HIGH YIELD PEDIATRICS2 SE2 REVIEWER (PEDIATRIC INFECTIOUS DISEASES)

I.

OVERVIEW: PEDIATRICS INFECTIOUS DISEASES

Top 5 leading causes of morbidity:

1.
Pneumonia
2.
Diarrhea
3.
Bronchitis/ Bronchiolitis
4.
Influenza
5.
Hypertension

Special problems of children:

1.
First exposure - exposure to an agent for the first time often produces fever and severe illness; re-exposure likely to produce a
mild illness, modified by the serum antibodies from the first infection
2.
Small passages - more easily obstructed by edema or secretions
3.
Young cells - rapid growth rates of cells make cells more susceptible to infection with most viral agents; decreased interferon
production
4.
Immature immunologic defenses - IgM are not usually synthesized by the fetus unless exposed to maternal infection; decreased
complement activity; decreased neutrophil chemotaxis; less effective cell-mediated immunity

Clinical approach to infectious diseases:

1.
Infectious diseases intertwined with microbiology
2.
All living cells appear to be parasitized by microbes throughout their lifetimes
3.
Pathogenesis both of virulence of infecting microbes and of defense of invaded host
4.
In-depth knowledge of the immune system indispensable in understanding the basic nature of management and prevention of
human diseases
5.
Microbial infection may be responsible for most human diseases

Two approaches to the study:

1.
Etiologic agent approach - identify the characteristics of the infecting organism and the disease it may cause
2.
Anatomic syndrome approach - can be defined in purely empirical and mutually exclusive terms and a patient's illness often can
be classified into a single anatomic syndrome

General approach to the child with possible infection:

1.
Detailed history and PE is essential
2.
Use of the laboratory - pathogens identified through direct methods, indirect methods and non-specific laboratory indications of
infection (elevation of acute-phase reactants: CRP and ESR)

II.

FEVER OF UNKNOWN ORIGIN (FUO)

Fever - symptom and not a disease; elevation of body temperature above normal (37.4C orally or 38C rectally)
Physiologic Fever States
Pathologic Causes

Digestion

Infection (most common)

Exercise

Inflammation

Ovulation

Neoplasms (hematologic or solid tumors)

Pregnancy

Vaccines

Warm environment

Dehydration

Emotion

Common causes of fever:

Major Illnesses
Minor Illnesses

Bacterial meningitis

URTI

UTI

Viral exanthema

Pneumonia

Gastroenteritis

Malaria

Mechanisms of a protective effect of fever:

o Enhanced neutrophil migration
o Increased production of antibacterial substances by neutrophils
o Increased production of interferon
o Increased anti-viral and anti-tumor activity of interferon
o Increased T-cell proliferation
o Decreased growth of microorganisms in iron-poor environment

Extreme hyperpyrexia (>41C) and hypothermia:

Extreme Pyrexia
Hypothermia

Central fevers

Elderly and the newborn

Drug fever

Cold exposure

Heat stroke

Hypothyroidism

HIV

Overwhelming infection

Malignant hyperthermia

Sepsis in CRF

Malignant neuroleptic syndrome

Overzealous treatment with antipyretics

Fever patterns:
o Intermittent pattern - exaggerated circadian rhythm thst includes a period of normal temperature on most days; high spikes
with return to normal
o Remitting pattern - persistent and varies by more than 0.5C for >24 hours; like intermittent but never returns to normal

o Sustained pattern - persistent and does not vary more than 0.5C for >24 hours; like remittent but with less marked swings of
temperature
o Relapsing pattern - febrile periods that are separated by intervals of normal temperature; seen in malaria
Tertian - 1st and 3rd days (P.vivax)
Quartan - 1st and 4th days (P.malariae)
o
*Biphasic pattern - seen in dengue
o
*Stepladder pattern - seen in typhoid/ enteric fever

Classic working definition (FUO):

o
Continuous fever of at least 3 weeks duration with daily temperature elevation above 38.3C and remaining undiagnosed
after 1 week of intensive study in the hospital

Working definition in pediatrics (FUO):

o
Fever >38C persisting as a predominant feature for more than 7-10 days in a child in whom a careful, thorough history and
physical examination and preliminary laboratory data fail to establish a diagnosis

Etiology:
1.
Infectious causes - most common
40-50%

Typhoid fever - stepladder pattern; fever will not be shorter than 5 days to a week; Faget sign
(pulse fever disproportion)

Differential diagnosis:
o Salmonella infection (Typhoid fever) - seriously and obviously ill with no apparent
cause; osteomyelitis/ arthritis (infant)
o Malaria - history of travel/ residence in endemic areas; enlarged spleen; jaundice
(hemolysis of RBCs)
o Miliary (Disseminated) TB - #1 in the Philippines; weight loss; anorexia; tuberculin test
(+) or (-); sterile pyuria; small discrete (hyperdense) shadows in both lung fields on CXR;
millet seeds
o Pyelonephritis - CVA/ suprapubic tenderness; crying on passing urine/ straining;
passing urine more frequently than usual; incontinence; WBC and/or bacteria in urine on
microscopy
o Abscess - fever with no obvious focus of infection; local tenderness (suppuration/
inflammation); fluctuant mass (subphrenic/ psoas)
o Septicemia - seriously and obviously ill, highly-febrile child with no focus of infection;
purpura, petechiae
2.
Collagen vascular
10-20%
10%

Juvenile rheumatoid arthritis - most common

3%

SLE
1%

HSP
1%

Vasculitis
3. Malignancies/ neoplasms
5-10%

Occult solid tumors (neuroblastoma - most common, Wilm's tumor, retinoblastoma)

Hematologic tumors (leukemia, lymphoma, Hodgkin's)

4. Miscellaneous
10-15%

Factitious fever

Metabolic

Genetic
5. Unknown/ undiagnosed
20-30%

Adage:
o
"FUO is more likely to be caused by an unusual presentation of a common disorder than a common manifestation of a rare
disorder"

Clues in the history that may localize the infection:

Sore throat
Streptococcus tonsillitis
Cough, rusty sputum
Lobar pneumonia
Severe joint pain/ swelling
Pyogenic arthritis
Severe pain in head and back of the neck with stiffness
Meningitis
Severe pain in a bone
Osteomyelitis
Tender liver
Amoebic liver abscess
Viral hepatitis
Ill-defined subcutaneous inflammation
Cellulitis
Pyomyositis
Bloody diarrhea
Shigella dysentery
Campylobacter
Chronic Diseases:
Congenital heart diseases
Cyanotic heart disease
Rheumatic fever
Shunted hydrocephalus

Complications:
Bacterial endocarditis
Cerebral abscess
Bacterial endocarditis
Recurrence of RF
Shunt infection
Ventriculitis

Septicemia
Chronic renal disease
UTI
Congenital or acquired immunodeficiency
Opportunistic organisms, fungi, parasites
Recent surgery
Concealed abscesses

History:
o Thorough history is essential

Physical examination:
o Complete, detailed, thorough and diligent
o Repeated frequently
o Look for focus of infection or findings

Laboratory:
o The most broadly-based, highest-yield, and most cost-effective testing is done first
o The lowest-yield, most esoteric testing is done last if no diagnosis is made despite persistence of symptoms
III.

