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Congenital Syndromes
Syndrome
Presentation
Genetic
Change
Incidence of
Wilms’ Tumor
WAGR
Aniridia,
genitourinary
anomalies,
mental
retardation
Monoallelic
deletion at
chromosom
e 11p13
> 30%
Denys-Drash
Intersexual
disorders,
nephropathy
WT1 point
mutation
> 90%
Beckwith-
Wiedemann
Macroglossia,
organomegaly,
neonatal
hypoglycemia,
gigantism
Duplication
of paternal
allele
< 5%