Glucocorticoid-remediable aldosteronism (GRA) is a monogenic form of human hypertension that predisposes to cerebral hemorrhage. As a result of a chimeric. Glucocorticoid-remediable aldosteronism (GRA), alternatively called dexamethasone-suppressible hyperaldosteronism (DSH) or familial hyperaldosteronism. Aldosterone suppression by dexamethasone, and high hydroxycortisol and oxocortisol levels are used to differentiate glucocorticoid-remediable.

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Case Reports in Endocrinology

Glucocorticoid-remediable aldosteronism is associated with severe hypertension in early childhood. Abstract Glucocorticoid remediable aldosteronism GRA is rare familial form of primary aldosteronism characterized by a normalization of hypertension with the administration of glucocorticoids. A prospective study by Rich et al.

The prevalence of GRA in primary aldosteronism remains to be established. Genetic analysis of GRA kindreds has revealed that the disorder is inherited as an autosomal dominant trait 6.

A new form of congenital adrenal hyperplasia. Because of the side effects of glucocorticoids, in children eplerenone can be given remeviable. Blood samples were taken on the fifth day, 2 h after the morning dose of dexamethasone at h.

The patient had a 4 pack-year smoking history. Potassium levels Normokalemia in GRA-affected patients was first described by Grim and Weinberger 4Abut it was believed to be uncommon.

The chimerism also results in the production of the hybrid steroids oxocortisol and hydroxycortisol that can be used as diagnostic glucocorricoid 5. Evaluation of the dexamethasone suppression test for the diagnosis of glucocorticoid-remediable aldosteronism. GRA is usually characterized by moderate to severe hypertension with onset early in life.


His cardiovascular history was notable for 3 aortic dissections, at ages of 10, 17, and 22, mid-thoracic aortic aneurysm requiring endovascular repair at the age of 18, and remeviable aortic aneurysm which required open surgical repair at the age of Views Read Edit View history. Glucocorticoid remediable aldosteronism is a rare form of familial hyperaldosteronism remediwble by an autosomal dominant pattern of inheritance [ 3 ].

Email alerts New issue alert. However, in subjects with glucocorticoid-remediable aldosteronism, ACTH increases the activity of existing aldosterone synthaseresulting in vlucocorticoid abnormally high rate of aldosterone synthesis and hyperaldosteronism.

Glucocorticoid remediable aldosteronism – Wikipedia

Amiloride blocks the gemediable sodium epithelial channel in the distal nephron and is an alternative to spironolactone treatment. It is not clear whether oxo-F and OH-F remediablee sodium-retaining properties and contribute to the phenotypic variability of this disorder. A case report of insulinoma relapse on background nesidioblastosis: Hyperaldosteronism therefore results in volume expansion, hypertension and usually hypokalemia. Thus, glucocorticoid treatment reverses the volume expansion associated with GRA, leading to reactivation of the suppressed renin-angiotensin system and restoration of function of the previously suppressed zona glomerulosa.

Screening targeted at these features performed at one hypertension clinic discovered two index families and four further families containing 40 mutation-positive individuals in one year From Wikipedia, the free encyclopedia.

Glucocorticoid-remediable aldosteronism.

This is an open access article distributed under the Creative Commons Attribution Licensewhich permits unrestricted use, aldosterronism, and reproduction in any medium, provided the original work is properly cited. Primary adrenal hyperplasia and renin responsive adenoma. Also, the early use of mineralocorticoid antagonists may have a significant preventive and attenuating effect in aneurysm formation, an association which needs to be confirmed in future studies.


We present a case of GRA and thoracoabdominal aneurysm complicated by multiple aortic dissections requiring complex surgical rwmediable endovascular repairs.

A Case of Glucocorticoid Remediable Aldosteronism and Thoracoabdominal Aneurysms

All the contents of this journal, except where aldotseronism noted, is licensed under a Creative Commons Attribution License. The authors wish to thank Dr. A PCR-based method aldosteronis screening individuals of all ages, from neonates to the elderly, for familial hyperaldosteronism type I.

The extended-release formulation of the dihydropyridine calcium channel blocker nifedipine has also been advocated in the medical management of primary aldosteronism because this agent has also been shown to inhibit aldosterone biosynthesis in vitro.

PRA will be suppressed unless mineralocorticoid antagonists such as spironolactone or amiloride have been used remedaible therapeutic agents. However, to date, no conversions of the CYP11B1 gene expected to cause GRA involving exons 4, 5, and 6 from CYP11B2 have been found in a sample of low renin hypertensive patients, patients with primary aldosteronism, and 90 normotensive individuals Sign In or Create an Account.

Clinicians should consider the diagnosis, particularly in hypertensive children, and those with a family history of either early-onset hypertension or early cerebral hemorrhage.

Hypokalemia Most patients with GRA have normal potassium levels 8 despite alrosteronism evidence for primary hyperaldosteronism.