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

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 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.
AuthorsJulianna Bailey, Mary Rozga, Catherine M McDonald, Ellen K Bowser, Kristen Farnham, Mark Mangus, Laura Padula, Kathleen Porco, Jessica A Alvarez
JournalJournal of the Academy of Nutrition and Dietetics (J Acad Nutr Diet) Vol. 121 Issue 7 Pg. 1364-1378.e2 (07 2021) ISSN: 2212-2672 [Print] United States
PMID32532673 (Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't, Systematic Review)
CopyrightCopyright © 2021 Academy of Nutrition and Dietetics. Published by Elsevier Inc. All rights reserved.
Chemical References
  • Membrane Transport Modulators
  • Cystic Fibrosis Transmembrane Conductance Regulator
Topics
  • Adolescent
  • Adult
  • Anthropometry
  • Body Mass Index
  • Body Weight (drug effects)
  • Child
  • Cystic Fibrosis (drug therapy, genetics, physiopathology)
  • Cystic Fibrosis Transmembrane Conductance Regulator (drug effects, genetics)
  • Female
  • Humans
  • Male
  • Membrane Transport Modulators (adverse effects)
  • Mutation
  • Randomized Controlled Trials as Topic
  • Treatment Outcome
  • Young Adult

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