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Bartter III Syndrome

Inicio Tubulopathies Bartter III Syndrome

Features

Chronic metabolic alkalosis with hypokalemia. Low to normal blood pressure and normal renal function although high plasma renin activity and aldosterone levels.

Clinical Manifestations

Symptoms begin during the first two years and include polyuria, polydipsia and proclivity to dehydration. Other symptoms are vomiting, anorexia, craving for salty foods, growth failure, muscle cramps and weakness.

Diagnosis

Metabolic alkalosis and a decreased level of potassium and chloride in blood, high chloride in urine and increased plasmatic renin and aldosterone levels.

Genetics

Classic Bartter syndrome or type III is caused by mutations CLCNKB which regulates the chloride channel function. The pattern of inheritance is autosomal recessive. For the genetic study it will be necessary samples of the index case and the parents.

Treatment

Prostaglandin inhibitors or NSAIDs like indomethacin or ibuprofen. Sometimes is necessary to use sodium and chlorine supplements, salty foods and provide large volume of water.

Prognosis

Permanent hereditary disease with favorable outcome and prognosis if treated.

Complications

Cardiac arrhythmias, muscle weakness, spasms or cramping. Hyperreninemia and hypokalemia can lead to chronic tubulointerstitial nephritis with progressive renal function impairment.