• 四川大學(xué)華西醫(yī)院呼吸內(nèi)科,四川成都 610041;

目的:肺泡蛋白沉積癥(pulmonary alveolar proteinosis,PAP)是一種少見的肺部疾病,由于臨床醫(yī)生認(rèn)識(shí)不足,容易誤診,而PAP通過灌洗治療常?？色@得較好的預(yù)后。本文通過分析PAP病例,總結(jié)PAP患者的臨床特點(diǎn)、影像學(xué)表現(xiàn)和治療方法,以提高該病的診療水平。方法:回顧性分析2003年2月～2008年5月四川大學(xué)華西醫(yī)院呼吸科經(jīng)病理確診的15例PAP患者臨床資料,并觀察了全肺灌洗治療PAP的效果。結(jié)果:PAP患者常見臨床癥狀為咳嗽和進(jìn)行性呼吸困難,體征正常或無特異性。胸部CT可表現(xiàn)為“地圖樣”、“鋪路石樣”或間質(zhì)纖維化改變。全肺灌洗治療的14例患者臨床癥狀明顯緩解。結(jié)論:肺泡蛋白沉積癥雖然少見,但只要提高認(rèn)識(shí),診斷并不困難。支氣管肺泡灌洗和(或)肺活檢是確診PAP的重要方法,全肺灌洗是治療PAP的主要方法。

引用本文： 黃娜,劉藺,袁泉,劉丹,李曉亮,楊小東. 肺泡蛋白沉積癥15例臨床分析. 華西醫(yī)學(xué), 2009, 24(3): 711-714. doi: 復(fù)制

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.I(xiàn)nvolvement 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. 王選錠,羅發(fā)滿,劉富光,等.粒巨噬細(xì)胞集落刺激因子在肺泡蛋白沉積癥患者中的表達(dá)[J].中華結(jié)核和呼吸雜志,2001,24(7):417-419..
7. 蕭淑華,鐘韻,彭穗瑋,等.肺泡蛋白沉積癥7例誤診分析[J].新醫(yī)學(xué),2005,36(6):345-346..
8. 吳升,王選錠,沈華浩,等.肺泡蛋白沉積癥10例臨床分析及全肺灌洗治療體會(huì)[J].臨床內(nèi)科雜志,2005,22(11):755-757..
  1. 1. ROSEN SH,CASTLEMAN B,LIEBOW AA.Pulmonary alveolar proteinosis[J].N Engl J Med,1958,258 (23):1523-1543..
  2. 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. 3. DRANOFF G,CRAWFORD AD,SADELAINM,et al.I(xiàn)nvolvement of granulocytemacrophage colonystimulating factor in pulmonary homeostasis[J].Science,1994,264(3):713-716.
  4. 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. 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. 6. 王選錠,羅發(fā)滿,劉富光,等.粒巨噬細(xì)胞集落刺激因子在肺泡蛋白沉積癥患者中的表達(dá)[J].中華結(jié)核和呼吸雜志,2001,24(7):417-419..
  7. 7. 蕭淑華,鐘韻,彭穗瑋,等.肺泡蛋白沉積癥7例誤診分析[J].新醫(yī)學(xué),2005,36(6):345-346..
  8. 8. 吳升,王選錠,沈華浩,等.肺泡蛋白沉積癥10例臨床分析及全肺灌洗治療體會(huì)[J].臨床內(nèi)科雜志,2005,22(11):755-757..