Abstract
Pseudohypoparathyroidism (PHP) is a term that refers to a heterogeneous group of metabolic disorders in which resistance to parathyroid hormone (PTH), characterized by hypocalcemia, hyperphosphatemia, and elevation of serum PTH in the setting of normal renal function, is the major clinical feature. In the original report by Fuller Albright, PHP patients showed reduced calcemic and phosphaturic responses to injected bovine parathyroid extract compared to patients with primary hypoparathyroidism (1). This observation led to the speculation that PHP is caused by a defect in PTH action within its target tissues, and was the first description of a hormone resistance syndrome. Subsequent studies describing parathyroid hyperplasia and elevation of immunoreactive serum PTH in untreated PHP patients confirmed that PTH resistance was the underlying defect (2,3).
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