Abstract

1. The child presented with a hypertensive emergency and had accompanying hypokalemic metabolic alkalosis. Further investigation revealed low renin activity, low serum aldosterone level, and a normal steroid profile, which is suggestive of Liddle syndrome. Other conditions that present with hypertension, hypokalemia, and suppressed renin activity include the syndrome of apparent mineralocorticoid excess, hyperaldosteronism, glucocorticoid–remediable aldo-steronism (GRA), and congenital adrenal hyperpla-sia, which were all excluded for this patient. 2. Liddle syndrome is caused by hyperactivity of the amiloride-sensitive epithelial sodium channel (ENaC), which is highly selective for sodium. Increased Na reabsorption in the distal convoluted tubules and

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