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Pediatrics Childhood tumors 01192e5e

Which of the following are not associated with Wilm's tumor

A
Aniridia
B
genitourinary abnormalities
C
Cataract
D
Hemihyperophy
High-Yield Explanation
WT1 is the best characterised Wilms tumor gene.It is located at chromosome 11p13 and encodes for a transcription factor that is critical for normal development of kidneys & gonads.WT2 is localised to a cluster of genes at 11p15.Children with some genetic syndromes are predisposed to developing Wilms tumor.These include WAGR Wilms tumor,aniridia,genitourinary abnormalities like horseshoe or fused kidney and mental retardation,del 11p13, Denys Drash syndrome(renal failure,renal mesangial sclerosis,male hermaphrodism,WT1 misense mutation) and Beckwith-Wiedeman syndrome(hemihyperophy,macroglossia,omphalocoele,organomegaly,del 11p15.5-WT2). Reference:Essential pediatrics-Ghai,8th edition,page no:617

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