Lysosomes/ Hurlers Disease

2 Pages 407 Words

Hurler’s disease could quite possibly be considered as one of the most dangerous types of lysosomal diseases because it is not really detectable until 6 and 24 months after birth. Once the disease is recognized though many times it is too late considering that the individual is already mentally retarded. Children that are affected by this disease have enlarged stomachs because of the fact that their spleens have swollen up.
The skeleton also is affected by the lysosomal malfunction, suffers normally have hunched backs and stunted growth. Also the thorax becomes stiff which causes those affected to appear to walk rigidly. As Hurler’s disease progresses the children start to develop similar appearances. Well-marked eyebrows and an abnormal amount of very thick coarse hair are among the most common. Also common to those suffering Hurler’s disease is broad mouths and enlarged tongues. Hearing and vision are also affected because of the build up of fluids in the tissue of the children. Finally because of the mucouspolysaccharide build up the heart is affected which makes the children very susceptible to heart problems.
Hurler’s disease is a type of lysosomal disease, which is caused by the lysosmal enzymes not producing, as they should. Hurler’s disease is caused when the carbohydrates that should be metabolized are not done correctly which causes the mucouspolysaccarides to build up in the actual cell. Because of this build up the cell is ultimately damaged along with all the tissue and organs that are involved, which is what causes many of the vision, hearing and heart problems.
Although there are no cures for Hurler’s disease there do happen to be a few procedures which aim to enhance suffers quality of life. The most common treatment for Hurler’s disease is bone marrow transplants; these need to be done within the first 18 months before mental retardation is in full effect. The bone marrow transplant allo...