Cystic fibrosis in Turkey: First data from the national registry
| Autor: | Ayşen Bingöl, Zeynep Tamay, Cem Murat Bal, Haluk Cokugras, Sevgi Pekcan, Abdurrahman Erdem Başaran, Hasan Yuksel, Şenay Şaşihüseyinoğlu, Ayse Tana Aslan, Deniz Dogru, Ali Özdemir, Ayse Ayzit Kilinc, Güzin Cinel, Ugur Ozcelik, Ömür Ercan, Gönül Çaltepe, Şükrü Çekiç, Derya Ufuk Altintas, Gokcen Dilsa Tugcu, Nural Kiper, Nagehan Emiralioglu, Ozge Yilmaz, Erkan Cakir, Mehmet Köse, Tugba Sismanlar, Hakan Yazan, Nazan Cobanoglu, Ebru Yalcin, Fazil Orhan, Lina Muhammed Al Shadfan, Melih Hangül, Erdem Topal, Ozlem Keskin, Ayşe Süleyman, Zeynep Gökçe Gayretli Aydın, Demet Can, Velat Şen, Sevgi Bilgic Eltan, Nihat Sapan, Tugba Ramasli Gursoy, Hadice Selimoglu Sen |
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| Přispěvatelé: | Tıp Fakültesi, Ondokuz Mayıs Üniversitesi, ÇAKIR, Erkan, Çukurova Üniversitesi, Bursa Uludağ Üniversitesi/Tıp Fakültesi/Dahili Tıp Bilimleri/Çocuk Sağlığı ve Hastalıkları Bölümü., Sapan, Nihat, Çekiç, Şükrü, L-1933-2017, Division of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara, Turkey, Division of Pediatric Pulmonology, Faculty of Medicine, Bezmialem University, Istanbul, Turkey, Division of Pediatric Pulmonology, Dr Sami Ulus Maternity and Children Training and Research Hospital, Ankara, Turkey, Division of Pediatric Pulmonology, Faculty of Medicine, Ankara University, Ankara, Turkey, Division of Pediatric Pulmonology, Meram Medicine Faculty, Necmettin Erbakan University, Konya, Turkey, Division of Pediatric Pulmonology, Ankara Children's Hematology Oncology Training and Research Hospital, Ankara, Turkey, Division of Pediatric Pulmonology, Faculty of Medicine, Dicle University, Diyarbakır, Turkey, Department of Pulmonology, Faculty of Medicine, Dicle University, Diyarbakır, Turkey, Division of Pediatric Allergy, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey, Division of Pediatric Allergy and Immunology, Faculty of Medicine, Çukurova University, Adana, Turkey, Division of Pediatric Allergy and Immunology, Faculty of Medicine, Bursa Uludağ University, Bursa, Turkey, Division of Pediatric Allergy and Pulmonology, Cerrahpaşa Medicine Faculty, Istanbul University, Istanbul, Turkey, Division of Pediatric Pulmonology, Faculty of Medicine, Gazi University, Ankara, Turkey, Division of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine, Akdeniz University, Antalya, Turkey, Division of Pediatric Pulmonology, Mersin City Training and Research Hospital, Mersin, Turkey, Division of Pediatric Pulmonology, Faculty of Medicine, Erciyes University, Kayseri, Turkey, Division of Pediatric Pulmonology, Gaziantep Cengiz Gökçek Maternity and Children Hospital, Gaziantep, Turkey, Division of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine, Celal Bayar University, Manisa, Turkey, Division of Pediatric Allergy, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey, Division of Pediatric Infectious Disease, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey, Division of Pediatric Allergy, Faculty of Medicine, İnönü University, Malatya, Turkey, Division of Pediatric Allergy, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey, Division of Pediatric Pulmonology, Faculty of Medicine, Balıkesir University, Balıkesir, Turkey, Division of Pediatric Pulmonology, Atatürk Regional Training and Research Hospital, Erzurum, Turkey, Division of Pediatric Gastroenterology, Hepatology and Nutrition, Faculty of Medicine, Ondokuz Mayıs University, Samsun, Turkey |
| Jazyk: | angličtina |
| Rok vydání: | 2019 |
| Předmět: |
Newborn screening
Male Hemoptysis Turkey Turkish Azithromycin Pediatrics Cystic fibrosis cystic fibrosis Diabetes mellitus 0302 clinical medicine Child death Corticosteroid Insulin Medicine Child Gastrostomy Patient registry National Registry Chronic liver disease Pneumothorax Dornase alfa Gastroesophageal reflux Patient Registry Ursodeoxycholic acid Child Preschool Bisphosphonic acid derivative Forced expiratory volume Human Pulmonary and Respiratory Medicine Health care facility medicine.medical_specialty Genotype First data from the national registry.- Pediatric pulmonology cilt.55 ss.541-548 2020 [Dogru D. Çakır E. Şişmanlar T. Çobanoğlu N. Pekcan S. Cinel G. Yalçın E. Kiper N. Şen V. S Ş. et al. -Cystic fibrosis in Turkey] Meconium Ileus Major clinical study national registry Proton pump inhibitor Article 03 medical and health sciences Age Neonatal Screening Allergic bronchopulmonary aspergillosis Genetics Humans Access to information Sinusitis Genotyping Demography Internet Infant Missing data medicine.disease Gene frequency Malignant neoplasm 030228 respiratory system Pancreas enzyme Mutation Osteoporosis School child Bronchodilating agent Complication Cystic Fibrosis Cftr gene Cystic Fibrosis Transmembrane Conductance Regulator Turkey (republic) patient registry Information processing Turkey (bird) Mannitol Registries Meconium ileus Priority journal Antibiotic agent Register Middle Aged Bacterial colonization Pseudo-bartter-syndrome Pseudomonas aeruginosa Tobramycin Pseudomonas infection Female Mutations Sweat test Adult Adolescent Sodium chloride Patient care Respiratory system Chloride Vitamin 030225 pediatrics Gene mutation Disease registry Colistin business.industry Infant Newborn Bartter Syndrome Alkalosis Newborn Bartter syndrome Lung function Preschool child Pediatrics Perinatology and Child Health Calcium Data analysis software National registry Prediction business |
| Popis: | Can, Demet (Balikesir Author) 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. |
| Databáze: | OpenAIRE |
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