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

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...

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Veröffentlicht in:Pediatric pulmonology 2020-08, Vol.55 (8), p.2011-2016
Hauptverfasser: Sismanlar Eyuboglu, Tugba, Dogru, Deniz, Çakır, Erkan, Cobanoglu, Nazan, Pekcan, Sevgi, Cinel, Guzin, Yalçın, Ebru, Kiper, Nural, Sen, Velat, Selimoglu Sen, Hadice, Ercan, Omur, Keskin, Ozlem, Bilgic Eltan, Sevgi, Alshadfan, Lina, Yazan, Hakan, Altıntas, Derya Ufuk, Sasihuseyinoglu, Ayse Senay, Sapan, Nihat, Cekic, Sükrü, Cokugraş, Haluk, Kılınc, Ayşe Ayzıt, Ramaslı Gursoy, Tugba, Aslan, Ayse Tana, Bingol, Aysen, Başaran, Abdurrahman Erdem, Ozdemir, Ali, Kose, Mehmet, Hangul, Melih, Emiralioglu, Nagehan, Tugcu, Gokçen, Yuksel, Hasan, Yılmaz, Ozge, Orhan, Fazıl, Gayretli Aydın, Zeynep Gokce, Topal, Erdem, Tamay, Zeynep, Suleyman, Ayse, Can, Demet, Bal, Cem Murat, Caltepe, Gönül, Ozcelik, Uğur
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Sprache:eng
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Zusammenfassung: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 
ISSN:8755-6863
1099-0496
DOI:10.1002/ppul.24805