title: Children with idiopathic hemihypertrophy and beckwith-wiedemann syndrome have different constitutional epigenotypes associated with wilms tumor.
abstract: Idiopathic hemihypertrophy (IH) is a congenital overgrowth syndrome associated with an increased risk of embryonal cancers in childhood. A related developmental disorder is Beckwith-Wiedemann syndrome (BWS), which increases risk for embryonal cancers, including Wilms tumor. Constitutional epigenetic alterations associated with BWS have been well characterized and include epigenetic alterations of imprinted genes on 11p15. The frequency of hypermethylation of H19 in children with IH and Wilms tumor, 20% (3/15), was significantly lower than the frequency in children with BWS and Wilms tumor, 79% (11/14; P = .0028). These results indicate that children with IH and Wilms tumor have different constitutional epigenotypes from those of children with BWS and Wilms tumor.
authors.affiliation: Predoctoral Program in Human Genetics, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
authors.forename: Emily L
authors.initials: EL
authors.lastname: Niemitz
authors.forename: Andrew P
authors.initials: AP
authors.lastname: Feinberg
authors.forename: Sheri A
authors.initials: SA
authors.lastname: Brandenburg
authors.forename: Paul E
authors.initials: PE
authors.lastname: Grundy
authors.forename: Michael R
authors.initials: MR
authors.lastname: DeBaun
mesh_terms: D001506:Beckwith-Wiedemann Syndrome / Q000235:genetics* / Q000473:pathology*; D002648:Child; D002880:Chromosomes, Human, Pair 11; D019175:DNA Methylation; D005838:Genotype; D006801:Humans; D009396:Wilms Tumor / Q000235:genetics*