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Medullary sponge kidneys and unilateral Wilms tumour in a child with Beckwith-Wiedemann syndrome
Authors:R Beetz  O Schofer  H Riedmiller  R Schumacher  P Gutjahr
Institution:(1) Department of Paediatrics, University Hospital, Langenbeckstrasse 1, W-6500 Mainz 1, Germany;(2) Department of Urology, University Hospital, Langenbeckstrasse 1, W-6500 Mainz 1, Germany
Abstract:The occurrence of a Wilms tumour in a 4-year-old girl with bilateral medullary sponge kidney, Beckwith-Wiedemann syndrome and congenital hemihypertrophy demonstrates the close relationship between these disorders. Another six cases from the literature with congenital hemihypertrophy and with medullary sponge kidney are discussed, two of them also developed intra-abdominal neoplasm.
Keywords:Beckwith-Wiedemann syndrome  Congenital hemihypertrophy  Medullary sponge kidney  Wilms tumour
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