{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T21:15:41Z","timestamp":1793049341283,"version":"4.5.0"},"reference-count":27,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2019,11,11]],"date-time":"2019-11-11T00:00:00Z","timestamp":1573430400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2020,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The data were collected using a data\u2010entry software system, which was accessed from the internet. Demographic and annually recorded data consisted of 15 and 79 variables, respectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>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 <jats:italic>Pseudomonas aeruginosa<\/jats:italic> was seen in 245 patients. The most common complication was pseudo\u2010Bartter syndrome in 120 patients. The median age of death was 13.5 years in a total of 15 patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.24561","type":"journal-article","created":{"date-parts":[[2019,11,11]],"date-time":"2019-11-11T12:54:03Z","timestamp":1573476843000},"page":"541-548","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":48,"title":["Cystic fibrosis in Turkey: First data from the national registry"],"prefix":"10.1002","volume":"55","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9931-9473","authenticated-orcid":false,"given":"Deniz","family":"Dogru","sequence":"first","affiliation":[{"name":"Division of Pediatric Pulmonology Faculty of Medicine, Hacettepe University Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1438-7854","authenticated-orcid":false,"given":"Erkan","family":"\u00c7ak\u0131r","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology Faculty of Medicine, Bezmialem University Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tu\u011fba","family":"\u015ei\u015fmanlar","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology Dr Sami Ulus Maternity and Children Training and Research Hospital Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nazan","family":"\u00c7obano\u011flu","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, Faculty of Medicine Ankara University Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sevgi","family":"Pekcan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, Meram Medicine Faculty Necmettin Erbakan University Konya Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00fczin","family":"Cinel","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology Ankara Children's Hematology Oncology Training and Research Hospital Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ebru","family":"Yal\u00e7\u0131n","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology Faculty of Medicine, Hacettepe University Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nural","family":"Kiper","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology Faculty of Medicine, Hacettepe University Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Velat","family":"\u015een","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, Faculty of Medicine Dicle University Diyarbak\u0131r Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hadice","family":"S. \u015een","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, Faculty of Medicine Dicle University Diyarbak\u0131r Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00d6m\u00fcr","family":"Ercan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, Meram Medicine Faculty Necmettin Erbakan University Konya Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00d6zlem","family":"Keskin","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy, Faculty of Medicine Gaziantep University Gaziantep Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sevgi","family":"B. Eltan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy, Faculty of Medicine Gaziantep University Gaziantep Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lina M.","family":"Al Shadfan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology Faculty of Medicine, Bezmialem University Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hakan","family":"Yazan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology Faculty of Medicine, Bezmialem University Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Derya U.","family":"Alt\u0131nta\u015f","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy and Immunology, Faculty of Medicine \u00c7ukurova University Adana Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4085-0256","authenticated-orcid":false,"given":"\u015eenay","family":"\u015ea\u015fih\u00fcseyino\u011flu","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy and Immunology, Faculty of Medicine \u00c7ukurova University Adana Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nihat","family":"Sapan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy and Immunology, Faculty of Medicine Bursa Uluda\u011f University Bursa Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u015e\u00fckr\u00fc","family":"\u00c7eki\u00e7","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy and Immunology, Faculty of Medicine Bursa Uluda\u011f University Bursa Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haluk","family":"\u00c7oku\u011fra\u015f","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy and Pulmonology, Cerrahpa\u015fa Medicine Faculty Istanbul University Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ay\u015fe","family":"A. K\u0131l\u0131n\u00e7","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy and Pulmonology, Cerrahpa\u015fa Medicine Faculty Istanbul University Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tu\u011fba","family":"R. G\u00fcrsoy","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, Faculty of Medicine Gazi University Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ay\u015fe T.","family":"Aslan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, Faculty of Medicine Gazi University Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ay\u015fen","family":"Bing\u00f6l","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine Akdeniz University Antalya Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9092-6936","authenticated-orcid":false,"given":"Abdurrahman E.","family":"Ba\u015faran","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine Akdeniz University Antalya Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7340-0409","authenticated-orcid":false,"given":"Ali","family":"\u00d6zdemir","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology Mersin City Training and Research Hospital Mersin Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3003-918X","authenticated-orcid":false,"given":"Mehmet","family":"K\u00f6se","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, Faculty of Medicine Erciyes University Kayseri Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melih","family":"Hang\u00fcl","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, Faculty of Medicine Erciyes University