• 第二軍醫(yī)大學附屬長海醫(yī)院呼吸內(nèi)科(上海 200433);

肺泡蛋白沉積癥(PAP)是一種罕見的疾病,其特征是肺泡內(nèi)間歇蓄積PAS染色陽性的富含磷脂的蛋白質(zhì)樣物質(zhì),從而影響到肺泡的氣體交換,導致呼吸困難、低氧血癥等一系列臨床綜合征。PAP可分為原發(fā)性、繼發(fā)性和先天性三種類型,其中90%是原發(fā)性PAP,其發(fā)病原因不明。目前原發(fā)性PAP最常用的治療方法是全肺灌洗術,但該治療需在全
身麻醉下進行,設備要求高,有一定的風險,且療效難以持久?,F(xiàn)報告1例經(jīng)過全肺灌洗術后效果不佳,再聯(lián)合皮下注射重組人粒細胞-巨噬細胞集落刺激因子(rHuGlV1.CSF,特爾立,廈門特寶生物工程有限公司)治療后病情明顯好轉(zhuǎn)的原發(fā)性PAP患者,并結(jié)合相關文獻,以加深對這種新療法的認識。

引用本文: 張偉,李強,董宇超,姚小鵬. 全肺灌洗術聯(lián)合皮下注射重組人粒細胞-巨噬細胞集落刺激因子治療原發(fā)性肺泡蛋白沉積癥一例并文獻復習. 中國呼吸與危重監(jiān)護雜志, 2008, 08(4): 298-300. doi: 復制

1. 8 汪琪琪,劉守智.全肺灌洗聯(lián)合粒細胞-巨噬細胞集落刺激因子治療肺泡蛋白沉積癥一例.中國呼吸與危重監(jiān)護雜志,2007,6:225.
2. Seymour JF,Presneill JJ.Pulmonary alveolar proteinosis:progress in the first 44 years.Am J Respir Crit Care Med,2002,166:215-235.
3. Stanley E,Lieschke GJ,Grail D,et al.Granulocyte/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology.Proc Natl Acad Sci USA,1994,91:5592-5596.
4. Dranoff G,Crawford AD,Sadelain M,et al.Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis.Science,1994,264:713-716.
5. Doerschuk CM.Pulmonary alveolar proteinosis--is host defense awry? N Engl J Med,2007,356:547-549.
6. Uchida K,Beck DC,Yamamoto T,et al.GM-CSF autoantibodies and neutrophil dysfunction in pulmonary alveolar proteinosis.N Engl J Med,2007,356:567-579.
7. Bonfield TL,Russell D,Burgess S,et al.Autoantibodies against granulocyte macrophage colony-stimulating factor are diagnostic for pulmonary alveolar proteinosis.Am J Respir Cell Mol Biol,2002,27:481-486.
8. Reed JA,Ikegami M,Cianciolo ER,et al.Aerosolized GM-CSF ameliorates pulmonary alveolar proteinosis in GM-CSF-deficient mice.Am J Physiol,1999,276:556-563.
9. Kavuru MS,Sullivan EJ,Piccin R,et al.Exogenous granulocyte-macrophage colony-stimulating factor administration for pulmonary alveolar proteinosis.Am J Respir Crit Care Med,2000,161:1143-1148.
  1. 1. 8 汪琪琪,劉守智.全肺灌洗聯(lián)合粒細胞-巨噬細胞集落刺激因子治療肺泡蛋白沉積癥一例.中國呼吸與危重監(jiān)護雜志,2007,6:225.
  2. 2. Seymour JF,Presneill JJ.Pulmonary alveolar proteinosis:progress in the first 44 years.Am J Respir Crit Care Med,2002,166:215-235.
  3. 3. Stanley E,Lieschke GJ,Grail D,et al.Granulocyte/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology.Proc Natl Acad Sci USA,1994,91:5592-5596.
  4. 4. Dranoff G,Crawford AD,Sadelain M,et al.Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis.Science,1994,264:713-716.
  5. 5. Doerschuk CM.Pulmonary alveolar proteinosis--is host defense awry? N Engl J Med,2007,356:547-549.
  6. 6. Uchida K,Beck DC,Yamamoto T,et al.GM-CSF autoantibodies and neutrophil dysfunction in pulmonary alveolar proteinosis.N Engl J Med,2007,356:567-579.
  7. 7. Bonfield TL,Russell D,Burgess S,et al.Autoantibodies against granulocyte macrophage colony-stimulating factor are diagnostic for pulmonary alveolar proteinosis.Am J Respir Cell Mol Biol,2002,27:481-486.
  8. 8. Reed JA,Ikegami M,Cianciolo ER,et al.Aerosolized GM-CSF ameliorates pulmonary alveolar proteinosis in GM-CSF-deficient mice.Am J Physiol,1999,276:556-563.
  9. 9. Kavuru MS,Sullivan EJ,Piccin R,et al.Exogenous granulocyte-macrophage colony-stimulating factor administration for pulmonary alveolar proteinosis.Am J Respir Crit Care Med,2000,161:1143-1148.