Suppression and stimulation of mineralocorticoid hormones (MCH) in the simple virilizing form of congenital adrenal hyperplasia (CAH) evaluated by the quantitation in adrenal venous blood

Abstract

Four untreated female patients with the nonsalt-losing form of congenital virilizing adrenal hyperplasia (21-hydroxylase deficiency) (21-OHD) maintained on a daily sodium intake of 120 m-equiv were studied by bilateral adrenal vein catheterization. Simultaneous right and left adrenal and peripheral blood samples were collected for determination of cortisol (F), progesterone (P), 17-hydroxyprogesterone (17-OHP), aldosterone (Aldo), and deoxycorticosterone (DOC). All patients were studied during sequential ACTH suppression (30 min after intravenous administration of 4 mg of dexamethasone) and stimulation (5 min after intravenous administration of 250 μg β-ACTH [cosyntropin]). Basal peripheral concentrations of Aldo, DOC, P and 17-OHP were increased, whereas F concentrations were in the lower limit of the normal range. Dexamethasone suppressed adrenal secretion in all subjects. Subsequent adrenal stimulation by ACTH increased P, 17-OHP and DOC, whereas F returned to only control levels. DOC responses to ACTH in the adrenal vein effluents correlated significantly with Aldo responses but not with the 17-OHP increments, suggesting that the adrenal responses of Aldo and DOC to ACTH are events that probably occur in the same zone.