WAGR Flashcards

1
Q

What is the genetic defect?

A

Deletion of one copy of chromosome 11p13
WT1 gene = wilms
PAX6 gene = aniridia

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2
Q

What are the clinical manifestations of WAGR?

A
W = Wilms tumor (60%)
A = Aniridia
G = GU anomalies
R = mental Retardation
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