Glucocorticoid-remediable aldosteronism

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Disease Overview

Glucocorticoid-remediable aldosteronism is one of three types of familial hyperaldosteronism. Aldosterone is a hormone manufactured by the adrenal glands which helps the body retain water and sodium and excrete potassium. It is caused by a fusion of the CYP11B1 and CYP11B2 genes and is inherited in an autosomal dominant manner. Individuals with this condition usually have hypertension (high blood pressure) before age 21. These individuals are also at an increased risk for a certain type of stroke known as a hemorrhagic stroke. First-line therapy consists of a steroid such as prednisone, dexamethasone, or hydrocortisone. This will often correct the overproduction of aldosterone, lower the blood pressure, and correct the potassium levels.[9061]


Synonyms

  • Familial hyperaldosteronism type 1
  • Hyperaldosteronism, familial type 1
  • Dexamethasone sensitive hypertension
  • Glucocorticoid sensitive hypertension

For more information, visit GARD.

National Organization for Rare Disorders