Term Name: | Denys-Drash syndrome |
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Synonyms: | |
Definition: | A syndrome that is characterized by the association of diffuse mesangial sclerosis (DMS), male pseudohermaphroditism with a 46,XY karyotype, and nephroblastoma that derives_from an abnormality in the WT1 gene (Wilms' tumor suppressor gene). |
Ontology: | Human Disease [DOID:3764] ( DOID:3764 ) |