Preferred Label : Familial hyperaldosteronism type 1;
ICD-11 definition : Type I familial aldosteronism, also called dexamethasone suppressible aldosteronism,
is a rare inherited disorder due to the ectopic expression of the aldosterone synthase
in the fascicular zone of the adrenal gland and marked with early severe hypertension
(often occurring before the age of 20), biological signs of primary aldosteronism
of variable intensity, and an abnormal elevated level of 18-oxo- and 18-hydroxycortisol.;
ICD-11 synonym : Glucocorticoid sensitive hypertension; Dexamethasone sensitive hypertension; GRA - [Glucocorticoid-remediable aldosteronism]; Glucocorticoid-remediable aldosteronism; Hereditary hyperaldosteronism type 1; FH1 - [Familial hyperaldosteronism type 1]; Primary hyperaldosteronism, glucocorticoid-sensitive;
ICD-11 acronym : GRA; FH1;
Origin ID : 1994802431;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
Type I familial aldosteronism, also called dexamethasone suppressible aldosteronism,
is a rare inherited disorder due to the ectopic expression of the aldosterone synthase
in the fascicular zone of the adrenal gland and marked with early severe hypertension
(often occurring before the age of 20), biological signs of primary aldosteronism
of variable intensity, and an abnormal elevated level of 18-oxo- and 18-hydroxycortisol.
