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De·nys-Drash syndrome(de-nēs' drash),
syndrome comprising nephropathy, Wilms tumor, and genital abnormalities.
Denys-Drash syndromeA hereditary condition (OMIM:194080) characterised by male pseudohermaphroditism, Wilms tumor, hypertension and degenerative renal disease.
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.
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.