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Article

Prophylactic bilateral nephrectomies in two paediatric patients with missense mutations in the WT1 gene

11 Dec 2003

Abstract excerpt

BACKGROUND: Denys-Drash syndrome (DDS) is associated with mutations of the Wilms' tumour 1 (WT1) gene, and is characterized by pseudohermaphroditism, a progressive glomerulopathy, and the development of Wilms' tumour. More than 90% of patients with DDS who carry constitutional intragenic WT1 mutations are at high risk (90%) for the development of Wilms' tumour. WT1 is a signalling protein with 90% of WT1...

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PMID: 14671061
DOI: 10.1093/ndt/gfg473

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