Thursday, January 12, 2012

Beckwith-Wiedemann Syndrome - BWS Support Network UK and Europe

Beckwith-Wiedemann Syndrome (BWS) is an overgrowth syndrome.

An overgrowth syndrome can cause part or all of the body to grow larger than expected.

In BWS, overgrowth is associated with body overgrowth (increased birth weight, height, and head circumference), macroglossia (large tongue), hemihypertrophy (one side of the body -in part or in whole - growing larger than the other side),
Omphalocele (Abdominal wall defect) and certain types of tumours as well as other physical characteristics.

The syndrome was first described by Dr Beckwith in 1963 and Dr Wiedemann in 1964.

BWS occurs in approximately 1:14,500 births.

There are roughly thirty characteristics that can be associated with BWS. Those which are used to make a diagnosis (the 5 major characteristics) are listed below.

It is very rare for a child with BWS to have all the characteristics; most cases have only a few of them. Some of the distinctive features of BWS may need to be corrected.

Some of the main characterisics to look for in Beckwith-Wiedemann Syndrome (BWS)Large birth weight and length (over 90-95th %)
Hypoglycemia (low blood sugar) in the first four months of life
Macroglossia (large tongue)

Omphalocele: Abdominal wall defect (including even a mild navel hernia)
Ear grooves (creases) or pits

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BWS Support Network UK and Europe

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1 comment:

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