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补体介导的肾脏疾病与活体供肾移植:定制改善预后的方法。

Complement-Mediated Kidney Disease and Living Donor Transplantation: Tailoring Approaches to Improve Outcomes.

作者信息

Memon Aliza Anwar, Lentine Krista L, Caliskan Yasar

机构信息

Division of Nephrology, Department of Medicine, SSM Health Saint Louis University Hospital, St. Louis, MO, USA.

Saint Louis University Transplant Center, SSM Health Saint Louis University Hospital, St. Louis, MO, USA.

出版信息

Curr Transplant Rep. 2025;12(1). doi: 10.1007/s40472-025-00466-8. Epub 2025 Apr 16.

Abstract

PURPOSE OF REVIEW

To provide a comprehensive update on the evaluation of kidney transplant recipients with complement-mediated kidney diseases and their living donor (LD) candidates.

RECENT FINDINGS

Atypical hemolytic syndrome (aHUS) and C3 glomerulopathy (C3G) are rare complement-mediated diseases characterized by excessive activation of the alternative complement pathway. The evaluation of living kidney donor candidates for complement-mediated kidney diseases is evolving in response to emerging evidence and advancements in risk assessment tools. Criteria once considered contraindications to living donation are now part of standard practice, while novel genetic markers and risk factors are being identified. For complement-mediated kidney diseases, genetic testing is particularly relevant as it can identify variants that influence disease recurrence risk and donor suitability. Despite these advances, data to guide the evaluation of LD candidates for aHUS and C3G are still very limited. The application and interpretation of novel genetic testing technologies remain in the early stages, and standardized guidance is lacking. In this review, we summarize the approach to LD kidney transplantation for complement-mediated kidney diseases, addressing utility of genetic testing, risks, and ongoing challenges for recipients and LDs.

SUMMARY

The present review highlights the importance and complexity of kidney transplantation from an LD for patients with complement-related kidney disorders and motivates further research to determine the optimal risk-assessment for LD candidates to recipients with aHUS and C3G.

摘要

综述目的

全面更新对患有补体介导性肾脏疾病的肾移植受者及其活体供者(LD)候选人的评估。

最新发现

非典型溶血性尿毒症综合征(aHUS)和C3肾小球病(C3G)是罕见的补体介导性疾病,其特征为替代补体途径过度激活。鉴于新出现的证据和风险评估工具的进展,对补体介导性肾脏疾病的活体肾供者候选人的评估正在不断发展。曾经被视为活体捐赠禁忌的标准如今已成为常规做法的一部分,同时新的基因标记和风险因素也在不断被识别。对于补体介导性肾脏疾病,基因检测尤为重要,因为它可以识别影响疾病复发风险和供者适用性的变异。尽管有这些进展,但指导aHUS和C3G的LD候选人评估的数据仍然非常有限。新型基因检测技术的应用和解读仍处于早期阶段,且缺乏标准化指南。在本综述中,我们总结了补体介导性肾脏疾病的LD肾移植方法,探讨了基因检测的效用、风险以及受者和LD面临的持续挑战。

总结

本综述强调了为患有补体相关肾脏疾病的患者进行LD肾移植的重要性和复杂性,并促使进一步开展研究,以确定针对aHUS和C3G受者的LD候选人的最佳风险评估方法。

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本文引用的文献

1
Evaluation for genetic disease in kidney transplant candidates: A practice resource.
Am J Transplant. 2025 Feb;25(2):237-249. doi: 10.1016/j.ajt.2024.10.019. Epub 2024 Oct 31.
2
Prognosis is still poor in patients with posttransplant C3 glomerulopathy despite eculizumab use.
Clin Kidney J. 2024 Jun 20;17(7):sfae190. doi: 10.1093/ckj/sfae190. eCollection 2024 Jul.
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Int J Mol Sci. 2024 Jun 13;25(12):6508. doi: 10.3390/ijms25126508.
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Rare Variants in Complement Gene in C3 Glomerulopathy and Immunoglobulin-Mediated Membranoproliferative GN.
Clin J Am Soc Nephrol. 2023 Nov 1;18(11):1435-1445. doi: 10.2215/CJN.0000000000000252. Epub 2023 Aug 24.
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Genetic evaluation of living kidney donor candidates: A review and recommendations for best practices.
Am J Transplant. 2023 May;23(5):597-607. doi: 10.1016/j.ajt.2023.02.020. Epub 2023 Mar 1.

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