- 期刊
Therapeutic Bronchoscopic Segmental/Lobar Lavage for Unilateral Pulmonary Alveolar Proteinosis-A Case Report
支氣管鏡肺葉灌洗術在單側肺泡蛋白質沉著症的治療－病例報告
摘要
肺泡蛋白質沉著症是一罕見的疾病，其特徵是肺泡中堆積periodic acid-Schiff染色陽性物質，病因目前仍不明。我們報告一位51歲女性，於住院前五個月出現乾咳及漸進性呼吸困難。胸部X光片顯示，右肺中葉及下葉有實質化病灶，左肺有肺氣腫變化。胸部電腦斷層攝影呈現，毛玻璃樣的病灶以及不規則石板拼鋪型態(crazy-paving pattern)。經支氣管鏡檢查暨診斷性支氣管肺泡灌洗術和經支氣管肺切片檢查，確診為肺泡蛋白質沉著症。基於病人有嚴重低血氧症以及左肺部呈現肺氣腫變化，在給予鎮靜劑及置放氣管內管下，為病人施行經支氣管鏡肺葉灌洗術。治療後，病人症狀明顯改善而出院，五個月後的門診追蹤顯示病人的復原情形良好。經支氣管鏡肺葉灌洗術之施行難度不高，可在大多數醫院執行，特別是針對接受全肺灌洗術可能具有較高風險的肺泡蛋白質沉著症病人。
並列摘要
Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by an accumulation of periodic acid-Schiff-positive materials in the alveolar space. The typical image findings are bilateral diffuse interstitial and parenchymal infiltrates shown on chest radiographs and/ or computed tomography (CT) of the chest. We report a case of unilateral PAP in a 51-year-old woman with unilateral emphysema. Chest radiographs and thoracic CT demonstrated findings that were highly suggestive of PAP in the right lung and emphysema in the left lung. Bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBLB) were performed and a diagnosis of idiopathic PAP (iPAP) was made based on the gross appearance of retrieved BAL fluid (BALF), cytological examination of the BALF, and the pathologic findings of TBLB specimens. Because the patient had significant hypoxemia, therapeutic bronchoscopic segmental-lobar lavage was performed under sedation and endotracheal tube intubation. After 4 cycles of lobar lavage, the clinical symptoms improved remarkably and the patient was discharged and followed up at the outpatient department. Therapeutic bronchoscopic segmental/lobar lavage may be effective in patients with iPAP, especially in those who are at high risk for therapeutic whole lung lavage.
延伸閱讀
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