BECKWITH WIEDEMANN SYNDROME
by Beckwith Wiedemann Support Group
Like many such disorders, Beckwith-Wiedemann Syndrome can vary in its
effects from child to child i.e. some children are relatively mildly affected
while others have a wider range of physical problems. There are certain main
features common to most children but there are others, which occur in some
cases. Every child does not have every feature and the features present may
vary in severity.
What is BWS?
Beckwith-Wiedemann Syndrome is a congenital overgrowth syndrome, which can
affect all systems of the body. It was first recognised in 1963-64 by Dr J.
Bruce Beckwith, a paediatric pathologist in America and, independently, by Dr
H.E. Wiedemann, a German geneticist. Each had found a similar set of congenital
abnormalities in children which could not be found in any other disorders - in
other words, a new syndrome.
BWS occurs once in approximately every 15,000 births. This figure may be an
under-estimate due to mild cases not being diagnosed. Cases have been reported
in most developed countries. In the great majority of cases it appears to be an
isolated event with no known relatives but there is some evidence that the
condition can be inherited.
The effects of BWS vary from mild to severe. Most problems can be helped or
even solved provided that accurate diagnosis is made and appropriate treatment
started. Those children who survive infancy, the great majority, are usually
healthy with their growth and appearance gradually becoming normal.
What causes BWS?
Recent research has shown that BWS is caused be an alteration in genetic
material. The alteration in most cases occurs only in the effected child. In
these cases there is a low risk of parents having other BWS children. In a few
cases the altered gene is inherited from one parent who may or may not be
mildly affected themselves. In these cases there is a 1 in 2 chance of another
child being affected. Research is continuing into the mechanism of action of
the gene responsible for BWS.
BWS Support Group
This was started in 1990 by a group of parents with BWS children to share
problems and information and to act as a self-help group. It aims to promote
both public and professional awareness of BWS and to support and encourage
research. The group has links with BWS groups in Holland and America. It
publishes a quarterly newsletter and booklet and a yearly meeting is arranged.
For further information about the group please contact:
Beckwith Wiedemann Support Group
The Drum and Monkey
Hazelbury Bryan
Dorset DT10 2EE
 01258 817573 (evenings)
0589 211000 (mobile)
 01202 205325
 rbaker5165@aol.com
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