全肺灌洗治疗肺泡蛋白沉积症6例的临床分析

Clinical Analysis of Whole Lung Lavage in Treatment of 6 Cases With Pulmonary Alveolar Proteinosis

目的:总结肺泡蛋白沉积症(PAP)的临床特征,评价大容量全肺灌洗治疗的临床疗效和安全性。方法:回顾性分析6例PAP患者临床资料。结果:6例患者均行支气管镜肺活检(TBLB)和支气管肺泡灌洗液(BALF)镜检,PAS染色阳性。1例肺活检病理检查符合肺泡蛋白沉积症。6例患者行全肺灌洗治疗,治疗后临床症状、胸部影像学、肺功能、动脉血气均明显改善。结论:胸部CT双肺弥漫性病变呈"地图样"或"铺路石样"特征性改变应警惕本病的可能,TBLB、BALF及肺活检病理检查可确诊PAP,大容量全肺灌洗治疗PAP安全有效。

Objective：To summarize clinical characteristics of pulmonary alveolar proteinosis （PAP） and evaluate the efficacy and safety of large volume whole lung lavage in the treatment of PAP. Methods ： Clinical data of 6 cases with PAP were analyzed retrospectively. Results： Six cases were diagnosed by transbronchoscopic lung biopsy （TBLB） and bronchoalveolar lavage fluid （BALF） with periodic acid-schiff staining. One case showed PAP by lung biopsy. Six cases were treated by whole lung lavage. The clinical symptoms, chest radiographs, pulmonary function and arterial blood gas parameters were markedly improved after whole lung lavage. Conclusion： It should be alert to PAP when, ＂geographic＂ or ＂crazy paving＂ changes in chest CT, TBLB,BALF and lung biopsy can make the accurate diagnosis. Large volume whole lung lavage is an effective and safe therapeutic method in treatment of PAP.