%0刊期刊文章戴维斯,Gwyneth%的股票,珍妮特%A Thia,Lena P.%A Hoo,Ah-Fong%A Bush,Andrew%A Aurora,Paul%A Brennan,Lucy%A Lee,Simon%alum,sooky%a cottam,philippa%英里,joanne%a chudleigh,简%a kirkby,jane%a balfour-lynn,伊恩m.%a carr,siobhánb.%a wallis,colin%wyatt,hilary%涉及涉水,Angie%患有标准英国护理囊性纤维化的新生儿筛查婴儿的%T肺功能缺陷是轻度和瞬态%D 2017%R 10.1183 / 13993003.00326-2017%J欧洲呼吸期轴颈%p 1700326%v 50%n5%X随着新型设计师分子的出现,用于囊性纤维化(CF)治疗,巨大需要早期临床试验结果,如婴幼儿肺功能(ILF)。我们调查了在CF新生儿筛查婴儿的前2年寿命期间的ILF异常的程度和跟踪。在0.5秒(FEV0.5)中,肺部间隙指数(LCI)和血液测量功能残留能力在〜3中测量了〜3 months, 1 year and 2 years in 62 infants with CF and 34 controls.By 2 years there was no significant difference in FEV0.5 z-score between CF and controls, whereas mean LCI z-score was 0.81 (95% CI 0.45–1.17) higher in CF. However, there was no significant association between LCI z-score at 2 years with either 3-month or 1-year results. Despite minimal average group changes in any ILF outcome during the second year of life, marked within-subject changes occurred. No child had abnormal LCI or FEV0.5 on all test occasions, precluding the ability to identify “high-risk” infants in early life.In conclusion, changes in lung function are mild and transient during the first 2 years of life in newborn screened infants with CF when managed according to a standardised UK treatment protocol. Their potential role in tracking disease to later childhood will be ascertained by ongoing follow-up.Lung function changes in newborn screened infants with cystic fibrosis are mild and transient up to age 2 years http://ow.ly/7gkW30etHtb %U //www.qdcxjkg.com/content/erj/50/5/1700326.full.pdf