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.
Ponte
P
P
Department of Pediatrics, Hahnemann University, Philadelphia, Pennsylvania 19102.
Katz
S M
SM
Falkner
B
B
D001506:Beckwith-Wiedemann Syndrome / Q000473:pathology*; D006801:Humans; D007223:Infant; D007668:Kidney / Q000002:abnormalities / Q000473:pathology*; D008297:Male; D008854:Microscopy, Electron