局部肺叶灌洗治疗合并呼吸衰竭的重症肺泡蛋白沉积症三例并文献复习_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

刘美红 , 李艳霞 ,  董畅

大连医科大学附属第一医院呼吸与危重症医学科（辽宁大连 116011）;

Corresponding author：

Keywords：

肺泡蛋白沉积症; 呼吸衰竭; 局部肺叶灌洗; 自愈倾向

DOI：

10.7507/1671-6205.202012015

Video：

Export PDF Favorites Scan Get Citation

Abstract Full text Figures/Tables Video References Cited by

目的探讨局部肺叶灌洗治疗合并呼吸衰竭的重症肺泡蛋白沉积症（pulmonary alveolar proteinosis，PAP）的可行性及安全性。方法回顾3例合并呼吸衰竭的PAP患者通过局部肺叶灌洗后逐渐自愈的诊治经过，并复习相关中外文献进行总结分析。结果 3例患者均为男性，年龄50～55岁，有长期的粉尘、生物燃料或者消毒物质的密切接触史，以“咳嗽、呼吸困难”入院。入院时呼吸空气动脉血气分析均提示I型呼吸衰竭，胸部高分辨率CT（high-resolution computed tomography，HRCT）呈“铺路石征”；1例患者取支气管肺泡灌洗液、2例患者取支气管镜肺活检行过碘酸–雪夫染色结果均为阳性而确诊PAP。2例患者接受了1次局部肺叶灌洗，1例患者接受了2次局部肺叶灌洗，灌洗后1～5 d复查胸部HRCT与灌洗前相仿，但呼吸困难症状均较前明显改善，动脉血气分析提示呼吸衰竭纠正。出院后密切随诊1～6个月，患者均无呼吸困难复发，胸部HRCT提示双肺弥漫性斑片影几乎全部吸收。截止到2021年1月，在中国知网、维普、万方等数据库以“肺泡蛋白沉积症”和“支气管肺泡灌洗”为检索词，在PubMed数据库以“pulmonary alveolar proteinosis”和“lobar lavage”为关键词，共检索到相关中英文文献64篇，其中合并呼吸衰竭的重症PAP患者43例。大部分报道是利用反复、多次的局部肺叶灌洗，将双肺的所有肺叶逐一进行灌洗，最多者局部肺叶灌洗次数多达20次，时间跨度达4个月；一些研究是将局部肺叶、肺段灌洗作为“预洗”或者“桥梁”，改善临床症状后再进行全肺灌洗；一些研究对比了接受全肺灌洗或肺叶灌洗患者的治疗效果，认为两者疗效相仿，大部分患者仅需要1次灌洗，临床症状即有明显改善。结论肺叶局部灌洗治疗合并呼吸衰竭的重症PAP是安全有效的，同时在1～2次局部肺叶灌洗后，1～6个月随访PAP患者病情呈自愈倾向，短时间内反复、多次灌洗可能是不必要的。

Citation： 刘美红, 李艳霞, 董畅. 局部肺叶灌洗治疗合并呼吸衰竭的重症肺泡蛋白沉积症三例并文献复习. Chinese Journal of Respiratory and Critical Care Medicine, 2022, 21(10): 737-740. doi: 10.7507/1671-6205.202012015 Copy