GRAM (+) BACTERIAL INFECTIONS: STAPHYLOCOCCUS AND STREPTOCOCCUS

1.
Staphylococcus species
a)
S. aureus
b)
S. epidermidis
2.
Streptococcus species
a)
S. pneumoniae
b)
Group A Streptococcus

a)

b.)

Staphylococcus species
Either coagulase (+) S. aureus or coagulase (-) S.
epidermidis, S. saprophyticus, S. haemolyticus
Part of the normal flora of humans
Coagulase (+) - more virulent; coagulase may
cause clumping > bigger > lesser chance to be
phagocytosed
Disease may result from tissue invasion or injury
caused by various toxins and enzymes produced
by the organisms
S. aureus
Most common cause of pyogenic infections of the
skin and soft tissue
Clinical manifestations vary with the location of
infection, which is most commonly the skin but
may be any tissue
More prevalent among person living in low
socioeconomic circumstances and those in tropical
climates
Toxin-mediated diseases
o Food poisoning
o Staphylococcal scarlet fever
o Staphylococcal scalded skin syndrome
(SSSS)
Ritter disease
Prodrome of malaise, fever, and
irritability associated with exquisite
tenderness of the skin or the appearance of
generalized erythema
Flaccid blisters and erosions develop
Circumoral erythema or radial crusting
and fissuring around the eyes, mouth, and
nose (sunburst pattern)
Areas of epidermis may separate in
response to gentle shear force (Nikolsky
sign)
o Toxic shock syndrome (TSS)
S. epidermidis
Coagulase Negative Staphylococci/ CONS
o Phlebitis
o Cause infections in patients with indwelling
foreign devices
o Common cause of nosocomial neonatal
infection

Streptococcus species

a.)

b.)

S. pneumoniae (Pneumococcus)
Gram (+), lancet-shaped polysaccharide
encapsulated diplococcus
Encapsulated strains cause most serious disease
Capsular polysaccharides impede phagocytosis
Common clinical syndromes: otitis media,
sinusitis, pneumonia and sepsis (bacteremia)

Group A Streptococcus (Streptococcus pyogenes)

Gram (+) coccoid-shaped bacteria that tend to
grow in chains
Classified on the basis of the M protein antigen,
the virulent factor
Also cause distinct clinical entities (scarlet fever
and erysipelas, as well as toxic shock syndrome
and necrotizing fascitis
Pathogenesis: direct invasion and toxin mediation
Diagnosis: throat swab culture or rapid antigen

o
o
o
o
o

o
o

o
o
IV.

Impetigo bullosa
Exclusively staphylococcal in origin
The characteristic lesion are caused by
epidermolytic toxin
Staphylococcal pneumonia
Pneumatoceles (air-filled) vs. Streptococcus
pneumoniae (consolidation)
Rare

o
o
o
o

detection test
Non-specific pharyngotonsillitis - kissing tonsils
Highly suggestive of Group A B- Streptococcal
infection: palatal petechiae
Scarlet fever
Toxin-mediated disease
Skin has goose-pimple appearance and feels
rough
Face usually spared, although the cheeks may be
erythematous with circumoral pallor
After 3-4 days, the rash begins to fade and is
followed by desquamation (Measles and Scarlet
fever)
The tongue is usually coated and the papillae are
swollen (strawberry tongue)
Impetigo contagiosa
The classic lesion begins as erythematous papules
in traumatized areas
Vesiculopustular lesion
Quickly evolve into honey-colored crusted plaques
with surrounding erythema
GAS and S. aureus chief causative agents

DISEASES PRESENTING WITH A VESICULOPAPULAR RASH AND PETECHEAL/ PURPURIC ERUPTIONS

Vesicles: measure <5 mm in diameter & bullae >5 mm

Varicella (Chicken Pox )

o
In normal immunocompetent children, systemic symptoms usually mild, serious complications unusual
o
Mode of transmission: respiratory route or direct contact with skin lesions of varicella/ herpes zoster patients
o
characterized by short or absent prodromal period
o
Characterized as dewdrops on a rose petal
o
Most striking manifestation of lesion: rapidity of progression from macule to papule to vesicle with clouding and umbilication
within 24 to 48 hours and ends in crusting
o
Lesions appear in crops
o
Papulovesicular eruptions associated with fever and mild constitutional symptoms
o
Appearance of lesions simultaneously in one anatomic area
o
Predominant central distribution of lesions including the scalp
o
Eventual crusting of all skin lesions
o
Congenital (Fetal) Varicella Syndrome - cicatricial skin lesions; ocular abnormalities; hypoplastic limbs; cortical atrophy
o
Herpes zoster (Shingles) - varicella zoster virus (VZV) establishes latent infection in dorsal root ganglia and reactivate

Herpes simplex
o
Herpes simplex type 1 (HSV-1) - associated chiefly with infections of the mouth, lips, eyes and central nervous system
(above the waist)
o
Herpes simplex type 2 (HSV-2) - associated with genital and neonatal infections
o
HSV-1 - site of latency is the trigeminal ganglia
o
HSV-2 - site of latency is the sacral ganglia
o
Viral infection begins at a cutaneous portal of entry such as oral cavity, genital mucosa, conjunctiva or breaks in keratinized
epithelia
o
Acute Herpetic Gingivostomatitis
o
Herpetic Whitlow

Enteroviral exanthems
o
Hand-foot-and-mouth: Coxsackievirus A16, also A5, A7, A9, A10, B2, B5
o
Herpangina: Coxsackievirus A22, also A1-A10, A16
o
Insect bite-like: Coxsackievirus A4; crops, last 1-2 weeks
o
Non-pustule forming: Coxsackievirus A9; also Echovirus 11, 30

Erythema multiforme
o
Erythema multiforme major (Stevens-Johnson Syndrome)

Cutaneous and mucosal lesions have an abrupt onset following a prodromal respiratory illness

Bullae involved the lips, the mouth, and conjunctiva

Purulent conjunctivitis is usual as is uveitis

o
Erythema multiforme minor

Lesions begin as macules or wheals and evolve into papules or plaques

Center of the lesion may be vesicular, purpuric or necrotic

Iris or target lesions - pathognomonic sign

_________________________________________________________________________________________________________________

Dengue fever
o
Benign syndrome
o
Biphasic fever
o
Headache, myalgia, arthralgia, rash - morbilliform, maculopapular, leukopenia, lymphadenopathy, petecchiae

Phases of dengue:

Febrile phase - flu like symptoms and dehydration

Critical phase - cardiovascular compromise to shock from leaky capillaries, thrombocytopenia and
coagulopathy

Recovery phase - reabsorption of fluid, may develop hypervolemia in overly aggressive fluid management
o
Torniquet test:

Positive test: 20 or more petechiae per 1 inch2 (6.25 cm2)

o
Clinical case definition for Dengue Hemorrhagic Fever:

Fever, or recent history of acute fever

Hemorrhagic manifestations

Low platelet count (100,000/mm3 or less)

