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題 名 | The Hyperimmunoglobulin E Syndrome=高免疫球蛋白E症候群 |
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作 者 | 許振東; 林于粲; 楊謹旭; 江伯倫; | 書刊名 | 微免與感染雜誌 |
卷 期 | 37:2 2004.04[民93.04] |
頁 次 | 頁121-123 |
分類號 | 415.96 |
關鍵詞 | 高免疫球蛋白E症候群; Furunculosis; Hyper-lgE syndrome; Methicillin resistance; Staphylococcus aureus; |
語 文 | 英文(English) |
英文摘要 | Hyperimmunoglobulin E syndrome is a primary immunodeficiency disease characterized by markedly high titers of serum immunoglobulin E (IgE), chronic eczema, recurrent staphylococcal infections, pneumatoceles, reduced neutrophil chemotaxis, and variable impaired T cell function. There are no clinical tools for diagnosis and definitive laboratory investigation. Variability of presentation makes it easy to confuse the diagnosis with that of severe atopy or other rare immunodeficiencies. We report a case of a 6-year-old boy with hyperimmunoglobulin E syndrome with recurrent methicillin-resistant Staphylococcus aureus furunculosis. Physical examination revealed a peculiar facial appearance, pruritic dermatitis, and furunculosis over the scalp, neck, and back. Laboratory investigation revealed mild leukocytosis with eosinophilia, a very high immunoglobulin E level, defective neutrophil chemotaxis, and impaired lymphocyte proliferation to anti-CD3/CD28 monoclonal antibodies. The boy was discharged without incident after 2 weeks of antibiotic therapy and debridement. |
本系統中英文摘要資訊取自各篇刊載內容。