Urinary Phosphate and Cyclic AMP in Pseudohypoparathyroidism
In a 1942 article in Endocrinology, Albright, Burnett, Smith, and Parson (1) described a syndrome characterized by hypocalcemia and hyperphosphatemia, in the absence of renal insufficiency, in which there was little or no restoration of serum calcium and phosphorus values to normal after administration of parathyroid extract. Albright et al termed this syndrome, which resembled hypoparathyroidism, pseudohypoparathyroidism and postulated that its pathophysiology was due to resistance of the kidney and the skeleton to the action of parathyroid hormone. Albright et al, in their first report, were struck by the physical appearance of the patients with this syndrome. They were of short stature, had round faces and short necks, short phalanges, metacarpal and metatarsal bones, exostosis, and ectopic calcium deposits. It was shown subsequently that some patients with certain of these physical characteristics had a normal response to the action of parathyroid hormone and have normal serum calcium and phosphorus,so called pseudo-pseudohypoparathyroidism (2,3). In addition, patients with hypocalcemia and renal resistance to the phosphaturic action of parathyroid hormone but without the somatic characteristics of classic pseudohypoparathyroidism have also been described (3).
KeywordsParathyroid Hormone Phosphate Excretion Osteitis Fibrosa Adenyl Cyclase System Urinary Phosphate
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