Browsing by Author "Emiralioglu, N"
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Item Children's ILDs survivors in adulthood: a European StudyManali, ED; Griese, M; Nathan, N; Uzunhan, Y; Borie, R; Michel, K; Schwerk, N; Fijolek, J; Radzikowska, E; Chua, F; Pabary, R; Mogulkoc, N; Mccarthy, C; Kallieri, M; Papaioannou, A; Kiper, N; Vasakova, MK; Lacina, L; Molina-MOLINA, M; Torrent-Vernetta, A; Tsiligiannis, T; Karadag, B; Kokosi, M; Renzoni, EA; Moorsel, CHV; Campo, I; Bendstrup, E; Prior, TS; Prasse, A; Bonella, F; Cottin, V; Diesler, R; Froidure, A; Kolilekas, L; Fotis, L; Douros, K; Kaditis, AG; Jeny, F; Chauveau, S; Nunes, H; Dahbia, A; Mariani, F; van der Vis, J; Groen, K; Eralp, EE; Gokdemir, Y; Kocakaya, D; Yildizeli, SO; Yildizeli, O; Yalçin, E; Emiralioglu, N; Buyuksahin, HN; O'Brien, H; Karcioglu, O; Can, D; Ezircan, A; Ozturk, GK; Ozturk, K; Ocal, N; Yuksel, H; Tongal, N; Safrankova, M; Kourtesi, K; Louvrier, C; Kannengiesser, C; Fabre, A; Legendre, M; Crestani, B; Pohunek, P; Bush, A; Papiris, SItem Geographical barriers to timely diagnosis of cystic fibrosis and anxiety level of parents during newborn screening in TurkeyGokdemir, Y; Eyuboglu, TS; Emiralioglu, N; Er, B; Sen, V; Pekcan, S; Ergenekon, AP; Hizal, MG; Eryilmaz, S; Kose, M; Hangul, M; Cakir, E; Cokugras, H; Kilinc, AA; Sasihuseyinoglu, AS; Altintas, DU; Gulen, F; Eski, A; Bingol, A; Ozdemir, A; Topal, E; Gursoy, TR; Girit, S; Ay, P; Yilmaz, OBackground Despite the availability of cystic fibrosis (CF) screening countrywide, diagnostic delay is still a crucial issue. The objectives of this study were to explore the stages of the NBS process, determine the risk factors associated with diagnostic delay and evaluate parent anxiety and experience throughout the process. Methods This is a multicenter cross-sectional study. A questionnaire was completed by parents of newborns diagnosed with CF via NBS in 17 centers. Socio-demographic characteristics, parent knowledge and experiences related to NBS, sweat test availability in the region of residence, and time to the definitive CF diagnosis were assessed through this questionnaire. Parents' anxiety levels were evaluated through the State-Trait Anxiety Inventory scales 1 and 2. Delayed diagnosis (DD) was defined as a definite CF diagnosis beyond the 8th week of life. Predictors of delayed CF diagnosis were evaluated by univariate and multivariate analysis. Results A total of 220 CF patients diagnosed via NBS were enrolled; 82 (37.3%) babies had DD. Multivariable analysis indicated that residence in the Southeast Anatolia region of Turkey (OR = 10.79, 95% CI = 2.37-49.2) was associated with a higher incidence of DD compared with other regions in Turkey. Of the total, 216 (98.1%) of the caregivers regarded the NBS program as useful and 180 (82%) reported high anxiety levels. Conclusion The organization of newborn screening should take into account regional and socio-cultural characteristics to improve the early diagnosis of CF and also reduce the anxiety level of parents.Item ERS International Virtual Congress 2021: Highlights from the Turkish Thoracic Society Early Career