Beckwith Wiedemann

Beckwidth-Wiedemann Syndrome is a disease that affects most parts of the body. It is mostly present in babies during birth. Unlike their peers, people with this condition tend to be taller or bigger in size. At the age of 8 the growth slows down and most grown up people are not abnormally tall. Beckwidth-Wiedemann Syndrome may affect some parts of the body and sometimes it affects one side of the body causing uneven growth of the body.

Characteristics of the Beckwidth-Wiedemann Syndrome

The sign and symptoms of the condition vary in different people.
At birth, some children are born with an opening at the side of their abdomen mostly at the belly button that has the internal organs protruding.
Presence of a soft pouch around the belly button is also seen in some infants.
The average length of the infant and weight is usually abnormal. Low blood sugar levels may also be witnessed at birth.
Infants with this condition are sometimes born with an abnormally large tongue. The tongue mostly interferes with air passage and speaking.
The risk of children with the condition suffering from cancerous tumors is very high. About 10% of the people with Wiedemann syndrome get the cancerous tumors mostly during their childhood.
The condition does not imply that people with it have medical conditions, children and adults with wiedemann syndrome have a normal life expectancy.

Management of the Beckwidth-Wiedemann Syndrome

The abdominal wall defects present in most of the infants can be fixed through surgery to prevent any form of infections. The umbilical hernias does not need any treatment, by the age of four they close spontaneously. The low blood sugar levels in children can lead to brain damaged if they are left untreated. The treatment used should be in accordance to the neonatal hypoglycemia protocol. The sugar levels can be raised by feeding the infant glucose. The large tongue can hinder the mouth from fully closing. However, the tongue becomes less noticeable overtime and therefore treatment is not necessary. Surgery can be performed in serious cases where the tongue hinders air passage.

In summary, the chances of infants being born with the BWS increases four fold in children conceived using in vitro fertilization. This is because of the genes being turned on and off during the procedures. The cases of an infant being born with BWS is one in 13600. BWS has been recorded to have some ethnic groups and is equally present in males and females.

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