Partial Characterization of Urinary Adrenocortical

Following the demonstration by Wilkins, Lewis, Klein, and Rosenberg (1) that cortisone controls the clinical manifestations and abnormal laboratory findings in virilizing adrenal hyperplasia, Bartter, Albright, Forbes, Leaf, Dempsey, and Carroll (2) postulated a deficient synthesis of "sugar hormone" (17-hydroxycorticosterone, Compound F) in this disease, reasoning from the unusual response of such patients to the administration of adrenocorticotropin (ACTH). This hypothesis has been supported by the demonstration of low blood levels of 17-hydroxylated-corticosteroids ("Porter-Silber chromogens") in untreated patients with adrenal hyperplasia and the usual failure of these levels to rise, following administration of ACTH (3,4). The discovery that, routinely, large quantities of pregnane-3a, 17a,20a-triol are excreted in the urine of patients with virilizing adrenal hyperplasia (5) has suggested a possible site of the metabolic block in the synthesis of Compound F. The present studies were undertaken to characterize the corticosteroids excreted in the urine in adrenal hyperplasia, in an attempt to define more precisely the specific defect in steroid biosynthesis.