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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.

See also: Aldosterone test



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:

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.



Support Groups:



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


Prevention:



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.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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789 Central Avenue, Dover, NH 03820
Phone: (603) 742-5252
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