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Glucocorticoid-Remediable Aldosteronism

McMahon, Graham T. MB, BCh, MRCPI; Dluhy, Robert G. MD

Cardiology in Review:
Original Articles
Abstract

Glucocorticoid remediable aldosteronism (GRA) appears to be the most common monogenic form of human hypertension. As a result of chimeric gene duplication, aldosterone is ectopically synthesized in the zona fasciculata of the adrenal gland under the control of adrenocorticotropin (ACTH). Affected individuals are typically hypertensive, often with onset in youth, and demonstrate refractoriness to standard antihypertensives such as angiotensin-converting enzyme inhibitors and β-blockers. GRA subjects are normokalemic but often develop hypokalemia when treated with a potassium-wasting diuretic. Analysis of affected kindreds has demonstrated a high prevalence of early cerebral hemorrhage, largely as a result of aneurysms. Identification of affected individuals should allow direct neurovascular screening and targeted antihypertensive therapy.

In 1966, Sutherland et al. 1 described a father and son with an autosomal-dominant hypokalemic hypertensive syndrome. These and subsequent patients 2 had typical biochemical features of primary hyperaldosteronism, including hypertension, suppressed plasma renin activity, and hypokalemia. However, their cases differed from others with hyperaldosteronism because their hypertension and hyperaldosteronism were reversed by the administration of the glucocorticoid dexamethasone. The molecular basis of this disorder, which was known earlier as dexamethasone-suppressible hyperaldosteronism, and more recently as glucocorticoid-remediable aldosteronism (GRA), is now fully understood. GRA has now been reported worldwide, and it appears to be the most common monogenic form of human hypertension.

Author Information

From the Division of Endocrinology, Diabetes & Hypertension, Brigham & Women’s Hospital, and Harvard Medical School, Boston, Massachusetts.

Reprints: Graham T. McMahon, MB, BCh, Division of Endocrinology, Diabetes & Hypertension, Brigham & Women’s Hospital, 221 Longwood Ave., RFB 2, Boston, MA 02115. E-mail: gmcmahon@partners.org

© 2004 Lippincott Williams & Wilkins, Inc.