Article
Association of partial gonadal dysgenesis, nephropathy and WT1 gene mutation without Wilms' tumor: incomplete Denys-Drash syndrome.
Journal of pediatric endocrinology & metabolism : JPEM - 1 May 2001
Cetinkaya E, Ocal G, Berberoğlu M, Adiyaman P, Ekim M, Yalçinkaya F, Orün E
Abstract excerpt
The concurrence of ambiguous genitalia, nephropathy and predisposition to Wilms' tumor are characteristics of Denys-Drash syndrome. Some of the reported patients do not express the full spectrum of the syndrome, while the occurrence of nephropathy has become a generally accepted common feature of this syndrome. We report an infant with male pseudohermaphroditism due to partial gonadal dysgenesis and nephropathy...
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