肺泡蛋白沉着症9例临床分析  

Pulmonary alveolar proteinosis:A clinical analysis of 9 cases

作　　者：张曙[1] 磨国鑫[1] 管希周[1] 胡红[1] 陈良安[1] 

机构地区：[1]解放军总医院呼吸科,北京100853

出　　处：《军医进修学院学报》2012年第2期151-152,164,共3页Academic Journal of Pla Postgraduate Medical School

摘　　要：目的探讨肺泡蛋白沉着症(PAP)的临床特征、疗效和预后。方法回顾性分析经治的9例PAP患者的临床资料,并复习文献。结果 9例患者中,男4例,女5例;发病年龄29-65岁,平均年龄42岁;主要临床表现为进行性呼吸困难6例,咳嗽4例,咳痰2例,发热1例,2例无症状;胸部影像呈多样性,胸部高分辨率CT(HRCT)可呈地图样、铺路石样特征性改变。9例均经支气管镜肺活检(TBLB)和支气管肺泡灌洗(BALF)明确诊断。结论 PAP以呼吸困难、咳嗽为主要临床表现,HRCT地图样、铺路石样表现为影像学特征,TBLB和BALF是确诊的主要依据;全肺灌洗术是最佳治疗方法。Objective To study the clinical features, therapeutic effect, and prognosis of pulmonary alveolar proteinosis（PAP）. Methods Clinical data about 9 cases of PAP（4 males and 5 females） were retrospectively analyzed with its related literature reviewed. Results The onset age of PAP was 42 years（mean 29-65 years）. The main clinical manifestations included dyspea in 6 cases, cough in 4 cases, expectoration in 2 cases, and fever in l case. No symptom was observed in 2 cases. Chest X-ray showed different lesions and high resolution computerized tomography （HRCT） revealed geographic and ＂crazy-paving＂ lesions throughout both lungs. Diagnosis of PAP was established by radio-assisted transbronchoscopic lung biopsy（TBLB） and bronchoalveolar lavage fluid（BALF）. Conclusion PAP is mainly characterized by dyspea and cough, geographic and ＂crazy-paving＂ lesions throughout both lungs found in HRCT, and is diagnosed based on the findings in TBLB or BALF. Large volume lavage of the whole lung is an effective and safe therapeutic procedure for it.

关 键 词：肺泡蛋白沉着症 肺疾病 诊断 治疗 

分 类 号：R563[医药卫生—呼吸系统]