title: Recurrent focal segmental glomerulosclerosis in grafts treated with plasma exchange and increased immunosuppression.
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.
authors.affiliation: Department of Nephrology, Great Ormond Street Hospital for Children NHS Trust, London, UK.
authors.forename: M A
authors.initials: MA
authors.lastname: Saleem
authors.forename: A V
authors.initials: AV
authors.lastname: Ramanan
authors.forename: L
authors.initials: L
authors.lastname: Rees
mesh_terms: D000293:Adolescent; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000209:etiology* / Q000628:therapy*; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007165:Immunosuppression Therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016030:Kidney Transplantation; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D010951:Plasma Exchange; D011183:Postoperative Complications; D012008:Recurrence
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone