What is Wiskott-Aldrich syndrome?

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It is an immune deficiency disorder associated with irregularity in immunoglobulin M (IgM) production. A defective gene on the X chromosome is responsible for this disease.

Hence females tend to be carriers of the syndrome while males with the defective gene develop symptoms. This rare immune deficiency leaves the body vulnerable to infection. It also causes low numbers of blood platelets. There may be serious infections such as pneumonia, meningitis, and blood poisoning (sepsis).

Wiskott-Aldrich syndrome occurs in 4 per million live male births and affects people of all ethnic backgrounds. Eczema – a skin disorder develops in more than three-quarters of individuals with Wiskott-Aldrich syndrome.

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Individuals with the syndrome may be more allergy- prone and may develop asthma. As many as 40% of individuals with Wiskott- Aldrich syndrome may develop an autoimmune disorder, the most common are autoimmune hemolytic anemia, vasculitis, renal disease, arthritis, and Henoch- Schonlein purpura.

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