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Recurrent focal segmental glomerulosclerosis in grafts treated with plasma exchange and increased immunosuppression
M A Saleem1, A V Ramanan, L Rees
1Department of Nephrology, Great Ormond Street Hospital for Children NHS Trust, London, UK.
Insights
This study investigated a treatment protocol for recurrent focal segmental glomerulosclerosis (FSGS) in pediatric kidney transplant patients. The protocol showed potential for improving renal function and reducing proteinuria, even in anuric patients, but carries significant risks.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Transplantation Immunology
Background:
- Focal segmental glomerulosclerosis (FSGS) is a leading cause of nephrotic syndrome in children, often leading to kidney transplant.
- Recurrence of FSGS post-transplant is a significant challenge, necessitating effective treatment strategies.
- Previous studies suggested a treatment protocol involving methylprednisolone, plasma exchange, and cyclophosphamide for recurrent FSGS.
Observation:
- Three pediatric patients with severe, recurrent FSGS in their first allografts were treated with methylprednisolone, plasma exchange, and cyclophosphamide, alongside cyclosporine A and prednisolone.
- All patients experienced recurrence within 24 hours post-transplant, with treatment initiated within 48 hours.
- All three patients developed anuria and required dialysis.
Findings:
- Despite anuria, one boy achieved stable chronic renal failure (CRF) with no proteinuria at 3 years post-transplant.
- One girl experienced improvement with CRF and non-nephrotic proteinuria at 3 years, after 4 months of dialysis.
- The third patient remained dialysis-dependent and unfortunately died from septic complications.
Implications:
- The reported treatment protocol may offer long-term benefits in renal function and proteinuria reduction for recurrent FSGS, even in anuric transplant recipients.
- Prolonged treatment duration may be necessary, highlighting the need for careful monitoring.
- The intensive immunosuppression regimen carries substantial risks, including infection, necessitating a balanced approach to treatment intensity.
Abstract:
We report on three children with severe, recurrent focal segmental glomeruloscerosis (FSGS) in their first allografts, treated with methylprednisolone, plasma exchange and cyclophosphamide. This protocol is based on a previous publication showing its successful use in three children. Our patients were 2 girls and 1 boy, aged 14.5, 14.6 and 13.2 years, respectively, at transplant. Concomitant immunosuppression included cyclosporin A and prednisolone. Recurrence occurred in all three patients within 24 h, and specific treatment was commenced within 48 h. All patients developed anuria and were dialysed. The boy stopped dialysis after 4 weeks, and has stable chronic renal failure (CRF) and no proteinuria 3 years later. One girl required dialysis for 4 months, and 3 years later has CRF with non-nephrotic range proteinuria. The other girl remained dialysis-dependent and died from septic complications. We conclude that even anuric patients treated with this protocol may have an improvement in renal function and reduction of proteinuria, which can last for over 3 years. However, treatment may need to be prolonged and carries the substantial risks of heavy immunosuppression.