Browsing by Author "Ozcakar, Zeynep Birsin"

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Clinical Course Of Adolescent Onset Atypıcal Hemolytic Uremic Syndrome: A Study Of Turkish Ahus Registry
(2022) Celegen, Kubra; Gulhan, Bora; Fidan, Kibriya; Yuksel, Selcuk; Yilmaz, Neslihan; Yilmaz, Aysun Caltik; Kilic, Beltinge Demircioglu; Gokce, Ibrahim; Tufan, Asli Kavaz; Kalyoncu, Mukaddes; Nalcacioglu, Hulya; Ozlu, Sare Gulfem; Sukur, Eda Didem Kurt; Canpolat, Nur; Bayazit, Aysun K.; Koyun, Mustafa; Tabel, Yilmaz; Tulpar, Sebahat; Celakil, Mehtap; Bek, Kenan; Zeybek, Cengiz; Duzova, Ali; Ozcakar, Zeynep Birsin; Topaloglu, Rezan; Soylemezoglu, Oguz; Ozaltin, Fatih
Could Plasma Based Therapies Still Be Considered in Selected Cases with Atypical Hemolytic Uremic Syndrome?
(2021) Ozlu, Sare Gulfem; Gulhan, Bora; Aydog, Ozlem; Atayar, Emine; Delibas, Ali; Parmaksiz, Gonul; Ozdogan, Elif Bahat; Comak, Elif; Tasdemir, Mehmet; Acar, Banu; Ozcakar, Zeynep Birsin; Topaloglu, Rezan; Soylemezoglu, Oguz; Ozaltin, Fatih; 35023648
Background. Atypical hemolytic uremic syndrome (aHUS) occurs due to defective regulation of the alternative complement pathway (ACP) on vascular endothelial cells. Plasma based therapy (PT) was the mainstay of the treatment for aHUS for many years until the introduction of therapies targeting blockage of the complement system. The aim of this study was to evaluate patients with aHUS who had been treated with plasma based therapies alone. Methods. The outcomes of seven genetically confirmed aHUS patients (2 girls, 5 males) were evaluated by means of clinical presentation, response to plasma therapy, course of the disease during the follow-up period and last status. Results. The median age of the patients at admission was 6.7 years (IQR 0.7-7.8). Three patients received plasma exchange therapy and the other four patients were treated with plasma infusions. One patient was lost to follow-up after one year; the median duration of follow-up for other patients was 3.7 years (IQR 2.7-6.5). During the follow up, two patients from our historical records when complement blocking therapies had not been in clinical use yet in Turkey, underwent kidney transplantation. One transplant patient experienced an acute rejection episode without graft loss. The remaining five patients had a glomerular filtration rate of more than 90 ml/min./1.73 m(2) at the last visit. Conclusion. Although we had a relatively small patient population, our findings indicate that PT might still be considered in selected patients particularly in countries where complement blocking therapies are difficult to reach due to their unavailability or costs that are not covered by the health care systems.
Transplantation in pediatric aHUS within the era of eculizumab therapy
(2020) Ozcakar, Zeynep Birsin; Ozaltin, Fatih; Gulhan, Bora; Comak, Elif; Parmaksiz, Gonul; Baskin, Esra; Topaloglu, Rezan; Kasap Demir, Belde; Canpolat, Nur; Yuruk Yildirim, Zeynep; Demircioglu Kilic, Beltinge; Yuksel, Selcuk; Soylemezoglu, Oguz; 0000-0003-4361-8508; 33217100; B-5785-2018
aHUS is caused by the over-activation and dysregulation of the alternative complement pathway. Data regarding outcomes of pediatric aHUS patients after kidney transplantation are still very scarce. Accordingly, the aim of this study was to describe the clinical findings and outcomes of pediatric aHUS patients after renal transplantation. This is a retrospective, multicenter study including 12 patients from the national registry system. Among the 12 patients, eight had received prophylactic eculizumab and none of those patients (except one) had experienced aHUS recurrence during a median follow-up period of 58.5 (min-max, 4-94) months. Although eculizumab had been started on the day before transplantation in one of them, aHUS recurrence occurred during the transplantation procedure. Eculizumab had been stopped in only one patient who had no complement gene mutation after 35 months of therapy, and recurrence had not been observed during the 19 months of follow-up. In three patients, maintenance doses had been spaced out without any recurrence. One additional patient with anti-CFH antibody received only two doses of eculizumab for transplantation and had been followed for 46 months without aHUS recurrence. The remaining three patients had not received anti-C5 therapy and none of those patients experienced aHUS recurrence during a median follow-up period of 21 (min-max, 9-42) months. Prophylactic eculizumab is a safe and effective treatment for the prevention of aHUS recurrence. Eculizumab interval prolongation, discontinuation, and transplantation without eculizumab prophylaxis can be tried in selected patients with close follow-up.