q ultrasound
infection (cholestasis vs.
Endoscopic retrograde cholangitis)
cholangiopancreatography
o r i ma g i n g e q u i v a l e n t
H ID A scan can
d e mo n s t r a t e b i l i a r y
function in the setting of
an obstructive
presentation but an
i n d e t e r mi n a t e u l t r a s o u n d
M a l i g n a n c y ( p r i ma r y o r
MRI
me t a s t a t i c )
S erum -fetoprotein
D e t e r mi n a t i o n o f p r i ma r y
s o u r c e o f ma l i g n a n c y w i t h
t e s t i n g s p e c i f i c t o p r i ma r y
602 / 652
tmdmss

a c o mb i n a t i o n
profile
Known or
unknown
ma l i g n a n c y
A b d o mi n a l
pain
We a k n e s s
A n o r e xi a
History of
hepatitis B or
C infection
( Vi r c h o w ' s ) o r
p e r i u mb i l i c a l ( S i s t e r
M a r y J o s e p h ) l y mp h
n o d e e n l a r g e me n t
Other findings
specific to the
p r i ma r y s i t e
ma l i g n a n c y s i t e
Use of:
Often none
HMG-CoA
Reductase
inhibitor
I s o n i a zi d
P h e n o t h i a zi n e
E r y t h r o my c i n
Progesterone
Halothane
Opiates
I n d o me t h a c i n
Corticosteroids
in question
Medication effect
R e p e a t A LT a n d A S T
after discontinuation of
t h e me d i c a t i o n
Other testing specific to
me d i c a t i o n e f f e c t s ( e . g . ,
creatinine kinase levels
w i t h t o xi c i t y o f H M G - C o A
reductase inhibitors)
Hepatic steatosis and

Ultrasound, C T scan, or nonalcoholic
MRI
steatohepatitis
Liver biopsy is necessary
t o d e t e r mi n e i n f l a mma t i o n
Fatigue,
ma l a i s e , a n d
vague right
upper
a b d o mi n a l
d i s c o mf o r t
A S T : A LT r a t i o
<1
Hepatitis with
no clear
etiology
Obesity
Polyuria,
polyphagia,
and polydipsia
We a k n e s s a n d
lethargy
Arthralgia
I mp o t e n c e i n
me n
We a k n e s s
Skin
h y p e r p i g me n t a t i o n
D i a b e t e s me l l i t u s
Electrocardiographic
a b n o r ma l i t i e s
Serum iron
T IBC
Iron saturation (ratio of
serum iron to T IB C) >45
Ferritin
Liver biopsy for
d e t e r mi n a t i o n o f h e p a t i c
iron index
H b AI c
May describe
mu s c l e p a i n o r
weakness
Muscle pain or
weakness on
e xa mi n a t i o n
Creatine kinase
Aldolase
Muscle biopsy
Unintentional
weight loss or
Goiter and/or
thyroid nodule(s) in
T hyroid disorders (5)

TSH
T hyroid function testing if
Muscle disorders
603 / 652
tmdmss

gain
Skin and hair
changes
Heat or cold
intolerance
certain conditions
Hair loss
A b n o r ma l r e f l e xe s
Hepatitis or
liver failure
E mp h y s e ma
when young or
out of
proportion with
s mo k i n g
history
Physical
e xa mi n a t i o n r a n g i n g
from acute hepatitis
to end-stage liver
disease
P u l mo n a r y
e xa mi n a t i o n
suggestive of ends t a g e p u l mo n a r y
disease
Child or young
adult (525 y)
Hepatitis
Dysarthria
Dysphagia
Kayser-Fleischer
rings in cornea
T r e mo r s
T S H i s a b n o r ma l
-1-antitrypsin deficiency
-1-antitrypsin phenotype
Serum protein
electrophoresis
Wilson's disease
Liver function tests often
nonspecific
C e r u l o p l a s mi n ( r e d u c e d i n
85% of patients)
24-h urine for quantitative
c o p p e r e xc r e t i o n > 1 0 0
g/d is suggestive)
A LT , a l a n i n e a mi n o t r a n s f e r a s e ; A S T , a s p a r t a t e a mi n o t r a n s f e r a s e ; C B C , c o mp l e t e b l o o d c o u n t ; I g M ,
i mmu n o g l o b u l i n M ; G I , g a s t r o i n t e s t i n a l ; R U Q , r i g h t u p p e r q u a d r a n t ; M R I , ma g n e t i c r e s o n a n c e i ma g i n g ; H M
C o A , 3 - h y d r o xy - 3 - me t h y l g l u t a r y l c o e n zy me A ; T I B C , t o t a l i r o n b i n d i n g c a p a c i t y ; T S H , t h y r o i d - s t i mu l a t i n g
h o r mo n e .
References
1. C ohen J A, K aplan M M . T he S G O T / S G P T ratio an indicator of alcoholic liver
d i s e a s eD
. i g D i s S c1i 9 7 9 ; 2 4 : 8 3 5 .
2 . K a p l a n M M . A l a n i n e a mi n o t r a n s f e r a s e l e v e l s : w h a t ' s n o r ma l ?A nn
(editorial).
I nt er n M ed2 0 0 2 ; 1 3 7 : 5 0 .
3 . P r a t t D S , K a p l a n M M . E v a l u a t i o n o f a b n o r ma l l i v e r - e n zy me r e s u l t s i n
a s y mp t o ma t i c p a t i e nNt sE. ngl J M ed2 0 0 0 ; 3 4 2 : 1 2 6 6 .
6 . S o r b i D , M c G i l l D B , T h i s t l e J L , e t a l . A n a s s e s s me n t o f t h e r o l e o f l i v e r
b i o p s i e s i n a s y mp t o ma t i c p a t i e n t s w i t h c h r o n i c l i v e r t e s t a bAnmo rJma l i t i e s .
G as t r oent er ol
2000;95:3206.
4 . H u a n g M J , L i a w Y F. C l i n i c a l a s s o c i a t i o n s b e t w e e n t h y r o i d a n d l i v e r d i s e a s e s .
J G as t r oent er ol H epat1 9ol9 5 ; 1 0 : 3 4 4 .
5 . A b d o A , M e d d i n g s J , S w a i n M . L i v e r a b n o r ma l i t i e s i n c e l iC
a cl i nd i s e a s e .
G as t r oent er ol H epat2 ol
004;2:107.
604 / 652
tmdmss
17.3 - Antinuclear Antibody Titer, Elevated
I mmu n o l o g y > 1 7 . 3 - A n t i n u c l e a r A n t i b o d y T i t e r, E l e v a t e d
17.3
Antinuclear Antibody Titer, Elevated
Jose ph B. Straton
I . Background
Antinuclear antibodies ( AN As) include antibodies to double-stranded D N A, histones,
c h r o ma t i n , a l o n g w i t h o t h e r n u c l e a r p r o t e i n s a n d R N A p r o t e i n c o mp l e xe s . T h e
p r e s e n c e o f s i g n i f i c a n t A N A t i t e r s i s a n e c e s s a r y c o mp o n e n t i n d i a g n o s i n g s y s t e mi c
a u t o i mmu n e d i s e a s e s , b u t t h e y c a n a l s o b e f o u n d i n o t h e r w i s e n o r ma l i n d i v i d u a l s .
B e c a u s e o f t h e l o w s p e c i f i c i t y o f a p o s i t i v e A N A t i t e r, a n A N A t i t e r s h o u l d o n l y b e
ordered when there is clinical suspicion of a disease process in which the AN A value
p l a y s a s i g n i f i c a n t r 1)
o l .e (
AN A-staining patterns are not as useful as was once believed in correlating patterns
w i t h c l i n i c a l d i s e a s e , b u t a r e o f t e n r e p o r t e d w i t h t i t e r s . I t i s i mp o r t a n t t o r e me mb e r
t h a t t h e s p e c i f i c i t y o f a p o s i t i v e A N A t i t e r f o r a l l r h e u ma t i c d i s e a s e s i s o n l y 5 0 % .
Five percent of young adults and 18% of individuals older than 65 years have a
mi l d l y e l e v a t e d A N A a n d n o d i s e a s e p r o c e s s ( a f a l s e l y p o2)
s i.t i v e A N A ) (
I I I . Evaluation
A c a r e f u l h i s t o r y a n d p h y s i c a l e xa mi n a t i o n a l o n g w i t h a n u n d e r s t a n d i n g o f t h e
p r e v a l e n c e o f s y s t e mi c a u t o i mmu n e d i s e a s e s s h o u l d g u i d e t h e o r d e r i n g a n d
i n t e r p r e t a t i o n o f A N A t i t e r s . F o r f u r t h e r d eT
t aai bl sl e, s1e7e. 3.. 1
P. 3 7 6
P. 3 7 7
P. 3 7 8
I V. Diagnosis
S e e T a b l e 1 7 . 3.. 1
TAB L E 17.3.1 Evaluation of the Patient with an Elevated

Antinuclear Antibody Titer
History
Furthe r te sting
Diagnosis
A. Conne ctiv e tissue disorde rs

SLE
Fatigue
Fever
Malar rash (presents

in 1/31/2 of patients)
Anti-ds- D N A (high
specificity for
605 / 652
tmdmss

We i g h t l o s s
Pain, redness,
or heat in two
o r mo r e j o i n t s
Photosensitivity
reaction
Rash
Oral ulcers
Chest pain
Shortness of
breath
S e i zu r e s o r
psychosis
without history
of of-fending
me d i c a t i o n s o r
drug use
A b d o mi n a l p a i n
M o r e c o mmo n
i n w o me n a n d
AfricanA me r i c a n s
Discoid rash
Joint effusion or
d e r a n g e me n t i n
chronic disease
(present in 2/3 of
patients)
Focal neurologic
deficits (15% of
patients)
Pleural effusions
Cardiac or pleural
rubs
SLE)
Urinalysis with
24-h collection
(look for
persistent
proteinuria or
casts)
C B C (look for
evidence of
a n e mi a )
S p e c i a l i ze d
nuclear antigen
tests:
ribonucleoprotein,
antibodies to antiS mi t h , a n t i - S S A/ R o, anti- S S B/La (patients
w i t h S L E ma y
produce different
autoantibodies)
Creatinine (look
for occult renal
disease)
X-rays of involved
joints
S y s t e mi c
s y mp t o ms :
f e v e r, w e i g h t
loss, and
fatigue
Morning
stiffness
Chronic,
s y mme t r i c j o i n t
c o mp l a i n t s
( t h r e e o r mo r e
f o r 6 o r mo r e
wk)
J o i n t s y mp t o ms
are often
i n t e r mi t t e n t a n d
mi g r a t o r y
Red, hot, swollen

j o i n t ( s ) ( mo s t c o mmo n
are the wrist,
me t a c a r p o p h a l a n g e a l ,
o r p r o xi ma l i n t e r p h a langeal joints)
Subcutaneous
nodules (usually on
t h e e xt e n s o r o r
pressure surfaces)
R h e u ma t o i d f a c t o r
(20% of patients
with R A are
negative)
C B C (look for an
a n e mi a o f c h r o n i c
disease)
X-rays of involved
joints (typically
d e mo n s t r a t e s
erosions or bone
loss)
Joint aspiration
( i n f l a mma t o r y
profile to synovial
fluid)
E xc e s s i v e
dryness of eyes
a n d / o r mo u t h
Recurrent oral
ulcers
Sensation
Enlarged salivary
glands
D r y mu c o u s
me mb r a n e s
Decreased salivation
Decreased tearing
RA
S jgren's
Anti- S S - A/ R o
s y n d r o me
Anti- S S - B / L a
Biopsy of salivary
glands or lip to
assess
l y mp h o c y t i c
606 / 652
tmdmss

disturbance
over the hands
and/or feet
Va g i n a l d r y n e s s
and
dyspareunia
Dysphagia
Purpura
Peripheral
neuropathy
infiltration
C B C (looking for
a n e mi a o f c h r o n i c
disease)
Cryoglobulins (if
positive, should
screen for
hepatitis C)
I mmu n o g l o b u l i n
electrophoresis
( t o d e mo n s t r a t e a
mo n o c l o n a l s p i k e )
Objective testing
of tear and saliva
production
( S c h i r me r t e s t
and salivary
s c i n t i g r a m)
C h e s t x- r a y t o
differentiate from
possible
sarcoidosis
Fever and
ma l a i s e
We i g h t l o s s
Muscle
tenderness
Muscle
weakness is
usually
s y mme t r i c ,
gradual in
onset, greater
loss in lower
l i mb s
Skin rash
Arthralgia
Chest pain or
shortness of
breath with
p u l mo n a r y
M u s c l e s t r e n g t h ma y
b e d i mi n i s h e d
Tenderness to
palpation over
affected areas
Skin rashes:
heliotrope rash on
eyelids or
e r y t h e ma t o u s p a p u l e s
o v e r e xt e n s o r
surfaces of joints
( p r o xi ma l i n t e r p h a l a n g e a l , e l b o w, o r
knee)
Presence of antiU1-R N P in
MCT D
AN A likely to
have a high titer
and a speckled
pattern in M C T D
in contrast to D M
or P M where high
titers are
suggestive of a
separate
overlapping
i n f l a mma t o r y
condition
Muscle biopsy
can be definitive
for a diagnosis.
Specific patterns
on
e l e c t r o my e l o g r a m
in DM
Idiopathic
i n f l a mma t o r y
my o p a t h y
(e.g., D M,
PM)
MCT D
B. Drugs
P a t i e n t i s mo r e
P h y s i c a l e xa mi n a t i o n a s
likely to be older
per S L E
a n d me n
Patient likely to be
Antihistone
antibodies
(present in 95%
Drug-induced
lupus
607 / 652
tmdmss
Patient likely to be
taking:
of cases)
Erythrocyte
rate is often
elevated
Anti-ds- D N A
testing is usually
negative and can
be used to
differentiate this
condition from
SLE
Antibodies to
neutrophil
c y t o p l a s mi c
a n t i g e n s ma y b e
positive
P r o c a i n a mi d e
(10% develop
lupus, 50%
have elevated
AN As)
C h l o r p r o ma zi n e
Quinidine
H y d r a l a zi n e
S y mp t o ms
consistent with S L E
described above
C. Sy ste m ic illne ss
Tuberculosis
Fever (in 50%
80%)
Malaise
We i g h t l o s s
Night sweats
Cough
(nonproductive
in early stages,
ma y e v e n t u a l l y
be productive
of sputum
and/or blood
tinged)
Pleuritic pain
Dyspnea
E xt r a p u l mo n a r y
(15% of cases)
Pleural effusion
Adventitial lung
sounds
L y mp h n o d e
e n l a r g e me n t w i t h o r
without tenderness
Organ-specific
findings
Tuberculin skin
test
C h e s t x- r a y i f
positive skin test
Acid-fast staining
and culture of
induced sputum
f o r my c o b a c t e r i a
Possibly
bronchoscopy
C B C looking for
a n e mi a
E l e c t r o l y t e s ma y
d e mo n s t r a t e
h y p o n a t r e mi a i n
patients
c o mp l i c a t e d b y
t h e s y n d r o me o f
inappropriate
adrenocortical
h o r mo n e
secretion
e n u me r a t e
Fatigue
Fever
Chills
Nonspecific
sore throat
Headache
Sleep
O f t e n n o r ma l
May have
l y mp h a d e n o p a t h y
J oint swelling
Right upper quadrant
tenderness or liver
e n l a r g e me n t
C B C ( ma y
d e mo n s t r a t e
l y mp h o c y t o s i s )
Vi r a l t i t e r s
(Epstein-Barr or
c y t o me g a l o v i r u s )
Other laboratory
Chronic viral
infections (e.g.,
Epstein-Barr
virus, cytome g a l o v i r u s )
608 / 652
tmdmss

disturbances
Wo me n ( 9 5 %
for P B C and
c o mmo n f o r
AH) and young
(AH)
A b d o mi n a l p a i n
Fever
A me n o r r h e a
Diarrhea
Pleuritic pain
and/or
polyarthritis
We i g h t l o s s
Fatigue
Malaise
Cigarette
s mo k i n g
of cancer
a b n o r ma l i t i e s a r e
u n u s u a l b u t ma y
be specific to the
particular viral
agent suspected
Jaundice
Advanced disease
c a n b e c h a r a c t e r i ze d
b y s p i d e r a n g i o ma s ,
a s c i t e s , p a l ma r
e r y t h e ma , c a p u t
me d u s a e ,
g y n e c o ma s t i a , p a r o t i d
e n l a r g e me n t , a n d
testicular atrophy
Liver disease
Liver function
(P B C, AH, and
t e s t i n g ( A S T , A LT ,p r i ma r y
g a mma - g l u t a my l a u t o i mmu n e
transferase,
cholangitis)
alkaline
phosphatase)
Serial testing of
a mi n o t r a n s f e r a s e s
if elevated
CBC
Hepatitis testing
( A, B and C )
Anti-ss- D N A and
a n t i s mo o t h
mu s c l e a n t i - b o d y
testing
Anti
mi t o c h o n d r i a l
antibodies
h a l l ma r k o f P B C
Liver biopsy
Physical findings are