Objective evidence of leaky capillaries

o
Complications:

Carditis

Encephalitis

Severe pleural effusion

End organ damage in severe shock - CNS, renal

o
Dengue Shock Syndrome

Severe protein-losing shock syndrome

Rapid and weak pulse

Narrow pulse pressure (</= 20 mm Hg) OR hypotension for age

Cold, clammy skin and altered mental status

Frank shock is direct evidence of circulatory failure

Hermans rash
o
Management:

No hemorrhagic manifestations and patient is well-hydrated: home treatment

Hemorrhagic manifestations or hydration borderline: consider hospitalization

Warning signs (even without profound shock) or DSS: hospitalize

Menigococcal infections
o
Caused by Neisseria meningitidis
o
Cell wall has lipid A containing lipo-oligosaccharides, with endotoxin and covered by a polysaccharide capsule
o
Serogroup B - most common in infants
o
Occult meningococcal bacteremia - fever with or without symptoms
o
Meningitis develops in 58% of cases
o
Acute meningococcemia:

Maculopapupar rash

Limb pain and myalgia

Cold hands and feet - early signs

o
Meningococcemia

Earliest finding: grayish cyanosis that does not blanch on pressure (legs, lips, nose, earlobes, genitalia)

Evolve to become purpuric lesions (some may be stellate shaped) > large confluent ecchymoses that blister,
necrose and develop eschar

Purpura fulminans

Waterhouse-Friderichsen Syndrome

Adrenal hemorrhage
Congenital Rubella Syndrome
o
IUGR
o
Blueberry muffin spots
o
Thrombocytopenic purpura with petechiae
o

V.

COMMUNICABLE VACCINE-PREVENTABLE DISEASES

Vaccine Type:
Live attenuated

Live bacterial
Killed inactivated
Killed bacteria

Subunits

Examples:
MMR
OPV
Varicella
Yellow Fever
BCG
Oral typhoid
HepA
Polio
Rabies
Cholera
Pertussis
Plague
Typhoid
Acellular pertussis
HepaB
influenza
typhoid

Toxoid
Polysaccharide
(ineffective in children)
Conjugate polysaccharide

lyme
Tetanus
Diphtheria
Meningococcal disease
Pneumococcal disease
Hib
Pneumococcal disease

Vaccine:
(Birth to 2 years old)
BCG **
Hepa B
DTP
Hib
Polio
Pneumococcal conjugate
MMR
Measles
Varicella

Number of doses:

Vaccine:
(4 to 6 years old)
DTP
Polio
MMR
Hepa A

Number of doses:

1
3
4
4
4** (3 in USA)
4
1
1
1

1
1
1
2

DISEASE

TYPE OF
ORGANISM

Diphtheria

Corynebacterium
diptheria

Gram (+)

MODE OF
TRANSMISSION
Person to person,
direct contact

Haemophilus
influenza B

Gram (-)

Person to person,
direct contact or airborne
droplets

Hepatitis B

Virus

Exposure to infected blood

or body fluids
In children, primarily
prenatally spread

Mumps

Virus

Pertussis

Bordetella
pertussis
Gram (-)

Person to person contact

or droplet
Communicable the day
before to 9 days after
swelling
Most often occurs in
children
Person to person contact
Very contagious

SYMPTOMS

COMPLICATIONS

Bullneck appearance
(Tonsillar and Pharyngeal
Diptheria),
sorethroat,
fever,
yellow-white to grayish
membrane tonsils
(leathery)
Laryngeal diphtheria:
brassy or metallic cough
Otitis media,
sinusitis,
epiglotitis,
upper respiratory tract
infection
Generally flu-like
symptoms (may be
asymptomatic)
Liver may be enlarged,
dark urine, light stool,
jaundice
Symptoms last 4-6 weeks
Low-grade fever,
headache, carache
Pain and swelling of
parotid glands lasting
about a week

Respiratory distress,
myocarditis

Non-productive cough with

quick expiratory phase
followed by inspiratory
whoop (whooping cough)
Scleral or conjunctival
hemorrhage due to
coughing

Pneumonia, fever, ear

infections

ADDITIONAL
NOTES

Bacterial meningitis
Most invasive disease
occur in children 3 mos to
3 y/o
Chronic hepatitis,
cirrhosis,
liver cancer

Orchitis in males who

have reached puberty but
sterility is rare
Infrequent complications
are encephalitis and
meningitis
Leukocytosis/
leukemoid
reaction with
lymphocytosis

Pneumococcus

Gram (+)

Polio

Polio virus +
enterovirus with
serotypes 1,2,3
Virus

Tetanus

Clostridium
tetani
Neurotoxin
produced by
anaerobe Gram
(+)

Influenza

Virus
type A or B

Rotavirus

Virus

Fecal-oral route, usually

from contaminated water

Human

Virus

Sexually transmitted
especially among
promiscuous adolescents

Papillomavirus

Person to person contact

or droplets
Many people are colonized
in the upper respiratory
tract
Direct contact of virus with
mouth

Tetanospamin
Exposure of wound to the
bacterium
Affected muscles sustain
maximal contraction and
annot relax
Deep puncture wounds are
at greatest risk
Not transmissible from
person to person
Neonatal tetanus results
from contamination of the
umbilical cord stump
Person to person, contact
with airborne droplets
(most common mode of
transmission)

Otitis media,
sinusitis,
invasive bacterial
infections

Pneumonia,
meningitis

Low-grade fever and sore

throat (most cases are
asymptomatic)
Differential diagnosis:
Assymetric ascending
paralysis
Occur only in humans
Rope sign (Paralytic
bulbar polio)
Severe generalized muscle
spasms
Trismus (masseter muscle
spasm: lock jaw) most
common presenting
manifestation
Rissus sardonicus
(sardonic smile of tetanus)
- intractable spasms of
facial and buccal muscles
Opisthotonus rigidity of
abdominal or thoracic
muscles
Fever, myalgia, headache
(flu-like symptoms) are
prominent in older children
and adults
Younger children
asymptomatic
Epiglotittis
Watery diarrhea
Child <2 years old have
more episodes due to
absence of antibodies

Muscle weakness
progressing to paralysis
0.1 2% of the cases
May affect any muscle
group

VI.

