Download - Hypoparathyroidism Hasan AYDIN, MD Endocrinology and Metabolism Yeditepe University Medical Faculty
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HypoparathyroidismHasan AYDIN, MD
Endocrinology and Metabolism
Yeditepe University Medical Faculty
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Hypoparathyroidism
• Clinically– Symptoms of neuromuscular hyperactivity
• Biochemically – hypocalcemia, – hyperphosphatemia, – diminished to absent circulating iPTH.
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Etiology
• Surgical (most common)
• Familial
• Idiopathic
• Functional
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Etiology
Surgical hypoparathyroidism• Most common cause is neck surgery eg.total thyroidectomy
Idiopathic hypoparathyroidism• Age of onset is 2-10 years• Female preponderance• Circulating parathyroid antibodies common
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Autoimmune hypoparathyroidism
• Component of autoimmune polyglandular syndrome
• Associated with primary adrenal insufficiency, mucocutaneous candidiasis
• Age of onset 5-9 years
Familial hypoparathyroidism
• Autosomal dominant
• Mutation in PTH gene leads to defective PTH
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• Di George’s syndrome
• MEDAC syndrome (multiple endocrine deficiency, autoimmune
candidiasis)
• HAM syndrome (hypoparathyroidism, Addison's disease, and
mucocutaneous candidiasis)
• Congenital aplasia of the parathyroids
• Iron deposition in the glands
• Copper deposition
• Aluminum deposition
• Infiltration with metastatic carcinoma
Others
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Functional Hypoparathyroidism
• Long periods of hypomagnesemia
– selective gastrointestinal magnesium absorption defects
– generalized gastrointestinal malabsorption
– alcoholism.
• Serum PTH low
• Hypocalcemia
(Mg is required for PTH release and peripheral action of PTH)
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Clinical Features
• PTH deficiency leads to hypocalcemia
• Effects depend on severity and rate of drop
• Neuromuscular features:
– Paresthesias (perioral, fingertips)
– Muscle weakness and cramps, fasciculations
– Tetany (Chvostek’s and Trousseau’s signs)
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Signs of Hypocalcemia
• Neuromuscular
• CNS
• CVS
• Ophtalmalogical
• Skin
• Dental
• GIS
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Neuromuscular Manifestations
• Paresthesias
• Tetany
– Chvostek's sign.
– Trousseau's sign
• Hyperventilation
• Adrenergic symptoms
– anxiety, tachycardia, sweating, and peripheral and circumoral pallor
• Convulsions
– more common in young people: generalized form of tetany followed
by prolonged tonic spasms; typical epileptiform seizure
• Extrapyramidal signs- Classic parkinsonism
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Chvostek’s sign
Elicited by tapping over facial nerve
causing twitching of ipsilateral facial
muscles
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Trousseau’s sign
Carpal spasm in response to inflation of BP cuff to 20 mm Hg above SBP for 3 min
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Clinical Features
CNS manifestations
– Depression
– Irritability
– Confusion
– Focal or generalized seizures
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Clinical Features
CVS manifestations– Decreased myocardial contractility
– Hypotension
– Congestive heart failure
• CVS features seen particularly in patients with underlying
cardiac disease, or those on digoxin or diuretics
• ECG: prolonged QT
• Laryngeal or bronchospasm (rare)
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Other Clinical Manifestations
• Posterior lenticular cataract
• Dental manifestations
– Abnormalities in enamel formation
– Delayed or absent dental eruption
– Defective dental root formation with short or
blunted roots
• Malabsorption syndrome
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Diagnosis
• Serum Calcium:
– Decreased
• Serum Phosphorus:
– Increased
• Serum iPTH:
– Decreased
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Serum iPTH
• Increased values in a range appropriate to the degree of
hypocalcemia
– pseudohypoparathyroidism, vit D deficiency, vit D
dependency (end-organ resistance to PTH)
– secondary hyperparathyroidism (dietary deficiency of
calcium, intestinal malabsorption of calcium, or excessive
intake of absorbable phosphate- containing drugs)
• Undetectable serum iPTH
– hypoparathyroidism
– functional hypoparathyroidism due to hypomagnesemia
