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Liddle Syndrome

By James I. McMillan, MD

Liddle syndrome is a rare hereditary disorder in which the collecting tubules of the kidneys excrete potassium but retain too much sodium and water, leading to high blood pressure.

The gene that causes Liddle syndrome is dominant, meaning that children of a person with the disorder have a 50% chance of inheriting the defective gene.

Liddle syndrome does not always cause symptoms. When it does, symptoms such as high blood pressure often begin during childhood or young adulthood. People also have low levels of potassium and high levels of bicarbonate in the blood.


  • Blood pressure measurement

  • Urine and blood tests

In addition to finding high blood pressure in a young person, doctors also measure a low amount of sodium in the urine and find low blood levels of hormones that help regulate sodium levels in the blood and thus blood pressure (renin and aldosterone ).

Genetic testing may also be done.


  • Drugs to increase sodium excretion

The condition is effectively treated by drugs that increase sodium excretion and lessen potassium excretion, such as triamterene or amiloride. These drugs effectively lower the blood pressure. The prognosis is very good.

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