The Denys-Drash syndrome.

Drash et all described two unrelated children who presented with ambiguous genitalia, both of whom had a unilateral Wilms' tumour, and subsequently developed progressive renal fail- ure.Denys et aP had previously described a child with male pseudohermaphroditism, Wilms' tumour, and a nephropathy in associ- ation with sex chromosomal mosaicism.Review of published reports shows 150 cases 64 of what has subsequently been called the Drash or, more correctly, the Denys-Drash syndrome.