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Liddle Syndrome


L. Aimee Hechanova

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

Last full review/revision Nov 2020| Content last modified Nov 2020
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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.

Diagnosis of Liddle Syndrome

  • Blood pressure measurement

  • Urine and blood tests

In addition to finding high blood pressure in a young person, doctors also find a low amount of sodium in the urine and 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.

Treatment of Liddle Syndrome

  • 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.

More Information

The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource.

NOTE: This is the Consumer Version. DOCTORS: Click here for the Professional Version
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Test your knowledge
Tubulointerstitial Nephritis
The kidneys contain tubules, which are structures in the kidneys that are involved in filtering fluids. Tubulointerstitial nephritis is inflammation that affects the tubules and the tissue surrounding them, called “interstitial tissue.” Onset of tubulointerstitial nephritis may be acute (sudden) or chronic (gradual) and often results in kidney failure. Which of the following is the most common cause of acute tubulointerstitial nephritis?
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