Recurrence of membranoproliferative glomerulonephritis after renal transplantation in Denys–Drash
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Thomas J. Neuhaus &Walter Arnold & Ariana Gaspert & Helmut Hopfer & Andreas Fischer
- چاپ و سال / کشور: 2011
Description
Denys–Drash syndrome (DDS) consists of the triad of nephropathy, male pseudohermaphroditism, and Wilms tumor caused by mutations within exons 8 or 9 of the Wilms tumor suppressor gene 1. Early onset nephrotic syndrome progresses to end-stage renal failure. The characteristic histological lesion is diffuse mesangial sclerosis. Here, we report on a boy withDDS who presented early with diffuse mesangial sclerosis, but subsequently also developed immune complex glomerulonephritis with a membranoproliferative pattern (MPGN-pattern GN) in his native kidneys. Four years after renal transplantation, immune complex glomerulonephritis with an MPGN pattern recurred in the renal graft resulting in proteinuria and progressive renal insufficiency.
Pediatr Nephrol (2011) 26:317–322 DOI 10.1007/s00467-010-1669-9 Received: 20 July 2010 / Revised: 30 August 2010 / Accepted: 10 September 2010 / Published online: 28 October 2010