Apparent mineralocorticoid excess syndromes.

Abstract:

:Apparent mineralocorticoid excess (AME) is a syndrome attributable to congenital deficiency of the enzyme 11 beta-dehydrogenase (11 beta-OHSD) which converts active glucocorticoid cortisol to inactive cortisone. When 11 beta-OHSD activity is impaired, cortisol acts as a potent mineralocorticoid and causes hypertension and hypokalemia with a suppression of the renin-angiotensin-aldosterone system. The increased ratio of urinary cortisol/cortisone metabolites and a prolonged half-life of cortisol are useful for the diagnosis. Dexamethasone and/or potassium sparing diuretics have been used for medication of AME. Licorice ingestion induces a mineralocorticoid excess state, and it seems that this is the result of acquired inhibition of 11 beta-DH by glycyrrhetinic acid. The existence of a second 11 beta-OHSD isoform has been suggested strongly for a long time, and recently, a human 11 beta-OHSD 2 cDNA has been isolated. It appears that 11 beta-OHSD 2 conveys specificity upon the renal MR, and a defect in its activity seems likely to account for the phenotype of AME.

journal_name

J Endocrinol Invest

authors

Shimojo M,Stewart PM

doi

10.1007/BF03349763

subject

Has Abstract

pub_date

1995-07-01 00:00:00

pages

518-32

issue

7

eissn

0391-4097

issn

1720-8386

journal_volume

18

pub_type

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