Tıp Fakültesi / Faculty of Medicine

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Author: search.filters.author.Baskin, EsraSubject: search.filters.subject.Atypical hemolytic uremic syndrome

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    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.
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    Unusual eye findings in a patient with atypical hemolytic uremic syndrome: Answers
    (2022) Avci, Begum; Ozdek, Sengul; Akkoyun, Imren; Baskin, Esra; 0000-0002-5375-379X; 0000-0002-2860-7424; 35084569; AAK-7713-2021
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    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] .