Laboratory testing is Malignancy
d e p e n d e n t o n t h e t y p e o f d e p e n d e n t o n t h e t y p e( p o s i t i v e A N A i s
ma l i g n a n c y s u s p e c t e d
o f ma l i g n a n c y
a rare finding)
suspected
(3)
A H , a u t o i mmu n e h e p a t i t i s ; A LT , a l a n i n e a mi n o t r a n s f e r a s e ; A S T , a s p a r t a t e a mi n o t r a n s f e r a s e ;
C B C , c o mp l e t e b l o o d c o u n t ; R N P, a n t i r i b o n u c l e o p r o t e i n ; D M , d e r ma t o my o s i t i s ; M C T D , mi xe d
c o n n e c t i v e t i s s u e d i s o r d e r ; P B C , p r i ma r y b i l i a r y c i r r h o s i s ; P M , p o l y my o s i t i s ; R A , r h e u ma t o i d
a r t h r i t i s ; S L E , s y s t e mi c l u p u s e r y t h e ma t o s u s ; A N A , a n t i n u c l e a r a n t i b o d i e s .
References
1 . P h a n T G , Wo n g R C , A d e l s t e i n S . A u t o a n t i b o d i e s t o e xt r a c t a b l e n u c l e a r
a n t i g e n s : ma k i n g d e t e c t i o n a n d i n t e r p r e t a t i o n mo r e me
C l ai nn i D
n gi agn
f u l . L ab
I mmunol2 0 0 2 ; 9 : 1 .
2 . S o l o mo n D H , K a v a n a u g h A J , S c h u r P H . E v i d e n c e - b a s e d g u i d e l i n e s f o r t h e
u s e o f i mmu n o l o g i c t e s t s : a n t i n u c l e a r a n t i b o d A
y rttehrs it ti ni sg .R heum
609 / 652
tmdmss
2002;47:434.
3 . L a n e S K , G r a v e l J W J r. , C l i n i c a l u t i l i t y o f c o mmo n s e r u m r h e u ma t o l o g i c t e s t s .
2002;65(6):10731080.
610 / 652
tmdmss
17.4 - Brain Natriuretic Peptide
I mmu n o l o g y > 1 7 . 4 - B r a i n N a t r i u r e t i c P e p t i d e
17.4
Brain Natriuretic Peptide
Nicole Otto
P e r r y W. S e x t o n
I . Background
B r a i n n a t r i u r e t i c p e p t i d e ( B N P ) w a s f i r s t d i s c o v e r e d i n t h e b r a i n s o f p i g s . I n h u ma n
B N P i s p r o d u c e d i n t h e v e n t r i c l e s o f t h e h e a r t a n d r e l e a s e d a s c a r d i a c my o f i b r i l s
w h i c h a r e s t r e t c h e d d u r i n g v o l u me o r p r e s s u r e o v e r l o a d . T h e r e a r e t h r e e o t h e r
k n o w n h u ma n n a t r i u r e t i c p e p t i d e s ( a t r i a l , C - t y p e , a n d d e n d r o a s p i s ) .
P h y s i o l o g i c l e v e l s o f B N P c a u s e s o d i u m a n d w a t e r e xc r e t i o n t h r o u g h t h e k i d n e y s
a l t h o u g h i t s p r i ma r y e f f e c t i s t h r o u g h v e n o u s d i l a t i o n , s u b s e q u e n t l y d e c r e a s i n g
c a r d i a c p r e l o a d . B N P i s s e c r e t e d i n i n c r e a s i n g a mo u n t s a s h e a l t h y i n d i v i d u a l s a g e .
Wo me n s e c r e t e mo r e B N P t h a n me n . T h e r o l e o f B N P i n me d i c i n e
P. 3 7 9
is evolving; although it was initially used solely in the diagnosis of congestive hear
failure (C H F), other uses continue to be established. B N P is also useful in
d i a g n o s i n g , p r e d i c t i n g p r o g n o s i s , a n d ma n a g i n g l e f t v e n t r i c u l a r d y s f u n c t i o n ( LV D )
a n d c o r o n a r y i s c h e mi a . S o me e xp e r t s s u g g e s t t h a t B N P c o u l d b e u s e d t o mo n i t o r
s y mp t o ma t i c a n d a s y mp t o ma t i c p a t i e n t s w i t h c a r d i o v a s c u l a r r i s k f a c t o r s a n d p r e d i c t
their overall risk of death from cardiovascular events. Recent evidence suggests th
B N P ma y e v e n h a v e a r o l e i n d i a g n o s i n g a n d t r e a t i n g c h i l d r e n w i t h C H F i n r e n a l
d i s e a s e 1)( .
I I I . Evaluation
C u r r e n t B N P t e s t s a r e r a p i d i mmu n o f l u o r e s c e n c e , w h i c h c o s t a b o u t $ 2 6 . 0 0 p e r k i t .
W i t h i n 3 0 mi n u t e s , t h e a n t i - B N P a n t i b o d i e s b i n d q u a n t i t a t i v e l y t o t h e B N P i n t h e
s e r u m a n d f l u o r e s c e p r o p o r t i o n a l l y t o t h e a mo u n t o f B N P pTr aebs leen t1 7( s. 4e).e.1
I n t h e p a t i e n t w i t h C H F a n d c o r o n a r y i s c h e mi a , r a p i d b e d s i d e B N P me a s u r e me n t i n
t h e e me r g e n c y d e p a r t me n t s e t t i n g h a s b e e n f o u n d t o d e c r e a s e mo r b i d i t y a n d
mo r t a l i t y. T h e h a l f - l i f e o f B N P i s o n l y 2 2 mi n u t e s ; t h e r e f o r e , s e r i a l t e s t i n g c a n b e
i n f o r ma t i v e2)(.
I V. Diagnosis
611 / 652
tmdmss
A. Congestive heart failure
B N P i s c u r r e n t l y u s e d f o r d i a g n o s i s , g u i d e t r e a t me n t , a n d p r e d i c t p r o g n o s i s i n C H F
A n e l e v a t e d B N P i s 8 6 % s e n s i t i v e a n d 9 8 % s p e c i f i c f o r C H F. A n o r ma l B N P h a s
96% negative predictive value and can be used to rule out C H F in the patient with
dyspnea.
1. C H F i s mo s t o f t e n d i a g n o s e d t h r o u g h h i s t o r y a n d p h y s i c a l e xa mi n a t i o n a n d t h e n
c o n f i r me d b y c h e s t x- r a y. I n p a t i e n t s w h o p r e s e n t w i t h s y mp t o ms o f c o u g h o r
dyspnea, an elevated B N P can help distinguish a cardiac cause from other
d i f f e r e n t i a l d i a g n o s e s ( s u c h a s p u l mo n a r y e mb o l u s , a h i s t o r y o f c h r o n i c
o b s t r u c t i v e l u n g d i s e a s e , a s t h ma , o r p n e u mo n i a ) . A d d i n g B N P t o c l i n i c a l
j u d g me n t i n c r e a s e s t h e d i a g n o s t i c a c c u r a c y f r o m 7 4 % t o 8 1 % . A B N P v a l u e o f
< 1 0 0 p g / n L ma y b e n o r ma l , w h e r e a s mo s t p a t i e n t s w i t h d y s p n e a w i t h C H F
a c h i e v e v a l u e s o f > 4 0 0 p g / n LT (asbel e 1 7 . 4). (3)
1 .
2. B N P l e v e l s f a l l a f t e r e f f e c t i v e t h e r a p e u t i c i n t e r v e n t i o n . M o r b i d i t y, mo r t a l i t y, a n d
h o s p i t a l a d mi s s i o n a n d r e a d mi s s i o n a r e d e p e n d e n t o n a g g r e s s i v e t r e a t me n t o f
C H F, a n d a d e c l i n e i n B N P l e v e l s o f t e n c o r r e l a t e w i t h c l i n i c a l i mp r o v e me n t s . A
suggested goal at hospital discharge is a B N P <500 pg/nL.
3. B N P me a s u r e d a t i n i t i a l c l i n i c a l p r e s e n t a t i o n p r o v i d e s u s e f u l p r o g n o s t i c
i n f o r ma t i o n o n p a t i e n t s w i t h c h r o n i c h e a r t f a i l u r e . P e r s i s t e n t l y e l e v a t e d B N P,
d e s p i t e o p t i ma l me d i c a l i n t e r v e n t i o n c a r r i e s a p o o r e r p r o g n o s i s . N o t e t h a t
p a t i e n t s w i t h c h r o n i c h e a r t f a i l u r e ma y h a v e p e r s i s t e n t l y e l e v a t e d B N P b u t ma y
be clinically
P. 3 8 0
s t a b l e . A s e r i e s o f B N P l e v e l s i s o f t e n mo r e h e l p f u l i n u n d e r s t a n d i n g t h e r e l a t i v
trend in a particular patient.
TAB L E 17.4.1 Helpful Values for Brain Natriuretic

Peptide
BNP
level
(pg/nL)
I nte rpre tation
<100
M a y b e n o r ma l
96% negative predictive value for C H F
>100
Possible C H F
84.3% positive predictive value for C H F

S e e t e xt f o r d i s c u s s i o n o f c o n f o u n d i n g f a c t o r s
>400
Most patients
with dyspnea
due to heart
failure
T hese patients should be under intense

mo n i t o r i n g a n d t r e a t me n t i n e i t h e r t h e
o u t p a t i e n t o r i n p a t i e n t s e t t i n gC h( saepet e r
7 . 5)
Othe r
612 / 652
tmdmss
<500
Goal at hospital
discharge
>700
Severely
d e c o mp e n s a t e d
CHF
B N P, b r a i n n a t r i u r e t i c p e p t i d e ; C H F, c o n g e s t i v e h e a r t f a i l u r e .
B. Left ventricular dysfunction

1. E l e v a t e d B N P l e v e l s c a n h e l p p r e d i c t t h e e xi s t e n c e o f LV D i n b o t h s y mp t o ma t i c
a n d a s y mp t o ma t i c p a t i e n t s . S o me a u t h o r s s u g g e s t t h a t me a s u r e me n t o f B N P
c a n b e u s e d a s a s c r e e n i n g t e s t f o r LV D i n p a t i e n t s w i t h c a r d i o v a s c u l a r r i s k
f a c t o r s . T h e e v i d e n c e a n d p a r a me t e r s t o s u p p o r t t h i s c l a i m a r e c u r r e n t l y
e me r g i n g a n d a r e c o n t r o v e r s i a l .
2. B N P l e v e l s c o r r e l a t e s t r o n g l y w i t h l e f t v e n t r i c u l a r ma s s ( i n c l u d i n g l e f t v e n t r i c u l a
h y p e r t r o p h y ) a n d l e f t v e n t r i c u l a r d i s t e n s i o n . T h e r e f o r e , ma n y o t h e r c o n d i t i o n s
( p r i ma r y p u l mo n a r y h y p e r t e n s i o n , p r i ma r y h y p e r a l d o s t e r o n i s m, C u s h i n g ' s
s y n d r o me , o r a t h l e t i c h e a r t ) mi g h t p r e c i p i t a t e a n d i n c r e a s e t h e B N P. B N P
i n c r e a s e s a s LV D w o r s e n s i n s e v e r e s e p s i s d e s p i t e t h e f r e q u e n t p r e s e r v a t i o n o f
e j e c t i o n f r a c t i o7)n . (
C. Acute coronary syndromes and known coronary

ischemia
I n t h e p a t i e n t p r e s e n t i n g w i t h c h e s t p a i n a n d q u e s t i o n a b l e c o r o n a r y i s c h e mi a , B N P
ma y b e e l e v a t e d . E l e v a t e d l e v e l s d e n o t e o n g o i n g o r r e c e n t i s c h e mi a w h e n me a s u r e d
6 hours to 10 days after an event. T his can be especially helpful in patients who
h a v e n o n s p e c i f i c S T - T w a v e c h a n g e s , a n o n q w a v e my o c a r d i a l i n f a r c t i o n , o r
s y mp t o ms o f a t y p i c a l c h e s t p a i n . P a t i e n t s w i t h a n y c o n d i t i o n c a u s i n g c a r d i a c
i s c h e mi a ma y h a v e a n e l e v a t e d B N P v a l u e . S o me e xp e r t s e v e n b e l i e v e t h a t B N P
ma y b e mo r e u s e f u l t h a n t h e me a s u r e me n t o f t r o p o n i n i n c a r d i a c i s c h e mi a . I t i s n o t
y e t k n o w n w h e t h e r t h e l e v e l o f B N P p r e d i c t s t h e s e v e r i t y a n d u l t i ma t e t h e p r o g n o s i s
o f t h e c a r d i a c i s c h e mi a i n t h e s e p o p u9)
l a.t i o n s (
D. Special considerations
1. O l d e r i n d i v i d u a l s a n d w o me n h a v e n o r ma l l y h i g h e r B N P l e v e l s . B N P i s e l e v a t e d
i n p r i ma r y a n d s e c o n d a r y p u l mo n a r y h y p e r t e n s i o n , r e n a l d i s e a s e , a n d c i r r h o s i s .
P a t i e n t s w i t h r e n a l f a i l u r e ( o n d i a l y s i s o r a w a i t i n g t r a n s p l a n t a t i o n ) ma y h a v e
u n r e l i a b l e B N P l e v e l s , l i k e l y r e s u l t i n g f r o m c h r o n i c v o l u me e xp a n s i o n . H o w e v e r,
patients with nondialysis-dependent renal dysfunction have B N P levels that
reliably correlate with echocardiographic evidence of heart failure. Patients with
c i r r h o s i s h a v e B N P l e v e l s t h r e e t i me s t h a t o f h e a l t h y s u b j e c t s i n s o me s t u d i e s .
613 / 652
tmdmss
2. C a r d i a c i n f l a mma t i o n o f a n y t y p e o f my o c a r d i t i s , i n c l u d i n g c a r d i a c t r a n s p l a n t
r e j e c t i o n , K a w a s a k i d i s e a s e , o r a n a r r h y t h mo g e n i c r i g h t v e n t r i c l e w i t h d e c r e a s e
e j e c t i o n f r a c t i o n , ma y l e a d t o i n c r e a s e d B N P.
3. O b e s e p a t i e n t s ( b o d y ma s s i n d e x > 3 0 ) w i t h h e a r t f a i l u r e t e n d t o h a v e l o w e r
l e v e l s o f B N P w h e n c o mp a r e d w i t h n o n o b e s e p a t i e n t s w i t h f a i l u r e .
4. O t h e r p o t e n t i a l u s e s o f B N P a r e b e i n g s t u d i e d a n d d e v e l o p e d , i n c l u d i n g t h e u s e
o f p h a r ma c o l o g i c d o s e s o f B N P ( k n ones
w n i rai st i de
) t o t r e a t C H 1F 9)( .
References
1 . W i e c zo r e k S J , Wu A H , C h r i s t e n s o n R , e t a l . A r a p i d B - t y p e n a t r i u r e t i c p e p t i d e
assay accurately diagnoses left ventricular dysfunction and heart failure: a
mu l t i c e n t e r e v a l u a t iAom
n . H ear t J2 0 0 2 ; 1 4 4 : 8 3 4 .
2 . M c C u l l o u g h PA , N o w a k R M , M c C o r d J , e t a l . B - t y p e n a t r i u r e t i c p e p t i d e a n d
c l i n i c a l j u d g me n t i n e me r g e n c y d i a g n o s i s o f h e a r t f a i l u r e : a n a l y s i s f r o m B r e a t h i n g
N o t P r o p e r l y ( B N P ) mu l t i n a t i o n a l Cs it rucduly.at i on
2002;106:416422.
3 . H o b b s R E . U s i n g B N P t o d i a g n o s e , ma n a g e , a n d t r e a t h eCalrev
t fea i l u r e .
C l i n J M ed2 0 0 3 ; 7 0 ( 4 ) : 3 3 3 3 3 6 .
4 . H e J , O g d e n L G , B a zza n o L A , e t a l . R i s k f a c t o r s f o r c o n g e s t i v e h e a r t f a i l u r e i n
U S me n a n d w o me n : N H A N E S I e p i d e mi o l o g i c f o l l o w -Aurpc hs tIuntd er
y. n M ed
2001;161:996.
5 . L u b i e n E , D e M a r i a A , K r i s h n a s w a my P, e t a l . U t i l i t y o f B - n a t r i u r e t i c p e p t i d e i n
d e t e c t i n g d i a s t o l i c d y s f u n c t i o n : c o mp a r i s o n w i t h D o p p l e r v e l o c i t y r e c o r d i n g s .
C i r c ul at i on
2002;105:595.
6 . N a g a y N , N i s h i k i m T , O k a n o Y, e t a l . P l a s ma b r a i n n a t r i u r e t i c p e p t i d e l e v e l s
i n c r e a s e i n p r o p o r t i o n t o t h e e xt e n t o f r i g h t v e n t r i c u l a r d y s f u n c t i o n i n p u l mo n a r y
h y p e r t e n s i o nJ . A m C ol l C ar di 1ol9 9 8 ; 3 1 : 2 0 2 .
7 . K o g l i n J , P e h l i v a n i S , S c h w a i b l ma i r M , e t a l . R o l e o f b r a i n n a t r i u r e t i c p e p t i d e
i n r i s k s t r a t i f i c a t i o n o f p a t i e n t s w i t h c o n g e s t i v e h eJa A
r t mf aCi l ol
u rle .C ar di ol
2001;38:1934.
8 . Wa n g T J , L a r s o n M G , L e v y D , e t a l . P l a s ma n a t r i u r e t i c p e p t i d e l e v e l s a n d t h e
r i s k o f c a r d i o v a s c u l a r e v e n t s a n d Nd eEangl
t h . J M ed2 0 0 4 ; 3 5 0 : 6 5 5 .
9 . D e L e mo s J A , M o r r o w D A , B e n t l e y J H , e t a l . T h e p r o g n o s t i c v a l u e o f B - t y p e
n a t r i u r e t i c p e p t i d e i n p a t i e n t s w i t h a c u t e c o r o n a r y sNy nEdngl
r o me
J sM. ed
2001;345:1014.
1 0 . M e h r a M R , U b e r PA , P a r k M H , e t a l . O b e s i t y a n d s u p p r e s s e d B - t y p e
614 / 652
tmdmss
n a t r i u r e t i c p e p t i d e l e v e l s i n h e a r t Jf aAi lm
u r eC.ol l C ar di 2ol0 0 4 ; 4 3 : 1 5 9 0 .
11 . H a l l C . N T - P r o B N P : t h e me c h a n i s m b e h i n d t h eJ ma
C ar
r kde r.
F ai l
2 0 0 5 ; 11 ( s u p p 5 ) : 8 1 8 3 .
1 2 . M i l l s R M . H o w t o u s e n e s i r i t i d e i n t r e a t i n g d e c o mp e n s a t e d h e a r t f a i l u r e .
C l ev e C l i n J M ed
2002;69(3):252256.
1 3 . P e a c o c k W F. B - t y p e n a t r i u r e t i c p e p t i d e a s s a y : a r a p i d t e s t f o r h e a r t f a i l u r e .
2002;69(3):244251.
1 4 . H o u b e n A J . Va s c u l a r a n d r e n a l a c t i o n s o f b r a i n n a t r i u r e t i c p e p t i d e i n ma n :
p h y s i o l o g y a n d p h a r ma c o lFoundam
g y.
C l i n P har mac2 ol
0 0 5 ; 1 9 ( 4 ) : 4 11 4 1 9 .
1 5 . B r u c k ma n M , e t a l . P r o g n o s t i c v a l u e o f p l a s ma N T p r o - N P i n p a t i e n t s w i t h
s e v e r e s e p s iC
s .i r c ul at i on
2 0 0 5 ; 11 2 : 5 2 7 5 3 4 .
1 6 . S c h mi t t B P, K u s h n e r M S , W i e n e r S L . T h e d i a g n o s t i c u s e f u l n e s s o f h i s t o r y o f
t h e p a t i e n t w i t h d y s p nJe G
a .en I nt er n M ed
1986;1(6):386393.
1 7 . M c C u l l o u g h PA , O ml a n d T , M a i s e l A S . B - t y p e n a t r i u r e t i c p e p t i d e s : a
d i a g n o s t i c b r e a k t h r o u g h f o r c l i n iRc ev
i a nC
s .ar di ov as c M ed
2003;4(2):7280.
1 8 . A r i c e t a G , B r o o k s E R , L a n g ma n C B . A s s e s s i n g c a r d i o v a s c u l a r r i s k i n
children with chronic kidney disease. B-natriuretic peptide: a potential new
ma r k e r.P edi at r N ephr ol
2005;20:17011707.
1 9 . T o p o l E J . N e s i r i t i d e n o t v e rNi f iEe ngl
d . J M ed2 0 0 5 ; 3 5 3 ( 2 ) : 11 3 11 6 .
2 0 . M c D o n a g h T A , M o r r i s o n C , L a w r e n c e A , e t a l . S y mp t o ma t i c a n d
a s y mp t o ma t i c l e f t - v e n t r i c u l a r s y s t o l i c d y s f u n c t i o n i n a n u r b a nL anc
p o petu l a t i o n .
1997;350(9081):829833.
2 1 . P a c k e r M . S h o u l d B - t y p e n a t r i u r e t i c p e p t i d e b e me a s u r e d r o u t i n e l y t o g u i d e
t h e d i a g n o s i s a n d ma n a g e me n t o f c h r o n i c h e a rC
t if raci ul
l u at
r e i?on
2003;108:29502957.
2 2 . J a n u zzi J L , M a i s e l A S . C o r r e s p o n d e n c e : r o u t i n e me a s u r e me n t o f n a t r i u r e t i c
p e p t i d e t o g u i d e t h e d i a g n o s i s a n d ma n a g e me n t o f c h r o n i c h e a r t f a i l u r e .
C i r c ul at i on
2004;109:e325e326.
615 / 652
tmdmss
17.5 - D-Dimer
I mmu n o l o g y > 1 7 . 5 - D - D i me r
17.5
D-Dimer
P e r r y W. S e x t o n
Nicole Otto
I . Background
A D - d i me r i s a d e g r a d a t i o n p r o d u c t o f c r o s s - l i n k e d f i b r i n t h a t h a s u n d e r g o n e
f i b r i n o l y s i s i n t h e f i n a l s t a g e s o f t h e c l o t t i n g c a s c a d e . A l t h o u g h t h e D - d i me r t e s t i s
u s e f u l i n t h e d i a g n o s i s o f v e n o u s t h r o mb o e mb o l i s m ( V T E ) ( d e e p v e i n t h r o mb o s i s
a n d / o r p u l mo n a r y e mb o l i s m) , v e n o g r a m, D o p p l e r u l t r a s o u n d , i mp e d a n c e
p l e t h y s mo g r a p h y, a r t e r i o g r a m, a n d / o r h i g h - r e s o l u t i o n c h e s t c o mp u t e d t o mo g r a p h y
(C T ) scan are the definitive diagnostic tests for these conditions. T he challenge of
s o me o f t h e s e d i a g n o s t i c t e s t s i s t h a t t h e r e s u l t s a r e o p e r a t o r d e p e n d e n t a n d c a n
s o me t i me s b e i n d e t e r mi n a t e . T h e D - d i me r t e s t w a s d e v e l o p e d t o p o t e n t i a l l y
e l i mi n a t e t h e n e e d f o r f u r t h e r d i a g n o s t i c t e s t i n g i n t h o s e w h o p r e s e n t w i t h a n u n s u r
diagnosis of suspect VT E.
I I . Evaluation
A. T h e f i r s t s t e p i n a p p r o a c h i n g a p a t i e n t w i t h V T E i s t o e s t a b l i s h t h e p r e t e s t
p r o b a b i l i t y o n t h e b a s i s o f c l i n i c a l c r i t e r i a s u c h a s t h e We l l s TCa br iltee r i a ( s e e
1 7 . 5 . 1). I n p a t i e n t s w i t h a l o w c l i n i c a l p r e t e s t p r o b a b i l i t y o f V T E , a D - d i me r l e v e
c a n b e o b t a i n e d t o g u i d e t h e d i a g n o s i s . A r e s u l t o f < 5 0 0 n g / mL b y r a p i d e n zy me
l i n k e d i mmu n o s o r b e n t a s s a y ( E L I S A ) h a s a n e g a t i v e p r e d i c t i v e v a l u e o f 9 5 %
( s e e T a b l e 1 7 . 5). 2a n d e l i mi n a t e s t h e n e e d f o r f u r t h e r t e s t i n g . P a t i e n t s w i t h a
l o w p r e t e s t p r o b a b i l i t y a n d a D - d i me r o f 5 0 0 n g / mL s h o u l d g o o n t o t h e
a p p r o p r i a t e i ma g i n g s t u d y ( v e n o g r a m, D o p p l e r u l t r a s o u n d , i mp e d a n c e
p l e t h y s mo g r a p h y, a r t e r i o g r a m, a n d / o r h i g h - r e s o l u t i o n c h e s t C T s c a n ) . P a t i e n t s
w i t h a h i g h p r e t e s t p r o b a b i l i t y o f V T E s h o u l d b e e v a l u a t e d f i r s t b y a n i ma g i n g
s t u d y ; D - d i me r i s u s e d o n l y w h e n t h e i ma g i n g r e s u l t s a r e i n d e t e r mi n a t e .
B. T w o t y p e s o f D - d i me r a s s a y s e xi s t . T h e l a t e x a g g l u t i n a t i o n a s s a y u t i l i ze s
a n t i b o d i e s s p e c i f i c f o r D - d i me r. T h e a l t e r n a t e me t h o d o f D - d i me r t e s t i n g i s t h e
E L I S A . S e c o n d - g e n e r a t i o n l a t e x a g g l u t i n a t i o n t e s t s f o r D - d i me r a r e a s s e n s i t i v e
( 9 5 % ) a s t h e c u r r e n t E L I S A , a n d t h e c o s t s a r e c o mp a r a b l e .
616 / 652
tmdmss
17.5 - D-Dimer
TAB L E 17.5.1 Wells Criteria for Predicting

