Beckwith-Wiedemann syndrome

Beckwith-Wiedemann syndrome (BWS) is a congenital overgrowth disorder characterised by unique set features that can consist of:

The incidence is reported at ~1:13,000-37,000 live births.

The majority of cases are sporadic while a small proportion (~15%) is autosomal dominant with abnormality related to chromosome 11p15.5. The syndrome carries a well recognised increased risk for the development of neoplasia 4. Another 10-20 of cases show paternal disomy 11.

Associations

First described by Beckwith and Wiedemann in 1965.

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Article Information

rID: 7412
System: Paediatrics
Section: Syndromes
Tag: cases
Synonyms or Alternate Spellings:
  • BWS
  • Beckwith Wiedemann syndrome (BWS)

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    BWS
    Case 1: chest radiograph
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    BWS

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    Case 1: pelvic radiograph
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    BWS
    Case 1: scintigraphy
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    Case 2
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