Ataxia-telangiectasia is a hereditary disorder characterized by no coordination, enlargement of capillaries, and increased susceptibility to infection. Increased susceptibility to infections in people with ataxia-telangiectasia due to failure of the function of B and T lymphocytes Often times, the levels of IgA and IgE antibody classes are also lacking. Recurrent sinus and respiratory infections, often leading to pneumonia and chronic lung diseases such as bronchitis. The risk of cancer, especially leukemia, brain tumors, and stomach cancer, is increased.
Abnormalities in the cerebellum (which is not associated with immunodeficiency disorders) cause there is no coordination (ataxia). There is no coordination usually develops when the child begins to walk but may be delayed until age 4. Speech becomes slurred, and the muscles progressively weaken, leading to severe disability. Mental retardation may occur and evolve. Between ages 1 and 6, capillaries in the skin and eyes become enlarged and visible. Enlarged capillaries (telangiectasia), called spider veins, usually most noticeable in the eyes and ears. Endocrine system may be affected, causing small testes (in boys), infertility, and diabetes.
Antibiotics and immune globulin to help prevent infection but does not reduce the problem to the nervous system. Ataxia-telangiectasia is usually developed to paralysis, dementia, and death, usually before the age of 30 years.
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