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Bartter syndrome
Potassium wasting; Salt-wasting nephropathy
Bartter syndrome is a group of rare conditions that affect the kidneys.
Causes
There are 5 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:
-
Constipation
Constipation
Constipation in infants and children occurs when they have hard stools or have problems passing stools. A child may have pain while passing stools o...
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Rate of weight gain is much lower than that of other children of similar age and gender (
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 gender....
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Needing to urinate more often than usual (
urinary frequency
)
Urinary frequency
Frequent urination means needing to urinate more often than usual. Urgent urination is a sudden, strong urge 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 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 hypokal...
-
High levels of
potassium
, calcium, and
chloride in the urine
Potassium
The potassium urine test measures the amount of potassium in a certain amount of urine.
ImageRead Article Now Book Mark ArticleChloride in the urine
The urine chloride test measures the amount of chloride in a certain volume of urine.
ImageRead Article Now Book Mark Article - High levels of the hormones renin and aldosterone in the blood
- Low blood chloride
-
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
Call your health care provider if your child is:
- Having muscle cramps
- Not growing well
- Urinating frequently
References
Bakkaloglu SA, Schaefer F. Diseases of the kidney and urinary tract in children. In: Skorecki K, Chertow GM, Marsden PA, Taal MW, Yu ASL, eds. Brenner and Rector's The Kidney . 10th ed. Philadelphia, PA: Elsevier; 2016:chap 74.
Guay-Woodford LM. Hereditary nephropathies and developmental abnormalities of the urinary tract. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 128.
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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: 10/13/2015
Reviewed By: Walead Latif DO, nephrologist, Medical Director of Fresenius Vascular Care, and Clinical Assistant Professor of Rutgers Medical School, Newark, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
