Published in:
01-02-2011 | Brief Report
Recurrence of membranoproliferative glomerulonephritis after renal transplantation in Denys–Drash
Authors:
Thomas J. Neuhaus, Walter Arnold, Ariana Gaspert, Helmut Hopfer, Andreas Fischer
Published in:
Pediatric Nephrology
|
Issue 2/2011
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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.