肺泡蛋白沉积症一例并文献回顾

目的 探讨肺泡蛋白沉积症的临床特征及诊断.方法 分析就诊于上海交通大学医学院附属新华医院呼吸科的1例经支气管肺泡灌洗及左肺活榆术后病理确诊的肺泡蛋白沉积症患者的临床资料,并结合文献进行复习.结果 肺泡蛋白沉积症病程慢性迁延,根据发病可分为三大类:先天性、获得性和继发性.以进行性呼吸困难为主要表现,胸部CT示舣肺呈磨玻璃...

One case of pulmonary alveolar proteinosis with literature review

Objective To discuss the clinical manifestations and diagnosis of a patient with pulmonary alveolar proteinosis (PAP). Methods One case of PAP confirmed by pathology of broncho alveolar lavage (BAD and left lung biopsy in our hospital was reported. Relevant references about PAP published were reviewed. Results PAP presented a protracted chronic clinical course, occurring in three clinically distinct forms:congenital, acquired and secondary. Progressive dyspnea proved to be main clinic manifestation of PAP. Chest computed tomography scan of PAP revealed diffuse, multifocal, patchy ground glass opacities. Broncho alveolar lavage fluid was milky in appearance. PAP was characterized by the accumulation of a lipoproteinaceous, eosinophilic, PAS positive material within alveoli. Conclusions The manifestations of PAP is not specific, image examinations and diagnostic BAL are very useful for diagnosis. Whole lung lavage was considered to be effective while segmental lavage should be more feasible.