Continuing need for mineralocorticoid therapy in salt-losing congenital adrenal hyperplasia.
Four patients with salt-losing congenital adrenal hyperplasia (CAH) who had stopped mineralocorticoid therapy for several years, showed raised plasma concentrations of 17OH-progesterone and plasma renin activity, despite adequate glucoticoid therapy. One patient was able to reduce urinary sodium excretion when the sodium intake was restricted. Another patient who was a salt-loser, developed signs of an adrenal crisis when salt deprived. In comparison, one nonsalt-loser and 2 normal subjects decreased urinary sodium excretion in response to sodium restriction. The addition of fludrocortisone (100 micrograms) to usual maintenance doses of glucocorticoid, resulted in normal levels of plasma 17OH-progesterone and plasma renin activity in all 4 salt-losers. Two female salt-losers, with raised plasma testosterone concentrations, began menstruating when their plasma testosterone concentrations returned to normal after treatment with fludrocortisone. It is recommended that salt-losing CAH patients should be given mineralocorticoid, in addition to glucocorticoid therapy, at least until adult life.
D000293:Adolescent; D000308:Adrenocortical Hyperfunction / Q000097:blood / Q000150:complications / Q000188:drug therapy*; D000328:Adult; D002648:Child; D004359:Drug Therapy, Combination; D005260:Female; D005438:Fludrocortisone / Q000627:therapeutic use*; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006908:Hydroxyprogesterones / Q000097:blood; D007010:Hyponatremia / Q000097:blood / Q000150:complications / Q000188:drug therapy*; D008297:Male; D012083:Renin / Q000097:blood; D012964:Sodium / Q000008:administration & dosage
D005938:Glucocorticoids; D006908:Hydroxyprogesterones; D012964:Sodium; D012083:Renin; D005438:Fludrocortisone