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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.

Year introduced: 2002

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Tree Number(s): C04.557.435.595.220, C04.588.945.947.535.585.220, C04.700.900.220, C12.050.351.875.253.096.562, C12.050.351.937.820.535.585.220, C12.050.351.968.419.473.585.220, C12.200.706.316.096.562, C12.200.758.820.750.585.220, C12.200.777.419.473.585.220, C12.800.316.096.562, C12.900.820.535.585.220, C12.950.419.473.585.220, C12.950.983.535.585.220, C16.131.939.316.096.562, C16.320.700.900.220, C19.391.119.096.562

MeSH Unique ID: D030321

Entry Terms:

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

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