Pulm onary Em bolus in the Sym ptom atic Patient
Num be r of
points
Crite ria
C l i n i c a l s i g n s a n d s y mp t o ms o f d e e p v e n o u s
t h r o mb o s i s
3.0
An alternative diagnosis that is less likely than

p u l mo n a r y e mb o l i s m
3.0
P u l s e r a t e > 1 0 0 b e a t s / mi n
1.5
I mmo b i l i za t i o n o r s u r g e r y i n t h e p r e v i o u s 4 w k
1.5
P r e v i o u s d e e p v e n o u s t h r o mb o s i s / p u l mo n a r y e mb o l i s m
1.5
H e mo p t y s i s
1.0
Malignancy
1.0
4 p o i n t s , p u l mo n a r y e mb o l i s m u n l i k e l y ( p r e t e s t p r o b a b i l i t y w i t h
p r e v a l e n c e f o r p u l mo n a r y e mb o l i s m o f 5 % t o 8 % ) .
> 4 p o i n t s , p u l mo n a r y e mb o l i s m l i k e l y ( p r e t e s t p r o b a b i l i t y w i t h p r e v a l e n c e
f o r p u l mo n a r y e mb o l i s m o f 3 9 % t o 4 1 % ) .
P. 3 8 2
TAB L E 17.5.2 I nterpreting D-dim er Results

Ddim e r
v a l u ea
Pre te st probability for V T E,

de e p v e nous throm bosis, or
Diagnostic
pulm onary e m bolism
I nte rpre tation de cision
<500
n g / mL
Low pretest probability
N o r ma l
Can
safely
e xc l u d e
the
diagnosis
of VT E
( Any
M a l i g n a n c y o r < 3 mo
VT E cannot
Need
617 / 652
tmdmss
17.5 - D-Dimer
value)
postsurgery
b e e xc l u d e d
further
i ma g i n g
>500
n g / mL
Any clinical probability
VT E cannot
b e e xc l u d e d
Need
further
i ma g i n g
aB y e n zy me - l i n k e d i mmu n o s o r b e n t a s s a y ( E L I S A ) t e s t i n g .
V T E , v e n o u s t h r o mb o e mb o l i s m.
C. I t i s i mp o r t a n t t o n o t e t h a t a l o w D - d i me r t e s t i s a b l e t o r u l e o u t V T E . B y
c o n t r a s t , a n e l e v a t e d D - d i me r l e v e l i s n o t a b l e t o e s t a b l i s h a d i a g n o s i s o f V T E
( t h i s t e s t i s n o t s u f f i c i e n t l y s p e c i f i c ) . T h e r e a r e ma n y o t h e r s y s t e mi c c a u s e s o f
a n e l e v a t e d D - d i me r (Ts aebel e 1 7 . 5). .3
I I I . Diagnosis
T h e D - d i me r t e s t i s a q u i c k , n o n i n v a s i v e , a n d n o n o p e r a t o r - d e p e n d e n t l a b o r a t o r y
t e s t t h a t c a n e xc l u d e t h e d i a g n o s i s o f V T E w h e n n e g a t i v e i n p a t i e n t s w i t h l o w
pretest probability but does not have sufficient specificity to rule in VT E with
certainty when positive.
TAB L E 17.5.3 Factors that Affect the Predictability

of the D-dim er Test
Show n not to
incre ase false I n c r e a s e f a l s e - I n c r e a s e r i s k o f f a l s e - n e g a t i vpeo s i t i v e o r f a l s e positiv e re sults
re sults
ne gativ e re sults
states
Patient at high risk for deep

Chronic renal
v e n o u s t h r o mb o s i s ( k n o w n
failure
h y p e r c o a g u l a b l e s t a t e , ma l i g n a n c y,
r e c e n t s u r g e r y, p r e g n a n c y )
Pregnancy
P r e s e n c e o f s ma l l s u b s e g me n t a l I n t r a v e n o u s
e mb o l i ( v s . l a r g e p u l mo n a r y
p l a c e me n t
e mb o l i )
Infection
Osteoarthritis
Sepsis
Superficial
618 / 652
tmdmss
17.5 - D-Dimer
t h r o mb o p h l e b i t i s
T r a u ma
Wo u n d s ( p o s t o p e r a t i v e p a t i e n t ' s n o r ma l r a n g e i s 2 0 0 5 0 0 n g / mL )
H o s p i t a l i za t i o n
P. 3 8 3
M a n y c o n d i t i o n s a f f e c t t h e p r e d i c t i v e v a l u e o f a p o s i t i v e D - d i me r t e s t . T h e s e
c o n d i t i o n s a n d t h e i r e f f e c t a r e s hToawbnl ei n1 7 . 5.. 3
Suggested Reading
A me r i c a n T h o r a c i c S o c i e t y. T h e d i a g n o s t i c a p p r o a c h t o a c u t e v e n o u s
t h r o mb o e mb o l i s m. C l i n i c a l p r a c t i c e g uAi dme l Ji n eR.es pi r C r i t C ar e M ed
1999;160(3):10431066.
K r u i p M J , L e c l e r c q M G , v a n d e r H e u l C , e t a l . D i a g n o s t i c s t r a t e g i e s f o r e xc l u d i n g
p u l mo n a r y e mb o l i s m i n c l i n i c a l o u t c o me s As tnn
u d iI ents er
. n M ed
2003;138:941.
G i n s b e r g J S , B r i l l - E d w a r d s PA , D e me r s C , e t a l . D - d i me r i n p a t i e n t s w i t h
c l i n i c a l l y s u s p e c t e d p u l mo n a r y e mbCohes
l i s m.
t1 9 9 3 ; 1 0 4 : 1 6 7 9 .
Schutgens R E, Esseboom E U, Haas F J, et al. Usefulness of a
s e mi q u a n t i t a t i v e D - d i me r t e s t f o r t h e e xc l u s i o n o f d e e p v e n o u s t h r o mb o s i s i n
o u t p a t i e n t sA. m J M ed2 0 0 2 ; 11 2 : 6 1 7 .
B r o t ma n D J , S e g a l J B , J a n i J T , e t a l . L i mi t a t i o n s o f D - d i me r t e s t i n g i n
u n s e l e c t e d i n p a t i e n t s w i t h s u s p e c t e d v e n o u s t h r o mb oAem
mbJo lMi sed
m.
2 0 0 3 ; 11 4 : 2 7 6 .
F e d u l l o P F, T a p s e n V F. E v a l u a t i o n o f s u s p e c t e d p u l mo n a r yN eEmb
nglo l iJs m.
M ed 2 0 0 3 ; 3 4 9 ( 1 3 ) : 1 2 4 7 1 2 5 6 .
C harles L A, E dwards T, M acik B G , et al. E valuation of sensitivity and specificity
o f s i x D - d i me r l a t e x a s sAarycsh. P at hol L ab M 1ed
9 9 4 ; 11 8 ( 11 ) : 11 0 2 11 0 5 .
W i l d b e r g e r J E , Vo r w e r k D , K i l b i n g e r M , e t a l . B e d s i d e t e s t i n g ( S i mp l i R E D ) i n t h e
d i a g n o s i s o f D V T . E v a l u a t i o n o f 2 5 0 p Ianv
t i ees
n tts R
. adi ol
1998;33(4):232235.
J a n s s e n M C , Wo l l e r s h e i m H , Ve r b r u g g e n B , e t a l . R a p i d D - d i me r a s s a y s t o
e xc l u d e d e e p v e i n t h r o mb o s i s a n d p u l mo n a r y e mb o l i s m: c u r r e n t s t a t u s a n d n e w
d e v e l o p me n t sS.emi n T hr omb H emos
1 9t9 8 ; 2 4 ( 4 ) : 3 9 3 4 0 0 .
619 / 652
tmdmss
17.5 - D-Dimer
We l l s P S , A n d e r s o n D R , R o d g e r M , e t a l . E v a l u a t i o n o f D - d i me r i n t h e d i a g n o s i s
o f s u s p e c t e d d e e p v e i n t h r o mbNo sEi ngl
s . J M ed2 0 0 3 ; 3 4 9 ( 1 3 ) : 1 2 2 7 1 2 3 5 .
Wo l f , S J . P r o s p e c t i v e v a l i d a t i o n o f We l l s c r i t e r i a i n t h e e v a l u a t i o n o f p a t i e n t s
w i t h s u s p e c t e d p u l mo n a r y e mb oAl nn
i s m.E mer M ed2 0 0 4 ; 4 4 ( 5 ) : 5 0 3 5 1 0 .
620 / 652
tmdmss
17.6 - Hypercalcemia
I mmu n o l o g y > 1 7 . 6 - H y p e r c a l c e mi a
17.6
Hypercalcemia
Jose ph B. Straton
I . Background
T h e n o r ma l r a n g e o f s e r u m c a l c i u m i s 8 . 5 1 0 . 5 mg / d L . H y p e r c a l c e mi a , d e f i n e d a s
s e r u m c a l c i u m c o n c e n t r a t i o n s a b o v e 1 0 . 5 mg / d L , o c c u r s w h e n t h e c a l c i u m e n t e r s t h e
c i r c u l a t o r y s y s t e m mo r e r a p i d l y t h a n i t i s e xc r e t e d i n u r i n e o r d e p o s i t e d i n b o n e s . A n
i n i t i a l e l e v a t e d c a l c i u m l e v e l s h o u l d b e r e p e a t e d t o c o n f i r m t h e2)a. b n o r ma l i t y (
N i n e o u t o f t e n c a s e s o f h y p e r c a l c e mi a i n a d u l t s a r e c a u s e d b y e i t h e r
h y p e r p a r a t h y r o i d i s m o r ma l i g n a n c y. H y p e r p a r a t h y r o i d i s m c a u s e s mo s t o f t h e
h y p e r c a l c e mi a i n t h e o u t p a t i e n t s e t t i n g . M a l i g n a n c y c a u s e s mo s t o f t h e
h y p e r c a l c e mi a f o u n d i n h o s p i t a l i ze d p a t i e n t s . L e s s c o mmo n c a u s e s o f h y p e r c a l c e mi a
a r e c h r o n i c r e n a l f a i l u r e , h y p e r t h y r o i d i s m, h y p e r v i t a mi n o s i s A , h y p e r v i t a mi n o s i s D ,
i mmo b i l i za t i o n , P a g e t ' s d i s e a s e , a n d g r a n u l o ma t o u s d i s e a s e s . P s e u d o h y p e r c a l c e mi a
o c c u r s w h e n p a t i e n t s h a v e i n c r e a s e d s e r u m c a l c i u m- b i n d i n g p r o t e i n s . F o r e xa mp l e ,
d e h y d r a t i o n s e v e r e e n o u g h t o c a u s e h y p e r a l b u mi n e mi a ma y r e s u l t i n h i g h s e r u m
c a l c i u m l e v e l s . S u c h p s e u d o h y p e r c a l c e mi a s h o u l d r e s o l v e a f t e r t h e
h e mo c o n c e n t r a t i o n i s c o r r e c t e d .
P. 3 8 4
I I I . Evaluation
A. History
P a t i e n t s a r e o f t e n a s y mp t o ma t i c u n t i l t h e c a l c i u m l e v e l r i s e s a b o v e 1 2 mg / d L , a n d
t h e s y mp t o ms a r e o f t e n n o n s p e c i f i c : g e n e r a l i ze d mu s c l e w e a k n e s s , mu s c l e a c h e s ,
decreased coordination, decreased level of consciousness, headache, loss of
a p p e t i t e , n a u s e a , v o mi t i n g , c o n s t i p a t i o n , i n c r e a s e d s a l i v a t i o n , d y s p h a g i a , a n d
a b d o mi n a l p a i n o r d i s t e n s i o n . A r e v i e w o f s y s t e ms ma y t u r n u p a h i s t o r y o f a r e n a l
c a l c u l u s o r a h i s t o r y o f a ma l i g n1)a.n c y (
D e p e n d i n g o n t h e s e v e r i t y o f h y p e r c a l c e mi a , p h y s i c a l e xa mi n a t i o n ma y r e v e a l me n t a l
c o n f u s i o n , p o o r me mo r y, s l u r r e d s p e e c h , a c u t e p s y c h o t i c b e h a v i o r, l e t h a r g y o r c o ma ,
a t a xi a , p o o r o v e r a l l mu s c l e s t r e n g t h , h y p o t o n i a , h y p e r e xt e n s i b l e j o i n t s , i n c r e a s e d
d e e p t e n d o n r e f l e xe s , p o s i t i v e B a b i n s k i ' s r e f l e xe s , i n c o o r d i n a t i o n , d e c r e a s e d p a i n o r
vibration sense, calcium deposits on the conjunctiva near the palpebral fissure or on
t h e c o r n e a a r o u n d t h e i r i s , o r a n a c u t e a b d o me n o r a n i l e u s .
C. Testing
S e e T a b l e 1 7 . 6.. 1
I V. Diagnosis
S e e T a b l e 1 7 . 6.. 1C a l c i u m l e v e l s a b o v e 1 2 . 5 mg / d L c a n b e l i f e t h r e a t e n i n g . I n s u c h
621 / 652
tmdmss
s i t u a t i o n s , o n e s h o u l d p e r f o r m a n e l e c t r o c a r d i o g r a m a n d b e g i n t r e a t me n t
i mme d i a t e l y, b e c a u s e c a r d i a c a r r e s t , c o n v u l s i o n s , o r c o ma c a n o c c u r. F u r t h e r t e s t i n g
c a n b e p e r f o r me d w h i l e t h e s e r u m c a l c i u m i s l o w e r e d .
P. 3 8 5
P. 3 8 6
TAB L E 17.6.1 Evaluation of the Patient with Hypercalcem ia

