Browsing by Author "Gulhan, Bora"

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Audiological Findings In Distal Renal Tubular Acidosis
(2021) Ay, Ezgi; Alniacik, Asuman; Gurses, Emre; Arslan, Filiz; Gulhan, Bora; Alniacik, Asuman; Duzova, Ali; Bajin, Munir Demir; Sennaroglu, Levent; Genc, Gulsum Aydan; Ozaltin, Fatih; Topaloglu, Rezan; Başkent Üniversitesi
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.
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.
Follow-Up Results of Patients with ADCK4 Mutations and the Efficacy of Coq10 Treatment
(2017) Atmaca, Mustafa; Gulhan, Bora; Korkmaz, Emine; Inozu, Mihriban; Soylemezoglu, Oguz; Candan, Cengiz; Bayazit, Aysun Karabay; Elmaci, Ahmet Midhat; Parmaksiz, Gonul; Duzova, Ali; Besbas, Nesrin; Topaloglu, Rezan; Ozaltin, Fatih; https://orcid.org/0000-0003-2373-1837; 28337616; AAM-2935-2021
ADCK4-related glomerulopathy is an important differential diagnosis in adolescents with steroid-resistant nephrotic syndrome (SRNS) and/or chronic kidney disease (CKD) of unknown origin. We screened adolescent patients to determine the frequency of ADCK4 mutation and the efficacy of early CoQ10 administration. A total of 146 index patients aged 10-18 years, with newly diagnosed non-nephrotic proteinuria, nephrotic syndrome, or chronic renal failure and end-stage kidney disease (ESKD) of unknown etiology were screened for ADCK4 mutation. Twenty-eight individuals with bi-allelic mutation from 11 families were identified. Median age at diagnosis was 12.4 (interquartile range [IQR] 8.04-19.7) years. Upon first admission, all patients had albuminuria and 18 had CKD (6 ESKD). Eight were diagnosed either through the screening of family members following index case identification or during genetic investigation of proteinuria in an individual with a history of a transplanted sibling. Median age of these 8 patients was 21.5 (range 4.4-39) years. CoQ10 supplementation was administered following genetic diagnosis. Median estimated glomerular filtration rate (eGFR) just before CoQ10 administration was 140 (IQR 117-155) ml/min/1.73m(2), proteinuria was 1,008 (IQR 281-1,567) mg/m(2)/day. After a median follow-up of 11.5 (range 4-21) months following CoQ10 administration, proteinuria was significantly decreased (median 363 [IQR 175-561] mg/m(2)/day, P=0.025), whereas eGFR was preserved (median 137 [IQR 113-158] ml/min/1.73m(2), P=0.61). ADCK4 mutations are one of the most common causes of adolescent-onset albuminuria and/or CKD of unknown etiology in Turkey. CoQ10 supplementation appears efficacious at reducing proteinuria, and may thereby be renoprotective.
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
Results of Turkish Multicentric National Cystinosis Registry
(2015) Topaloglu, Rezan; Gulhan, Bora; Ozaltin, Fatih; Bodur, Ilknur; Besbas, Nesrin; Dursun, Hasan; Yilmaz, Alev; Gurgoze, Metin Kaya; Gokce, Ibrahim; Akinci, Nurver; Erdogan, Ozlem; Dursun, Ismail; Canpolat, Nur; Donmez, Osman; Cayci, Fatma Semsa; Serdaroglu, Erkin; Comak, Elif; Nalcacioglu, Hulya; Gok, Faysal; Yuksel, Selcuk; Soylu, Alper; Bahat, Elif; Hacihamdioglu, Duygu Ovunc; Candan, Cengiz; Bastug, Funda; AAW-8783-2020
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.
Turkish Atypical Hemolytic Uremic Syndrome Registry: Eculizumab Treatment İn Ahus Patients
(2018) Baskin, Esra; Canpolat, Nur; Gulleroglu, Kaan; Yilmaz, Alev; Melek, Engin; Yuksel, Selcuk; Gulhan, Bora; Kalyoncu, Mukaddes; Parmaksiz, Gonul; Ozcakar, Birsin; Ozaltin, Fatih; Soylemezoglu, Oguz; https://orcid.org/0000-0003-4361-8508; https://orcid.org/0000-0003-1434-3824; https://orcid.org/0000-0003-2373-1837; B-5785-2018; F-3294-2013; AAM-2935-2021