Hereditary Adrenocortical Unresponsiveness to ACTH—Another Case

Abstract

We read with interest the excellent article on hereditary adrenocortical unresponsiveness to ACTH. (Pediatrics, 45:43, 1970) by Drs. Franks and Nance. We recently had a newborn infant with adrenocortical dysfunction, deficient cortisol production, and normal aldosterone production whom we feel fits the syndrome. In previous reports of this syndrome, the earliest age of diagnosis was 2 months. Those children who developed adrenal crisis shortly after delivery died before diagnosis or therapy could be initiated. Because of unusual hyperpigmentation and severe hypoglycemia, we were able to initiate studies necessary to make the diagnosis during the neonatal period.