Abstract
Cystic 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. Articles were screened, risk of bias was assessed, data were
synthesized narratively, and each outcome was graded for certainty of evidence. The
databases search retrieved 2,519 articles, and 7 cross-sectional articles were included
in the final narrative analysis. Three studies examined pediatric participants and
4 examined adults. None of the included studies reported on outcomes of mortality
or quality of life. Very low certainty evidence described no apparent relationship
between dietary macronutrient distribution and lung function, anthropometric measures,
or lipid profile in individuals with CF. The current systematic review demonstrates
wide ranges in the dietary macronutrient intakes of individuals with CF with little
to no demonstrable relationship between macronutrient distribution and nutrition-related
outcomes. No evidence is presented to substantiate an outcomes-related benefit to
a higher fat-diet except in the context of achieving higher energy intakes in a lesser
volume of food.
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References
- Cystic fibrosis: An update for clinicians. Part 1: Nutrition and gastrointestinal complications.J Gastroenterol Hepatol. 2014; 29: 1344-1355
- Cystic fibrosis mortality and survival in the UK: 1947-2003.Eur Respir J. 2007; 29: 522-526
- A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto.J Clin Epidemiol. 1988; 41: 583-591
- A rational approach to the nutritional care of patients with cystic fibrosis.J R Soc Med. 1989; 82: 11-20
- ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis.Clin Nutr. 2016; 35: 557-577
- Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand.Thoracic Society of Australia and New Zealand, Sydney2017
- Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: Results of a systematic review.J Am Diet Assoc. 2008; 108: 832-839
- The relative contribution of food groups to macronutrient intake in children with cystic fibrosis: A European multicenter assessment.J Acad Nutr Diet. 2019; 119: 1305-1319
- Dietary intake of energy-dense, nutrient-poor and nutrient-dense food sources in children with cystic fibrosis.J Cyst Fibros. 2018; 17: 804-810
- Metabolic syndrome and dietary patterns: A systematic review and meta-analysis of observational studies.Eur J Nutr. 2017; 56: 925-947
- Nutrition and cardiovascular health.Int J Mol Sci. 2018; 19
- Possible role of diet in cancer: Systematic review and multiple meta-analyses of dietary patterns, lifestyle factors, and cancer risk.Nutr Rev. 2017; 75: 405-419
- 2017 Annual Data Report.Cystic Fibrosis Foundation, Bethesda, MD2018
- Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: A population-based cohort study.Am J Clin Nutr. 2013; 97: 872-877
- Prevalence and factors associated with overweight and obesity in adults with cystic fibrosis: A single-center analysis.J Cyst Fibros. 2020; 19: 139-145
- Academy of Nutrition and Dietetics methodology for conducting systematic reviews for the Evidence Analysis Library.J Acad Nutr Diet. 2016; 116: 311-318
- Preferred reporting items for systematic reviews and meta-analyses: The PRISMA statement.J Clin Epidemiol. 2009; 62: 1006-1012
- PROSPERO 2018 CRD42018097373. National Institute for Health Research.(Published 2018. Accessed December 3, 2019)
- Opportunities for Consumer Engagement.(Published 2016. Accessed December 3, 2019)
- Nutrition care for patients with cystic fibrosis: An Evidence Analysis Center scoping review.J Acad Nutr Diet. 2019; 119: 137-151.e131
- Rayyan-a web and mobile app for systematic reviews.Syst Rev. 2016; 5: 210
- Evidence Analysis Manual: Steps in the Academy Evidence Analysis Process: A Systematic Review and Guideline Manual.Academy of Nutrition and Dietetics, Chicago, IL2016
- GRADE guidelines: 1. Introduction-GRADE evidence profiles and summary of findings tables.J Clin Epidemiol. 2011; 64: 383-394
- GRADE guidelines: 11. Making an overall rating of confidence in effect estimates for a single outcome and for all outcomes.J Clin Epidemiol. 2013; 66: 151-157
- GRADE guidelines: 3. Rating the quality of evidence.J Clin Epidemiol. 2011; 64: 401-406
- Anthropometric and dietary intake indicators as predictors of pulmonary function in cystic fibrosis patients.J Bras Pneumol. 2012; 38: 470-476
- Nutrient status of adults with cystic fibrosis.J Am Diet Assoc. 2007; 107: 2114-2119
- Dietary intake and nutritional status in a Scandinavian adult cystic fibrosis-population compared with recommendations.Food Nutr Res. 2011; 55
- Serum phospholipid fatty acid profile and dietary intake in an adult Mediterranean population with cystic fibrosis.Br J Nutr. 2006; 96: 343-349
- Nutritional intake and status in children with cystic fibrosis: Does age matter?.J Pediat Gastroenterol Nutr. 2007; 44: 116-123
- Dietary intake and lipid profile in children and adolescents with cystic fibrosis.J Cyst Fibros. 2017; 16: 410-417
- Glucose fluctuations are not modulated by the proportion of calories from macronutrients or spontaneous total energy expenditure in adults with cystic fibrosis.Can J Diabetes. 2016; 40: 389-392
- Dietary Reference Intakes for Energy, Carbohydrate, Fiber, Fat, Fatty Acids, Cholesterol, Protein, and Amino Acids.The National Academies Press, Washington, DC2005
- Dietary Guidelines for Americans 2015-2020, 8th ed.(Published December 2015. Accessed February 28, 2020)
- Programmes and projects. Population nutrient intake goals for preventing diet-related chronic diseases.(Published 2018. Accessed December 3, 2019)
- Prevalence of dyslipidemia in adults with cystic fibrosis.J Cyst Fibros. 2010; 9: 24-28
- Dyslipidemia in racially admixtured children with cystic fibrosis.Indian J Endocrinol Metab. 2012; 16: 585-588
- Abnormal lipid concentrations in cystic fibrosis.Am J Clin Nutr. 2002; 75: 1005-1011
- Cystic fibrosis dyslipidaemia: A cross-sectional study.J Cyst Fibros. 2019; 18: 566-571
- Visceral adipose tissue is associated with poor diet quality and higher fasting glucose in adults with cystic fibrosis.J Cyst Fibros. 2019; 18: 430-435
- Healthy Diet. Fact sheet 394. 2018.(Available at)https://www.who.int/nutrition/publications/nutrientrequirements/healthy_diet_fact_sheet_394.pdf?ua=1(Published August 2018. Accessed Februry 28, 2020)
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
J. A. Alvarez is an assistant professor of medicine, Emory University School of Medicine, Atlanta, GA.
Biography
L. Padula is an advanced practice dietitian, Children's Hospital of Philadelphia, Philadelphia, PA.
Biography
M. Rozga is a nutrition researcher, Academy of Nutrition and Dietetics, Evidence Analysis Center, Chicago, IL.
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). No other potential conflict of interest was reported by the authors.
FUNDING/SUPPORT This work was supported by the Academy of Nutrition and Dietetics and the Medical Nutrition Dietetic Practice Group of the Academy of Nutrition and Dietetics.
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- Effect of CFTR Modulators on Anthropometric Parameters in Individuals with Cystic Fibrosis: An Evidence Analysis Center Systematic ReviewJournal of the Academy of Nutrition and DieteticsVol. 121Issue 7
- PreviewThere 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.
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