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Familial hyperaldosteronism type 1

Orpha number ORPHA403
Synonym(s) Dexamethasone sensitive hypertension
FH1
GRA
Glucocorticoid sensitive hypertension
Glucocorticoid-remediable aldosteronism
Prevalence Unknown
Inheritance
  • Autosomal dominant
Age of onset Variable
ICD-10
  • E26.0
OMIM
UMLS -
MeSH -
MedDRA -
SNOMED CT -

Summary

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


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