Cystic fibrosis in Turkey: First data from the national registry

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dc.authoridSismanlar Eyuboglu, Tugba/0000-0001-7284-4999
dc.authoridOrhan, Fazil/0000-0002-4850-932X
dc.authoridEmiralioglu, Nagehan/0000-0002-1405-8401
dc.authoridTugcu, Gokcen Dilsa/0000-0002-9804-1200
dc.authoridDogru, Deniz/0000-0001-9931-9473
dc.authoridtopal, erdem/0000-0002-4439-2689
dc.authorid, erdembasaran/0000-0002-9092-6936
dc.authorwosidÇokuğraş, Haluk C/D-2598-2019
dc.authorwosidSismanlar Eyuboglu, Tugba/AAK-5467-2020
dc.authorwosidOrhan, Fazil/AAL-6641-2021
dc.authorwosidEmiralioglu, Nagehan/S-7221-2016
dc.authorwosidTugcu, Gokcen Dilsa/ABG-5531-2021
dc.authorwosidTamay, Zeynep/AAE-5668-2020
dc.authorwosidDogru, Deniz/AAG-8365-2019
dc.contributor.authorDogru, Deniz
dc.contributor.authorCakir, Erkan
dc.contributor.authorSismanlar, Tugba
dc.contributor.authorCobanoglu, Nazan
dc.contributor.authorPekcan, Sevgi
dc.contributor.authorCinel, Guzin
dc.contributor.authorYalcin, Ebru
dc.date.accessioned2024-08-04T20:46:55Z
dc.date.available2024-08-04T20:46:55Z
dc.date.issued2020
dc.departmentİnönü Üniversitesien_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.doi10.1002/ppul.24561
dc.identifier.endpage548en_US
dc.identifier.issn8755-6863
dc.identifier.issn1099-0496
dc.identifier.issue2en_US
dc.identifier.pmid31710166en_US
dc.identifier.scopus2-s2.0-85075130359en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage541en_US
dc.identifier.urihttps://doi.org/10.1002/ppul.24561
dc.identifier.urihttps://hdl.handle.net/11616/99051
dc.identifier.volume55en_US
dc.identifier.wosWOS:000495493500001en_US
dc.identifier.wosqualityQ2en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherWileyen_US
dc.relation.ispartofPediatric Pulmonologyen_US
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.typeArticleen_US

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