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頁籤選單縮合
題名 | Hemophagocytic Syndrome: A Review of 18 Pediatric Cases=噬血症候群:18個兒科病例的分析 |
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作者姓名(中文) | 陳志榮; 黃玉成; 江東和; 洪悠紀; 楊兆平; 張鑾英; 林奏延; | 書刊名 | 微免與感染雜誌 |
卷期 | 37:3 2004.06[民93.06] |
頁次 | 頁157-163 |
分類號 | 417.519 |
關鍵詞 | 噬血症候群; 兒科; Child; Epstein-barr virus; Hemophagocytic syndrome; |
語文 | 英文(English) |
英文摘要 | This retrospective study included 18 pediatric cases (median age, 3 years) with pathologically proved hemophagocytic syndrome (HPS) from a single institution during 1992 and 2001. There were 9 males and 9 females. Prolonged fever, cytopenia, liver dysfunction and hepatomegaly were the most common features at presentation. Sixteen (88.9%) cases were previously healthy. The case fatality rate was 61.1%, and all fatal cases died within 2 months of disease onset. The infectious agents associated with HPS were identified in 11 cases (61.1%), and 8 (72.7%) of them had evidence of Epstein-Barr virus (EBV) infection or reactivation. Underlying immunologic disorder or neoplastic disease was identified in 11.1% of the cases. Children less than 3 years of age with HPS were more vulnerable to neutropenia-associated bloodstream infection (85.7% vs 27.3%; p=0.025). Pseudomonas aeruginosa (3) and Candida tropicalis (2) were the 2 most commonly isolated pathogens. Regarding specific management of HPS, intravenous immunoglobulin and steroids were the first-line agents and were administered in 16 cases and 11 cases, respectively, while etoposide was administered in 5 refractory cases during the late phase of disease. Most HPS occurred in previously healthy children, and a substantial proportion of cases rapidly progressed to death. Most cases were associated with viral infection, particularly EBV, and young children tended to develop neutropenia-associated bacteremia during the active phase of the disease. |
本系統之摘要資訊系依該期刊論文摘要之資訊為主。