Children with idiopathic hemihypertrophy and beckwith-wiedemann syndrome have different constitutional epigenotypes associated with wilms tumor.

Niemitz EL, Feinberg AP, Brandenburg SA, Grundy PE, DeBaun MR
Am J Hum Genet. 2005 77 (5): 887-91

PMID: 16252245 · PMCID: PMC1271394 · DOI:10.1086/497540

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.

MeSH Terms (7)

Beckwith-Wiedemann Syndrome Child Chromosomes, Human, Pair 11 DNA Methylation Genotype Humans Wilms Tumor

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