Browsing by Author "Çekiç, S"

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    Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey
    Çobanoglu, 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, D
    Background 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.
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    Evaluation of respiratory function at 6 years of age in patients with cystic fibrosis with frequent pulmonary exacerbations in the first 2 years of life
    Tabakci, 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, DD
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    Clinical characteristics of patients requiring lung transplantation referral in national cystic fibrosis registry data
    Onay, 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, D
    Background. 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.
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    Cystic Fibrosis Patients Eligible for Modulator Drugs: Data from Cystic Fibrosis Registry of Turkey
    Çobanoglu, 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, U
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    Cystic fibrosis in Turkey: First data from the national registry
    Dogru, 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, U
    Background 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.
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    CFTR mutations unidentified in CFTR2 database and their phenotypic characteristics: Data from cystic fibrosis registry of Turkey
    Cinel, 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, U