Current Status of Cystic Fibrosis in Türkiye: Data from the National Registry.
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Ademhan Tural Dilber, Şişmanlar Eyüboğlu Tuğba, Cinel Güzin, Çakır Erkan, Pekcan Sevgi, Özçelik Uğur, Emiralioğlu Ordukaya Nagehan, Yalçın Ebru, Nayır Büyükşahin Halime, Serbes Mahir, Altıntaş Derya Ufuk, Hizal Mina, İnal Gaye, Keskin Özlem, Eryılmaz Polat Sanem, Savaş Suat, Ayman Fatma Nur, Erdal Meltem, Demir Havva İpek, Yüksel Hasan, Polat Uluğ Demet, Özdemir Ali, Tuğcu Gökçen Dilşa, Demirdöğen Ezgi, Korkmaz Merve, Canıtez Yakup, Kekeç Handan, Tana Aslan Ayşe, Harmancı Koray, Şen Velat, Selimoğlu Şen Hadice, Ayzıt Kılınç Ayşe, Kılıç Başkan Azer, Öztosun Berrak, Damadoğlu Ebru, Karcıoğlu Oğuz, Kartal Öztürk Gökçen, Gülen Figen, Zirek Fazılcan, Ocak Ece, Baş İkizoğlu Nilay, Çakır Eylül Pınar, Topal Erdem, Kılıç Mehmet, Çaltepe Gönül, Asfuroğlu Pelin, Çobanoğlu Nazan, Hangül Melih, Dağdelen Erdi Çağrı, Köse Mehmet, Ersoy Ali, Bingöl Ayşen, Başaran Abdurrahman Erdem, Özsezen Beste, Ramaslı Gürsoy Tuğba, Korkmaz Pervin, Gayretli Aydın Zeynep Gökçe, Doğru Deniz
Аннотация
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.