Liddle’s Syndrome:
Liddle’s Syndrome is an autosomal dominant disorder that mimics hyperaldosteronism, resulting in Hypokalemia associated with hypertension.
Pathogenesis of Liddle’s syndrome:
It is due to deletional mutation of the amiloride-sensitive ENaC (epithelial Sodium channel) resulting in unregulated sodium absorption by overactive ENaC in the late distal collecting tubule/ cortical collecting duct.
Clinical features and laboratory findings of Liddle’s syndrome:
- Hypertension (due to volume expansion as well as excessive sodium retention)
- Hypernatremia
- Hypokalemia
- Alkalosis
- Decrease renin
- Decrease aldosterone
Liddle’s syndrome= Hypokalemia + Hypertension
Treatment of Liddle’s syndrome:
- Sodium restriction
- Amiloride or triamterene: Both are a potassium-sparing diuretic, though another member of this group i.e. spironolactone is ineffective in this case
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