The very young and old

are high risk of
pneumonia

Severe dehydration,
metabolic imbalance,
malnutrition,
intussusception
Cervical cancer, warts

Bacterial
superinfection
most common
complication
Myositis
Myocarditis

Vaccination powerful
dynamic tool

MUCOCUTANEOUS COMPLEX SYNDROMES: DISEASES PRESENTING WITH A MACULOPAPULAR RASH

Definition:
o
A febrile illness in children associated with skin manifestation (exanthem) and mucous membrane involvement (conjunctiva,
throat, respiratory or gastrointestinal tract)

Classification:
o
Maculopapular eruption
o
Vesiculobullous or vesiculopustular
o
Petechial or purpuric eruption

Descriptive dermatologic terms:

Lesions:
Description:
Examples:
Discrete
Lesions remain separate
Childhood exanthems
(Rubelliform)
Confluent
Lesions run together
Childhood exanthems
(Morbilliform)
Reticulated
Lace-like network
Erythema infectiosum
Multiform
(Polymorphous)
Iris
Grouped
Generalized
Zosteriform

More than one type of shape or

lesion
Circle within a circle;
A bulls eye lesion
Lesions clustered
together
Widespread
Linear arrangement along a
nerve distribution

Erythema multiforme
Erythema multiforme
Herpes simplex

Hesrpes zoster


o
o
o
o
o
o
o

1.
2.
3.
DISEASE OR
SYNDROME

RUBEOLA
(Measles)

RUBELLA
(German
Measles)

General statements:
Maculopapular rashes are non-specific
These are the most common primary lesions seen during acute febrile illness in children
Associated with mild, febrile, upper respiratory or GIT illnesses
Most exanthematous illnesses in children are benign
Enteroviruses are the leading cause of infection-related exanthematous diseases
Their differential diagnoses is critical because the early cutaneous manifestations of potentially fatal bacterial and rickettsial
diseases frequently are similar
Many conditions that will ultimately manifest purpuric, vesicular, urticarial or ulcerative cutaneous lesions may first appear as
erythematous macules or papules
Pathogenesis:
Dissemination of infectious agents by blood (viremia, bactermia) which results in secondary infection at the cutaneous site
a.
Direct result of infectious agents in the epidermis, dermis or dermal capillary endothelium
b.
An immune response between the organism and antibody or cellular factors in the cutaneous location.
Dissemination of known specific toxins of infectious agents
A combination of these mechanisms
INFECTIOUS
AGENT

AGE

Infants,
Adolescents

Infants,
young adults

INCUBATION
PERIOD
(DAYS)

10 12

14 21

CLINICAL
CHARACTERISTICS

LESIONS

DISTRIBUTION

Onset with fever,

cough, coryza, and
conjunctivitis, nasal
catarrh
About 2 days after
onset, appearance of
enanthem (Koplik
spots), and 2 days
later, onset of
exanthem
With severe prodromal
period
Modified measles:
Attenuated form of
infection that may
occur in individuals
who have received Ig
after exposure to
measles
Clinical manifestations
are milder than those
of typical infection and
incubation period is
prolonged from 14 to
20 days
Atypical measles:
Individuals infected
with natural virus and
who previously
received killed
measles vaccines
Sudden onset of high
fever accompanied by
abdominal pain,
cough, vomitting, and
pleuritic chest pain
Koplik spots are rarely
present and rash
begins distally and
progresses in a
cephalad direction

Erythematous,
maculopapular,
and confluent
Develop a
brownish
appearance,
and fine branny
desquamation
occurs

Starts behind ears

and on forehead
Spreads downward
over body
Confluence most
prominent on face,
trunk, and proximal
end of extremities

Mild symptoms with

onset 1-5 days before
rash
Fever usually <38.50C
Headache, malaise,
and suboccipital and
postauricular
lymphadenopathy

Erythematous,
maculopapular,
discrete

Starts on face and

spreads downward
to trunk and
extremities

ROSEOLA
INFANTUM
(Exanthem
Subitum)

ENTERO
VIRAL
INFECTION

ERYTHEMA
INFECTIOSUM

INFECTIOUS

MONONUCLEOSIS

HHV-6 & 7

Coxsackievirus
es A2, A4, A5,
A7, A9, A10,
A16, B1-B5;
Echoviruses 17, 11-14, 1619, 22, 24, 25,
30, 38;
Enterovirus 71

Parvovirus B19

Epstein-Barr
Virus

6 mos 2 yrs

Infants &
young
children

Prepubertal
children &
Adults

Children &
Adolescents

47

4-28

30-50

Low grade fever then

rash
Forchheimer spots
Most important
consequence in
pregnant women is
Congenital Rubella
Syndrome (salt and
pepper retinopathy
and blueberry muffin
spots)
Fever 3-5 days in
duration, rapid
defervescence, and
then the appearance
of rash
No severe prodromal
period
Nagayama spots
Most prominent clinical
manifestation:
irritability
High-grade fever then
rash
Bulging fontanels
Seizures
Fever and mild to
moderate pharyngitis
Occasionally,
herpangina,
meningitis, and other
manifestations of
systemic viral infection
Exanthem occurs in 550% of infections,
depending on virus
type
Rash may occur
during fever or after
defervescence
Hand, foot, and mouth
syndrome
Biphasic illness with
mild prodromal period
with headache and
malise for 2-3 days,
then 7-day symptomfree period, followed
by typical exanthem
Affects the bone
marrow

Fever, pharyngitis,
lymphadenopathy and
severe sore throat
Generalized
lymphadenopathy
Anterior, posterior
cervical and
submandibular
lymphadenopathy
(most common)
Hepatitis involvement

Erythematous,
macular or
maculopapular

Most prominent of
neck and trunk
Face and
extremities may be
affected

Most
commonly
erythematous,
maculopapular,
and discrete.
May have
macular,
petechial,
vesicular, and
urticarial
components.
Rarely
erythema
multiforme

Usually starts on
face and spreads
downward to trunk
and extremitities
May have
peripheral
distribution (hand,
foot, and mouth
syndrome)

Three stage
exanthem:
Initially, rash on
cheeks
(slapped-cheek
appearance)
and then
erythematous
maculopapular
rash on trunk
and limbs
Finally rash
develops a
reticular pattern
of lesions
Lace-like
eruption
Most
commonly
erythematous,
macular,
maculopapular,
and discrete
(rubelliform)
In association
with ampicillin
administration,
the rash may

Starts on face
More prominent on
extensor surfaces
of extremities

Mainly on trunk and

proximal end of
extremities

SCARLET
FEVER

Streptococcus
pyogenes

School age

3-4

7-14

TYPHOID
FEVER

Salmonella
typhi

MENINGOCOCCEMIA

N. Meningitidis

LEPTOSPIROSIS

Leptospira spp.

KAWASAKI
DISEASE

Unknown

Older
children &
adults

Any (<5 yr)

is common
Exanthem occurs in 313% of cases
If ampicillin
administered, then
exanthem in 50% of
cases (ampicillin
rash)
30-50% of primary
infections in
adolescence or early
adulthood are
symptomatic
Clinical triad of
exudative pharyngitis,
cervical
lymphadenopathy and
splenomegaly
Hoaglands sign: lid
edema
Atypical lymphocytes
(Downey cell)
Glandular fever
Fever, pharyngitis, and
cervical lymphadenitis
Rash onset within 2
days of first symptoms
Goose-pimple
appearance

be more vivid
Erythema
multiforme and
urticaria may
occur

Diffuse
erythematous
and fine
maculopapular
(looks and
feels like red
sandpaper).
Rash darker in
skin folds
(Pastia lines)
Desquamation
occurs
Rose spots (24 mm macular
lesions)

Circumoral pallor
Generalized rash,
with trunk and
proximal end
ofextremities being
most involved

Fever and pharyngitis,

Sudden onset of rash
PenG - treatment
Rifampicin postexposure prophylaxis
for acute
menigococcemia

Characteristic
rash is
petechial or
purpuric.
Early lesions
may be
erythematous,
maculopapular,
or urticarial

Generalized

Fever, conjunctivitis,
and anorexia. Rash
rarely noted
5 days or more of high
fever, sore throat,
cervical
lymphadenopathy,
mucosal erythema,
conjunctivitis without
exudates, skin rash,
and periungal
desquamation of the

Erythematous
maculopapular
rash
Maculopapular
rash

Mainly on trunk

Malaise, headache,
and marked fever
Rash onset 10 days
after onset of fever
Stepladder fever
Relative bradycardia
Intestinal symptoms
Chloramphenicol
gold standard of
treatment
Ceftriaxone short
course therapy

Discrete lesion on
abdomen

Generalized

skin on the fingers and

toes
Thrombocytosis
Dryness with fissuring
and erythema of the
lips, red strawberry
tongue and infected
pharynx
Complication:
Aneurysm
VII.

