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題名 | Idiopathic Pulmonary Fibrosis in a Child=幼童原發性肺纖維病變 |
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作者姓名(中文) | 張魁文; 吳昌騰; 黃秀芬; 江東和; 黃健燊; | 書刊名 | 長庚醫學 |
卷期 | 26:1 2003.01[民92.01] |
頁次 | 頁60-64 |
分類號 | 415.46 |
關鍵詞 | 原發性肺纖維病變; 類固醇脈衝治療; Childhood idiopathic pulmonary fibrosis; Corticosteroid pulse therapy; |
語文 | 英文(English) |
中文摘要 | 原發性肺纖維病變原因不明,雖?此病在幼童極為罕見,但預後通常極差。此病在國內尚未被報告過。在此我們報告一個二歲幼童的原發性肺纖維病變以快速進行的呼吸因難繼發呼吸衰竭來表現,肺部切片證實為原發性肺纖維病變。雖然即時給予類固醇脈衝治療並嘗試使用一氧化氮治療,此病患仍然在住院後35持續低血氧死亡。原發性肺纖維病變應包含在呼吸困難的鑑別診斷中,並儘早給予類固醇治療。幼童原發性肺纖維病變給予類固醇的確實時機及劑量亦待研究,其他新式的藥物治療亦有待發展。 |
英文摘要 | Idiopathic pulmonary fibrosis (IPF) is a rare disease of unknown etiology and is usually associated with a poor prognosis. Up to the present, less than 50 cases of IPF in children have been reported in the English literature, and no case has ever been reported from Taiwan. Herein we report on a 2-year-old boy with IPF was verified with an open lung biopsy. Despite intravenous methylprednisolone pulse therapy and empiric nitric oxide treatment, he expired on the 35th day after admission due to profound hypoxemia. A diagnosis of IPF should be included in the differential diagnosis for patients presenting with unexplained shortness of breath and pulmonary interstitial infiltrations. |
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