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Whole Lung Lavage in a Patient with Pulmonary Alveolar Proteinosis: A Case Report

肺泡蛋白質沉積症進行全肺部沖洗術之病例報告

摘要


肺泡蛋白質沉積症是非常罕見的疾病,病理組織呈現肺泡中充滿蛋白質類的物質,到目前病理機轉並不清楚。因為是未知病理機轉,對於這個疾病最有效也是最正確的治療方式則是全肺部沖洗術。治療性全肺部沖洗術必須經由全身麻醉及置放雙口徑氣管內管。而這個治療方法會碰到問題則是低血氧,心肺循環問題和置放雙口徑氣管內管技術問題。因此必須要有經驗的麻醉專科醫師實行,手術後也需要加護病房照顧避免發生病發症。在此我們報告一位44歲男性,因吸入柴油,合併呼吸困難,咳嗽有痰,發燒,診斷為肺泡蛋白質沉積症。在全身麻醉下病患接受全肺部沖洗術。沖洗後臨床症狀不但改善,肺功能也明顯好轉。

並列摘要


Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of protein and lipid material, called surfactant, within the air sacs or alveoli of the lungs with for unknown reasons. Because of the unknown pathogenesis, the most effective treatment for PAP is the mechanical removal of the proteinaceous material via whole-lung lavage (WLL) with isotonic saline as the lavage solution, and mechanical chest percussion during lavage. Serial arterial blood-gas determinations and measurement of lung compliance are done in during the intraoperative and postlavage periods. The major complications of WLL are hypoxia, circulatory disturbance and difficulty in positioning the double lumen endotracheal tube. An experienced team is needed in the operating room and a postoperative care facility is also required to reduce complications. Here we report a 44-year-old man with a history of diesel fuel inhalation, suffered from dyspnea on exertion, a productive cough, and low-grade fever. An open lung biopsy confirmed the diagnosis of PAP. The patient underwent WLL under general anesthesia. His clinical symptoms were improved and discharged with a relatively stable condition.

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