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Pulmonary Alveolar Proteinosis Treated with Whole Lung Lavage: A Case Report and Literature Review

肺蛋白質沉積症-病例報告與文獻回顧

摘要


肺蛋白質沉積症是少見之疾病。它於1958年被提出,特色是在肺泡中有periodic acid-Schiff特殊染色陽性物質的沉積。一個55歲男性於住院前九個月開始有呼吸困難以及輕微咳嗽。胸腔x光片顯示瀰漫性間質浸潤。動脈血氣分析顯示低血氣濃度以及擴散能力降低。胸部電腦斷層發現區域毛玻璃樣病變與碎石路模式(crazy-paving Pattern)。肺部病理切片確認肺蛋白沉積症診斷。病人後續接受多次全肺灌洗術治療。其臨床症狀以及動脈血氧濃度逐漸改善。

並列摘要


Pulmonary alveolar proteinosis is a rare disease which was described first in 1958 as an ”accumulation of periodic acid-Schiff (PAS)-positive material in the alveolar space”. A 55-year- old male smoker was admitted due to slowly progressive exertional dyspnea with mild productive cough for 9 months. Chest radiography revealed bilateral diffuse lung infiltrates. Arterial blood gas showed hypoxemia with impaired diffusion capacity. Chest tomography showed patchy areas of ground glass opacities with a crazy-paving pattern. Alveolar proteinosis was proved by open- lung biopsy. Therapeutic whole lung lavage was performed 3 times monthly. His symptoms and arterial oxygen tension improved thereafter.

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