History
Te s t i n g
Diagnosis
A. Spurious
E xc e s s i v e t h i r s t
Vo mi t i n g o r
diarrhea with
poor oral intake
D r y mu c o u s
me mb r a n e s
Decreased skin
turgor
Confusion/lethargy
Repeat calcium
after rehydration
P s e u d o h y p e r c a l c e mi a
due to dehydration
B. Endocrine disorde rs
F e ma l e
>60 y
Aches and pains
Va g u e
a b d o mi n a l p a i n
Depressive
s y mp t o ms
Renal calculi
N e c k ma s s ( u n likely)
No clinical
evidence of
ma l i g n a n c y
Serum calcium
< 1 4 . 5 mg / d L
Parathyroid
h o r mo n e
(elevated)
Serum chloride
> 1 0 2 mg / d L
Alkaline
phosphatase
( n o r ma l )
Serum phosphate
Bicarbonate
X-ray hands and
clavicles
Hyperparathyroidism
( 3 ) ( p r i ma r y o r
secondary)
A n xi e t y / t r e mo r
We i g h t l o s s
Heat intolerance
Vi s i o n p r o b l e ms
E xo p h t h a l mo s
E y e l i d t r e mo r
Tachycardia
Perspiration
H y p e r r e f l e xi a
T hyroids t i mu l a t i n g
h o r mo n e , f r e e4 T
T h y r o t o xi c o s i s
Fatigue
We i g h t l o s s
N a u s e a / v o mi t i n g
Hypotension
Lethargy
I n c r e a s e d mu c o s a l
or skin
p i g me n t a t i o n
Elevated
potassium
Decreased
sodium
Decreased blood
glucose
A b n o r ma l a d r e n a l
c o r t i c a l h o r mo n e
s t i mu l a t i o n t e s t
Addison's disease
622 / 652
tmdmss
C. Malignancy (4)
Malignancy with or
Serum calcium
w i t h o u t me t a s t a s i s
> 1 4 mg / d L
Serum chloride
Lung cancer
< 1 0 0 mg / d L
R e n a l c e l l c a r c i n o ma
Alkaline
S q u a mo u s c e l l
phosphatase >2
c a r c i n o ma
t i me s n o r ma l
Parathyroid
h o r mo n e < 2 t i me s
n o r ma l
C B C ( a n e mi a
frequent)
Further workup
according to the
suspected site of
p r i ma r y t u mo r
Bone scan
>50 y
We i g h t l o s s
S mo k e r
O t h e r s y mp t o ms
specific to
p a r t i c u l a r t u mo r
site
We i g h t l o s s
Signs specific to
site
>50 y
F e ma l e
We i g h t l o s s
Signs specific to
site
Further workup
according to the
s u s p e c t e d p r i ma r y
t u mo r s i t e
Alkaline
phosphatase
Bone scan
Breast cancer
Ovarian cancer
Metastatic disease
>50 y
Male
Urinary
c o mp l a i n t s
P r o s t a t e ma s s o n
r e c t a l e xa mi n a t i o n
or a diffusely
enlarged hard
prostate
Prostate-specific
antigen
Alkaline
phosphatase
Bone scan
Ultrasoundguided prostate
biopsy
Prostate cancer
>60 y
Bone pain
We i g h t l o s s
Fatigue
Pallor
Tenderness over
bones
C B C ( a n e mi a )
Serum and urine
protein
electrophoresis
Creatinine
Erythrocyte
rate
M u l t i p l e my e l o ma ( 5 )
Repeat serum
Medication use
D. Me dications/v itam ins
Lithium
Physical
623 / 652
tmdmss
F u r o s e mi d e
T h i a zi d e
diuretics
A mi n o p h y l l i n e
e xa mi n a t i o n
n o r ma l o r s h o w s
signs of
h y p e r c a l c e mi a
calcium (elevated)
Other serum
electrolyte values
ma y b e a b n o r ma l
C a l c i u m- b a s e d
antacids
N o r ma l o r s h o w s
signs of
Phosphate
BUN
Creatinine
Bicarbonate
M i l k - a l k a l i s y n d r o me
Repeat serum
calcium
Serum phosphate
and chloride
levels
F o r v i t a mi n A
overuse, C T scan
of the head
Vi t a mi n D o v e r d o s e
Vi t a mi n A o v e r d o s e
N o r ma l p h y s i c a l
Vi t a mi n p i l l u s e e xa mi n a t i o n o r
Bone pain
Headaches
Tenderness over
bones
p a p i l l e d e ma
E. Othe r
Prolonged bed
rest or chair rest
Physical
e xa mi n a t i o n
signs of
Consider D E XA
scan for
osteoporosis
I mmo b i l i za t i o n
History of acute
renal failure
Physical
e xa mi n a t i o n
signs of
BUN
Creatinine
Urinalysis
Chronic or diuretic
phase of acute renal
failure
Fever
Fatigue
Malaise
A n o r e xi a
Cough
Dyspnea
Retrosternal
c h e s t d i s c o mf o r t
Polyarthritis
Findings
dependent on
sites involved
E r y t h e ma
nodosum
Uveitis
CBC
( l y mp h o c y t o p e n i a )
C h e s t x- r a y
P u l mo n a r y
function testing
Transbronchial
biopsy
Sarcoidosis or other
g r a n u l o ma t o u s
disease
F a mi l y h i s t o r y o f
M a y b e n o r ma l o r
show signs of
Low 24-h urine

calcium level
F a mi l i a l h y p o c a l c i u r i c
C B C , c o mp l e t e b l o o d c o u n t ; B U N , b l o o d u r e a n i t r o g e n ; C T , c o mp u t e d t o mo g r a p h y ; D E X A , d u a l e n e r
624 / 652
tmdmss
x- r a y a b s o r p t i o me t r y.
References
1 . S h a n e E . H y p e r c a l c e mi a : p a t h o g e n e s i s , c l i n i c a l ma n i f e s t a t i o n s , d i f f e r e n t i a l
d i a g n o s i s , a n d ma n a g e me n t . I n : F a v u s P
MrJi ,mer
e d . on t he met abol i c bone
di s eas es and di s or der s of mi ner al met,abol
4 t h i es m
d . P h i l a d e l p h i a , PA :
L i p p i n c o t t W i l l i a ms & W i l k i n s , 1 9 9 9 : 1 8 3 1 8 7 .
2 . B u s h i n s k y D A , M o n k R D . C a l cLianc
u m.et1 9 9 8 ; 3 5 2 : 3 0 6 3 11 .
3 . A l Z a h r a n i A , L e v i n e M A . P r i ma r y h y p e r p a r a t h Ly ranc
o i det
i s1m.
997;349:1233
1238.
4 . S t e w a r t A F, C l i n i c a l p r a c t i c e . H y p e r c a l c e mi a a s s o c i a t e d w
Ni tEh ngl
c a nJc e r.
M ed 2 0 0 5 ; 3 5 2 : 3 7 3 3 7 9 .
5 . M u n d y G R , G u i s e T A . H y p e r c a l c e mi a o f ma l iAgm
n aJn cMy.ed1 9 9 7 ; 1 0 3 : 1 3 4
145.
625 / 652
tmdmss
17.7 - Hyperkalemia
I mmu n o l o g y > 1 7 . 7 - H y p e r k a l e mi a
17.7
Hyperkalemia
D a v i d We b n e r
I . Background
H y p e r k a l e mi a i s d e f i n e d a s a s e r u m p o t a +
s )s i >u 5m. 0( KmE q / L . H y p e r k a l e mi a c a n
b e l i f e t h r e a t e n i n g w h+e ni sK> 6 . 5 mE q / 2)
L .(
H y p e r k a l e mi a c a n b e d i v i d e d i n t o f o u r e t i o l o g i c g r o u p s : s p u r i o u s c a u s e s ,
r e d i s t r i b u t i o n a b n o r ma l i t i e s , r e n a l d i s o r d e r s , a n d h o r mo n e d e f i c i e n c i e s . S p u r i o u s
h y p e r k a l e mi a i s h y p e r k a l e mi a i n a h e a l t h y p a t i e n t ; i t i s ma n i f e s t e d a s t h r o mb o c y t o s i s ,
l e u k o c y t o s i s ( a l s o ma y c a u s e h y p o k a l e mi a ) , h e mo l y s i s , a n d r e p e a t e d f i s t c l e n c h i n g
i n b l o o d d r a w i n g . T h e mo s t c o mmo n r e d i s t r i b u t i o n a b n o r ma l i t y i s a c i d o s i s . T h e mo s t
frequently occurring renal disorders are renal insufficiency or failure with a
c o n c o mi t a n t p o t a s s i u m l o a d . T h e p r i ma r y c a u s e o f e n d o c r i n o l o g i c h y p e r k a l e mi a i s
uncontrolled diabetes.
I I I . Evaluation
H y p e r k a l e mi a i s u s u a l l y n o t s u s t a i n e d u n l e s s t h e r e i s a d i s o r d e r o f t h e p o t a s s i u m
r e g u l a t o r y s y s t e m. I n a n o t h e r w i s e h e a l t h y i n d i v i d u a l , r o u t i n e s c r e e n i n g o f p o t a s s i u m
i s n o t i n d i c a t e d . P o t a s s i u m s h o u l d b e mo n i t o r e d i n p a t i e n t s o n c e r t a i n me d i c a t i o n s
( s e e T a b l e 1 7 . 7). ,1 w i t h a c i d b a s e d i s o r d e r s , a b n o r ma l i t i e s i n r e n a l f u n c t i o n , a n d
disorders of aldosterone secretion. T hese patients are at risk of potentially fatal
h y p e r k a l e mi a3) (.
P. 3 8 8
P. 3 8 9
P. 3 9 0
TAB L E 17.7.1 Evaluation of the Patient with Hyperkalem ia

History
Te s t i n g
Diagnosis
A. Spurious (2)
Laboratory reading Healthy patient
i s h e mo l y s i s o n
electrolyte panel
H e mo l y s i s i n
R e p e a t s e r u m+ K
t e s t i n g ; n o r ma l i ze d c o l l e c t i o n t u b e
626 / 652
tmdmss
17.7 - Hyperkalemia
electrolyte panel
Platelet count >1
mi l l i o n
i n r e d r a w n s a mp l e
H e a l t h y p a t i e n t , e xc e pRt e p e a t p l a t e l e t
for disorder causing count with
t h r o mb o c y t o s i s
h e p a r i n i ze d
s p e c i me n
T h r o mb o c y t o s i s
(platelets
r e l e a s e +K
during clotting)
WB C count
>200,000
H e a l t h y p a t i e n t , e xc e pRt e p e a t W B C c o u n t L e u k o c y t o s i s
f o r u n d e r l y i n g d i s o r d e rw i t h r a p i d
causing leukocytosis processing/spinning
d o w n s p e c i me n
K + n o r ma l i ze d i n
repeat draw with
careful draw
technique
Healthy patient
R e p e a t s e r u m+ K
Tight or
prolonged
tourniquet or
fist clenching
during blood
draws
B. Re distribution (3)
Acidosis
- B lockers
Angiotensinconverting
e n zy me
inhibitors
Angiotensinreceptor
blockers
Cardiac
glycosides
N e u r o mu s c u l a r
blocking
agents
Salt
substitutes
T r i me t h o p r i m
P e n t a mi d i n e
Often no signs specific

to acidosis in an
otherwise ill patient
No signs of
h y p e r k a l e mi a P h y s i c a l
e xa mi n a t i o n ma y
reveal underlying
illness leading to
me d i c a t i o n u s e
pH <7.35
I mme d i a t e
ECG
Monitor
cardiac rhythm
AB G
Sequential
testing
Medication/diet
I mme d i a t e
effect
ECG
Cardiac rhythm
should be
mo n i t o r e d
627 / 652
tmdmss
17.7 - Hyperkalemia
Crush injury
Tissue
breakdown
Bruising, other signs

o f t r a u ma , o r a
necrotic wound
Cell breakdown
I mme d i a t e
ECG
Monitor
cardiac rhythm
R h a b d o my o l y s i s
S i g n s c e l l i n j u r y, h e a t
stroke, crush injury
Cell breakdown
Urinalysis for and kidney
my o g l o b i n
dysfunction
B U N/creatinine
I mme d i a t e
ECG
Monitor
cardiac rhythm
Large bruise or
h e ma t o ma
H e ma t o ma
H e ma t o ma
I mme d i a t e
breakdown
ECG
Monitor
cardiac rhythm
Muscle contraction Endurance athlete

i n ma r a t h o n
runners
C a c h e xi a
S y mp t o ms
related to the
etiology of the
c a c h e xi a
Cachetic
Signs related to
reason for
c a c h e xi a
Cell breakdown
a n d mu s c l e
r e l e a s e o f +K
Tissue
I mme d i a t e
catabolism
ECG
Monitor
cardiac rhythm
H e mo l y t i c a n e mi a
H e mo l y s i s
Pallor
Petechiae
Orthostatic
hypotension
Bleeding from any
orifice
I mme d i a t e
ECG
Monitor
cardiac rhythm
CBC
Other signs as
related to the
etiology of
h e mo l y t i c
a n e mi a
H y p e r k a l e mi c
Quadriplegia with sparing of the cranial
periodic paralysis nerves
Transient hyper
K+
628 / 652
tmdmss
17.7 - Hyperkalemia
(4)
C. Re nal disorde rs
U s e o f p o t a s s i u m- s p a r i n g d i u r e t i c s ( e . g . ,
t r i a mt e r e n e , s p i r o n o l a c t o n e )
H i s t o r y o f t r e a t me n t f P
o rneumoc
a
ysti s
c ar i ni ii n f e c t i o n
Known creatinine clearance <50
S y s t e mi c l u p u s
Malar rash
Discoid rash
Recurrent oral
ulcers
Focal neurologic
deficits
Sickle-cell disease or trait
Signs due to
Diuretic use
BUN
Creatinine
Creatinine
c l e a r a n c e ma y
be low
F EK +
BUN
Creatinine
N o r ma l
creatinine
clearance
T r i me t h o p r i m o r
p e n t a mi d i n e
effect
Renal
Creatinine
insufficiency or
clearance
failure with a
I mme d i a t e
potassium load
E C G , mo n i t o r
cardiac rhythm
AB G
Creatinine
clearance
F EK +
Evaluation of
underlying
disease
Decreased
GFR
Decreased
BUN
GFR
Creatinine
Creatinine
be low
L o w F EK +
Evaluation of
underlying
disease
Decreased
629 / 652
tmdmss
Signs due to
a my l o i d o s i s
BUN
Creatinine
Creatinine
be low
L o w F EK +
Evaluation of
underlying
disease
17.7 - Hyperkalemia
Decreased
GFR
D. Horm one de ficie ncy

Diabetes
Signs of D K A
Evaluate for
DKA
Check
E C G/cardiac
rhythm
Sequential
testing
Pseudo and actual

aldosterone
deficiency
H y p e r p i g me n t a t i o n
Hypotension
We i g h t l o s s
Vo mi t i n g
Acidosis
causing
redistribution
w i t h K+ / H+
e xc h a n g e
Addison's
Aldosterone
disease
levels
Renin levels
Check
E C G/cardiac
rhythm
Cosyntropin
s t i mu l a t i o n t e s t
Heparin use
Aldosterone
levels
Check
E C G/cardiac
rhythm
Aldosterone
e xc r e t i o n l o w
second to
heparin
W B C , w h i t e b l o o d c e l l ; E C G , e l e c t r o c a r d i o g r a m; C B C , c o mp l e t e b l o o d c o u n t ; A B G ,
ar t er i al bl ood gases; B U N , bl ood ur ea ni t rK
o g+,e nl o; wF E
f r a c t i o n a l e xc r e t i o n o f
p o t a s s i u m; G F R , g l o me r u l a r f i l t r a t i o n r a t e ; D K A , d i a b e t i c k e t o a c i d o s i s .
I V. Diagnosis
H y p e r k a l e mi a i s u s u a l l y a s y mp t o ma t i c . L o w e r l e+ veellesv oa ft i oKn ma y c h a n g e
n o r ma l c a r d i a c f u n c t i o n , p r o d u c i n g e l e c t r o c a r d i o g r a p h i c ( E C G ) c h a n g e s t h a t b e c o me
630 / 652
tmdmss
17.7 - Hyperkalemia
p r o g r e s s i v e l y mo r e l i f e t h r e a t e n i n g . P a t i e n t s+ w
i 6t h. 5a mE
K q/L require
i mme d i a t e c a r d i a c e v a l u a t i o n . T h e e a r l i e s t E C G c h a n g e s a r e e l e v a t i o n o f T w a v e s ,
f o l l o w e d b y a w i d e n i n g o f t h e Q R S c o mp l e x, a t r i o v e n t r i c u l a r c o n d u c t i o n d e l a y s ; t h i s
s l o w e r r h y t h m ma y l e a d t o l e t h a l v e n t r i c u l a r f i b r i l l a t i o n a n d a s y s t o l e . A r r h y t h mi a s
o c c u r a t h i g h l e v e l s +o fa nKd i f +K r i s e s v e r y r a p i d l y i n i t s c o n c e n t r a t i o n . A s i n
h y p o k a l e mi a , s e v e r e c a s e s o f h y p e r k a l e mi a c a n a l s o o c c u r w i t h a n a s c e n d i n g
paralysis.
References
1 . H a l p e r i n M L , K a me l K S . P o t a sLs anc
i u m.et1 9 9 8 ; 3 5 2 ( 9 1 2 2 ) : 1 3 5 1 4 0 .
2 . Wa l l a c h J , e Idnt
. er pr et at i on of di agnos t i c, t6es
t ht se d . N e w Yo r k : L i t t l e ,
B r o w n a n d C o mp a n y, 1 9 9 6 .
3 . G e n n a r i F. D i s o r d e r s o f p o t a s s i u m h o me o s t a s i s : h y p o k a l e mi a a n d
h y p e r k a l e mi aC.r i t C ar e C l i2n0 0 2 ; 1 8 : 2 7 3 2 8 8 .
4 . E v e r s S , E n g e l i e n A , K a r s c h V, e t a l . S e c o n d a r y h y p e r k a l e miJc p a r a l y s i s .
N eur ol N eur os ur g P s y c hi 1at9r9y8 ; 6 4 ( 2 ) : 2 4 9 2 5 2 .
5 . M a r t i n e z- M a l d o n a d o M . A p p r o a c h t o t h e p a t i e n t w i t h h y p e r k a l e mi a . I n : K e l l e y
W N , e dTex
. t book of i nt er nal medi. cPi ne
h i l a d e l p h i a PA : L i p p i n c o t t - R a v e n , 1 9 9 7 .
6 . R a s t e r g a r A , S o l e i ma n i M . H y p o k a l e mi a a n d h y p ePr kos
a ltegrmiad
a . M ed J
2001;77:759764.
631 / 652
tmdmss
17.8 - Hypokalemia
I mmu n o l o g y > 1 7 . 8 - H y p o k a l e mi a
17.8
Hypokalemia
D a v i d We b n e r
I . Background
H y p o k a l e mi a i s o n e o f t h e mo s t c o mmo n e l e c t r o l y t e a b n o r ma l i t i e s f o u n d i n b a s i c
l a b o r a t o r y e l e c t r o l y t e p a n e l s . A l t h o u g h t h e d e f i n i t i o n v a r i e s , h y p o k a l e mi a i s g e n e r a l l y
s a i d t o o c c u r w h e n s e r u m p o t a s s+i u) m
c o(nKc e n t r a t i o n f a l l s b e l o w 3 . 6 mE q / L .
W i t h t h e e xc e p t i o n o f f a c t i t i o u s c a u s e s ( l e u k e mi c p a t i e n t s w i t h e xt r e me l e u k o c y t o s i s ,
+ i s t a k e n u p b y t h e a b n o r ma l w h i t e b l o o d c e l l s ) , t r u e h y p o k a l e mi a r e s u l t s
where K
f r o m i n c r e a s e d p o t a s s i u m e xc r e t i o n , t r a n s c e l l u l a r s h i f t , o r d e c r e a s e d d i e t a r y i n t a k e
o f p o t a s s i u m. M o s t c o mmo n l y, h y p o k a l e mi a i s c a u s e d b y a b n o r ma l l o s s e s o f
p o t a s s i u m t h r o u g h t h e g a s t r o i n t e s t i n a l t r a c t o r k iTdanbel ye (1s7e.e8). .1 T h e mo s t
c o mmo n o f t h e s e c a u s e s i s d i u r e t i c u s e l e a+d idnegptl oe t K
ion. Both the loop
( b l o c k e d r e s o r p t i o n i n t h e l o o p o f H e n l e ) a n d t h i a zi d e d i u r e t i c s ( b l o c k r e s o r p t i o n i n
e a r l y d i s t a l t u b u l e ) c o n t r i b u t e t o t h e d e v e l o p me n t o f h y p o k a l e mi a . S o d i u m a n d
c h l o r i d e d e l i v e r y a r e i n c r e a s e d b y t h e d i u r e t i c s , r e s u l t i n g i n t h e +s. e c r e t i o n o f K
F u r t h e r +K l o s s i s f o s t e r e d b y i n c r e a s e d ma g n e s i u m e xc r e t i o n , w h i c h i s a l s o l i n k e d
t o t h e l o o p a n d t h i a zi d e d i u r e t i c s . O t h e r me d i c a t i o n s , me t a b o l i c a l k a l o s i s , r e n a l
t u b u l a r a c i d o s i s , a n d a l d o s t e r o n i s m d u e t o s y s t e mi c d i s e a s e s a n d g e n e t i c d i s o r d e r s
a r e i mp l i c a t e d i n t h e d e v e l o p me n t o f h y p o 1)
k a. l e mi a (
I I I . Evaluation
S e e T a b l e 1 7 . 8.. 1
P. 3 9 2
P. 3 9 3
I V. Diagnosis
S e e T a b l e 1 7 . 8.. 1
TAB L E 17.8.1 Evaluation of the Patient with Hypokalem ia

