Orphanet: Denys Drash syndrome

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Denys-Drash syndrome

Disease definition

A rare genetic, syndromic glomerular disorder characterized by the association of nephropathy presenting as persistent proteinuria or overt nephrotic syndrome, Wilms tumor and genitourinary structural defects. In addition, disorders of testicular development are common in subjects with 46,XY karyotype.


Classification level: Disorder
  • Synonym(s):
    • Drash syndrome
    • Wilms tumor-DSD syndrome
    • Wilms tumor-disorder of sex development syndrome
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: Childhood, Adolescent, Infancy, Neonatal
  • ICD-10: N04.1
  • OMIM: 194080
  • UMLS: C0950121
  • MeSH: D030321
  • GARD: 5576
  • MedDRA: 10070179

Detailed information


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