Department of Pediatrics, Pediatric Nephrology Unit, Kasr Al-Ainy Faculty of Medicine, Cairo University, Cairo, Egypt.
Department of Pediatrics, National Research Center, Cairo, Egypt.
Ital J Pediatr. 2021 Dec 14;47(1):236. doi: 10.1186/s13052-021-01188-0.
Although kidney transplantation (KTX) is the treatment of choice for pediatric end stage kidney disease (ESKD); concerns for recurrence in cases of focal segmental glomerulosclerosis (FSGS) are still present. This study aimed to investigate the outcome of KTX in children with ESKD secondary to FSGS, with implementation of preemptive perioperative plasma exchange (PE) for non-genetically proven patients.
Forty FSGS pediatric kidney transplant recipients were studied. Of them: 12 patients (30%) had genetically proven NPHS2 mutations/familial and 28 (70%) were sporadic FSGS patients. All sporadic patients electively received 6 perioperative PE sessions. Patients with recurrence of proteinuria (n = 13; including 3 patients with genetic/familial and 10 patients with sporadic FSGS) were managed with PE and Rituximab (RTX). Kaplan-Meier curves were used to analyze graft and recurrence free survival data.
The mean follow-up duration after KTX was 3.8 ± 2.86 years. Recurrence of proteinuria was encountered early postoperative in 11 patients (27.5%) and late (1.6 and 2.9 years after KTX) in 2 patients (5%). All patients with early recurrence achieved complete remission, while patients with late recurrence developed graft failure. Current serum creatinine and proteinuria levels were not different in patients received PE (n = 31) and patients did not PE (n = 9) (p = 0.308 and 0.287 respectively). Current serum creatinine and proteinuria levels in sporadic patients (n = 28) after prophylactic perioperative PE were not different from those of genetic/ familial patients (n = 12) (p = 0.303 and 0.144 respectively). Proteinuria was less in patients underwent native nephrectomy than others immediately postoperative and at assessment (p = 0.002 & 0.0031 respectively). One-year graft and patient survival was 93.8% with a mean 1-year serum creatinine of 0.67 ± 0.25 mg/dl. Three graft losses (7.5%) were due to chronic rejection 3.3, 3.75 and 4.17 years after KTX and 2 patients' mortality (5%) occurred early postoperative (first 2 weeks).
FSGS transplanted children have favorable outcomes with perioperative PE for non-genetically proven cases. Early recurrence after KTX can be successfully managed with PE and RTX.
虽然肾移植（KTX）是儿科终末期肾病（ESKD）的首选治疗方法，但对于局灶节段性肾小球硬化症（FSGS）患者的复发仍存在担忧。本研究旨在探讨对 FSGS 继发的 ESKD 患儿进行 KTX 的结果，对非遗传性证实的患者实施预防性围手术期血浆置换（PE）。
研究了 40 例 FSGS 儿科肾移植受者。其中：12 例（30%）患者有遗传性 NPHS2 突变/家族性，28 例（70%）为散发性 FSGS 患者。所有散发性患者均选择性接受 6 次围手术期 PE 治疗。出现蛋白尿复发（n=13；包括 3 例遗传性/家族性和 10 例散发性 FSGS）的患者接受 PE 和利妥昔单抗（RTX）治疗。采用 Kaplan-Meier 曲线分析移植物和无复发存活率数据。
KTX 后平均随访 3.8±2.86 年。11 例（27.5%）患者在术后早期出现蛋白尿复发，2 例（5%）患者在术后晚期（1.6 和 2.9 年后）出现蛋白尿复发。所有早期复发的患者均达到完全缓解，而晚期复发的患者则出现移植物失功。接受 PE（n=31）和未接受 PE（n=9）的患者当前的血清肌酐和蛋白尿水平无差异（p=0.308 和 0.287）。接受预防性围手术期 PE 的散发性患者（n=28）的当前血清肌酐和蛋白尿水平与遗传性/家族性患者（n=12）无差异（p=0.303 和 0.144）。与其他患者相比，即刻术后和评估时行肾切除术的患者蛋白尿更少（p=0.002 和 0.0031）。1 年移植物和患者存活率为 93.8%，平均 1 年血清肌酐为 0.67±0.25mg/dl。3 例移植物失功（7.5%）归因于慢性排斥反应，分别发生在 KTX 后 3.3、3.75 和 4.17 年，2 例患者（5%）在术后早期（前 2 周）死亡。
FSGS 移植患儿接受围手术期 PE 治疗非遗传性证实的患者，预后良好。KTX 后早期复发可通过 PE 和 RTX 成功治疗。
