Pseudohypoparathyroidism medical therapy

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 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.

Medical Therapy

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

Adverse drug reactions
Calcium chloride	Rapid Intravenous injection may cause the patient to complain of tingling sensations, a calcium taste Injections of calcium chloride are accompanied by peripheral vasodilation as well as a local “burning” sensation, and there may be a moderate fall in blood pressure
Calcium gluconate	Patients may complain of tingling sensations, a sense of heat waves and a calcium or chalky taste following the intravenous administration of calcium gluconate Rapid intravenous injection of calcium salts may cause vasodilation, decreased blood pressure, bradycardia, cardiac arrhythmias, syncope and cardiac arrest. Use in digitalized patients may precipitate arrhythmias Local necrosis and abscess formation may occur with intramuscular injection

References

↑ Clarke BL, Brown EM, Collins MT, Jüppner H, Lakatos P, Levine MA, Mannstadt MM, Bilezikian JP, Romanischen AF, Thakker RV (2016). “Epidemiology and Diagnosis of Hypoparathyroidism”. J. Clin. Endocrinol. Metab. 101 (6): 2284–99. doi:10.1210/jc.2015-3908. PMC 5393595. PMID 26943720.

Template:WikiDoc Sources

[pmid26943720-1] Clarke BL, Brown EM, Collins MT, Jüppner H, Lakatos P, Levine MA, Mannstadt MM, Bilezikian JP, Romanischen AF, Thakker RV (2016). “Epidemiology and Diagnosis of Hypoparathyroidism”. J. Clin. Endocrinol. Metab. 101 (6): 2284–99. doi:10.1210/jc.2015-3908. PMC 5393595. PMID 26943720.

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