肺泡蛋白沉积症的影像学诊断与临床分析.docVIP

肺泡蛋白沉积症的影像学诊断与临床分析 何顺清 唐西平 何飞鸿 单伯文 （广州增城市新塘医院影像科，广州511340） 【摘要】　目的　通过探讨肺泡蛋白沉积症（PAP）的X线、CT影像学表现与临床特点，提高对本病的认识。方法　回顾分析7例经病理证实的肺泡蛋白沉积症患者的X线、CT影像学及临床表现。结果 影像学表现多样化，主要为地图样表现、碎石路样表现、肺实变表现（支气管充气征）、肺水肿样表现及肺间质纤维化样表现等，肺部阴影明显而且相对稳定，但是临床症状较轻。典型的影像学表现与临床症状常不相称是PAP的特点。结论 肺泡蛋白沉积症影像学表现具有一定的特征性，结合临床症状，基本能作出正确诊断，确诊有赖病理。 【关键词】　肺泡蛋白沉积症；体层摄影术；X线计算机；支气管肺泡灌洗 【中图分类号】R563.9 R816.41 【文献标识码】A Imaging Diagnosis and Clinical Analysis of Pulmonary Alreolar Proteinosis HE Shun-qing,TANG Xi-ping,HE Fei-hong,DAN Bo-wen (Department of Imaging ,Zengcheng Xintang Hospital,Guangzhou511340,China) 【Abstract】　Objective To investigate the X-ray、CT imaging features and clinical Characteristics of pulmonary alreolar proteinosis,Improving the understanding of this disease. Methods X-ray、CT imaging and clinical findings of 7 patients with pathology-confirmed pul- monary alreolar proteinosis were retrospectively reviewed. Results Imaging features of PAP were Diversified, characterized as geographic, the crazy- paving pattern, lobar or segmental consolidation ( air- bronchogram sign) and like pulmonary edema or intersititial fibrosis. The manifestations were obvious and relatively stabile ,were more serious than the symptoms.The typ- ical imaging findings which were not matched with clinical symptoms were the features of the PAP Conclusion A careful analysis of the typical imaging features of PAP, combining with clinical symptoms , should be conducted in order to make the accurate diagnosis. and final diagnosis was depended on pathology. 【Key words】Pulmonary alreolar proteinosis;Tomagraphy;X-ray computed ; Bronchial alveolar lavage , BAL 肺泡蛋白沉积症(pulmonary alveolarp roteinosis , PA P)是一种罕见的，以肺泡腔及终末呼吸性细支气管内大量沉积磷脂蛋白样物质为特征的肺部弥漫性疾病[1，2]。本病病因未明，起病隐匿，临床症状没有特征性，主要是活动后呼吸困难，咳嗽，咳痰，且影像医师对本病缺乏足够的认识，因此误诊率极高。本研究回顾我院2003年6月至2013年7月期间发现的7例肺泡蛋白沉积症，分析其X线胸片、胸部CT表现特征及临床特点，以提高对本病的认识和诊断能力。 1 材料与方法 1.1 一般资料 本组7例肺泡蛋白沉积症例患者中，男5例，女2例，年龄29～63岁，平均年龄46岁，其中40—50岁4例，占57%，病程3个月～8年，平均26个月。6例有吸烟史，农民3名，制衣厂（灰尘大）工人2名，电焊工人1名，洗水厂（接触化学