Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosis


Sismanlar Eyuboglu T., Dogru D., ÇAKIR E., Cobanoglu N., Pekcan S., Cinel G., ...More

Pediatric Pulmonology, vol.55, no.8, pp.2011-2016, 2020 (Peer-Reviewed Journal) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 55 Issue: 8
  • Publication Date: 2020
  • Doi Number: 10.1002/ppul.24805
  • Journal Name: Pediatric Pulmonology
  • Journal Indexes: Science Citation Index Expanded, Scopus, CAB Abstracts, EMBASE, MEDLINE, Veterinary Science Database
  • Page Numbers: pp.2011-2016
  • Keywords: cystic fibrosis, Pseudo-Bartter syndrome, registry

Abstract

© 2020 Wiley Periodicals, Inc.Background: Pseudo-Bartter syndrome (PBS) is a rare complication of cystic fibrosis (CF) and there are limited data in the literature about it. We aimed to compare clinical features and accompanying findings of patients with PBS in a large patient population. Methods: The data were collected from the Cystic Fibrosis Registry of Turkey where 1170 CF patients were recorded in 2017. Clinical features, diagnostic test results, colonization status, complications, and genetic test results were compared in patients with and without PBS. Results: Totally 1170 patients were recorded into the registry in 2017 and 120 (10%) of them had PBS. The mean age of diagnosis and current age of patients were significantly younger and newborn screening positivity was lower in patients with PBS (P <.001). There were no differences between the groups in terms of colonization status, mean z-scores of weight, height, BMI, and mean FEV1 percentage. Types of genetic mutations did not differ between the two groups. Accompanying complications were more frequent in patients without PBS. Conclusion: PBS was detected as the most common complication in the registry. It could be due to warm weather conditions of our country. It is usually seen in younger ages regardless of mutation phenotype and it could be a clue for early diagnosis of CF.