Pseudohypoparathyroidism Treatment & Management

Updated: Sep 17, 2017
  • Author: Mini R Abraham, MD; Chief Editor: George T Griffing, MD  more...
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Treatment

Approach Considerations

The goals of therapy are to maintain serum total and ionized calcium levels within the reference range to avoid hypercalciuria and to suppress PTH levels to normal. This is important because elevated PTH levels in patients with PHP can cause increased bone remodeling and lead to hyperparathyroid bone disease.

All patients with severe symptomatic hypocalcemia should be initially treated with intravenous calcium. Administration of oral calcium and 1alpha-hydroxylated vitamin D metabolites, such as calcitriol, remains the mainstay of treatment and should be initiated in every patient with a diagnosis of PHP.

In adults, infuse approximately 100 mg of elemental calcium (either calcium chloride or calcium gluconate) over 10 to 20 minutes. If this measure does not alleviate the clinical manifestation, 100 mg/hr of elemental calcium can be infused (in adults), with close monitoring of calcium levels. Do not rapidly infuse calcium because of the possible adverse effects of cardiac conduction defects; cardiac monitoring may help to guide therapy.

The 2 most readily available formulations for parenteral use 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.

Pseudohypoparathyroidism type 1b patients could develop tertiary hyperparathyroidism and/or hyperparathyroid bone disease. Therefore, it is important to treat them with sufficient doses of calcium and vitamin D to maintain serum calcium and PTH levels within or as close to the normal range as possible. [22]

Rarely, extraskeletal osteomas require surgical removal to relieve pressure symptoms. Parathyroidectomy is the treatment of choice in patients with tertiary hyperparathyroidism.

Monitor therapy through regular serum and urinary calcium measurements. Exercise caution to avoid renal or hypercalcemic complications. In addition, monitor serum PTH levels with a goal of maintaining serum PTH levels within the reference range.