THE RELATIONSHIP BETWEEN NEPHROMEGALY AND WILMS TUMOR IN CHILDREN WITH BECKWITH WIEDEMANN SYNDROME. † 849

Beckwith–Wiedemann syndrome and Wilms’ tumour

Patients with rare overgrowth disorders, such as Beckwith–Wiedemann syndrome and Simpson–Golabi– Behmel syndrome, are predisposed to embryonal tumours, including Wilms’ tumour of the kidney. Therefore, these disorders offer a link between hyperplastic growth and cancer. Genetic lesions at chromosome 11p15 have been associated with Beckwith–Wiedemann syndrome and Wilms’ tumour for several years ...

Screening for Wilms tumor and hepatoblastoma in children with Beckwith-Wiedemann syndromes: a cost-effective model.

BACKGROUND We undertook a cost-benefit analysis of screening for Wilms tumor and hepatoblastoma in children with Beckwith-Wiedemann syndrome (BWS), a known cancer predisposition syndrome. The purpose of this analysis was twofold: first, to assess whether screening in children with BWS has the potential to be cost-effective; second, if screening appears to be cost-effective, to determine which p...

[Beckwith-Wiedemann syndrome].

Beckwith-Wiedemann syndrome.

some llp15.3-.pter and monosomic for chromosome 5p15.3-.pter (karyotype 46,XY,der(5)t(5;ll)(pl5.3;pl5.3)pat). Paternally derived duplications of llpl5.5 are associated with Beckwith-Wiedemann syndrome (BWS) and both family members trisomic for llpl5.5 had prenatal overgrowth (birth weights >97th centile), macroglossia, coarse facial features, and broad hands. We review the clinical features of ...

Beckwith-Wiedemann Syndrome