Requirement of mineralocorticoid in congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency.
Marginal salt loss occurs in patients with congenital adrenal hyperplasia due to 11 beta-hydroxylase (11-OHase) deficiency treated with dexamethasone and is accompanied by increased PRA. The present study was undertaken to evaluate the effect of the stimulated renin-angiotensin system on pituitary-adrenal suppression. Seven patients with 11-OHase deficiency were subjected to a series of treatments with dexamethasone, cortisol, and combined cortisol and 9 alpha-fluorohydrocortisone. The latter combination suppressed PRA and sodium excretion, and produced better control of the pituitary-adrenal axis, as measured by plasma ACTA and serum 11-deoxycortisol. We conclude that in children with 11-OHase deficiency, PRA needs to be monitored, and when it is elevated, mineralocorticoid replacement is indicated.
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy* / Q000378:metabolism; D000324:Adrenocorticotropic Hormone / Q000097:blood; D002648:Child; D002675:Child, Preschool; D003350:Cortodoxone / Q000097:blood; D003907:Dexamethasone / Q000627:therapeutic use; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D005438:Fludrocortisone / Q000627:therapeutic use; D006801:Humans; D006854:Hydrocortisone / Q000031:analogs & derivatives / Q000627:therapeutic use; D008297:Male; D008901:Mineralocorticoids / Q000172:deficiency / Q000627:therapeutic use*; D012084:Renin-Angiotensin System / Q000187:drug effects; D013250:Steroid Hydroxylases / Q000172:deficiency*; D014882:Water-Electrolyte Balance / Q000187:drug effects
D008901:Mineralocorticoids; C021650:hydrocortisone acetate; D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone; D013250:Steroid Hydroxylases; D005438:Fludrocortisone; D003350:Cortodoxone; D006854:Hydrocortisone