| 摘要: |
| ［摘要］ 目的 提高肺淋巴管平滑肌瘤病（PLAM）的诊疗水平。方法 收集2007-07～2018-07广西壮族自治区人民医院收治的5例PLAM的临床影像学和病理学资料进行分析总结和文献复习。结果 5例患者均为女性，症状以活动性呼吸困难或咳嗽为主，胸部薄层高分辨率CT（HRCT）均显示PLAM特征性的双肺弥漫性薄壁小囊腔病变，最后均诊断为PLAM。结论 PLAM是一种主要见于女性的罕见病，典型的临床影像学为两肺弥漫性薄壁囊腔改变，肺病理活检有助于诊断，PLAM可用西罗莫司治疗。 |
| 关键词: 肺淋巴管平滑肌瘤病 高分辨率计算机体层X线摄影术 西罗莫司 |
| DOI：10.3969/j.issn.1674-3806.2019.08.18 |
| 分类号:R 734.2 |
| 基金项目: |
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| Clinical diagnosis and treatment of pulmonary lymphangioleiomyomatosis and review of literature |
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LUO Ling, Ning Yu, LIU Hang, et al.
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Department of Respiratory Medicine, the People′s Hospital of Guangxi Zhuang Autonomous Region, Nanning 530021, China
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| Abstract: |
| ［Abstract］ Objective To improve the diagnosis and treatment of pulmonary lymphangioleiomyomatosis(PLAM). Methods The clinical imaging and pathological data of 5 patients with PLAM treated in the People′s Hospital of Guangxi Zhuang Autonomous Region during July 2007 and July 2018 were respectively analyzed and the related literature was reviewed. Results All of the 5 patients were female with dyspnea on exertion or cough as the main symptoms, and the results of their chest high-resolution computed tomography（HRCT） scans all showed bilateral diffuse small thin-walled cystic lesions which were consistent with radiological features of PLAM, resulting in a diagnosis of PLAM. Conclusion PLAM is a rare disease occurring frequently in women. Chest HRCT shows characteristic diffuse small thin-walled cystic lesions in both lungs. Lung biopsy is helpful for diagnosis and PLAM can be treated with sirolimus. |
| Key words: Pulmonary lymphangioleiomyomatosis（PLAM） High-resolution computed tomography（HRCT） Sirolimus |