MembersSenel, MY; Karadogan, D; Vardaloglu, I; Develi, E; Çelik, S; Hizal, M; Özsezen, B; Öncel, A; Can, I; Hürsoy, N; Uyar, K; Karakas, FG; Er, B; Asfuroglu, P; Gürsoy, TR; Eyuboglu, TS; Çakir, EP; Ademhan, D; Karaoglanoglu, S; Emiralioglu, N; Öztürk, NAA; Marim, F; Güçlü, ÖA; Çetin, N; Topçu, DÖ; Çelik, P; Akgün, MThis review aimed to highlight some important points derived from the presentations of the European Respiratory Society 2021 Virtual International Congress by a committee formed by the Early Career Task Group of the Turkish Thoracic Society. We summarized a wide range of topics including current developments of respiratory diseases and provided an overview of important and striking topics of the congress. Our primary motivation was to give some up-to-date information and new developments discussed during congress especially for the pulmonologists who did not have a chance to follow the congress. This review also committed an opportunity to get an overview of the newest data in the diverse fields of respiratory medicine such as post-coronavirus disease 2019, some new interventional and technologic developments related to respiratory health, and new treatment strategies.Item Barriers to Timely Diagnosis of Cystic Fibrosis Via Newborn Screening in TurkeyGokdemir, Y; Emiralioglu, N; Sismanlar, T; Er, B; Pekcan, S; Ergenekon, A; Sen, V; Eryilmaz, S; Hizal, MG; Kose, M; Hangul, M; Cakir, E; Cokugras, H; Atabek, A; Sasihuseyinoglu, S; Gulen, F; Bingol, A; Ozdemir, A; Topal, E; Gursoy, TR; Ay, P; Yilmaz, OItem Evaluation of factors affecting bone mineral density in CF patients through CF registry of TurkeySoydas, SSA; Tugcu, GD; Gençoglu, MY; Cinel, G; Emiralioglu, N; Yalçin, E; Kiper, N; Sen,; Altintas, DU; Çokugras, H; Kilinç, AA; Yazan, H; Ünal, G; Yilmaz, A; Çaglar, HT; Damadoglu, E; Irmak, I; Demir, E; Öztürk, GK; Bingöl, A; Basaran, E; Sapan, N; Aslan, AT; Asfuroglu, P; Harmanci, K; Köse, M; Hangül, M; Özdemir, A; Özcan, G; Gayretli, ZG; Keskin, Ö; Yüksel, H; Özdogan, S; Topal, E; Çaltepe, G; Can, D; Ekren, PK; Kiliç, M; Eyüboglu, TS; Pekcan, S; Çobanoglu, N; Çakir, E; Özçelik, U; Dogru, DItem Patients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1 year?Uytun, S; Cinel, G; Polat, SE; Tabakçi, SO; Kiper, N; Yalçin, E; Tural, DA; Özsezen, B; Sen, V; Sen, HS; Altintas, DU; Çokugras, H; Kilinç, AA; Baskan, AK; Yazan, H; Çollak, A; Uzuner, S; Ünal, G; Yilmaz, AI; Çaglar, HT; Damadoglu, E; Irmak, I; Demir, E; Öztürk, GK; Bingöl, A; Basaran, E; Sapan, N; Canitez, Y; Aslan, AT; Asfuroglu, P; Harmanci, K; Köse, M; Hangül, M; Özdemir, A; Çobanoglu, N; Özcan, G; Keskin, Ö; Yüksel, H; Özdogan, S; Topal, E; Çaltepe, G; Can, D; Ekren, PK; Kiliç, M; Emiralioglu, N; Eyüboglu, TS; Pekcan, S; Çakir, E; Özçelik, U; Dogru, DBackgroundCystic fibrosis (CF) is an autosomal recessive disorder caused by CF transmembrane conductance regulator (CFTR) genetic variants. CFTR modulators improve pulmonary function and reduce respiratory infections in CF. This study investigated the clinical and laboratory follow-up parameters over 1 year in patients with CF who could not receive this treatment. MethodsThis retrospective cohort study included 2018 and 2019 CF patient data from the CF registry of Turkey. Demographic and clinical characteristics of 294 patients were assessed, who had modulator treatment indications in 2018 but could not reach the treatment. ResultsIn 2019, patients