FURTHER STUDIES ON THE TREATMENT OF CONGENITAL ADRENAL HYPERPLASIA WITH CORTISONE

Abstract

PREVIOUSLY published studies (1, 2, 3) have shown that the administration of cortisone to patients with congenital adrenal hyperplasiacaused markedly decreased excretion of 17-ketosteroids and biologically active androgen. Both chemical tests and bioassays showedthat there was a simultaneous decrease in urinary estrogen, presumably derived from the adrenals. The output of “corticosteroids” was less uniformly affected, probably due to the fact that while the endogenous secretion of these steroids was suppressed a portion of the cortisone was excreted in this fraction. The patients were all treated with cortisone injected intramuscularly. The last report dealt with 8 patients, 6 females with pseudohermaphrodism and 2 males with macrogenitosomia praecox. At the time of its publication 2 patients had been treated continuously for five months, 1 for three months, the others for periods of only ten to fiftyeight days. It was found that initial doses of 50 mg. or 25 mg. of intramuscular cortisone per day were as effective as 100 mg. per day in causing adrenal suppression. It appeared that 25 mg. of intramuscular cortisone daily would maintain this suppression in the older patients and that probably considerably smaller amounts would be required in infants. In this series, the 2 female pseudohermaphrodites (aged 8½ and 18½ years) who had been treated for five months had developed breasts, and theolder had had regular menstrual cycles. Decreased growth of sexual hair and lessened acne suggested that virilization was diminished. With the doses of cortisone administered, no changes in carbohydrate metabolism were observed. Most of the patients showed no symptoms suggestive of excessive cortisone effect. One boy of 2½ years, however, given 50 mg. of cortisone daily for ten days, became euphoric and manic and his previously existing hypertension increased. Another infant of 11 weeks, after receiving 25 mg. of cortisone daily for two or three months, became obese with a round “moon face” suggestive of Cushing's syndrome.