Clinical Analysis of 15 Cases with Pulmonary Alveolar Proteinosis_West China Medical Journal

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Department of Respiratory Medicine,West China Hospital,Sichuan University,Chengdu 610041,China;

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Keywords：

肺泡蛋白沉积症; 全肺灌洗

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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。

Citation： HUANG Na,LIU Lin,YUAN Quan,et al. Clinical Analysis of 15 Cases with Pulmonary Alveolar Proteinosis. West China Medical Journal, 2009, 24(3): 711-714. doi: Copy

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6.	王选锭,罗发满,刘富光,等.粒巨噬细胞集落刺激因子在肺泡蛋白沉积症患者中的表达[J].中华结核和呼吸杂志,2001,24(7):417-419..
7.	萧淑华,钟韵,彭穗玮,等.肺泡蛋白沉积症7例误诊分析[J].新医学,2005,36(6):345-346..
8.	吴升,王选锭,沈华浩,等.肺泡蛋白沉积症10例临床分析及全肺灌洗治疗体会[J].临床内科杂志,2005,22(11):755-757..

1. ROSEN SH,CASTLEMAN B,LIEBOW AA.Pulmonary alveolar proteinosis[J].N Engl J Med,1958,258 (23):1523-1543..
2. SHAH PL,HANSELL D,LAWSON DR,et al.Pulmonary alveolar proteinosis:clinical aspects and current concepts on pathogenesis[J].Thorax,2000,55 (1):67-77..
3. DRANOFF G,CRAWFORD AD,SADELAINM,et al.Involvement of granulocytemacrophage colonystimulating factor in pulmonary homeostasis[J].Science,1994,264(3):713-716.
4. TANAKA N,WATANABE J,KITAMURA T,et al.Lungs of patients with idiopathic pulmonary alveolar proteinosis express a factor which neutralizes granulocytemacrophage colony stimulating factor[J].FEBS Letters,1999,442(2-3):246-250..
5. GARRAWAY MS,GHIO AJ,CARTER JD,et al.Detection of granulocytemacrophage Colonystimulating factor in patients with pulmonary alveolar proteinosis[J].AM J Respire Crit Care Med,2000,161 (4 Pt 1):1294-1299..
6. 王选锭,罗发满,刘富光,等.粒巨噬细胞集落刺激因子在肺泡蛋白沉积症患者中的表达[J].中华结核和呼吸杂志,2001,24(7):417-419..
7. 萧淑华,钟韵,彭穗玮,等.肺泡蛋白沉积症7例误诊分析[J].新医学,2005,36(6):345-346..
8. 吴升,王选锭,沈华浩,等.肺泡蛋白沉积症10例临床分析及全肺灌洗治疗体会[J].临床内科杂志,2005,22(11):755-757..

West China Medical Journal

Clinical Analysis of 15 Cases with Pulmonary Alveolar Proteinosis

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