What is Liddle’s Syndrome?

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 potassium-sparing diuretic, tough another member of this group i.e. spironolactone is ineffective in this case

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