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| {{CMG}}; {{AE}} {{SSW}} | | {{CMG}}; {{AE}} {{SSW}} |
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| ==Overview==
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| __NOTOC__
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| {{CMG}}; {{AE}}
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| ==Overview==
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| == Overview ==
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| '''Genetic counseling'''
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| The frequency of malformations observed in patients with Wilms tumor underlines the need for genetic counseling, molecular and genetic explorations, and follow-up.
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| A French study concluded that patients need to be referred for genetic counseling if they have one of the following:
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| One major abnormality such as:
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| :*Beckwith-Wiedemann symptoms (macroglossia, neonatal or postnatal [[macrosomia]], abdominal wall defects, or visceromegaly); or
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| One condition such as:
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| :*Hemihyperplasia.
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| :*Overgrowth syndrome or [[mental retardation]].
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| :*Aniridia.
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| :*[[Diffuse mesangial sclerosis]].
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| Two or more minor malformations such as:
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| :*Inguinal or [[umbilical hernia]].
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| :*[[Hypospadias]].
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| :*Renal abnormalities.
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| :*[[Ectopic testis]].
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| Simple oncological follow-up is indicated when there is no malformation or when there is only one minor malformation.
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| After genetic counseling takes place, a search for WT1 mutations should be considered for patients who have the following:
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| :*Bilateral Wilms tumor.
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| :*Familial Wilms tumor.
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| :*Wilms tumor and age younger than 6 months.
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| :*Genitourinary abnormality.
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| :*Mental retardation association.
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| A search for an 11p15 abnormality should be considered for patients exhibiting any symptoms of Beckwith-Wiedemann syndrome, hemihyperplasia, or bilateral or familial Wilms tumor.<div align="left"></div>
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| <div align="left"></div>
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| ==References==
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| {{reflist|2}}
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| {{Metabolic pathology}}
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| [[Category:Hepatology]]
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Revision as of 22:00, 11 June 2018