基础医学与临床2024，Vol.44Issue(7)：1044-1048,5.DOI:10.16352/j.issn.1001-6325.2024.07.1044
淋巴管肌瘤病进展及死亡风险相关因素
Factors associated with lymphangioleiomyomatosis progression and mortality
摘要
Abstract
Lymphangioleiomyomatosis(LAM)is a rare progressive lung disease characterized by diffuse cystic le-sions that primarily affects women of reproductive ages and leads to respiratory failure at the end stage of the dis-ease,and significantly impacts patients'quality of life.The clinical use of mammalian target of rapamycin(mTOR)inhibitors(e.g.sirolimus)has moderated the rate of disease progression and significantly improved the survival in LAM patients.During the clinical diagnosis,treatment,and follow-up of LAM disease,the clinical characteristics and the course of the disease of different LAM patients are heterogeneous which might suggest differences in disease progression and long-term prognosis.The present review summarizes the progress of research on risk factors for LAM progression and mortality in order to optimize individualized intervention protocols in clinical practice and to improve long-term prognosis of patients.关键词
淋巴管肌瘤病/肺功能/血管内皮增长因子D(VEGF-D)/西罗莫司Key words
lymphangioleiomyomatosis/pulmonary function/vascular endothelial growth factor D(VEGF-D)/sirolimus分类
临床医学引用本文复制引用
王路易,徐凯峰..淋巴管肌瘤病进展及死亡风险相关因素[J].基础医学与临床,2024,44(7):1044-1048,5.基金项目
中央高水平医院临床科研专项(2022-PUMCH-B-107) （2022-PUMCH-B-107）
