Cure of Apparent Mineralocorticoid Excess by Kidney Transplantation

To the Editor: Kidney 11β-hydroxysteroid dehydrogenase 2 protects against mineralocorticoid excess by oxidatively converting cortisol, which binds to mineralocorticoid receptors as well as does aldosterone, to cortisone. Loss-of-function mutations in the gene for this enzyme cause the syndrome of apparent mineralocorticoid excess, with severe hypertension and hypokalemia.1,2 Most patients with the syndrome can be treated effectively with mineralocorticoid-receptor antagonists, potassium-sparing diuretics, and dexamethasone. We describe a woman with apparent mineralocorticoid excess who was cured by renal transplantation. Hypertension was diagnosed in a woman at 19 years of age, and apparent mineralocorticoid excess at 28 years of age because of . . .