Hyperactive channels and inherited hypertension: Liddle's syndrome – an epithelial sodium channelopathy

in European Journal of Endocrinology
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Liddle's syndrome (pseudoaldosteronism) represents an inherited, autosomal dominant form of severe hypokalaemic hypertension which clinically resembles primary hyperaldosteronism. Urine assessment in patients with Liddle's syndrome reveals excessive renal sodium absorption and concomitant potassium wasting (1). However, in contrast to primary hyperaldosteronism, serum and urine aldosterone levels are suppressed in patients with this disorder. Further, Liddle's syndrome is frequently refractory to sodium restriction and various regimens of antihypertensive drugs (1). Diuretics acting on the distal tubule, such as triamterene and amiloride, may be effective in controlling the profound hypertension and marked potassium loss in patients with Liddle's syndrome if dietary sodium intake is restricted, while aldosterone antagonists are ineffective. In his original description, Liddle (2) hypothesized that the crucial mechanism in this disorder was that 'the renal tubules transport ions with such abnormal facility that the end result simulates that of a mineralocorticoid excess'. Complete recovery of signs and symptoms in


     European Society of Endocrinology

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