علاج متلازمة فرط القشرانيات المعدنية الظاهري في ارتفاع ضغط الدم ذو البدء في مرحلة الطفولة مع نقص بوتاسيوم الدم

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.