Denys-Drash Syndrome

**Definition:** 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.

**Tree numbers:** - 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

**Synonyms:** - Drash Syndrome - Wilms Tumor and Pseudohermaphroditism - Nephropathy, Wilms Tumor, and Genital Anomalies - Pseudohermaphroditism, Nephron Disorder and Wilms' Tumor