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目的 对肺泡蛋白沉着症 (PAP)患者的诊治情况加以总结 ,希望提高对本病的认识。方法 从诊断和治疗方面分析了 1997～ 2 0 0 2年上海市肺科医院呼吸内科经病理确诊的 6例PAP患者。结果 6例患者中 3例行肺灌洗 ,其中 2例经纤支镜检 ,1例插Carlen’s双腔管灌洗 ,后者症状、X线胸片、血气、肺功能明显改善。结论 综合文献复习 ,认识本病主要表现为咳嗽、进行性呼吸困难、呼吸音降低 ,X线胸片、CT示双肺弥漫性阴影自肺门向外放射。确诊靠病理检查结果。支气管肺泡灌洗 (BAL)仍是迄今唯一有效的治疗方法。
Objective To summarize the diagnosis and treatment of patients with pulmonary alveolar proteinosis (PAP) and hope to improve the understanding of this disease. Methods From diagnosis and treatment, 6 cases of pathologically diagnosed PAP in respiratory department of Shanghai Pulmonary Hospital from 1997 to 2002 were analyzed. Results Of the 6 patients, 3 patients underwent lung lavage. Among them, 2 patients underwent microscopic bronchoscopy and Carlen’s double-lumen tube lavage. The latter symptoms, X-ray, blood gas and pulmonary function were significantly improved. Conclusions Comprehensive literature review, understanding the disease mainly manifested as cough, progressive dyspnea, decreased respiratory sounds, chest X-ray, CT showed diffuse shadow of the lungs from the hilar outward emission. Diagnosis by pathological examination results. Bronchoalveolar lavage (BAL) is by far the only effective treatment.