同期双侧序贯全肺灌洗术治疗肺泡蛋白沉积症3例并文献复习

目的探讨肺泡蛋白沉积症的诊断及同期序贯全肺灌洗的疗效。方法总结本院3例肺泡蛋白沉积症的临床资料及诊治经过,并复习相关文献。结果肺泡蛋白沉积症临床表现以胸闷、呼吸困难为主,CT表现为双肺弥漫性渗出或磨玻璃影,伴小叶间隔增厚,肺功能表现为弥散功能下降,肺泡灌洗液和肺组织活检PAS染色阳性可确诊,全肺灌洗为主要治疗方法。结论肺泡蛋白沉积症临床表现不典型,确诊需行支气管肺泡灌洗和肺活检,同期双肺序贯灌洗值得推广。

Report of 3 cases of pulmonary alveolar proteinosis treated by bilateral sequential whole lung lavage

Objective To investigate the diagnosis of pulmonary alveolar proteinosis （ PAP） and the thera-peutic effect of bilateral sequential whole lung lavage in the same treatment session. Methods The clinical data and management of 3 cases were summarized, followed by pertinent literatures review. Results The main symptom of PAP was exertional dyspnea. Computed tomography （ CT） showed diffused infiltrates and ground-glass opacities with interlobular septal thickening in both lung fields. Pulmonary function test demonstrated decreased diffusion capacity for carbon monoxide. The diagnosis was relied on PAS staining of bronchoalveolar lavage fluid and lung tissue biopsy. Whole lung lavage under general anesthesia was the most effective therapy. Conclusion The clinical manifestation of PAP is not typical, thus it is needed to take bronchoalveolar lavage and lung biopsy. Bilateral sequential whole lung lavage in the same treatment session is worth of widely promotion.