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Evidence for persistent dysfunction of wild-type aldosterone synthase gene in glucocorticoid-treated familial hyperaldosteronism type I.

Abstract：

BACKGROUND:In familial hyperaldosteronism type I (FH-I), glucocorticoid treatment suppresses adrenocorticotrophic hormone-regulated hybrid gene expression and corrects hyperaldosteronism. OBJECTIVE:To determine whether the wild-type aldosterone synthase genes, thereby released from chronic suppression, are capable of functioning normally. METHODS:We compared mid-morning levels of plasma potassium, plasma aldosterone, plasma renin activity (PRA) and aldosterone: PRA ratios, measured with patients in an upright position, and responsiveness of aldosterone levels to infusion of angiotensin II (AII), for 11 patients with FH-I before and during long-term (0.8-14.3 years) treatment with 0.25-0.75 mg/day dexamethasone or 2.5-10 mg/day prednisolone. RESULTS:During glucocorticoid treatment, hypertension was corrected in all. Potassium levels, which had been low (< 3.5 mmol/l) in two patients before treatment, were normal in all during treatment (mean 4.0+/-0.1 mmol/l, range 3.5-4.6). Aldosterone levels during treatment [13.2+/-2.1 ng/100 ml (mean+/-SEM)] were lower than those before treatment (20.1+/-2.5 ng/100 ml, P< 0.05). PRA levels, which had been suppressed before treatment (0.5+/-0.2 ng/ml per h), were unsuppressed during treatment (5.1+/-1.5 ng/ml per h, P< 0.01) and elevated (> 4 ng/ml per h) in six patients. Aldosterone: PRA ratios, which had been elevated (> 30) before treatment (101.1+/-25.9), were much lower during treatment (4.1+/-1.0, P< 0.005) and below normal (< 5) in eight patients. Surprisingly, aldosterone level, which had not been responsive (< 50% rise) to infusion of AII for all 11 patients before treatment, remained unresponsive for 10 during treatment. CONCLUSIONS:Apparently regardless of duration of glucocorticoid treatment in FH-I, aldosterone level remains poorly responsive to AII, with a higher than normal PRA and a low aldosterone: PRA ratio. This is consistent with there being a persistent defect in functioning of wild-type aldosterone synthase gene.

journal_name

J Hypertens

journal_title

Journal of hypertension

authors

Stowasser M,Taylor WL,Gartside MG,Tunny TJ,Gordon RD

doi

10.1097/00004872-199715120-00080

subject

Has Abstract

pub_date

1997-12-01 00:00:00

pages

1729-33

issue

12 Pt 2

eissn

0263-6352

issn

1473-5598

journal_volume

pub_type

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Evidence for persistent dysfunction of wild-type aldosterone synthase gene in glucocorticoid-treated familial hyperaldosteronism type I.