younger than 18 years had significantly lower BMI z-scores than in 2018. During the 1-year follow-up, forced expiratory volumes (FEV1) and FEV1 z-scores a trend toward a decrease. In 2019, chronic Staphylococcus aureus colonization, inhaled antipseudomonal antibiotic use for more than 3 months, oral nutritional supplement requirements, and oxygen support need increased. ConclusionsPatients who had indications for modulator treatments but were unable to obtain them worsened even after a year of follow-up. This study emphasized the importance of using modulator treatments for patients with CF in our country, as well as in many countries worldwide.Item Patients eligible for modulator drugs: Data from cystic fibrosis registry of TurkeyÇobanoglu, N; Özçelik, U; Çakir, E; Eyüboglu, TS; Pekcan, S; Cinel, G; Yalçin, E; Kiper, N; Emiralioglu, N; Sen, V; Sen, HS; Ercan, Ö; Çokugras, H; Kilinç, AA; Al Shadfan, LM; Yazan, H; Altintas, DU; Karagöz, D; Demir, E; Öztürk, GK; Bingöl, A; Basaran, AE; Sapan, N; Çekiç, S; Celebioglu, E; Aslan, AT; Gürsoy, TR; Tugcu, G; Özdemir, A; Harmanci, K; Yildirim, GK; Köse, M; Hangül, M; Tamay, Z; Süleyman, A; Yüksel, H; Yilmaz, O; Özcan, G; Topal, E; Can, D; Ekren, PK; Çaltepe, G; Kiliç, M; Özdogan, S; Dogru, DBackground A better understanding of cystic fibrosis transmembrane conductance regulator biology has led to the development of modulator drugs such as ivacaftor, lumacaftor-ivacaftor, tezacaftor-ivacaftor, and elexacaftor-tezacaftor-ivacaftor. This cross-sectional study evaluated cystic fibrosis (CF) patients eligible for modulator drugs. Methods Data for age and genetic mutations from the Cystic Fibrosis Registry of Turkey collected in 2018 were used to find out the number of patients who are eligible for modulator therapy. Results Of registered 1488 CF patients, genetic analysis was done for 1351. The numbers and percentages of patients and names of the drugs, that the patients are eligible for, are as follows: 122 (9.03%) for ivacaftor, 156 (11.54%) for lumacaftor-ivacaftor, 163 (11.23%) for tezacaftor-ivacaftor, and 57 (4.21%) for elexacaftor-tezacaftor-ivacaftor. Among 1351 genotyped patients total of 313 (23.16%) patients are eligible for currently licensed modulator therapies (55 patients were shared by ivacaftor and tezacaftor-ivacaftor, 108 patients were shared by lumacaftor-ivacaftor and tezacaftor-ivacaftor, and 22 patients were shared by tezacaftor-ivacaftor and elexacaftor-tezacaftor-ivacaftor groups). Conclusions The present study shows that approximately one-fourth of the registered CF patients in Turkey are eligible for modulator drugs. As, frequent mutations that CF patients have in Turkey are different from North American and European CF patients, developing modulator drugs effective for those mutations is necessary. Furthermore, as modulator drugs are very expensive currently, financial support of the government in developing countries like Turkey is noteworthy.Item Clinical findings of patients with cystic fibrosis according to newborn screening resultsGursoy, TR; Aslan, AT; Asfuroglu, P; Eyuboglu, TS; Cakir, E; Cobanoglu, N; Pekcan, S; Cinel, G; Dogru, D; Ozcelik, U; Yalcin, E; Sen, V; Ercan, O; Kilinc, AA; Yazan, H; Altintas, DU; Ozturk, GK; Bingol, A; Sapan, N; Celebioglu, E; Tugcu, GD; Ozdemir, A; Harmanci, K; Kose, M; Emiralioglu, N; Tamay, Z; Yuksel, H; Ozcan, G; Topal, E; Can, D; Ekren, PK; Caltepe, G; Kilic, M; Ozdogan, SBackground