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Nephrology & Renal Medicine

Apparent Mineralocorticoid Excess (AME)

ICD-10 Code
E43_1

Autosomal recessive deficiency of 11-beta-hydroxysteroid dehydrogenase type 2. Cortisol is not inactivated to cortisone and excessively stimulates mineralocorticoid receptors. Mimics Liddle syndrome but caused by cortisol. Also acquired via chronic licorice ingestion.

Clinical Presentation & Protocol

Patient Usually Complains Of

Patient presents with refractory hypertension and persistent hypokalemia. History significant for early-onset hypertensive complications. No history of exogenous mineralocorticoid use. Evaluation for secondary hypertension, specifically focusing on 11β-HSD2 deficiency. Inquire regarding chronic licorice ingestion or herbal supplement use.

Clinical Examination Findings

Physical examination reveals elevated blood pressure readings consistent with hypervolemia. Absence of edema despite mineralocorticoid excess. Patient may exhibit signs of chronic hypertension, including potential retinopathy or left ventricular hypertrophy.

Treatment Protocol

Initiate therapy with potassium-sparing diuretics, specifically amiloride or triamterene, to block epithelial sodium channels. Consider dexamethasone to suppress endogenous cortisol production if indicated. Monitor serum electrolytes and blood pressure closely. Discontinue any licorice-containing products immediately.

Detailed clinical guide coming soon.