Cystic Fibrosis Center, Hospices Civils de Lyon, Lyon, France; UMR 5558 CNRS Equipe EMET Université Claude Bernard Lyon 1 Lyon, France.
Pediatric Cystic Fibrosis Center, Hôpital d'Enfants, Centre Hospitalier Universitaire de Nancy, Nancy, France.
J Cyst Fibros. 2022 Jan;21(1):155-159. doi: 10.1016/j.jcf.2021.06.002. Epub 2021 Jun 26.
Lung clearance index (LCI) is a biomarker of ventilation inhomogeneity. Data are scarce on its usefulness in daily practice for monitoring the effects of treatments in older children and adults with CF. In this French observational study of lumacaftor-ivacaftor, 63 of 845 patients (7.5%) had available LCI performed at baseline and at six (M6; n=34) or 12 months (M12; n=46) after lumacaftor-ivacaftor initiation. At inclusion, median [IQR] age was 16 years [13-17], ppFEV was 72.8 [59.6-80.7], and LCI was 12.3 [10.3-15.0]. At both M6 and M12, no statistically significant LCI increases of 0.13 units or 1.34% (95% CI: -4.85-7.53) and 0.6 units or 6.66% (95% CI: -0.03-13.5) were observed. Discordant results between LCI and ppFEV were observed in one-third of the patients. In daily practice, LCI monitoring in adolescents and young adults with moderate lung disease gives results that are more heterogenous than those reported in children with milder disease.
肺清除指数 (LCI) 是不均一性通气的生物标志物。关于其在监测 CF 中年龄较大的儿童和成人治疗效果方面的日常应用的有用性的数据很少。在这项关于 lumacaftor-ivacaftor 的法国观察性研究中，845 名患者中有 63 名（7.5%）在基线时和 lumacaftor-ivacaftor 开始后 6 个月（M6；n=34）或 12 个月（M12；n=46）时进行了可获得的 LCI 检测。纳入时，中位 [IQR] 年龄为 16 岁 [13-17]，ppFEV 为 72.8 [59.6-80.7]，LCI 为 12.3 [10.3-15.0]。在 M6 和 M12 时，LCI 分别增加了 0.13 个单位或 1.34%（95%CI：-4.85-7.53）和 0.6 个单位或 6.66%（95%CI：-0.03-13.5），但均无统计学显著意义。三分之一的患者的 LCI 和 ppFEV 结果不一致。在日常实践中，中重度肺疾病的青少年和年轻成人的 LCI 监测结果比轻度疾病儿童的报告结果更为多样化。
