The Drash syndrome: male pseudohermaphroditism, nephritis, and Wilms tumor.

Abstract

The radiographic and clinical findings of 5 infants and children who had the combination of male pseudohermaphroditism, progressive nephritis and Wilms tumor are reported. The components of this syndrome are so striking that the radiologist can often suggest the diagnosis months to years before all 3 components are apparent if proteinuria, hypertension or an abdominal mass develop in any child with male pseudohermaphroditism. Since the progressive renal failure probably can be managed by dialysis or renal transplantation in some cases, prompt recognition and treatment of the Wilms tumor may permit the child to live a long and relatively normal life.