Young Girl
PROF.DR.MAGESHKUMARS UNIT
Devendra Patil
HISTORY
I7 year old female pt presented with
Dc p -65. l-35
Hb 11.8 gms
Pcv- 35%
Platelets- 2 lakhs.
RFT:
Urea- 34 mg, creat- 0.9 mg
Na- 137 meq/l, k- 4, cl- 100, bicarb- 20meq/l
Ecg :
Rate- 90/min
Regular ; normal axis ; p wave - normal
Pr interval- 120 msec
Qrs-0.08 sec
Qt interval- 448 msec
Bazets formula : Qtc- 546 msec
Rule of thumb in QT interval
CAUSES OF PROLONGED QTC
Hypokalemia
Hypocalcemia
CARDIAC -
Acute myocarditis, acute MI, HOCM,
torsade de pointes.
CONGENITAL-
Jervell-lange-nielsen syndrome
Romano-ward synd
Cerebral injury.
Hypothermia
DRUGS:
QUINIDINE,
Procainamide,
Tricyclic antidepressants.
Antimalarials- halofantrine
Chest radiograph : NAD
Ct brain:- Normal study
EEG : Normal Study
Problems :
seizures
Prolonged QT in ECG
CT Brain normal
Serum calcium: 5.7 mg/dl[normal-8-10]
Investigations needed to work up a case of
HYPOCALCEMIA
Sr. Calcium ( total / ionised / unionised )
Sr. Albumin levels
Sr. Phosphate levels
Sr. Parathormone levels
ABG
24 Hrs urinary calcium
Sr. Magnesium
Sr. 25 (OH)vit D levels
Sr. Alkaline Phosphatase levels
Serum calcium: 5.7 mg/dl[normal-8-10]
Serum albumin : 3.8 gm /dl
Ionised calcium- 0.452 mmol/l[ 1.16-1.32]
Unionised calcium- 0.458 mmol/l
Serum Phosphorus : 6.2 (2.5 4.5 mmol/dl )
Serum PTH : 5.2pg/ml ( 8-51 pg/ml )
24 Hrs urine Calcium : 7mg/L (< 300mg/day)
Sr. Alkaline Phosphatase : 37 IU/l ( 33-100)
ABG ( room air , at rest )
Po2- 102.4mm hg
Pco2 -39.4 mm hg
Ph- 7.427
Na -138.6. K- 3.17, Cl - 92.9 ( all in meq/l)
Hco3 25.4 mmol/l
Osmololity: 287.6 mosm/kg
Impression : normal study.
Functional classification of
hypocalcemia
PTH low/ absent: PTH ineffective Pth overwhelmed:
Abstract
We report 10 patients with primary hypoparathyroidism. Age at onset varied from 7 months to 52
years (mean 28); 7 were female. Diagnosis was established at a mean of 4.1 years after the
appearance of clinical manifestations. Unexplained hypocalcemia (mean 5.3 mg/dl) and
hyperphosphatemia (mean 6.4 mg/dl) were present in all patients. Prevalent symptoms included
Clinical
tetany (9 patients), seizures (5) and hypocalcemic cataracts (4).
manifestations may be grouped into 5 types 1) tetany; 2)
seizures; 3) other neurologic disorders (basal ganglia calcification,
pseudotumor of the brain, ataxia, nystagmus, hypertonus, paresis); 4) disorders of the lens
including fully developed cataracts and 5) skin alterations like psoriasis and others. Some of these
run on acute course (seizures, tetany), others a subacute one (skin alterations) while others are
rather chronic (cataract and other neurologic disorders). Seizures and electroencephalographic
disorders predominate in younger patients while tetany is more prevalent in older subjects.
The Journal of Clinical Endocrinology & Metabolism Vol. 88, No. 4 1476-1477 , 2003
IMAGES IN CLINICAL ENDOCRINOLOGY
Calcification of the Basal Ganglia in Chronic Hypoparathyroidism
An 80-yr-old man with a more than 60-yr history of postsurgical hypoparathyroidism was evaluated
for hypocalcemia detected during hospitalization after a fall. The patient had long-standing
intermittent paresthesias in his distal extremities, but denied muscle cramps, tetany, or seizures.
