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Pseudo-Bartter syndrome presenting a preschooler with cystic fibrosis

Children with cystic fibrosis (CF) are at increased risk of developing electrolyte abnormalities. In cases of extreme electrolyte and fluid losses (e.g., vomiting and diarrhea) they may present pseudo-Bartter syndrome (PBS)1 characterized by hypokalemic and hypochloremic metabolic alkalosis in the absence of renal tubular disease2. In this condition, the renin-angiotensin-aldosterone is activated, in response to extracellular volume depletion and hyponatremia. PBS most commonly presents in infants under 6 months old. However, an association with high ambient temperatures and seasonal epidemics has been reported in countries with warm weather3, when losses of chlorine and sodium in sweat are increased. There is no data on the incidence of PBS as a complication of CF in Latin America. Retrospective case series studies from Jordan, Turkey and Saudi Arabia suggested an incidence range of 12-18.3% in their pediatric populations2,4,5.