Apparent mineralocorticoid excess (AME) is a form of hereditary hypertension that is cortisol-mediated.


Inheritance: autosomal recessive


Mutation: Type 2 isoenzyme of 11-beta hydroxysteroid dehydrogenase


Effect: reduced activity, with decreased metabolism of cortisol to cortisone


Clinical features:

(1) hypertension

(2) low plasma renin and aldosterone concentrations

(3) normal serum cortisol level with increased half life

(4) increased ratio of urine cortisol to cortisone in a 24 hour urine

(5) therapeutic response to high doses of spironolactone or amiloride


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