儿童和青少年C3肾小球病变或免疫复合物膜增殖性肾小球肾炎的肾移植:当前国际实践调查

IF 1.4 4区 医学 Q3 PEDIATRICS
Christian Patry, Nicholas J A Webb, Matthias Meier, Lars Pape, Alexander Fichtner, Britta Höcker, Burkhard Tönshoff
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引用次数: 0

摘要

背景:大约50%由C3肾小球病变(C3G)或原发性免疫复合物膜增生性肾小球肾炎(IC-MPGN)引起的慢性肾病患者在发病的前10年内需要透析和/或肾移植(KTx)。目前,没有关于KTx的适应症或移植后管理的指南。方法:因此,我们通过survey Monkey发起了一项关于CKD 5期儿童C3G和IC-MPGN的国际在线调查。欧洲儿科肾病学会(ESPN)的所有KTx中心都被邀请参加了这项调查,该调查于2023年8月23日至11月25日进行。结果:65个(63%)中心(n = 103)参与了研究。26%的人至少有一次决定反对为患有C3G或IC-MPGN的儿童进行活体捐赠。88.2%的决定的主要原因是担心在任何潜在的移植中潜在疾病的复发。88%的人表示死者捐赠是一种选择;12%的人决定根本不进行移植。对于KTx的决策和管理,没有人提及任何国家或地区指南的现有建议。对于移植后C3G或IC-MPGN的复发,60%的应答者建议使用eculizumab治疗。结论:这项调查显示,儿科肾病学家相当不愿意将由于C3G或IC-MPGN导致的CKD 5期患者列入活体供肾移植名单。这一决定主要是基于对潜在疾病复发的恐惧,以及缺乏可靠的治疗方案。受影响的患者获得最佳肾衰竭治疗方案的机会有限,这需要进一步的行动。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Kidney Transplantation in Children and Adolescents With C3 Glomerulopathy or Immune Complex Membranoproliferative Glomerulonephritis: An International Survey of Current Practice.

Kidney Transplantation in Children and Adolescents With C3 Glomerulopathy or Immune Complex Membranoproliferative Glomerulonephritis: An International Survey of Current Practice.

Kidney Transplantation in Children and Adolescents With C3 Glomerulopathy or Immune Complex Membranoproliferative Glomerulonephritis: An International Survey of Current Practice.

Kidney Transplantation in Children and Adolescents With C3 Glomerulopathy or Immune Complex Membranoproliferative Glomerulonephritis: An International Survey of Current Practice.

Background: Approximately 50% of patients with chronic kidney disease due to C3 glomerulopathy (C3G) or primary immune-complex membranoproliferative glomerulonephritis (IC-MPGN) will require dialysis and/or kidney transplantation (KTx) within the first 10 years of disease onset. Currently, there are no guidelines regarding the indications for KTx or post-transplant management.

Methods: We therefore initiated an international online survey via Survey Monkey on C3G and IC-MPGN in children with CKD stage 5. All KTx centers of the European Society for Paediatric Nephrology (ESPN) were invited to participate in the survey, which was conducted from August 23 to November 25, 2023.

Results: Sixty-five (63%) of the centers (n = 103) participated. Twenty-six percent had made at least one decision against living donation for a child with C3G or IC-MPGN. The main reason for 88.2% of these decisions was concern about the recurrence of the underlying disease in any potential transplant. Eighty-eight percent indicated deceased donation as an option; 12% decided not to proceed with transplantation at all. Regarding KTx decision-making or management, none of them referred to an existing recommendation by any national or regional guideline. For the recurrence of C3G or IC-MPGN post-transplant, eculizumab treatment was suggested by 60% of respondents.

Conclusion: This survey shows a considerable reluctance of pediatric nephrologists to list patients with CKD stage 5 due to C3G or IC-MPGN for living donor kidney transplantation. This decision is mainly based on the fear of recurrence of the underlying disease combined with the lack of reliable treatment options. This limited access of affected patients to the best treatment option for kidney failure requires further action.

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来源期刊
Pediatric Transplantation
Pediatric Transplantation 医学-小儿科
CiteScore
2.90
自引率
15.40%
发文量
216
审稿时长
3-8 weeks
期刊介绍: The aim of Pediatric Transplantation is to publish original articles of the highest quality on clinical experience and basic research in transplantation of tissues and solid organs in infants, children and adolescents. The journal seeks to disseminate the latest information widely to all individuals involved in kidney, liver, heart, lung, intestine and stem cell (bone-marrow) transplantation. In addition, the journal publishes focused reviews on topics relevant to pediatric transplantation as well as timely editorial comment on controversial issues.
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