Browsing by Author "Fidan, Kibriya"

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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
Eculizumab treatment and discontinuation in pediatric patients with atypical hemolytic uremic syndrome: a multicentric retrospective study
(2022) Baskin, Esra; Fidan, Kibriya; Gulhan, Bora; Gulleroglu, Kaan; Canpolat, Nur; Yilmaz, Alev; Parmakiz, Gonul; Ozcakar, Birsin Z.; Ozaltin, Fatih; Soylemezoglu, Oguz; 35060104
Introduction Eculizumab is effective treatment of pediatric atypical hemolytic uremic syndrome (aHUS). However, the optimal duration of treatment is not clearly defined. The aim of this study was to retrospectively analyze the outcome of pediatric patients with aHUS, who started eculizumab treatment but discontinued it during the follow-up period. Methods The clinical and laboratory findings of the pediatric patients with aHUS were recorded on a web-based, national registry system, known as the Turkish aHUS Registry. The study included 63 patients who had to have received more than four doses of eculizumab during the acute phase of the disease. Results The median age at diagnosis was 3.62 (IQR: 1.29-6.14) years. During the follow-up period, 39 patients continued to receive standard eculizumab treatment (standard treatment group, treatment every 2 weeks) while 24 received an extended dose of eculizumab at three-four-week intervals (non-standard treatment group). There was no significant difference between both groups in terms of clinical and laboratory parameters. Eculizumab treatment was discontinued in 18 patients (30.7%, F/M:11/7), and the median age of these patients at diagnosis and their median follow-up duration were 4.0 (IQR:2.7-10.2) and 4.2 (IQR:2.2-7) years respectively. The median eGFR at the last visit was 110 (84.7-146.1)ml/min/1.73 m(2). Fourteen patients remained in remission without any sign of the disease. Recurrence occurred in four (22.2%) patients, in which eculizumab was immediately started again and complete remission was achieved. Conclusion Eculizumab is a successful treatment option in pediatric patients with aHUS and it can be safely discontinued with close monitoring in a selected group of patients. In case of recurrence, eculizumab should be restarted immediately to achieve complete remission. [GRAPHICS] .
Extra-Renal Manifestations of Atypical Hemolytic Uremic Syndrome in Children
(2018) Fidan, Kibriya; Goknar, Nilufer; Gulhan, Bora; Melek, Engin; Yildirim, Zeynep Y.; Baskin, Esra; Hayran, Mutlu; Gulleroglu, Kaan; Ozcakar, Zeynep B.; Ozaltin, Fatih; Soylemezoglu, Oguz; https://orcid.org/0000-0003-4361-8508; https://orcid.org/0000-0003-1434-3824; 29610995; B-5785-2018; AAJ-8833-2021
Atypical hemolytic uremic syndrome (aHUS) is a chronic disease characterized by thrombotic microangiopathy and a high risk of end-stage kidney disease. Dysregulation and/or excessive activation of the complement system results in thrombotic microangiopathy. Interest in extrarenal manifestations of aHUS is increasing. This study aimed to determine the clinical characteristics of patients with extrarenal manifestations of aHUS in childhood. This study included 70 children with extrarenal manifestations of HUS from the national Turkish aHUS Registry. The demographics, clinical characteristics, genetic test results, all treatments, and renal/hematologic status of aHUS patients with extrarenal involvement were recorded. The most common extrarenal manifestation was neurological system involvement (n = 46 [27.2%]), followed by gastrointestinal (n = 20 [11.8%]), cardiovascular (n = 12 [7%]), and respiratory (n = 12 [7%]) involvement. The patients with neurological involvement had a higher mortality rate and a lower estimated glomerular filtration rate (eGFR) than the other patients at last follow-up. Eculizumab (with or without plasma exchange/plasma infusion) treatment increased the renal and hematologic recovery rates. The most common and serious extrarenal manifestation of aHUS is neurological involvement and treatment outcome findings presented herein are important to all relevant clinicians.
Gastrointestinal System Involvement in Atypical Hemolytic Uremic Syndrome
(2018) Fidan, Kibriya; Yildirim, Zeynep Yuruk; Goknar, Nilufer; Gulhan, Bora; Gulleroglu, Kaan; Ozcakar, Zeynep Basin; Baskin, Esra; Hayran, Mutlu; Ozaltin, Fatih; Soylemezoglu, Oguz; https://orcid.org/0000-0003-1434-3824; https://orcid.org/0000-0003-4361-8508; F-3294-2013; B-5785-2018
Micronuclei and other nuclear anomalies in buccal epithelial cells of children with chronic kidney disease
(2016) Baskin, Esra; Aykanat, Banu; Demircigil, Gonca Cakmak; Buyan, Necla; Gulleroglu, Kaan; Fidan, Kibriya; Bayrakci, Umut Selda; Dalgic, Aydin; Karakayali, Hamdi; Haberal, Mehmet; Burgaz, Sema; 0000-0003-1434-3824; 0000-0003-4361-8508; 0000-0002-3462-7632; 28033104; B-5785-2018; AAJ-8833-2021; AAJ-8097-2021
The objective of this study was to reveal the likely genomic instability in children with chronic kidney disease (CKD) using micronucleus (MN) assay on buccal epithelial cells (BEC). We investigated the frequencies of micronuclei and other nuclear anomalies, such as nuclear buds, binucleated cells, condensed chromatin, and karyorrhectic and pyknotic cells in BEC. Children with CKD were grouped as follows: children in the pre-dialysis (PreD) stage (N=17), children on regular haemodialysis (HD) (N=14), and children who have undergone transplantation (Tx) (N=17). As a control group, twenty age-and gender-matched healthy children were selected. The MN frequency in BEC of all groups of children with CKD was significantly elevated (5-to 7-fold) as compared to the control group (p<0.001). In contrast, the frequencies of nuclear buds were not significantly higher in the study groups compared to the control group. The frequencies of binucleated cells and condensed chromatin cells were significantly higher in all subgroups of children with CKD relative to the control group (p<0.001). Our results show that the BEC of pediatric PreD, HD, and Tx patients with CKD display increased cytogenetic, cytokinetic, and cytotoxic effects. They also point to the sensitivity and usefulness of the BEC MN assay in the assessment of genetic susceptibility of patients with CKD.