FURTHER STUDIES ON THE TREATMENT OF CONGENITAL ADRENAL HYPERPLASIA WITH CORTISONE

Abstract

IT IS generally accepted that the adrenal cortex plays a role in the maintenance of blood pressure. Hypotension is a symptom of Addison's disease, and hypertension occurs in Cushing's syndrome. The administration of excessive amounts of desoxycorticosterone or of cortisone raises the blood pressure. Thorn and his associates (1) have treated patients with essential hypertension by bilateral complete adrenalectomy followed by substitution therapy with cortisone and desoxycorticosterone. There was no evidence in the cases treated that the hypertension was caused by excessive or abnormal adrenal hormones. In spite of its frequency in Cushing's syndrome, hypertension is uncommon in the adrenogenital syndrome. It has been described in a number of cases of adrenocortical tumors of the virilizing type (2, 3). One male patient with congenital adrenal hyperplasia and hypertension who improved with cortisone therapy has been reported by Shepard and Clausen (4). In a previous paper (5) the association of these conditions was mentioned in 3 patients whom we shall describe now in more detail. In addition, studies will be reported suggesting that one of these patients had deficiency of the carbohydrate-regulating hormones of the adrenal. We shall take the opportunity to discuss the variations of symptomatology in cases of congenital adrenal hyperplasia and the probability that there may be a number of different patterns of abnormal adrenal activity.