@Article {Ackrivo1802237,作者= {Ackrivo,Jason和Hansen-Flaschen,John和Wileyto,E. Paul和Schwab,Richard〜J。和Elman,Lauren和Kawut,Steven M.},标题= {肌营养的侧面硬化硬化术后呼吸功能不全或死亡的预后模型},体积= {53},Number = {4},Elocation-ID = {1802237},年份= {2019},DOI = {10.1183 / 13993003.02237-2018},出版商= {欧洲呼吸社会},摘要=188bet官网地址 {临床上有用的模型预测肌营养的外侧硬化症(ALS)的呼吸不足发作会通知疾病干预,通信和临床试验设计。我们旨在导出并验证呼吸功能不全的临床预后模型,以便在介绍的呼吸内容术中呈现给门诊ALS诊所。我们使用多变量的逻辑回归和内部交叉验证来使用单中心队列的765艾尔患者培养临床预后模型谁在2006年至2015年期间呈现。使用Multicentre汇总资源开放访问ALS临床试验(PRO-ACT)数据库进行外部验证,其中包含7083年患者。预测器包括首次门诊访问的基线特征。主要结果是6个月内的呼吸功能不全,通过开始非侵入性通气,强迫致命能力(FVC)\ 5%的预测,气管造口术或死亡。765名患者在我们的中心,300名(39%)有呼吸系统6个月内不足或死亡。六种基线特征(诊断年龄,症状发作和诊断的延迟,FVC,症状发病现场,肌萎缩侧面硬化功能评级规模修订(ALSFRS-R)总得分和ALSFRS-R呼吸困难评分)用于预测风险 primary outcome. The derivation cohort c-statistic was 0.86 (95\% CI 0.84{\textendash}0.89) and internal cross-validation produced a c-statistic of 0.86 (95\% CI 0.85{\textendash}0.87). External validation of the model using the PRO-ACT cohort produced a c-statistic of 0.74 (95\% CI 0.72{\textendash}0.75).We derived and externally validated a clinical prognostic rule for respiratory insufficiency in ALS. Future studies should investigate interventions on equivalent high-risk patients.ALS has a heterogenous progression to respiratory failure. A clinician can use the clinical prognostic rule to estimate a 6-month risk of respiratory failure onset, thus facilitating referrals and respiratory interventions http://ow.ly/LLzP30nqm2D}, issn = {0903-1936}, URL = {//www.qdcxjkg.com/content/53/4/1802237}, eprint = {//www.qdcxjkg.com/content/53/4/1802237.full.pdf}, journal = {European Respiratory Journal} }