Experience With Long-Term Cortisone Therapy in Congenital Adrenocortical Hyperplasia:report of 4 Cases*

CONGENITAL adrenocortical hyperplasia is a condition marked by a high degree ofandrogenicity. It is manifested as pseudohermaphrodism in the female and macrogenitosomiapraecox in the male. Characteristically, there is an increased urinary excretion of 17-ketosteroids. Treatment prior to 1950 was unsatisfactory; in fact, the intensity of the virilization was often such that many clinicians considered it preferable to transform some of the more severely masculinized genetic females into passable males than to condemn them to live as unhappy and inadequate females. The male prototype was usually better off, unlesshe had the misfortune to be the victim of an associated electrolyte disturbance, which attimes superimposes the addisonian complex upon the adrenogenital syndrome. Usually his only complaint, once maturity was attained, was his short, even at times dwarfed, stature due to premature epiphyseal closure, the result of early excessive androgenicity. Early in 1950, Sprague et al. (1) reported that the administration of cortisone in man reduced the urinary excretion of 17-ketosteroids. It followed inevitably that attempts would be made to utilize therapeutically this androgenic depressant activity of cortisone.