Author(s): Gomes, Melanie ; Almeida, Manuela Ferreira ; Barbosa, Catarina Sousa ; Gama, Maria Inês ; Peres, Maria ; Pinto, Élia ; MacDonald, Anita ; Rocha, Júlio César
Date: 2023
Persistent ID: http://hdl.handle.net/10362/161519
Origin: Repositório Institucional da UNL
Subject(s): European PKU guidelines; natural protein; phenylalanine; phenylketonuria; protein intake; protein intake adequacy; protein substitutes; Food Science; Nutrition and Dietetics
Funding Information: CINTESIS-UIDB/04255/2020 and UIDP/04255/2020, a program financially supported by Fundação para a Ciência e Tecnologia/Ministério da Ciência, Tecnologia e Ensino Superior, through national funds is acknowledged. Publisher Copyright: © 2023 by the authors.
In PKU, the protein requirements are contentious. In 2018, we evaluated the protein intake in patients with PKU. Ninety-nine early treated patients aged 19.3 ± 8.2 years (54% males) were studied. A total of 24 had hyperphenylalaninemia (HPA), 48 mild and 27 classical PKU. All had an annual nutritional status evaluation. A total of 83% were on diet therapy only, and 17% were on diet with tetrahydrobiopterin therapy. Anthropometry, metabolic control and nutritional intake [total protein (TP, g/kg), natural protein (NP, g/kg), protein equivalent from protein substitutes (PE, g/kg)] were collected. TP adequacy (TPA) was calculated as a % of WHO (2007) safe levels of protein intake. Results were compared with the European PKU Guidelines (EPG). The median % contribution NP of TP intake was 53% [31–100]. Most patients (78%) had a TP intake above the EPG recommendations. The median TPA was 171% [146–203], with 79% [51–165] from NP and 84% [0–109] from PE. A TPA of 100–140% was observed in 16 (16%) patients. Only n = 6 (6%) patients had a TPA < 100%. These results emphasize the heterogeneity of PKU. More research is needed to understand the necessity of a single protein recommendation for all, as a ‘one-size-fits-all’ solution might not be appropriate.
