Introduction: Phenylketonuria (PKU) is a inborn error of amino acid metabolism in
which there is reduced activity of the enzyme that converts phenylalanine into
tyrosine. The diagnosis is made by the neonatal screening test and its treatment
must be started as soon as possible and maintained throughout life. The main
treatment is a low phenylalanine diet, following the patient's tolerance; therefore the
objective of the present work is to develop a booklet with nutritional guidelines for
patients diagnosed with PKU, to be used as support in their treatment. Methodology:
This is a descriptive study with a qualitative approach through an Integrative
Literature Review, carried out from August to November 2022. Articles from the
Lilacs, Pubmed, Google Scholar and Scielo were selected, in addition to manuals
from the Ministério da Saúde (MS), from 2017 to 2022 based on the following
keywords: health education, diet therapy in PKU, breastfeeding in PKU and child
health. Articles not available in full, not related to the topic, theses and dissertations
were excluded. The study was carried out in two stages, the survey of scientific
information and the construction of the booklet. Results: Three articles and Clinical
Practice Guidelines Phenylketonuria were used in the construction of the booklet. A
digital booklet was then prepared, with the topics: Introduction, Food groups,
Breastfeeding, Allowed Foods, Moderate Consumption Foods and Prohibited Foods.
Conclusion: The booklet is an educational technology that can assists in the
nutritional guidance of children with PKU.
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