Effect of CFTR Modulators on Anthropometric Parameters in Individuals with Cystic Fibrosis: An Evidence Analysis Center Systematic Review
| Autor: | Jessica A. Alvarez, Ellen K. Bowser, Catherine M. McDonald, Mark Mangus, Kathleen Porco, Mary Rozga, Julianna Bailey, Laura Padula, Kristen Farnham |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
0301 basic medicine
Adult Male medicine.medical_specialty Adolescent Cystic Fibrosis Cystic Fibrosis Transmembrane Conductance Regulator 030209 endocrinology & metabolism CINAHL Cystic fibrosis law.invention Body Mass Index Ivacaftor 03 medical and health sciences chemistry.chemical_compound Young Adult 0302 clinical medicine Randomized controlled trial law Internal medicine Membrane Transport Modulators Medicine Humans Child Randomized Controlled Trials as Topic 030109 nutrition & dietetics Nutrition and Dietetics Anthropometry business.industry Lumacaftor Body Weight General Medicine medicine.disease Treatment Outcome chemistry Mutation Female medicine.symptom business Body mass index Weight gain Food Science medicine.drug |
| Zdroj: | Journal of the Academy of Nutrition and Dietetics. 121(7) |
| ISSN: | 2212-2672 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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