Peripheral Muscle Function and Body Composition in People With Cystic Fibrosis on Elexacaftor/Tezacaftor/Ivacaftor: A Cross-Sectional Single-Centre Study.

IF 2.7 3区 医学 Q1 PEDIATRICS
Lauren J Clayton, Anthony I Shepherd, Jo Corbett, Mathieu Gruet, Gary Connett, Mark Allenby, Julian Legg, Thomas Daniels, Don S Urquhart, Zoe L Saynor
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引用次数: 0

Abstract

Background: People with cystic fibrosis (pwCF) often have multifactorial peripheral muscle abnormalities attributed to, for example, malnutrition, steroid use, altered redox balance and, potentially, CF-specific intrinsic alterations. Malnutrition in CF now includes an increasing prevalence of overweight and obesity, particularly in those receiving CF transmembrane conductance regulator (CFTR) modulator therapy (CFTRm). We aimed to characterise peripheral muscle function and body composition in pwCF on Elexacaftor/Tezacaftor/Ivacaftor (ETI) CFTRm, compared to healthy controls.

Methods: Fifteen pwCF on ETI, and 15 healthy age- and sex-matched controls (CON), underwent whole-body dual-energy X-ray absorptiometry scans, and a comprehensive evaluation of peripheral muscle function. Tests included quadriceps maximal isometric force measurement, an intermittent isometric quadriceps fatiguing protocol, handgrip strength dynamometry, squat jump height assessment, and 1-min sit-to-stand testing.

Results: No significant differences in quadriceps maximal isometric force (CON: 181.60 ± 92.90 Nm vs. CF: 146.15 ± 52.48 Nm, p = 0.21, d = 0.47), handgrip strength (CON: 34 ± 15 kg vs. CF: 31 ± 11 kg, p = 0.62, d = 0.18), peripheral muscle endurance, fatigue, or power were observed between the groups. Moreover, no significant differences in whole-body, trunk or limb lean mass, fat-free mass, fat mass, or whole-body bone mineral density were evident.

Conclusion: Comparable peripheral muscle mass and function has been demonstrated in pwCF on ETI, albeit a group with good lung function. Research is needed to confirm these findings longitudinally in pwCF, including those with more severe lung disease, who are less physically active, and have less optimal nutrition and exercise support.

背景:囊性纤维化患者(pwCF)通常会出现多因素导致的外周肌肉异常,例如营养不良、使用类固醇、氧化还原平衡改变以及潜在的 CF 特异性内在改变。目前,CF患者营养不良包括超重和肥胖,尤其是在接受CF跨膜传导调节器(CFTR)调节剂治疗(CFTRm)的患者中,超重和肥胖的发病率越来越高。我们的目的是与健康对照组相比,了解接受 Elexacaftor/Tezacaftor/Ivacaftor (ETI) CFTRm 治疗的贫困儿童的外周肌肉功能和身体成分:15名服用 ETI 的 pwCF 和 15 名年龄和性别匹配的健康对照组 (CON) 接受了全身双能量 X 射线吸收扫描,并对外周肌肉功能进行了全面评估。测试包括股四头肌最大等长力测量、间歇等长股四头肌疲劳方案、手握力测功法、蹲跳高度评估和 1 分钟坐立测试:结果:两组在股四头肌最大等长力(CON:181.60 ± 92.90 牛米 vs. CF:146.15 ± 52.48 牛米,P = 0.21,D = 0.47)、手握力(CON:34 ± 15 千克 vs. CF:31 ± 11 千克,P = 0.62,D = 0.18)、外周肌肉耐力、疲劳或力量方面均无明显差异。此外,全身、躯干或四肢的瘦体重、去脂体重、脂肪量或全身骨矿物质密度均无明显差异:结论:尽管肺功能良好,但在接受 ETI 治疗的慢性阻塞性肺疾病患者中,外周肌肉质量和功能具有可比性。还需要对患有更严重肺病、运动量较少、营养和运动支持较差的慢性阻塞性肺病患者进行纵向研究,以证实这些发现。
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来源期刊
Pediatric Pulmonology
Pediatric Pulmonology 医学-呼吸系统
CiteScore
6.00
自引率
12.90%
发文量
468
审稿时长
3-8 weeks
期刊介绍: Pediatric Pulmonology (PPUL) is the foremost global journal studying the respiratory system in disease and in health as it develops from intrauterine life though adolescence to adulthood. Combining explicit and informative analysis of clinical as well as basic scientific research, PPUL provides a look at the many facets of respiratory system disorders in infants and children, ranging from pathological anatomy, developmental issues, and pathophysiology to infectious disease, asthma, cystic fibrosis, and airborne toxins. Focused attention is given to the reporting of diagnostic and therapeutic methods for neonates, preschool children, and adolescents, the enduring effects of childhood respiratory diseases, and newly described infectious diseases. PPUL concentrates on subject matters of crucial interest to specialists preparing for the Pediatric Subspecialty Examinations in the United States and other countries. With its attentive coverage and extensive clinical data, this journal is a principle source for pediatricians in practice and in training and a must have for all pediatric pulmonologists.
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