Browsing by Author "Dogru D."

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    Reliability and validity of the Cystic Fibrosis Questionnaire-Revised for children and parents in Turkey: Cross-sectional study
    (Kluwer Academic Publishers, 2013) Yuksel H.; Yilmaz O.; Dogru D.; Karadag B.; Unal F.; Quittner A.L.
    Purpose: The purpose of study was to translate Cystic Fibrosis Questionnaire-Revised (CFQ-R) into Turkish for children with cystic fibrosis (CF) and evaluate its reliability and validity. This is the first CF-specific health-related quality of life (HRQOL) measure validated in a Muslim country. Methods: Fifty-one children aged 6-13 years treated at four centers in Turkey and 30 parents participated in this cross-sectional study. Demographic characteristics and disease severity parameters were recorded for all participants. All participants completed the parent or child versions of CFQ-R and KINDL questionnaires at enrollment. Reliability and construct validity analysis were carried out. Results: Both children and parents endorsed a range of responses, with no evidence of floor or ceiling effects. Item-to-total correlations indicated that most items were more highly correlated with their intended scale than competing scales. Good internal consistency was found for majority of child and parent scales. CFQ-R scales correlated significantly with clinical indices of disease severity. Good evidence of convergent validity with a generic HRQOL scale was found. Conclusion: Turkish versions of CFQ-R Child and Parent instruments have demonstrated adequate reliability and validity and can be utilized in clinical trials or integrated into clinical evaluation and follow-up of Turkish children with CF. © 2012 Springer Science+Business Media B.V.
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    Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosis
    (John Wiley and Sons Inc., 2020) Sismanlar Eyuboglu T.; Dogru D.; Çakır E.; Cobanoglu N.; Pekcan S.; Cinel G.; Yalçın E.; Kiper N.; Sen V.; Selimoglu Sen H.; Ercan O.; Keskin O.; Bilgic Eltan S.; Alshadfan L.; Yazan H.; Altıntas D.U.; Sasihuseyinoglu A.S.; Sapan N.; Cekic S.; Cokugraş H.; Kılınc A.A.; Ramaslı Gursoy T.; Aslan A.T.; Bingol A.; Başaran A.E.; Ozdemir A.; Kose M.; Hangul M.; Emiralioglu N.; Tugcu G.; Yuksel H.; Yılmaz O.; Orhan F.; Gayretli Aydın Z.G.; Topal E.; Tamay Z.; Suleyman A.; Can D.; Bal C.M.; Caltepe G.; Ozcelik U.
    Background: 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. © 2020 Wiley Periodicals, Inc.
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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.
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    Clinical findings of patients with cystic fibrosis according to newborn screening results
    (John Wiley and Sons Inc, 2022) Ramasli Gursoy T.; Aslan A.T.; Asfuroglu P.; Sismanlar Eyuboglu T.; Cakir E.; Cobanoglu N.; Pekcan S.; Cinel G.; Dogru D.; Ozcelik U.; Yalcin E.; Sen V.; Ercan O.; Kilinc A.A.; Yazan H.; Altintas D.U.; Kartal Ozturk G.; Bingol A.; Sapan N.; Celebioglu E.; Tugcu G.D.; Ozdemir A.; Harmanci K.; Kose M.; Emiralioglu N.; Tamay Z.; Yuksel H.; Ozcan G.; Topal E.; Can D.; Korkmaz Ekren P.; Caltepe G.; Kilic M.; Ozdogan S.
    Background: 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. © 2021 Japan Pediatric Society.