Studies in Cushing's syndrome. III. Urinary 17-ketosteroids in patients with bilateral adrenal cortical hyperplasia.

There are several reports in the literature concerning urinary 17-ketosteroids in patients with Cushing's syndrome associated with bilateral adrenal cortical hyperplasia (1-7). In most of the published studies, identification of the individual 17-ketosteroids was incomplete, so that the sum of detailed information available is limited. With the development of improved techniques for steroid fractionation and identification, a re-evaluation of the problem seemed indicated. The present investigation was therefore undertaken with a twofold purpose: 1) to make a detailed study of the urinary 17-ketosteroids in patients with this form of Cushing's syndrome; and 2) to determine whether or not the administration of corticotropin to normal subjects was capable of reproducing the excretory pattern of 17-ketosteroids found in Cushing's syndrome with bilateral adrenal corti-cal hyperplasia. It seemed likely that the data resulting from such studies might provide useful information bearing upon the pathogenesis of this disorder. MATERIALS AND METHODS Patients with Cushing's syndrome and bilateral adrenal cortical hyperplasia. Seven patients having the typical clinical and laboratory features of Cushing's syndrome served as the subjects for the study. Pertinent data obtained from these patients are presented in Table I. The absence of adrenal cortical tumor was proved in six of the seven patients by operation (bilateral total or sub-total adrenalectomy). In the seventh patient (Bon.), intravenous pyelography and retroperitoneal insufflation of air gave no evidence of a suprarenal mass. Two of the subjects (Bon. and Fan.) were studied before and during the administration of corticotropin. Bon. received a single intravenous infusion of lyophilized adreno-hour period. Fan. received corticotropin gel, 40 I.U. intramuscularly twice daily for three days. Subjects uwthout adrenal cortical disease. Normal values for the individual 17-ketosteroids were derived from a study of 8 normal males (age range, 21 to 35) and 13 normal females (age range, 20 to 31). These subjects were presumably healthy laboratory workers who were carrying on their usual activities. The average values are summarized in Table II. The data are to be published in detail elsewhere. Two normal females aged 26 and 27 years were studied before and during three day periods of corticotropin administration (ACTH gel, 40 I.U. intramuscularly, twice a day). In addition, studies were carried out in two patients who had been treated with corticotropin for prolonged periods. Patient M.S., a 41 year old white female with bronchial asthma, had received intramuscular doses of 5 to 20 I.U. (lyophilized and gel preparations) with a …