Hypoparathyroidism and PTH resistance

Effect · Ca low, PO4 high PTH low

Too little PTH, or a receptor that cannot hear it. Calcium falls and phosphate rises because the phosphaturic drive has gone, while alkaline phosphatase stays normal: the pattern that separates it from vitamin D deficiency.

Traced from the start

  1. Fall in ionised calciumRise in ionised calcium
  2. Calcium-sensing receptor on parathyroid chief cells
  3. Parathyroid hormone
  4. Thyroid parafollicular (C) cells
  5. Calcitonin
  6. Distal convoluted and connecting tubule: calcium reabsorptionBone: osteoblast-osteoclast coupling
  7. Hypoparathyroidism and PTH resistance

Detail

Biochemistry
Calcium low, phosphate HIGH, PTH low or inappropriately normal, alkaline phosphatase normal; always measure magnesium as well
Causes
Anterior neck surgery is by far the commonest; then autoimmune disease (isolated, or autoimmune polyendocrine syndrome type 1), 22q11.2 deletion, infiltration by iron or copper, radiation, and severe hypomagnesaemia
Pseudohypoparathyroidism type 1a
GNAS loss of function causing PTH resistance: low calcium and high phosphate with a HIGH PTH, plus Albright hereditary osteodystrophy with short stature, a round face and shortened fourth and fifth metacarpals
Treatment
Calcium with calcitriol or alfacalcidol, since PTH-driven activation of vitamin D is what has been lost; watch for hypercalciuria and nephrocalcinosis, because renal calcium reabsorption is no longer being supported

When it goes wrong

Day one to three after total thyroidectomy

Perioral paraesthesia progressing to carpopedal spasm, with a positive Chvostek sign (facial twitch on tapping over the facial nerve) and Trousseau sign (carpal spasm within three minutes of cuff inflation above systolic pressure), a prolonged QT interval, laryngospasm and seizures

Chronic untreated hypoparathyroidism

Cataracts, basal ganglia calcification with extrapyramidal features, and dental enamel hypoplasia in children

Practise this structure

1 question in the bank tagged Parathyroid.