Pseudohypoaldosteronism type 2 I15.1

Rare genetic form of hypertension characterized by hyperkalemia, mild hyperchloremic metabolic acidosis, normal or elevated aldosterone, low renin, with normal renal glomerular filtration rate (GFR).

The prevalence of the disorder is unknown. To date, slightly more than 180 affected individuals and families have been described in the literature.

Hypertension in adulthood; furthermore, hyperkalemia, mild hyperchloremic acidosis, and low renin are present at a young age and are constant findings.

Aldosterone levels may be normal to high. Hypervolemia is frequently observed. Growth failure has been described in some cases. Patients do not present with renal failure.