Elevated lung clearance index in infants with cystic fibrosis shortly after birth
Elevated lung clearance index in infants with cystic fibrosis shortly after birth
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DOI：
10.1183/13993003.00580-2017
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发表时间：
2017-11-01
影响因子：
24.3
通讯作者：
Latzin, Philipp
中科院分区：
文献类型：
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作者：
Kieninger, Elisabeth;Yammine, Sophie;Latzin, Philipp
目前尚不清楚囊性纤维化（CF）患儿在什么年龄可能出现肺功能损害。我们评估了新生儿筛查诊断为CF的婴儿出生后不久的肺功能。我们在CF患儿和健康对照的前瞻性队列中进行了婴儿肺功能测量。我们评估了肺清除率指数（LCI）、功能剩余容量（FRC）和潮汐呼吸参数。主要结局是CF的患病率和肺功能异常的严重程度（+/- 1.64 z评分）。我们招募了53名CF婴儿（平均年龄7.8周）和57名对照组（平均年龄5.2周）。与对照组相比，CF患儿LCI和FRC升高（平均差异为0.30,95% CI 0.02-0.60, p= 0.034和14.5 mL, 95% CI 7.7-21.3 mL, p< 0.001），而呼气潮流量峰值时间与呼气时间之比降低。在22例（41.5%）CF患儿中，LCI或FRC均超过1.64 z-score；3例患儿LCI和FRC均升高。CF婴儿出生后不久，肺功能异常很普遍。通气不均匀性或恶性充气可作为监测CF肺部疾病和特异性治疗效果的无创标志物，因此可作为该年龄组未来干预研究的结局参数。
It is not known at what age lung function impairment may arise in children with cystic fibrosis (CF). We assessed lung function shortly after birth in infants with CF diagnosed by newborn screening.We performed infant lung function measurements in a prospective cohort of infants with CF and healthy controls. We assessed lung clearance index (LCI), functional residual capacity (FRC) and tidal breathing parameters. The primary outcome was prevalence and severity of abnormal lung function (+/- 1.64 z-scores) in CF.We enrolled 53 infants with CF (mean age 7.8 weeks) and 57 controls (mean age 5.2 weeks). Compared to controls, LCI and FRC were elevated (mean difference 0.30, 95% CI 0.02-0.60; p= 0.034 and 14.5 mL, 95% CI 7.7-21.3 mL; p< 0.001, respectively), while ratio of time to peak tidal expiratory flow to expiratory time was decreased in infants with CF. In 22 (41.5%) infants with CF, either LCI or FRC exceeded 1.64 z-scores; three infants had both elevated LCI and FRC.Shortly after birth, abnormal lung function is prevalent in CF infants. Ventilation inhomogeneity or hyperinflation may serve as noninvasive markers to monitor CF lung disease and specific treatment effects, and could thus be used as outcome parameters for future intervention studies in this age group.