Pseudohypoparathyroidism medical therapy

The mainstay of treatment for pseudohypoparathyroidism is oral calcium and 1alpha-hydroxylated vitamin D analogues, such as calcitriol. Other forms of Vitamin D cannot be used as parathyroid hormone resistance in the proximal tubule decreases the efficiency of production of 1,25(OH)2 vitamin D from 25-hydroxyvitamin D.Intravenous calcium is recommended for all patients who develop severe symptomatic hypocalcemia.

• The mainstay of treatment for pseudohypoparathyroidism is oral calcium and 1alpha-hydroxylated vitamin D metabolites, such as calcitriol ^[1]
• Other forms of Vitamin D cannot be used as parathyroid hormone resistance in the proximal tubule decreases the production of 1,25(OH)2 vitamin D from 25-hydroxyvitamin D.Intravenous calcium is recommended for all patients who develop severe symptomatic hypocalcemia
• The majority of cases of pseudohypoparathyroidism are self-limited and require only supportive care
• The goal of therapy is to:
  • Serum calcium within the normal range to prevent hypercalciuria
  • Maintain parathyroid hormone levels within reference range which helps prevent bone remodeling and hyperparathyroid bone disease
  • Intravenous calcium is recommended for all patients who develop severe symptomatic hypocalcemia
• Adult
  • Preferred regimen (1):elemental calcium (either calcium chloride or calcium gluconate) 100 mg of over 10 to 20 minute
  • An additional dose of 100 mg/hr of elemental calcium can be infused if symptoms do not resolve, with close monitoring of calcium levels
  • Cardiac monitoring may help to guide therapy as rapid infusion of calcium can cause cardiac conduction defects
  • Preferred regimen (2):Parenteral formulations available are calcium chloride and calcium gluconate 100 mg of over 10 to 20 minute
    •  10% calcium chloride a 10-mL ampule contains 360 mg of elemental calcium
    •  10% calcium gluconate 10-mL ampule contains 93 mg of elemental calcium
• For neonates, infants, and children
  • Preferred regimen (1):10% calcium gluconate   0.5-1 mL/kg of administered over 5 minutes

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