title: Beckwith-Wiedemann syndrome. Renal pathology at 4 months of age.
abstract: 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.
authors.affiliation: Department of Pediatrics, Hahnemann University, Philadelphia, Pennsylvania 19102.
authors.forename: P
authors.initials: P
authors.lastname: Ponte
authors.forename: S M
authors.initials: SM
authors.lastname: Katz
authors.forename: B
authors.initials: B
authors.lastname: Falkner
mesh_terms: D001506:Beckwith-Wiedemann Syndrome / Q000473:pathology*; D006801:Humans; D007223:Infant; D007668:Kidney / Q000002:abnormalities / Q000473:pathology*; D008297:Male; D008854:Microscopy, Electron