Patry Christian, Webb Nicholas J A, Meier Matthias, Pape Lars, Fichtner Alexander, Höcker Britta, Tönshoff Burkhard
Department of Pediatrics I, University Children's Hospital Heidelberg, Medical Faculty, Heidelberg University, Heidelberg, Germany.
Novartis Pharma AG, Basel, Switzerland.
Pediatr Transplant. 2025 Mar;29(2):e70048. doi: 10.1111/petr.70048.
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.
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.
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.
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.
约50%因C3肾小球病(C3G)或原发性免疫复合物膜增生性肾小球肾炎(IC-MPGN)导致慢性肾脏病的患者在疾病发作后的前10年内需要透析和/或肾移植(KTx)。目前,尚无关于KTx适应症或移植后管理的指南。
因此,我们通过Survey Monkey对5期慢性肾脏病儿童的C3G和IC-MPGN开展了一项国际在线调查。邀请了欧洲儿科肾脏病学会(ESPN)的所有KTx中心参与该调查,调查于2023年8月23日至11月25日进行。
103个中心中有65个(63%)参与。26%的中心至少做出过一次反对为患有C3G或IC-MPGN的儿童进行活体捐赠的决定。这些决定中88.2%的主要原因是担心任何潜在移植中基础疾病的复发。88%的中心表示接受 deceased捐赠作为一种选择;12%的中心决定完全不进行移植。关于KTx决策或管理,没有一个中心参考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%的受访者建议使用依库珠单抗治疗。
这项调查显示,儿科肾脏病医生相当不愿意将因C3G或IC-MPGN导致5期慢性肾脏病的患者列入活体供肾移植名单。这一决定主要基于对基础疾病复发的担忧以及缺乏可靠的治疗选择。受影响患者获得肾衰竭最佳治疗选择的机会有限,这需要进一步采取行动。