Browsing by Author "Kose M."

Now showing 1 - 5 of 5
  • No Thumbnail Available
    Item
    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.
  • No Thumbnail Available
    Item
    Geographical barriers to timely diagnosis of cystic fibrosis and anxiety level of parents during newborn screening in Turkey
    (John Wiley and Sons Inc, 2021) Gokdemir Y.; Eyuboglu T.S.; Emiralioglu N.; Er B.; Sen V.; Pekcan S.; Ergenekon A.P.; Hizal M.G.; Eryilmaz S.; Kose M.; Hangul M.; Cakir E.; Cokugras H.; Kılınc A.A.; Sasıhuseyinoglu A.S.; Altintas D.U.; Gulen F.; Eski A.; Bingol A.; Ozdemir A.; Topal E.; Gursoy T.R.; Girit S.; Ay P.; Yılmaz O.
    Background: 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. © 2021 Wiley Periodicals LLC
  • No Thumbnail Available
    Item
    Epidemiological, Clinical, and Laboratory Features of Children With COVID-19 in Turkey
    (Frontiers Media S.A., 2021) Karbuz A.; Akkoc G.; Bedir Demirdag T.; Yilmaz Ciftdogan D.; Ozer A.; Cakir D.; Hancerli Torun S.; Kepenekli E.; Erat T.; Dalgic N.; Ilbay S.; Karaaslan A.; Erdeniz E.H.; Aygun F.D.; Bozdemir S.E.; Hatipoglu N.; Emiroglu M.; Sahbudak Bal Z.; Ciftci E.; Bayhan G.I.; Gayretli Aydin Z.G.; Ocal Demir S.; Kilic O.; Hacimustafaoglu M.; Sener Okur D.; Sen S.; Yahsi A.; Akturk H.; Cetin B.; Sutcu M.; Kara M.; Uygun H.; Tural Kara T.; Korukluoglu G.; Akgun O.; Üstündağ G.; Demir Mis M.; Sali E.; Kaba O.; Yakut N.; Kılıc O.; Kanik M.K.; Cetin C.; Dursun A.; Cicek M.; Kockuzu E.; Sevketoglu E.; Alkan G.; Guner Ozenen G.; İnce E.; Baydar Z.; Ozkaya A.K.; Ovali H.F.; Tekeli S.; Celebi S.; Cubukcu B.; Bal A.; Khalilova F.; Kose M.; Hatipoglu H.U.; Dalkiran T.; Turgut M.; Basak Altas A.; Selcuk Duru H.N.; Aksay A.; Saglam S.; Sari Yanartas M.; Ergenc Z.; Akin Y.; Duzenli Kar Y.; Sahin S.; Tuteroz S.K.; Bilen N.M.; Ozdemir H.; Senoglu M.C.; Pariltan Kucukalioglu B.; Besli G.E.; Kara Y.; Turan C.; Selbest Demirtas B.; Celikyurt A.; Cosgun Y.; Elevli M.; Sahin A.; Bahtiyar Oguz S.; Somer A.; Karadag B.; Demirhan R.; Turk Dagi H.; Kurugol Z.; Taskin E.C.; Sahiner A.; Yesil E.; Ekemen Keles Y.; Sarikaya R.; Erdem Eralp E.; Ozkinay F.; Konca H.K.; Yilmaz S.; Gokdemir Y.; Arga G.; Ozen S.; Coksuer F.; Vatansever G.; Tezer H.; Kara A.
    Objectives: The aim of this study is to identify the epidemiological, clinical, and laboratory features of coronavirus disease 2019 (COVID-19) in children. Methods: A retrospective study was conducted by pediatric infectious disease specialists from 32 different hospitals from all over Turkey by case record forms. Pediatric cases who were diagnosed as COVID-19 between March 16, 2020, and June 15, 2020 were included. Case characteristics including age, sex, dates of disease onset and diagnosis, family, and contact information were recorded. Clinical data, including the duration and severity of symptoms, were also collected. Laboratory parameters like biochemical tests and complete blood count, chest X-ray, and chest computed tomography (CT) were determined. Results: There were 1,156 confirmed pediatric COVID-19 cases. In total, male cases constituted 50.3% (n = 582) and females constituted 49.7% (n = 574). The median age of the confirmed cases was 10.75 years (4.5–14.6). Of the total cases, 90 were younger than 1 year of age (7.8%), 108 were 1–3 years of age (9.3%), 148 were 3–6 years of age (12.8%), 298 were 6–12 years of age (25.8%), 233 were 12–15 years of age (20.2%), and 268 cases were older than 15 years of age (23.2%). The most common symptom of the patients at the first visit was fever (50.4%) (n = 583) for a median of 2 days (IQR: 1–3 days). Fever was median at 38.4°C (38.0–38.7°C). The second most common symptom was cough (n = 543, 46.9%). The other common symptoms were sore throat (n = 143, 12.4%), myalgia (n = 141, 12.2%), dyspnea (n = 118, 10.2%), diarrhea (n = 112, 9.7%), stomachache (n = 71, 6.1%), and nasal discharge (n = 63, 5.4%). When patients were classified according to disease severity, 263 (22.7%) patients were asymptomatic, 668 (57.7%) patients had mild disease, 209 (18.1%) had moderate disease, and 16 (1.5%) cases had severe disease. One hundred and forty-nine (12.9%) cases had underlying diseases among the total cases; 56% of the patients who had severe disease had an underlying condition (p < 0.01). The need for hospitalization did not differ between patients who had an underlying condition and those who do not have (p = 0.38), but the need for intensive care was higher in patients who had an underlying condition (p < 0.01). Forty-seven (31.5%) of the cases having underlying conditions had asthma or lung disease (38 of them had asthma). Conclusions: To the best of our knowledge, this is one of the largest pediatric data about confirmed COVID-19 cases. Children from all ages appear to be susceptible to COVID-19, and there is a significant difference in symptomatology and laboratory findings by means of age distribution. © Copyright © 2021 Karbuz.
  • No Thumbnail Available
    Item
    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.
  • No Thumbnail Available
    Item
    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.