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    Erysipelas-like erythema on elbow in a child with familial Mediterranean fever: an unexpected localization
    (Springer London Ltd, 2025) Yüksel, Selçuk; Yüksel, Gülten
    [No abstract available]
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    Exploring gastrointestinal manifestations in childhood onset systemic lupus erythematosus - Insights from a multicenter study
    (Sage Publications Ltd, 2024) Sönmez, Hafize Emine; Batu, Ezgi Deniz; İşgüder, Rana; Şahin, Nihal; Aliyev, Emil; Aslan, Esma; Çoban, Sümeyra; Yüksel, Selçuk
    Objective Systemic lupus erythematosus (SLE) constitutes an autoimmune disorder with potential involvement of the gastrointestinal system (GIS). Our objective was to assess the gastrointestinal (GI) manifestations in patients diagnosed with childhood onset SLE.Methods The study cohort consisted of 123 patients with childhood onset-SLE and GIS involvement from 16 referral departments of pediatric rheumatology. All participants met the Systemic Lupus International Collaborating Clinics criteria.Results Out of 123 patients, 78 (63.4%) exhibited GIS involvement at the initial SLE diagnosis, whereas the remaining 45 (36.6%) developed GI symptoms after a median duration of 12 (3-140) months. Eighty-two (66.7%) individuals experienced symptoms related to the GI tract, whereas the remaining patients received a diagnosis of GI involvement through laboratory assessments. The predominant initial GIS involvement symptom was abdominal pain, observed in 77 (62.6%) patients, followed by elevated hepatic transaminases in 70 (56.9%), hepatomegaly in 40 (32.5%), diarrhea in 26 (21.1%), and jaundice in 11 (8.9%) patients. The GIS involvement was associated with SLE in 82 (78.6%), while it resulted from drug-related adverse events in 35 (28.5%) patients or comorbidities in 6 (0.5%) patients.Conclusion GIS involvement should be considered in all childhood onset-SLE patients, especially in the presence of suggestive symptoms or elevated hepatic transaminases. It is also crucial to consider SLE in the differential diagnosis of GIS manifestations in children. Apart from GIS involvement directly associated with SLE, adverse events of drugs should be kept in mind.
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    Insights from ADPedKD, ERKReg and RaDaR registries provide a multi-national perspective on the presentation of childhood autosomal dominant polycystic kidney disease in high- and middle-income countries
    (Elsevier B.V., 2025) Gimpel, Charlotte; Fieuws, Steffen; Hofstetter, Jonas; Pitcher, David; Vanmeerbeek, Lotte; Haeberle, Stefanie; Dachy, Angélique; Yüksel, Selçuk
    Data on the presentation of Autosomal Dominant Polycystic Kidney Disease (ADPKD) in children have been based on small/regional cohorts and practices regarding both asymptomatic screening in minors and genetic testing differ greatly between countries. To provide a global perspective, we analyzed over 2100 children and adolescents with ADPKD from 32 countries in six World Health Organization regions: 1060 children from the multi-national ADPedKD registry were compared to 269 pediatric patients from the United Kingdom (RaDaR) and 825 from the European Rare Kidney Disease Registry (ERKReg). Asymptomatic family screening was a common mode of presentation (48% in ADPedKD, 62% in ERKReg) with broad international variability (19%-75%), but fairly stable temporal trends in both registries with no correlation to genetic testing. The national rates of genetic testing varied and correlated significantly with healthcare expenditure (odds ratio 1.030 per 100 United States Dollars/capita/year, in the ERKReg cohort), with little variation over time. Diagnosis due to prenatal abnormalities was more common than anticipated at 14% increasing steadily from 2000 onward in both registries. Realistically, a high proportion of children were diagnosed with ADPKD by active screening, underlining that families affected by ADPKD have a high need for counselling on the complex issues around presymptomatic diagnosis. Regional variations in rate of genetic testing appeared to be driven by economic factors. However, large differences in rate of active screening were not correlated to healthcare spending and probably reflect the influence of different of cultural, legal and ethical frameworks on families and clinicians in different healthcare systems. © 2025 International Society of Nephrology
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    Kidney transplantation in pediatric and young adults: a single-center experience
    (Pamukkale University, 2023) Özgen, Utku; Özban, Murat; Akbudak, İlknur Hatice; Yüksel, Selçuk; Girişgen, İlknur; Aykota, Muhammed Raşid; Becerir, Tülay
    Purpose: Pediatric kidney transplantation (PKTx) is one of the most comfortable renal replacement therapies preferred in children with end-stage renal disease (ESRD) worldwide. Donor selection and identification of the underlying cause of renal failure in the recipient and individualisation of treatment are decisive factors for graft survival. The aim of this study is to present our results. Materials and methods: This single-center, retrospective study was conducted at Pamukkale University, Faculty of Medicine, Organ Transplantation Center. The PKTx was performed in 11 patients between December 2014 and November 2019. Results: The mean time from the beginning of the first dialysis session to transplantation was 40.2 months, and two patients were transplanted preemptively. The mean age of LD and DD transplants was 41.6 years and 17.1 years, respectively. This was attributed to the fact that, in the donation of cadaveric organs, donors under the age of 18 years are only registered for the waiting list for the recipients under the age of 18 years in our country. Conclusion: Our study showed that patients who used basiliximab for induction treatment were more advantageous in terms of infections than patients using anti-thymocyte globulin (ATG). © 2023, Pamukkale University. All rights reserved.
