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Beckwith-Wiedemann syndrome

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Definition:

Beckwith-Wiedemann syndrome is a congenital (present from birth) growth disorder that causes large body size, large organs, and other problems.

Causes, incidence, and risk factors:

The cause of Beckwith-Wiedemann syndrome is unknown, but it may be genetic. Eighty percent of cases are associated with a defect in chromosome number 11.

Infancy can be a critical period because of low blood sugar ( hypoglycemia ), omphalocele (when present), and an increased rate of tumor development, with Wilm's tumor and adrenal carcinoma being most common.

• Review Date: 6/13/2007
• Reviewed By: A.D.A.M. Editorial Team: Greg Juhn, M.T.P.W., David R. Eltz, Kelli A. Stacy. Previously reviewed by Daniel Rauch, M.D., FAAP, Director, Pediatric Hospitalist Program, Associate Professor of Pediatrics, NYU School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network (10/25/2006).

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