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移植肾后局灶节段性肾小球硬化症的复发:策略与结果。

Recurrence of focal segmental glomerulosclerosis after kidney transplantation: strategies and outcome.

机构信息

Department of Pediatric Nephrology, Armand Trousseau Hospital (APHP) and University Paris 6 (UPMC), Paris, France.

出版信息

Curr Opin Organ Transplant. 2010 Oct;15(5):628-32. doi: 10.1097/MOT.0b013e32833dee3a.

Abstract

PURPOSE OF REVIEW

Steroid-resistant nephrotic syndrome/focal segmental glomerulonephritis (FSGS) is the primary renal disease in approximately 10% of pediatric patients receiving a renal allograft. Risk factors for recurrence are a chronological age between 6 and 15 years at onset of the nephrotic syndrome and a rapid progression of the disease in the native kidneys leading to end-stage renal disease in less than 3 years. With rapid recurrence of FSGS and loss of the allograft, further renal transplants also carry a high likelihood of recurrence of nephrotic syndrome.

RECENT FINDINGS

Different pathogenic factors have been discussed for the recurrence of proteinuria/FSGS in the transplanted kidney, especially the involvement of a proteinuric circulating factor. Treatment strategies are divided into two phases: induction of remission by plasma exchanges combined with high-dose intravenous or oral cyclosporine A; stabilization of remission by cyclophosphamide or rituximab, which showed promising results in several case reports.

SUMMARY

No controlled studies have been performed yet to address the management of recurrent FSGS posttransplant. Complications related to the high-degree immunosuppression are not rare and should be regularly investigated. Therefore, the benefit: risk ratio for all immunosuppressive treatment strategies should be carefully evaluated for each individual patient.

摘要

目的综述

类固醇耐药性肾病综合征/局灶节段性肾小球硬化症(FSGS)是约 10%接受肾移植的儿科患者的主要肾脏疾病。复发的危险因素是肾病综合征发病时的年龄在 6 至 15 岁之间,以及在不到 3 年内导致终末期肾病的肾病快速进展。由于 FSGS 的快速复发和移植物的丧失,进一步的肾移植也极有可能导致肾病综合征复发。

最新发现

对于移植肾中蛋白尿/FSGS 的复发,已经讨论了不同的发病机制,特别是蛋白尿循环因子的参与。治疗策略分为两个阶段:通过血浆置换联合大剂量静脉或口服环孢素 A 诱导缓解;通过环磷酰胺或利妥昔单抗稳定缓解,在几项病例报告中显示出良好的效果。

总结

尚未进行对照研究来解决移植后 FSGS 的复发问题。与高度免疫抑制相关的并发症并不罕见,应定期进行调查。因此,对于每个患者,都应仔细评估所有免疫抑制治疗策略的获益:风险比。

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