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- Pulmonary Alveolar Proteinosis after Whole Lung Lavage Treatment--A Case Report
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- Pulmonary Alveolar Proteinosis Treated with Whole Lung Lavage: A Case Report and Literature Review
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- 肺蛋白沉著症的診斷與治療
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題 名 | Pulmonary Alveolar Proteinosis after Whole Lung Lavage Treatment--A Case Report=肺泡蛋白質沈積症經全肺灌洗術治療--病例報告 |
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作 者 | 康育誠; 林清基; 許清寅; 陳碧芳; | 書刊名 | 胸腔醫學 |
卷 期 | 16:3 2001.09[民90.09] |
頁 次 | 頁183-189 |
分類號 | 415.46 |
關鍵詞 | 肺蛋白質沈積症; 全肺灌洗術; Pulmonary alveolar proteinosis; Whole-lung lavage; |
語 文 | 英文(English) |
中文摘要 | 肺泡蛋白質沈積症亦即肺泡磷脂蛋白沈積症;在患者的遠端氣道內可發現填充了無定形、periodic acid-Schiff (PAS)染色陽性的脂蛋白樣特質;它主要是由磷脂蛋白肺張力素及肺張力素的前蛋白所構成。我們的病例是一位中年男性其症狀為發燒、呼吸困難以咳血。他在這次求診之前也曾經因為上述的不適而住院,當時診斷為支氣管擴張症合併續發性感染。而在這次住院中,理學檢查發現兩側下肺野有肺囉音,動脈血液氣體分析呈現低血壓,且肺功能可見一氧化碳擴散能力減低,胸部X光片則呈現兩側肺門旁及兩側下肺野有不規則網狀的肺浸潤。在高解像胸部電腦斷層掃描下則明顯可見多處毛玻璃樣的分布在兩側肺野,同時併有肺小葉間隔增厚的現象。此患者後來接受開胸肺組織切片檢查,病理學報告發現有嗜伊紅性強PAS染色陽性的顆粒狀物質;檢查証實為肺泡蛋白質沈積症,並且在隨後進行了兩側肺的全肺灌洗術治療;其灌洗液在電子顯徵鏡下呈現典型的肺張力素層狀構造,更確定此診斷。病患在接受兩側肺的全肺灌洗術治療之後,症狀及肺功能明顯改善,故出院並於門診追?。 |
英文摘要 | Pulmonary alveolar proteinosis (PAP), also known as pulmonary alveolar phospholipoprotenosis, is a diffuse lung disease characterized by the accumulation of amorphous, periodic acid-Shiff-positive lipoproteinaceous material, composed principally of the phospholipid surfactant and surfactant apoproteins deposited in the distal air spaces. We report a middle-aged male who was admitted to our hospital with the chief complaint of fever, dyspnea on exertion, and hemoptysis. He had been diagnosed as having bronchiectasis with secondary infection on a previous admission, due to the same symptoms and signs. During this admission, the physical examination revealed coarse breathing sounds in the bilateral basal lungs. Arterial blood gas showed hypoxemia, and pulmonary function tests showed decreased diffusion capacity. A chest radiograph revealed reticulonodular infiltrates in both perihilar regions and in both lower lobes, and the high resolution computed tomography revealed a multifocal ground glass appearance throughout both lungs, with interlobular septal thickening. The diagnosis of pulmonary alveolar proteinosis was finally confirmed by open lung biopsy and an electron microscopic examination of the bronchoalveolar lavage fluid, which showed eosinophilic granular PAS-positive, diastase-resistant material in the alveolar spaces, and the characteristic lamellar structure of the surfactant vesicles. Therapeutic whole-lung lavage was performed in both lungs, after which the patient felt better. He was then discharged for follow-up at the OPD. |
本系統中英文摘要資訊取自各篇刊載內容。