Cystic fibrosis (CF) is a lethal recessive genetic disease caused by loss of function associated with mutations in the CF trans-membrane conductance regulator. It is highly prevalent (approximately 1 in 3,500) in Caucasians. The aim of this study was to compare demographic and clinical features, diagnostic tests, treatments, and complications of patients with CF whose newborn screening (NBS) with twice-repeated immune reactive trypsinogen testing was positive, normal, and not performed. Methods In this study, 359 of all 1,488 CF patients recorded in the CF Registry of Turkey in 2018, who had been born through the process of NBS, were evaluated. Demographic and clinical features were compared in patients diagnosed with positive NBS (Group 1), normal (Group 2), or without NBS (Group 3). Results In Group 1, there were 299 patients, in Group 2, there were 40 patients, and in Group 3, there were 20 patients. Among all patients, the median age at diagnosis was 0.17 years. The median age at diagnosis was higher in Groups 2 and 3 than in Group 1 (P = 0.001). Fecal elastase results were higher in Group 2 (P = 0.033). The weight z-score was lower and chronic Staphylococcus aureus infection was more common in Group 3 (P = 0.017, P = 0.004, respectively). Conclusions Frequency of growth retardation and chronic S. aureus infection can be reduced with an early diagnosis using NBS. In the presence of clinical suspicion in patients with normal NBS, further analyses such as genetic testing should be performed, especially to prevent missing patients with severe mutations.Item Evaluation of respiratory function at 6 years of age in patients with cystic fibrosis with frequent pulmonary exacerbations in the first 2 years of lifeTabakci, SO; Cinel, G; Uytun, S; Polat, SE; Yalçin, E; Kiper, N; Erdal, MA; Sen, V; Savas, S; Çelebi, Ö; Altintas, DU; Serbes, M; Kilinç, AA; Çokugras, H; Arslan, H; Yazan, H; Kafi, HM; Çollak, A; Ünal, G; Yilmaz, AI; Çaglar, HT; Irmak, I; Damadoglu, E; Öztürk, GK; Demir, E; Basaran, E; Bingöl, A; Canitez, Y; Çekiç, S; Asfuroglu, P; Aslan, AT; Harmanci, K; Kiliç, G; Köse, M; Ersoy, A; Hangül, M; Özdemir, A; Özcan, G; Çobanoglu, N; Aydin, ZGG; Keskin, Ö; Yüksel, H; Özdogan, S; Topal, E; Çaltepe, G; Can, D; Ekren, PK; Kiliç, M; Eyüboglu, TS; Pecan, S; Çakir, E; Emiralioglu, N; Özçelik, U; Ersöz, DDItem Clinical characteristics of patients requiring lung transplantation referral in national cystic fibrosis registry dataOnay, ZR; Eyüboglu, TS; Aslan, AT; Gürsoy, TR; Yalçin, E; Kiper, N; Emiralioglu, N; Sen, HS; Sen, V; Ünal, G; Yilmaz, AI; Kilinç, AA; Çokugras, H; Baskan, AK; Yazan, H; Çollak, A; Uzuner, S; Sasihüseyinoglu, AS; Özcan, D; Altintas, DU; Öztürk, GK; Demir, E; Bingöl, A; Basaran, E; Çekiç, S; Sapan, N; Irmak, I; Damadoglu, E; Tugcu, GD; Polat, SE; Özdemir, A; Harmanci, K; Kiliç, G; Hangül, M; Köse, M; Tamay, Z; Yüksel, H; Özcan, G; Topal, E; Can, D; Korkmaz, P; Çaltepe, G; Kiliç, M; Özdogan, S; Çakir, E; Çobanoglu, N; Pekcan, S; Cinel, G; Özçelik, U; Dogru, DBackground. We aimed to determine the number of cystic fibrosis (CF) patients recorded in the Cystic