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Job syndrome-hyperimmunoglobulin E syndrome


Job syndrome-hyperimmunoglobulin E syndrome

المعلومة

hyperimmunoglobulin E (hyper-IgE or HIE) syndrome is a rare immunodeficiency disorder that has an autosomal dominant inheritance pattern.

HIE has variable expressivity and is associated with multiple abnormalities. The most common findings are recurrent skin abscesses pneumonia with pneumatocele development, and high serum levels of IgE. Facial, dental, and skeletal features are also associated with this syndrome.

The pathophysiology of Job syndrome is not completely understood. Patients consistently have a poor, delayed hypersensitivity response to antigens. This delayed response is associated with alterations in T-lymphocyte populations and various interleukin and cytokine abnormalities.

المرجع

e-medicine

mbs2380's picture
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بعد التخرج

Quote:
recurrent skin abscesses

recurrent staphylococcal infections of the skin
thanks...

Vince Carter
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