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Hypoparathyroidism

Definition

Hypoparathyroidism is defined as a disorder characterised by inadequate secretion or action of PTH

Aetiology

Important causes: [Ref1][Ref2]

  • Surgery – leading overall cause
    • Parathyroidectomy
    • Unindented injury from thyroidectomy
  • Autoimmune destruction – leading non-surgical cause
  • Genetic cause (e.g. DiGeorge syndrome)

Other causes: [Ref1][Ref2]

  • Infiltrative diseases (e.g. haemochromatosis, Wilson’s disease)
  • Neck irradiation
  • Hypomagnesium (causes functional hypoparathyroidism, therefore it’s important to correct any underlying hypomagnesium before correcting hypocalcaemia)

Clinical Features

Clinical features are typically secondary to hypocalcaemia [Ref]

Early / mild hypocalcaemia features [Ref]
  • Paraesthesia (peri-oral and in extremities)
  • Muscle cramps / stiffness / myalgia
  • Signs:
    • Chvostek sign (facial twitching upon tapping the facial nerve)
    • Trousseau sign (carpopedal spasm induced by brachial artery occlusion with a BP cuff)
Features of more severe hypocalcaemia: [Ref]
  • Laryngospasm
  • Tetany
  • Seizures
  • QTc prolongation and torsades de pointes

Hypocalcaemia increases neuromuscular excitability because ionised calcium normally stabilises voltage-gated sodium channels. When calcium levels fall, these channels open more easily, leading to spontaneous depolarisation.

Rare features typically only seen in chronic hypoparathyroidism[Ref]

  • Neuropsychiatric symptoms (e.g. confusion, depression, irritability, cognitive dysfunction)
  • Basal ganglia calcification (→ secondary Parkinsonism)
  • Ophthalmologic
    • Cataracts
    • Papilloedema

Investigation and Diagnosis

Biochemical changes: [Ref]

  • ↓ PTH
  • ↓ Calcium
  • ↑ Phosphate
  • ↓ / normal 1,25 dihydroxyvitamin D (due to lack of PTH-stimulated renal conversion)

Rationising the biochemical changes:

  • Actions of PTH
    • ↑ Serum calcium (via ↑ bone resorption, ↑ renal calcium reabsorption, ↑ intestinal calcium absorption)
    • ↓ Serum phosphate (via ↑ renal phosphate excretion)

The initiating event is low PTH, which results in the subsequent biochemical changes (opposite of PTH’s action).

Management

Conventional therapy: [Ref1][Ref2]

  • Oral calcium supplementation
  • Active vitamin D (calcitriol / 1,25-hydroxyvitamin D)

 

  • Phosphate binders for hyperphosphataemia
  • Thiazide diuretic may be added in persistent hypercalciuria

If conventional therapy failed: PTH replacement therapy (e.g. teriparatide)

Therapeutic goal in hypoparathyroidism is to:

  • Maintain serum calcium in the low-normal range
  • Avoid hypercalcaemia and hypecalciuria
  • Maintain serum phosphate in the high-normal range

Notes on various types of vitamin D and their use:

Vitamin D type Description Indication
 

Native forms of vitamin D

  • Preferred: Vitamin D3 (cholecalciferol)
  • Alternative: Vitamin D2  (ergocalciferol)
 

Must be hydroxylated in the liver to 25-hydroxyvitamin D (calcifediol), then in the kidney into 1,25-dihydroxyvitamin D (calcitriol) for biological activity

  • Routine vitamin D deficiency
  • Osteoporosis
  • Supplementation in the general population
25-hydroxyvitamin D (calcifediol) Major circulating form of vitamin D (produced by 25-hydroxylation in the liver)

(If vitamin D deficiency is suspected, 25-hydroxyvitamin D is measured)

  • Considered in liver impairment (calcifediol does not require hepatic hydroxylation, it can be readily hydroxylated by the kidneys)
Activated vitamin D metabolites (bypassing the renal activation process)
  • 1-α-hydroxycholecalciferol (alfacalcidol)
  • 1,25-hydroxyvitamin D (calcitriol)

 

Both alfacalcidol / calcitriol can be given as supplements to bypass the renal activation process (thus considered activated vitamin D metabolites):
  • Calcitriol is the fully active form of vitamin D (produced naturally by the kidney’s 1-alpha hydroxylase)
  • Alfacalcidol is synthetic, and it requires liver activation to be fully active

 

Impaired renal 1-alpha hydroxylase activity:
  • Stage 4/5 CKD (specific indication in CKD article)
  • Hypoparathyroidism 

Not used for routine deficiency replacement or for general population supplementation due to increased risk of hypercalcaemia

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