CONGENITAL DEFECTS AND THE EMBRYONIC STAGES

Neural Tube Defects:

Spina bifida occulta

o
Midline defect of the vertebral bodies without protrusion of the spinal cord or meninges
o
Usually asymptomatic; at the level of L5 to S1
o
A dermal sinus tract or opening may be a cause of recurrent meningitis

Meningocele
o
Meninges herniate through a defect in the posterior vertebral arches

Myelomeningocele
o
Most severe form of dysraphism
o
Spinal cord and meninges protrude into the defect
o
Etiology: unknown
o
Clinical Manifestations:

Dysfunction of many organs as skin, gut, skeleton, peripheral nervous system

o
May be associated with hydrocephalus (Chiari type 2)
o
Management: multi-disciplinary

Encephalitis
o
Affects the skull through a midline bony defect

Anencephaly
o
Primitive brain with connective tissue, hypoplastic pituitary, absent cerebrum and cerebellum
o
Death in several days (not compatible with life)
o
Prevention: maternal intake of 0.4 mg folic acid
Disorders of Neural Migration:

Lissencephaly
o
Absence of cerebral convolutions and poorly formed Sylvian fissure with appearance of a 3-4 month fetal brain
o
With 4-layered cortex instead of 6
o
Clinical Manifestations:

Failure to thrive, MR, seizure disorder, chromosomal features (Miler-Dieker Syndrome)

o
CT and MRI: smooth brain

Schizencephaly
o
Unilateral or bilateral clefts with the cerebral hemispheres due to an abnormality in morphogenesis
o
Clinical Manifestations:

MR, seizures, microcephaly, spastic paresis

Porencephaly
o
Presence of cyst or cavities within the brain

Holoprosencephaly
o
Results from defective cleavage
o
Polymicrogyria opposite of Lissencephaly

Microcephaly
o
Head size > 3SD below the mean for age and sex
o
Primary: genetic; Secondary: non-genetic

Hydrocephalus
o
Results from conditions with impaired circulation and absorption of the CSF or an increased production by a choroid plexus
papilloma
o
Types:

Obstructive or non-communicating within the ventricular system

Non-obstructive or communicating obliteration of cisterns/ malfunction of arachnoid villi aqueduct

o
Clinical Manifestations:

Enlarged head with big fontanel, wide sutures, prominent scalp veins and setting sun sign

Long tract sign

Irritability, lethargy, vomiting

o
Diagnosis:

Complete Hx and PE

Ultrasound large amount of fluid

MR/CT scan enlarged ventricles

Craniosynostosis

Premature closure of cranial sutures

Types:

Primary: due to abnormalities in the skull development

Secondary: failure of brain growth and expansion

Pseudotumor Cerebri
o
Mimics brain tumors, increased ICP
o
Caused by metabolic disorders

Drugs (Vitamin A, steroids)

Hydrancephaly Ex Vacuo

CHILD WITH INCREASED INTRACRANIAL PRESSURE

Amount of CSF depends on age of patient
Normal intracranial pressure in the resting state = 10mmHg (136mmH2O)
Intracranial Hypertension+ >200 mmH2O
ICP surrounding pressure exerted by the brain, blood and CSF

o
o

VIII.

In Infants
Bulging fontanel
Failure to thrive
Impaired upward gaze
Large head
Shrill cry

In Children
Diplopia
Headache
Papilledema
Mental changes
Nausea and vomiting

Cerebral Edema

Increase in brain volume caused by an increase in water and Na content

3 categories:
Vasogenic
Cytotoxic
Increased capillary permeability; fluid in white Swelling of neurons, glia, endothelial cells,
matter
constricts the extracelluar space

Interstitial
Transependymal movement of fluid from
ventricles to brain, a block on CSF
absorption and ventricles enlarge

Ex. Brain tumors, trauma, hemorrhage

Ex. Hypoxia, ischemia, CNS infection

Ex. Hydrovcephalus

Tx: Dexamethasone

Tx. Mannitol

Tx: acetazolamide, furosemide

(Steroids and mannitol ineffectice)

Brain Tumors in Children:

Clinical Presentation:
o
Increased ICP
o
Deficits according to location of tumor

Supratentorial personality, speech, seizure

Infratentorial cerebellar signs, cranial nerve deficits

Intracerebral Hemorrhage

May occur in the subarachnoid space or n the parenchyma of the brain

Severe headache, nuchal rigidity, loss of consciousness

Intracerebral hematoma increased ICP, focal deficits, seizures

Common causes:
o
AVM, blood dyscrasia, dengue hemorrhagic fever and septicemia, perinatal asphyxia, APCD

Diagnosis: contrast CT scan, cranial MRI, angiogram, MRA, blood profile, septic work-up

Management: Treat ICP elevation, give specific management

Brain Abscess:

Common 4-8 years old

Causes:
o
Congenital Heart Diseases
o
Meningitis
o
Otitis Media
o
Mastoiditis
o
Contiguous Infection
o
Head injuries
o
Immunodeficiency states

Manifestations:
o
Early cerebritis, non-specific
o
Later signs of increased ICP, focal neurologic deficits
Management:
o
Antibiotics if abscess is < 2cm, illness of short duration, no ICP signs and normal neurologic exam

Use 3rd generation cephalosporins + metronidazole or ampicillin sulbactam for 4-6 weeks
o
Surgery

CNS Infection:

Acute Bacterial Meningitis

o
Produces increased ICP because of cerebral edema, obstruction to flow and absorption of CSF and impairing venous flow
o
Etiologic agents:
Newborn to 3 months
3 months to 6 years
> 6 years
GBS
H. influenza, Type B
N. meningitides
E.coli
S. pneumonia
S. pneumonia
L. monocytogenes
N. meningitides
Enterococci
S. pneumonia
Complications:

Seizures, subdural effusions, SIADH, prolonged fever, DIC

o
Sequelae:

Cerebral atrophy

Microcephaly
o
Treatment:

