Oleg Shapiro1, Thomas R. Welch2, Maryellen Sheridan1, Susan Stred2, Jyoti Upadhyay1
Canadian Journal of Urology, Vol.14, No.6, pp. 3767-3769, 2007
Abstract Objective: We present a child with Denys-Drash syndrome recognized after surgery for mixed gonadal dysgenesis, and discuss screening procedures the urologist should consider in similar circumstances.
Case report: A 1-year-old child with XY gonadal dysgenesis underwent genital reconstruction. The postoperative period was complicated by incisional drainage, which led to the recognition of a nephrotic syndrome. Molecular analysis of the WT-1 gene confirmed a mutation associated with the Denys-Drash syndrome.
Conclusion: The Denys-Drash syndrome should be suspected in children with XY gonadal dysgenesis. The presence of urine protein should be sought in such children, and if present, consultation More >
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