肺泡蛋白沉积症6例临床分析

目的了解肺泡蛋白沉积症(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。方法对6例经支气管肺泡灌洗液病检或胸腔镜肺活检证实的PAP患者的症状、体征和影像学特点进行了回顾性分析,并观察了全肺灌洗治疗PAP的效果。结果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。5例患者支气管肺泡灌洗沉淀物PAS染色阳性。全肺灌洗治疗的5例患者临床症状明显缓解,影像学表现明显改善。结论支气管肺泡灌洗和(或)肺活检是确诊PAP的重要方法.典型的胸部CT呈“地图”样和“铺路石”样改变。全肺灌洗是治疗PAP的主要方法。

Clinical Analysis of 6 Patients with Pulmonary Alveolar Proteinosis

Objective To investigate clinical manifestations,image feature and treatment of pulmonary alveolar proteinosis(PAP).Methods A retrospective analysis was conducted on 6 patients with PAP who were diagnosed by lung biopsy and pathological examination of bronchoalveolar lavage fluid(BALF).The changes of clinical symptoms,signs and image feature were studied.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.The periodic acidic Schiff staining(PAS)in BALF was positive in 5 patients with PAP.After whole lung lavage,5 patients were relieved from clinical symptoms and image features was proved.Conclusion Bronchoalveolar lavage and lung biopsy were important diagnostic tool for assessment PAP.Computed tomography scan of the chest has a characteristic appearance of geographic and/or "crazy-paving" pattern.Therapeutic whole lung lavage is the principle option to treat patients with PAP.