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Ency. home > Disease > B > Beckwith-Wiedemann syndrome

Beckwith-Wiedemann syndrome    See images

Overview | Symptoms | Treatment | Prevention

Definition:

Beckwith-Wiedemann syndrome is a consistent grouping of findings of unknown etiology (cause) and characterized by a large tongue (macroglossia), large organs (visceromegaly) and large body size (macrosomia), umbilical hernia or omphalocele (hernia of the navel), and low blood sugar in the newborn (neonatal hypoglycemia).

Causes and Risks

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

Affected children are often large at birth. Many have an abdominal wall defect, such as an umbilical hernia or omphalocele. They have a characteristic facial appearance with a gaping mouth and large tongue.

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

Ency. home > Disease > B > Beckwith-Wiedemann syndrome

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