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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.

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Department of Pediatrics, Children's Hospital Lucerne, Spitalstrasse, 6000, Lucerne 16, Switzerland.


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.

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