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Pediatrics General 2aa016b1

Wilm's tumor is associated with all the following except :

A
Aniridia
B
Beckwith syndrome
C
Polycystic kidney
D
Hemi – hypertrohy
High-Yield Explanation
Warn' s tamer : Wilm's tumor, also known as nephroblastoma, is the most common malignant tumor of kidney in children. Etiology. Most of the tumor are sporadic. 1- 2% may have family history. Familial predisposition to wilm's tumor is inherited in an autosornal dominant manner. Mutation of the following genes are seen in wilms tumor. Wilms tumor gene (WT 1) on chromosome 11. CTNNB I gene encoding the protooncogene beta - ca ten in. p53. Wilm's tumor is associated with three distinct syndromes. WAGR syndrome Beck with Wiedemnn syndrome Denys - Drash syndrome Wilm's tumor Aniridia Genital abnormalities Mental Retardation Enlargement of body organs Hemihypertrophy Renal medullary cysts Adrenal cytomegaly : abnormal large cells in adrenal cortex Gonadal dysgenesis (Male pseudo - hermaphrodite) Nephropathy leading to Renal failure

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