TREATMENT OF CONGENITAL ADRENAL HYPER-PLASIA WITH CORTISONE*

Abstract

CONGENITAL adrenal hyperplasia causes pseudohermaphrodism in females and macrogenitosomia precox in males. The differentiation of these conditions from other types of intersexuality and from various disorders causing male sexual precocity is well known (1). This type of hyperadrenocorticism in contrast to the Cushing type is characterized by accelerated growth, muscular development and epiphysial ossification, early growth of sexual hair and progressive virilization, all of which are due to excessive secretion of adrenal androgen. In most cases there are no evidences of disturbance of electrolyte or carbohydrate regulation; in others there is a disorder of electrolyte regulation causing symptoms similar to those of Addison's disease and often leading to death. Unsuccessful attempts (unpublished) have been made by us to suppress the secretion of androgen in patients with congenital adrenal hyperplasia by the administration of steroids, such as 17-ethyl testosterone, 17-vinyl testosterone, 17-methyl androstenediol and 17-methyl androstanediol, which have a chemical structure similar to that of androgens but possess relatively little androgenic activity. Treatment of patients with cortisone (ll-dehydro-17-hydroxycorticosterone acetate) was begun in January 1950 and preliminary reports of the first cases have been published (2, 3). This paper presents in more detail the results we have observed following the administration of cortisone to 6 females and 2 males with congenital adrenal hyperplasia and includes the findings of other observers in 2 additional cases.