ty -jour t1-两个突变的 mbl -2 等位基因JF的囊性纤维化成人的肺状况受损 - 欧洲呼吸杂志-24 IS -5 au -davies，J.C。au -Turner，M.W。Au -Klein，N.Au-，Y1-2004/11/01 UR -http：//www.qdcxjkg.com/content.com/content/24/5/5/798/798.Abtract N2-甘露糖结合凝集素最近被确定为囊性纤维化严重程度的修饰符，尽管研究产生了不同的结果，并且作用机理尚不清楚。目前的作者研究了大量的成年人（n = 298）和儿童（n = 260），以进一步探索这种明显的关系。具有两个结构性突变但没有杂合子的成年人的肺功能和氧饱和度显着降低，医院入院率更高，并且系统性炎症标记升高。这与铜绿假单胞菌的感染率增加无关，并且对Burkholderia cepacia的敏感性没有增加。这些发现都没有在小儿队列中反映出来。总之，尽管杂合子不受影响，但严重的甘露糖结合凝集素缺乏似乎对囊性纤维化成年有害。建议这与补体介导的细菌杀伤受损无关，并且假设与宿主炎症反应的联系。如果开发甘露糖结合凝集素作为该疾病的新方法，那么本研究将表明，有两个结构性突变的严重缺陷患者可能会受益。 This work was supported by the Cystic Fibrosis Research Trust, by a Wellcome Trust Senior Clinical Fellowship (E.W.F.W. Alton), and a Wellcome Trust Clinical Fellowship (K. Fidler). Research at the Institute of Child Health and Great Ormond Street for Children National Health Service (NHS) Trust benefits from research and development funding received from the UK NHS executive. Consumables for this study were funded in part by NatImmune A/S, Copenhagen, Denmark. M.W. Turner and N. Klein are consultants for this company, which is developing a recombinant form of mannose-binding lectin. ER -