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포도상구균 폐렴을 동반한 고면역글로불린 E 증후군 1례
오흥원,서원석,신상만 순천향의학연구소 1995 Journal of Soonchunhyang Medical Science Vol.1 No.1
The hyperimmunoglobulin E syndrome is a rare primary immunodeficiency disorder characterized by extremely high serum IgE levels and eosinophilia, and serious recurrent bacterial infections of the skin and sinopulmonary tract in early life. Associated clinical features are coarse facial appearance and chronic eczematoid dermatitis. The most common organism that infect these patients are S. aureus and C. albicans. The patients' serum IgE levels are elevated but the basic immunologic mechanism is not fully understood. It is believed that the elevated IgE levels reflect a T-cell imbalance characterized by T-cell activation and a deficiency of suppressor T cells to inhibit IgE production. We have experienced a case of hyperimmunoglobulin E syndrome in a ??-year-old boy who had suffered from recurrent staphylococcal pneumonia and onychomycosis and chronically pruritic dermatitis from 3 months of age with elevated serum IgE level. A brief review of the related literature is presented.