Clinical Spectrum and Tumour Risk Analysis in Patients with Beckwith-Wiedemann Syndrome Due to CDKN1C Pathogenic Variants

LCA Cardoso, A Parra, CR Gil, P Arias, N Gallego… - Cancers, 2022 - mdpi.com
Simple Summary Beckwith–Wiedemann syndrome (BWS) is an overgrowth disorder caused
by imprinting or genetic alterations at the 11p15. 5 locus. BWS is considered a spectrum …

Cancer risk in Beckwith-Wiedemann syndrome: a systematic review and meta-analysis outlining a novel (Epi) genotype specific histotype targeted screening protocol

A Mussa, C Molinatto, G Baldassarre, E Riberi… - The Journal of …, 2016 - Elsevier
Objective To compare tumor risk in the 4 Beckwith-Wiedemann syndrome (BWS) molecular
subgroups: Imprinting Control Region 1 Gain of Methylation (ICR1-GoM), Imprinting Control …

Improved molecular detection of mosaicism in Beckwith-Wiedemann Syndrome

SW Baker, KA Duffy, J Richards-Yutz… - Journal of medical …, 2021 - jmg.bmj.com
Background Beckwith-Wiedemann Syndrome (BWS) is characterised by overgrowth and
tumour predisposition. While multiple epigenetic and genetic mechanisms cause BWS, the …

(Epi) genotype–phenotype correlations in Beckwith–Wiedemann syndrome

A Mussa, S Russo, A De Crescenzo, A Freschi… - European journal of …, 2016 - nature.com
Beckwith–Wiedemann syndrome (BWS) is characterized by cancer predisposition,
overgrowth and highly variable association of macroglossia, abdominal wall defects …

Mutations of the Imprinted CDKN1C Gene as a Cause of the Overgrowth Beckwith–Wiedemann Syndrome: Clinical Spectrum and Functional Characterization

F Brioude, I Netchine, F Praz, M Le Jule… - Human …, 2015 - Wiley Online Library
Beckwith–Wiedemann syndrome (BWS) is an imprinting disorder associating macroglossia,
abdominal wall defects, visceromegaly, and a high risk of childhood tumor. Molecular …

High frequency of copy number variations (CNVs) in the chromosome 11p15 region in patients with Beckwith–Wiedemann syndrome

B Baskin, S Choufani, Y Chen, C Shuman, N Parkinson… - Human Genetics, 2014 - Springer
Abstract Beckwith–Wiedemann syndrome (BWS), an overgrowth and tumor predisposition
syndrome is clinically heterogeneous. Its variable presentation makes molecular diagnosis …

Imprinting status of 11p15 genes in Beckwith–Wiedemann syndrome patients with CDKN1C mutations

M Li, J Squire, C Shuman, J Atkin, R Pauli, A Smith… - Genomics, 2001 - Elsevier
Beckwith–Wiedemann syndrome (BWS) is an imprinting disorder characterized by somatic
overgrowth, congenital malformations, and predisposition to childhood tumors. Aberrant …

Molecular basis of Beckwith–Wiedemann syndrome spectrum with associated tumors and consequences for clinical practice

T Eggermann, ER Maher, CP Kratz, D Prawitt - Cancers, 2022 - mdpi.com
Simple Summary Beckwith–Wiedemann syndrome (BWS, OMIM 130650) is an inborn
overgrowth disorder caused by molecular alterations in chromosome 11p15. 5. These …

Introduction to the Beckwith–Wiedemann Syndrome and Cancer Special Issue

A Mussa, JM Kalish - Cancers, 2023 - mdpi.com
Beckwith–Wiedemann syndrome (BWS) is a genetic imprinting disorder that most commonly
presents as overgrowth, macroglossia, abdominal wall defects, lateralized overgrowth, and …

Variable Expressivity of the Beckwith-Wiedemann Syndrome in Four Pedigrees Segregating Loss-of-Function Variants of CDKN1C

A Sparago, F Cerrato, L Pignata, F Cammarata-Scalisi… - Genes, 2021 - mdpi.com
Beckwith-Wiedemann syndrome (BWS) is an imprinting disorder characterized by prenatal
and/or postnatal overgrowth, organomegaly, abdominal wall defects and tumor …