TY - JOUR T1 - Celecoxib in LAM:摩根富林明I期临床试验的结果——欧洲呼吸杂志》乔和J - 10.1183/13993003.02370 -2019欧元SP - 1902370 AU El-Chemaly Souheil AU - Taveira-DaSilva,安吉洛盟——Bagwe Shefali盟——Klonowska Katarzyna AU -马查多,塔尼亚AU - Lamattina,安东尼·m . AU -戈德堡,希拉里·J . AU -琼斯,阿曼达·m . AU - Julien-Williams,帕特丽夏盟,毛雷尔Rie AU -罗萨斯,伊万·o . AU - Henske伊丽莎白p . AU -莫斯,乔尔盟——Kwiatkowski摘要N2淋巴管平滑肌瘤病(angioleiomyomatosis, LAM)是一种与进行性肺部疾病相关的多系统疾病，几乎只影响女性。LAM的病理特征是异常的平滑肌样细胞的增殖，这些细胞主要在结节性硬化复合体(TSC)基因TSC2中携带突变，很少在TSC1[1]中携带突变。LAM可偶发于TSC或与TSC相关。TSC基因突变导致雷帕霉素复合物1 (mTORC1)[2]的哺乳动物/机制靶点激活。该手稿最近已被《欧洲呼吸杂志》接受发表。在我们的制作团队进行编辑和排版之前，它以公认的形式在这里出版。当这些生产过程完成并且作者已经批准了结果证明之后，文章将转移到最新一期的在线ERJ。请打开或下载PDF阅读本文。利益冲突:El-Chemaly博士没有什么要披露的。Conflict of interest: Angelo Taveira-DaSilva has no conflicts of interest.Conflict of interest: Dr. Bagwe has nothing to disclose.Conflict of interest: Dr. Klonowska has nothing to disclose.Conflict of interest: Tania MachadoConflict of interest: Dr. Lamattina reports personal fees from Wave Life Sciences, outside the submitted work.Conflict of interest: Dr. Goldberg has nothing to disclose.Conflict of interest: Amanda M. JonesConflict of interest: Patricia Julien-WilliamsConflict of interest: Rie MaurerConflict of interest: Dr. Rosas has nothing to disclose.Conflict of interest: Dr. Henske has nothing to disclose.Conflict of interest: Dr. Moss has nothing to disclose.Conflict of interest: Dr. Kwiatkowski has nothing to disclose. ER -