Cystic fibrosis in Turkey: First data from the national registry.

Autor: Dogru D; Division of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara, Turkey., Çakır E; Division of Pediatric Pulmonology, Faculty of Medicine, Bezmialem University, Istanbul, Turkey., Şişmanlar T; Division of Pediatric Pulmonology, Dr Sami Ulus Maternity and Children Training and Research Hospital, Ankara, Turkey., Çobanoğlu N; Division of Pediatric Pulmonology, Faculty of Medicine, Ankara University, Ankara, Turkey., Pekcan S; Division of Pediatric Pulmonology, Meram Medicine Faculty, Necmettin Erbakan University, Konya, Turkey., Cinel G; Division of Pediatric Pulmonology, Ankara Children's Hematology Oncology Training and Research Hospital, Ankara, Turkey., Yalçın E; Division of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara, Turkey., Kiper N; Division of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara, Turkey., Şen V; Division of Pediatric Pulmonology, Faculty of Medicine, Dicle University, Diyarbakır, Turkey., S Şen H; Department of Pulmonology, Faculty of Medicine, Dicle University, Diyarbakır, Turkey., Ercan Ö; Division of Pediatric Pulmonology, Meram Medicine Faculty, Necmettin Erbakan University, Konya, Turkey., Keskin Ö; Division of Pediatric Allergy, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey., B Eltan S; Division of Pediatric Allergy, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey., Al Shadfan LM; Division of Pediatric Pulmonology, Faculty of Medicine, Bezmialem University, Istanbul, Turkey., Yazan H; Division of Pediatric Pulmonology, Faculty of Medicine, Bezmialem University, Istanbul, Turkey., Altıntaş DU; Division of Pediatric Allergy and Immunology, Faculty of Medicine, Çukurova University, Adana, Turkey., Şaşihüseyinoğlu Ş; Division of Pediatric Allergy and Immunology, Faculty of Medicine, Çukurova University, Adana, Turkey., Sapan N; Division of Pediatric Allergy and Immunology, Faculty of Medicine, Bursa Uludağ University, Bursa, Turkey., Çekiç Ş; Division of Pediatric Allergy and Immunology, Faculty of Medicine, Bursa Uludağ University, Bursa, Turkey., Çokuğraş H; Division of Pediatric Allergy and Pulmonology, Cerrahpaşa Medicine Faculty, Istanbul University, Istanbul, Turkey., A Kılınç A; Division of Pediatric Allergy and Pulmonology, Cerrahpaşa Medicine Faculty, Istanbul University, Istanbul, Turkey., R Gürsoy T; Division of Pediatric Pulmonology, Faculty of Medicine, Gazi University, Ankara, Turkey., Aslan AT; Division of Pediatric Pulmonology, Faculty of Medicine, Gazi University, Ankara, Turkey., Bingöl A; Division of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine, Akdeniz University, Antalya, Turkey., Başaran AE; Division of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine, Akdeniz University, Antalya, Turkey., Özdemir A; Division of Pediatric Pulmonology, Mersin City Training and Research Hospital, Mersin, Turkey., Köse M; Division of Pediatric Pulmonology, Faculty of Medicine, Erciyes University, Kayseri, Turkey., Hangül M; Division of Pediatric Pulmonology, Faculty of Medicine, Erciyes University, Kayseri, Turkey., Emiralioğlu N; Division of Pediatric Pulmonology, Gaziantep Cengiz Gökçek Maternity and Children Hospital, Gaziantep, Turkey., Tuğcu G; Division of Pediatric Pulmonology, Ankara Children's Hematology Oncology Training and Research Hospital, Ankara, Turkey., Yüksel H; Division of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine, Celal Bayar University, Manisa, Turkey., Yılmaz Ö; Division of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine, Celal Bayar University, Manisa, Turkey., Orhan F; Division of Pediatric Allergy, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey., Gayretli Aydın ZG; Division of Pediatric Infectious Disease, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey., Topal E; Division of Pediatric Allergy, Faculty of Medicine, İnönü University, Malatya, Turkey., Tamay Z; Division of Pediatric Allergy, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey., Süleyman A; Division of Pediatric Allergy, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey., Can D; Division of Pediatric Pulmonology, Faculty of Medicine, Balıkesir University, Balıkesir, Turkey., Bal CM; Division of Pediatric Pulmonology, Atatürk Regional Training and Research Hospital, Erzurum, Turkey., Çaltepe G; Division of Pediatric Gastroenterology, Hepatology and Nutrition, Faculty of Medicine, Ondokuz Mayıs University, Samsun, Turkey., Özçelik U; Division of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
Jazyk: angličtina
Zdroj: Pediatric pulmonology [Pediatr Pulmonol] 2020 Feb; Vol. 55 (2), pp. 541-548. Date of Electronic Publication: 2019 Nov 11.
DOI: 10.1002/ppul.24561
Abstrakt: Background: Cystic fibrosis (CF) care has been implemented in Turkey for a long time; however, there had been no patient registry. For this purpose, the Turkish National CF Registry was established. We present the first results of registry using data collected in 2017.
Methods: The data were collected using a data-entry software system, which was accessed from the internet. Demographic and annually recorded data consisted of 15 and 79 variables, respectively.
Results: There were 1170 patients registered from 23 centers; the estimated coverage rate was 30%. The median age at diagnosis was 1.7 years (median current age: 7.3 years); 51 (4.6%) patients were aged over 18 years. Among 293 patients who were under 3 years of age, 240 patients (81.9%) were diagnosed through newborn screening. Meconium ileus was detected in 65 (5.5%) patients. Genotyping was performed in 978 (87.4%) patients and 246 (25.2%) patients' mutations were unidentified. The most common mutation was deltaF508 with an allelic frequency of 28%, followed by N1303K (4.9%). The median FEV1% predicted was 86. Chronic colonization with Pseudomonas aeruginosa was seen in 245 patients. The most common complication was pseudo-Bartter syndrome in 120 patients. The median age of death was 13.5 years in a total of 15 patients.
Conclusions: Low coverage rate, lack of genotyping, unidentified mutations, and missing data of lung functions are some of our greatest challenges. Including data of all centers and reducing missing data will provide more accurate data and help to improve the CF care in Turkey in the future.
(© 2019 Wiley Periodicals, Inc.)
Databáze: MEDLINE
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