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Total Protein Intake in Patients with PKU

2023-12Artigo científico Acesso aberto
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dc.contributor.authorGomes, Melanie
dc.contributor.authorAlmeida, Manuela Ferreira
dc.contributor.authorBarbosa, Catarina Sousa
dc.contributor.authorGama, Maria Inês
dc.contributor.authorPeres, Maria
dc.contributor.authorPinto, Élia
dc.contributor.authorMacDonald, Anita
dc.contributor.authorRocha, Júlio César
dc.contributor.institutionNOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)
dc.contributor.institutionCentro de Investigação em Tecnologias e Serviços de Saúde (CINTESIS)
dc.contributor.pblMDPI AG
dc.date.accessioned2023-12-20T23:54:33Z
dc.date.available2023-12-20T23:54:33Z
dc.date.issued2023-12
dc.descriptionFunding 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.
dc.description.abstractIn 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.en
dc.description.versionpublishersversion
dc.description.versionpublished
dc.format.extent1041453
dc.identifier.doi10.3390/nu15234883
dc.identifier.issn1422-8599
dc.identifier.otherPURE: 78405263
dc.identifier.otherPURE UUID: 040e56f0-bfd2-466b-a0b4-b0740467af47
dc.identifier.otherScopus: 85178958174
dc.identifier.otherWOS: 001117500300001
dc.identifier.urihttp://hdl.handle.net/10362/161519
dc.identifier.urlhttps://www.scopus.com/pages/publications/85178958174
dc.language.isoeng
dc.peerreviewedyes
dc.subjectEuropean PKU guidelines
dc.subjectnatural protein
dc.subjectphenylalanine
dc.subjectphenylketonuria
dc.subjectprotein intake
dc.subjectprotein intake adequacy
dc.subjectprotein substitutes
dc.subjectFood Science
dc.subjectNutrition and Dietetics
dc.titleTotal Protein Intake in Patients with PKUen
dc.title.subtitleAdequacy Evaluation According to the European PKU Guidelines from 2017en
dc.typejournal article
degois.publication.issue23
degois.publication.titleNutrients
degois.publication.volume15
dspace.entity.typePublication
rcaap.rightsopenAccess

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