Article
Surgical management and genotype/phenotype correlations in WT1 gene-related diseases (Drash, Frasier syndromes).
Journal of pediatric surgery - 1 Jan 2003
Auber F, Lortat-Jacob S, Sarnacki S, Jaubert F, Salomon R, Thibaud E, Jeanpierre C, Nihoul-Fékété C
Abstract excerpt
BACKGROUND/PURPOSE: The WT1 gene plays a role in urogenital and gonadal development. Germline mutations of this gene have been observed in patients with Drash or Frasier syndrome (Sd). The purpose of this report is to compare phenotype and genotype of these patients. METHODS: Retrospective study of 12 patients treated since 1980 for WT1 gene-related disorders was conducted. RESULTS: End-stage renal disease (ESRD)...
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