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Diseases

Genetic and Rare Diseases Information Center (GARD)

Glucocorticoid-remediable aldosteronism


Other Names for this Disease
  • Dexamethasone sensitive hypertension
  • Familial hyperaldosteronism type 1
  • Familial hyperaldosteronism type I
  • FH-I
  • FH1
Related Diseases
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Overview

Glucocorticoid-remediable aldosteronism is one of three types of familial hyperaldosteronism and was first described in 1966. 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.[1]
Last updated: 6/12/2015

References

  1. Young, William and Kaplan, Norman. Familial hyperaldosteronism. UpToDate. May 6, 2014; http://www.uptodate.com/contents/familial-hyperaldosteronism. Accessed 6/12/2015.
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In Depth Information

  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
Other Names for this Disease
  • Dexamethasone sensitive hypertension
  • Familial hyperaldosteronism type 1
  • Familial hyperaldosteronism type I
  • FH-I
  • FH1
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.