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Laboratory Evaluation
• Hypocalcemia
– Corrected total calcium (mg/dL) = (measured total
calcium mg/dL) + 0.8 (4.0 - measured albumin g/dL)
• PO4, Mg, iPTH, BUN/Cr, 25 (OH) Vit D, 1,25 (OH) vit D3, Alk
Phos
• ECG: prolonged QT interval
• Skeletal X-rays
• Bone biopsy
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Differential Diagnosis
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Differential for Hypocalcemia
Vitamin D Deficiency– Congenital rickets– Malnutrition– Malabsorption– Liver disease– Renal disease
• Acute on chronic RF• Nephrotic syndrome
– Hypomagnesemia– Sepsis– Anticonvulsants
(phenytoin, primidone)
Pseudohypoparathyroidism
– PTH resistance
Ca Chelation
– Hyperphosphatemia
– Citrate
– Free fatty acids
– Alkalosis
– Fluoride Poisoning
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Treatment
• Physiologic replacement of PTH
• Pharmacologic doses of vitamin D
– (ergocalciferol or its more potent analog
dihydrotachysterol, in combination with oral
calcium administration)
• Diets low in phosphate (restriction of dairy products
and meat) and oral aluminum hydroxide gels
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Emergency Measures for Tetany
• Intravenous calcium (10-20 ml of a 10% solution of calcium gluconate
(40 mg elemental calcium per 10 mL)
• Vitamin D
• Oral calcium 200 mg of elemental calcium (as the carbonate salt)
every 2 hours and gradually increasing to 500 mg every 2 hours if
necessary.
• Continuous calcium infusion (500 ml of 5% glucose and water
containing 10 ml of 10% calcium gluconate is given over 6 hours
initially)
• Anticonvulsive agents (phenytoin, phenobarbital)
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Severe Hypocalcemia
• Hypocalcemia may be profound and resistant to treatment (“bone hunger” syndrome).
• 10 g of elemental calcium IV infusion over 24 hours
• 1,25(OH)2D3 (calcitriol [Rocaltrol]) in doses ranging from 0.5 to 2 µg daily
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Marked Hypoparathyroidism
• Long-term vitamin D treatment
– dihydrotachysterol ( 1 mg is equivalent to about 120,000 units
or 3 mg of vitamin D2), 4 mg/d as a single dose for 2 days,
then 2 mg/d for 2 days, then 1 mg/d
– Ergocalciferol (vitamin D2 40,000 units/mg).
– Cholecalciferol metabolites calcifediol and calcitriol
• Calcium- total (dietary and supplemental) intake of 1 g or more of
the element daily in patients under age 40 and 2 g in patient over
age 40.
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Complications
• Hypercalcemia
• Hypercalciuria
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PTH Resistance Syndromes
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Pseudohypoparathyroidism
• Abnormal target tissue responses
– receptor binding of the hormone
– final expression of the cellular actions of PTH
• Resistance to several other hormones (vasopressin, glucagon).
• Secretion of a biologically inert form of PTH,
• Circulating inhibitors of PTH action,
• An intrinsic abnormality of PTH receptors,
• Autoantibodies to the PTH receptor,
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Pseudohypoparathyroidism
• Rare familial disorder
• Target tissue resistance to PTH
• Hypocalcemia, hyperphosphatemia
• Increased parathyroid gland function
• Short stature and short metacarpal and metatarsal bones.
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Pseudopseudohypoparathyroidism
• Developmental defects without biochemical
abnormalities of pseudohypoparathyroidism.
• Lack evidence of PTH resistance
• 50% reduction in Gs alpha function
• Autosomal dominant
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PsHP Type Ia (Albright Syndrome)
• Hypoparathyroidism, short stature, round facies, obesity,
brachydactily, neck webbing, sc calcifications
• Defect in the function of Gs protein
• TSH, Glucagon, Gonadotropin resistance
• Autosomal dominant
• Intermittant hypocalcemia, elevated PTH,
low urine Ca
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Diagnosis• Developmental abnormalities
• Serum calcium and phosphorus normal
pseudopseudohypoparathyroidism
• Hypocalcemia and hyperphosphatemia
pseudohypoparathyroidism
• Increased serum iPTH and markedly diminished phosphaturic
and nephrogenous cAMP responses to PTH distinguish
• Serum phosphorus normal, low in a hypocalcemic patient
secondary hyperparathyroidism due to vitamin D or dietary
calcium deficiency and intestinal malabsorption of calcium
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T h a n k y o u !