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目的提高对肺泡蛋白沉积症(PAP)的认识。方法回顾性分析20例确诊为肺泡蛋白沉积症患者的病例资料。结果肺泡蛋白沉积症以呼吸困难、咳嗽、咳痰为主要表现,影像学多表现为双肺弥漫性磨玻璃影及斑片影,部分呈“地图征”及“铺路石征”改变。20例患者均行支气管肺泡灌洗液(BALF)病理分析,过碘酸雪夫(PAS)染色阳性;10例经支气管镜肺活检(TBLB)提示肺泡腔见蛋白物沉积。结论肺泡蛋白沉积症临床症状及实验室检查无特异性,对于以咳嗽伴进行性呼吸困难为主要临床表现,CT双肺弥漫性病变,呈“地图征”及“铺路石征”改变的病例应警惕本病,尽早行BALF及TBLB,必要时行胸腔镜肺活检确诊,避免误诊。
Objective To improve the understanding of pulmonary alveolar proteinosis (PAP). Methods Retrospective analysis of 20 cases of patients diagnosed as pulmonary alveolar proteinosis case data. Results Pulmonary alveolar proteinosis mainly manifested as dyspnea, cough and expectoration. The imaging showed diffuse glassy mildew and patchy shadow of the lungs, some of which were “map sign” and “paving stone sign” change. Twenty patients underwent pathological analysis of bronchoalveolar lavage fluid (BALF), positive by periodic acid-lipoic acid (PAS) staining and 10 by bronchoscopic lung biopsy (TBLB). Conclusions The clinical symptoms and laboratory tests of pulmonary alveolar proteinosis are nonspecific. For patients with cough with progressive dyspnea as the main clinical manifestations, CT pulmonary diffuse lesions showed “map sign” and “paving stone sign” Change the case should be wary of this disease, as early as possible BALF and TBLB, if necessary, line thoracoscopic lung biopsy confirmed to avoid misdiagnosis.