Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2005 Nov;77(5):887-91.
doi: 10.1086/497540. Epub 2005 Oct 3.

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

Emily L Niemitz  1 , Andrew P Feinberg, Sheri A Brandenburg, Paul E Grundy, Michael R DeBaun
Affiliations

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

Emily L Niemitz et al. Am J Hum Genet. 2005 Nov.

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.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Analysis of H19 methylation in patients with BWS and IH. A 1.0-kb fragment of the H19 gene was hybridized to genomic DNA digested with PstI and the methylcytosine-sensitive restriction enzyme SmaI. The upper band represents the 1.8-kb methylated fragment, and the lower band represents the 1.0-kb unmethylated digested fragment. Patient numbers corresponding to those in the tables are shown below the lanes. All DNA specimens were from the normal kidney. fk = fetal kidney.
Figure 2
Figure 2
Analysis of LIT1 methylation in patients with BWS and IH. An EST probe from the LIT1 gene was hybridized to genomic DNA digested with BamH1 and the methylcytosine-sensitive restriction enzyme NotI. The upper band represents the 6.0-kb unmethylated fragment, and the lower band represents the 4.2-kb unmethylated digested fragment. Patient numbers corresponding to those in the tables are shown below the lanes. All DNA specimens were from the normal kidney except samples for IH13, IH14, and IH17, which were from the Wilms tumor. fk = fetal kidney.
See this image and copyright information in PMC

References

Web Resources

    1. Online Mendelian Inheritance in Man (OMIM), http://www.ncbi.nlm.gov/Omim/ (for BWS and IH) - PubMed

References

    1. Breslow N, Beckwith JB, Ciol M, Sharples K (1988) Age distribution of Wilms’ tumor: report from the National Wilms’ Tumor Study. Cancer Res 48:1653–1657 - PubMed
    1. Cui H, Horon IL, Ohlsson R, Hamilton SR, Feinberg AP (1998) Loss of imprinting in normal tissue of colorectal cancer patients with microsatellite instability. Nat Med 4:1276–128010.1038/3260 - DOI - PubMed
    1. DeBaun MR, Niemitz EL, McNeil DE, Brandenburg SA, Lee MP, Feinberg AP (2002) Epigenetic alterations of H19 and LIT1 distinguish patients with Beckwith-Wiedemann syndrome with cancer and birth defects. Am J Hum Genet 70:604–611 - PMC - PubMed
    1. DeBaun MR, Tucker MA (1998) Risk of cancer during the first four years of life in children from The Beckwith-Wiedemann Syndrome Registry. J Pediatr 132:398–400 - PubMed
    1. Feinberg AP, Tycko B (2004) The history of cancer epigenetics. Nat Rev Cancer 4:143–153 - PubMed

Publication types