Syndrome of Apparent Mineralocorticoid Excess
ICD-10 E26.1 · ICD-11 5A76.Y

Treatment of Syndrome of Apparent Mineralocorticoid Excess with Hypertension and High Cortisol/Cortisone Ratio

This protocol covers the management of non-classic apparent mineralocorticoid excess presenting in adolescents to adults with a high cortisol/cortisone (F/E) ratio, low serum cortisone, low plasma renin, increased urinary potassium excretion, and elevated blood pressure or established hypertension.

Clinical Scenario

Hypertension High F/E ratio Low serum cortisone Low plasma renin Increased urinary K⁺ excretion Adolescents – Adults

Patients are characterised by a high cortisol/cortisone ratio, low plasma renin activity, and increased urinary potassium excretion. Elevated blood pressure or frank hypertension is present, reflecting unregulated mineralocorticoid receptor activation — with consequences extending beyond blood pressure to other tissues including the heart and blood vessels.

Treatment Approach

Management centres on low-dose mineralocorticoid receptor antagonist therapy, combined with potassium supplementation and dietary modification. The complete regimen — including the choice of agent, dose range, and full dietary guidance — is set out in the structured protocol.

Treatment Goals

The primary clinical targets are correction of blood pressure and improvement — ideally normalisation — of plasma renin levels.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1210/clinem/dgz315

Subjects with NC-AME are characterized by a high F/E ratio, low E levels, normal to elevated blood pressure, low plasma renin and increased urinary potassium excretion.

Subjects with NC-AME and elevated BP, or those who are hypertensive should be treated with low-dose MR antagonists and potassium supplementation to control BP and BP-independent damage occurring in other tissues, such as the heart and blood vessels, among others, by unregulated activation of MR.

According to Funder (2017), treatment of mild AME or NC-AME with elevated BP should be straightforward with low-dose spironolactone (12.5–25 mg/day) or eplerenone (25–50 mg/day) with the intention to correct BP and improve renin levels.

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