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中华临床医师杂志(电子版) ›› 2021, Vol. 15 ›› Issue (07) : 556 -560. doi: 10.3877/cma.j.issn.1674-0785.2021.07.015

综述

肺淋巴管肌瘤病治疗的研究进展
陈雪芬1, 邓静敏1,()   
  1. 1. 530021 南宁,广西医科大学第一附属医院呼吸与危重症医学科
  • 收稿日期:2021-03-02 出版日期:2021-07-15
  • 通信作者: 邓静敏

Progress in treatment of pulmonary lymphangiomyomatosis

Xuefen Chen1, Jingmin Deng1,()   

  1. 1. Department of Respiratory Medicine, the First Affiliated Hospital of Guangxi Medical University, Nanning 530021, China
  • Received:2021-03-02 Published:2021-07-15
  • Corresponding author: Jingmin Deng
引用本文:

陈雪芬, 邓静敏. 肺淋巴管肌瘤病治疗的研究进展[J/OL]. 中华临床医师杂志(电子版), 2021, 15(07): 556-560.

Xuefen Chen, Jingmin Deng. Progress in treatment of pulmonary lymphangiomyomatosis[J/OL]. Chinese Journal of Clinicians(Electronic Edition), 2021, 15(07): 556-560.

肺淋巴管肌瘤病(PLAM)是一种罕见的肺部疾病,好发于育龄期女性,主要临床表现为反复气胸、乳糜胸、呼吸困难等,典型影像学表现为两肺弥漫性囊性病变,治疗较困难,患者预后差。PLAM的治疗包括药物治疗、气胸及乳糜胸的处理、肺移植等,其中西罗莫司是主要的治疗药物。本文就PLAM的治疗研究进展进行综述,以期为临床治疗及后续研究提供参考。

Pulmonary lymphangiomyomatosis is a rare lung disease that occurs in women of reproductive age. Main clinical manifestations are repeated pneumothorax, chylothorax, dyspnea and so on. Typical imaging manifestation is diffuse cystic lesions of both lungs. Clinical treatment is difficult, and prognosis is poor. Treatments for PLAM includes drugs treatment, managment of pneumothorax and chylothorax, and lung transplantation. Among them, Sirolimus is the main therapeutic drug. This article reviews the progress in treatment of PLAM to provide references for clinical treatment and future research of this disease.

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