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    Pediatric kidney care experience after the 2023 Turkey/Syria earthquake
    (Oxford University Press, 2024) Bakkaloğlu, Sevcan A.; Delibaş, A.; Sürmeli Döven, Serra; Taner, Sevgin; Yavuz, Sevgi; Yüksel, Selçuk
    Background/aims: Two earthquakes on February 6th, 2023 destroyed ten cities in Türkiye. We report our experience with pediatric victims during these catastrophes, with a focus on crush syndrome related-acute kidney injury (Crush-AKI) and death. Method: A web-based software was prepared. Patient demographics, time under rubble (TUR), admission laboratory data, dialysis, and kidney and overall outcomes were asked. Results: 903 injured children (median age: 11.62 years) were evaluated. Mean TUR was 13 h (Interquartile range-IQR: 32.5), max 240 h). 31 of 32 patients with a TUR of >120 h survived. The patient who rescued after ten days survived.Two-thirds of the patients were given 50 mEq/L sodium-bicarbonate in 0.45% sodium-chloride solution on admission day. 58% of patients were given intravenous fluid (IVF) at a volume of 2000-3000 mL/m2 body surface area (BSA), 40% of 3000-4000 mL/m2 BSA, and only 2% of >4000 mL/m2 BSA. 425 patients had surgeries, 48 suffered from major bleeding. Amputations were recorded in 96 patients. Eighty-two and 66 patients required ventilator and inotropic support, respectively.Crush-AKI developed in 314 patients (36% of all patients). 189 patients were dialyzed. Age > 15 years, creatine phosphokinase (CK)≥20 950 U/L, TUR≥10 h, and the first-day IVF volume < 3000-4000 mL/m2 BSA were associated with Crush-AKI development. 22 deaths were recorded, 20 of 22 occurred in patients with Crush-AKI and within the first 4 days of admission. All patients admitted after 7 days survived. Conclusions: This is the most extensive pediatric kidney disaster data after an earthquake. Serum CK level was significantly associated with Crush-AKI at the levels of >20 950 U/L, but not with death. Adolescent age and initial IVF of less than 3000-4000 mL/m2 BSA were also asscoiated with Crush-AKI. Given that mildly injured victims can survive longer periods in the disaster field, we suggest uninterrupted rescue activity for at least 10 days.
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    Towards a standardized program of transitional care for adolescents with juvenile idiopathic arthritis for Turkey: a national survey study
    (BioMed Central Ltd, 2024) Sözeri, Betül; Şahin, Nihal; Açarı, Ceyhun; Avar Aydın, Pınar Özge; Baba, Özge; Yüksel, Selçuk
    Background: Juvenile idiopathic arthritis (JIA) is a prevalent childhood chronic arthritis, often persisting into adulthood. Effective transitional care becomes crucial as these patients transition from pediatric to adult healthcare systems. Despite the concept of transitional care being recognized, its real-world implementation remains inadequately explored. This study aims to evaluate the thoughts and practices of healthcare providers regarding transitional care for JIA patients. Methods: A cross-sectional survey was conducted among pediatric and adult rheumatologists in Turkey. Based on the American Academy of Pediatrics’ six core elements of transitional care, the survey included 86 questions. The respondents’ demographic data, attitudes towards transitional care, and practical implementation were assessed. Results: The survey included 48 rheumatologists, with 43.7% having a transition clinic. The main barriers to establishing transition programs were the absence of adult rheumatologists, lack of time, and financial constraints. Only 23.8% had a multidisciplinary team for transition care. Participants agreed on the importance of coordination and cooperation between pediatric and adult healthcare services. The timing of the transition process varied, with no consensus on when to initiate or complete it. Participants advocated for validated questionnaires adapted to local conditions to assess transition readiness. Conclusions: The study sheds light on the challenges and perspectives surrounding transitional care for JIA patients in Turkey. Despite recognized needs and intentions, practical implementation remains limited due to various barriers. Cultural factors and resource constraints affect the transition process. While acknowledging the existing shortcomings, the research serves as a ground for further efforts to improve transitional care and ensure better outcomes for JIA patients transitioning into adulthood.