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Recurrence of membranoproliferative glomerulonephritis after renal transplantation in Denys-Drash


Neuhaus, T J; Arnold, W; Gaspert, A; Hopfer, H; Fischer, A (2011). Recurrence of membranoproliferative glomerulonephritis after renal transplantation in Denys-Drash. Pediatric Nephrology, 26(2):317-322.

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.

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.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Pathology and Molecular Pathology
Dewey Decimal Classification:610 Medicine & health
Date:February 2011
Deposited On:12 Mar 2011 13:25
Last Modified:17 Feb 2018 13:11
Publisher:Springer
ISSN:0931-041X
Additional Information:The original publication is available at www.springerlink.com
OA Status:Closed
Publisher DOI:https://doi.org/10.1007/s00467-010-1669-9
PubMed ID:21046168

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