肺淋巴管平滑肌瘤病1例并文献复习

目的探讨肺淋巴管平滑肌瘤病（PLAM）的临床特征、疗效和预后，提高对该病的认识。方法回顾性分析1例PLAM病人的临床资料，并复习文献。结果该病主要发生于育龄期妇女，临床症状无特异性，以咳嗽、胸闷、气短和呼吸困难为主；常发生乳糜胸和（或）气胸；肺功能检查有弥散功能下降，以阻塞性通气功能障碍为多，兼有限制性通气功能障碍；HRCT见双肺弥漫薄壁囊状阴影为影像学特征性表现；肺组织活检是确诊的主要依据。结论本病病因目前尚不清，孕激素疗效不理想，有待进一步探讨病因及寻找有效的疗法。

Clinical analysis of pulmonary lymphangioleiomyomatosis

Objective The clinical features,effects and the prognosis of the pulmonary lymphangioleiomyomatosis (PLAM) were discussed to strengthen the recognization of this disease.Methods The clinical materials of the 1 case of the PLAM and relevant materials were reviewed.Results PLAM primarily affects young women. The main clinical manifestations of PLAM were nonspecific,including the cough,suffocated chest,short-breathing and dysnpea,it often occurred with chylothorax and/or pneumothorax. Pulmonary function test s...