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Arq Bras Endocrinol Metabol. 2004 Oct;48(5):682-6. Epub 2005 Mar 7.

Glucocorticoid-remediable aldosteronism.

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  • 1Division of Endocrinology, Diabetes & Hypertension, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA. gmcmahon@partners.org

Abstract

Glucocorticoid-remediable aldosteronism (GRA) is a monogenic form of human hypertension that predisposes to cerebral hemorrhage. As a result of a chimeric gene duplication, aldosterone is ectopically synthesized in the cortisol-secreting zona fasciculata of the adrenal gland under the control of adrenocorticotropin (ACTH). Hypertension frequently has its onset during childhood and is usually refractory to standard anti-hypertensives such as ACE inhibitors and beta-blockers. Hypokalemia can develop in those treated with a potassium-wasting diuretic, but random potassium levels are usually normal. Diagnosis has been facilitated by the availability of a genetic test. Suppression of ACTH release with exogenous dexamethasone is a useful diagnostic and therapeutic strategy. Treatment with the mineralocorticoid receptor antagonists spironolactone and epleronone is also efficacious. The diagnosis of GRA facilitates directed therapies and screening of at-risk individuals and kindreds.

PMID:
15761539
[PubMed - indexed for MEDLINE]
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