Adrenal Hyperplasia—a Case Report of Delayed Onset of the Congenital Form or an Acquired Form1

Abstract

A mildly virilized female age 55 was studied. The first signs of heterogenous precocity presented at the age of 8. The patient menstruated irregularly until ovarian ablation at the age of 38. Urinary steroid excretion studies done during a control period, after ACTH stimulation, on metyrapone administration, and after dexamethasone suppression, were compatible with a compensated deficiency of 21-hydroxylation in the adrenals. Low normal cortisol secretion rate and plasma cortisol were additional evidence in favor of the diagnosis. The upper normal levels of 11-deoxy-17-ketosteroids and elevated levels of 11-oxygenated-17-ketosteroids encountered in the patient might account for the mild virilizing manifestations. It is not possible to determine at this time whether this was a case of the delayed onset of congenital adrenal hyperplasia or an acquired form of the disease.