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小儿肾移植后补体相关肾脏疾病的复发和结局。

Recurrence and Outcomes of Complement-Related Renal Disease After Pediatric Renal Transplantation.

机构信息

From the Department of Pediatric Nephrology, Baskent University, Ankara, Turkey.

出版信息

Exp Clin Transplant. 2020 Jan;18(Suppl 1):82-83. doi: 10.6002/ect.TOND-TDTD2019.P28.

Abstract

Complement dysregulation is related to different glomerular pathologies. Patients with complement dysregulation have high recurrence risk after transplant; however, with trough-effective therapeutics, renal transplant can be an option for these patients. Here, we present 2 boys with renal disease related to complement dysregulation and their outcomes after renal transplant. Patient 1 had atypical hemolytic uremic syndrome, which was treated with eculizumab before renal transplant; eculizumab therapy was also continued after transplant as preventive therapy. Eculizumab therapy was stopped at year 2 post-transplant. At year 4 post-transplant, his serum creatinine level was 0.87 mg/dL. Patient 2, who had chronic renal disease related to C3 glomerulopathy, was not responsive to eculizumab before renal transplant. At month 4 posttransplant, C3 glomerulopathy recurrence was demonstrated with biopsy, and serum creatinine level was 1.96 mg/dL at this time. Eculizumab was started as a rescue therapy. At year 4 posttransplant, his serum creatinine level was 2.07 mg/dL. In our 2 patients with complement dysregulation, eculizumab was an effective and preventive therapy after renal transplant. However, more studies are needed to understand the long-term efficacy and safety of eculizumab after renal transplant.

摘要

补体失调与不同的肾小球病理有关。补体失调的患者在移植后复发风险较高;然而,通过谷值有效的治疗,肾移植可以成为这些患者的选择。在此,我们介绍了 2 例与补体失调相关的肾脏疾病患儿及其肾移植后的结局。患者 1 患有非典型溶血尿毒综合征,在肾移植前接受依库珠单抗治疗;移植后也继续进行依库珠单抗治疗作为预防治疗。移植后 2 年停用依库珠单抗。移植后 4 年,其血清肌酐水平为 0.87mg/dL。患者 2 患有与 C3 肾小球病相关的慢性肾脏疾病,在肾移植前对依库珠单抗无反应。移植后 4 个月,活检显示 C3 肾小球病复发,此时血清肌酐水平为 1.96mg/dL。开始依库珠单抗作为挽救治疗。移植后 4 年,其血清肌酐水平为 2.07mg/dL。在我们的 2 例补体失调患者中,肾移植后依库珠单抗是一种有效且预防性的治疗方法。然而,需要更多的研究来了解肾移植后依库珠单抗的长期疗效和安全性。

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