肺泡蛋白沉积症15例临床分析  

Clinical Analysis of 15 Cases with Pulmonary Alveolar Proteinosis

作　　者：黄娜[1] 刘蔺[1] 袁泉[1] 刘丹[1] 李晓亮[1] 杨小东[1] 

机构地区：[1]四川大学华西医院呼吸内科,四川成都610041

出　　处：《华西医学》2009年第3期711-714,共4页West China Medical Journal

摘　　要：目的：肺泡蛋白沉积症（pulmonary alveolar proteinosis，PAP）是一种少见的肺部疾病，由于临床医生认识不足，容易误诊，而PAP通过灌洗治疗常常可获得较好的预后。本文通过分析PAP病例，总结PAP患者的临床特点、影像学表现和治疗方法，以提高该病的诊疗水平。方法：回顾性分析2003年2月-2008年5月四川大学华西医院呼吸科经病理确诊的15例PAP患者临床资料，并观察了全肺灌洗治疗PAP的效果。结果：PAP患者常见临床症状为咳嗽和进行性呼吸困难，体征正常或无特异性。胸部CT可表现为“地图样”、“铺路石样”或间质纤维化改变。全肺灌洗治疗的14例患者临床症状明显缓解。结论：肺泡蛋白沉积症虽然少见，但只要提高认识，诊断并不困难。支气管肺泡灌洗和（或）肺活检是确诊PAP的重要方法，全肺灌洗是治疗PAP的主要方法。Objective：pulmonary alveolar proteinosis （PAP） is a rare respiratory disease. Misdiagnosis frequently happen because of unfamiliarity with the disease. However, prognosis is determined by correct diagnosis, whole or segmental lung lavage therapy will improve the prognosis. We summarized our experience to investigate clinical manifestations,image feature and treatment of PAP by the analysis of the PAP cases. Methods：A retrospective analysis of 15 cases who were diagnosed of PAP with pathological evidences from February 2003 to May 2008 was made. The effects of therapeutic whole lung lavage on PAP were evaluated. Results：The PAP is clinically characterized by cough and progressive dyspnea. Physical examination is normal or reveals relatively minor and nonspecific pulmonary findings. Computed tomography scanning may show geographic air-space＂ground-glass＂opacities, ＂crazy-paving＂ pattern or interstitial fibrosis. 14 patients were relieved from clinical symptoms and image features. Conclusion： Pulmonary alveolar proteinosis is rare,but the diagnosis is not difficult if we are familiar with the syndrome. Bronchoalveolar lavage and lung biopsy are important diagnostic tool for assessment PAP. Therapeutic whole lung lavage is the principle option to treat patients with PAP.

关 键 词：肺泡蛋白沉积症 全肺灌洗 

分 类 号：R563.9[医药卫生—呼吸系统] R562[医药卫生—内科学]