Ty-jour t1 - 淋巴胆管瘤瘤中的甲素反应性与FEV _{1 和VEGF-D JF - 欧洲呼吸期刊Jo - Eur Respir J Do - 10.1183 / 13993003.04270-2020 VL - 57是 - 6 sp- 2004270 AU - Cassandro，Roberto Au - Elia，Davide Au - Caminati，Antonella Au - Pacheco-Rodriguez，Gustavo Au - Styrianou，Mario Au - Moss，Joel Au - Harari，Sergio Y1 - 2021/06/01 UR - HTTP：//www.qdcxjkg.com/content/57/6/2004270.abstract N2 - Lymphangioleiomyomatosis (LAM) is a multisystem disease characterised by cystic lung destruction, leading to respiratory failure, and associated with kidney (e.g. angiomyolipomas (AML)) and lymphatic involvement (e.g. lymphangioleiomyomas, chylous effusions) [1, 2]. LAM occurs sporadically or in association with tuberous sclerosis complex (TSC), an autosomal-dominant disorder characterised by mutations of the TSC1 or TSC2 genes. Lung destruction results from the proliferation of LAM cells, which possess neoplastic properties and are found in LAM lung nodules, in association with fibroblasts, mast cells, lymphocytes and lymphatic endothelial cells [3, 4]. LAM patients may show increases in serum levels of the lymphangiogenic factor, vascular endothelial growth factor-D (VEGF-D), a LAM biomarker used in differential diagnosis of cystic lung diseases and to identify LAM patients likely to respond to sirolimus treatment [5–7].Methacholine bronchoprovocation in LAM patients was used to assess airway reactivity. A negative methacholine challenge was associated with a lack of a response to β-adrenergic agonist, higher levels of FEV1, and higher levels of VEGF-D https://bit.ly/3aVTFl2 ER -}