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.
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Biography
J. Bailey is a clinical dietitian, University of Alabama at Birmingham.
Biography
M. Rozga is a nutrition researcher, Academy of Nutrition and Dietetics, Evidence Analysis Center, Chicago, IL.
Biography
C. M. McDonald is a clinical dietitian, Primary Children's Hospital, Salt Lake City, UT.
Biography
E. K. Bowser is an associate in pediatrics and faculty nutritionist, Pediatric Pulmonary Division, University of Florida College of Medicine, Gainesville.
Biography
K. Farnham is a clinical pediatric dietitian, Nemours Children's Specialty Care, Jacksonville, FL.
Biography
M. Mangus is a patient advocate, San Antonio, TX.
Biography
L. Padula is an advanced practice dietitian, Children's Hospital of Philadelphia, Philadelphia, PA.
Biography
K. Porco is a patient advocate, Attain Health Foundation, Red Lodge, MT.
Biography
J. A. Alvarez is an assistant professor, Emory University School of Medicine, Atlanta, GA.
Article info
Publication history
Published online: June 10, 2020
Footnotes
STATEMENT OF POTENTIAL CONFLICT OF INTEREST J. A. Alvarez receives funding from the National Institutes of Health (R03 DK117246, K01 DK102851), and the Cystic Fibrosis Foundation (ALVARE19A0). There were no other conflicts of interest to disclose.
FUNDING/SUPPORT The Academy of Nutrition and Dietetics and the Medical Nutrition Dietetic Practice Group provided funding for this systematic review.
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© 2021 by the Academy of Nutrition and Dietetics.
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- Dietary Macronutrient Distribution and Nutrition Outcomes in Persons with Cystic Fibrosis: An Evidence Analysis Center Systematic ReviewJournal of the Academy of Nutrition and DieteticsVol. 121Issue 8
- PreviewCystic fibrosis (CF) increases risk for undernutrition and malabsorption. Individuals with CF traditionally have been counseled to consume a high-fat diet. However, a new era of CF care has increased lifespan and decreased symptoms in many individuals with CF, necessitating a re-examination of the high-fat CF legacy diet. A literature search was conducted of Medline (Ovid), Embase, and CINAHL (EBSCO) databases to identify articles published from January 2002 to May 2018 in the English language examining the relationships between dietary macronutrient distribution and nutrition outcomes in individuals with CF.
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