Catalog

Familial hyperaldosteronism type 1

No MMS code

Link to WHO Definition

Familial hyperaldosteronism type 1 – http://id.who.int/icd/entity/1994802431

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.

Parents