WAGR Syndrome

A contiguous gene syndrome associated with hemizygous deletions of chromosome region 11p13. The condition is marked by the combination of WILMS TUMOR; ANIRIDIA; GENITOURINARY ABNORMALITIES; and INTELLECTUAL DISABILITY.

Also Known As:

Syndrome, WAGR; 11p Partial Monosomy Syndrome; Chromosome 11p13 Deletion Syndrome; WAGR Complex; WAGR Contiguous Gene Syndrome; Wilms Tumor, Aniridia, Genitourinary Anomalies, And Mental Retardation Syndrome; Wilms Tumor-Aniridia-Genital Anomalies-Retardation Syndrome; Complex, WAGR; WAGR Complices; WAGR Syndromes; Contiguous Gene Syndrome, WAGR; Wilms Tumor, Aniridia, Genitourinary Anomalies, Mental Retardation Syndrome; Wilms Tumor-Aniridia-Genitourinary Anomalies-MR Syndrome; Wilms Tumor-Aniridia-Gonadoblastoma-Mental Retardation Syndrome

Neoplasms: 1324753
- Hereditary Neoplastic Syndromes: 112
  - Wilms Tumor: 3872
    - WAGR Syndrome: 26
- Neoplasms by Site: 4
  - Urogenital Neoplasms: 471
    - Urologic Neoplasms: 880
      - Kidney Neoplasms: 6284
        Wilms Tumor: 3872
        WAGR Syndrome: 26
- Neoplasms by Histologic Type: 2
  - Complex and Mixed Neoplasms
    - Wilms Tumor: 3872
      - WAGR Syndrome: 26
Nervous System Diseases: 14178
- Neurologic Manifestations: 7102
  - Neurobehavioral Manifestations: 1586
    - Intellectual Disability: 8035
      - WAGR Syndrome: 26
Eye Diseases: 3522
- Eye Abnormalities: 95
  - Aniridia: 470
    - WAGR Syndrome: 26
- Hereditary Eye Diseases: 18
  - Aniridia: 470
    - WAGR Syndrome: 26
- Uveal Diseases: 16
  - Iris Diseases: 5
    - Aniridia: 470
      - WAGR Syndrome: 26
Urogenital Diseases: 126
- Urologic Diseases: 799
  - Kidney Diseases: 12250
    - Kidney Neoplasms: 6284
      - Wilms Tumor: 3872
        WAGR Syndrome: 26
  - Urologic Neoplasms: 880
    - Kidney Neoplasms: 6284
      - Wilms Tumor: 3872
        WAGR Syndrome: 26
- Urogenital Neoplasms: 471
  - Urologic Neoplasms: 880
    - Kidney Neoplasms: 6284
      - Wilms Tumor: 3872
        WAGR Syndrome: 26
- Urogenital Abnormalities: 76
  - Disorders of Sex Development: 730
    - 46,XY Disorder of Sex Development: 472
      - WAGR Syndrome: 26
- Female Urogenital Diseases and Pregnancy Complications
  - Female Urogenital Diseases
    - Urologic Diseases: 799
      - Kidney Diseases: 12250
        Kidney Neoplasms: 6284
        Wilms Tumor: 3872
        WAGR Syndrome: 26
    - Urogenital Neoplasms: 471
      - Urologic Neoplasms: 880
        Kidney Neoplasms: 6284
        Wilms Tumor: 3872
        WAGR Syndrome: 26
    - Urogenital Abnormalities: 76
      - Disorders of Sex Development: 730
        46,XY Disorder of Sex Development: 472
        WAGR Syndrome: 26
- Male Urogenital Diseases
  - Urologic Diseases: 799
    - Kidney Diseases: 12250
      - Kidney Neoplasms: 6284
        Wilms Tumor: 3872
        WAGR Syndrome: 26
  - Urogenital Neoplasms: 471
    - Urologic Neoplasms: 880
      - Kidney Neoplasms: 6284
        Wilms Tumor: 3872
        WAGR Syndrome: 26
  - Urogenital Abnormalities: 76
    - Disorders of Sex Development: 730
      - 46,XY Disorder of Sex Development: 472
        WAGR Syndrome: 26
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Congenital Abnormalities: 25723
  - Chromosome Disorders: 136
    - WAGR Syndrome: 26
  - Eye Abnormalities: 95
    - Aniridia: 470
      - WAGR Syndrome: 26
  - Urogenital Abnormalities: 76
    - Disorders of Sex Development: 730
      - 46,XY Disorder of Sex Development: 472
        WAGR Syndrome: 26
- Inborn Genetic Diseases: 11939
  - Chromosome Disorders: 136
    - WAGR Syndrome: 26
  - Hereditary Neoplastic Syndromes: 112
    - Wilms Tumor: 3872
      - WAGR Syndrome: 26
  - Hereditary Eye Diseases: 18
    - Aniridia: 470
      - WAGR Syndrome: 26
Endocrine System Diseases: 968
- Gonadal Disorders: 30
  - Disorders of Sex Development: 730
    - 46,XY Disorder of Sex Development: 472
      - WAGR Syndrome: 26

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