肺泡微结石症一例报告并文献复习  

Pulmonary Alveolar Microlithiasis:One Case Report and Literature Review

作　　者：叶俊[1] 赵黎明[1] 方正[1] 修清玉[1] 李兵[1] 

机构地区：[1]第二军医大学附属长征医院呼吸内科,上海200003

出　　处：《中国呼吸与危重监护杂志》2013年第6期601-604,共4页Chinese Journal of Respiratory and Critical Care Medicine

摘　　要：目的 了解肺泡微结石症（ PAM） 的临床特征。方法 复习国内发表的肺泡微结石症的病例, 结合近期我院收治的1 例肺泡微结石症病例进行分析。结果 85 例患者中, 男41 例, 女44例; 确诊时年龄7 ～69 岁, 平均（ 27 ±15） 岁; 其中35 例患者分属15 个家庭, 均为同胞兄弟姐妹, 8 个家庭父母为近亲婚配。多数患者无症状或症状轻微, 肺功能检查基本正常或为限制性通气功能障碍。典型的影像学表现为双肺弥漫的以中下肺野为主的高密度小结节, 73 例患者结合X 线表现和轻微的临床症状而确诊。无有效治疗方法。结论 肺泡微结石症是一种常染色体隐性遗传疾病, 临床症状和影像学表现分离高度提示本病。Objective To explore the clinical features of pulmonary alveolar microlithiasis （ PAM） .Methods One case of PAM diagnosed on January 2012 in Changzheng Hospital was described, and 84cases in the medical literature in chinese after 1990 were reviewed.？Results There were 41 males and 44females, aged from 7 to 69 years （ mean 27 ±15 years） . 35 cases were sibling and were belong to 15families, inbreeding was found in 8 families. Most patients were asymptomatic and the majority of patientseither had normal or restrictive pulmonary function. The characteristic chest radiograph was fine sand-likemicronodulation of calcific densities bilaterally, involving mainly the middle and lower zones. According toclinical and radiological manifestation, the diagnosis of PAM was established in 73 cases. There is no knowneffective treatment for the disease.？Conclusion PAMis an autosomal recessive disorder. A hallmark of thedisease is a striking dissociation between the radiologic findings and the only mild clinical signs andsymptoms.

关 键 词：肺泡微结石症 临床特征 

分 类 号：R563.9[医药卫生—呼吸系统]