Browsing by Subject "meconium ileus"

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    Cystic fibrosis in Turkey: First data from the national registry
    (John Wiley and Sons Inc., 2020) Dogru D.; Çakır E.; Şişmanlar T.; Çobanoğlu N.; Pekcan S.; Cinel G.; Yalçın E.; Kiper N.; Şen V.; S. Şen H.; Ercan Ö.; Keskin Ö.; B. Eltan S.; Al Shadfan L.M.; Yazan H.; Altıntaş D.U.; Şaşihüseyinoğlu Ş.; Sapan N.; Çekiç Ş.; Çokuğraş H.; A. Kılınç A.; R. Gürsoy T.; Aslan A.T.; Bingöl A.; Başaran A.E.; Özdemir A.; Köse M.; Hangül M.; Emiralioğlu N.; Tuğcu G.; Yüksel H.; Yılmaz Ö.; Orhan F.; Gayretli Aydın Z.G.; Topal E.; Tamay Z.; Süleyman A.; Can D.; Bal C.M.; 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. © 2019 Wiley Periodicals, Inc.
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    The success of the Cystic Fibrosis Registry of Turkey for improvement of patient care
    (John Wiley and Sons Inc, 2022) Asfuroglu P.; Sismanlar Eyuboglu T.; Aslan A.T.; Gursoy T.R.; Emiralioglu N.; Yalcin E.; Kiper N.; Sen V.; Sen H.S.; Altintas D.U.; Ozcan D.; Kilinc A.A.; Cokugras H.; Baskan A.K.; Yazan H.; Erenberk U.; Dogan G.; Unal G.; Yilmaz A.I.; Keskin O.; Arik E.; Kucukosmanoglu E.; Irmak I.; Damadoglu E.; Ozturk G.K.; Gulen F.; Basaran A.E.; Bingol A.; Cekic S.; Sapan N.; Kilic G.; Harmanci K.; Kose M.; Ozdemir A.; Tugcu G.D.; Polat S.E.; Hangul M.; Ozcan G.; Aydin Z.G.G.; Yuksel H.; Topal E.; Ozdogan S.; Caltepe G.; Suleyman A.; Can D.; Ekren P.K.; Bal C.M.; Kilic M.; Cinel G.; Cobanoglu N.; Pekcan S.; Cakir E.; Ozcelik U.; Dogru D.
    Background: 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. © 2022 Wiley Periodicals LLC.