II. Epidemiology

  1. Rare

III. Pathophysiology

  1. Low Parathyroid Hormone (PTH) levels
    1. Inadequate PTH Secretion OR
    2. Activating mutation of CASR gene
  2. Low PTH results in Hypocalcemia
    1. Renal Calcium loss (Hypercalciuria)
    2. Decreased Osteoclast activity
    3. Decreased 1,25 - Dihydroxyvitamin D synthesis

IV. Causes

  1. Hereditary
    1. Idiopathic Hypoparathyroidism
    2. DiGeorge Syndrome
    3. Pseudohypoparathyroidism (rare)
      1. Tissue-resistance to PTH
      2. Hypoparathyroidism despite increased PTH levels
  2. Acquired
    1. Anterior neck surgery (75% of Hypoparathyroidism cases)
      1. Thyroidectomy with operative Parathyroid removal (most common cause)
      2. Post-surgical monitoring includes Serum Calcium and PTH levels
    2. Radiation (rare)
    3. Hemochromatosis
  3. Autoimmune Syndrome
    1. Multiple endocrine deficiency syndrome
    2. Adrenal, Ovarian and Parathyroid failure
    3. Mucocutaneous Candidiasis
    4. Alopecia
    5. Vitiligo
    6. Pernicious Anemia

V. Symptoms and signs

VI. Labs

VII. Differential Diagnosis

VIII. Evaluation

  1. See Hypocalcemia for protocol

IX. Management: Calcium Replacement

  1. See Calcium Replacement
  2. General
    1. Adjust Calcium dosing to Serum Calcium
    2. Check for Hypomagnesemia and replace if low
    3. Avoid Hypercalciuria (risk of Nephrolithiasis)
      1. Hypercalciuria can be evaluated with 24 hour Urine Calcium and Urine Creatinine
      2. Thiazide Diuretics may be used to reduce Hypercalciuria
  3. Preparations
    1. Calcium supplement 1.5 to 3 g/day (25 to 50 mg/kg/day) of elemental Calcium in divided doses
      1. Calcium Citrate (21% elemental Calcium) 950 mg two tablets orally three times daily
    2. Calcitriol (Vitamin D Analogue) 0.5 mcg/day

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Related Studies

Ontology: Hypoparathyroidism (C0020626)

Definition (MSH) A condition caused by a deficiency of PARATHYROID HORMONE (or PTH). It is characterized by HYPOCALCEMIA and hyperphosphatemia. Hypocalcemia leads to TETANY. The acquired form is due to removal or injuries to the PARATHYROID GLANDS. The congenital form is due to mutations of genes, such as TBX1; (see DIGEORGE SYNDROME); CASR encoding CALCIUM-SENSING RECEPTOR; or PTH encoding parathyroid hormone.
Definition (NCI) An endocrine disorder characterized by decreased production of parathyroid hormone by the parathyroid glands. It is usually caused by damage of the parathyroid glands during head and neck surgery. Signs and symptoms include muscle cramps, abdominal pain, dry skin, brittle nails, cataracts, tetany, and convulsions.
Definition (NCI_CTCAE) A disorder characterized by a decrease in production of parathyroid hormone by the parathyroid glands.
Definition (CSP) condition produced by greatly reduced function of the parathyroids possibly due to autoimmune disease or genetic factors, or by the removal of those bodies; lack of parathyroid hormone leads to a fall in plasma calcium level, which may result in increase neuromuscular excitability and ultimately tetany followed by a rise in plasma phosphate level.
Concepts Disease or Syndrome (T047)
MSH D007011
ICD9 252.1
ICD10 E20 , E20.9
SnomedCT 190457001, 154697005, 267479004, 36976004
French HYPOPARATHYROIDIE, Hypoparathyroïdie, Insuffisance parathyroïdienne, Hypoparathyroïdisme
English HYPOPARATHYROIDISM, Hypoparathyroidism NOS, Hypoparathyroidism, unspecified, hypoparathyroidism (diagnosis), hypoparathyroidism, Hypoparathyroidism [Disease/Finding], hypoparathyroid, Hypoparathyroidism NOS (disorder), Deficiency of PTH, Deficiency of parathyrin, Deficiency of parathyroid hormone, Hypoparathyroidism (disorder), deficiency; parathyroid, insufficiency; parathyroid, parathyroid; deficiency, parathyroid; insufficiency, Hypoparathyroidism, NOS, Hypoparathyroidism
Spanish HIPOPARATIROIDISMO, hipoparatiroidismo, SAI, hipoparatiroidismo, SAI (trastorno), deficiencia de PTH, deficiencia de hormona paratiroidea, deficiencia de paratohormona, déficit de PTH, déficit de hormona paratiroidea, hipoparatiroidismo (trastorno), hipoparatiroidismo, Hipoparatiroidismo
Swedish Hypoparatyreos
Japanese フクコウジョウセンキノウテイカショウ, 上皮小体機能低下, 上皮小体機能低下症, 上皮小体低下, 上皮小体低下症, 副甲状腺機能低下症, 副甲状腺機能低下
Czech hypoparatyreoidismus, Hypoparatyreóza
Finnish Hypoparatyreoosi
Russian GIPOPARATIREOZ, GIPOPARATIREOIDIZM, ГИПОПАРАТИРЕОЗ, ГИПОПАРАТИРЕОИДИЗМ
Portuguese HIPOPARATIROIDISMO, Hipoparatiroidismo, Hipoparatireoidismo
German HYPOPARATHYROIDISMUS, Hypoparathyreoidismus, nicht naeher bezeichnet, Hypoparathyreoidismus
Korean 상세불명의 부갑상샘 기능저하증, 부갑상샘 기능저하증
Polish Niedoczynność przytarczyc
Hungarian Hypoparathyreosis
Norwegian Hypoparathyreodisme, Hypoparatyreoidisme, Hypoparatyreose
Dutch bijschildklier; deficiëntie, bijschildklier; insufficiëntie, deficiëntie; bijschildklier, insufficiëntie; bijschildklier, Hypoparathyroïdie, niet gespecificeerd, hypoparathyroïdie, Hypoparathyreosis, Hypoparathyreoïdie, Hypoparathyroïdie, Parathyroïdie, hypo-
Italian Ipoparatiroidismo