Fibrosis Registry of Turkiye (CFRT) who were in need of lung transplantation (LT) referral and examine clinical differences between patients who were LT candidates due to rapid forced expiratory volume in one second (FEV1) decline and LT candidates without rapid FEV1 decline in the last year to identify a preventable cause in patients with such rapid FEV1 decline. Methods. All CF patients recorded in the CFRT in 2018 were evaluated in terms of LT. Patients were divided into those with FEV1 below 50% and in need of LT due to a decrease of 20% or more in the previous year (Group 1) and those who did not have FEV1 decline of more than 20% in the previous year but had other indications for LT (Group 2). Demographic and clinical features were compared between the two groups. Results. Of 1488 patients registered in CFRT, 58 had a need for LT. Twenty patients were included in Group 1 and others in Group 2. Our findings did not reveal any significant variations in treatment, chronic infection status, or complications between the two groups. The average weight z-score was significantly higher in Group 1. Positive correlations were detected between weight z-score and FEV1 in 2017 in Group 1 and between FEV1 values in 2017 and 2018 in Group 2. Conclusions. There appears to be a relationship between the nutritional status and weight z-scores of CF patients and pulmonary function, which may indirectly affect the need for lung transplantation referral.Item The efficacy of inhaled hypertonic saline in children with cystic fibrosis who receive daily DNase.Gencoglu, MY; Tugcu, GD; Soydas, SSA; Cinel, G; Emiralioglu, N; Yalçin, E; Kiper, N; Sen,; Altintas, DU; Çokugras, H; Kilinç, AA; Yazan, H; Ünal, G; Yilmaz, A; Çaglar, HT; Damadoglu, E; Irmak, I; Demir, E; Öztürk, G; Bingöl, A; Basaran, E; Sapan, N; Aslan, AT; Asfuroglu, P; Harmanci, K; Köse, M; Hangül, M; Özdemir, A; Özcan, G; Gayretli, ZG; Keskin, Ö; Yüksel, H; Özdogan, S; Topal, E; Çaltepe, G; Can, D; Ekren, PK; Kiliç, M; Eyüboglu, TS; Pekcan, S; Çobanoglu, N; Çakir, E; Özçelik, U; Dogru, DItem Cystic Fibrosis Patients Eligible for Modulator Drugs: Data from Cystic Fibrosis Registry of TurkeyÇobanoglu, FN; Ersöz, DD; Çakir, E; Eyüboglu, TS; Pekcan, S; Cinel, G; Yalçin, E; Kiper, N; Sen, V; Sen, HS; Ercan, Ö; Keskin, Ö; Eltan, SB; Al Shadfan, LM; Yazan, H; Altintas, DU; Sasihüseyinoglu, S; Sapan, N; Çekiç, S; Çokugras, H; Atabek, AA; Gürsoy, TR; Aslan, AT; Bingöl, A; Basaran, AE; Özdemir, A; Köse, M; Hangül, M; Emiralioglu, N; Tugcu, G; Yuksel, H; Yilmaz, Ö; Orhan, F; Aydin, ZGG; Topal, E; Tamay, Z; Süleyman, A; Can, D; Bal, CM; Çaltepe, G; Özçelik, UItem Early Pseudomonas aeruginosa colonization in cystic fibrosis patientsOcak, E; Tugcu, GD; Polat, SE; Cinel, G; Emiralioglu, N; Yalçin, E; Kiper, N; Sen,; Altintas, DU; Çokugras, H; Kilinç, AA; Yazan, H; Ünal, G; Yilmaz, AI; Damadoglu, E; Irmak, I; Demir, E; Öztürk, G; Basaran, AE; Bingöl, A; Sapan, N; Aslan, AT; Asfuroglu, P; Harmanci, K; Köse, M; Hangül, M; Özdemir, A; Özcan, G; Aydin, ZGG; Keskin, AÖ; Yüksel, H; Özdogan, S; Topal, E; Çaltepe, G; Can, D; Ekren, PK; Kiliç, M; Eyüboglu, TS; Pekcan, S; Çobanoglu, N; Çakir, E; Özçelik, U; Ersöz, DDItem Cystic fibrosis in Turkey: First data from the national