Dietary intake in individuals with phenylketonuria: an integrative review

Introduction: the dietary intake of individuals with phenylketonuria (PKU) may vary widely according to different cultural eating habits, lifestyle, access to multidisciplinary team, and metabolic formulas available. Thus, knowing the dietary intake of this population makes it possible to tailor nutritional treatment strategies to impact their health. Objective: to analyze the evidence on the dietary intake of individuals with PKU. Methods: an integrative literature review was conducted on the dietary intake of individuals with PKU in the databases PUBMED, BIREME and Science Direct. Original articles that addressed the energy and macronutrient food intake of children, adolescents and/or adults with PKU were included in the study, without time restriction, in any language. A total of 384 articles were found and 27 articles were selected and analyzed. Results: evidence about the nutritional composition of their diet showed that individuals with PKU consume between 1160-2721 kcal of energy -7.2-17.4 % (32.4-76.9 g) of energy as protein, 45.9-69.2 % of energy as carbohydrates, 16.6-39 % of energy as lipids- and between 7.6 and 20 g of fiber. Conclusion: most individuals with PKU have low energy, protein and fiber intake, adequate lipid intake, and high carbohydrate intake. Metabolic control of the disease is still a challenge in all countries. Nutritional strategies to improve dietary nutritional composition and phenylalanine blood levels in individuals with PKU remain an urgent issue.