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

Autor: Sismanlar Eyuboglu T; Department of Pediatric Pulmonology, Faculty of Medicine, Gazi University, Ankara, Turkey., Dogru D; Department of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara, Turkey., Çakır E; Department of Pediatric Pulmonology, Faculty of Medicine, Bezmialem Vakif University, İstanbul, Turkey., Cobanoglu N; Department of Pediatric Pulmonology, Faculty of Medicine, Ankara University, Ankara, Turkey., Pekcan S; Department of Pediatric Pulmonology, Meram Medicine Faculty, Necmettin Erbakan University, Konya, Turkey., Cinel G; Department of Pediatric Pulmonology, Faculty of Medicine, Ankara Yıldırım Beyazıt University, Ankara, Turkey., Yalçın E; Department of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara, Turkey., Kiper N; Department of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara, Turkey., Sen V; Department of Pediatric Pulmonology, Faculty of Medicine, Dicle University, Diyarbakır, Turkey., Selimoglu Sen H; Department of Pulmonology, Faculty of Medicine, Dicle University, Diyarbakır, Turkey., Ercan O; Department of Pediatric Pulmonology, Meram Medicine Faculty, Necmettin Erbakan University, Konya, Turkey., Keskin O; Department of Pediatric Allergy, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey., Bilgic Eltan S; Department of Pediatric Allergy, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey., Alshadfan L; Department of Pediatric Pulmonology, Faculty of Medicine, Bezmialem Vakif University, İstanbul, Turkey., Yazan H; Department of Pediatric Pulmonology, Faculty of Medicine, Bezmialem Vakif University, İstanbul, Turkey., Altıntas DU; Department of Pediatric Allergy and Immunology, Faculty of Medicine, Çukurova University, Adana, Turkey., Sasihuseyinoglu AS; Department of Pediatric Allergy and Immunology, Faculty of Medicine, Çukurova University, Adana, Turkey., Sapan N; Department of Pediatric Allergy and Immunology, Faculty of Medicine, Uludag University, Bursa, Turkey., Cekic S; Department of Pediatric Allergy and Immunology, Faculty of Medicine, Uludag University, Bursa, Turkey., Cokugraş H; Department of Pediatric Allergy and Pulmonology, Cerrahpasa Medicine Faculty, İstanbul University, İstanbul, Turkey., Kılınc AA; Department of Pediatric Allergy and Pulmonology, Cerrahpasa Medicine Faculty, İstanbul University, İstanbul, Turkey., Ramaslı Gursoy T; Department of Pediatric Pulmonology, Faculty of Medicine, Gazi University, Ankara, Turkey., Aslan AT; Department of Pediatric Pulmonology, Faculty of Medicine, Gazi University, Ankara, Turkey., Bingol A; Department of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine, Akdeniz University, Antalya, Turkey., Başaran AE; Department of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine, Akdeniz University, Antalya, Turkey., Ozdemir A; Department of Pediatric Pulmonology, Mersin City Research & Training Hospital, Mersin, Turkey., Kose M; Department of Pediatric Pulmonology, Faculty of Medicine, Erciyes University, Kayseri, Turkey., Hangul M; Department of Pediatric Pulmonology, Faculty of Medicine, Erciyes University, Kayseri, Turkey., Emiralioglu N; Department of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara, Turkey., Tugcu G; Department of Pediatric Pulmonology, Ankara Children's Hematology Oncology Training and Research Hospital, Ankara, Turkey., Yuksel H; Department of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine, Celal Bayar University, Manisa, Turkey., Yılmaz O; Department of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine, Celal Bayar University, Manisa, Turkey., Orhan F; Department of Pediatric Allergy, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey., Gayretli Aydın ZG; Department of Pediatric Infectious Disease, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey., Topal E; Department of Pediatric Allergy, Faculty of Medicine, İnonu University, Malatya, Turkey., Tamay Z; Department of Pediatric Allergy and Immunology, İstanbul Facultyof Medicine, İstanbul University, İstanbul, Turkey., Suleyman A; Department of Pediatric Allergy and Immunology, İstanbul Facultyof Medicine, İstanbul University, İstanbul, Turkey., Can D; Department of Pediatric Pulmonology, Faculty of Medicine, Balıkesir University, Balıkesir, Turkey., Bal CM; Department of Pediatric Pulmonology, Doctor Lufti Kirdar Kartal Training and Research Hospital, ıstanbul, Turkey., Caltepe G; Department of Pediatric Gastroenterology, Faculty of Medicine, Ondokuz Mayıs University, Samsun, Turkey., Ozcelik U; Department of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
Jazyk: angličtina
Zdroj: Pediatric pulmonology [Pediatr Pulmonol] 2020 Aug; Vol. 55 (8), pp. 2011-2016. Date of Electronic Publication: 2020 May 04.
DOI: 10.1002/ppul.24805
Abstrakt: 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 FEV 1 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.
(© 2020 Wiley Periodicals, Inc.)
Databáze: MEDLINE
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