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  • Síndrome caracterizada por FALÊNCIA RENAL CRÔNICA e DISGENESIA GONADAL nos fenótipos femininos com cariótipo de 46,XY ou indivíduo feminino com cariótipo normal 46,XX. É causada por mutações nos sítios de processamento do gene supressor do tumor de Wilms (GENES DO TUMOR DE WILMS) do doador no cromossomo 11.
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