" /> Familial hyperaldosteronism type 1 - CISMeF





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;

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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.

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28/07/2025


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