Treatment of Syndrome of Apparent Mineralocorticoid Excess in Childhood-Onset Hypertension with Hypokalemia
Syndrome of Apparent Mineralocorticoid Excess (AME) presenting from the newborn period through childhood produces severe hypertension alongside a biochemical profile that sets it apart from primary aldosteronism. Recognising this distinct pattern is essential for selecting appropriate treatment.
Clinical Scenario
- Childhood-onset hypertension (newborns to children)
- Hypokalemia and metabolic alkalosis
- Low plasma renin and low serum aldosterone
- High serum or urinary cortisol/cortisone (F/E) ratio
- Nephrocalcinosis may be present
Treatment Approach
Management is built around mineralocorticoid receptor antagonists combined with glucocorticoid therapy aimed at reducing endogenous cortisol. The clinical goal is normalisation of blood pressure.
The complete age-appropriate regimen — including additional agents and potassium considerations — is in the full protocol.
References
DOI: 10.1210/clinem/dgz315
- The clinical features are childhood-onset hypertension, hypokalemia, and alkalosis with low plasma renin, but unlike PA, AME displays low aldosterone levels in the presence of a high serum or urinary cortisol/cortisone (F/E) ratio.
- Affected subjects with classic AME display a low birth weight, severe hypertension, nephrocalcinosis, hypokalemia, suppressed renin, and low aldosterone.
- Mineralocorticoid receptor antagonists at age-appropriate doses are mandatory: relatively low doses are sufficient, given the side effects (spironolactone) or cost (eplerenone), with amiloride and calcium channel blockers occasionally required to normalize BP.
- In addition, patients can also be treated with glucocorticoids that have a long half-life, such as dexamethasone or betamethasone, decreasing ACTH levels and endogenous cortisol.
- The treatment of AME includes MR antagonists, glucocorticoid and potassium supplementation, and additional antihypertensive therapy.
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