An epidemic of pseudo-Bartter syndrome in cystic fibrosis patients

Abstract

Introduction Pseudo-Bartter syndrome (PBS) is characterized by hyponatremic, hypochloremic metabolic alkalosis that mimicks Bartter syndrome but with no pathology in the renal tubules [5]. This article reports the manifestation of PBS in nine cystic fibrosis (CF) patients diagnosed within a 15-day-period in the hottest summer since 1975 in Ankara, Turkey. Methods Nine children participated in the study; all were diagnosed with CF-PBS between August 15 and August 31, 2006 in the Hacettepe University Pediatric Pulmonary Diseases Unit. The medical records of CF patients between January 1989 and November 2005 were retrospectively investigated. The main air temperatures of all Augusts from 1975 to 2006 were also obtained. Results A total of 57 episodes of PBS were found in 43 CF patients between January 1989 and November 2005 in our center [4, 6]; of these, nine occurred between August 15 and August 31, 2006. The demographic features and laboratory characteristics of these nine CF-PBS patients are given in Table 1. All patients, with the exception of patient no. 8, who was newly diagnosed, had been admitted prior to these dates with PBS. Eight patients were using oral salts, as recommended. A 23-month-old-boy died 6 h after admission despite intensive therapy. While between 1975–2005 the mean air temperature in August in the Ankara region was 22.9°C (range: 7.2– 37.2°C), in August 2006, it was 27.2°C (range: 14.6°C– 38°C). August 2006 was the hottest August since 1975