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WAGR syndrome

Disease definition

WAGR syndrome (Wilms tumor - aniridia - genitourinary anomalies - intellectual disability mental retardation) is a rare genetic disorder characterized by an unusual complex of congenital developmental abnormalities with intellectual disability, and an increased risk of developing Wilms tumor.

ORPHA:893

  • Synonym(s):
    • Del(11)(p13)
    • Deletion 11p13
    • Monosomy 11p13
    • Wilms tumor-aniridia-genitourinary anomalies-intellectual disability syndrome
  • Prevalence: -
  • Inheritance: Autosomal dominant 
  • Age of onset: Neonatal
  • ICD-10: Q87.8
  • OMIM: 194072  612469
  • UMLS: C0206115  C2931803
  • MeSH: C538295  D017624
  • GARD: 5528
  • MedDRA: -

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