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