STUDIES OF THE ADRENAL HYPERFUNCTION IN 2 PATIENTS WITH ATYPICAL CUSHING'S SYNDROME

Abstract

Case studies of 2 patients with adrenocortical hyperplasia are reported. Both patients presented a clinical syndrome of marked atrophy of the bones, muscles and skin without other features commonly observed in Cushing''s syndrome. It is postulated that the adrenocortical hyperfunction in these cases was characterized by excessive 17-hydroxycorti-coid excretion with either a relative or an absolute decrease in protein-anabolic adrenal steroids. The urinary 17-hydroxy-corticoid level was clearly elevated in only one of the patients. Metabolic derangement was established in the other by the demonstration of a high 17-hydroxycorticoid-creatinine ratio (steroid index). Both patients responded to small doses of corticotropin with marked increases in 17-hydroxycorticoid excretion. This increased sensitivity of the adrenal cortex is interpreted to mean that endogenous corticotropin levels are normal or low in these cases, and that the primary derangement is probably adrenocortical hyperfunction. In patients with pituitary basophilism, on the other hand, the adrenocortical hyperfunction is thought to be secondary to increased elaboration of corticotropin, and the response to administered corticotropin is not greater than normal. Both patients of this report underwent bilateral total adrenalectomy successfully. Adrenocortical hyperplasia was the pathologic finding in both cases.