Pseudohypoparathyroidism with raised plasma alkaline phosphatase.

Abstract

Pseudohypoparathyroidism was described by Albright and his colleagues in 1942. These workers showed that some of the clinical and biochemical manifestations of the condition were due to end- organ insensitivity to parathyroid hormone, and suggested that hypersecretion of the hormone would occur in response to the low plasma calcium level resulting from this insensitivity. This idea was supported by the finding of hyperplastic parathyroid glands in some of these patients (Elrick et al., 1950). However, 5 cases have so far been reported in which there have been raised plasma alkaline phosphatase levels and radiological and, in some cases, histological evidence of osteitis fibrosa (Garceau and Miller, 1956; Kolb and Steinbach, 1962; Costello and Dent, 1963; Bell, Gerard, and Bartter, 1963). In these cases at least it seems likely that, though the kidney is insensitive to the hormone, the bones remain sensitive. If this is so, the low plasma calcium level requires explanation.