Total Protein Intake in Patients with PKU: Adequacy Evaluation According to the European PKU Guidelines from 2017

Melanie Gomes, Manuela Ferreira Almeida, Catarina Sousa Barbosa, Maria Inês Gama, Maria Peres, Élia Pinto, Anita MacDonald, Júlio César Rocha

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Abstract

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.

Original languageEnglish
Article number4883
JournalNutrients
Volume15
Issue number23
DOIs
Publication statusPublished - Dec 2023

Keywords

  • European PKU guidelines
  • natural protein
  • phenylalanine
  • phenylketonuria
  • protein intake
  • protein intake adequacy
  • protein substitutes

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