Denys-Drash syndrome

De·nys-Drash syndrome

(de-nēs' drash),

syndrome comprising nephropathy, Wilms tumor, and genital abnormalities.

Denys-Drash syndrome

A hereditary condition (OMIM:194080) characterised by male pseudohermaphroditism, Wilms tumor, hypertension and degenerative renal disease.

Molecular pathology
Defects of WTI, which encodes a transcription factor that plays an essential role in the normal development of the urogenital system, cause Denys-Drash syndrome.

Denys-Drash syndrome

(dĕn′ĭs-drăsh′)

A rare congenital syndrome caused by a mutation in the Wilms' tumor gene that includes male pseudohermaphroditism, progressive renal failure, and an increased risk for genitourinary tumors.