Beckwith-Wiedemann syndrome

Question 1

Beckwith Widemann syndrome sketch:

Answer 1

• picture Kip from Napoleon dynamite to remind you of KIP2 on p57 gene
• he has a big tongue (Macroglossia)
• picture him with an omphalocele
• he is rubbing his ears to remind you of the linear earlobe
• Remember Wilm’s tumor! is the number one embryonal tumor in these patients