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溶血性尿毒症综合征与肾移植:病例系列及文献综述

Hemolytic Uremic Syndrome and Kidney Transplantation: A Case Series and Review of the Literature.

作者信息

Milan Manani Sabrina, Virzì Grazia Maria, Giuliani Anna, Clementi Anna, Brocca Alessandra, Dissegna Daniela, Martino Francesca, d''Amore Emanuele Stefano Giovanni, Ronco Claudio

机构信息

Department of Nephrology, Dialysis and Transplantation, San Bortolo Hospital, Vicenza, Italy.

出版信息

Nephron. 2017;136(3):245-253. doi: 10.1159/000468528. Epub 2017 Apr 19.

Abstract

BACKGROUND

Hemolytic uremic syndrome (HUS) can be triggered by Shiga toxin producing Escherichia coli (STEC) infection or it can be defined as atypical HUS (aHUS) if it is related to uncontrolled complement activation. aHUS is characterized by a high incidence of recurrence after kidney transplantation, and it can also occur de novo in transplant recipients. Eculizumab is used both to prevent and to treat aHUS following kidney transplantation. In this paper, we report our centre experience and we present 4 cases of HUS in patients who underwent kidney transplantation.

METHODS

This is a single-center experience about HUS development in transplanted patients.

RESULTS

Patient 1 with end-stage renal disease (ESRD) due to STEC-HUS undergoing kidney transplantation without prophylactic therapy with eculizumab. Patient 2 with HUS secondary to an episode of diarrhea at 8 years old. After a slow progression to ESRD, she underwent kidney transplantation and she received prophylactic therapy with eculizumab due to the presence of anti-complement factor H antibodies. Patient 3 underwent pre-emptive living donor ABO-incompatible kidney transplantation and developed HUS secondary to antibody-mediated rejection. Patient 4 developed de novo HUS 16 years after kidney transplantation without a known cause.

CONCLUSION

The correct diagnosis of HUS and the identification of the complement component alterations in case of aHUS are important parameters required to predict the risk of post-transplant recurrence of the disease. In the cases we reported, eculizumab has been found to be effective both to prevent and to treat aHUS following kidney transplantation.

摘要

背景

溶血尿毒综合征(HUS)可由产志贺毒素大肠杆菌(STEC)感染引发,若与补体激活失控相关则可定义为非典型HUS(aHUS)。aHUS的特征是肾移植后复发率高,也可在移植受者中新发。依库珠单抗用于预防和治疗肾移植后的aHUS。在本文中,我们报告了我们中心的经验,并介绍了4例肾移植患者发生HUS的病例。

方法

这是一项关于移植患者发生HUS的单中心经验。

结果

患者1因STEC-HUS导致终末期肾病(ESRD),在未接受依库珠单抗预防性治疗的情况下接受肾移植。患者2在8岁时因腹泻发作继发HUS。在缓慢进展至ESRD后,她接受了肾移植,由于存在抗补体因子H抗体,她接受了依库珠单抗预防性治疗。患者3接受了抢先的活体供体ABO血型不相容肾移植,并因抗体介导的排斥反应继发HUS。患者4在肾移植16年后无已知原因新发HUS。

结论

HUS的正确诊断以及aHUS病例中补体成分改变的识别是预测疾病移植后复发风险所需的重要参数。在我们报告的病例中,已发现依库珠单抗在预防和治疗肾移植后的aHUS方面均有效。

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