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Liddle Syndrome

By

L. Aimee Hechanova

, MD, Texas Tech University Health Sciences Center, El Paso

Last full review/revision Jul 2020| Content last modified Jul 2020
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Liddle syndrome is a rare hereditary disorder involving increased activity of the epithelial sodium channel (ENaC), which causes the kidneys to excrete potassium but retain too much sodium and water, leading to hypertension. Symptoms are of hypertension, fluid retention, and metabolic alkalosis. Diagnosis is through measurement of urinary electrolytes. Potassium-sparing diuretics provide the best treatment.

Diagnosis of Liddle Syndrome

  • Urine sodium level

  • Plasma renin and aldosterone levels

Diagnosis is suggested by the presence of hypertension in a young patient, particularly one with a positive family history. Low urine sodium (< 20 mEq, or 20 mmol/L), low plasma renin and aldosterone levels, and response to empiric treatment usually are considered sufficient to confirm the diagnosis. Definitive diagnosis can be achieved through genetic testing (see GeneTests for more information) .

Treatment of Liddle Syndrome

  • Triamterene or amiloride

Triamterene 100 to 200 mg po bid and amiloride 5 to 20 mg po once/day are both effective because they close sodium channels. Spironolactone is ineffective.

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