History
Phy sical
e x am ination
Te s t i n g
Diagnosis
632 / 652
tmdmss
17.8 - Hypokalemia
A. G astrointestinal losses (2)
E me s i s
Nasogastric
drainage
Pyloric/duodenal
obstruction
Pancreatic fistulas
Diarrhea
L a xa t i v e a b u s e
C o l o n i c n e o p l a s ms
Often no
signs on
physical
e xa mi n a t i o n
G e n e r a l i ze d
mu s c l e
weakness
Constipation
C a c h e xi a
A b d o mi n a l
e xa mi n a t i o n
for signs of
peritonitis or
l o c a l i ze d
pain
Gastrointestinal
Serum
potassium
electrolytes
losses
Spot urine
electrolytes if
needed
Urine and serum
o s mo l a l i t y a s
needed
Stool electrolytes
as appropriate
Electrocardiogram
(look for U
w a v e s , o r, i f
rapidly replacing
potassium by the
intravenous route,
look for
a r r h y t h mi a s )
B. Re nal losse s
Diuretics (loop or
t h i a zi d e )
O s mo t i c d i u r e s i s
(uncontrolled
diabetes)
Metabolic alkalosis
( v o mi t i n g / d i a r r h e a )
P r i ma r y
hyperaldosteronism
Cortisol responsive
aldosteronism
Congenital adrenal
hyperplasia
L i d d l e ' s s y n d r o me
11 h y d r o xy s t e r o i d
dehydrogenase
deficiency
B a r t t e r s y n d r o me
G i t e l ma n ' s
s y n d r o me
Inappropriate
secretion of
antidiuretic
h o r mo n e
As above
May also
ask about
history of
diabetes or
use of loop
o r t h i a zi d e
diuretics
Renal
As above
potassium
May also check losses and
f o r k e t o n e s , b l o o dc o n g e n i t a l
s u g a r, s e r u m p H s y n d r o me s
633 / 652
tmdmss
17.8 - Hypokalemia
H y p o ma g n e s e mi a
Licorice use
Corticosteroids
Renal tubular
acidosis
Renal artery
stenosis
C . Transce llular shift (3)
Bronchodilators
Tocolytics
T heophylline
Caffeine
Insulin
D e l i r i u m t r e me n s
Hyperthyroidism
F a mi l i a l
h y p o k a l e mi c
periodic paralysis
B a r i u m t o xi c i t y
Pancreatitis
Congestive heart
failure
T o xi c s h o c k
Pleural effusion
Ascites
Anasarca
Burns
(second/third
degree)
As above
May also
ask about
alcohol
abuse and
careful
me d i c a t i o n
history
Drug therapy
As above
a n d s y s t e mi c
May also check diseases
for serum alcohol
level, drug screen
as appropriate
D. I nade quate intake
A n o r e xi a / b u l i mi a
F o r c e d v o mi t i n g o r
diarrhea for any
reason
Neurotic spitting
Poor diet
As above
May also
check for
evidence to
support the
history such
as weight
loss,
staining, or
pitting of the
teeth
As above
Patient
e xc e s s e s
634 / 652
tmdmss
17.8 - Hypokalemia
References
1 . G e n n a r i F. C u r r e n t c o n c e p t s : h y p o k aNl eEmi
ngl
a . J M ed1 9 9 8 ; 3 3 9 ( 7 ) : 4 5 1
458.
2 . G e n n a r i F. D i s o r d e r s o f p o t a s s i u m h o me o s t a s i s : h y p o k a l e mi a a n d
h y p e r k a l e mi aC.r i t C ar e C l i2n0 0 2 ; 1 8 : 2 7 3 2 8 8 .
3 . R a s t e r g a r A , S o l e i ma n i M . H y p o k a l e mi a a n d h y p ePr kos
a ltegrmiad
a . M ed J
2001;77:759764.
635 / 652
tmdmss
18.1 - Bone Cyst
> T a b l e o f C o n t e n t s > 1 8 - D i a g n o s t i c I ma g i n g A b n o r ma l i t i e s > 1 8 . 1 - B o n e C y s t
18.1
Bone Cyst
Mark R. Ne e dham
I . Background
B o n e c y s t s p r e s e n t e i t h e r a s i n c i d e n t a l l e s i o n s o n x- r a y s o b t a i n e d f o r o t h e r
diagnostic purposes or as the underlying cause of a pathologic fracture. When
confronted with a bone cyst, the physician has to decide if further diagnostic testin
i s r e q u i r e d t o c o n f i r m t h e d i a g n o s i s , w h e t h e r t r e a t me n t i s r e q u i r e d , a n d w h a t t y p e o
f o l l o w - u p p l a n i s n e e d1)e.d (
A. Etiology
T h e c a u s e o f s i mp l e b o n e c y s t s i s u n k n o w n . T y p i c a l l y, a b o n e c y s t e xp a n d s t h e
cortex of the bone, with the periosteum covering the thin cortical shell of the cyst. A
me mb r a n e l i n e s t h e c y s t , a n d i f t r a u ma t i ze d , ma y l e a k b l o o d o r f l u i d i n t o t h e c y s t o r
d e v e l o p s e p t a t i o n s w i t h i n t h e c y s t t h a t ma y g i v e t h e c y s t a mo r e c o mp l e x a p p e a r a n c
o n x- r a y o r c o mp u t e d t o mo g r a p h y ( C T 2)
) s. c a n (
B. Epidemiology
M o s t c y s t s o c c u r d u r i n g t h e f i r s t o r s e c o n d d e c a d e s , mo s t c o mmo n l y i n c h i l d r e n
y o u n g e r t h a n 1 0 y e a r s o f a g e . T h e mo s t c o mmo n l o c a t i o n i s t h e h u me r u s , f o l l o w e d
b y t h e f e mu r, a n d r a r e l y t h e t i b i a , f i b u l a , r a d i u s , a n d u l n a . O l d e r p a t i e n t s mo r e
c o mmo n l y p r e s e n t w i t h b o n e c y s t s i n t h e i l e u m a n d c a l c a n e u s . M u l t i p l e c y s t s ma y
o c c u r i n t h e s a me p a t i e n t . W i t h i n t h e l o n g b o n e s , t h e mo s t c o mmo n l o c a t i o n s a r e t h
p r o xi ma l me t a p h y s i s , b u t i n v o l v e me n t o f t h e e p i p h y s i s c a n o c c u r.
I I I . Evaluation
A. History
B o n e c y s t s a r e t y p i c a l l y a s y mp t o ma t i c u n l e s s a s p o n t a n e o u s f r a c t u r e h a s o c c u r r e d
t h r o u g h t h e c y s t . P a i n a s s o c i a t e d w i t h a b o n e l e s i o n ma y s i mp l y b e d u e t o r a p i d
g r o w t h o f a b e n i g n l e s i o n b u t ma y b e a mo r e o mi n o u s s i g n o f a ma l i g n a n t b o n e
t u mo r.
E xa mi n a t i o n o f t h e a f f e c t e d e xt r e mi t y s h o u l d i n c l u d e a n i n s p e c t i o n o f t h e o v e r l y i n g
636 / 652
tmdmss
18.1 - Bone Cyst
s o f t t i s s u e f o r e v i d e n c e o f a s s o c i a t e d s o f t t i s s u e ma s s . P r e s e n c e o f a n a s s o c i a t e d
ma s s s h o u l d a l e r t t h e p h y s i c i a n t o t h e p o s s i b i l i t y o f a mo r e s i g n i f i c a n t p a t h o l o g y,
b e c a u s e b o n e c y s t s a r e c o n f i n e d t o t h e b o n e i t s e l f . S i mi l a r l y, a s s o c i a t e d
i n f l a mma t o r y r e a c t i o n s , i n c l u d i n g t e n d e r n e s s , i n d u r a t i o n , o r e r y t h e ma , ma y b e
w a r n i n g s i g n s o f mo r e s e r i o u s c o n d i t i o n s . L i mb l e n g t h s s h o u l d b e me a s u r e d a n d
d o c u me n t e d s o t h a t s e r i a l mo n i t o r i n g f o r l i mb l e n g t h d i s c r e p a n c y c a n b e
a c c o mp l i s h e d , p a r t i c u l a r l y w i t h l e s i o n s a d j a c e n t t o o r i n v o l v i n g t h e e p i p h y s i s .
C. Testing
P l a i n x- r a y i s u s u a l l y t h e o n l y d i a g n o s t i c t e s t r e q u i r e d t o e s t a b l i s h t h e d i a g n o s i s o f
b o n e c y s t . T h e x- r a y s h o w s a w e l l - d e f i n e d , w e l l - c i r c u ms c r i b e d l e s i o n w i t h a t h i n
s c l e r o t i c ma r g i n w i t h o u t r e a c t i o n o r d i s r u p t i o n o f t h e p e r i o s t e u m. S u s p e c t e d c y s t s i
t h e s p i n e o r p e l v i s ma y r e q u i r e ma g n e t i c r e s o n a n c e i ma g i n g ( M R I ) t o e l i c i t t h e
a n a t o mi c d e t a i l n e c e s s a r y f o r t h e d i a g n o s i s . I f a n a s s o c i a t e d s o f t t i s s u e l e s i o n i s
s u s p e c t e d i n a l o n g b o n e , a n M R I c a n b e h e l p f u l i n d e t e r mi n i n g t h e p r e s e n c e o r
a b s e n c e o f a s s o c i a t e d ma s s . A n M R I i s i n d i c a t e d i f t h e c y s t a p p e a r s a d j a c e n t t o o r
involves the epiphysis, because such cysts that involve the growth plate need
r e f e r r a l f o r i n t e r v e n t i o n o r, a t t h e l e a s t , mo r e c a r e f u l mo n i t o r i n g f o r g r o w t h
i mp a i r me n t . I f a n y a d d i t i o n a l i ma g i n g i s r e q u i r e d , M R I i s s u p e r i o r t o C T s c a n f o r t h i
i n d i c a t i o n . N u c l e a r me d i c i n e s t u d i e s , i n c l u d i n g p o s i t r o n - e mi s s i o n t o mo g r a p h y, a r e
n o t u s u a l l y h e l p f3)
u l. (
I V. Diagnosis
T h e d i a g n o s i s o f b o n e c y s t s i s c o n f i r me d b y p l a i n x- r a y. T h e d i f f e r e n t i a l d i a g n o s i s
i n c l u d e s ma l i g n a n t b o n e t u mo r o r me t a s t a s e s . T h e d i a g n o s t i c d i l e mma w h e n b o n e
cysts are
P. 3 9 8
i d e n t i f i e d i n c i d e n t a l l y i s t o d i s t i n g u i s h a b e n i g n c o n d i t i o n f r o m a ma l i g n a n c y. T h e
t h e r a p e u t i c d i l e mma s w h e n b o n e c y s t s a r e i d e n t i f i e d a r e t o d e t e r mi n e w h i c h c y s t s
require intervention to prevent pathologic fracture, how to treat pathologic fractures
t h r o u g h b o n e c y s t s w h e n t h e y o c c u r, a n d h o w t o mo n i t o r f o r a n d p r e v e n t a g r o w t h
d i s c r e p a n c y4)(.
1. R e f e r p a t h o l o g i c f r a c t u r e s t h r o u g h b o n e c y s t s f o r t h e t r e a t me n t b y o r t h o p a e d i c
surgeon.
2. O r d e r a n M R I o f t h e a f f e c t e d e xt r e mi t y i f t h e b o n e l e s i o n d e t e c t e d o n x- r a y i s
p a i n f u l , t e n d e r t o p a l p a t i o n , o r h a s a n a s s o c i a t e d s o f t t i s s u e ma s s . A n y a t y p i c a l
characteristics detected on M R I warrant referral.
3. R e f e r l a r g e b o n e c y s t s t h a t h a v e e xp a n d e d a n d t h i n n e d t h e o v e r l y i n g c o r t e x f o r
t r e a t me n t i n o r d e r t o p r e v e n t p a t h o l o g i c f r a c t u r e .
4. C a r e f u l l y d o c u me n t l i mb l e n g t h s w h e n t h e b o n e c y s t i s a d j a c e n t t o o r i n v o l v e s
the epiphysis and arrange for serial follow-up.
5. R e f e r f o r t r e a t me n t i f l i mb l e n g t h o r g r o w t h d i s c r e p a n c y o c c u r s .
637 / 652
tmdmss
18.1 - Bone Cyst
B o n e c y s t s t h a t p r e s e n t w i t h p a t h o l o g i c f r a c t u r e s ma y h a v e t o b e t r e a t e d b y
curettage of the cyst and packing with bone graft in addition to standard fracture
i mmo b i l i za t i o n . L a r g e c y s t s w i t h t h i n n i n g o f t h e c o r t e x n e e d p r o p h y l a c t i c t r e a t me n t
w i t h c u r e t t a g e a n d b o n e g r a f t i n g , c r y o t h e r a p y, a n d i n j e c t i o n o f b o n e ma r r o w o r
steroids in order to resolve the cyst and prevent pathologic fracture.
References
1 . T e o , E L H , P e h W CSGi .mpl e B one C y. set M e d i c i n e f r o m We b M D . Av a i l a b l e
a t : h t t p : / / w w w. e me d i c i n e . c o m/ r a d i o / t o p i c 6 4 2 . h t m. L a s t u p d a t e d : J a n u a r y 5 , 2 0 0 5 .
2 . C a p a n n a R , C a mp a n a c c i D A , M a n f r i n i M . U n i c a me r a l a n d a n e u r y s ma l b o n e
c y s t s .O r t hop C l i n N or t h A1 9m9 6 ; 2 7 : 6 0 5 6 1 4 .
3 . M e t t l e r F.
P r i mar y c ar e r adi ol.ogy
P h i l a d e l p h i a , PA : W B S a u n d e r s , 2 0 0 0 .
4 . N o v e l l i n e R AS.qui r e' s f undament al s of r adi.olCogy
a mb r i d g e : H a r v a r d
638 / 652
tmdmss
18.2 - Mediastinal Mass
> T a b l e o f C o n t e n t s > 1 8 - D i a g n o s t i c I ma g i n g A b n o r ma l i t i e s > 1 8 . 2 - M e d i a s t i n a l
Mass
18.2
M ediastinal M ass
Ronnie Coutinho
Enrique S. Fe rnande z
I . Background
M o s t me d i a s t i n a l ma s s e s a r e d i s c o v e r e d i n c i d e n t a l l y d u r i n g r o u t i n e r a d i o g r a p h i c
s t u d i e s . B e c a u s e t h e s e a r e f r e q u e n t l y ma l i g n a n t , i t i s i mp e r a t i v e t o i n i t i a t e a t i me l y
i n v e s t i g a t i o n o f a n y s y mp t o ms t h a t ma y b e a s s o c i a t e d w i t h a me d i a s t i n a l p r o c e s s .
W h e n a me d i a s t i n a l ma s s i s s u s p e c t e d o r d e t e c t e d , k n o w l e d g e o f t h e b o u n d a r i e s o f
i n d i v i d u a l me d i a s t i n a l c o mp a r t me n t s a n d t h e i r c o n t e n t s f a c i l i t a t e s t h e f o r mu l a t i o n o
a d i f f e r e n t i a l d i a g n o s i s . C a u s e s o f a me d i a s t i n a l ma s s a r e n u me r o u s , r a n g i n g f r o m
i n f e c t i o u s e t i o l o g i e s t o b e n i g n c y s t i c l e s i o n s t o ma l i g n a n c i e s .
T h e medi as t i num
i s t h e s p a c e i n t h e t h o r a x b e t w e e n t h e p l e u r a l c a v i t i e s . I t e xt e n d s
f r o m t h e s t e r n u m a n t e r i o r l y t o t h e v e r t e b r a l c o l u mn p o s t e r i o r l y a n d c o n t a i n s a l l t h e
t h o r a c i c v i s c e r a e xc e p t t h e l u n g s . T r a d i t i o n a l l y, t h e me d i a s t i n u m h a s b e e n d i v i d e d
i n t o t h eant er i or medi as t i num
( t h e s p a c e i n f r o n t o f t h e p e r i c a r d i umi
m)ddl
, t hee
medi as t i num
(the portion that contains the pericardium and its contents), and the
pos t er i or medi as t i num
( t h e p o r t i o n b e h i n d t h e p e r i c a r d i u m) . I t i s v i t a l t o e s t a b l i s h
t h e l o c a t i o n o f t h e ma s s i n a p a r t i c u l a r c o mp a r t me n t , a s ma s s e s i n t h e a n t e r i o r
c o mp a r t me n t a r e mo r e l i k e l y t o b e ma l i g n a n t t h a n t h o s e f o u n d i n o t h e r c o mp a r t me n t
(1) .
T h e p a t h o p h y s i o l o g y o f me d i a s t i n a l ma s s e s i s d e t e r mi n e d b y b o t h t h e a g e o f t h e
p a t i e n t a n d l o c a t i o n o f t h e ma s s i t s e l f . O n t h e c h e s t x- r a y, a l i n e d r a w n t h r o u g h t h e
a n t e r i o r a s p e c t o f t h e t r a c h e a a n d t h e p o s t e r i o r a s p e c t o f t h e h e a r t ma y b e
c o n s i d e r e d t o b e i n t h e mi d d l e me d i a s t i n u m. A l i n e d r a w n t h r o u g h t h e a n t e r i o r
P. 3 9 9
ma r g i n s o f t h e v e r t e b r a l b o d i e s i s i n t h e p o s t e r i o r me d i a s t i n u m. I t i s o f t e n d i f f i c u l t t
t e l l i f a n a b n o r ma l i t y i s i n t h e mi d d l e o r t h e p o s t e r i o r me d i a s t i n u m. T h e r e f o r e , t h e s e
t w o c o mp a r t me n t s ma y a t t i me s b e c o n s i d e r e d t o g e t h e r w h e n f o r mu l a t i n g a
differential diagnosis.
A. Anterior mediastinum
T h e a n t e r i o r me d i a s t i n u m c o n t e n t s i n c l u d e t h e t h y mu s g l a n d , a q u a n t i t y o f l o o s e
639 / 652
tmdmss
a r e o l a r t i s s u e , l y mp h a t i c v e s s e l s , a f e w a n t e r i o r me d i a s t i n a l l y mp h g l a n d s , b r a n c h e
o f t h e i n t e r n a l ma mma r y a r t e r y, a n d t h e s t e r n o p e r i c a r d i a l l i g a me n t s . L e s i o n s s e e n i
t h i s r e g i o n i n c l u d e t h y mi c l e s i o n s ( i n c l u d i n g t h y mi c c y s t s ) , l y mp h o i d p r o l i f e r a t i o n s ,
t h y r o i d l e s i o n s , p a r a t h y r o i d l e s i o n s , a n d g e r m c e l l t u mo r s . A d d i t i o n a l t u mo r s t h a t m
b e s e e n i n t h e a n t e r i o r me d i a s t i n u m i n c l u d e l y mp h a n g i o ma s , h e ma n g i o ma s , a n d
l i p o ma s . O n e s h o u l d c o n s i d e r i n f e c t i o u s e t i o l o g i e s , s u c h a s t u b e r c u l o u s
l y mp h a d e n i t i s , a s w e l l a s n o n i n f e c t i o u s e t i o l o g i e s , s u c h a s s a r c o i d o s i s . C o mmo n
a n t e r i o r me d i a s t i n a l l e s i o n s i n c l u d e t h y mo ma s , t e r a t o ma s t(er
a lat
s ooicda l l e d
l e s i o n s ) , l y mp h o ma s , a n d t h y r o i d l e3)s .i o n s (
1. T h y m o m aT h i s i s t h e mo s t c o mmo n n e o p l a s m o f t h e me d i a s t i n u m, a n d o f t e n i t
i s d i s c o v e r e d i n c i d e n t a l l y. B e s i d e s t h y mo ma s , o t h e r t u mo r s o f t h e t h y mu s
i n c l u d e t h y mi c c a r c i n o ma s , t h y mi c l y mp h o ma s , t h y mi c c y s t s , a n d t h y mo l i p o ma s .
A p p r o xi ma t e l y 2 5 % o f a l l me d i a s t i n a l ma s s e s a r e t h y mo ma s a n d a r o u n d h a l f o f
t h e a n t e r i o r me d i a s t i n a l ma s s e s a r e t h y mo ma s . T h y mo ma s g e n e r a l l y p r e s e n t i n
t h e f i f t h d e c a d e , e q u a l l y i n me n a n d w o me n ; t h e y a r e r a r e i n c h i l d r e n . M o s t
t h y mo ma s a r e b e n i g n l e s i o n s .
2. Te r a t o m aF r e q u e n t l y, a n t e r i o r me d i a s t i n a l ma s s e s a r e t e r a t o ma s ( a l s o r e f e r r e d
t o a st er at oi d l es i ons
) . T e r a t o ma s a r e g e r m c e l l t u mo r s t h a t u s u a l l y a r i s e i n
y o u n g a d u l t s f r o m a b n o r ma l l y d e r i v e d e mb r y o n i c l a y e r s . T h e y a r e o f t e n
a s y mp t o ma t i c , b u t i f l a r g e , t h e y ma y h a v e a ma s s e f f e c t w i t h c o mp r e s s i o n o f
a d j a c e n t s t r u c t u r e s . H i s t o l o g i c a l l y, mo s t t e r a t o ma s c o n t a i n e c t o d e r ma l
c o mp o n e n t s ( e . g . , s e b u m, h a i r, t e e t h ) . C o mp u t e d t o mo g r a p h y ( C T ) s c a n s h o w s
t h e c y s t i c c o mp o n e n t a s w e l l a s r e g i o n s o f c a l c i f i c a t i o n . T h e t r e a t me n t o f
t e r a t o ma i s s u r g i c a l e xc i s i o n , a n d t h e p r o g n o s i s i s e xc e l l e n t .
3. L y m p h o m a sT h e s e c o n s t i t u t e a p p r o xi ma t e l y 1 0 % t o 2 0 % o f a l l a n t e r i o r
me d i a s t i n a l ma s s e s . I n c h i l d r e n , l y mp h o ma s a c c o u n t f o r a p p r o xi ma t e l y 2 5 % o f
me d i a s t i n a l ma s s e s . I n a d o l e s c e n t s , a c u t e l y mp h o b l a s t i c l y mp h o ma u s u a l l y
p r e s e n t s a s a n a n t e r i o r me d i a s t i n a l ma s s , o f t e n w i t h t h y mi c i n v o l v e me n t . I n
a d u l t s , mo s t me d i a s t i n a l l y mp h o ma t o u s ma s s e s a r e s e e n i n p a t i e n t s b e t w e e n 3 0
a n d 4 0 y e a r s o f a g e . O n e o f t h e mo s t c o mmo n l y mp h o ma t o u s l e s i o n s i n
r e l a t i v e l y y o u n g a d u l t s , e s p e c i a l l y w o me n , t h a t mo s t o f t e n p r e s e n t s i n t h e
a n t e r i o r me d i a s t i n u m, i s H o d g k i n ' s l y mp h o ma .
4. T h y r o i d l e s i o nPsr e s e n t i n g a s me d i a s t i n a l ma s s e s , t h e s e a r e u s u a l l y
s u b s t e r n a l g o i t e r s e xt e n d i n g i n t o t h e a n t e r i o r me d i a s t i n u m. A t h y r o i d ma s s h e r e
ma y b e a s y mp t o ma t i c , o r i f l a r g e e n o u g h , ma y p r e s e n t w i t h p a i n o r d y s p h a g i a .
M o s t p a t i e n t s a r e w o me n , u s u a l l y o l d e r t h a n 4 0 y e a r s o f a g e a t p r e s e n t a t i o n .
B e s i d e s t h e c o mmo n t y p e s o f l e s i o n s me n t i o n e d , v a r i o u s o t h e r t y p e s o f c y s t i c
n e c k ma s s e s ma y b e p r e s e n t . T h e s e v a r y i n t h e i r h i s t o l o g y a n d e mb r y o g e n e s i s .
C T s c a n a n d r a d i o n u c l e o t i d e s t u d i e s a r e h e l p f u l i n ma k i n g a d e4)t .e r mi n a t i o n (
B. M iddle mediastinum
A p p r o xi ma t e l y 2 0 % o f a l l me d i a s t i n a l ma s s e s a r e l o c a t e d i n t h e mi d d l e me d i a s t i n u m
T hese include the heart; the great vessels to and from the heart; the bifurcation of
t h e t r a c h e a i n t o t h e b r o n c h i ; t h e p e r i c a r d i u m; t h e p h r e n i c n e r v e a r o u n d t h e
640 / 652
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p e r i c a r d i u m; p o r t i o n s o f t h e v a g u s n e r v e ; t h e e s o p h a g u s ; a n d t h e l y mp h n o d e s i n
t h i s r e g i o n , w h i c h i n c l u d e t h e p a r a t r a c h e a l a n d t h e t r a c h e o b r o n c h i a l l y mp h n o d e s .
T h e l e s i o n s t h a t ma y a r i s e f r o m t h e mi d d l e me d i a s t i n u m i n c l u d e a o r t i c a n e u r y s ms ,
