ADRENAL-STEROID DYNAMICS IN CONGENITAL ADRENAL-HYPERPLASIA - EVALUATION BY SIMULTANEOUS BILATERAL ADRENAL VENOUS CATHETERIZATION

Ten patients with untreated congenital virilizing adrenal hyperplasia (partial 21-hydroxylase deficiency) were studied by bilateral adrenal vein catheterization. Simultaneous right and left adrenal and peripheral venous blood samples were collected for determination of cortisol, progesterone (P), 17- hydroxyprogesterone (17-OHP), androstenedione, dehydroepiandrosterone, testosterone, and dihydrotestosterone. The concentrations of the above steroids were always higher in the adrenal effluents than in the peripheral blood samples, indicating their secretion by the adrenals. Five of the patients had their steroid dynamics studied during a sequential test of suppression (0.5 h after iv administration of 4 mg dexamethasone) and stimulation [5 min after iv administration of 250 μg β ACTH-(1- 24)]. Peripheral steroid concentrations did not change significantly during these dynamics studies, except for P and 17-OHP which decreased significantly during suppression and increased above control after stimulation. In contrast, significant suppression of adrenal venous blood concentrations of all of the above steroids was demonstrated for every patient. During stimulation, adrenal vein concentration of steroids returned to control values, except for P and 17-OHP which attained higher levels. The results indicate that in congenital adrenal hyperplasia, the adrenal glands secrete significant amounts of testosterone and dihydrotestosterone in addition to androstenedione and dehydroepiandrosterone. The results also indicate that all steroid concentrations are increased after ACTH administration. © 1979 by The Endocrine Society.