Uncomplicated penicillin sensitive s. pneumonia mengitis: 10 -14 days

Resistant strep: vancomycin

Uncomplicated N. meningitides: IV penicillin 5-7 days

Uncomplicated h. influenza type B: 7-10 days

Gram (-) meningitis: 3rd generation cephalosporin

Viral Meningitis
o
Aseptic meningitis
o
Usually accompanying the viral infection as diarrhea, respiratory, mumps, measles, varicella
o
Treatment is supportive as the course is self-limiting
TB meningitis
o
3 stages:

Stage 1 prodromal (fever, headache, irritability)

Stage 2 neurologic signs (meningeal signs, decreased level of sensorium)

Stage 3 coma, irregular respiration, rigidity, opisthotonus

o
Diagnosis:

Clinical: subacute course, (+) exposure to TB, (+) tuberculin test

CSF: groundglass appearance with tendency to coagulate, increased WBC with lymphocytes, extremely high
protein, low sugar

(+) Acid fast staining, PCR, ELISA, or latex agglutination antigen

o

Normal

Treatment:

Quadruple anti-TB regimen:

2 HRZES/ 10 HR
WBC
0-5 lymphocyte

15-45

Protein

Sugar
50-75
>50% of blood sugar

Clear

Others

ABM

High neutrophils
(After 5 days lymphocyte)

High

Low

Turbid

Viral

Normal

N or slight increase

Normal

Clear

High lymphocyte

High

Low <40

xanthochromic

TB

IX.

Encephalitis
o
Usually viral

Herpes Simplex

Dengue encephalopathy

Subacute sclerosing paraencephalitis (SSPE)

Related to measles

Subacute and degenerative disease of the entire brain

Symptoms: behavioral and personality change

Diagnosis: (+) measles antibody in the CSF, characteristic EEG findings

Treatment: no treatment
ABNORMALITIES OF TONE AND MOVEMENT IN CHILDREN
UMN

Bulk
Tone

Minimal atrophy
Increased, spastic

DTRa
Fasciculations
Babinski
Sensory Deficit

Hyperreflexia

LMN
Profound atrophy
Decreased, spastic
Hyporeflexia

+
May be present

+/May be present

Cerebral Palsy:

Refers to a group of disorders characterized by motor abnormalities (tone, posture or movement) which are neither progressive nor
episodic

Most common and costly form of chronic motor disability with prevalence of 2/1000

Clinical Manifestations:
o
Delay in development

Poor head control, delays in gross motor or fine motor development

o
Motor deficit

Depending on the area of the brain involved and usually the risk factors present
o
Associated developmental disabilities

Mental retardation, epilepsy, visual, hearing, speech and behavioral abnormalities

Physiologic
Spastic
Athetoid
Rigid
Ataxic
Tremor
Atonic
Mixed
Unclassified

Topographic
Monoplegia
Paraplegia
Hemiplegia
Triplegia
Quadriplegia
Diplegia
Double hemiplegia

Etiologic
Prenatal infection, metabolic, anoxia,
toxic, genetic, infarction
Perinatal anoxia
Postnatal toxins, trauma, infection

Functional
Class I no limitations of activity
Class II slight to moderate
Class III moderate to great
Class IV no useful physical activity

Spastic Quadriplegic Cerebral Palsy

o
Most severe form because of involvement of all 4 limbs and the high association of mental retardation and seizures
o
Feeding problems are common due to supranuclear bulbar palsies
o
Stiffness with the knees bent or with leg separated, occurs more commonly in the child with spasticity and athetotis
combined
o
Body stiffens like a board
o
Arm may stiffen straight out while the other stiffens bent, head twists to one side, legs stiffen together and knees press
together
o
Shoulders and head press back, fist grips thumb, legs turn in

Athetoid Cerebral Palsy

o
Less common that the spastic type
o
Most likely associated with birth asphyxia
o
Hypotonic infants who do not have UMN signs but later develop increased variable tone (rigidity) and dystonia and other
dyskinesias
o
Intellect is preserved in many cases
Autism Spectrum Disorder:

Behavioral Phenotype:
o
Qualitative impairment in language/ communication
o
Impaired social interactions and reciprocity
o
Lack of imaginative play

Signs and Symptoms:

o
No pathognomonic symptom or behavior
o
Most will present with:

Impairment in joint attention

Use of eye contact and pointing to share experiences with others normally develops at 18 months

Lack of protoimperative pointing (to get an object of desire)

Lack of imaginative play

Attention Deficit Hyperactivity Disorders:

Most common neurobehavioral disorder in childhood

Characterized by (DSM IV):

o
Inattention
o
Poor impulse control
o
Motor overactivity/ restlessness

**Symptoms should be present for > 6 months, in at least 2 settings and significantly affects social, academic or occupational functioning

X.

SEIZURES:

Simple Partial Seizures/ Focal Seizures/ Jacksonian Seizures:

o
Consciousness is preserved
o
May have motor, sensory, somatosensory or special sensory, autonomic or psychic symptomatology.
o
No post-ictal confusion
Absence
o
Brief (less than 30 seconds)
o
With impairment of consciousness
o
No aura
o
No post-ictal symptoms
o
Typical EEG: generalized 3-4 Hz slow spike and wave discharges
o
Fundamentally and pharmacologically unique from other seizures
Myoclonic
o
Brief jerks of whole body or individual muscle groups, usually without impairment of consciousness
o
Shock-like contractions are commonly precipitated by the patients falling asleep or awakening.

o
May evolve into a tonic-clonic seizure
Atonic Seizures:
o
Sudden loss or diminution of muscle tone without apparent preceding myoclonic or tonic events, lasting for 1-2 seconds
involving the head, trunk, jaw or limb musculature

Diagnosis:

No routine laboratory requests

EEG
Neuroimaging: MRI>CT
Others for specific indications:
o
CSF, head-up tilt table test and/or other cardiologic work-up
o
Endocrinological, metabolic, genetic work-up, neurppsychological investigations
Use of EEG:

Abnormal epileptiform activities:

o
Absent 10-40% of epileptic patients
o
Present in 1-5% on non-epileptic people
Indications for Neuroimaging:

Partial onset seizures

Findings suggest of progressive neurologic disease

Intractable seizures

Seizures increasing in frequency and intensity despite adequate treatment.

MRI- has higher sensitivity than CT Scan for structural epileptogenic foci.
Infantile Spasm or West Syndrome:

Triad: Infantile spasm, mental retardation and hypsarrythmia on EEG

Lennox Gastaut Syndrome:

Triad of seizure disorder, mental retardation and atypical petit mal on EEG

Mixed seizure type

Benign Rolandic Epilepsy:

Male preponderance

EEG: characteristic centro-temporal spikes

Childhood Absence:

3 times more common in girls

Triggered by hyperventilation for no less than 3 minutes

Treatment: anti-epileptic drug

Petit mal seizure

Febrile Seizure:

A seizure in association with a febrile illness with a temperature greater than 38.4 C (although the fever may not be evident until after the
seizure).

Without evidence of any causative disease such as CNS infection, metabolic abnormality, intoxication, etc., in children older than 1
month of age without prior afebrile seizures.
Classification:

Simple:
o
Short
o
Generalized
o
Occurs only once in a febrile illness.