Kayseri Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1405-8401","authenticated-orcid":false,"given":"Nagehan","family":"Emiralio\u011flu","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology Gaziantep Cengiz G\u00f6k\u00e7ek Maternity and Children Hospital Gaziantep Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00f6k\u00e7en","family":"Tu\u011fcu","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology Ankara Children's Hematology Oncology Training and Research Hospital Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hasan","family":"Y\u00fcksel","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine Celal Bayar University Manisa Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6051-5020","authenticated-orcid":false,"given":"\u00d6zge","family":"Y\u0131lmaz","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine Celal Bayar University Manisa Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Faz\u0131l","family":"Orhan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy, Faculty of Medicine Karadeniz Technical University Trabzon Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zeynep G.","family":"Gayretli Ayd\u0131n","sequence":"additional","affiliation":[{"name":"Division of Pediatric Infectious Disease, Faculty of Medicine Karadeniz Technical University Trabzon Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erdem","family":"Topal","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy, Faculty of Medicine \u0130n\u00f6n\u00fc University Malatya Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zeynep","family":"Tamay","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy Istanbul Faculty of Medicine, Istanbul University Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ay\u015fe","family":"S\u00fcleyman","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy Istanbul Faculty of Medicine, Istanbul University Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Demet","family":"Can","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology, Faculty of Medicine Bal\u0131kesir University Bal\u0131kesir Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cem M.","family":"Bal","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology Atat\u00fcrk Regional Training and Research Hospital Erzurum Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00f6n\u00fcl","family":"\u00c7altepe","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology and Nutrition, Faculty of Medicine Ondokuz May\u0131s University Samsun Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"U\u011fur","family":"\u00d6z\u00e7elik","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology Faculty of Medicine, Hacettepe University Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,11,11]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23790"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2017.11.013"},{"key":"e_1_2_6_4_1","first-page":"165","article-title":"Newborn screening for cystic fibrosis with the chloride electrode and neutron activation analysis","volume":"28","author":"G\u00fcrson CT","year":"1973","journal-title":"Helv Paediatr Acta"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.4274\/balkanmedj.galenos.2018.2018.1332"},{"key":"e_1_2_6_6_1","unstructured":"ECFS Patient Registry.https:\/\/www.ecfs.eu\/ecfspr\/. Accessed August 14 2019."},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00034805"},{"key":"e_1_2_6_8_1","first-page":"343","article-title":"Significance of contemporary methods of lung function testing for the detection of airway obstruction in children and adolescents","volume":"149","author":"Zapletal A","year":"1977","journal-title":"Z Erkr Atmungsorgane"},{"key":"e_1_2_6_9_1","unstructured":"Centers for Disease Control and Prevention. A SAS program for the 2000 CDC growth charts (ages 0 to <20 years).https:\/\/www.cdc.gov\/nccdphp\/dnpao\/growthcharts\/resources\/sas.htm\/. Accessed August 14 2019."},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clnu.2016.03.004"},{"key":"e_1_2_6_11_1","unstructured":"Turkish Statistical Institute.http:\/\/www.turkstat.gov.tr\/. Accessed August 14 2019."},{"key":"e_1_2_6_12_1","unstructured":"Zolin A Orenti A Naehrlich L et al.ECFSPR Annual Report 2017. 2019."},{"issue":"62","key":"e_1_2_6_13_1","first-page":"PA621","article-title":"Newborn screening for CF: first three years experience in Hacettepe University","volume":"52","author":"Gharibzadeh Hizal M","year":"2018","journal-title":"Eur Respir J"},{"key":"e_1_2_6_14_1","first-page":"781","volume-title":"Kendig's Disorders of the Respiratory Tract in Children","author":"Ahmed NN","year":"2019"},{"key":"e_1_2_6_15_1","unstructured":"Cystic Fibrosis Foundation.Cystic Fibrosis Foundation Patient Registry 2015 Annual Data Report. Bethesda MD2016."},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004390050683"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1089\/gte.2007.0046"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.3109\/15513819409024278"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02428283"},{"key":"e_1_2_6_20_1","first-page":"144","article-title":"Molecular genetic analysis of Turkish cystic fibrosis patients","volume":"36","author":"K\u00f6pr\u00fcbasi FF","year":"1993","journal-title":"Ann Genet"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1159\/000154281"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24299"},{"key":"e_1_2_6_23_1","first-page":"407","article-title":"Sequencing of the CFTR gene in selected Turkish patients with cystic fibrosis","volume":"67","author":"\u00c7ankaya T","year":"2015","journal-title":"Minerva Pediatr"},{"issue":"1","key":"e_1_2_6_24_1","first-page":"19","article-title":"Episodic seasonal pseudo\u2010Bartter syndrome in cystic fibrosis","volume":"15","author":"Kintu B","year":"2014","journal-title":"Paediatr Respir Rev"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-007-0413-3"},{"key":"e_1_2_6_26_1","doi-asserted-by":"publisher","DOI":"10.1179\/146532805X45719"},{"key":"e_1_2_6_27_1","doi-asserted-by":"publisher","DOI":"10.4103\/1319-2442.109579"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-013-2712-4"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fppul.24561","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.24561","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ppul.24561","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.24561","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,6]],"date-time":"2023-09-06T22:17:54Z","timestamp":1694038674000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.24561"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,11,11]]},"references-count":27,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2020,2]]}},"alternative-id":["10.1002\/ppul.24561"],"URL":"https:\/\/doi.org\/10.1002\/ppul.24561","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,11,11]]},"assertion":[{"value":"2019-07-19","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-10-09","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-11-11","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}