Total Protein Intake in Patients with PKU

Detalhes bibliográficos
Autor(a) principal: Gomes, Melanie
Data de Publicação: 2023
Outros Autores: Almeida, Manuela Ferreira, Barbosa, Catarina Sousa, Gama, Maria Inês, Peres, Maria, Pinto, Élia, MacDonald, Anita, Rocha, Júlio César
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10362/161519
Resumo: 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.
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spelling Total Protein Intake in Patients with PKUAdequacy Evaluation According to the European PKU Guidelines from 2017European PKU guidelinesnatural proteinphenylalaninephenylketonuriaprotein intakeprotein intake adequacyprotein substitutesFood ScienceNutrition and DieteticsFunding 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.NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)Centro de Investigação em Tecnologias e Serviços de Saúde (CINTESIS)RUNGomes, MelanieAlmeida, Manuela FerreiraBarbosa, Catarina SousaGama, Maria InêsPeres, MariaPinto, ÉliaMacDonald, AnitaRocha, Júlio César2023-12-20T23:54:33Z2023-122023-12-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10362/161519eng1422-8599PURE: 78405263https://doi.org/10.3390/nu15234883info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2024-03-11T05:44:26Zoai:run.unl.pt:10362/161519Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T03:58:33.936151Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Total Protein Intake in Patients with PKU
Adequacy Evaluation According to the European PKU Guidelines from 2017
title Total Protein Intake in Patients with PKU
spellingShingle Total Protein Intake in Patients with PKU
Gomes, Melanie
European PKU guidelines
natural protein
phenylalanine
phenylketonuria
protein intake
protein intake adequacy
protein substitutes
Food Science
Nutrition and Dietetics
title_short Total Protein Intake in Patients with PKU
title_full Total Protein Intake in Patients with PKU
title_fullStr Total Protein Intake in Patients with PKU
title_full_unstemmed Total Protein Intake in Patients with PKU
title_sort Total Protein Intake in Patients with PKU
author Gomes, Melanie
author_facet Gomes, Melanie
Almeida, Manuela Ferreira
Barbosa, Catarina Sousa
Gama, Maria Inês
Peres, Maria
Pinto, Élia
MacDonald, Anita
Rocha, Júlio César
author_role author
author2 Almeida, Manuela Ferreira
Barbosa, Catarina Sousa
Gama, Maria Inês
Peres, Maria
Pinto, Élia
MacDonald, Anita
Rocha, Júlio César
author2_role author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)
Centro de Investigação em Tecnologias e Serviços de Saúde (CINTESIS)
RUN
dc.contributor.author.fl_str_mv Gomes, Melanie
Almeida, Manuela Ferreira
Barbosa, Catarina Sousa
Gama, Maria Inês
Peres, Maria
Pinto, Élia
MacDonald, Anita
Rocha, Júlio César
dc.subject.por.fl_str_mv European PKU guidelines
natural protein
phenylalanine
phenylketonuria
protein intake
protein intake adequacy
protein substitutes
Food Science
Nutrition and Dietetics
topic European PKU guidelines
natural protein
phenylalanine
phenylketonuria
protein intake
protein intake adequacy
protein substitutes
Food Science
Nutrition and Dietetics
description 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.
publishDate 2023
dc.date.none.fl_str_mv 2023-12-20T23:54:33Z
2023-12
2023-12-01T00:00:00Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://hdl.handle.net/10362/161519
url http://hdl.handle.net/10362/161519
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 1422-8599
PURE: 78405263
https://doi.org/10.3390/nu15234883
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.source.none.fl_str_mv reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
repository.mail.fl_str_mv
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