肺泡蛋白沉积症1例及文献复习

目的探讨肺泡蛋白沉积症（PAP）的临床特征及诊断。方法对1例患者的临床资料进行分析并复习相关文献。结果 PAP病程慢性迁延,以渐进性呼吸困难为主要表现。高分辨CT表现双肺弥漫分布的磨玻璃影,小叶间隔增厚,呈地图样分布并可见铺路石征;支气管肺泡灌洗液呈＂牛奶样＂改变静置有沉淀,且提示细支气管及肺泡腔内充满过碘酸雪夫（PAS）阳性的无细胞颗粒状物质。结论 PAP临床表现不具有特异性,HRCT检查及诊断性支气管肺泡灌洗有助于诊断,局部或全肺灌洗疗效肯定。

Report on 1 example pulmonary alveolar proleinoss

Objective To discuss the clinical manifestations and diagnosis of a patient with pulmonary alveolar proteinosis（PAP）.Methods one case of PAP was analyzed and the recent literature was reviewed.Results PAP presented a protracted chronic clinical course,It was characterized by progressived Dyspnea.Typical CT scan showed diffused ground-grass opacities and thick interlobular septa in both lower fields,Typically with ＂map＂ changed and ＂crazy paving＂ patterns and ＂milky＂ effluent from bronchoalveolar lavage fluid（BALF）.the microscopic characteristics of BALF from bronchoalveolar lavage of PAP indicated the alveolar space and terminal bronchioles almost completely filled with Periodic Acid Schiff（PAS）-positive surfactant.Conclusion The manifestations of PAP is not specific,image examinations and diagnostic bronchoalveolar lavege are very useful and lung lavage especially whole lung lavage is effective..