Browsing by Author "Kilic M."
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Item Experience with mesocaval shunt with autologous jugular vein interposition in patients with Budd-Chiari syndrome(2005) Ilkgul Ö.; Kilic M.; Içöz G.; Zeytunlu M.; Demirpolat G.; Akyildiz M.; Tokat Y.; Parildar M.; Memis A.Background/Aims: In the present era of interventional radiology and liver transplantation, the role of mesocaval shunt surgery for portal hypertension in Budd-Chiari syndrome is reviewed. Methodology: This study analyzed the management of 35 patients with Budd-Chiari syndrome between June 1994 and June 2004 in our institution. During this 10-year interval, 31 of the 35 patients with Budd-Chiari syndrome underwent shunt procedures and four patients underwent liver transplantation. Mesocaval shunts were preferred in 27 patients and seven of these patients required prior caval stenting. One portocaval shunt was performed in a patient having a thrombosed mesocaval shunt. In all mesocaval shunt procedures the patient's internal jugular vein was used as an interposition graft between the superior mesenteric vein and inferior vena cava. In four patients with thrombosed vena cava a mesoatrial shunt was performed using polytetrafluoroethylene graft while four patients with established cirrhosis underwent orthotopic liver transplantation. Results: In the group of mesocaval shunts, 3 patients were lost in the early postoperative period with a mortality rate of 11%, 2 of them due to thrombosed shunts and one of them due to pneumonia. The median follow-up was 42 months (6-120 months) and one patient experienced shunt thrombosis and died afterwards due to the complications of portal hypertension. In the whole series the patency rate of the mesocaval shunt was 89%. Conclusions: Patients with Budd-Chiari syndrome can be managed by a combination of shunt surgery, interventional radiology and liver transplantation. Our results demonstrate the effectiveness of mesocaval shunt procedure with autologous jugular vein interposition to maintain long-term patency and survival. © H.G.E. Update Medical Publishing S.A.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.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.