Carvajal Abreu Karla, Loos Sebastian, Fischer Lutz, Pape Lars, Wiech Thorsten, Kemper Markus J, Tönshoff Burkhard, Oh Jun, Schild Raphael
Department of Pediatric Nephrology, Pediatric Hepatology and Pediatric Transplantation, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Department of Hepatobiliary Surgery and Transplantation, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Front Pediatr. 2023 Sep 26;11:1280521. doi: 10.3389/fped.2023.1280521. eCollection 2023.
Early onset focal segmental glomerular sclerosis (FSGS) in the kidney allograft in patients without FSGS in the native kidney is a rare disorder in children. It usually occurs mostly beyond the first year after kidney transplantation and often leads to graft loss. Standardized treatment protocols have not yet been established.
We describe a boy with early onset FSGS in the transplanted kidney and non-selective glomerular proteinuria (maximum albumin-to-creatinine ratio of 3.8 g/g; normal range, ≤0.03 g/g creatinine). Manifestation occurred at 30 days posttransplant and was accompanied by a significant graft dysfunction (eGFR 61 ml/min per 1.73 m). Treatment with 25 sessions of plasmapheresis over 14 weeks and three consecutive days of methylprednisolone pulse therapy (10 mg/kg per day) followed by oral prednisolone as rejection prophylaxis (3.73 mg/m per day) led to sustained remission of proteinuria (albumin-to-creatinine ratio of 0.028 g/g) and normalization of graft function (eGFR 92 ml/min per 1.73 m) after 14 weeks. The follow-up period was 36 months.
This case underlines the efficacy of immunosuppressive and antibody eliminating therapy in early onset FSGS after kidney transplantation.
在无原发性局灶节段性肾小球硬化症（FSGS）的患儿中，肾移植后早期发生的FSGS是一种罕见疾病。它通常大多发生在肾移植后第一年之后，且常导致移植肾失功。目前尚未建立标准化的治疗方案。
我们报告一名患有移植肾早期FSGS且伴有非选择性肾小球性蛋白尿（最大白蛋白与肌酐比值为3.8 g/g；正常范围≤0.03 g/g肌酐）的男孩。症状在移植后30天出现，同时伴有显著的移植肾功能障碍（估算肾小球滤过率为61 ml/min/1.73m²）。在14周内进行了25次血浆置换治疗，并连续3天给予甲泼尼龙冲击治疗（每天10 mg/kg），随后口服泼尼松龙预防排斥反应（每天3.73 mg/m²），14周后蛋白尿持续缓解（白蛋白与肌酐比值为0.028 g/g），移植肾功能恢复正常（估算肾小球滤过率为92 ml/min/1.73m²）。随访期为36个月。
该病例强调了免疫抑制和抗体清除疗法在肾移植后早期FSGS治疗中的有效性。
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