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Article de Coréen | WPRIM | ID: wpr-160985

RÉSUMÉ

The Job syndrome is a primary immunodeficiency disorder characterized by markedly elevated serum IgE levels, recurrent severe infections of the skin and sinoplmonary tract, including staphylococcal pneumonias, chronic eczematoid dermatitis, coarse facial features, and mild eosinophilia. In 1966, Davis et al discribed two patients with severe eczema, chroic sinopulmonary infections, and recurrent staphylococcal abscesses originally. The reason for immunocompromise in the Job syndrome remains unclear, but a chemotactic defect in neutrophils has been described. We have experienced a case of Job syndrome in a 9-year-old girl who had suffered from severe recurrent multiple infections since 2 month of age. After treatment with thymopentin, she was improved clinically. A brief review of related literature is presented.


Sujet(s)
Enfant , Femelle , Humains , Abcès , Dermatite , Eczéma , Éosinophilie , Immunoglobuline E , Syndrome de Job , Granulocytes neutrophiles , Pneumopathie à staphylocoques , Peau , Thymopentine
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