A case of Denys-Drash syndrome without renal manifestations followed by end stage renal disease after completion of treatment for Wilms tumor

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  • ウイルムス腫瘍発症時は腎症状なく治療終了後に末期腎不全に至ったDenys-Drash症候群の1例

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Abstract

A six-year-old girl was diagnosed to have Wilms tumor. Urinalysis at that time showed no proteinuria, but proteinuria was detected at the age of eight, after completion of treatment for lung metastasis. Gene analysis showed mutation of the WT1 gene, and she was diagnosed with Denys-Drash syndrome. The impaired kidney function later progressed to renal failure, and she had renal transplantation from her mother at the age of 14. Currently she is doing well with no signs of recurrence, eight years after lung metastasis. It is advised that patients with risk factors for renal failure should be identified at as early a stage of diagnosis as passible.

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