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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Purpose: To evaluate a patient with hypertension for apparenent mineralocorticoid excess (AME).
Specialty: Endocrinology, Clinical Laboratory, Nephrology, Cardiology
Objective: criteria for diagnosis
ICD-10: E24.8,