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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 pulmonary disease that is often misdiagnosed due to inadequate knowledge of clinicians. However, PAP often achieves better prognosis through lavage. This paper analyzes the PAP cases, summarizes the clinical features of PAP patients, imaging findings and treatment methods to improve the diagnosis and treatment of the disease. Methods: The clinical data of 15 PAP patients diagnosed by pathology in Department of Respiratory Medicine, West China Hospital of Sichuan University from February 2003 to May 2008 were retrospectively analyzed. The effect of whole lung lavage on PAP was also observed. Results: The common clinical symptoms of PAP were cough and progressive dyspnea with normal or non-specific signs. Chest CT can be manifested as “map ”, “paving stone ” or interstitial fibrosis changes. The clinical symptoms of 14 patients treated with whole lung lavage were significantly relieved. Conclusions: Alveolar proteinosis is rare, but as long as awareness is raised, diagnosis is not difficult. Bronchoalveolar lavage and / or lung biopsy is an important method for the diagnosis of PAP, and whole lung lavage is the main method for the treatment of PAP.