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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.

ORPHA:403

  • 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: -
  • MeSH: -
  • GARD: 2790
  • MedDRA: -

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