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Denys–Drash syndrome


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A rare disorder consisting of the triad of nephroblastoma (Wilms’ tumour), congenital nephropathy, and intersex disorders, resulting from mutations in the Wilm’s tumour suppressor gene (WT1). Incomplete forms exist; congenital nephropathy, with diffuse mesangial sclerosis, is the constant feature with either Wilms’ tumour or intersex disorders, usually in the form of male pseudohermaphroditism. P. Denys (20th century), French physician; A. Drash (20th century), British physician

Subjects: Medicine and Health.


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