Complex:
o
Prolonged (>15mins)
o
Focal
o
Recurrent within a 24-hour period
Lumbar Puncture:
American Academy of Pediatrics recommendations for Lumbar Puncture:

A must for children under 12 months.

Strongly recommended for children between 12-18 months.

Not necessary in a child > 18 months.

First complex febrile seizures and or persistent lethargy.

Strongly recommended for children who have received prior antibiotic therapy.
XI.

PEDIATRIC RHEUMATIC DISEASES:

5 presentations of pediatric rheumatic diseases:

Chronic Arthritis7

Systemic Connective Tissue Disease

Systemic Vasculitis

Periodic Fevers
Non-articular musculoskeletal pain

Chronic Arthritis (Rheumatic Diseases Presenting as Chronic Arthritis > 6 weeks):

Juvenile Idiopathic Arthritis

o
Erosive type of arthritis
o
RF is important for prognosis if JRA will progress to adult RA
o
Diagnose 6 weeks or more; classify 6 months

Diagnosed as JRA if it lasts > 6 weeks

Onset type defined by type of disease in the first 6 months

o
ACR criteria for JRA:

Onset: <16 years of age

Persistent arthritis in > 1 joints

Duration: >6 weeks

Exclusion of other forms of juvenile arthritis

o
ACR criteria for Classification of JRA:

Polyarthritis - >5 inflamed joints

Oligoarthritis -<4 inflamed joints

Systemic arthritis with rash and characteristic quotidian fever

o
Types of JRA:
Systemic
Pauciarticular (Oligoarthritis)
Polyarthritis
F:M 1:1
F:M 7:1
F:M 8:1
Least common and most difficult to treat
Most common and with good prognosis
Arthritis usually abates
(-) ANA, (-) RF
50% (+) ANA chroic uveitis risk
(-) RF: 10% disabling disease
RF rarely (+)
(+) RF: >50% disabling disease (poor
prognosis)
Arthritis in 1 or more joints plus:
Affects 4 or less joints
Affects 5 or more joints
Often asymmetric
Often symmetric

Fever (quotidian pattern)

Affects large and small joints

Evanescent rash (rheumatoid Affects large joints (knees/ ankles)

rash)

Serositis

Organomegaly

High WBC/ platelets

Elevated acute phase reactants

Most difficult to treat
Majority achieving remission
More prolonged course
Poor prognostic factors:
Poor prognostic factors for uveitis:
Poor prognostic factors:

Polyarticular

(+) ANA

Young age at onset

Fever >3 months

Onset < 6 years

(+) RF/ rheumatoid nodules

Increased inflammatory markers

(+) Anti-CCP
>6 months

Large number of affected joints

(hips/hands/wrist)
**Quotidian fever -1-2 spikes of fever per day with sudden decline to normal or subnormal temperature
**Evanescent rash (rheumatoid rash) salmon pink macular rash, which appears only during fever
**Chronic uveitis can lead to blindness; children usually do not complain of eye pain, photophobia, etc; treat with steroids (MTX) even arthritis
stops; risk factors: oligoarticular, female, young age, (+) ANA

Juvenile Spondyloarthropathy
o
Inflammation of the joints of the axial skeleton, large joints of the lower extremities and of entheses (attachment of tendons
into the bones)
o
RF always absent
o
Types:

Juvenile ankylosing spondylitis

Psoriatic arthirits

Reactive arthritis (Reiters syndrome urethritis, conjunctivitis, arthirits) Arthropathies associated with IBD

Undifferentiated Spondyloarthropathy
o
High frequency of HLA-B27
o
Presence of extra-articular features (eyes, kin, GIT, GUT)
o
Treatment

Medications

NSAIDS (aspirin, indomethacin, ibuprofen, diclofenac, naproxen)

DMARDS (Sulfasalazine, Methotrexate)

Glucocorticoids

TNF-blockers
o
Physical and Occupational Therapy
o
Nutrition

Differentiating Points
JRA
Younger children
F>M
Large and small joints
Symmetric

JSpA
Adolescents
M>F
Axial and large joint involvement
Asymmetric
Extra-articular features
Associated with HLA-B27

Systemic Connective Tissue Diseases:

Systemic Lupus Erythematosus

o
Presence of autoantibodies (hallmark of SLE)
o
Treatment:

Glucocorticoids, Hydroxychloroquine, Immunosuppressive drugs, IV gammaglobulin, biologics

1997 ACR Criteria for SLE
(Any 4 of the 11 criteria should be present, serially or simultaneously, during any interval of observation
1. Malar Rash
Crosses the nasal bridges
Not pathognomonic for SLE
2. Discoid Rash
Coin-shaped; affects face and scalp, scars with hyperpigmentation when
inflammation resolves
3. Photosensitivity
Rashes after exposure
4. Oral or nasopharyngeal ulcers
Usually painless; found in palate and nasal mucosa
5. Serositis
Pleural and pericarditis effusion
6. Arthritis
Non-erosive; intermittent usually in MCPs and PIPs; symmetrical
7. Neurologic diseases
Psychosis and/or seizures; (+) anti-ribosomal P and neuronal antibodies
8. Hematologic diseases
Hemolytic anemia (Coombs test)
Leukopenia (<4000 x 2 occasions)
Lymphopenia (<1500 x 2 occasions)
Thrombocytopenia (<100,000)
9. Renal Disease
Proteinuria (4+/ >500 mgday)
Cellular cast
10. Positive immunoserologies
(+) anti-dsDNA
(+) anti-Smith
(+) antiphospholipid antibodies (Thrombosis in MI)
11. (+) ANA

Neonatal Lupus
o
Syndrome of passively acquired autoimmune injury

Cutaneous lupus

Congenital heart block

Systemic manifestations (hematologic, hepatic, GI, neurologic, pulmonary, thrombotic)

o
Pathogenesis

Anti-Ro/SSA and Anti-La/SSB were found to be causative for neonatal lupus

Ro and La antigens were found in fetal heart and neonatal skin

Anti-Ro and Anti-La were present in the fetal circulation

Anti-Ro and Anti-La were shown to interfere with cardiac function

o
Treatment of Congenital Heart Block

In utero

Fluorinated steroid treatment for incomplete block or hydropic changes

Direct intraumbilical fetal therapy with digoxin and diuretics

Plasmapharesis

In utero pacing
o
Prognosis

Skin, liver and hematologic complications generally resolve with minimal sequelae

All children with CAVB require pacemaker insertion by the age of 15

Only few reported cases of children developing systemic rheumatic disease during adolescence or adulthood

Juvenile Dermatomyositis
o
Most common inflammatory myositis in children
o
Features:

Symmetric proximal muscle weakness

Cutaneous abnormalities (Heliotrope rash, Gottrons rash)

Helitrope rash violaceous eruptions on the upper eyelids and in rare cases on the lower eyelids

Gottrons rash discrete erythematous eruptions or red patches overlying bony areas (knuckles,
elbows, knees)

Elevated muscle enzymes (CK, aldolase, LDH, AST, ALT)

