Pediatric Respiratory Medicine, Dept of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
Eur Respir J. 2021 Jul 8;58(1). doi: 10.1183/13993003.02686-2020. Print 2021 Jul.
Although the lung clearance index (LCI) is a sensitive marker of small airway disease in individuals with cystic fibrosis (CF), less is known about longitudinal changes in LCI during routine clinical surveillance. Here, our objectives were to describe the longitudinal course of LCI in children with CF during routine clinical surveillance and assess influencing factors.
Children with CF aged 3-18 years performed LCI measurements every 3 months as part of routine clinical care between 2011 and 2018. We recorded clinical data at every visit. We used a multilevel mixed effect model to determine changes in LCI over time and identify clinical factors that influence LCI course.
We collected LCI measurements from 1204 visits (3603 trials) in 78 participants, of which 907 visits had acceptable LCI data. The average unadjusted increase in LCI for the entire population was 0.29 (95% CI 0.20-0.38) LCI units·year. The increase in LCI was more pronounced in adolescence (0.41 (95% CI 0.27-0.54) LCI units·year). Colonisation with either or , pulmonary exacerbations, CF-related diabetes and bronchopulmonary aspergillosis were associated with a higher increase in LCI over time. Adjusting for clinical risk factors reduced the increase in LCI over time to 0.24 (95% CI 0.16-0.33) LCI units·year.
LCI measured during routine clinical surveillance is associated with underlying disease progression in children with CF. An increased change in LCI over time should prompt further diagnostic intervention.
虽然肺清除指数（LCI）是囊性纤维化（CF）患者小气道疾病的敏感标志物，但在常规临床监测期间，LCI 的纵向变化知之甚少。在此，我们的目的是描述 CF 儿童在常规临床监测期间 LCI 的纵向变化，并评估影响因素。
2011 年至 2018 年间，年龄在 3-18 岁的 CF 儿童每 3 个月进行一次 LCI 测量，作为常规临床护理的一部分。我们在每次就诊时记录临床数据。我们使用多水平混合效应模型来确定 LCI 随时间的变化，并确定影响 LCI 过程的临床因素。
我们从 78 名参与者的 1204 次就诊（3603 次试验）中收集了 LCI 测量值，其中 907 次就诊有可接受的 LCI 数据。整个人群未经调整的 LCI 平均增加值为 0.29（95%CI 0.20-0.38）LCI 单位·年。青春期 LCI 的增加值更为明显（0.41（95%CI 0.27-0.54）LCI 单位·年）。或 定植、肺部恶化、CF 相关糖尿病和支气管曲霉病与 LCI 的增加呈正相关。调整临床危险因素后，LCI 的年增长率降至 0.24（95%CI 0.16-0.33）LCI 单位·年。
在常规临床监测期间测量的 LCI 与 CF 儿童的潜在疾病进展相关。LCI 随时间的变化增加应促使进一步进行诊断干预。
