/glucocorticoid-remediable aldosteronism

glucocorticoid-remediable aldosteronism

Rare Disease

MONDO:0007080

Also Known As

FH-IFH1GRAaldosteronism, glucocorticoid-remediabledexamethasone-sensitive hypertensionfamilial hyperaldosteronism type 1glucocorticoid-remediable aldosteronismglucocorticoid-sensitive hypertensionACTH-dependent hyperaldosteronism syndromeFH 1HALD1aldosteronism, sensitive to dexamethasonedexamethasone sensitive hypertensionfamilial hyperaldosteronism type Iglucocorticoid sensitive hypertensionglucocorticoid-suppressible hyperaldosteronismhyperaldosteronism, familial type 1hyperaldosteronism, familial, type 1hyperaldosteronism, familial, type I

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.