Protocol for ensuring the food and nutritional security of students with Phenylketonuria through translational research

Abstract

Introduction: Phenylketonuria is characterized by a mutation in the enzyme phenylalanine hydroxylase, which metabolizes phenylalanine, an amino acid present in almost all foods. The loss of activity of this enzyme results in an increase in the concentration of phenylalanine, to toxic levels. The standard treatment for this condition mainly comprises two strategies: a diet restricted in phenylalanine, and the use of a metabolic formula rich in amino acids, but free of phenylalanine. Objective: In Brazil, there are protocols to guarantee food and nutritional security in schoolchildren with specific conditions, but those with phenylketonuria are not covered. Feeding this group is costly and government support may be necessary to ensure adequate nutrient consumption, especially for those families in situations of social vulnerability. The objective of this work is to propose a protocol, through translational research, that guarantees the food and nutritional security of students with phenylketonuria in public schools in the Federal District (DF), which can serve as a basis for the creation of a national protocol. Methods: The work reviewed existing policies through searches in databases (PubMed, BVS and Embase) and government websites. The suggested protocol was created from a new approach and adaptation of protocols already in progress, which makes its implementation feasible. Conclusion: The protocol is extremely important for the proper management of this population and will ensure the necessary intake of amino acids for the full cognitive development of students with phenylketonuria.