The Denys-Drash syndrome.

sent with normal male external genitalia, the vast majority will appear phenotypically female or have ambiguous genitalia (table 1). The majority of cases of the Denys-Drash syndrome with any one of these three phenotypes of their external genitalia will have a normal male karyotype. The relative paucity of cases with a female karyotype may be because of underdiagnosis of the syndrome in phenotypic females with the nephropathy or as a result of underascertainment because of the previous poor survival of children with renal failure in infancy or early childhood. The findings in the internal genitalia in the Denys-Drash syndrome are extremely variable (table 2). While the internal genitalia in some of the cases will be appropriate for their exter-