Denys-Drash syndrome


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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.
References in periodicals archive ?
Louise suffers from Denys-Drash syndrome, a very rare condition typically affecting young children, which results in the infant developing a Wilms tumour.
People with Denys-Drash syndrome have a 90% chance of developing this rare form of tumour.
horseshoe kidney, hypospadias, undescended testes), congenital aniridia, WAGR syndrome (aniridia, mental retardation, genito-urinary abnormalities), congenital hemihypertrophy, Beckwith-Wiedemann syndrome, and Denys-Drash syndrome (renal disease, pseudo-hermaphroditism).
Background: Denys-Drash Syndrome (DDS) is an uncommon disorder that appears sporadically and in rare cases may be inherited as an autosomal dominant trait.
Rudin C, Pritchard J, Fernando ON: Renal transplantation in the management of bilateral Wilms' tumour (BWT) and of Denys-Drash syndrome (DDS).
1,8,10] In order to rule out the presence of Denys-Drash syndrome, which occurs as a consequence of mutations at the WT1 gene on chromosome 11, patients with DMP must be examined periodically for the presence of Wilms tumor and nephropathy with progressive renal failure.
One patient (patient 12) had features of Denys-Drash syndrome.