Recurrence of membranoproliferative glomerulonephritis after renal transplantation in Denys-Drash.
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.
Department of Pediatrics, Children's Hospital Lucerne, Spitalstrasse, 6000, Lucerne 16, Switzerland. thomas.neuhaus@ksl.ch
Thomas J
TJ
Neuhaus
Walter
W
Arnold
Ariana
A
Gaspert
Helmut
H
Hopfer
Andreas
A
Fischer
D030321:Denys-Drash Syndrome / Q000235:genetics* / Q000473:pathology; D016162:Genes, Wilms Tumor; D015432:Glomerulonephritis, Membranoproliferative / Q000235:genetics* / Q000473:pathology*; D006801:Humans; D007231:Infant, Newborn; D007676:Kidney Failure, Chronic / Q000473:pathology / Q000601:surgery*; D016030:Kidney Transplantation; D008297:Male; D009154:Mutation; D012008:Recurrence; D009396:Wilms Tumor / Q000235:genetics / Q000473:pathology / Q000601:surgery