Effect of CFTR Modulators on Anthropometric Parameters in Individuals with Cystic Fibrosis: An Evidence Analysis Center Systematic Review.

Journal of the Academy of Nutrition and Dietetics
Julianna BaileyJessica A Alvarez

Abstract

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 iv...Continue Reading

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Jun 3, 2021·Journal of Personalized Medicine·Renske van der MeerHarry G M Heijerman
Aug 28, 2021·Journal of Personalized Medicine·Bente L AalbersHarry G M Heijerman
Oct 28, 2021·Pediatric Pulmonology·Marc A SalaMichelle Prickett
Nov 6, 2021·Pediatric Pulmonology·Megan E GabelSuzanne H Michel
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Feb 5, 2022·Nutrition in Clinical Practice : Official Publication of the American Society for Parenteral and Enteral Nutrition·Jessica M Shape, Marc A Sala

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