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Immediate post-transplant nephrosis in a patient with congenital nephrotic syndrome
Authors:Sahar S Barayan  Samhar I Al-Akash  Mohammed Malekzadeh  Jennifer L Marik  Arthur H Cohen  Robert B Ettenger  O Yadin
Institution:(1) Division of Pediatric Nephrology, UCLA Children’s Hospital, 10833 Le Conte Ave., Los Angeles, CA 90095-1752, USA e-mail: Oyadin@mednet.ucla.edu Tel.: +1-310-2066987, Fax: +1-310-8250442, US;(2) Cedar-Sinai Medical Center, Department of Pathology, Los Angeles, California, USA, US
Abstract:A 19-month-old girl with congenital nephrotic syndrome of the Finnish type underwent a living-related renal transplant; 24 h after transplantation she became massively nephrotic. She did not respond to steroids, plasmapheresis, and high-dose cyclosporine. A month later, a renal biopsy showed only glomerular foot process effacement. She was treated with high-dose methylprednisolone pulses and oral cyclophosphamide. She rapidly went into complete remission with no further relapses. Graft function has been stable 2 years after transplantation. Received: 24 June 1999 / Revised: 21 February 2001 / Accepted: 23 February 2001
Keywords:  Congenital nephrotic syndrome  Renal transplantation  Corticosteroids  Cyclophosphamide  Post-transplant nephrotic syndrome
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