Denys-Drash syndromeDenys-Drash syndrome is an autosomal dominant complex consisting of gonadal dysgenesis, nephropathy, and Wilms tumor. The condition shows no racial predilection. The accurate estimate of male-to-female ratio is problematic because the syndrome may manifest with intersex disorders. The majority of the cases is caused by a mutation in the WT-1 gene, the Wilms tumor associated gene, on chromosome 11 (11q13).
The key morphologic feature of Denys-Drash syndrome is diffuse mesangial sclerosis. Clinically it presents shortly after birth with proteinuria and progresses to renal failure within one to four years. Renal involvement may occur in isolation or in association with male pseudohermaphroditism and nephroblastoma (Wilms tumor).