Orphanet: Familial hyperaldosteronism type III

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Familial hyperaldosteronism type III

Disease definition

Familial hyperaldosteronism type III (FH-III) is a rare heritable form of primary aldosteronism (PA) that is characterized by early-onset severe hypertension, non glucocorticoid-remediable hyperaldosteronism, overproduction of 18-oxocortisol and 18-hydroxycortisol, and profound hypokalemia.


Classification level: Disorder
  • Synonym(s):
    • FH-III
    • FH3
    • Familial hyperaldosteronism type 3
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Infancy, Childhood, Adolescent
  • ICD-10: E26.0
  • OMIM: 613677
  • UMLS: -
  • MeSH: -
  • GARD: 12362
  • MedDRA: -

Detailed information


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