His past medical history was remarkable for an episode of nephrolithiasis in his 20s. He had been
. On
taking up to 4000 mg calcium carbonate (oral, daily) before admission
examination, Chvostek and Trousseau signs, tremor,
and rigidity were absent. Laboratory studies included: 5.9 mg/dl serum
calcium (normal range, 8.410.5), 0.78 mmol/liter ionized calcium (normal range, 1.131.32), 6.7
mg/dl phosphate (normal range, 2.74.5), 3.2 g/dl albumin (normal range, 3.55.3), and 1 pg/ml
intact PTH (normal range, 1065). In Fig. 1, brain computed tomography showed diffuse, symmetric
parenchymal calcifications involving the dentate nuclei (A), thalami (B, curved arrow), globus palladi
(B, arrowhead), caudate heads (B, straight arrow), and deep cerebral white matter (C). The patient
was treated with 0.5 g calcitriol and 1000 mg calcium carbonate (oral, daily). After 1 week, his
chronic paresthesias resolved and serum calcium rose to 9.2 mg/dl.
Absence of Basal Ganglia calcification
Upto 30 % of cases of Hypocalcemia have
basal ganglia calcification
BASAL GANGLIA
CALCIFICATION
Birth anoxia
Idiopathic (most common)
bilateral and symmetrical
Hypoparathyroidism /
pseudohypoparathyroidism
Fahr syndrome
Cockayne syndrome
Garg R, Garg N, Tandon N, Khurana ML, Ammini AC. Idiopathic
hypoparathyroidism presenting as epilepsy in a 40 years female. Neurol
India 1999;47:244
The patient was a 40 year old female who complained of generalized tonic
clonic seizures for the last 4 years. The seizures used to occur once in
every 2-3 months. positive Chvostek's and Trousseau's signs. Nervous
system examination was normal. Investigations revealed a serum calcium
level of 6.4 mg/dl (normal - 8.5-10.5 mg/dl) and serum phosphate level of
6.4 mg/dl (normal - 2.5-4.5 mg/dl). Serum albumin was 4.0 G/dl (normal -
3.8-4.0 G/dl) and renal function tests were normal. Serum alkaline
phosphatase level was 8.8 KA units (normal - 3-13 KA units). Serum
parathyroid hormone levels were 28 pg/ml (normal - 11-55 pg/ml) which
were inappropriately low in the presence of hypocalcaemia. Plasma
cortisol, thyroxin and gonadotropins were within normal range. EEG
showed generalized spike and wave pattern. CT scan of the head
was normal.
Idiopathic hypoparathyroidism presenting as
dementia.
D Eraut
Br Med J. 1974 March 9; 1(5905): 429430.
PMCID: PMC1633226
Summary: 80 yr old male
Difference of treatment
1.High dose of vitamin D ( 20000 IU /day ) (
100-120 times the RDA of Vit D )
Why ?
2.High dose of oral Calcium
3.Monitoring of urinary Ca to avoid the risk of
nephrolithiasis
Prophylactic use of Thiazide
Final Diagnosis :
Hypocalcemia induced seizures
Hypoparathyroidism
Mostly Idiopathic variety
Hereditary hypoparathyroidism
May occur without any other endocrinological and
dermatological manifestations.
Manifest within first decade or later.
Syndrome associated with hypopara thyroidism
Di George syndrome[ DSG1-chromosome-22. DSG1-
Chromosome10].
Kenney- Caffey syndrome: hypo parathyroidism, short
stature, osteo sclerosis, thick cortical bones.
Sanjad-Sakati synd: growth failure and dysmorphic
features.
Basal ganglia calcification and extra pyramidal symptoms
are more common and earlier in onset.
