Dilber Ademhan Tural, Tuğba Şişmanlar Eyüboğlu, Güzin Cinel, Erkan Çakır, Sevgi Pekcan, Uğur Özçelik, Nagehan Emiralioğlu Ordukaya, Ebru Yalçın, Halime Nayır Büyükşahin, Mahir Serbes, Derya Ufuk Altıntaş, Mina Hizal, Gaye İnal, Özlem Keskin, Sanem Eryılmaz Polat, Suat Savaş, Fatma Nur Ayman, Meltem Erdal, Havva İpek Demir, Hasan Yüksel, Demet Polat Uluğ, Ali Özdemir, Gökçen Dilşa Tuğcu, Ezgi Demirdöğen, Merve Korkmaz, Yakup Canıtez, Handan Kekeç, Ayşe Tana Aslan, Koray Harmancı, Velat Şen, Hadice Selimoğlu Şen, Ayşe Ayzıt Kılınç, Azer Kılıç Başkan, Berrak Öztosun, Ebru Damadoğlu, Oğuz Karcıoğlu, Gökçen Kartal Öztürk, Figen Gülen, Fazılcan Zirek, Ece Ocak, Nilay Baş İkizoğlu, Eylül Pınar Çakır, Erdem Topal, Mehmet Kılıç, Gönül Çaltepe, Pelin Asfuroğlu, Nazan Çobanoğlu, Melih Hangül, Erdi Çağrı Dağdelen, Mehmet Köse, Ali Ersoy, Ayşen Bingöl, Abdurrahman Erdem Başaran, Beste Özsezen, Tuğba Ramaslı Gürsoy, Pervin Korkmaz, Zeynep Gökçe Gayretli Aydın, Deniz Doğru
{"title":"Current Status of Cystic Fibrosis in Türkiye: Data from the National Registry.","authors":"Dilber Ademhan Tural, Tuğba Şişmanlar Eyüboğlu, Güzin Cinel, Erkan Çakır, Sevgi Pekcan, Uğur Özçelik, Nagehan Emiralioğlu Ordukaya, Ebru Yalçın, Halime Nayır Büyükşahin, Mahir Serbes, Derya Ufuk Altıntaş, Mina Hizal, Gaye İnal, Özlem Keskin, Sanem Eryılmaz Polat, Suat Savaş, Fatma Nur Ayman, Meltem Erdal, Havva İpek Demir, Hasan Yüksel, Demet Polat Uluğ, Ali Özdemir, Gökçen Dilşa Tuğcu, Ezgi Demirdöğen, Merve Korkmaz, Yakup Canıtez, Handan Kekeç, Ayşe Tana Aslan, Koray Harmancı, Velat Şen, Hadice Selimoğlu Şen, Ayşe Ayzıt Kılınç, Azer Kılıç Başkan, Berrak Öztosun, Ebru Damadoğlu, Oğuz Karcıoğlu, Gökçen Kartal Öztürk, Figen Gülen, Fazılcan Zirek, Ece Ocak, Nilay Baş İkizoğlu, Eylül Pınar Çakır, Erdem Topal, Mehmet Kılıç, Gönül Çaltepe, Pelin Asfuroğlu, Nazan Çobanoğlu, Melih Hangül, Erdi Çağrı Dağdelen, Mehmet Köse, Ali Ersoy, Ayşen Bingöl, Abdurrahman Erdem Başaran, Beste Özsezen, Tuğba Ramaslı Gürsoy, Pervin Korkmaz, Zeynep Gökçe Gayretli Aydın, Deniz Doğru","doi":"10.4274/ThoracResPract.2025.2025-1-11","DOIUrl":null,"url":null,"abstract":"<p><strong>Objective: </strong>The Cystic Fibrosis Registry of Türkiye (CFRT) was established by the Turkish Pediatric Respiratory Diseases and Cystic Fibrosis Society and has provided detailed information on demographic, clinical, genetic, and treatment-related aspects of cystic fibrosis (CF) patients since 2017. We aimed to describe the current status of CF in Türkiye using CFRT's 2023 annual data.</p><p><strong>Material and methods: </strong>Demographic, clinical, and treatment data were taken from CFRT's 2023 record.</p><p><strong>Results: </strong>In 2023, 2,258 patients from 34 centers were recorded. The median age of patients was 9.1 years, and 46.9% were female, with a median age at diagnosis of 0.3 years. Only 14.9% of the patients were older than 18 years. Genetic analyses were completed in 97.3% of patients. The most common variant, F508del, had a total variant frequency of 22.1%. The median percent predicted FEV1 and FVC were 88.0 and 94.0 in those aged 6-17 years 71.0 and 84.0 in those aged ≥18 years, respectively. The median values of body mass index z-scores were -0.5, and -0.5 for patients 2-18 and older than 18 years, respectively. Chronic colonization with <i>Pseudomonas aeruginosa</i> was present in 17.2% of the patients. Most patients used inhaled recombinant human DNase (87.1%) and oral pancreatic enzyme replacement treatment (83.0%). CF transmembrane conductance regulator (CFTR) modulators were used by 15.9% of patients. Over the year, 24 patients died, with a median age at death of 13.3 years.</p><p><strong>Conclusion: </strong>The CFRT report provides a valuable resource showing the clinical and laboratory data of patients with CF in the country.</p>","PeriodicalId":75221,"journal":{"name":"Thoracic research and practice","volume":" ","pages":"238-247"},"PeriodicalIF":0.6000,"publicationDate":"2025-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12365561/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Thoracic research and practice","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4274/ThoracResPract.2025.2025-1-11","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/8/4 0:00:00","PubModel":"Epub","JCR":"0","JCRName":"RESPIRATORY SYSTEM","Score":null,"Total":0}
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Abstract
Objective: The Cystic Fibrosis Registry of Türkiye (CFRT) was established by the Turkish Pediatric Respiratory Diseases and Cystic Fibrosis Society and has provided detailed information on demographic, clinical, genetic, and treatment-related aspects of cystic fibrosis (CF) patients since 2017. We aimed to describe the current status of CF in Türkiye using CFRT's 2023 annual data.
Material and methods: Demographic, clinical, and treatment data were taken from CFRT's 2023 record.
Results: In 2023, 2,258 patients from 34 centers were recorded. The median age of patients was 9.1 years, and 46.9% were female, with a median age at diagnosis of 0.3 years. Only 14.9% of the patients were older than 18 years. Genetic analyses were completed in 97.3% of patients. The most common variant, F508del, had a total variant frequency of 22.1%. The median percent predicted FEV1 and FVC were 88.0 and 94.0 in those aged 6-17 years 71.0 and 84.0 in those aged ≥18 years, respectively. The median values of body mass index z-scores were -0.5, and -0.5 for patients 2-18 and older than 18 years, respectively. Chronic colonization with Pseudomonas aeruginosa was present in 17.2% of the patients. Most patients used inhaled recombinant human DNase (87.1%) and oral pancreatic enzyme replacement treatment (83.0%). CF transmembrane conductance regulator (CFTR) modulators were used by 15.9% of patients. Over the year, 24 patients died, with a median age at death of 13.3 years.
Conclusion: The CFRT report provides a valuable resource showing the clinical and laboratory data of patients with CF in the country.
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