Bartter syndrome

Alternative Names

Potassium wasting; Salt-wasting nephropathy

Definition of Bartter syndrome

Bartter syndrome refers to a group of rare conditions that affect the kidneys.

Causes, incidence, and risk factors

There are five gene defects known to be associated with Bartter syndrome. The condition is present from before birth (congenital).

Symptoms

This disease usually occurs in childhood. Symptoms include:

Signs and tests

The diagnosis of Bartter syndrome is usually suspected by finding low levels of potassium in the blood. The potassium level is usually less than 2.5 mEq/L. Unlike other forms of kidney disease, this condition does not cause high blood pressure and there is a tendency toward low blood pressure. Other signs of this syndrome include:

Treatment

Bartter syndrome is treated by keeping the blood potassium level above 3.5 mEq/L. This is done by following a diet rich in potassium or taking potassium supplements.

Expectations (prognosis)

The long-term outlook for patients with Bartter syndrome is not certain. Infants who have severe growth failure may grow normally with treatment. Although most patients remain well with ongoing treatment, some develop kidney failure.

Review

David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Herbert Y. Lin, MD, PHD, Nephrologist, Massachusetts General Hospital; Associate Professor of Medicine, Harvard Medical School. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. – 11/30/2009

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