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Long-term kidney function in children with Wilms tumour and constitutional WT1 pathogenic variant.

Maria Pia FalconeKathryn Pritchard-JonesJesper BrokWilliam MifsudRichard D WilliamsKayo NakataSuzanne TugnaitReem Al-SaadiLucy SideJohn AndersonCatriona DuncanStephen D MarksDetlef BockenhauerTanzina Chowdhury
Published in: Pediatric nephrology (Berlin, Germany) (2021)
Despite the known high risk of kidney disease in patients with WT and constitutional WT1 pathogenic variant, nearly two-thirds of patients had sustained native kidney function, suggesting that nephron-sparing surgery (NSS) should be attempted when possible without compromising oncological risk. Larger international studies are needed for accurate assessment of WT1genotype-kidney function phenotype correlation.
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