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SÃndrome de Beckwith-Wiedemann
Definición:
El sÃndrome de Beckwith-Wiedemann es un agrupamiento consistente de hallazgos de etiologÃa desconocida (causa) y caracterizados por lengua grande ( macroglosia ), órganos grandes ( visceromegalia ), talla corporal grande (macrosomÃa), hernia umbilical u onfalocele ( hernia del ombligo) y azúcar baja en la sangre en el recién nacido ( hipoglicemia neonatal).
Causas, incidencia y factores de riesgo:
La causa del sÃndrome de Beckwith-Wiedemann se desconoce, pero parece tener origen genético. De hecho, algunos casos pueden estar asociados con un defecto en el cromosoma 11.
Los niños afectados son grandes al nacer y muchos tienen un defecto en la pared abdominal, como una hernia umbilical o un onfalocele. Tienen una apariencia facial caracterÃstica con una boca abierta y una lengua grande.
La infancia es un perÃodo crÃtico debido a la hipoglicemia (azúcar bajo en la sangre) severa, al onfalocele y a una tasa de aumento en el desarrollo de un tumor (siendo los más comunes el tumor de Wilms y carcinoma suprarrenal .
- Fecha de revisión: 6/13/2007
- Versión en inglés revisada por: 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).
- Traducción y localización realizada por: DrTango, Inc.
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