registryDogru, D; Çakir, E; Sismanlar, T; Çobanoglu, N; Pekcan, S; Cinel, G; Yalçin, E; Kiper, N; Sen, V; Sen, HS; Ercan, Ö; Keskin, Ö; Eltan, SB; Al Shadfan, LM; Yazan, H; Altintas, DU; Sasihüseyinoglu, S; Sapan, N; Çekiç, S; Çokugras, H; Kilinç, AA; Gürsoy, TR; Aslan, AT; Bingöl, A; Basaran, AE; Özdemir, A; Köse, M; Hangül, M; Emiralioglu, N; Tugcu, G; Yüksel, H; Yilmaz, Ö; Orhan, F; Gayretli Aydin, ZG; Topal, E; Tamay, Z; Süleyman, A; Can, D; Bal, CM; Çaltepe, G; Özçelik, UBackground 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.Item One-Year Follow-Up of Cystic Fibrosis Patients Who Cannot Reach the Modulator Therapies Although Having Indications: What are Their Losses?Uytun, S; Polat, SE; Tabakçi, SÖ; Cinel, G; Emiralioglu, N; Yalçin, E; Kiper, N; Sen,; Altintas, DU; Çokugras, H; Kilinç, AA; Yazan, H; Çollak, A; Uzuner, S; Ünal, G; Yilmaz, A; Çelik, S; Damadoglu, E; Irmak, I; Demir, E; Öztürk, G; Basaran, E; Bingöl, A; Sapan, N; Aslan, AT; Asfuroglu, P; Harmanci, K; Köse, M; Hangül, M; Özdemir, A; Özcan, G; Gayretli, ZG; Keskin, Ö; Yüksel, H; Özdogan, S; Topal, E; Çaltepe, G; Can, D; Ekren, PK; Kiliç, M; Süleyman, A; Eyüboglu, TS; Pekcan, S; Çobanoglu, N; Çakir, E; Özçelik, U; Dogru, DItem Turkish Thoracic Society Early Career Members Task Force Group's Virtual Congress Notes: European Respiratory Society International Congress 2020Kizilirmak, D; Karadogan, D; Yildirim, H; Akyil, FT; Eyüboglu, TS; Emiralioglu, N; Sertçelik, ÜÖ; Günaydin, FE; Ataoglu, Ö; Oguz, MS; Çakmakci, S; Özçelik, N; Öncel, A; Firincioglulari, A; Kara, BY; Ömer, D; Karaoglanoglu, S; Cetin, N; Karakas, FG; Gurkan, CG; Marim, F; Önyilmaz, T; Yulug, DP; Öztürk, NAA; Güçlü, ÖA; Küsbeci, TÇ; Serifoglu, I; Arikan, H; Töreyin, ZN; Çelik, P; Akgün, MIn this article, Early Career Task Force Group members of the Turkish Thoracic Society summarize the European Respiratory Society 2020 virtual congress. Current developments in the field of respiratory diseases were compiled with the addition of sessions specific to coronavirus disease 2019 this year. Almost all of the congress sessions were examined, and the important and striking results of the congress were highlighted. Congress sessions were attended by expert researchers, and the prominent messages of each session were highlighted in short summaries. They were then grouped under relevant titles and ranked in order of meaning and relation. It was finalized by a team of researchers.Item Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosisEyuboglu, TS; Dogru, D; Çakir, E; Cobanoglu, N; Pekcan, S; Cinel, G; Yalçin, E; Kiper, N; Sen, V; Sen, HS; Ercan, O; Keskin, O; Eltan, SB; Alshadfan, L; Yazan, H; Altintas, DU; Sasihuseyinoglu, AS; Sapan, N; Cekic, S; Cokugras, H; Kilinc, AA; Gursoy, TR; Aslan, AT; Bingol, A; Basaran, AE; Ozdemir, A; Kose, M; Hangul, M; Emiralioglu, N; Tugcu, G; Yuksel, H; Yilmaz, O; Orhan, F; Aydin, ZGG; Topal, E; Tamay, Z; Suleyman, A; Can, D; Bal, CM; Caltepe, G; Ozcelik, UBackground Pseudo-Bartter syndrome (PBS) is a rare complication of cystic fibrosis (CF) and there are limited data in the literature about it. We aimed to compare clinical features and accompanying findings of patients with PBS in a large patient population. Methods The data were collected from the Cystic Fibrosis Registry of Turkey where 1170 CF patients