Renin-angiotensin-aldosterone system: a long-term follow-up study in 17 alpha-hydroxylase deficiency syndrome (17OHDS).
We studied the mineralocorticoid pattern in 4 patients with 17OHD during long-term glucocorticoid treatment. We observed reduction of BP, normalization of K levels, a gradual increase in PRA and in urinary Aldosterone (ALDO); a normal response of plasma ALDO to ACTH and to angiotensin II was present only in one case. We observed a prompt decrease of mineralocorticoid hormones, normalized by long-term therapy only in one case. Discontinuation of treatment induced an increase of ALDO that became suppressed in late off-treatment. Thus, glucocorticoid treatment decreases abnormal steroid levels and activates zona glomerulosa (ZG) function, even if it may take years for ALDO to normalize. Brief discontinuation of therapy induces a surge in ALDO levels, revealing no biosynthetic defect in ZG, while in late off-treatment mineralocorticoids seem to come exclusively from zona fasciculata.
D000293:Adolescent; D000311:Adrenal Glands / Q000187:drug effects / Q000378:metabolism; D000312:Adrenal Hyperplasia, Congenital; D000328:Adult; D000450:Aldosterone / Q000652:urine; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006973:Hypertension / Q000188:drug therapy / Q000209:etiology / Q000378:metabolism; D008297:Male; D008901:Mineralocorticoids / Q000378:metabolism; D012083:Renin / Q000097:blood; D012084:Renin-Angiotensin System; D013250:Steroid Hydroxylases / Q000172:deficiency*; D013577:Syndrome
D005938:Glucocorticoids; D008901:Mineralocorticoids; D000450:Aldosterone; D013250:Steroid Hydroxylases; D012083:Renin