Article ; Online: Total Protein Intake in Patients with PKU: Adequacy Evaluation According to the European PKU Guidelines from 2017.
2023 Volume 15, Issue 23
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 ...
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. |
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MeSH term(s) | Male ; Humans ; Female ; Phenylalanine ; Phenylketonurias ; Nutritional Status ; Diet ; Anthropometry |
Chemical Substances | Phenylalanine (47E5O17Y3R) |
Language | English |
Publishing date | 2023-11-22 |
Publishing country | Switzerland |
Document type | Journal Article |
ZDB-ID | 2518386-2 |
ISSN | 2072-6643 ; 2072-6643 |
ISSN (online) | 2072-6643 |
ISSN | 2072-6643 |
DOI | 10.3390/nu15234883 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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