Pseudohypoparathyroidism medical therapy
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Differentiating Pseudohypoparathyroidism from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2]
Overview
The mainstay of treatment for pseudohypoparathyroidism is oral calcium and 1alpha-hydroxylated vitamin D metabolites, 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.
Medical Therapy
- The mainstay of treatment for pseudohypoparathyroidism is oral calcium and 1alpha-hydroxylated vitamin D metabolites, 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 majority of cases of pseudohypoparathyroidism are self-limited and require only supportive care.
- The goal is to maintain serum calcium within the normal range to prevent hypercalciuria and to maintain parathyroid hormone levels within reference range which helps prevent bone remodeling and hyperparathyroid bone disease that can result from elevated parathyroid hormone level.
- Intravenous calcium is recommended for all patients who develop severe symptomatic hypocalcemia. Adult dosage is started 100 mg of elemental calcium (either calcium chloride or calcium gluconate) over 10 to 20 minutes. 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;
- Parenteral formulations available are calcium chloride and calcium gluconate; a 10-mL ampule of 10% calcium chloride contains 360 mg of elemental calcium,and a 10-mL ampule of 10% calcium gluconate contains 93 mg of elemental calcium.
- For neonates, infants, and children, the recommended initial dose is 0.5-1 mL/kg of 10% calcium gluconate administered over 5 minutes.