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目的探讨肺泡蛋白沉积症(PAP)的影像特点及诊断。方法回顾性分析9例经病理证实的PAP患者胸部X线和CT表现。结果PAPX线表现为两肺对称或不对称分布磨玻璃样影和实变影,自肺门向外放射,形似“蝶翼征”。CT表现呈多样化改变:①两肺弥漫性斑片状模糊影和磨玻璃样改变,呈“地图样”分布(8例);②蝶翼征(7例);③支气管充气征(4例);④“碎石路”征(7例)。结论肺部影像表现可充分显示肺泡蛋白沉积症上述典型的X线、CT表现,结合临床综合分析可作出正确诊断,纤维支气管镜肺活检及肺泡灌洗术是本病确诊的主要依据。
Objective To investigate the imaging features and diagnosis of pulmonary alveolar proteinosis (PAP). Methods The chest X-ray and CT findings of 9 pathologically confirmed PAP patients were retrospectively analyzed. Results PAPX line showed two symmetrical or asymmetrical distribution of ground glass-like shadow and real shadow, radiating outward from the hilar and shaped like a “butterfly wing syndrome.” CT showed a variety of changes: ① diffuse blurring of both lungs and ground glass-like changes were “map-like” distribution (8 cases); ② butterfly wing sign (7 cases); ③ bronchial air sign 4 cases); ④ “gravel road” sign (7 cases). Conclusion The pulmonary manifestations of pulmonary alveolar proteinosis can fully display the typical X-ray and CT findings of the above-mentioned diseases. Correct diagnosis can be made in combination with clinical comprehensive analysis. Fiberoptic bronchoscopy lung biopsy and alveolar lavage are the main basis for the diagnosis of this disease.