title: 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 micrograms beta-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.
mesh_terms: D019326:17-alpha-Hydroxyprogesterone; D000293:Adolescent; D000311:Adrenal Glands / Q000098:blood supply / Q000378:metabolism*; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications / Q000503:physiopathology*; D000328:Adult; D000450:Aldosterone / Q000097:blood; D002648:Child; D003366:Cosyntropin; D003900:Desoxycorticosterone / Q000097:blood; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D006908:Hydroxyprogesterones / Q000097:blood; D008901:Mineralocorticoids / Q000097:blood / Q000378:metabolism*; D011374:Progesterone / Q000097:blood; D012083:Renin / Q000097:blood; D014770:Virilism / Q000209:etiology / Q000503:physiopathology*
chemical_list: D006908:Hydroxyprogesterones; D008901:Mineralocorticoids; D003366:Cosyntropin; D003900:Desoxycorticosterone; D000450:Aldosterone; D011374:Progesterone; D019326:17-alpha-Hydroxyprogesterone; D012083:Renin; D006854:Hydrocortisone