d i l a t i o n o f t h e s u p e r i o r v e n a c a v a , d i l a t i o n o f t h e p u l mo n a r y a r t e r y, o r
d i l a t i o n / e n l a r g e me n t o f t h e a zy g o s a n d t h e h e mi a zy g o s v e i n s . L y mp h o ma s , t u mo r s o
t h e h e a r t , p e r i c a r d i a l c y s t s , a n d me t a s t a t i c l e s i o n s a r e o t h e r d i a g n o s t i c
c o n s i d e r a t i o n s i n t h i s c o mp a r t me n t .
C. Posterior mediastinum
A p p r o xi ma t e l y 2 0 % t o 2 5 % o f a l l me d i a s t i n a l ma s s e s p r e s e n t i n t h e p o s t e r i o r
me d i a s t i n u m. C o n t e n t s o f t h e p o s t e r i o r me d i a s t i n u m i n c l u d e t h e e s o p h a g u s , t h e
descending portion of the thoracic aorta, the thoracic duct, a portion of the vagus
n e r v e s , a n d l y mp h n o d e s . N e u r o g e n i c t u mo r s p r e d o mi n a t e i n
P. 4 0 0
t h e p o s t e r i o r me d i a s t i n u m. T h e s e i n c l u d e n e u r o b l a s t o ma s , g a n g l i o n e u r o ma s ,
g a n g l i o n e u r o b l a s t o ma s , n e u r o f i b r o ma s , s c h w a n n o ma s , a n d p h e o c h r 5)
o mo
. c y t o ma s (
L e s s f r e q u e n t l y o c c u r r i n g l e s i o n s i n c l u d e p a r a g a n g l i o ma s , c h e mo d e c t o ma s , a n d
g i a n t l y mp h n o d e h y p e r p l a s i a ( C a s t l e ma n d i s e a s e ) . B e n i g n me s e n c h y ma l l e s i o n s
s u c h a s l i p o ma s , f i b r o ma s , my xo ma s , a n d l e i o my o ma s ma y a l s o b e s e e n . M a l i g n a n t
v e r s i o n s i n c l u d e l i p o s a r c o ma s , f i b r o s a r c o ma s , a n d l e i o my o s a r c o ma s .
D. M ediastinal widening
A o r t i c d i s s e c t i o n i s a n i mp o r t a n t c a u s e o f me d i a s t i n a l w i d e n i n g . O t h e r c a u s e s o f
me d i a s t i n a l w i d e n i n g i n c l u d e a o r t i c r u p t u r e , s t e r n a l f r a c t u r e , p u l mo n a r y c o n t u s i o n s ,
me d i a s t i n a l ma s s e s , t u mo r s o f t h e l u n g , i d i o p a t h i c me d i a s t i n a l f i b r o s i s , c a r d i a c
t a mp o n a d e , a n d l e a k i n g a o r t i c a n e u r y s ms . A l s o , l y mp h o ma s a n d me t a s t a t i c l e s i o n s
c a n c a u s e me d i a s t i n a l w i d e n i n g . S a r c o i d o s i s i s a n i mp o r t a n t a u t o i mmu n e
c o n s i d e r a t i o n . I n f e c t i o u s e t i o l o g i e s o f h i l a r l y mp h a d e n o p a t h y a n d / o r me d i a s t i n a l
w i d e n i n g i n c l u d e my c o b a c t e r i u m t u b e r c u l o s i s , t u l a r e mi a , p e r t u s s i s , v i r a l d i s e a s e s
( i n c l u d i n g h u ma n i mmu n o d e f i c i e n c y v i r u s [ H I V ] a n d E p s t e i n - B a r r v i r u s ) , r i c k e t t s i a l
i n f e c t i o n s , v a r i c e l l a p n e u mo n i a , a n d f u n g a l i n f e c t i o n s ( i n c l u d i n g h i s t o p l a s mo s i s ,
c o c c i d i o i d o my c o s i s ) , a n d t r o p i c a l e o s i n o p h i l i a . A n t h r a x a n d p l a g u e a r e i mp o r t a n t
i n f e c t i o u s c o n s i d e r a t i o n s i n t h e a g e o f b i o t e r r o r i s m. O t h e r mi s c e l l a n e o u s e n t i t i e s
c a u s i n g me d i a s t i n a l w i d e n i n g i n c l u d e G o o d p a s t u r e ' s s y n d r o me , h i s t i o c y t o s i s X ,
c y s t i c f i b r o s i s , a n d i d i o p a t h i c p u l mo n a r y h e mo s i d e r o s i s . F i n a l l y, o c c u p a t i o n a l l u n g
diseases such as silicosis and berylliosis should be considered.
I I I . Evaluation
A c a r e f u l l y d i r e c t e d h i s t o r y a n d p h y s i c a l e xa mi n a t i o n i s e s s e n t i a l b e f o r e i n v e s t i g a t i
s t u d i e s a r e p e r f o r me d t o d e t e r mi n e t h e l i k e l y c a u s e s o f a me d i a s t i n a l ma s s .
A. History
A h i s t o r y o f c o n s t i t u t i o n a l s y mp t o ms , i n c l u d i n g a d o c u me n t a t i o n o f f e v e r, s w e a t , a n d
w e i g h t l o s s i s i mp o r t a n t .
C h e c k v i t a l s i g n s a t t h e t i me o f e xa mi n a t i o n . C h e c k f o r s k i n l e s i o n s a n d e v a l u a t e f o
641 / 652
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s i g n s o f s k i n p a l l o r o r c o n j u n c t i v a l p a l l o r. E xa mi n e t h e n e c k f o r t h y r o me g a l y, ma s s e
o r a d e n o p a t h y. A u s c u l t a t e t h e l u n g s f o r w h e e ze s , r a l e s , r h o n c h i , o r p l e u r a l f r i c t i o n
r u b s . L i s t e n f o r p e r i c a r d i a l r u b s . O n a b d o mi n a l e xa mi n a t i o n , e v a l u a t e f o r l i v e r o r
s p l e e n e n l a r g e me n t . O n g e n i t o u r i n a r y a n d p e l v i c e xa mi n a t i o n s , e v a l u a t e f o r
t e s t i c u l a r a n d / o r s c r o t a l ma s s e s o r o v a r i a n ma s s e s . S i g n i f i c a n c e s h o u l d b e a t t a c h e d
t o t h e p r e s e n c e o f l o c a l i ze d a s w e l l a s g e n e r a l i ze d l y mp h a d e n o p a t h y ( d u e t o
me t a s t a t i c d i s e a s e , H I V o r o t h e r v i r a l i n f e c t i o n s , o r w h e n c o n s i d e r i n g a d i a g n o s i s o
l y mp h o ma ) . O n e s h o u l d r e c o g n i ze t h e s i g n i f i c a n c e o f a p a t h o l o g i c l y mp h n o d e ( 1 c m
o r l a r g e r, p e r s i s t i n g f o r a t l e a s t 4 w e e k s ) .
C. Testing
1. L a b o r a t o r y t e s R
t so u t i n e l a b o r a t o r y t e s t s s h o u l d i n c l u d e a c o mp l e t e b l o o d
c o u n t w i t h d i f f e r e n t i a l , a n d a n e r y t h r o c y t e s e d i me n t a t i o n r a t e , a s w e l l a s s p e c i f i
t e s t s t o b e p e r f o r me d , d e p e n d i n g o n t h e t y p e o f l e s i o n s u s p e c t e d . T h e s e ma y
i n c l u d e l a c t a t e d e h y d r o g e n a s e , f e t o p r o t e i n , f r a c t i o n h u ma n g r o w t h h o r mo n e ,
s e r u m c a l c i u m, p a r a t h o r mo n e , g l o b u l i n s , s e r u m a n t i a c e t y l c h o l i n e r e c e p t o r
a n t i b o d y, p u r i f i e d p r o t e i n d e r i v a t i v e s k i n t e s t , a n d H I V a n t i b o d y s c r e e n i n g .
S u s p i c i o u s o r p a t h o l o g i c p e r i p h e r a l l y mp h n o d e s s h o u l d b e b i o p s i e d .
CgT s c a n i s a v e r y u s e f u l a n d p r e c i s e t o o l f o r l o c a l i zi n g a n d
c h a r a c t e r i zi n g me d i a s t i n a l ma s s e s a n d f o r p r o v i d i n g h e l p f u l c l u e s a s t o t h e i r
n a t u r e 2)( . C T s c a n i s b e t t e r t h a n p l a i n f i l ms a t d e mo n s t r a t i n g t h e c a l c i f i c a t i o n
o f l y mp h n o d e s t h a t c o mmo n l y f o l l o w s t u b e r c u l o s i s a n d f u n g a6)l .i n f e c t i o n s (
M a g n e t i c r e s o n a n c e i ma g i n g i s l e s s o f t e n u s e d t o e v a l u a t e me d i a s t i n a l ma s s e s
b u t ma y h e l p i n t h e e v a l u a t i o n o f p a t i e n t s w i t h s u p e r i o r v e n a c a v a l o b s t r u c t i o n ,
a o r t i c a n e u r y s ms , a n d l a r g e r i n t r a t h o r a c i c b l o o d v e s s e l s . T r a n s t h o r a c i c
u l t r a s o u n d i s u s e f u l i n e v a l u a t i n g t h e t h y mu s i n a d u l t s a n d c h i l d r e n a n d c a n h e l
d i s t i n g u i s h c y s t i c f r o m s o l i d me d i a s t i n a l ma s s e s ; i t c a n a l s o a i d i n d i s t i n g u i s h i n
c a r d i a c f r o m p a r a c a r d i a c ma s s e s . B a r i u m s w a l l o w a n d e n d o s c o p i c u l t r a s o u n d
s t u d i e s a r e u s e f u l a d j u n c t s f o r e v a l u a t i n g ma s s e s w i t h i n t h e mi d d l e me d i a s t i n u m
a s w e l l a s f o r e v a l u a t i n g e s o p h a g e a l ma s s e s a n d l y mp h n o d e s a d j a c e n t t o t h e
e s o p h a g u s6)( .
3. B i o p s yS u s p i c i o u s o r p a t h o l o g i c p e r i p h e r a l l y mp h n o d e s s h o u l d b e b i o p s i e d .
I V. Diagnosis
A. W i d e n i n g o f t h e me d i a s t i n u m c a n b e d u e t o n u me r o u s c o n d i t i o n s . W h e n
evaluating a suspicious lesion, look for clues that help differentiate a
me d i a s t i n a l o r i g i n f r o m
P. 4 0 1
a l u n g , p l e u r a l , o r c h e s t w a l l o r i g i n . M a s s e s w i t h i r r e g u l a r, n o d u l a r, o r s p i c u l a t e
b o r d e r s t e n d t o a r i s e i n t h e l u n g , w h e r e a s b r o a d - b a s e d ma s s e s w i t h s mo o t h
e d g e s a r e mo r e l i k e l y t o a r i s e i n t h e me d i a s t i n u m o r me d i a s t i n a l p l e u r a .
B. T o ma k e a d e f i n i t i v e d i a g n o s i s , a b i o p s y o f t h e me d i a s t i n a l ma s s ma y b e
n e c e s s a r y. H i s t o l o g i c d i a g n o s i s g u i d e s mo r e s p e c i f i c l a b o r a t o r y a n d i ma g i n g
s t u d i e s . A t i s s u e d i a g n o s i s i s a l s o i mp o r t a n t f o r d i s e a s e s t a g i n g a n d t o i n f o r m
642 / 652
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t r e a t me n t o p t i o n s f o r t h e s p e c i f i c c o n d i t i o n .
Acknowledgm ent
T he authors appreciate the input and assistance from Pepi Granat, M D
References
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4 t h e d . N e w Yo r k : C h u r c h i l l L i v i n g s t o n e , 2 0 0 1 .
643 / 652
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18.3 - Osteopenia
> T a b l e o f C o n t e n t s > 1 8 - D i a g n o s t i c I ma g i n g A b n o r ma l i t i e s > 1 8 . 3 - O s t e o p e n i a
18.3
Osteopenia
Scott I ppolito
I . Background
F i f t e e n mi l l i o n p a t i e n t s h a v e o s t e o p o r o s i s a n d a n o t h e r 3 4 mi l l i o n p a t i e n t s h a v e l o w
b o n e ma s s , o r o s t e o p e1)
n i .a O( s t e o p o r o s i s i s c o n s i d e r e d t o b e a c h r o n i c
p r o g r e s s i v e c o n d i t i o n : w h e n l e f t u n t r e a t e d , i t r e s u l t s i n d e c r e a s e d b o n e ma s s a n d
s k e l e t a l f r a g i l i t y. I t i s p r e c e d e d b y o s t e o p e n i a , a l o w b o n e d e n s i t y c o n d i t i o n .
O s t e o p e n i a o c c u r s w h e n r e s o r p t i o n o c c u r s a t a f a s t e r r a t e t h a n f o r ma t i o n . I t h a s
b e e n t h o u g h t o f a s l o w b o n e ma s s o r d e c r e a s e d c a l c i f i c a t i o n o f b o n e w i t h o u t t h e
c l i n i c a l l y i n c r e a s e d r i s k o f f r a1)
c t. uIrfe t h( i s i mb a l a n c e i n t h e b o n e r e mo d e l i n g c y c l e
c o n t i n u e s , o s t e o p e n i a p r o g r e s s e s t o o s t e o2)
p o. rIof sliesf t( u n c h e c k e d ,
o s t e o p o r o s i s c a n l e a d t o f r a c t u r e , d e f o r mi t y, a n d 3)d .i s a b i l i t y (
A. Etiology
T h e p r o c e s s o f b o n e t h i n n i n g i s a n a t u r a l p a r t o f a g i n g : h o w e v e r, i t c a n b e d e l a y e d
me d i c a l l y o r mi n i mi ze d b y l i f e s t y l e c h3)a.n gTehse (d e v e l o p me n t o f o s t e o p o r o s i s
r e s u l t s f r o m d e f e c t i v e b o n e r e mo d e l i n g . O s t e o b l a s t s a r e r e s p o n s i b l e f o r t h e
f o r ma t i o n o f o s t e o i d , o r b o n e ma t r i x. M i n e r a l i za t i o n o f t h i s o s t e o i d ma t r i x p r o d u c e s
b o n e , a n d t h e o s t e o b l a s t s t h a t r e ma i n f o l l o w i n g mi n e r a l i za t i o n a r e t e r me d
os t eoc y t es
. O s t e o b l a s t s r e s p o n d t o a v a r i e t y o f h u mo r a l f a c t o r s s u c h a s e s t r o g e n ,
v i t a mi n D , c y t o k i n e s , a n d v a r i o u s g r o w t h f a c t o r s t h a t s t i mu l a t e 1)
b o. n e g r o w t h (
O s t e o c l a s t s a c t i n d i r e c t o p p o s i t i o n t o o s t e o b l a s t s a n d r e s p o n d t o ma n y s i g n a l s ,
mo s t i mp o r t a n t l y o s t e o p r o t e g e r i n l i g a n d , g r a n u l o c y t e c o l o n y - s t i mu l a t i n g f a c t o r,
P. 4 0 2
a n d i n t e r l e u k i n s t h a t a r e n e c e s s a r y f o r c e l l d e v e l o p me n t . O s t e o c l a s t s a t t a c h t o
e n d o s t e a l b o n e a n d s e c r e t e a c i d t o d i s s o l v e c a l c i u m c r y s t a l s . E n zy me s s u c h a s
me t a l l o p r o t e i n e a s e s t h e n a c t t o b r e a k d o w n t h e p r o t e i n ma t r i x, a n d t h e o s t e o c l a s t
u n d e r g o e s a p o p t o s i s . T h e b r e a k d o w n ma t e r i a l s f r o m t h i s p r o t e i n d e g r a d a t i o n c a n b e
me a s u r e d a s p o s s i b l e ma r k e r s o f b o n e r e s1)
o r. p t i o n (
T h e i mb a l a n c e o f o s t e o c l a s t i c a n d o s t e o b l a s t i c a c t i v i t y c a n b e c a u s e d b y s e v e r a l
age- and disease-related conditions. T hese causes are often classified into three
ma i n c a t e g o r i epsr: i m a r y o s t e o p o r o s( ni so r ma l a g i n g p r o c e s s e s d e c r e a s e g o n a d a l
f u n c t i o n , r e s u l t i n g i n d e c r e a s e d b o n e f o r ma t i o n w i t h o u t d e c l i n i n g o s t e o c l a s t i c
644 / 652
tmdmss
18.3 - Osteopenia
a c t i o n ) p, o s t m e n o p a u s a l o s t e o p o r o( dsei sc l i n i n g e s t r o g e n c a u s e s a n i n c r e a s e i n
o s t e o c l a s t i c a c t i v i t y a n d a r e s u l t i n g i mb a l a n c e b e t w e e n f o r ma t i o n a n d r e s o r p t i o n ) ,
a n d s e c o n d a r y o s t e o p o r o(sdi us e t o s e c o n d a r y c a u s e s , o f t e n f r o m d i s e a s e s , [ s e e
d i f f e r e n t i a l d i a g n o s i s ] , n u t r i t i o n a l d e f i c i e n c i e s o r me d i c a t i o n s t h a t h a v e e f f e c t s o n
c a l c i u m, a n d o t h e r f a c t o r s r e l a t e d t o b o n e f o r ma t i o n o r 1,
r e 4)
s o. r p t i o n ) (
B. Epidemiology
O s t e o p o r o s i s ma i n l y a f f e c t s p o s t me n o p a u s a l w o me n , a l t h o u g h y o u n g e r w o me n a n d
me n c a n a l s o b e a f f e c t e d . T h e p r e v a l e n c e o f o s t e o p o r o s i s b a s e d o n b o n e d e n s i t y a t
t h e f e mo r a l n e c k i s 1 8 % t o 2 8 % i n w o me n a n d 6 % t o 2 2 % i n me n o l d e r t h a n 5 0 y e a r
o f a g e a n d i n c r e a s e s w i t h i n c r e a s i n g a g e . F r a c t u r e s a r e t h e mo s t s e r i o u s
c o n s e q u e n c e o f o s t e o p o r o s i s . C u r r e n t l y, i t h a s b e e n e s t i ma t e d t h a t mo r e t h a n 5 0 %
o f w o me n a n d 2 0 % o f me n a t t h e a g e o f 5 0 w i l l e xp e r i e n c e a t l e a s t o n e o s t e o p o r o t i c
f r a c t u r e i n t h e i r l i f e t i me
5) . sT (h e d i s e a s e c u r r e n t l y r e s u l t s i n mo r e t h a n 3 5 0 , 0 0 0 h i p
f r a c t u r e s a n d 7 0 0 , 0 0 0 v e r t e b r a l f r a c t u r e s e a c h y e a r i n t h e U n4)i t. e d S t a t e s (
B e c a u s e me n a n d w o me n o l d e r t h a n 6 5 y e a r s o f a g e a r e t h e f a s t e s t g r o w i n g
s e g me n t o f t h e p o p u l a t i o n w o r l d w i d e , t h e a n n u a l n u mb e r o f f r a c t u r e s i s e xp e c t e d t o
d o u b l e b y 2 0 26)
5 .( W i t h r e s p e c t t o r a c e , w h i t e w o me n a r e t h e mo s t o f t e n a f f e c t e d
a n d A f r i c a n - A me r i c a n w o me n h a v e t h e l o w e s t p r e v a l e n c e o f o 1)
s t .e o p o r o s i s (
N u me r o u s r i s k f a c t o r s p r e d i c t l o w b o n e mi n e r a l d e n s i t y ( B M D ) a n d t h e d e v e l o p me n t
of osteoporosis and resulting fracture. Risk factors include age, white race, tobacc
u s e , f e ma l e g e n d e r, l o w b o d y w e i g h t , p h y s i c a l i n a c t i v i t y, f a mi l y h i s t o r y o f
o s t e o p o r o s i s , me d i c a t i o n s ( i . e . , g l u c o c o r t i c o i d s , a n t i c o n v u l s a n t s , e xc e s s t h y r o i d
h o r mo n e ) , p r e v i o u s f r a c t u r e , e s t r o g e n d e f i c i e n c y, e xc e s s i v e a l c o h o l i n t a k e , a n d l o w
c a l c i u m, p h o s p h o r u s , o r v i t a mi n D 1,
i n t3,a k4)e . ( C o n s u mi n g l a r g e q u a n t i t i e s o f s o f t
d r i n k s h a s b e e n l i n k e d t o c a u s i n g l o w b o n e d e n s i t y, b e c a u s e t h e y c o n t a i n h i g h l e v e
o f p h o s p h o r i c a c i d , w h i c h i n t e r f e r e s w i t h c a l c i u m a b s o r p t i o n . Vi t a mi n A , t a k e n i n
e xc e s s o f $ 1 , 2 0 0 g a d a y, a c c e l e r a t e s b o n e l o s s . F i n a l l y, t e s t o s t e r o n e p r o t e c t s
me n ; n e v e r t h e l e s s , b o n e l o s s ma y o c c u r w h e n h o r mo n e l e v 3)
e l .s d e c l i n e (
I I I . Evaluation
A. History
A s k f e ma l e p a t i e n t s a b o u t t h e i r me n s t r u a l h i s t o r y, a g e o f me n a r c h e a n d me n o p a u s e ,
p e r i o d s o f a me n o r r h e a , p r e g n a n c y, l a c t a t i o n , a n d o r a l c o n t r a c e p t i v e . A s k a l l p a t i e n t
a b o u t i l l n e s s e s , me d i c a t i o n s , f a l l s , a n o r e xi a a n d o t h e r e a t i n g d i s o r d e r s , s t e r o i d u s e
c a l c i u m a n d v i t a mi n D i n t a k e , a l c o h o l a n d t o b a c c o u s e , e xe r c i s e , a n d f a mi l y h i s t o r y
o f o s t e o p o r o s i s . A s k ma l e p a t i e n t s a b o u t s y mp t o ms s u g g e s t i v e o f l o w t e s t o s t e r o n e
levels.
O b s e r v e f o r a b n o r ma l g a i t , p o s t u r e , a n d b a l a n c e . L o o k f o r k y p h o s i s , a b n o r ma l s p i n a
c u r v a t u r e , a n d a s y mme t r y o f t h e p a r a v e r t e b r a l mu s c u l a t u r e . F o c a l t e n d e r n e s s o n
p a l p a t i o n o v e r t h e v e r t e b r a l p r o c e s s e s ma y s u g g e s t v e r t e b r a l f r a c t u r e . O b s e r v e
o t h e r b o n e s f o r d e f o r mi t i e s . N o t e s i g n s o f p o o r n u t r i t i o n . E xa mi n e f o r a n y p h y s i c a l
ma n i f e s t a t i o n s o f l o w t e s t o s t e r o n e i n ma l e p a t i e n t s .
645 / 652
tmdmss
18.3 - Osteopenia
C. Testing
B go n e d e n s i t o me t r y i s a n o n i n v a s i v e t e c h n i q u e t h a t i s u s e d
t o me a s u r e t h e b o n e mi n e r a l c o n t e n t i n o r d e r t o p r e d i c t f r a c t u r e r i s k s a n d t h e
n e e d f o r me d i c a l t h e r a p y. B M D i s t y p i c a l l y e xp r e s s e d a s t h e T s c o r e ( e . g . , t h e
n u mb e r o f s t a n d a r d d e v i a t i o n s ( S D s ) b e l o w t h e me a n f o r n o n o s t e o p e n i c , h e a l t h y
y o u n g w o me n7)) (. S i mi l a r l y, a Z s c o r e i s t h e n u mb e r o f S D s f r o m t h e me a n b o n e
d e n s i t y f o r a g e - ma t c h e d , s e x- ma t c h e d , a n d e t h n i c - ma t c h e1)d. pTaht iee n t s (
Wo r l d H e a l t h O r g a n i za t i o n d e f i n e s o s t e o p e n i a a s a T s c o r e o f b e t w e e n - 1 . 0 a n d
- 2 . 5 S D , a n d o s t e o p o r o s i s a s a s c o r e o f - 2 . 5 S D7)o. r Rmo
e creen t( l y, s e v e r a l
d i a g n o s t i c t e c h n i q u e s h a v e c o me t o t h e ma r k e t , mo s t n o t a b l y t h e d u a l - e n e r g y
P. 4 0 3
x- r a y a b s o r p t i o me t r y ( D E X A ) . H o w e v e r, u l t r a s o u n d , c o mp u t e d t o mo g r a p h y ( C T )
s c a n , ma g n e t i c r e s o n a n c e i ma g i n g ( M R I ) , r a d i o g r a p h i c a b s o r p t i o me t r y, d u a l p h o t o n a b s o r p t i o me t r y, a n d d u a l x- r a y a n d l a s e r h a v e a l s o b e e n s u g g e s t e d a s
me t h o d s o f b o n e me a s u r e me
1) .n t (
a. D E X A i s t h e mo s t c o mmo n l y u s e d g o l d s t a n d a r d t e c h n i q u e t o me a s u r e
B M D . D E X A u s e s t w o x- r a y b e a ms o f d i f f e r e n t e n e r g y l e v e l s t o s c a n t h e
