Hypoparathyroidism

Muhammad Ahda
152010101034
 Hypoparathyroidism

• Clinically
– Tanda tanda peningkatan aktivitas
neuromuscular

• Biochemically
– hypocalcemia,
– hyperphosphatemia,
– diminished to absent circulating iPTH.
 Etiologi

• Bedah (paling sering)

• Familial

• Idiopathic

• Functional
 Etiologi
Bedah hypoparathyroidism
• Paling umum pada operasi bedah di leher seperti total
thyroidectomy.

Idiopathic hypoparathyroidism
• Dimulai pada usia 2-10
• Wanita memiliki kecenderungan
• Sirkulasi parathyroid antibodies
Autoimmune hypoparathyroidism
• autoimmune polyglandular syndrome
• Dikaitkan dengan kekurangan adrenal primer, mucocutaneous
candidiasis

• Muncul saat 5-9 tahun

Familial hypoparathyroidism
• Autosomal dominant

• Mutasi di PTH gen membawa tidak ada aktivasi PTH

 Others
• Di George’s syndrome
• MEDAC syndrome (multiple endocrine deficiency, autoimmune
candidiasis)
• HAM syndrome (hypoparathyroidism, Addison's disease, and
mucocutaneous candidiasis)
• Congenital aplasia parathyroids
• Deposit besi pada gland
• Deposit tembaga
• Deposit Aluminum
• Infiltrasi metastatic carcinoma
 Functional Hypoparathyroidism
• Dampak panjang dari hypomagnesemia
– Gangguan absropsi gastrointestinal magnesium
– generalized gastrointestinal malabsorption
– alcoholism.
• Serum PTH rendah
• Hypocalcemia
(Mg dibutuhkan untuk PTH melepaskan dan aksi perifer dari PTH)
 Clinical Features

• Kekurangan PTH  hypocalcemia

• Neuromuscular features:
– Paresthesias (perioral, fingertips)
– Muscle weakness and cramps, fasciculations
– Tetany (Chvostek’s and Trousseau’s signs)
 Signs of Hypocalcemia
• Neuromuscular

• CNS

• Ophtalmalogica

• Cardiovaskular

• Dental
 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
 Chvostek’s sign

dipicu dengan tap di

sekitar nervus facialis
dekat anterior telinga
menyebabkan
berkedut nya otot
wajah
Trousseau’s sign

Carpal spasm
response dari pompa
BP sebanyak 20 mm
Hg di sekitar nilai SBP
for 3 min
 Clinical Features

CNS manifestations
– Depression
– Irritability
– Focal or generalized seizures
 Clinical Features

Cardiovaskular manifestasi
– Penurunan myocardial contractility
– Hypotension
– Congestive heart failure
• Cardiovaskular dapat dilihat umum nya pada pasien jantung
dari pengobatan diuretik
• ECG: prolonged QT
• Laryngeal atau bronchospasm (langka)
 Other Clinical Manifestations

• Posterior lenticular cataract

• Dental manifestasi

– Abnormal dalam pembentukan enamel

– Keterlambatan atau absent erupsi gigi

– Permasalahan pada akar gigi

 Diagnosis

• Serum Calcium:
– menurun
• Serum Phosphorus:
– meningkat
• Serum iPTH:
– menurun
 Serum iPTH
• Meningkat berdasarkan derajat hipocalcemia
– 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
 Laboratory Evaluation

• Hypocalcemia
– Corrected total calcium (mg/dL) = (measured total
calcium mg/dL) + 0.8 (4.0 - measured albumin g/dL)
• Fosfat
• ECG: prolonged QT interval
• Skeletal X-rays
• Bone biopsy
 Differential Diagnosis

Calcium PO4 PTH 25-vit D 1,25 -Vit D

Hypoparathyroidism Normal

/N
Pseudohypoparathyroidism Normal

/N
Liver disease

/N
Renal disease Normal
 Differential for Hypocalcemia
Vitamin D Deficiency Pseudohypoparathyroidism
– Congenital rickets – PTH resistance
– Malnutrition Ca Chelation
– Malabsorption
– Hyperphosphatemia
– Liver disease
– Citrate
– Renal disease
– Free fatty acids
• Acute on chronic RF
– Alkalosis
• Nephrotic syndrome
– Fluoride Poisoning
– Hypomagnesemia
– Sepsis
– Anticonvulsants
(phenytoin, primidone)
 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
 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)

 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
 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.

PTH Resistance Syndromes
 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,
 Pseudohypoparathyroidism

• Rare familial disorder

• Target tissue resistance to PTH

• Hypocalcemia, hyperphosphatemia

• Increased parathyroid gland function

• Short stature and short metacarpal and metatarsal

bones.
 Pseudopseudohypoparathyroidism

• Developmental defects without biochemical

abnormalities of pseudohypoparathyroidism.
• Lack evidence of PTH resistance
• 50% reduction in Gs alpha function
• Autosomal dominant
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
 Daftar pustaka
Matfin, G. Endorcrine and metabolic medical emergencies. Endocrine press