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Denys-Drash syndrome

Disease definition

Denys-Drash syndrome (DDS) is a rare urogenital disorder characterized by the association of diffuse mesangial sclerosis (DMS), male pseudohermaphroditism with a 46,XY karyotype, and nephroblastoma.


  • Synonym(s):
    • Drash syndrome
    • Wilms tumor-DSD syndrome
    • Wilms tumor-disorder of sex development syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: -
  • Age of onset: Infancy, Neonatal
  • ICD-10: N04.1
  • OMIM: 194080
  • UMLS: C0950121
  • MeSH: D030321
  • GARD: 5576
  • MedDRA: 10070179

Detailed information


Additional information

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