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Question
Posted by: es | 2003/01/14

Wiskott-Aldrick - sindrome

Is their a cure for this syndrome or any help to make is better? It is my aunt grandson that has it and I like to know more about this sindrome.

Thank
es

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Our expert says:
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ES, it's an X-linked recessive immunodeficiency disorder of male infants, characterized by eczema, thrombocytopenia, and recurrent infection.

The first manifestations often are hemorrhagic (usually bloody diarrhea), followed by recurrent respiratory infections. Malignancy (especially lymphoma and acute lymphoblastic leukemia) is common (10%) in survivors > 10 yr old. The characteristic immunologic defects include poor antibody responses to polysaccharide antigens, cutaneous anergy, partial T-cell immunodeficiency, elevated levels of IgE and IgA, low levels of IgM, and hypercatabolism of IgG but normal IgG levels. Because of the combined deficiency in both B- and T-cell function, infections occur with pyogenic bacteria, viruses, fungi, and P. carinii. Hematologically, these patients have small platelets and increased splenic destruction of platelets; accordingly, splenectomy may alleviate the thrombocytopenia. The gene defect has been localized to Xp11.

The information provided does not constitute a diagnosis of your condition. You should consult a medical practitioner or other appropriate health care professional for a physical exmanication, diagnosis and formal advice. Health24 and the expert accept no responsibility or liability for any damage or personal harm you may suffer resulting from making use of this content.

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