1.Advances in the Diagnosis, Treatment and Prognosis of Lymphangioleiomyomatosis
Chongsheng CHENG ; Song LIU ; Yanli YANG ; Guozhu HOU ; Wuying CHENH ; Tengyue ZHANG ; Danjing HU ; Xinlun TIAN ; Kaifeng XU
JOURNAL OF RARE DISEASES 2022;1(1):38-44
Lymphangioleiomyomatosis (LAM) is a rare, multisystemic, low-grade neoplasm character-ized by diffuse cystic lesions in the lung.In recent years, emerging imaging examination such as 68Ga-NEB PET-CT scan provides efficient and precise non-invasive diagnostic methods to detect lymphatic circulation abnormalities in LAM patients. The long-term efficacy and safety of sirolimus for LAM has accumulated further evidence, and genetic profiling studies have unveiled more information of genetic mechanisms. Prognosis of LAM has been much improved. We briefly reviewed the research advances of LAM in China and other countires.
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