肺泡蛋白沉积症二例诊治体会并文献复习

A Report of 2 cases of Pulmonary Alveolar Proteinosis,with Literature Review

目的 探讨肺泡蛋白沉积症 (PAP)的临床特征、诊断及治疗。方法 对 2例患者的临床资料进行分析并复习文献。结果 PAP以进行性呼吸困难为表现 ,CT示双肺毛玻璃样改变 ,支气管肺泡灌洗液呈“牛奶样”改变为特征 ,肺活检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。全肺灌洗后症状、动脉血氧分压及胸部CT可得到明显改善。结论 PAP临床表现不具有特异性 ,影像学检查尤其是高分辨率CT及诊断性支气管肺泡灌洗有助于诊断 ,确诊有赖于肺活检。全肺灌洗疗效肯定。

Objective To study the clinical manifestations, d ia gnosis and treatment of Pulmonary alveolar proteinosis(PAP).Methods Two cases of PAP was analyzed and the recent literature was reviewed.Res ults PAP was characterized by progressive dyspnea.Typical CT scan showed ground-glass opacities and “milky” effluent from bronchoalveolar lavage.The m icroscopic characteristics of PAP were the alveolar space and terminal bronchio les almost completely filled with Periodic Acid Schiff(PAS),-positive acellula r surfactant.There were great improvements in symptoms,PaO 2 and chest CT scann ing after whole-lung lavage.Conclusion The manifestations of PAP i s not specific. Image examinations especially high-resolution CT scanning and d iagnostic bronchoalveolar lavage are very useful.The gold-standard for diagnosi s is lung biopsy.Whole-lung lavage is effective.