r e g i o n o f i n t e r e s t a n d me a s u r e t h e a mo u n t o f x- r a y a b s o r b e d b y t h e b o n e a s
t h e b e a m p a s s e s t h r o u g h t h e 7)
b o. dD
y E( X A me a s u r e s t h e s u m o f c o r t i c a l
a n d t r a b e c u l a r b o n e a n d c a n d e t e c t a s l i t t l e a s 2 % 1)
b o. nTehleo s s (
c u r r e n t p r a c t i c e i s t o p e r f o r m D E X A o f t h e l u mb a r v e r t e b r a e ( L 1 - 4 ) ; t h e h i p ,
i n c l u d i n g t h e f e mo r a l n e c k , Wa r d ' s t r i a n g l e , t h e g r e a t e r t r o c h a n t e r, a n d t h e
t o t a l h i p ( w h i c h i n c l u d e s a l l t h e s e me a s u r e s ) ; o r b o t h . T h e r e s u l t s a r e
p r e s e n t e d v i s u a l l y, i n c l u d i n g b o t h T s c o r e s a n d 8)
Z.scores (
b. C T s c a n d e p e n d s o n t h e d i f f e r e n t i a l a b s o r p t i o n o f r a d i a t i o n b y c a l c i f i e d
b o n e a n d i s u s e d f o r c e n t r a l me a s u r e me n t s o n l y. C o mp a r e d t o D E X A , C T
scan is less readily available and is associated with relatively high radiation
e xp o s u r e7)( .
c. M R I i n d i a g n o s i n g o s t e o p o r o s i s i s s t i l l e v o l v i n g . M o r e r e s e a r c h mu s t b e
d o n e t o i mp r o v e t h e s e n s i t i v i t y a n d s p e c i f i c i t y o f M R I a s w e l l a s t o c a l c u l a t e
a p p r o p r i a t e T a n d Z s c o1)
r e. s (
d. U l t r a s o u n d me a s u r e s t h e b o n e ma s s a n d s t r e n g t h a n d a s s e s s e s t h e b o n e
mi c r o a r c h i t e c t u r e b y d e t e c t i n g t h e t r a n s mi s s i o n o f h i g h - f r e q u e n c y s o u n d
w a v e s t h r o u g h b o n7)e. (T h e c a l c a n e u s i s t h e p r i ma r y s i t e o f me a s u r e me n t
(1) . H o w e v e r, t h i s t e c h n i q u e h a s n o t b e e n s h o w n t o b e u s e f u l i n mo n i t o r i n g
s k e l e t a l r e s p o n s e t o t h e d i f f e r e n t t h e r a p i e s u s e d t o t r e a t o7)
s t.e o p o r o s i s (
e. R a d i o g r a p h i c a b s o r p t i o me t r y p r o v i d e s r a d i o l o g i c a s s e s s me n t o f t h e
me t a c a r p a l s a n d p h a l a n g e s o n t h e b a s i s o f p l a i n f i l m. H o w e v e r, r a d i o g r a p h s
a r e a n i n s e n s i t i v e me a s u r e o f b o n e l o s s a n d ma y d e mo n s t r a t e a b n o r ma l i t i e s
o n l y a f t e r 3 0 % o f b o n e l o s s h a s o c c u r r e d . G e n e r a l l y, i t i s n o t r e c o mme n d e d
a s a s c r e e n i n g t e s t f o r o s t e o p o r o s i s o r o s t 1)
e o. p e n i a (
f. D u a l - p h o t o n a b s o r p t i o me t r y me a s u r e s b o n e mi n e r a l c o n t e n t a t t h e s p i n e
a n d h i p u s i n g p h o t o n s e mi t t e d a t l o w e n e r g y l e v e l s . I t i s a l s o u s e d t o
me a s u r e t h e t o t a l b o d y c a l c i u m a n d p r o v i d e s a me a s u r e me n t o f t h e mi n e r a l
646 / 652
tmdmss
18.3 - Osteopenia
d e n s i t y i n b o t h l o n g b o n e s a n d b o n e s s u c h a s t h e h e e l b y me a s u r i n g t h e
t o t a l mi n e r a l c o n t e n t i n t h e p a t h o f t h 7)
e .b e a m (
g. D u a l x- r a y a n d l a s e r i s a n e w t e c h n i q u e t h a t i s p r e s e n t l y b e i n g r e s e a r c h e d .
I t u s e s t w o x- r a y b e a ms i n c o mb i n a t i o n w i t h a l a s e r. R e s e a r c h e r s h a v e
suggested that this technique has the advantage of filtering out any
i n f l u e n c e t h a t a d i p o s e t i s s u e ma y h a v e o n t h e a c c u r a c y o f D E X A
me a s u r e me n t s7) (.
2. L a b o r a t o r y t e s Lt sa b o r a t o r y a s s e s s me n t i s n o t r o u t i n e l y u s e d t o s c r e e n f o r t h e
p r e s e n c e o f o s t e o p o r o s i s b u t i t ma y b e u s e f u l i n p a t i e n t s w i t h l o w b o n e d e n s i t y
with the goal of identifying secondary causes (such as elevated serum calcium
l e v e l s , s u g g e s t i n g h y p e r p a r a t h y r o i d i s m) o r f a c t o r s t h a t c a n a g g r a v a t e b o n e
f r a g i l i t y ( s u c h a s a l o w l e v e l o f 2 5 - h y d r o xy v i t a mi n D ) . B i o c h e mi c a l ma r k e r s o f
i n c r e a s e d b o n e r e s o r p t i o n ( i . e . , c o l l a g e n c r o s s - l i n k s , c a l c i u m, a n d
h y d r o xy p r o l i n e ) o r i n c r e a s e d b o n e f o r ma t i o n ( i . e . , b o n e - s p e c i f i c a l k a l i n e
phosphatase and osteoclastin) are associated with an increased fracture risk.
H o w e v e r, t h e s e ma r k e r s s h o w s u b s t a n t i a l v a r i a b i l i t y, a n d t h e r e a r e i n s u f f i c i e n t
d a t a t o s u p p o r t t h e i r u s e i n d e c i d i n g f o r o r a g a i n s t b o n e d e n s i t o me t r y o r
p h a r ma c o t h e r a p8)
y .(
I V. Diagnosis
T h e r a d i o g r a p h i c f i n d i n g o f g e n e r a l i ze d l o s s o f b o n e d e n s i t y ( o s t e o p e n i a ) i s n o t
specific and can be seen in various conditions. Disorders associated with
g e n e r a l i ze d l o s s o f b o n e d e n s i t y i n c l u d e d i s o r d e r s o f mu l t i p l e o r u n c e r t a i n c a u s e
( s e n i l e o s t e o p o r o s i s , j u v e n i l e o s t e o p o r o s i s , o s t e o g e n e s i s i mp e r f e c t a ) , a d r e n a l c o r t e
disorders ( C ushing's disease, Addison's disease), gonadal disorders
( p o s t me n o p a u s a l o s t e o p o r o s i s , h y p o g o n a d i s m) , p i t u i t a r y d i s o r d e r s ( a c r o me g a l y,
h y p o p i t u i t a r i s m) , d i a b e t e s me l l i t u s , t h y r o i d d i s o r d e r s ( h y p e r t h y r o i d i s m,
h y p o t h y r o i d i s m) , h y p o p a r a t h y r o i d i s m, ma r r o w r e p l a c e me n t a n d e xp a n s i o n ( my e l o ma ,
l e u k e mi a , l y mp h o ma , me t a s t a t i c d i s e a s e , G a u c h e r ' s d i s e a s e ) , a n e mi a s ( s i c k l e c e l l ,
t h a l a s s e mi a ,
P. 4 0 4
h e mo p h i l i a ) , d r u g s a n d o t h e r s u b s t a n c e s ( s t e r o i d s , h e p a r i n , a n t i c o n v u l s a n t s ,
i mmu n o s u p p r e s s a n t s , a l c o h o l ) , a n d c h r o n i c d i s e a s e s ( c h r o n i c r e n a l d i s e a s e , h e p a t i c
i n s u f f i c i e n c y, g a s t r o i n t e s t i n a l ma l a b s o r p t i o n s y n d r o me s , c h r o n i c i n f l a mma t o r y
p o l y a r t h r o p a t h i e s , a my l o i d o s i s , s a r c o i d o s i s , c h r o n i c d e b i l i t y o r i mmo
4, 9)b. i l i za t i o n ) (
References
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G mb H . 2 0 0 4 ; 1 ( 2 3 ) : 2 0 4 2 1 4 .
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y.
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tmdmss
18.4 - Solitary Pulmonary Nodule
> T a b l e o f C o n t e n t s > 1 8 - D i a g n o s t i c I ma g i n g A b n o r ma l i t i e s > 1 8 . 4 - S o l i t a r y
P u l mo n a r y N o d u l e
18.4
Solitary Pulmonary Nodule
Mark R. Ne e dham
I . Background
P u l mo n a r y n o d u l e s , g e n e r a l l y c o n s i d e r e d t o b e i s o l a t e d a n d c i r c u ms c r i b e d l e s i o n s
< 3 c m i n d i a me t e r i n t h e l u n g s , a r e a c o mmo n a n d s o me w h a t v e xi n g c l i n i c a l f i n d i n g .
A l s o c a l l e d c o i n l e s i o n s , t h e y a r e t y p i c a l l y f o u n d i n c i d e n t a l l y o n c h e s t x- r a y s
o b t a i n e d f o r o t h e r p u r p o s e s . T h e y a r e a l s o v e r y c o mmo n l y d e t e c t e d ; c o mp u t e d
t o mo g r a p h y ( C T ) s c a n i s u s e d f o r p u r p o s e s o f s c r e e n i n g f o r l u n g c a n c e r. T h e e n t i r e
purpose of any evaluation is to resect potentially curable lesions while avoiding
unnecessary and risky interventions. Toward that end, the physician needs to
d e t e r mi n e w h i c h o f t h e f o l l o w i n g t h r e e a p p r o a c h e s t o u s e : o b s e r v a t i o n w i t h s e r i a l
mo n i t o r i n g , b i o p s y, o r i mme d i a t e e xc i s i o n . T h e p r i ma r y me d i c a l l e g a l p i t f a l l i s t o
i g n o r e a p o t e n t i a l l y r e s p e c t a b l e a n d c u r a b l e e a r l y - s t a g e l u n g c a n c e r. H o w e v e r, a n
o v e r l y a g g r e s s i v e a p p r o a c h ma y l e a d t o u n n e c e s s a r y p r o c e d u r e s a n d c o mp l i c a t i o n s
s u c h a s p n e u mo t h o r a x o r p u l mo n a r y h e mo r r h a g e .
P u l mo n a r y n o d u l e s ma y b e o f e i t h e r b e n i g n o r ma l i g n a n t e t i o l o g y. A l l t y p e s
c a n c e r ma y p r e s e n t i n i t i a l l y a s a s o l i t a r y n o d u l e , i n c l u d i n g c a r c i n o i d t u mo r
me t a s t a s i s t o t h e l u n g . B e n i g n c a u s e s i n c l u d e i n f e c t i o n , p a r t i c u l a r l y f u n g a l
g r a n u l o ma t o u s d i s e a s e , h a ma r t o ma s o r b e n i g n t u mo r s o f t h e l u n g , l i p o ma s ,
l e s i o n s , r h e u ma t o i d a r t h r i t i s , s a r c o i d o s i s , a n d b r o n c h o1)g .e n i c c y s t (
of lung
and
and
vascular
I I I . Evaluation
A. History
H i s t o r y i s n o t d e f i n i t i v e b u t c a n a s s i s t i n mo v i n g t h e o d d s t o w a r d b e n i g n o r
ma l i g n a n t . A g e y o u n g e r t h a n 4 0 y e a r s p r o b a b l y c a r r i e s < 3 % c h a n c e o f ma l i g n a n c y.
A g e o l d e r t h a n 6 0 y e a r s c a r r i e s > 5 0 % c h a n c e o f ma l i g n a n c y. P a s t s mo k i n g h i s t o r y i
s t r o n g l y a s s o c i a t e d w i t h ma l i g n a n c y. A s b e s t o s o r o c c u p a t i o n a l c a r c i n o g e n
P. 4 0 5
e xp o s u r e i n c r e a s e s t h e p r o b a b i l i t y o f ma l i g n a n c y. A p r e v i o u s l y d i a g n o s e d ma l i g n a n c
i n c r e a s e s t h e o d d s t h a t t h e n o d u l e i s a me t a s t a s i s . A l e s i o n l a r g e r t h a n 3 c m i s
h i g h l y l i k e l y t o b e ma l i g n a n t . D o u b l i n g t i me i s a s i g n i f i c a n t h i s t o r i c a l o b s e r v a t i o n t h
ma y c o n t r i b u t e t o t h e d e c i s i o n - ma k i n g p r o c e s s , t h e r e b y ma k i n g t h e r e v i e w o f a n y
649 / 652
tmdmss
a v a i l a b l e p r i o r c h e s t x- r a y s e s s e n t i a l . B e n i g n l e s i o n s h a v e e i t h e r a v e r y r a p i d g r o w
r a t e , w i t h d o u b l i n g t i me s < 2 0 d a y s s e e n i n i n f e c t i o u s c o n d i t i o n s , o r e xt r e me l y l o n g
d o u b l i n g t i me s . M a l i g n a n t n o d u l e s g e n e r a l l y h a v e d o u b l i n g t i me s b e t w e e n 2 0 d a y s
a n d 2 y e a r s . A n o d u l e t h a t h a s b e e n p r e s e n t a n d i s u n c h a n g e d f o r 2 y e a r s i s a l mo s t
c e r t a i n t o b e b e n i g n . N o t e t h a t b e c a u s e p l a i n x- r a y s p r o j e c t a n i ma g e o f a s p h e r e
o n t o a t w o - d i me n s i o n a l p l a n e , a n i n c r e a s e o f 2 6 % i n d i a me t e r, f o r e xa mp l e , f r o m 1
c m t o 1 . 3 c m, i s e q u i v a l e n t t o o n e d o u b l i n g i n v o l u me o f t h e n o d u l e . We l l c i r c u ms c r i b e d b o r d e r s f a v o r b e n i g n l e s i o n s , w h e r e a s i r r e g u l a r o r s p i c u l a t e d b o r d e r s
f a v o r ma l i g n a n c2)y . (
T h e p r e s e n c e o f f e v e r s u g g e s t s a p o s s i b l e i n f e c t i o u s e t i o l o g y. E xt r a p u l mo n a r y
ma n i f e s t a t i o n s o f c e r t a i n d i s e a s e s s u c h a s t u b e r c u l o s i s , s a r c o i d o s i s , o r r h e u ma t o i d
a r t h r i t i s ma y g i v e s o me a d d i t i o n a l c l u e s t o t h e d i a g n o s i s . H o w e v e r, t h e c o n t r i b u t i o n
o f p h y s i c a l d i a g n o s i s i s l i mi t e d .
C. Testing
I f t h e n o d u l e i s d e t e c t e d o n p l a i n x- r a y, t h e n s u b s e q u e n t a d d i t i o n a l s t u d i e s c a n
i n c l u d e C T s c a n , f i b e r o p t i c b r o n c h o s c o p y, p e r c u t a n e o u s f i n e n e e d l e a s p i r a t i o n ,
ma g n e t i c r e s o n a n c e i ma g i n g ( M R I ) , u l t r a s o u n d , s i n g l e p h o t o n e mi s s i o n c o mp u t e d
t o mo g r a p h y ( S P E C T ) , p o s i t r o n e mi s s i o n t o mo g r a p h y ( P E T ) , a n d v i d e o - a s s i s t e d
t h o r a c i c s u r g e r y ( VAT S ) f o r e xc i s i o n .
1. A C T s c a n c a n b e u s e f u l i n t h a t s ma l l n o d u l e s n o t s e e n o n p l a i n x- r a y ma y b e
d e t e c t e d b y i t . T h e p r e s e n c e o f mu l t i p l e n o d u l e s f a v o r s a b e n i g n c a u s e , e xc e p t
w i t h t h e h i s t o r y o f p r i o r n e o p l a s m, i n w h i c h c a s e me t a s t a t i c d i s e a s e i s mo r e
l i k e l y. A C T s c a n ma y a l s o d e mo n s t r a t e c e r t a i n p a t t e r n s o f c a l c i f i c a t i o n w i t h i n
t h e n o d u l e t h a t c a n n o t b e v i s u a l i ze d o n p l a i n x- r a y. A s t i p p l e d o r e c c e n t r i c
p a t t e r n o f c a l c i f i c a t i o n f a v o r s ma l i g n a n c y, w h e r e a s o t h e r c h a r a c t e r i s t i c p a t t e r n s
ma y f a v o r a b e n i g n e t i o l o g y. B e n i g n l e s i o n s t e n d t o h a v e h i g h e r H o u n s f i e l d u n i t
o n C T s c a n , a me a s u r e o f d e n s i t y, b u t t h e r e i s n o d i s t i n c t c u t o f f p o i n t b e t w e e n
b e n i g n a n d ma l i g n a n t l e s i o n s .
2. F i b e r o p t i c b r o n c h o s c o p y g e n e r a l l y h a s l o w y i e l d , p a r t i c u l a r l y w i t h p e r i p h e r a l
nodules.
3. F i n e n e e d l e a s p i r a t i o n h a s a r e a s o n a b l e y i e l d w i t h p e r i p h e r a l n o d u l e s > 2 c m b u
c a r r i e s a s i g n i f i c a n t r i s k o f p n e u mo t h o r a x.
4. M R I a n d u l t r a s o u n d d o n o t p l a y mu c h o f a r o l e i n t h e d i a g n o s t i c e v a l u a t i o n .
5. S P E C T a n d P E T , w i t h f l u o r o d e o xy g l u c o s e u s e d a s a b i o l o g i c a l ma r k e r, a r e
p r o mi s i n g t e c h n i q u e s f o r n o n i n v a s i v e d i a g n o s i s b u t a r e l i mi t e d b y f a l s e - p o s i t i v e
r e s u l t s i n me t a b o l i c a l l y a c t i v e n o d u l e s s u c h a s i n f e c t i o u s g r a n u l o ma s a n d
i n f l a mma t o r y c o n d i t i o n s . F a l s e - n e g a t i v e r e s u l t s c a n o c c u r b e c a u s e o f t h e l i mi t e d
s p e c i a l r e s o l u t i o n o f P E T , w h i c h c a n f o r m a n i ma g e o n l y d o w n t o a p p r o xi ma t e l y
8 mm, t h e r e b y p o s s i b l y n o t d e t e c t i n g ma l i g n a n c i e s s ma l l e r t h a n a b o u t 1 c m. I n
a d d i t i o n , s o me t u mo r s , s u c h a s c a r c i n o i d a n d b r o n c h o a l v e o l a r c a r c i n o ma , h a v e
v e r y l o w me t a b o l i c r a t e s a n d c a n b e mi
4) .s s e d (
650 / 652
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6. T h e a d v a n t a g e o f VAT S i s t h a t t h e p r o c e d u r e i s b o t h d i a g n o s t i c a n d t h e r a p e u t i c
H o w e v e r, i t i s mo r e c o s t l y a n d mo r e i n v a s i v e w i t h i n h e r e n t c o mp l i c a t i o n s o f
p n e u mo t h o r a x a n d h e mo r r h3)
a g. e (
I V. Diagnosis
T h e d i a g n o s i s o f s o l i t a r y p u l mo n a r y n o d u l e i s u s u a l l y ma d e t h r o u g h p l a i n x- r a y. T h e
d i f f e r e n t i a l d i a g n o s i s i n c l u d e s p r i ma r y o r me t a s t a t i c l u n g ma l i g n a n c y. F u r t h e r
e v a l u a t i o n o r mo n i t o r i n g d e p e n d s o n t h e c l i n i c a l s e t t i n g . W h e n e v e r p o s s i b l e , o b t a i n
o l d c h e s t x- r a y s f o r c o mp a r i s o n . A n o d u l e u n c h a n g e d o v e r a p e r i o d o f 2 y e a r s i s
a l mo s t c e r t a i n t o b e b e n i g n . O t h e r r e c o mme n d a t i o n s i n c l u d e t h e f o l l o w i n g :
A. F o r n o d u l e s d e t e c t e d i n p a t i e n t s y o u n g e r t h a n 4 0 y e a r s o f a g e , w i t h n o p r i o r
s mo k i n g h i s t o r y, n o o c c u p a t i o n a l e xp o s u r e r i s k s , n o k n o w n p r i o r ma l i g n a n c i e s ,
a n d s mo o t h w e l l - c i r c u ms c r i b e d b o r d e r s , c o n s i d e r s e r i a l mo n i t o r i n g . H o w e v e r, t h
p a t i e n t mu s t b e s t a b l e i n t h e p r a c t i c e s u c h t h a t s e r i a l c h e s t x- r a y s a t 3 - , 6 - , 1 2 a n d 2 4 - mo n t h i n t e r v a l s ma y b e o b t a i n e d . E l e c t VAT S f o r r e s e c t i o n f o r n o d u l e s
i n c r e a s i n g i n s i ze o v e r p r i o r x- r a y.
P. 4 0 6
B. F o r n o d u l e s d e t e c t e d i n p a t i e n t s o l d e r t h a n 4 0 y e a r s o f a g e , w i t h p r i o r s mo k i n g
h i s t o r y, a n y o c c u p a t i o n a l c a n c e r r i s k s , k n o w n p r i o r ma l i g n a n c i e s , a n d
s p e c u l a t e d b o r d e r s o r s u s p i c i o u s c a l c i f i c a t i o n p a t t e r n s , p r o c e e d t o VAT S f o r
r e s e c t i o n . I f t h e p a t i e n t i s a t a h i g h s u r g e r y r i s k b e c a u s e o f i mp a i r e d p u l mo n a r y
r e s e r v e o r o t h e r c o mp l i c a t i o n , c o n s i d e r b i o p s y t o v e r i f y d i a g n o s i s p r i o r t o
r e s e c t i o n f o r n o d u l e s > 2 c m i n s i ze l o c a t e d i n t h e p e r i p h e r y.
C. F o r p a t i e n t s w h o h a v e n o d u l e s t h a t d o n o t f i t i n t o t h e f i r s t t w o c a t e g o r i e s , t h e
o p t i o n s a r e n o t a s c l e a r, a n d p a t i e n t p r e f e r e n c e c a n p l a y a r o l e i n e s t a b l i s h i n g
t h e w o r k - u p . C h o i c e s i n c l u d e C T s c a n ; t h e p r e s e n c e o f mu l t i p l e n o d u l e s ,
d e n s i t y > 1 6 0 H U , o r c h a r a c t e r i s t i c c a l c i f i c a t i o n p a t t e r n ma y f a v o r b e n i g n
e t i o l o g y. A b s e n c e o f t h e s e f i n d i n g s ma y s u g g e s t ma l i g n a n c y. F o r n o d u l e s l a r g e r
t h a n 1 c m i n a n o t h e r w i s e i n d e t e r mi n a t e s i t u a t i o n , c o n s i d e r P E T , r e c o g n i zi n g
t h a t f a l s e - n e g a t i v e a n d f a l s e - p o s i t i v e r e s u l t s ma y o c c u r.
D. I n s i t u a t i o n s i n w h i c h t h e c h o i c e i s ma d e t o n o t p r o c e e d w i t h VAT S a n d
r e s e c t i o n , i mme d i a t e l y f o l l o w t h e p a t i e n t w i t h s e r i a l c h e s t x- r a y s a t 3 , 6 , 1 2 , a n d
2 4 mo n t h s a n d p r o c e e d t o r e s e c t i o n f o r a n y c h a n g e i n n5)
o d. u l e s i ze (
References
1 . We i n b e r g e r M D , S t e v e n E . D i f f e r e n t i a l d i a g n o s i s a n d e v a l u a t i o n o f t h e s o l i t a r y
p u l mo n a r y n o d u lUep. t o D at e O nl i ne R ef er,enc
2 0 e0 5 .
2 . M a n o c h a S a n j a y. S o l i t a r y P u l mo n a r y N o d u l e . E M e d i c i n e O n l i n e R e f e r e n c e ,
2005.
4 . G o u l d M K , M a c l e a n C C , K u s c h n e r W G , e t a l . A c c u r a c y o f p o s i t r o n e mi s s i o n
t o mo g r a p h y i n t h e d i a g n o s i s o f p u l mo n a r y n o d u l e s a n d ma s s l e s i o n s : a me t a
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a n a l y s i sJ. A M A 2 0 0 1 ; 2 1 : 9 1 4 9 2 4 .
3 . B e r n a r d A . R e s e c t i o n o f p u l mo n a r y n o d u l e s u s i n g v i d e o a s s i s t e d t h o r a c i c
s u r g e r y.A nn T hor ac S ur1g9 9 6 ; 6 1 : 2 0 2 .
5 . H a r t ma n T E . E v a l u a t i o n o f t h e s o l i t a r y p u l mo n a rRy adi
n o ol
d u lCe l. i n N or t h
Am 2005;43(3):459465.
652 / 652

