Health Encyclopedia

Bartter syndrome

Definition

Bartter syndrome refers to a rare group of conditions that affect the kidneys. People with Bartter syndrome have a loss of potassium (hypokalemic alkalosis) and a rise in the hormone aldosterone.

Alternative Names

Potassium wasting

Causes, incidence, and risk factors

In some cases, Bartter syndrome may be genetic and the condition is present from before birth (congenital).

The condition is thought to be caused by a defect in the kidney's ability to reabsorb potassium. As a result, the kidneys remove too much potassium from the body. This is also known as potassium wasting.

Symptoms

This disease usually occurs in childhood. Symptoms include:

Constipation
Growth failure
Increased frequency of urination
Muscle cramping and weakness

Signs and tests

The diagnosis of Bartter syndrome is usually made 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. Other signs of this syndrome include:

High levels of potassium, calcium, and chloride in the urine
High levels of the hormones renin and aldosterone in the blood
Low blood chloride
Metabolic alkalosis

These same signs and symptoms can also occur in people who have taken too many diuretics or laxatives. Urine tests can be done to rule out these causes.

In Bartter syndrome, a biopsy of the kidney typically shows too much growth of kidney cells called the juxtaglomerular apparatus. However, this is not found in all patients, especially in young children.

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.

Many patients also need salt and magnesium supplements, as well as medicine that blocks the kidney's ability to get rid of potassium. High doses of nonsteroidal anti-inflammatory drugs (NSAIDs) may also be used.

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.

Complications

Kidney failure is a possible complication.

Calling your health care provider

Call your health care provider if your child is:

Having muscle cramps
Not growing well
Urinating frequently

References

Tolkoff-Rubin N. Treatment of irreversible renal failure. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 133.

Mitch WE. Chronic kidney disease. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 131.

Review Date:10/15/2008
Reviewed By:Parul Patel, MD, Private Practice specializing in Kidney and Pancreas Transplantation, affiliated with California Pacific Medical Center, Department of Transplantation, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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