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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