TREATMENT OF CONGENITAL ADRENAL HYPER-PLASIA WITH CORTISONE*

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Abstract
Until now it has never been possible to prevent the precocious and progressive virilization which occurs in female pseudohermaphrodites and males with macrogenitosomia precox due to congenital adrenal hyperplasia. In the 10 cases of congenital adrenal hyperplasia studied, initial doses of 50-100 mg. of cortisone daily in older children, or 25-50 mg. daily in young infants, regularly caused rapid decrease of the urinary 17-ketosteroids to levels of 4-8 mg./day in the older group, and to levels of 0.3-1.4 mg./day in the younger group. The suppression of 17-ketosteroid excretion was subsequently maintained by the admn. of 25 mg. of cortisone daily, or 50 mg. every other day, in older children; whereas doses varying from 10-25 mg./day sufficed in the infants. The effects lasted as long as treatment was continued, the longest period in the series being 5-6 mos. In the 2 cases in which assays were made, the amts. of biologically active androgen in the urine were reduced to probably a greater degree than the 17-ketosteroids. In all cases studied the urinary estroids detd. chemically were high before treatment and were reduced con-comitantly with the 17-ketosteroids. In 1 case it was shown that biologically active estrogen followed the same pattern. The corticosteroids of the urine were affected less regularly. Although there was generally a moderate decrease, in 1 case the output rose again slightly while treatment was being continued, possibly because a portion of these steroids may be derived from the cortisone administered. In 1 case an acute infection occurring in the early stages of treatment with 25 mg. of cortisone daily caused a marked temporary increase in the urinary 17-ketosteroids, corticosteroids and estroids. No changes in carbohydrate metabolism were observed but urinary nitrogen excretion increased. In patients with congenital adrenal hyperplasia and defective electrolyte regulation, cortisone probably has some Na-retaining activity. It is probable that desoxycorticosterone in addition to cortisone will be required to maintain electrolyte equilibrium in some of these patients. In 1 patient erections of the stump of the clitoris stopped promptly. There has been some decrease of acne and seborrhea in a number of cases. In 2 patients treated for 5 mos., development of the breasts and estrogenic changes of the vaginal smear have occurred, and 1 girl has now had 4 regular menstrual periods. In 1 patient hypertension disappeared under treatment. In a patient showing Addisonian-like pigmentation there was marked fading. Treatment with cortisone in small doses offers the possibility of preventing or suppressing the progressive virilization which occurs with congenital adrenal hyperplasia, and may permit the normal development of feminine characteristics in female pseudohermaphrodites.