There is a strong positive association between nutrition status and lung function in cystic fibrosis (CF). Improvements in clinical care have increased longevity for individuals with CF, and it is unknown how cystic fibrosis transmembrane regulator (CFTR) modulation therapy affects nutrition status over time. The objective of this systematic review of the literature was to examine anthropometric (height, weight, and body mass index [BMI; calculated as kg/m²]) and body composition outcomes of CFTR modulation therapy. A literature search of Medline (Ovid), Embase, and CINAHL (EBSCO) databases was conducted for randomized controlled trials examining the effect of CFTR modulation therapy on anthropometric and body composition parameters, published in peer-reviewed journals from January 2002 until May 2018. Articles were screened, data were synthesized qualitatively, and evidence quality was graded by a team of content experts and systematic review methodologists. Significant weight gain with ivacaftor was noted in children and adults with at least 1 copy of G551D mutation. In adults with at least 1 copy of R117H the effect of ivacaftor on BMI was not significant. Effects on BMI were mixed in adults with class II mutations taking ivacaftor with lumacaftor. There was no significant change in BMI in children homozygous for F508del who took ivacaftor with tezacaftor. Elexacaftor-tezacaftor-ivacaftor increased BMI and body weight in individuals 12 years of age and older who were hetero- or homozygous for the F508del mutation. The effect of CFTR modulation therapy on anthropometric parameters depends on the genetic mutation and the type of modulation therapy used. More research is needed to understand the long-term clinical impact of these drugs on nutritional status, including body composition and the role of dietary intake.

囊性纤维化 (CF) 的营养状况与肺功能之间存在很强的正相关关系。临床护理的改进延长了 CF 患者的寿命，但尚不清楚囊性纤维化跨膜调节剂 (CFTR) 调节疗法如何随着时间的推移影响营养状况。这篇系统性文献综述的目的是检查人体测量学（身高、体重和体重指数 [BMI；计算为 kg/m ²]) 和 CFTR 调节疗法的身体成分结果。对 Medline (Ovid)、Embase 和 CINAHL (EBSCO) 数据库进行了文献检索，以研究 CFTR 调制疗法对人体测量学和身体成分参数的影响的随机对照试验，这些试验发表在 2002 年 1 月至 2018 年 5 月的同行评审期刊上。对文章进行筛选，对数据进行定性合成，并由内容专家和系统评价方法学家组成的团队对证据质量进行分级。在具有至少 1 个 G551D 突变拷贝的儿童和成人中注意到依伐卡托显着体重增加。在具有至少 1 个 R117H 拷贝的成人中，ivacaftor 对 BMI 的影响不显着。服用依伐卡托和 lumacaftor 的 II 类突变成人对 BMI 的影响是混合的。F508del 纯合子儿童服用依伐卡托和 tezacaftor 的 BMI 没有显着变化。Elexacaftor-tezacaftor-ivacaftor 增加了 12 岁及以上 F508del 突变杂合子或纯合子个体的 BMI 和体重。CFTR 调制疗法对人体测量参数的影响取决于基因突变和所使用的调制疗法类型。需要更多的研究来了解这些药物对营养状况的长期临床影响，包括身体成分和饮食摄入的作用。CFTR 调制疗法对人体测量参数的影响取决于基因突变和所使用的调制疗法类型。需要更多的研究来了解这些药物对营养状况的长期临床影响，包括身体成分和饮食摄入的作用。CFTR 调制疗法对人体测量参数的影响取决于基因突变和所使用的调制疗法类型。需要更多的研究来了解这些药物对营养状况的长期临床影响，包括身体成分和饮食摄入的作用。