肺泡蛋白沉积症的影像学表现4例报道并文献复习  

The imaging findings of pulmonary alveolar proteinosis: a report of 4 cases and literature review

作　　者：陈进[1] 何海青[1] 张敏鸽[1] 陈盈[1] 米玉成[1] 樊树峰[1] 

机构地区：[1]浙江省台州医院放射科,浙江临海317000

出　　处：《中国医药导报》2013年第33期163-165,168,共4页China Medical Herald

基　　金：浙江省台州市科技局项目(编号121KYA08)

摘　　要：目的 分析肺泡蛋白沉积症（PAP）的胸部平片、CT影像表现,旨在提高对本病的认识.方法 回顾性分析浙江省台州医院4例经病理证实的PAP患者的胸部影像资料,其中男3例,女1例,年龄29～61岁;4例患者均行螺旋CT、高分辨率CT（HRCT）检查,1例行胸部平片;其中3例肺泡灌洗,1例肺穿刺证实.结果 ①原发型3例,胸部平片表现为双肺弥漫性分布斑片状密度增高影,边界不清,两侧肋膈角锐利,心影未见增大.1例胸部CT示病变弥漫呈片状分布,边缘锐利,与周围正常肺组织界限清晰,并在其衬托下呈“地图样”分布,同时可显示磨玻璃影中的网格状影（铺路石征）.2例胸部CT示两肺弥漫分布磨玻璃影,肺尖及肺基底部均累及,部分胸膜下区未累及,边界不清,部分肺组织实变,见空气支气管征.②继发型l例,CT显现左下肺散在片状磨玻璃样高密度影,与周围正常组织分界清楚;右下肺大片状高密度影,内见支气管走形,周围见晕征.③1例原发型PAP纵隔内见肿大淋巴结影,短径未超过1.5 cm.4例均未见胸腔积液和心影增大.结论 弥漫、区域性磨玻璃样阴影,地图或铺路石样改变,胸膜下区未累及,临床与影像表现不符等征象是PAP具有特征性的表现;若有基础疾病合并上述影像表现,应考虑合并PAP可能.Objective To analyze the chest plain film and CT imaging findings of pulmonary alveolar proteinosis （PAP）,in order to improve the understanding of this disease.Methods The imaging finding data of 4 patients （3 men and 1 woman,aged from 29 to 61） with pulmonary alveolar proteinosis （PAP） confirmed by pathology in Taizhou Hospital of Zhejiang Province were retrospectively analyzed.All 4 patients were given the examination of spiral computed tomograph and high resolution CT （HRCT）,1 patient was given the examination of chest plain film.3 patients were given bronchoalveolar lavage and 1 patient was given lung puncture for confirming.Results ①3 patients were belonged to the primary type：the manifestations of chest plain film were mottling shadows with high density diffused in both lung fields,the boundary was not clear,there was the sharp costophrenic angle in both sides and heart shadow was not enlargement.Chest CT of 1 patient showed that disease＇s characters were patchy distribution,sharp-edged clear boundary with normal lung tissue,＂geographic distribution＂ under normal lung tissue,and thread net in ground-glass opacity （slabstone signs）.Chest CT of 2 patients showed that ground-glass opacity was diffused in both sides of lung including apex pulmonis and the bottom of lung,some parts of sub-pleural regions were involved,boundary was not clear,some parts of lung were consolidation shadow,and air bronchogram was seen.②1 patient was belonged to the secondary type：chest CT showed that there were scattering distribution of flake high density with ground-glass opacity in left lower lobe,sharp-edged clear boundary with normal lung tissue; there were large patchy shadows in right lower lobe with canal gas-path and halo sign surrounding.③There was mediastinal enlarged lymph node which short diameter was not above 1.5 cm in 1 case of the primary type of PAP.There was no pleural effusion or enlargement of the heart in all 4 patients.Conclusion Diffused and regional shadow of ground-glass o

关 键 词：肺泡蛋白沉积症 磨玻璃影 体层摄影术 X线计算机 

分 类 号：R563.9[医药卫生—呼吸系统]