Children with idiopathic hemihypertrophy and Beckwith-Wiedemann syndrome have different constitutional epigenotypes associated with Wilms tumor

Emily L. Niemitz, Andrew P. Feinberg, Sheri A. Brandenburg, Paul E. Grundy, Michael R. DeBaun

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)887-891
Number of pages5
JournalAmerican journal of human genetics
Volume77
Issue number5
DOIs
StatePublished - Nov 2005

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

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