摘要
肺泡蛋白沉积症是一种临床少见的病因不详的肺部疾病,以肺泡及细支气管腔内堆积过量的无定形过碘酸雪夫(PAS)染色阳性的蛋白性物质为特征。其发病呈先天性、获得性或继发于其他疾病;病程慢性迁延,特征性的表现为临床症状、体征与胸部影像学分离现象;支气管肺泡灌洗液检查及病理检查可为确诊提供依据;全肺灌洗治疗效果显著。
Pulmonary alveolar proteinosis （PAP） is a rare and cryptogenic lung disorder. It is characterized by excessive and abnormal accumulation of proteinaceous eosinophilic periodic acid/Schiff - positive material in the alveolar spaces of the lungs and bronchioles. It may be congenital, acquired or secondary to others. Its course is chronic and protracted. Clinically, PAP was characterized by its incompatibility among symptoms, signs and radiographic pictures. The examination of brochoalveolar lavage fluid and/or lung biopsy could be used to confirm the diagnosis of PAP. Whole lung lavage is an efficient therapy for PAP.
出处
《中国全科医学》
CAS
CSCD
2007年第7期575-577,共3页
Chinese General Practice
关键词
肺泡蛋白沉积症
全肺灌洗
Pulmonary alveolar proteinosis
Whole lung irrigation
