Cystic fibrosis in Turkey: First data from the national registry

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dc.contributor.authorDoğru, Deniz
dc.contributor.authorÇakır, Erkan
dc.contributor.authorŞişmanlar, Tuğba
dc.contributor.authorÇobanoğlu, Nazan
dc.contributor.authorPekcan, Sevgi
dc.contributor.authorCinel, Güzin
dc.contributor.authorYalçın, Ebru
dc.contributor.authorŞen, Velat
dc.contributor.authorŞen, Hadice S.
dc.date.accessioned2024-04-24T15:59:31Z
dc.date.available2024-04-24T15:59:31Z
dc.date.issued2020
dc.departmentDicle Üniversitesi, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümü, Çocuk Hastalıkları Ana Bilim Dalıen_US
dc.description.abstractBackground 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.en_US
dc.identifier.citationDoğru, D., Çakır, E., Şişmanlar, T., Çobanoğlu, N., Pekcan, S., Cinel, G. ve diğerleri. (2020). Cystic fibrosis in Turkey: First data from the national registry. Pediatric Pulmonology, 55(2), 541-548.
dc.identifier.doi10.1002/ppul.24561
dc.identifier.endpage548en_US
dc.identifier.issn8755-6863
dc.identifier.issn1099-0496
dc.identifier.issue2en_US
dc.identifier.pmid31710166
dc.identifier.scopus2-s2.0-85075130359
dc.identifier.scopusqualityQ1
dc.identifier.startpage541en_US
dc.identifier.urihttps://doi.org/10.1002/ppul.24561
dc.identifier.urihttps://hdl.handle.net/11468/14117
dc.identifier.urihttps://onlinelibrary.wiley.com/doi/10.1002/ppul.24561
dc.identifier.volume55en_US
dc.identifier.wosWOS:000495493500001
dc.identifier.wosqualityQ2
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.institutionauthorŞen, Velat
dc.institutionauthorŞen, Hadice S.
dc.language.isoenen_US
dc.publisherWileyen_US
dc.relation.ispartofPediatric Pulmonology
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectCystic fibrosisen_US
dc.subjectNational registryen_US
dc.subjectPatient registryen_US
dc.titleCystic fibrosis in Turkey: First data from the national registryen_US
dc.titleCystic fibrosis in Turkey: First data from the national registry
dc.typeArticleen_US

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