Studies of Cortisol Metabolism in a Case of the Hypertensive Form of Congenital Adrenal Hyperplasia: Demonstration of the Absence of 11β-Dehydroxylation1

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Abstract
Cortisol-4-C14 was administered on 2 occasions to a child with the hypertensive form of CAH, once before and once during ACTH administration. Five 11-deoxy C21-OH steroids (S, DHS, THS, DOC, THDOC) were isolated from the urines excreted following the cortisol-4-C14 administrations and were shown to be devoid of radioactivity. This finding demonstrated the absence of 11β-dehydroxylation in the patient and showed that the large amounts of 11-deoxy C21-OH steroids excreted did not result from metabolic transformation of cortisol or its metabolites. Cortisol secretion rates determined by the in vivo isotope dilution method were 2.0 and 1.8 mg/day before and during exogenous ACTH stimulation, respectively. These figures were interpreted as indicating maximal endogenous ACTH stimulation of the patient's adrenal glands under control conditions.