Familial hyperaldosteronism type I

Orpha code: 403OMIM code: 103900

Definition

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.

Disease data
Classification

Disease

Synonyms
Dexamethasone-sensitive hypertension
FH1
FH-I
GRA
Nadciśnienie wrażliwe na deksametazon
Nadciśnienie wrażliwe na glikokorykosteroidy
FH-I
FH1
Familial hyperaldosteronism type 1
GRA
Glucocorticoid-remediable aldosteronism
Glucocorticoid-sensitive hypertension
ORPHA code
403
OMIM code
103900
ICD10 code
E26.0
ICD11 code
5A72.0

No additional description.

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