The Beckwith-Wiedemann syndrome phenotype and the risk of cancer
β Scribed by Schneid, H.; Vazquez, M.P.; Vacher, C.; Gourmelen, M.; Cabrol, S.; Le Bouc, Y.
- Publisher
- John Wiley and Sons
- Year
- 1997
- Tongue
- English
- Weight
- 112 KB
- Volume
- 28
- Category
- Article
- ISSN
- 0098-1532
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β¦ Synopsis
Beckwith-Wiedemann syndrome (BWS) comprises of a number of childhood abnormalities, often associated with one or more tumors. Thirty-eight patients were investigated to determine clinical and/or biological signs associated with a tumor presence. Our patients exhibited a higher incidence of tumor development (21%) than that previously reported, underlying the care with which such patients should be followed, when particular clinical features are observed: visceromegaly affecting three organs (liver, kidney, spleen), and also family history with sign of BWS such as macroglossia, omphalocele, hemihypertrophy, embryonic tumor), high body weight at birth (> or = +2 standard deviations and diastasis recti.
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## INAUGURAL DR. ClULlO 1. D'ANGIO AWARD of adults who have been both cured of their tumor and In 1940, the diagnosis of Wilms' tumor was associated with the same poor prognosis as that of other forms of childhood cancer. The addition of radiation therapy, and then chemotherapy to the management
The Beckwith-Wiedemann syndrome (BWS) is an overgrowth malformation syndrome that occurs with an incidence of 1:13,700 births. There is a striking incidence of childhood tumors found in BWS patients. Various lines of investigation have localized "imprinted" genes involved in BWS and associated child