Membranoproliferative glomerulonephritis associated with a mutation in Wilms' tumour suppressor gene 1

Detlef Bockenhauer; William van't Hoff; Gil Chernin; Saskia F Heeringa; Neil J Sebire

doi:10.1007/s00467-009-1135-8

Membranoproliferative glomerulonephritis associated with a mutation in Wilms' tumour suppressor gene 1

Pediatr Nephrol. 2009 Jul;24(7):1399-401. doi: 10.1007/s00467-009-1135-8. Epub 2009 Feb 11.

Authors

Detlef Bockenhauer¹, William van't Hoff, Gil Chernin, Saskia F Heeringa, Neil J Sebire

Affiliation

¹ Nephrology, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London, WC1N 3JH, UK. Detlef.Bockenhauer@nhs.net

PMID: 19205749
DOI: 10.1007/s00467-009-1135-8

Abstract

Wilms' tumour suppressor gene 1 (WT1) encodes a transcription factor required for normal development of the genitourinary system. In the kidney, mutations in WT1 can cause diffuse mesangial sclerosis or focal segmental glomerulosclerosis. Here, we report on a girl with a mutation in WT1, who developed membranoproliferative glomerulonephritis (MPGN) 3 years after completion of treatment for Wilms' tumour. This finding extends the spectrum of glomerular disease seen with WT1 mutations and could have implications for the screening of children with MPGN.

Publication types

Case Reports

MeSH terms

Antineoplastic Agents / therapeutic use
Child
Child, Preschool
Combined Modality Therapy
Female
Genes, Wilms Tumor*
Glomerulonephritis, Membranoproliferative / complications
Glomerulonephritis, Membranoproliferative / genetics*
Glomerulonephritis, Membranoproliferative / physiopathology
Humans
Immunosuppressive Agents / therapeutic use
Infant
Kidney Transplantation
Mutation
Nephrectomy
Vincristine / therapeutic use
Wilms Tumor / complications
Wilms Tumor / genetics
Wilms Tumor / surgery

Substances

Antineoplastic Agents
Immunosuppressive Agents
Vincristine