Bartter Syndrome Presenting with Metabolic Alkalosis: A Case Series

Abstract

Bartter Syndrome (BS) is a rare, inherited renal tubulopathy characterised by hypokalaemic, hypochloraemic metabolic alkalosis. Children present with the complaint of polyuria, dehydration, failure to thrive and normal blood pressure despite hyperreninemia and hyperaldosteronism. This is a series of eight children (2 months -1 years of age, 5 males and 3 females children) diagnosed with BS. Mean age of onset was five months with male predominance. Most common presentation was failure to thrive and polyuria. All children showed metabolic alkalosis with hyponatremia, hypokalemia and hypochloremia. Urinary losses of sodium, potassium and chloride were noted in all the eight children. Diagnosis was based on clinical manifestation and electrolyte abnormalities. All children were started on indomethacin and positive response was noted. On follow-up correction of electrolyte abnormalities along with adequate weight gain was seen. Although, it is a rare disease requiring high index of suspicion, but with prompt clinical diagnosis and early treatment, morbidity and mortality can be significantly reduced.