A patient with pseudohypoparathyroidism with increased serum calcium and lα,25-dihydroxyvitamin d after exogenous parathyroid hormone administration

The cause for the normal calcemic response to exogenous parathyroid hormone (PTH) was explored in a case of pseudohypoparathyroidism. The diagnosis of pseudohypoparathyroidism in this 29-yr-old woman was well established, since she had hypocalcemia (6.6 mg/dl), hyperphosphatemia (5.0 mg/ dl), high serum immunoreactive PTH, and negligible urinary cAMP response to exogenous PTH. Treatment with Amphojel slightly increased serum calcium (to 7.4 mg/dl) and lowered serum phosphorus (to 4.1 mg/dl) without altering the serum concentration of lα,25-dihydroxyvitamin D [1,25(OH)₂D]. After im administration of parathyroid extract (400 U/day for 4 days), serum calcium increased to 9.2 mg/dl, commensurate with significant elevations in serum 1,25(OH)₂D (from 1.2 to 2.6 ng/dl) and fractional (intestinal) calcium absorption (from 0.22 to 0.43). The results suggest that the synthesis of 1,25(OH)₂D upon PTH challenge was relatively intact and may have accounted for the normal calcemic response.