@article {Franciosi2101410作者={亚历桑德罗·n·Franciosi和丹尼尔Fraughen和汤姆{\ '}s p·卡罗尔和诺尔g . McElvaney} title = {alpha - 1抗胰蛋白酶缺乏症”:明确公认的保护的作用阈值},体积= {59}= {2},elocation-id = {2101410} = {2022}, doi ={10.1183/13993003.01410 -2021},出版商={欧洲呼吸学会},文摘= {alpha - 1抗胰蛋白酶缺乏症”(AATD)是唯一容易识别单基因引起的慢性阻塞性肺病。188bet官网地址迄今为止唯一condition-specific AATD-associated治疗慢性阻塞性肺病是每周静脉plasma-purified人类alpha -抗胰蛋白酶(IV-AAT)。不确定性AATD基因型应该受益于IV-AAT持续下去。IV-AAT是昂贵的,包括每周等离子产品的管理。大部分的危险分层一直围绕{\ textquotedblleft}的长期被认可的假说假定的保护阈值{\ textquotedblright} {\ textmu} 11米(0.57 g {\ textperiodcentered} l - 1) AAT在血清。这个假设已经成为AATD保健的范式的核心,尽管它的推导和准确性定义疾病的风险尚不清楚。我们回顾了文献和研究之间的关系11 {\ textmu}米阈值和临床结果提供上下文和洞察周围的问题这个话题。我们发现没有数据证明慢性阻塞性肺病的风险增加依赖于11日{\ textmu} M阈值。此外,大量的近期临床数据研究这个阈值否定假设。相反,使用11 {\ textmu} M作为治疗目标适当ZZ个人临床证据支持,尽管更精致的剂量方案正在探索。继续使用的11个{\ textmu} M阈值作为一个行列式的临床风险问题,使得AAT-augmentation方法不当,可能驱动增加医疗支出,不应作为指标开始治疗。基因型代表一个更证明的风险指标,与ZZ和罕见的独立ZZ-equivalent基因型与慢性阻塞性肺病。 New and better risk assessment models are needed to provide individuals diagnosed with AATD with reliable risk estimation and optimised treatment goals.The protective threshold of 11 {\textmu}M has historically been used to predict risk of COPD in AATD. The evidence now conclusively shows this to be inaccurate. 11 {\textmu}M is a target for augmentation therapy, but should not be used as a determinant of risk of COPD. https://bit.ly/2TXXNus}, issn = {0903-1936}, URL = {//www.qdcxjkg.com/content/59/2/2101410}, eprint = {//www.qdcxjkg.com/content/59/2/2101410.full.pdf}, journal = {European Respiratory Journal} }