Control of aldosterone in 17 alpha-hydroxylase deficiency.
The control mechanism of aldosterone in 3 patients with 17 alpha-hydroxylase deficiency was compared to that in a patient with a deoxycorticosterone-producing tumor. The basal levels of plasma renin activity (PRA) and plasma aldosterone (PAC) were decreased in 2 of the 3 patients with 17 alpha-hydroxylase deficiency and in the patient with a tumor. However, in the third patient with accelerated hypertension, those levels were normal. In the 3 patients with low PRA and PAC, PAC was stimulated by various procedures, although the responses were lower than those in control subjects. In the patient with accelerated hypertension, the responses were similar to those of the control subjects. After 6 months' treatment with dexamethasone, the low levels of PRA and PAC gradually returned to the lower limit of normal in 2 of the patients with 17 alpha-hydroxylase deficiency. These results suggest that the suppression of PAC in patients with 17 alpha-hydroxylase deficiency is probably due to a suppression of the renin-angiotensin system.
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital; D000324:Adrenocorticotropic Hormone / Q000494:pharmacology; D000328:Adult; D000450:Aldosterone / Q000097:blood*; D003900:Desoxycorticosterone / Q000097:blood; D003907:Dexamethasone / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male; D011188:Potassium / Q000494:pharmacology; D012083:Renin / Q000097:blood; D013250:Steroid Hydroxylases / Q000172:deficiency*
D003900:Desoxycorticosterone; D000450:Aldosterone; D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone; D013250:Steroid Hydroxylases; D012083:Renin; D011188:Potassium