Poly glandular auto immune type-1 deficiency:
Mutation in APECED GENE.
childhood onset.
Disease association: muco cuateneous candidiasis,
hypo parathyroidism, adrenal insufficiency,
hypogonadism, alopecia, hypothyroidism, dental
enamel hypoplasia, mal absorption, vitiligo,
chronic active hepatitis, pernicious anemia.
Kearns - Sayre and MELAS syndrome
AD- hypocalacemic hypercalciuria:
Abnormality in calcium sensing receptor.
Receptor senses normal calcium level as
excessive and suppresses pth secretion.
Increased renal excretion of calcium.
External calcium and vit-D supplements
exacerbate the symptoms leading to
irreversible renal damage due to renal stones
or ectopic calcifications.
Acquired hypoparathyroidism
Surgery- para thyroidectomy for hyper para
thyroidism.
Irradiation .
Hemochromatosis.
hemosiderosis.
Calcium Regulation
99% of body calcium in skeleton
Miscible Pool: 40% bound to protein, 13%
complexed w/ anions, 47% free ionized
PTH: Increased Ca, Decreased PO4, Increased
Vitamin D
Vitamin D: Increased Ca, Increased PO4,
Decreased PTH (slow)
Kidney, Bones, GI Tract
Hypocalcemia/Hypoparathyroidism
Acute Hypocalcemia: Parathyroidectomy
PTH undetectable at 8 hours
Normalizes by 30 hours
Calcium nadir at 20 hours, normal Day 2-3
Hungry bone syndrome Old, Labs
Medications: I-131, Cimetidine, ETOH, Cisplatin,
Digoxin, Ampho-B
Hypomagnesemia
Hypocalcemia/
Hypoparathyroidism
Neuro: Paresthesias, fasciculations, muscle
spasm, tetany, irritability, movement disorder,
SEIZURE, laryngeal spasm.
Visual: Cataracts, optic neuritis, papilledema- in
association with increased ict
Pulmonary: Bronchospasm
CV: Prolonged QT, CHF, Hypotension, digitalis
effect may be reduced.
GI: Dysphagia, abdominal pain, biliary colic
GU: Preterm labor
Psychiatric- depression, psychosis.
Chvosteks sign and trousseaus sgn can be used
latent tetany.
Hypoparathyroidism: Treatment
Acute severe hypocalcemia
Check ionized Ca
100-300 mg (10-30 ml) 10% Ca-gluconate in 150
cc D5W over 10 minutes
Continuous infusion at 0.5 mg/kg/hr
EKG monitoring
Check Magnesium
Check Phosphorous
Hypoparathyroidism: Treatment
TREATMENT
Vitamin-D - 40000 to 1,20,000 iu/day[ 1-3
mg /day] combined with elemental calcium is
satisfactory.
Calcitriol- 0.5 to 1 micro gram/day.
They do not reverse the lowered urinary
calcium reabsorption.
Thiazide diuretics lower urinary calcium in
hypoparathyroid pts on vit-D.
HYPOMAGNESEMIA
Levels less than 0.4 mmol/l[ 0.8 meq/l]
Reduced PTH secretion or reduced response to
PTH
Severe hypomagnesemia is paradoxically
associated with low PTH level. Cause unknown.
Serum phosphate levels are usually not elevated.
When magnesium is repleted restoration of PTH
levels may preceed serum calcium by several
days.
Treatment- IV magnesium.
PTH overwhelmed
Loss of ca from ECF is so severe that PTH
cannot compensate.
Pancreatitis,
Acute hyper phosphatemia- due to cell
destruction, tissue damage.
Renal failure.
PTH raises in response to hypocalcemia but
serum calcium do not return to normal.
Differential diagnosis of hypoclcemia
Low calcium, high phosphate-
hypoparathroidism, psudo
hypoparathyroidism.
Low calcium, low phosphate-absent or
ineffective vitamin D.
Hypo magnesemia.
Nutritional deficiency in alcohol.