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Apparent mineralocorticoid excess syndrome: an overview

Palermo, Mario and Quinkler, Marcus and Stewart, Paul M. (2004) Apparent mineralocorticoid excess syndrome: an overview. Arquivos Brasileiros de Endocrinologia & Metabologia, Vol. 48 (5), p. 687-696. eISSN 1677-9487. Article.

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DOI: 10.1590/S0004-27302004000500015


Apparent mineralocorticoid excess (AME) syndrome results from defective 11ß-hydroxysteroid dehydrogenase type 2 (11b-HSD2). This enzyme is co-expressed with the mineralocorticoid receptor (MR) in the kidney and converts cortisol (F) to its inactive metabolite cortisone (E). Its deficiency allows the unmetabolized cortisol to bind to the MR inducing sodium retention, hypokalemia, suppression of PRA and hypertension. Mutations in the gene encoding 11ß-HSD2 account for the inherited form, but a similar clinical picture to AME occurs following the ingestion of bioflavonoids, licorice and carbenoxolone, which are competitive inhibitors of 11ß-HSD2. Reduced 11ß-HSD2 activity may explain the increased sodium retention in preeclampsia, renal disease and liver cirrhosis. Relative deficiency of 11ß-HSD2 activity can occur in Cushing's syndrome due to saturation of the enzyme and explains the mineralocorticoid excess state that characterizes ectopic ACTH syndrome. Reduced placental 11ß-HSD2 expression might explain the link between reduced birth weight and adult hypertension. Polymorphic variability in the HSD11B2 gene in part determines salt sensitivity, a forerunner for adult hypertension onset. AME represents a spectrum of mineralocorticoid hypertension with severity reflecting the underlying genetic defect in the 11ß-HSD2; although AME is a genetic disorder, several exogenous compounds can bring about the symptoms by inhibiting 11ß-HSD2 enzyme. Substrate excess as seen in Cushing's syndrome and ACTH ectopic production can overwhelm the capacity of 11ß-HSD2 to convert F to E, leading up to an acquired form of AME.

Item Type:Article
ID Code:257
Uncontrolled Keywords:Hypertension, 11beta-HSD2, 11alfa-HSD2, AME syndrome, cortisol, cortisone
Subjects:Area 06 - Scienze mediche > MED/13 Endocrinologia
Divisions:001 Università di Sassari > 03 Istituti > Clinica medica generale e terapia medica
Publisher:Sociedade Brasileira de Endocrinologia e Metabologia
Additional Information:Pubblicato online il 7 marzo 2005.
Deposited On:18 Aug 2009 10:01

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