EMG findings

Muscle Biopsy Findings

Bohan and Peter Criteria for Juvenile Dermatomyositis
Classic rash (Helitrope rash, Gottrons rash)

Plus 3 of the following:

Symmetrical proximal muscle weakness
Elevated muscle enzymes (CK, aldolase, LDH, AST, ALT)
EMG findings (myopathy, denervation)
Muscle biopsy findings (necrosis, inflammation)
Juvenile Scleroderma
o
Chronic disease of unknown cause
o
Salt and pepper skin
o
Types:

Localized/Linear Scleroderma

Systemic Sclerosis

Diffuse Cutaneous Systemic Scleroderma

Limited Cutaneous Systemic Scleroderma

o
Manifestations of Limited Systemic Scleroderma (CREST):

Calcinosis

Raynauds phenomenon

Esophageal dysmotility

Sclerodactyly

Telangiectasia

Systemic Vasculitis

Inflammation and necrosis of blood vessels caused by an aberrant immune response

Classification of Primary Vasculitis

Size of vessel
Small

Medium

Large

Henoch-Schonlein Purpura
o
IgA-mediated vasculitis of the small vessels
o
Most common cause of non-thrombocytopenic purpura in children
o
Also known as anaphylactoid purpura
o
Features:

Palpable purpuras

Abdominal symptoms

Arthralgias/arthritis

Renal manifestations
o
Occurs in all age groups and all races
o
Most frequent in children (50%) <5 y/o
o
Male preponderance
o
Duration of illness averages 4 weeks
o
Recurrences in 16-40%, most within 6 weeks
o
80-90% occur following an upper respiratory infection
o
Treatment

Steroids only if with GI and renal complications

o
Major complications of HSP

Nephrotic syndrome

Bowel peforation

Kawasaki Disease
o
Acute febrile vasculitis of infancy and early childhood
o
Also known as mucocutaneous lymph node syndrome

Diseases
Hypersensitivity angitis,
Serum sickness,
Henoch-Schonlein Purpura
Kawasakis disease,
Polyarteritis nodosa,
Wegeners Granulomatosis,
lymphomatoid granulomatosis,
Churg-Strauss disease
Takayasus arteritis,
Temporal arteritits,
Behcets syndrome

Diagnostic Criteria of Kawasaki Disease

Fever of at least 5 days duration

Plus at least 4 of the following principle features:

Polymorphous exanthema (any lesion except bullous or vesicular type; seldom pruritic; spread all over; disappear without
desquamation)
Cervical lymphadenopathy (>1.5 cm; unilateral)
Bilateral conjunctival infection (non-purulent; non-exudative)
Changes in lips and oral cavity (dry, red, cracked lips strawberry tongue also seen in strep infection)
Changes in extremities (swelling, palmar erythema, periungal desquamation) -2nd or 3rd week

Phases of Kawasaki Disease:

Acute
Subacute
1-2 weeks of illness
End of fever to day 25 (desquamation, arthritis,
thrombocytosis)
o

Convalescent
Usually 6-8 weeks after onset of illness
(disappearance of clinical signs and
normalization of acute phase reactants)

Cardiac features of KD:

Acute myocarditis (tachycardia, gallop, arrhythmias, muffled heart sounds)

Pericardial effusion (30%)

Mitral insufficiency (uncommon) Aortic insufficiency (chronic phase)

Coronary artery abnormalities (20-25%) aneurysm

Coronary artery occlusion

Acute myocardial infarction

Treatment during Acute Phase

IVIg : 2g/kg, single infusion over 10-12 hrs

Aspirin: 80-100 mg/kg/day in 4 divided doses until patient is afebrile for 3-4 days

High dose anti-inflammatory

Persistent/Recrudescent fever

Repeat IVIg

IV methylprednisolone (30mg/kg); total of 3 daily doses as necessary

Treatment during Subacute Phase

Subsequent antiplatelet Rx

Aspirin: 3-5 mg/kg/day OD

Low dose anti-platelet

Duration: 6-8 weeks from onset & with normal echo

If with 2D echo abnormality: indefinitely

With large aneurysms with or without thrombus

Warfarin INR 2-2.5

Low dose aspirin

Polyarteritis Nodosa
o
Chnonic, relapsing, febrile disease with protean clinical manifestations
o
Necrotizing vasculitis affecting the small and medium-sized vessels
o
Aneurysm formation
o
Etiology:

Viruses

Hepatitis B (most common in adults)

Bacteria

Group A Streptococcus (most common in children)

o
Clinical Features of PAN

Fever, anorexia, weight loss, myalgias, arthralgias/arthritis

Cutaneous lesions

Vasculitic rash

Abdominal pain

Hypertension

Hematuria

Proteinuria

Wegeners Granulomatosis
o
Necrotizing granulomatous vasculitis of the medium-sized vessels
o
Diagnosis of Wegener Granulomatosis

Clinical features + sinus/lung/kidney biopsy (necrotizing granulomatous angitis)

Confirmed by the presence of c-ANCA (anti-PR3)

Takayasu Arteritis
o
Chronic vasculitis of the large vessels (aorta and its main branches)
o
Also known as pulseless disease
o
Age of onset: 10-40 years old

Clinical Features:

Pre-pulseless phase

Fever, weight loss, anorexia, night sweats, fatigue, arthritis

Hypertension

Limb claudication

Pulseless phase

Bruit (over the carotid, subclavian and abdominal arteries)

Behcet Disease
o
Chronic relapsing inflammatory disease
o
Strongly associated with HLA-B5 and HLA-B51
o

o
o
o
o

Criteria of the International Study Group for the Diagnosis of Behcets Disease
Recurrent oral ulceration
Plus 2 of:
Recurrent genital ulcers (>3 times per year)
Eye lesions
Skin lesions
Pathergy (popular/ pustular response to local skin injury)

Genital ulcers

Ocular lesions

Usually occur after oral ulcers

Scar with healing
Most common in the scrotum (males) and vulva (females)

Most dominant feature is bilateral acute uveitis

Hypopyon (severe anterior uveitis with purulent material in the anterior chamber)
Retinal vasculitis, optic neuritis, vascular occlusion

Hereditary Periodic Fever Syndromes

Familial Mediterranean Fever (FMF)

Hyper-IgD Syndrome (HIDS)

TNF-Receptor-Associated Periodic Syndrome (TRAPS)

Familial Cold Urticaria

Muckle-Wells Syndrome

Chronic Infantile Neurological Cutaneous and Articular Disease (CINCA) / (NOMID)

Non-articular Musculoskeletal Pain
Growing Pains
Reflex Sympathetic Dystrophy
Erythromelalgia
Review:
JIA or JSpA or Both:
Adolescent males
JSpA
Axial involvement
JSpA
RF positivity
JIA
HLA-B27 positivity
JSpA
Ocular involvement
BOTH
Treatment with methotrexate
BOTH
SLE-NLE-JDMS-Scleroderma
Heart block
Neonatal Lupus
Heliotrope rash
JDMS
Anti-dsDNA
SLE
Anti-centromere
Scleroderma
Painless oral ulcers
SLE