Metabolic Studies in Patients With Congenital Adrenal Hyperplasia. Effects of Cortisone Therapy*

ADRENAL hyperplasia may be congenital or acquired and may occur in either male or female. In the female it may manifest itself as female pseudohermaphrodism or as virilism. If the onset of hyperplasia occurs in utero between the third and fifth fetal month, as is the usual case, female pseudohermaphrodism results (1). If the onset is at a later gestational period or postnatally, only virilism results, since the differentiation of the urogenital sinus has occurred before this time. In the male if the onset of adrenal hyperplasia occurs either in utero or before the age of puberty, macrogenitosomia praecox results. All recorded cases of the adrenogenital syndrome with onset after birth but before the age of 10 years have been due to tumor rather than to adrenal hyperplasia (2). In common with other poorly understood medical conditions, congenital adrenal hyperplasia has been approached therapeutically by a number of widely divergent regimens. The results of surgical extirpation of adrenal cortical tissue in general have been disappointing (1). Similarly, therapy with various hormonal preparations, until recently, has met with little success (1, 3, 4). However, during the past two years, since the introduction of cortisone therapy by Wilkins and coworkers (3, 5, 6), the approach to treatment of patients with this condition has been revolutionized.