What is Wiskott-Aldrich's syndrome?
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Wiskott-Aldrich syndrome, also known as immunodeficiency syndrome-meunodeficiency, is a rare disease that disrupts blood cells and immune system in most men of all backgrounds. The syndrome, a serious inherited state that can cause death, is characterized by a significantly low number of plates, frequent infections and inflammation of the skin. While the condition also increases the risk of leukemia and lymphoma, Wiskott-aldrich syndrome is treatable. The person suffering from this condition has disruption with a protein responsible for the transmission of signals from the cell surface to the actin cytoskeleton, a network of fibers that form the basic make -up cells. Mutations cause cells to have difficulty growth and connect to other cells.
gene related to Wiskott-aldrich syndrome is a found on chromosome X, sex chromosome. Males are more susceptible to illness because they need only one of the distorted x chromosomes to inherit the condition. In order to inherit the syndrome, it would have to receive two distorted x chromosomes.
with a low number of platelets is a person with a disorder prone to easy bruise and bleeding, because their lack of plates causes the body to face bleeding. Bleeding in the skin can lead to small blue and red spots that look like bruises. Low platelet levels also lead to bloody bowel movements, bloody gums and persistent nose bleeding. More seriously, one can develop brain bleeding and infants can be obliged to wear a helmet to protect themselves from the injury of the head until the levels of the plates are increased.
with compromised immune syndrome is also a susceptible individual with a disorder of infections. A person can obtain minor diseases such as ears, respiratory infections and sinusitis. It is not so common that an individual can develop more serious diseases such as blood poisoning and meningitis.
Another classical symptom of syndrome is eczema, serious skin inflammation. Inflammation may occurWhether all over the body of man. Eczema causes one itching so much, it can even scratch when they sleep. Often you scratch yourself until they bleed.
Wiskott-aldrich syndrome causes confusion of the immune system and can lead to inflammation of the blood vessels in the internal organs. Inflammation is often associated with fever and rash. Another immune disorder that may occur is anemia in which antibodies are killed by red blood cells.
While the disorder is potentially life -threatening, one can still lead a healthy and productive life. One of the most effective methods of treatment is donor marrow transplantation if it is carried out before age 6 years. Treatment often includes iron supplements to be protected from blood loss. In more serious cases, the transfusion of platelets and the spleen removal operation can be considered.