Taylors.10 Minute - Diagnosis.manual - Symptoms.and - Signs.in - The.time Limited - Encounter.

Diunggah oleh

Informasi Dokumen

Judul Asli

Hak Cipta

Format Tersedia

Bagikan dokumen Ini

Bagikan atau Tanam Dokumen

Opsi Berbagi

Apakah menurut Anda dokumen ini bermanfaat?

Apakah konten ini tidak pantas?

Hak Cipta:

Format Tersedia

Taylors.10 Minute - Diagnosis.manual - Symptoms.and - Signs.in - The.time Limited - Encounter.

Diunggah oleh

Hak Cipta:

Format Tersedia

Table of Contents

6.4 - Nosebleed ............................................................................................................................................................................. 185

7.1 - Atypical Chest Pain ........................................................................................................................................................... 212

9.5 - Dysphagia ............................................................................................................................................................................. 323

10 - Renal and Urologic Problems

10.1 - Dysuria ................................................................................................................................................................................. 361

11 - Problems Related to the Female Reproductive System .................................................................... 402

17.4 - Brain Natriuretic Peptide .............................................................................................................................................. 611

18 - Diagnostic Imaging Abnormalities

Editors and Authors

Editors and Authors

Lase rw ords Priv ate Lim ite d, Che nnai, I ndia

Editors and Authors

Editors and Authors

C ont ribut ors

Editors and Authors

Editors and Authors

Editors and Authors

10.8 Scrotal Mass

Editors and Authors

Editors and Authors

Associate C linical P rofessor

Editors and Authors

Editors and Authors

Editors and Authors

M edi c i ne, N ebr as k a M edi c al C ent er, O maha, N ebr as k a

Editors and Authors

Editors and Authors

Editors and Authors

Editors and Authors

Editors and Authors

Editors and Authors

Editors and Authors

Editors and Authors

S t udent H eal t h S er v i c es , U ni v er s i t y of P enns y l v ani a; S t af f P hy s i c i an, D epar t ment

Hal S. Shim azu M D

Editors and Authors

B r emer t on, Was hi ngt on

Editors and Authors

P enns y l v ani a, P hi l adel phi a

Preface to the Second Edition

Preface to the Second Edition

Tay l or ' s 10- M i nut e D i agnos i s M, anual

Preface to the First Edition

Preface to the First Edition

T he 10- M i nut e D i agnos i s M anual

[-] 2 - Undifferentiated Problems

[-] 6 - Ear, Nose, and Throat Problems

[+] 9.7 - Upper Gastrointestinal Bleeding

[+] 13.3 - Maculopapular Rash

[+] 18.3 - Osteopenia

1 - Principles of the 10-Minute Diagnosis

Search for Diagnostic Cues T hroughout the

1 - Principles of the 10-Minute Diagnosis

gastroesophageal reflux disease because the patient has a past history of

T hink Most Com m on First

1 - Principles of the 10-Minute Diagnosis

Use all Available Assistance

Consider the Psychosocial Aspects of the Problem

W hen el i c i t i ng a medi c al hi s t or y f r om J oan S . , i t wi l l be i mpor t ant t o l ear n t he

Seek Help When Needed

1 - Principles of the 10-Minute Diagnosis

T hink in Term s of a Continually Evolving