Pediatric Focal Segmental Glomerulosclerosis: Favorable Transplantation Outcome With Plasma Exchange

Abstract Background: Although kidney transplantation (KT) is the treatment of choice for pediatric kidney failure (KF); concerns for recurrence in cases of focal segmental glomerulosclerosis (FSGS) are still present. This study aimed to investigate the outcome of KT in children with KF secondary to FSGS, with implementation of preemptive perioperative plasma exchange (PE) for non-genetically proven patients.Methods: 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. Sporadic patients electively received 6 preoperative PE sessions. Recurrence of proteinuria was managed with PE and Rituximab (RTX). Kaplan-Meier curves were used to analyze graft and recurrence free survival data.Results: The mean follow-up duration after KT 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 KT) in 2 patients (5%). 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 KT and 2 patients' mortality (5%) occurred early postoperative (first 2 weeks) due to infection.Conclusion: FSGS transplanted children have favorable outcomes with perioperative PE for non-genetically proven cases. Early recurrence after KT can be successfully managed with PE and RTX.