Current Status of Cystic Fibrosis in Türkiye: Data from the National Registry
Makaleler > Tam Makale · 2025
Yazarlar: DİLBER ADEMHAN TURAL, TUĞBA ŞİŞMANLAR EYÜBOĞLU, GÜZİN CİNEL, ERKAN ÇAKIR, SEVGİ PEKCAN, HAYRİYE UĞUR ÖZÇELİK, NAGEHAN EMİRALİOĞLU, ELMAS EBRU YALÇIN, HALİME NAYIR BÜYÜKŞAHİN, MAHİR SERBES, DERYA UFUK ALTINTAŞ, MİNA HIZAL, GAYE İNAL, ÖZLEM KESKİN, SANEM ERYILMAZ POLAT, Suat Savaş, FATMA NUR AYMAN, Meltem Erdal, HAVVA İPEK DEMİR, HASAN YÜKSEL, Demet Polat Uluğ, Ali Özdemir, GÖKÇEN DİLŞA TUĞCU, EZGİ DEMİRDÖĞEN, Merve KORKMAZ, YAKUP CANITEZ, HANDAN KEKEÇ, AYŞE TANA ASLAN, KORAY HARMANCI, VELAT ŞEN, HADİCE SELİMOĞLU ŞEN, AYŞE AYZIT KILINÇ, AZER KILIÇ BAŞKAN, Berrak Öztosun, EBRU DAMADOĞLU, OĞUZ KARCIOĞLU, Gökçen KARTAL ÖZTÜRK, Figen GÜLEN, FAZILCAN ZİREK, Ece OCAK, NİLAY BAŞ İKİZOĞLU, EYLÜL PINAR ÇAKIR, ERDEM TOPAL, MEHMET KILIÇ, GÖNÜL ÇALTEPE, PELİN ASFUROĞLU, FATMA NAZAN ÇOBANOĞLU, MELİH HANGÜL, Erdi Çağrı Dağdelen, MEHMET KÖSE, ALİ ERSOY, AYŞEN BİNGÖL, ABDURRAHMAN ERDEM BAŞARAN, BESTE ÖZSEZEN, TUĞBA RAMASLI GÜRSOY, Pervin KORKMAZ, ZEYNEP GÖKÇE GAYRETLİ AYDIN, DENİZ DOĞRU ERSÖZ
Thoracic Research and Practice, Diğer Kurumların Hakemli Dergileri, Uluslararası, Scopus, 2025, cilt 26, ss 238-247
DOI: 10.4274/thoracrespract.2025.2025-1-11
Özet
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 treatmentrelated 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.