Article
Epigenotyping as a tool for the prediction of tumor risk and tumor type in patients with Beckwith-Wiedemann syndrome (BWS).
The Journal of pediatrics - 1 Dec 2004
Bliek Jet, Gicquel Christine, Maas Saskia, Gaston Véronique, Le Bouc Yves, Mannens Marcel
Abstract excerpt
OBJECTIVES: Patients with Beckwith-Wiedemann syndrome (BWS) have a risk of 7.5% to 10% of developing childhood tumors, 60% of which are Wilms' tumors. Aberrant methylation of two distinct clusters of imprinted genes on chromosome 11p15 is detected in approximately 70% of BWS cases. Our aim was to determine associations between the imprinting status of both imprinting clusters (BWSIC1/2) and the tumor incidence...
Topics
- Beckwith-Wiedemann Syndrome
- Child, Preschool
- France
- Genes, Wilms Tumor
- Genotype
- Humans
- Incidence
- Kidney Neoplasms
- Membrane Proteins
- Methylation
- Neoplasms
- Netherlands
- Potassium Channels, Voltage-Gated
- Predictive Value of Tests
- Risk Factors
- Wilms Tumor