Pulmonary Alveolar Proteinosis Treated with Whole Lung Lavage in Intensive Care Unit

肺泡蛋白質沈著症在加護病房進行全肺灌洗術治療

王耀章(Yao-Chang Wang) ； 盧朝勇(Jau-Yeong Lu) ； 郭文雄(Wein-Shung Kuo) ； 鍾明燈(Ming-Teng Chung) ； 葉本芳(Pen-Fang Yeh)

《重症醫學雜誌》 11卷4期 (2010/12) Pp. 267-274

https://doi.org/10.30035/TCCM.201012.0005

摘要

肺泡蛋白質沈著症是不常見的間質性肺病。胸部X光特徵是肺泡充滿PAS染色陽性的脂蛋白物質。肺泡蛋白質沈著症的標準治療是施行肺灌洗術將脂蛋白物質洗出肺部。大部份肺灌洗術是在手術房全身麻醉下進行的。在這篇文章，報告一位53歲病人住院前兩個月因進行性呼吸困難至本院門診。胸部X光片顯示兩肺浸潤性變化。胸部電腦斷層呈現毛玻璃樣病灶及不規則石板拼舖型表徵。經支氣管鏡檢查確診為肺泡蛋白質沈著症。病人在加護病房施行全肺灌洗術。治療後病人症狀及胸部X光片明顯改善。一年後追蹤病人狀況良好。肺灌洗術可以無需在手術室操作而在加護病房內安全的施行。

關鍵字

肺泡蛋白質沈著症 ； 全肺灌洗術

並列摘要

Pulmonary alveolar proteinosis (PAP) is an unusual interstitial lung disease. The characteristic of the disease is the revealing of periodic acid Schiff (PAS) positive lipoproteinaceous material-filled alveoli on chest radiographs. The standard treatment of PAP is to remove the lipoproteinaceous materials from the lung through the whole lung lavage (WLL) procedure, which is mostly performed in operating rooms under general anaesthesia. In this article, we report the case of a 53-year-old male patient who visited to our chest outpatient clinic due to progressive shortness of breath for 2 months prior to being hospitalized. Chest radiographs indicated bilateral perihilar infiltrate with haziness. A high resolution computed tomography (HRCT) scan of the chest showed ground glass opacification of alveolar spaces with typical crazy paving pattern. A subsequent bronchoscopic lung biopsy confirmed the diagnosis of PAP. The patient underwent the WLL procedure in the intensive care unit and significant improvements have been observed on patient symptoms and chest radiographs after the treatment. A one year of follow up showed that the patient is in stable condition. Thus, we conclude that the lung lavage procedure can also be safely performed in the intensive care unit instead of in an operating room.

並列關鍵字

Pulmonary alveolar proteinosis PAP ； Whole lung lavage WLL