Urological implications of the beckwith-wiedemann syndrome.
A significant number of children with the Beckwith-Wiedemann syndrome are prone to the development of nephroblastomas and adrenal carcinomas. Renomegaly, a prolonged tubular blush on excretory urography similar to that of medullary sponge kidney, and previously undescribed caliceal diverticula may be observed. Diligent followup is mandatory to detect early neoplasia. Suggestions are detailed based on reported case management.
D000310:Adrenal Gland Neoplasms / Q000150:complications; D002675:Child, Preschool; D003456:Cryptorchidism / Q000150:complications; D004431:Ear, External / Q000002:abnormalities; D006801:Humans; D007682:Kidney Pelvis / Q000002:abnormalities; D008260:Macroglossia / Q000150:complications*; D008265:Macrostomia / Q000150:complications*; D008297:Male; D009506:Nevus / Q000150:complications*; D013577:Syndrome; D013737:Testis / Q000637:transplantation; D014182:Transplantation, Autologous