1.	Sadeghi HA. Segmental lung lavage with fiberoptic bronchoscopy in a patient with special presentation of pulmonary alveolar proteinosis. Tanaffos, 2013, 12(4): 48-52.
2.	Edis EC, Tabakoglu E, Caglar T, et al. Treatment of a primary pulmonary alveolar proteinosis case with severe hypoxaemia by using segmental lavage technique. Ann Acad Med Singap, 2007, 36(10): 871-872.
3.	徐作军, 许文兵, 曹伟标, 等. 经纤维支气管镜分段肺泡灌洗治疗肺泡蛋白沉积症. 中华结核和呼吸杂志, 1997, 20(ZK): 89-90.
4.	Vast C, Demonet B, Mouveroux J. Value of selective pulmonary lavage under fibroscopic control in alveolar proteinosis. Poumon Coeur, 1978, 34(4): 305-307.
5.	Rodrigo D, Rathnapala A, Senaratne W. Therapeutic limited bronchoalveolar lavage with fiberoptic bronchoscopy as a bridging procedure prior to total lung lavage in a patient with pulmonary alveolar proteinosis: a case report. J Med Case Rep, 2015, 9: 93.
6.	Firat ND, Cıledağ A, Kabalak PA, et al. Pulmonary alveolar proteinosis and successful therapy with combined lavage procedures: Case reports. Exp Ther Med, 2011, 2(3): 569-573.
7.	Cheng SL, Chang HT, Lau HP, et al. Pulmonary alveolar proteinosis: treatment by bronchofiberscopic lobar lavage. Chest, 2002, 122(4): 1480-1485.
8.	Xu ZH, Jing JY, Wang HY, et al. Pulmonary alveolar proteinosis in China: a systematic review of 241 cases. Respirology, 2009, 14(5): 761-766.
9.	Skoczynski S, Wyskida K, Rzepka-Wrona P, et al. Novel method of noninvasive ventilation supported therapeutic lavage in pulmonary alveolar proteinosis proves to relieve dyspnea, normalize pulmonary function test results and recover exercise capacity: a short communication. J Thorac Dis, 2018, 10(4): 2467-2473.
10.	Boyce DSK, Lee JW, Shah P, et al. Combined-modality therapy for pulmonary alveolar proteinosis in a remote setting: a case report. BMC Pulm Med, 2019, 19(1): 61.
11.	Tazawa R, Trapnell BC, Inoue Y, et al. Inhaled granulocyte/macrophage-colony stimulating factor as therapy for pulmonary alveolar proteinosis. Am J Respir Crit Care Med, 2010, 181(12): 1345-1354.

1. Sadeghi HA. Segmental lung lavage with fiberoptic bronchoscopy in a patient with special presentation of pulmonary alveolar proteinosis. Tanaffos, 2013, 12(4): 48-52.
2. Edis EC, Tabakoglu E, Caglar T, et al. Treatment of a primary pulmonary alveolar proteinosis case with severe hypoxaemia by using segmental lavage technique. Ann Acad Med Singap, 2007, 36(10): 871-872.
3. 徐作军, 许文兵, 曹伟标, 等. 经纤维支气管镜分段肺泡灌洗治疗肺泡蛋白沉积症. 中华结核和呼吸杂志, 1997, 20(ZK): 89-90.
4. Vast C, Demonet B, Mouveroux J. Value of selective pulmonary lavage under fibroscopic control in alveolar proteinosis. Poumon Coeur, 1978, 34(4): 305-307.
5. Rodrigo D, Rathnapala A, Senaratne W. Therapeutic limited bronchoalveolar lavage with fiberoptic bronchoscopy as a bridging procedure prior to total lung lavage in a patient with pulmonary alveolar proteinosis: a case report. J Med Case Rep, 2015, 9: 93.
6. Firat ND, Cıledağ A, Kabalak PA, et al. Pulmonary alveolar proteinosis and successful therapy with combined lavage procedures: Case reports. Exp Ther Med, 2011, 2(3): 569-573.
7. Cheng SL, Chang HT, Lau HP, et al. Pulmonary alveolar proteinosis: treatment by bronchofiberscopic lobar lavage. Chest, 2002, 122(4): 1480-1485.
8. Xu ZH, Jing JY, Wang HY, et al. Pulmonary alveolar proteinosis in China: a systematic review of 241 cases. Respirology, 2009, 14(5): 761-766.
9. Skoczynski S, Wyskida K, Rzepka-Wrona P, et al. Novel method of noninvasive ventilation supported therapeutic lavage in pulmonary alveolar proteinosis proves to relieve dyspnea, normalize pulmonary function test results and recover exercise capacity: a short communication. J Thorac Dis, 2018, 10(4): 2467-2473.
10. Boyce DSK, Lee JW, Shah P, et al. Combined-modality therapy for pulmonary alveolar proteinosis in a remote setting: a case report. BMC Pulm Med, 2019, 19(1): 61.
11. Tazawa R, Trapnell BC, Inoue Y, et al. Inhaled granulocyte/macrophage-colony stimulating factor as therapy for pulmonary alveolar proteinosis. Am J Respir Crit Care Med, 2010, 181(12): 1345-1354.

Chinese Journal of Respiratory and Critical Care Medicine

局部肺叶灌洗治疗合并呼吸衰竭的重症肺泡蛋白沉积症三例并文献复习

Abstract Full text Figures/Tables Video References Cited by

Previous Article

Next Article

Format

Content