Bartter syndrome
Potassium wasting; Salt-wasting nephropathyBartter syndrome is a group of rare conditions that affect the kidneys.
Causes
There are five gene defects known to be associated with Bartter syndrome. The condition is present at birth (congenital).
The condition is caused by a defect in the kidneys' ability to reabsorb sodium. People affected by Bartter syndrome lose too much sodium through the urine. This causes a rise in the level of the hormone aldosterone, and makes the kidneys remove too much potassium from the body. This is known as potassium wasting.
The condition also results in an abnormal acid balance in the blood called hypokalemic alkalosis, which causes too much calcium in the urine.
Symptoms
This disease usually occurs in childhood. Symptoms include:
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Constipation
Constipation
Constipation in infants and children means they have hard stools or have problems passing stools. A child may have pain while passing stools or may ...
ImageRead Article Now Book Mark Article - Less weight gain than other children of similar age and sex (growth failure)
Growth failure
Failure to thrive refers to children whose current weight or rate of weight gain is much lower than that of other children of similar age and sex....
Read Article Now Book Mark Article - Frequent urination (urinary frequency)
Urinary frequency
Frequent urination means needing to urinate more often than usual. Urgent urination is a sudden, strong need to urinate. This causes a discomfort i...
ImageRead Article Now Book Mark Article - Low blood pressure
- Kidney stones
- Muscle cramping and weakness
Exams and Tests
Bartter syndrome is usually suspected when a blood test finds a low level of potassium in the blood. Unlike other forms of kidney disease, this condition does not cause high blood pressure. There is a tendency toward low blood pressure. Laboratory tests may show:
Low level of potassium in the blood
Low blood potassium level is a condition in which the amount of potassium in the blood is lower than normal. The medical name of this condition is h...
- High urine potassium, calcium, and chloride levels
Potassium
The potassium urine test measures the amount of potassium in the urine.
ImageRead Article Now Book Mark ArticleChloride levels
The urine chloride test measures the amount of chloride in the urine.
ImageRead Article Now Book Mark Article - High blood levels of the hormones, renin and aldosterone
- Low blood chloride level
- Metabolic alkalosis
Alkalosis
Alkalosis is a condition in which the body fluids have excess base (alkali). This is the opposite of excess acid (acidosis).
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These same signs and symptoms can also occur in people who take too many diuretics (water pills) or laxatives. Urine tests can be done to rule out other causes.
An ultrasound of the kidneys may be done.
Treatment
Bartter syndrome is treated by eating foods rich in potassium or taking potassium supplements.
Many people also need salt and magnesium supplements. Medicine may be needed that blocks the kidney's ability to get rid of potassium. High doses of nonsteroidal anti-inflammatory drugs (NSAIDs) may also be used.
Outlook (Prognosis)
Infants who have severe growth failure may grow normally with treatment. Over time, some people with the condition will develop kidney failure.
Kidney failure
Chronic kidney disease is the slow loss of kidney function over time. The main job of the kidneys is to remove wastes and excess water from the body...
When to Contact a Medical Professional
Contact your health care provider if your child is:
- Having muscle cramps
- Not growing well
- Urinating frequently
References
Guay-Woodford LM. Hereditary nephropathies and developmental renal/urinary abnormalities. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 113.
Kliegman RM, St. Geme JW, Blum NJ, et al. Inherited tubular transport abnormalities: Bartter syndrome. In: Kliegman RM, St. Geme JW, Blum NJ, et al, eds. Nelson Textbook of Pediatrics. 22nd ed. Philadelphia, PA: Elsevier; 2025:chap 571.
Mount DB. Disorders of potassium balance. In: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, eds. Brenner and Rector's The Kidney. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 17.
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Aldosterone level test - illustration
Aldosterone is a hormone released by the adrenal glands. It is part of the complex mechanism used by the body to regulate blood pressure by reabsorbing water in the kidneys.
Aldosterone level test
illustration
Review Date: 12/31/2023
Reviewed By: Walead Latif, MD, Nephrologist and Clinical Associate Professor, Rutgers Medical School, Newark, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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