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Pediatr Nephrol. 2011 Feb;26(2):317-22. doi: 10.1007/s00467-010-1669-9. Epub 2010 Oct 28.

Recurrence of membranoproliferative glomerulonephritis after renal transplantation in Denys-Drash.

Author information

1
Department of Pediatrics, Children's Hospital Lucerne, Spitalstrasse, 6000, Lucerne 16, Switzerland. thomas.neuhaus@ksl.ch

Abstract

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 with DDS 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.

PMID:
21046168
DOI:
10.1007/s00467-010-1669-9
[Indexed for MEDLINE]

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