were recorded in 2017. Clinical features, diagnostic test results, colonization status, complications, and genetic test results were compared in patients with and without PBS. Results Totally 1170 patients were recorded into the registry in 2017 and 120 (10%) of them had PBS. The mean age of diagnosis and current age of patients were significantly younger and newborn screening positivity was lower in patients with PBS (P < .001). There were no differences between the groups in terms of colonization status, mean z-scores of weight, height, BMI, and mean FEV1 percentage. Types of genetic mutations did not differ between the two groups. Accompanying complications were more frequent in patients without PBS. Conclusion PBS was detected as the most common complication in the registry. It could be due to warm weather conditions of our country. It is usually seen in younger ages regardless of mutation phenotype and it could be a clue for early diagnosis of CF.Item CFTR mutations unidentified in CFTR2 database and their phenotypic characteristics: Data from cystic fibrosis registry of TurkeyCinel, G; Dogru, D; Çakir, E; Eyüboglu, TS; Çobanoglu, N; Pekcan, S; Yalçin, E; Kiper, N; Sen, V; Sen, HS; Ercan, Ö; Keskin, Ö; Eltan, SB; Al Shadfan, LM; Yazan, H; Altintas, DU; Sasihüseyinoglu, S; Sapan, N; Çekiç, S; Çokugras, H; Atabek, AA; Gürsoy, TR; Aslan, AT; Bingöl, A; Basaran, AE; Özdemir, A; Köse, M; Hangül, M; Emiralioglu, N; Tugcu, G; Yüksel, H; Yilmaz, Ö; Orhan, F; Aydin, ZGG; Topal, E; Tamay, Z; Can, D; Bal, CM; Çaltepe, G; Özçelik, UItem Factors associated with pulmonary function decline of patients in the cystic fibrosis registry of Turkey: A retrospective cohort studyEmiralioglu, N; Çakir, B; Sertçelik, A; Yalçin, E; Kiper, N; Sen, V; Altintas, DU; Serbes, M; Çokugras, H; Kilinç, AA; Baskan, AK; Hepkaya, E; Yazan, H; Türel, O; Kafi, HM; Yilmaz, AI; Ünal, G; Çaglar, T; Damadoglu, E; Irmak, I; Demir, E; Öztürk, G; Bingöl, A; Basaran, E; Sapan, N; Aslan, AT; Asfuroglu, P; Harmanci, K; Köse, M; Hangül, M; Özdemir, A; Tugcu, G; Polat, SE; Özcan, G; Gayretli, ZG; Keskin, O; Bilgic, S; Yüksel, H; Özdogan, S; Topal, E; Caltepe, G; Can, D; Ekren, PK; Kiliç, M; Süleyman, A; Eyüboglu, TS; Cinel, G; Pekcan, S; Çobanoglu, N; Çakir, E; Özcelik, U; Dogru, DBackground: The decline in pulmonary function is a predictor of disease progression in patients with cystic fibrosis (CF). This study aimed to determine the decline rate of percent predicted forced expiratory volume in 1 s (ppFEV1) based on the data of the CF Registry of Turkey. The secondary aim was to investigate the risk factors related to the decline in ppFEV1. Methods: A retrospective cohort study of CF patients over 6 years old, with pulmonary function data over at least 2 years of follow-up was extracted from the national CF registry for years 2017-2019. Patients were classified according to disease severity and age groups. Multivariate analysis was used to predict the decline in ppFEV1 and to investigate the associated risk factors. Results: A total of 1722 pulmonary function test results were available from 574 patients over the study period. Mean diagnostic age was older and weight for age, height for age, and body mass index z scores were significantly lower in the group of ppFEV1 < 