| 肺淋巴管平滑肌瘤病 |
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| 引用本文： | 金美玲,蔡映云,张志凤. 肺淋巴管平滑肌瘤病[J]. 中华结核和呼吸杂志, 2000, 23(6): 355-357 |
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| 作者姓名： | 金美玲 蔡映云 张志凤 |
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| 作者单位： | 上海医科大学附属中山医院肺科(金美玲!200032，蔡映云!200032，张志凤!200032)，上海医科大学附属中山医院(潘文森!200032) |
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| 摘 要： | 目的 提高对罕见病淋巴管平滑肌瘤病（ＰＬＡＭ）的认为。方法 对１９９２年对来诊治的３例ＰＬＡＭ患者的临床资料进行分析，并结合文献复习。结果 ＰＬＡＭ是一种罕见的弥漫肺部疾病，临床表现为反复发作自发性气胸、活动后呼吸困难和痰血等。肺功能呈阻塞性或混合性通气功能障，动脉血气示低氧血症。胸部Ｘ线表现为两肺弥漫分布网格状影，高分辨ＣＴ（ＨＲＣＴ）示两肺弥漫分布囊状改变。病理学检查示肺组织淋巴管增生和扩张，
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| 关 键 词： | 肺淋巴管平滑肌瘤病 肺疾病 诊断 治疗 预后 |
| 修稿时间： | 1999-10-11 |
Pulmonary lymphangioleiomyomatosis |
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| JIN Meiling,CAI Yingyun,ZHANG Zhifeng,Pulmonary Department,Zhongshan Hospital,Shanghai Medical University,Shanghai ,China. Pulmonary lymphangioleiomyomatosis[J]. Chinese journal of tuberculosis and respiratory diseases, 2000, 23(6): 355-357 |
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| Authors: | JIN Meiling CAI Yingyun ZHANG Zhifeng Pulmonary Department Zhongshan Hospital Shanghai Medical University Shanghai China |
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| Affiliation: | Pulmonary Department, Zhongshan Hospital, Shanghai Medical University, Shanghai 200032, China. |
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| Abstract: | Objective To improve the diagnosis and treatment of pulmonary lymphangioleiomyomatosis(PLAM). Methods Three patients with PLAM confirmed by pathological assessment were presented and relevant literatures were reviewed. Results PLAM is a rare pulmonary disease of unknown cause. The clinical manifestations were pneumothorax, exertional dyspnea and hemoptysis. Pulmonary function test showed obstructive or compound ventilative defect and hypoxemia. HRCT showed bilateral diffuse cystic airspaces change. Pathological features showed abnormal smooth muscle proliferation occurred along lymphatics. Lymphatics dilated and proliferated. Conclusions The prognosis of PLAM is poor. There is no effective method for the treatment of this disease at present. |
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| Keywords: | Pulmonary lymphangioleiomyomatosis Proliferation of smooth muscle |
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