Orphanet: Familial hyperaldosteronism type I

Search for a rare disease

* (*) mandatory field

Other search option(s)

Suggest an update

(*) Required fields.


Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

Familial hyperaldosteronism type I

Disease definition

Familial hyperaldosteronism type I (FH-I) is a rare heritable, glucocorticoid remediable form of primary aldosteronism (PA) characterized by early-onset hypertension, hyperaldosteronism, variable hypokalemia, low plasma renin activity (PRA), and abnormal production of 18-oxocortisol and 18-hydroxycortisol.


Classification level: Disorder
  • Synonym(s):
    • Dexamethasone-sensitive hypertension
    • FH-I
    • FH1
    • Familial hyperaldosteronism type 1
    • GRA
    • Glucocorticoid-remediable aldosteronism
    • Glucocorticoid-sensitive hypertension
  • Prevalence: 1-5 / 10 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood, Adolescent, Adult
  • ICD-10: E26.0
  • OMIM: 103900
  • UMLS: C1260386
  • MeSH: -
  • GARD: 2790
  • MedDRA: -

Detailed information


The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.