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活体相关肾移植中伴有补体基因变异的急性血栓性微血管病导致早期移植物失功:病例系列报告。

Early graft loss due to acute thrombotic microangiopathy accompanied by complement gene variants in living-related kidney transplantation: case series report.

机构信息

National Clinical Research Center of Kidney Diseases, Jinling Hospital, Medical School of Nanjing University, Nanjing, Jiangsu, China.

Department of Kidney Transplantation, Nephropathy Hospital, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shanxi, China.

出版信息

BMC Nephrol. 2022 Jul 14;23(1):249. doi: 10.1186/s12882-022-02868-7.

Abstract

BACKGROUND

Recently, early graft loss has become very rare in living-related kidney transplantation (LKT) as a result of decreased risk of hyperacute rejection and improvements in immunosuppressive regimens. Post-transplant acute thrombotic microangiopathy (TMA) is a rare, multi-factorial disease that often occurs shortly after kidney transplantation and is usually resistant to treatment with dismal renal outcomes. The complement genetic variants may accelerate the development of TMA. However, the complement genetic test was seldom performed in unknown native kidney disease recipients scheduled for LKT.

CASE PRESENTATION

We reported three cases of unknown native kidney diseases who had fulminant TMA in the allograft shortly after LKT. Both the donors and the recipients were noted to carry complement genetic variants, which were identified by genetic testing after transplantation. However, all recipients were refractory to treatment and had allograft loss within 3 months after LKT.

CONCLUSION

This case series highlights the suggestion to screen complement gene variants in both the donors and the recipients with unknown native kidney diseases scheduled for LKT.

摘要

背景

由于急性排斥反应风险降低和免疫抑制方案的改进,活体相关肾移植(LKT)中的早期移植物失功已变得非常罕见。移植后急性血栓性微血管病(TMA)是一种罕见的多因素疾病,通常在肾移植后不久发生,且通常对治疗有抗性,导致预后不良。补体遗传变异可能会加速 TMA 的发展。然而,对于计划接受 LKT 的未知原发性肾脏疾病受者,很少进行补体遗传检测。

病例介绍

我们报告了 3 例在 LKT 后不久移植物中发生暴发性 TMA 的未知原发性肾脏疾病患者。供者和受者均携带补体遗传变异,这些变异是在移植后通过基因检测确定的。然而,所有受者均对治疗无反应,且在 LKT 后 3 个月内均发生移植物失功。

结论

本病例系列强调了对计划接受 LKT 的未知原发性肾脏疾病供者和受者进行补体基因变异筛查的建议。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/63d4/9284761/5dfed43aaf06/12882_2022_2868_Fig1_HTML.jpg

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