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題 名 | Orthopedic Manifestation in a Child with Hyperimmunoglobulin E Syndrome=高免疫球蛋白E症候群病童之骨骼症狀表現: 一病例報告 |
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作 者 | 林思偕; 黃璟隆; 顏大欽; 謝貴雄; | 書刊名 | 中華民國小兒科醫學會雜誌 |
卷 期 | 39:5 民87.09-10 |
頁 次 | 頁330-332 |
分類號 | 417.626 |
關鍵詞 | 高免疫球蛋白E症候群; 兒童; 骨骼; Hyperimmunoglobulin E syndrome; Osteomyelitis; Fracture; HIE; |
語 文 | 英文(English) |
中文摘要 | 吾人報告一罹患高免疫球蛋白E症候群之12歲女性病童。其臨床表現為慢性骨髓 炎及關節炎所導致之左膝外翻,雙手關節變型及經常骨折。過去病史顯示此病例有慢性濕疹 及反覆皮膚及軟組織發炎。住院期間並發現有pneumatocele。實驗室檢查發現病童有極高之 血清IgE值(18989lU/mL)。遲延性皮膚過敏試驗使用皮內注射PPD,candida,及tetanus toxoid均呈陰性反應。其單核細胞對各種分裂原(mitogen)之淋巴球增殖反應則為正常。顆 粒性白血球功能,如NBT試驗及趨化功能亦為正常。對反覆感染併有上述骨骼症狀表現之病 童應高度懷疑高免疫球蛋白E症候群。早期診斷並適當使用預防性抗生素為目前此症之唯一 有效治療。 |
英文摘要 | We report a 12-year-old girl with hyperimmunoglobulin E (HIE) syndrome who presented with genu vulgus of left knee, joint deformities involving both hands, and frequent fractures. She had had chronic eczema and recurrent skin and soft tissue infections since infancy, and was found to have a pneumatocele during admission. Immunologic abnormalities included extremely elevated serum IgE levels (18989 IU/ml) and cutaneous anergy to candida, purified protein derivative, and tetanus toxoid. The results of polymorphonuclear leukocyte function tests including the nitroblue tetrazolium test and chemotaxis were normal. A high index of suspicion for HIE syndrome should be given in patients with recurrent skin infections and orthopedic complaints. The physician should anticipate orthopedic problems in caring for patients with HIE syndrome, and optimal antibiotics prophylaxis should be used. |
本系統中英文摘要資訊取自各篇刊載內容。