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篇名
台灣地區的韋格納氏肉芽腫:12個病例分析
並列篇名
Wegener's granulomatosis in Taiwan: an analysis of twelve patients
作者 林永章陳得源藍忠亮
中文摘要
目的:韋格納氏肉芽腫在台灣是相當少見的疾病,關於其臨床病程及預後的資料亦相當有限。本篇研究目的在於分析台灣地區韋格納氏肉芽腫病例的臨床特徵、病程及併發症,期望在將來能有助於患者之診斷及處理。方法:研究資料來源為病歷回顧,收錄了1985至2005年間於台中榮民總醫院診斷為韋格納氏肉芽腫共12個病例,統計分析其臨床特徵、病程、實驗室檢查及預後。結果:研究對象包括5位男性及7位女性病例。平均診斷年們為55.4歲(16~77歲)。診斷時最常見的臨床表現為鼻炎及鼻竇炎(100%)。於追蹤期間最常受到侵犯的器官系統依序為上呼吸道(100%)、肺臟(75%)及腎臟(50%)。共有11位病換檢驗血清ANCA,結果全部皆呈現c-ANCA陽性。經治療後所有病患皆獲得部份或完全緩解,但追蹤期間有7位(58%)病人出現疾病復發。整體死亡率為50%,首要死因為細菌性肺炎。結論:台灣地區韋格納氏肉芽腫好發於中年成人,並且於疾病早期皆有上呼吸道侵犯情形。研究結果顯示病患合併有高罹病率及死亡率,並且於疾病活動期之血清c-ANCA陽性率相當高。面對這樣稀少又複雜的疾病,臨床醫師在診斷上需具備高度警覺性。當病患的臨床特徵及病理表現皆高度懷疑韋格納氏肉芽腫時,血清c-ANCA試驗陽性有助於支持診斷此疾病。
英文摘要
Objective: Wegener's granulomatosis (WG) is a rare disease in Taiwan and data on the disease course and outcome is limited. We analyzed the clinical characteristics of patients with WG in Taiwan in order to facilitate improved case recognition and management in the future. Methods: A retrospective study was conducted by chart review. Patients with WG diagnosed between 1985 and 2005 at Taichung Veteran's General Hospital were included. Their clinical features, disease course, and laboratory findings were analyzed. Results: The patient group consisted of 5 males and 7 females. The mean age at diagnosis was 55.4 years. The most common manifestation at diagnosis was rhinosinusitis (100%). During follow-up, the most common involved organ/system was the upper respiratory tract (100%), followed by lungs (75%) and kidneys (50%). Serum antineutrophil cytoplasmic antibodies (ANCA) were assessed in 11 of the 12 patients, and were positive in all patients with a cytoplasmic pattern. All 12 patients achieved at least partial remission after treatment, but 58% of patients (7/12) experienced one or more relapses. The overall mortality rate was 50%, and the leading cause of death was bacterial pneumonia. Conclusion: WG in Taiwan usually affects middle-aged adults, and nearly always involves the upper respiratory tract in the early stage of disease. Patients in this study are characterized by high morbidity and mortality, as well as high rates of seropositivity for cytoplasmic-AN CA. A high index of suspicion is needed in diagnosis of this rare and complex disease. In patients with clinical features and pathological findings that are highly suggestive of WG, seropositivity for cytoplasmic-ANCA could help support the diagnosis.
起訖頁 44-51
關鍵詞 ANCA診斷預後韋格納氏肉芽腫Antineutrophil cytoplasmic antibodydiagnosisprognosisWegener's granulomatosis
刊名 中華民國風濕病雜誌  
期數 200706 (21:1期)
出版單位 中華民國風濕病醫學會
該期刊-上一篇 皮膚血管炎--不同致病因之臨床分析
該期刊-下一篇 各種HLA-B27亞型在僵直性脊椎炎病人之中的盛行率
 

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