Kinsey Laura, Winterbottom Hannah, Hudson Lydia, Lal Simon, Jones Andrew M, Burden Sorrel
Manchester Adult Cystic Fibrosis Centre, Manchester University NHS Foundation Trust, Wythenshawe Hospital, Manchester, M23 9LT, United Kingdom.
Intestinal Failure Unit, Salford Royal Infirmary, Northern Care Alliance Foundation Trust, Manchester, United Kingdom.
Eur J Clin Nutr. 2025 Mar 22. doi: 10.1038/s41430-025-01589-y.
Elexacaftor/Tezacaftor/Ivacaftor (ETI) has led to improved lung function, life expectancy, and body mass index for people with Cystic Fibrosis (CF). The aim of this systematic review was to evaluate the impact that ETI has had on body composition in people with CF. A systematic review was performed using MEDLINE, EMBASE, CINAHL, and the Cochrane Central Register of Controlled Trials. Quality assessment using the Joanna Briggs Institute critical appraisal tools were performed. Results were summarised narratively. Five observational cohort studies involving a total of 185 participants were reviewed. Three studies showed an increase in fat mass (7.0-8.6 kg, 13.2-14.3 kg, and 13.4-15.5 kg). Two studies reported an increase in fat-free mass (49.4-50.1 kg, 52.5-55 kg), while one reported a decrease (50.5-48.9 kg). Two studies found an increase in fat mass index (4.1-6.3 kgm/ and 4.7-5.4 kg/m) and fat-free mass index (17.4-17.7 kg/m and 18.1-18.8 kg/m). Two studies observed an increase in percentage body fat mass (12.1-15.4% and 23.1-27.6%). Four studies were classified as low quality, while one was considered medium quality. This review suggest that commencing ETI results in changes in body composition. Firm conclusions about the type and distribution of change in body composition cannot be made due to limited studies, high heterogeneity, and methodical weaknesses. It highlights the necessity for higher quality and longer-term studies to explore the impact that ETI is having on body composition.
依列卡福妥/替扎卡福妥/依伐卡托（ETI）已使囊性纤维化（CF）患者的肺功能、预期寿命和体重指数得到改善。本系统评价的目的是评估ETI对CF患者身体成分的影响。使用MEDLINE、EMBASE、CINAHL和Cochrane对照试验中央注册库进行了系统评价。使用乔安娜·布里格斯研究所的批判性评价工具进行了质量评估。结果进行了叙述性总结。对五项观察性队列研究进行了综述，共涉及185名参与者。三项研究显示脂肪量增加（分别为7.0 - 8.6千克、13.2 - 14.3千克和13.4 - 15.5千克）。两项研究报告去脂体重增加（分别为49.4 - 50.1千克、52.5 - 55千克），而一项研究报告减少（从50.5千克降至48.9千克）。两项研究发现脂肪量指数增加（分别为4.1 - 6.3千克/平方米和4.7 - 5.4千克/平方米）以及去脂体重指数增加（分别为17.4 - 17.7千克/平方米和18.1 - 18.8千克/平方米）。两项研究观察到体脂百分比增加（分别为12.1 - 15.4%和23.1 - 27.6%）。四项研究被归类为低质量，而一项被认为是中等质量。本综述表明开始使用ETI会导致身体成分发生变化。由于研究有限、高度异质性和方法学缺陷，无法就身体成分变化的类型和分布得出确凿结论。它强调了开展更高质量和长期研究以探索ETI对身体成分影响的必要性。
