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

Abstract

The variation in both the clinical and biochemical response to administered adrenal steroids or corticotropin suggests that naturally occurring adrenal hypercorticism might not always be manifested as fully developed Cushing's syndrome. Until recently, the diagnosis of adrenal hyperfunction depended for the most part on typical clinical findings, since analytical methods for the corticosteroids were not quantitatively reliable. Within the past few years newer methods of steroid analysis have been developed which permit a more accurate measure of cortical function. One of these procedures (1, 2) has been used extensively in the course of evaluating many patients with one or more of the features of Cushing's syndrome. Among these were patients with obesity, hirsutism, diabetes, osteoporosis or hypertension. The 2 patients who are the subject of this report were found to have cortical hyperfunction. Their appearance so belied Cushing's syndrome that the correct diagnosis had not been made by many competent observers, even after prolonged study. The predominant feature was the extreme wasting of bone, muscle and skin, and in one patient this advanced atrophy represented the end-result of continued adrenal hypercorticism of thirty years' duration. In this clinical report we will refer to our observations of the adrenal defect in 6 additional patients with Cushing's syndrome (3). The endocrine data from these studies will be presented in a later report.