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肺泡蛋白質沉著症之高解像電腦斷層影像所見:病例報告

High Resolution CT Findings in a Case of Pulmonary Alveolar Proteinosis

摘要


肺泡蛋白質沉著症(pulmonary alveolar proteinosis,PAP)是一個少見而在肺泡中積存了一些呈現periodic acid-Schiff (PAS)陽性反應的蛋白樣物質的疾病,目前致病原因未明。我們報告一個病例是以乾咳,逐漸地造成呼吸困難為臨床症狀之主要表現,一般胸部X光檢查表現出兩側廣泛性間質及肺泡浸潤,高解像電腦斷層攝影(high resolution computed tomography,HRCT)顯示出兩側廣泛性毛玻璃狀(ground-glass)陰影,並雜有濃密的空氣腔(air-space)肺實質變化(consolidation)及肺間質變厚的影像,因此建議這個診斷。經開胸手術(open lung biopsy)做活體組織病理切片檢驗,最後診斷為PAP。本病例報告回顧相關文獻並討論此病例的整個療程中HRCT表現及臨床表現的對照。

並列摘要


Pulmonary alveolar proteinosis (PAP) is a rare disease with an unclear etiology, in which the alveoli became filled with a periodic acid-Schiff (PAS)-positive proteinaceous material. We present the case of a 32-year old man with prominent symptoms of dry cough and progessively dyspnea for two weeks. Routine chest radiography showed diffuse interstitial and alveolar opacity of both lungs. High resolution computed tomography (HRCT) revealed diffuse bilateral ground-glass opacity, areas of air-space consolidation, and thickened interstitial septa. These findings were very suggestive of the diagnosis of PAP. Open lung biopsy was performed providing pathologic confirmation of PAP. The clinical course and HRCT features of this patient are discussed.

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