Beckwith-Wiedemann syndrome. Renal pathology at 4 months of age

This report describes a child with nephromegaly and Beckwith-Wiedemann syndrome. The type and extent of the renal involvement could not be determined by imaging techniques. A pathologic examination of the renal biopsy tissue in this case demonstrated diffuse interstitial dysplasia. This is the first early pathologic description of the renal pathologic process in a living child with Beckwith-Wiedemann syndrome. The renal pathology indicates that children with this syndrome who survive other complications of the disorder have the potential for progressive renal disease.