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局灶节段性肾小球硬化症/激素耐药性肾病综合征复发的临床实践建议。

Clinical practice recommendations for recurrence of focal and segmental glomerulosclerosis/steroid-resistant nephrotic syndrome.

机构信息

Faculty of Medicine, University Hospital of Cologne, Children's and Adolescents' Hospital, Pediatric Nephrology, University of Cologne, Cologne, Germany.

Department of Pediatrics, University Children's Hospital Heidelberg, Heidelberg, Germany.

出版信息

Pediatr Transplant. 2021 May;25(3):e13955. doi: 10.1111/petr.13955. Epub 2020 Dec 30.

Abstract

Recurrence of primary disease is one of the major risks for allograft loss after pediatric RTx. The risk of recurrence of FSGS/SRNS after pediatric RTx in particular can be up to 86% in idiopathic cases. There is a need for consensus recommendations on its prevention and treatment. The CERTAIN study group has therefore performed a thorough literature search based on the PICO model of clinical questions to formulate educated statements to guide the clinician in the process of decision-making. A set of educated statements on prevention and treatment of FSGS/SRNS after pediatric RTx has been generated after careful evaluation of available evidence and thorough panel discussion. We do not recommend routine nephrectomy prior to transplantation; neither do we recommend abstaining from living donation. Special attendance needs to be given to those patients who had already experienced graft loss due to FSGS/SRNS recurrence. Early PE or IA with or without high-dose CsA and/or rituximab seems to be most promising to induce remission. The educated statements presented here acknowledge that FSGS/SRNS recurrence after pediatric RTx remains a major concern and is associated with shorter graft survival or even graft loss. The value of any recommendation needs to take into account that evidence is based on cohorts that differ in ethnicity, pre-transplant history, immunosuppressive regimen, definition of recurrence (eg, clinical and/or histological diagnosis) and treatment modalities of recurrence.

摘要

原发性疾病复发是小儿肾移植后移植物丢失的主要风险之一。特别是特发性 FSGS/SRNS 患儿在接受小儿肾移植后复发的风险可能高达 86%。因此,有必要就其预防和治疗达成共识建议。为此,CERTAIN 研究小组根据临床问题的 PICO 模型进行了全面的文献检索,制定了有教育意义的声明,以指导临床医生在决策过程中的工作。在仔细评估现有证据并进行深入的小组讨论后,我们制定了一套关于小儿肾移植后 FSGS/SRNS 预防和治疗的有教育意义的声明。我们不建议在移植前常规行肾切除术;也不建议放弃活体供肾。需要特别关注那些因 FSGS/SRNS 复发而已经经历过移植物丢失的患者。早期进行 PE 或 IA 联合或不联合大剂量 CsA 和/或利妥昔单抗似乎最有希望诱导缓解。这里提出的有教育意义的声明承认,小儿肾移植后 FSGS/SRNS 复发仍然是一个主要关注点,与移植物存活率降低甚至移植物丢失有关。任何建议的价值都需要考虑到证据是基于不同种族、移植前病史、免疫抑制方案、复发定义(例如,临床和/或组织学诊断)和复发治疗方式的队列。

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