在接受或未接受依列卡福妥/替扎卡福妥/依伐卡福妥治疗的成年囊性纤维化患者中习惯性身体活动的纵向变化
Longitudinal changes in habitual physical activity in adult people with cystic fibrosis in the presence or absence of treatment with elexacaftor/tezacaftor/ivacaftor.
作者信息
Gruber Wolfgang, Stehling Florian, Blosch Christopher, Dillenhoefer Stefanie, Olivier Margarete, Brinkmann Folke, Koerner-Rettberg Cordula, Sutharsan Sivagurunathan, Mellies Uwe, Taube Christian, Welsner Matthias
机构信息
Paediatric Pulmonology and Sleep Medicine, Cystic Fibrosis Center, Children's Hospital, University of Duisburg-Essen, Essen, Germany.
Institute of Human Nutrition and Food Science, Christian-Albrechts-Universität zu Kiel, Kiel, Germany.
出版信息
Front Sports Act Living. 2024 Feb 23;6:1284878. doi: 10.3389/fspor.2024.1284878. eCollection 2024.
BACKGROUND
Habitual physical activity (PA) and exercise training are accepted as important aspects of care for people with cystic fibrosis (pwCF) to improve health-related measures of physical fitness, which in turn have a positive impact on quality of life and prognosis. In the last decade, effective CFTR modulator therapies have become a promising treatment for pwCF by targeting the underlying cause of CF. This highly effective therapy improves clinical outcomes and quality of life in people with specific CFTR mutations. Little is known about the longitudinal pattern of PA or the impact of the highly effective modulator therapy with Elexacaftor/Tezacaftor/Ivacaftor (ETI) on PA in adult pwCF. This study assessed the course of device-based PA measurement in adult pwCF and evaluated the effects of ETI on habitual physical activity in those who were eligible for ETI.
METHODS
Data from adult pwCF (aged ≥18 years) were analysed at baseline and follow-up, using identical assessments at both time points. Outcome parameters were PA in steps/day and the intensity of PA. The group that received ETI was treated for an average of 33 weeks and not for the entire duration of the period. The data were collected between 2021 and 2022, following the removal of absolute pandemic restrictions/lockdowns.
RESULTS
Follow-up duration was 5.6 years in pwCF with ETI (ETI group, = 21) and 6.5 years in pwCF without ETI (non-ETI group, = 6). From baseline to follow-up, pwCF treated with ETI had a significant increase in steps/day (+25%, = 0.019) and a non-significant increase in moderate-to-vigorous intensity time (+5.6%, = 0.352). Conversely, individuals in the non-ETI group showed a non-significant decrease in both steps/day -3.2%, = 0.893) and moderate-to-vigorous intensity time (-25%, = 0.207). The ETI group showed a significant decrease in percent predicted forced expiratory volume in 1 s (ppFEV) and FEV z-score before the start of ETI treatment, both of which improved significantly after therapy initiation. Body weight and body mass index also improved significantly with ETI use.
CONCLUSIONS
These data suggest that ETI treatment has a positive effect on habitual physical activity behavior in the adult pwCF studied.
背景
习惯性体力活动（PA）和运动训练被认为是囊性纤维化患者（pwCF）护理的重要方面，有助于改善与健康相关的体能指标，进而对生活质量和预后产生积极影响。在过去十年中，有效的CFTR调节剂疗法通过针对CF的潜在病因，已成为pwCF的一种有前景的治疗方法。这种高效疗法改善了特定CFTR突变患者的临床结局和生活质量。关于成人pwCF的PA纵向模式或艾列卡福/替扎卡福/依伐卡福（ETI）这种高效调节剂疗法对PA的影响知之甚少。本研究评估了成人pwCF基于设备的PA测量过程，并评估了ETI对符合ETI治疗条件者的习惯性体力活动的影响。
方法
对成年pwCF（年龄≥18岁）在基线和随访时的数据进行分析，两个时间点采用相同的评估方法。结局参数为每日步数表示的PA和PA强度。接受ETI治疗的组平均治疗33周，而非整个研究期间。数据收集于2021年至2022年期间，当时已解除绝对的疫情限制/封锁措施。
结果
接受ETI治疗的pwCF随访时间为5.6年（ETI组，n = 21），未接受ETI治疗的pwCF随访时间为6.5年（非ETI组，n = 6）。从基线到随访，接受ETI治疗的pwCF每日步数显著增加（+25%，P = 0.019），中度至剧烈强度时间有不显著增加（+5.6%，P = 0.352）。相反，非ETI组个体的每日步数（-3.2%，P = 0.893）和中度至剧烈强度时间（-25%，P = 0.207）均有不显著下降。ETI组在ETI治疗开始前预测的1秒用力呼气容积百分比（ppFEV）和FEV z评分显著下降，治疗开始后均显著改善。使用ETI后体重和体重指数也显著改善。
结论
这些数据表明，ETI治疗对所研究的成年pwCF的习惯性体力活动行为有积极影响。
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