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Beckwith–Wiedemann syndrome

✍ Scribed by Sanaa Choufani; Cheryl Shuman; Rosanna Weksberg


Publisher
John Wiley and Sons
Year
2010
Tongue
English
Weight
323 KB
Volume
154C
Category
Article
ISSN
1552-4868

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The Wiedemann-Beckwith syndrome (WBS) comprises an accumulation of multiple congenital anomalies. Exomphalos, macroglossia and gigantism are considered the most common manifestations, hence the alternative designation EMG-syndrome. The syndrome carries with it an increased risk of developing specifi

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Wiedemann-Beckwith syndrome (WBS) is a heterogeneous overgrowth syndrome associated with malformations and an elevated risk of developing embryonal tumors. WBS is a multigenic disorder caused by dysregulation of imprinted growth regulatory genes within the 11p15 region. Elucidation of the genetic ca

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Wiedemann-Beckwith syndrome (WBS) has already been noted to have asymmetric expression in monozygous (MZ) twins. Up to now 12 sets of MZ twins with WBS have been reported (Table I). To this series we would like to add 2 more sets of female twins, both discordant for WBS. TWIN PAIR 1 This set of twi