40, while chronic Pseudomonas aeruginosa (p < .001) and mucoid P. aeruginosa colonization (p < .001) were significantly higher in this group (p < .001). Overall mean annual ppFEV1 decline was -0.97% (95% confidence interval [CI] = -0.02 to -1.92%). The mean change of ppFEV1 was significantly higher in the group with ppFEV1 >= 70 compared with the other (ppFEV1 < 40 and ppFEV1: 40-69) two groups (p = .004). Chronic P. aeruginosa colonization (odds ratio [OR] = 1.79 95% CI = 1.26-2.54; p = .01) and initial ppFEV1 >= 70 (OR = 2.98 95% CI = 1.06-8.36), p = .038) were associated with significant ppFEV1 decline in the whole cohort. Conclusions: This data analysis recommends close follow-up of patients with normal initial ppFEV1 levels at baseline; advocates for early interventions for P. aeruginosa; and underlines the importance of nutritional interventions to slow down lung disease progression.Item The success of the Cystic Fibrosis Registry of Turkey for improvement of patient careAsfuroglu, P; Eyuboglu, TS; Aslan, AT; Gursoy, TR; Emiralioglu, N; Yalcin, E; Kiper, N; Sen, V; Sen, HS; Altintas, DU; Ozcan, D; Kilinc, AA; Cokugras, H; Baskan, AK; Yazan, H; Erenberk, U; Dogan, G; Unal, G; Yilmaz, AI; Keskin, O; Arik, E; Kucukosmanoglu, E; Irmak, I; Damadoglu, E; Ozturk, GK; Gulen, F; Basaran, AE; Bingol, A; Cekic, S; Sapan, N; Kilic, G; Harmanci, K; Kose, M; Ozdemir, A; Tugcu, GD; Polat, SE; Hangul, M; Ozcan, G; Aydin, ZGG; Yuksel, H; Topal, E; Ozdogan, S; Caltepe, G; Suleyman, A; Can, D; Ekren, PK; Bal, CM; Kilic, M; Cinel, G; Cobanoglu, N; Pekcan, S; Cakir, E; Ozcelik, U; Dogru, DBackground Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established CF registry system in Turkey on follow-up, clinical, growth, treatment, and complications of people with this disease. Methods Age at diagnosis, current age, sex, z-scores of weight, height and body mass index (BMI), neonatal screening results, pulmonary function tests, history of meconium ileus, medications, presence of microorganisms, and follow-up were evaluated and compared to data of people with CF represented in both 2017 and 2019 registry data. Results There were 1170 people with CF in 2017 and 1637 in 2019 CF registry. Eight hundred and fourteen people were registered in both 2017 and 2019 of whom z-scores of heights and BMI were significantly higher in 2019 (p = 0.002, p =0.039, respectively). Inhaled hypertonic saline, bronchodilator, and azithromycin usages were significantly higher in 2019 (p =0.001, p = 0.001, p = 0.003, respectively). The percent predicted of forced expiratory volume in 1 sec and forced vital capacity were similar in 2017 and 2019 (88% and 89.5%, p = 0.248 and 84.5% and 87%, p =0.332, respectively). Liver diseases and osteoporosis were significantly higher, and pseudo-Bartter syndrome (PBS) was significantly lower in 2019 (p = 0.011, p = 0.001, p = 0.001, respectively). Conclusions The z-scores of height and BMI were higher, the use of medications that protect and improve lung functions was higher and incidence of PBS was lower in 2019. It was predicted that registry system increased the care of people with CF regarding their follow-up. The widespread use of national CF registry system across the country may be beneficial for the follow-up of people with CF.