Brazilian Journals Publicações de Periódicos e Editora Ltda.
Abstract
Objective: To evaluate the quality of life of adults with phenylketonuria (PKU) using different methodologies. Methods: review developed according to PRISMA report items and the guidelines of the Cochrane Handbook for Systematic Reviews. Research was performed in PubMed, EMBASE, BVS, SCOPUS and Cochrane Library databases in November 2021. Results: 500 articles were identified and following the inclusion and exclusion criteria, 09 selected studies evaluated 452 adults, with average age of 28 years old. 89% of the articles considered the sample insufficient, 66.6% used generic questionnaires and the remaining 33.3% used the specific questionnaire (PKU-QoL). Conclusion: The results of the impact of PKU on HRQoL were mostly positive. More research using PKU-QoL is needed to broaden the interpretation of data and expand knowledge on this topic, the development of robust studies will provide a conclusion on the effect of PKU and its treatment on HRQoL
