Congenital adrenal hyperplasia: renin and steroid values during treatment.
Plasma renin activity (PRA), aldosterone (Aldo), 17alpha-hydroxyprogesterone (17-OHP) and testosterone (T), together with urine sodium, pregnanetriol, 17-oxosteroids and the 11-oxygenation index (11-OH) were estimated in 23 patients (age 5.7--18 yrs.) with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency during glucocorticoid treatment. Elevated PRA levels (1400--17200 ng Al/l/hr) were found in 13 out of 15 patients with a history of salt loss. Three non-salt losers showed high PRA levels and in the remaining 5 the levels were in the upper normal range (540--900 ng Al/l/hr). Plasma Aldo levels were normal (25--620 pmol/l) in 18 patients and slightly elevated (690--2360 pmol/l) in 5. While these results indicate persistent impairment of sodium homeostasis in CAH patients, no significant correlations between log. PRA, log. Aldo and urinary sodium excretion were found. Mid-day 17-OHP levels ranged from 9 to 117 nmol/l and T from 0.3 to 18.0 nmol/l. Neither the 17-OHP nor the T results correlated well with the clinical assessment of therapeutic control. The results of the urinary steroid determinations showed better agreement with the clinical assessment of treatment and the 17-oxosteroid, pregnanetriol and 11-OH index results appeared to be better discriminants between good and poor control. Twelve of the patients with a history of early salt loss were reinvestigated after one month's treatment with oral 9 alpha-flurohydrocortisone (0.05 mg/day). PRA was reduced in 7 patients and 17-OHP fell in 10 patients. No consistent changes were found in Aldo, T, or urinary sodium and steroid excretion during this low-dose mineralocorticoid treatment.
D015068:17-Ketosteroids / Q000652:urine; D000293:Adolescent; D000308:Adrenocortical Hyperfunction / Q000097:blood / Q000188:drug therapy / Q000378:metabolism*; D000450:Aldosterone / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006908:Hydroxyprogesterones / Q000097:blood; D008297:Male; D009318:Natriuresis; D011279:Pregnanetriol / Q000652:urine; D012083:Renin / Q000097:blood*; D013250:Steroid Hydroxylases / Q000172:deficiency; D013256:Steroids / Q000097:blood*; D013739:Testosterone / Q000097:blood
D015068:17-Ketosteroids; D005938:Glucocorticoids; D006908:Hydroxyprogesterones; D013256:Steroids; D011279:Pregnanetriol; D013739:Testosterone; D000450:Aldosterone; D013250:Steroid Hydroxylases; D012083:Renin