Denys-Drash Syndrome

A disorder of sex development characterized by UROGENITAL ABNORMALITIES; GONADAL DYSGENESIS; and WILMS TUMOR. It is caused by a mutation in the Wilms tumor suppressor gene (GENES, WILMS TUMOR) on chromosome 11.

Also Known As:

Nephropathy, Wilms Tumor, and Genital Anomalies; Pseudohermaphroditism, Nephron Disorder and Wilms' Tumor; Wilms Tumor and Pseudohermaphroditism; Denys Drash Syndrome; Syndrome, Denys-Drash; Syndrome, Drash; Drash Syndrome

Neoplasms: 1361496
- Hereditary Neoplastic Syndromes: 115
  - Wilms Tumor: 3953
    - Denys-Drash Syndrome: 65
- Neoplasms by Site: 4
  - Urogenital Neoplasms: 488
    - Urologic Neoplasms: 918
      - Kidney Neoplasms: 6420
        Wilms Tumor: 3953
        Denys-Drash Syndrome: 65
- Neoplasms by Histologic Type: 2
  - Complex and Mixed Neoplasms
    - Wilms Tumor: 3953
      - Denys-Drash Syndrome: 65
Urogenital Diseases: 129
- Urologic Diseases: 817
  - Kidney Diseases: 12764
    - Kidney Neoplasms: 6420
      - Wilms Tumor: 3953
        Denys-Drash Syndrome: 65
  - Urologic Neoplasms: 918
    - Kidney Neoplasms: 6420
      - Wilms Tumor: 3953
        Denys-Drash Syndrome: 65
- Urogenital Neoplasms: 488
  - Urologic Neoplasms: 918
    - Kidney Neoplasms: 6420
      - Wilms Tumor: 3953
        Denys-Drash Syndrome: 65
- Urogenital Abnormalities: 77
  - Disorders of Sex Development: 740
    - 46,XY Disorder of Sex Development: 474
      - Denys-Drash Syndrome: 65
- Female Urogenital Diseases and Pregnancy Complications
  - Female Urogenital Diseases
    - Urologic Diseases: 817
      - Kidney Diseases: 12764
        Kidney Neoplasms: 6420
        Wilms Tumor: 3953
        Denys-Drash Syndrome: 65
    - Urogenital Neoplasms: 488
      - Urologic Neoplasms: 918
        Kidney Neoplasms: 6420
        Wilms Tumor: 3953
        Denys-Drash Syndrome: 65
    - Urogenital Abnormalities: 77
      - Disorders of Sex Development: 740
        46,XY Disorder of Sex Development: 474
        Denys-Drash Syndrome: 65
- Male Urogenital Diseases
  - Urologic Diseases: 817
    - Kidney Diseases: 12764
      - Kidney Neoplasms: 6420
        Wilms Tumor: 3953
        Denys-Drash Syndrome: 65
  - Urogenital Neoplasms: 488
    - Urologic Neoplasms: 918
      - Kidney Neoplasms: 6420
        Wilms Tumor: 3953
        Denys-Drash Syndrome: 65
  - Urogenital Abnormalities: 77
    - Disorders of Sex Development: 740
      - 46,XY Disorder of Sex Development: 474
        Denys-Drash Syndrome: 65
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 960
- Congenital Abnormalities: 26404
  - Urogenital Abnormalities: 77
    - Disorders of Sex Development: 740
      - 46,XY Disorder of Sex Development: 474
        Denys-Drash Syndrome: 65
- Inborn Genetic Diseases: 12342
  - Hereditary Neoplastic Syndromes: 115
    - Wilms Tumor: 3953
      - Denys-Drash Syndrome: 65
Endocrine System Diseases: 996
- Gonadal Disorders: 30
  - Disorders of Sex Development: 740
    - 46,XY Disorder of Sex Development: 474
      - Denys-Drash Syndrome: 65

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