肺泡微结石症8例临床分析及文献复习  

Clinical analysis of eight pulmonary alveolar microlithiasis and review of the literature

作　　者：李秋红[1] 周瑛[1] 胡洋[1] 张苑[1] 苏奕亮[1] 张芬[1] 沈瓅 尤小芳[2] 谢惠康[3] 李惠萍[1] Li Qiuhong, Zhou Ying, Hu Yang, Zhang Yuan, Su Yiliang,Zhang Fen, Shen Li, You Xiaofang, Xie Huikang, Li Hui ping(Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai 200433, China)

机构地区：[1]同济大学附属上海市肺科医院呼吸科,200433 [2]同济大学附属上海市肺科医院影像科,200433 [3]同济大学附属上海市肺科医院病理科,200433

出　　处：《国际呼吸杂志》2018年第20期1582-1587,共6页International Journal of Respiration

基　　金：上海申康医院发展中心临床创新计划资助（16CR3054A）

摘　　要：目的分析儿童及成人肺泡微结石症（pulmonary alveolar microlithiasis,PAM）的临床特点。方法结合我院的8例患者及复习国内发表的病理或胸部CT确诊的PAM共143例进行分析。结果儿童组共13例，成人组共130例，呈家族发病的比率为64.60%（73/113），临床表现不典型，体检发现者有53例（37.06%），常见的临床症状为气急（30.77%）、干咳（27.97%）。130例患者行胸部CT检查，儿童组与成人组比较，共同特点均以弥漫性小结节为主，伴有小叶间隔增厚或钙化。但儿童组磨玻璃影较成人组更为常见（儿童53.85%;成人26.50%），成人组较儿童组钙化融合更为常见（成人61.54%;儿童23.08%）,差异有统计学意义（χ2=7.091，P〈0.05）。有病理确诊的患者有81例，其中支气管镜和肺穿刺确诊的有69例。在两组病例中，经病理确诊和仅根据胸部CT明确诊断之间差异无统计学意义（χ2=0.923，P=0.337），说明本病的胸部CT表现特征性明显，可以作为独立的确诊手段。儿童组肺功能正常者有7例（70%），成人组正常者有29例（46.77%）。肺功能下降以限制性通气功能障碍为主，弥散功能可减退，下降的程度与合并肺纤维化、肺气肿等相关。病例随访期1个月至20年不等，仅1例死亡，其余患者在随访过程中病灶缓慢增多，部分病例可合并感染、气胸、肺纤维化、肺气肿、肺动脉高压、肺心病而导致病情加重。本病目前尚无特异的治疗方法，以对症治疗为主。结论PAM在我国仍属少见病。其影像学表现具有特征性，临床表现不典型，60%以上患者有家族史。胸部CT结合支气管镜下肺泡灌洗或活检可确诊。Objective To describe the clinical features and diagnosis of pulmonary alveolar microlithiasis （PAM） in adults and children. Methods We reviewed 143 cases from the literature from year 1994 to 2014 and report 8 cases in Shanghai Pulmonary Hospital which were tissue-confirmed or diagnosed by chest computed tomography （CT）. Results There were 13 children and 130 adults. The disease presented a high incidence of familial occurrence （64.60%,73/113）. In most patients, the disease was diagnosed in routine examinations （ 37.06%）, In symptomatic patients, typical complaints were dyspnoea （ 30.77%）,nonproductive cough （ 27.97%）. One hundred and thirty patients had chest tomography scans. The diffuse small parenchymal nodules and interlobular septal thickening or calcification were the same findings in children and adults. Ground-glass opacities were more common in children than in adults （child ：53.85% ; adult ：26.50%）. But the dense consolidations of calcification were usually found in adults （adult：61.54%; child： 23. 08 % ; χ2 =7 .091,P〈0.05）; Sixty-eight cases were diagnosed from the specimens and tissues under bronchoscopy and by lung puncture in 80 tissue-confirmed cases. There were no statistical significance between pathological diagnosed and CT diagnosed in children and adults （χ2 =0.923, P=0.337）. So CT had helped to characterize could be very useful in the diagnosis of PAM. Seven children （70%） and 2 lung functions. The lung function disorder were restrictive ventilation diffuse lung function. The extent of this disorder was concerned with pulm the imaging 9 of the disease and .77%） had normal and the decline of onary fibrosis or emphysema. It might remain static （from 1 month to 20 years in the literature） as regards to both symptoms and radiographic findings, only 1 patients died. While in others it might worsen over time during the follow-up period, complicated with pulmonary fibrosis, infection, pneumothorax, emphysema, leading to pulmonary hypertensi

关 键 词：肺泡微结石症 胸部CT 病理学 

分 类 号：R563[医药卫生—呼吸系统]