The association of Wilms' tumor with nephrologic disease.
Three cases of nephrologic disease characterized clinically by nephrotic syndrome and histologically with mesangial sclerosis associated with Wilms' tumor are documented. Two children were born with ambiguous genitalia and an XY chromosome constitution. A third patient had a 46,XY karyotype and developed a Wilms' tumor but had a normal female phonotype. The evaluation and current management of this syndrome are discussed.
Tank
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Division of Urologic Surgery, Oregon Health Sciences University, Portland 97201.
Melvin
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D002675:Child, Preschool; D012734:Disorders of Sex Development / Q000150:complications*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007680:Kidney Neoplasms / Q000150:complications*; D008297:Male; D009400:Nephrosclerosis / Q000150:complications* / Q000473:pathology; D013577:Syndrome; D009396:Wilms Tumor / Q000150:complications*