Protein intake and prevalence of overweight in patients with phenylketonuria

Bibliographic Details
Main Author: Rodrigues, Catarina
Publication Date: 2025
Other Authors: Sousa Barbosa, Catarina, de Almeida, Manuela Ferreira, Bandeira, Anabela, Martins, Esmeralda, Rocha, Sara, Guimas, Arlindo, Ribeiro, Rosa, Soares, António, Moreira-Rosário, André, Dias, Cláudia Camila, MacDonald, Anita, Borges, Nuno, Rocha, Júlio César
Format: Article
Language: eng
Source: Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
Download full: http://hdl.handle.net/10362/181238
Summary: Funding Information: This work was supported by an Individual Research Agreement between BioMarin Pharmaceutical Inc. and CINTESIS, R&D Unit (reference UIDB/4255/2020). The supporting source had no such involvement or restrictions regarding publication.Anita MacDonald received research funding and honoraria from Nutricia, Vitaflo International, and Merck Serono. She is a member of the Advisory Board entitled ELEMENT (Danone-Nutricia), and member of an Advisory Board for Arla, Applied Pharma Research and Vitaflo International. Catarina Rodrigues received honoraria as a speaker from PIAM. J\u00FAlio C\u00E9sar Rocha was a member of the European Nutritionist Expert Panel (Biomarin), the Advisory Board for Applied Pharma Research, Vitaflo, Synlogic, Biomarin, PTC Therapeutics and Nutricia, and received honoraria as speaker from APR, Merck Serono, Biomarin, Nutricia, Vitaflo, Cambrooke, PIAM, Lifediet and PTC Therapeutics. Funding Information: This work was supported by an Individual Research Agreement between BioMarin Pharmaceutical Inc. and CINTESIS , R&D Unit (reference UIDB/4255/2020 ). The supporting source had no such involvement or restrictions regarding publication. Publisher Copyright: © 2025
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spelling Protein intake and prevalence of overweight in patients with phenylketonuriaA 10-year longitudinal studyNatural proteinOverweightPhenylalaninePhenylketonuriaProtein intakeEndocrinology, Diabetes and MetabolismBiochemistryMolecular BiologyGeneticsEndocrinologySDG 3 - Good Health and Well-beingFunding Information: This work was supported by an Individual Research Agreement between BioMarin Pharmaceutical Inc. and CINTESIS, R&D Unit (reference UIDB/4255/2020). The supporting source had no such involvement or restrictions regarding publication.Anita MacDonald received research funding and honoraria from Nutricia, Vitaflo International, and Merck Serono. She is a member of the Advisory Board entitled ELEMENT (Danone-Nutricia), and member of an Advisory Board for Arla, Applied Pharma Research and Vitaflo International. Catarina Rodrigues received honoraria as a speaker from PIAM. J\u00FAlio C\u00E9sar Rocha was a member of the European Nutritionist Expert Panel (Biomarin), the Advisory Board for Applied Pharma Research, Vitaflo, Synlogic, Biomarin, PTC Therapeutics and Nutricia, and received honoraria as speaker from APR, Merck Serono, Biomarin, Nutricia, Vitaflo, Cambrooke, PIAM, Lifediet and PTC Therapeutics. Funding Information: This work was supported by an Individual Research Agreement between BioMarin Pharmaceutical Inc. and CINTESIS , R&D Unit (reference UIDB/4255/2020 ). The supporting source had no such involvement or restrictions regarding publication. Publisher Copyright: © 2025Background: Overweight has been identified as a comorbidity associated with phenylketonuria (PKU). A systematic review with meta-analysis found that although patients with PKU had a similar body mass index (BMI) when compared to non-PKU controls, there was a significantly higher BMI when measured in patients described as having classical PKU. Objective: The aim of this retrospective longitudinal study was to identify the prevalence of overweight in patients with PKU, over 10 years, in a Portuguese Reference Centre, following a phenylalanine-restricted diet. Methods: Inclusion criteria were diagnosis of PKU and completion of an annual nutritional status evaluation every 2 years. Information on anthropometry, dietary intake and blood phenylalanine levels was collected. Results: The sample consisted of 94 patients (aged 14.0 ± 7.8y, 46 females). Over the study period, there was a non-statistically significant trend towards an increase in the prevalence of overweight (24.5 vs 33.0 %), as defined by age-appropriate BMI. When compared with normal-weight patients, overweight patients had significantly higher blood phenylalanine levels in the first and fifth biennium. Total and natural protein intake were significantly higher in normal-weight patients, at all timepoints, compared to overweight patients. Univariate analysis showed that a higher protein intake, particularly of natural protein, is a protective factor against the development of overweight. This result remained after adjusting total protein intake for age, gender, and metabolic control. Conclusions: This study found a trend towards an increase in the prevalence of overweight in patients with PKU. Therefore, the nutritional status of patients with PKU should be regularly monitored, supported by preventive and attentive nutritional support.NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)Comprehensive Health Research Centre (CHRC) - pólo NMSCentro de Investigação em Tecnologias e Serviços de Saúde (CINTESIS)RUNRodrigues, CatarinaSousa Barbosa, Catarinade Almeida, Manuela FerreiraBandeira, AnabelaMartins, EsmeraldaRocha, SaraGuimas, ArlindoRibeiro, RosaSoares, AntónioMoreira-Rosário, AndréDias, Cláudia CamilaMacDonald, AnitaBorges, NunoRocha, Júlio César2025-03-24T21:15:54Z2025-042025-04-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10362/181238eng1096-7192PURE: 113624794https://doi.org/10.1016/j.ymgme.2025.109068info:eu-repo/semantics/openAccessreponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiainstacron:RCAAP2025-03-31T02:04:30Zoai:run.unl.pt:10362/181238Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-29T04:42:21.121482Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiafalse
dc.title.none.fl_str_mv Protein intake and prevalence of overweight in patients with phenylketonuria
A 10-year longitudinal study
title Protein intake and prevalence of overweight in patients with phenylketonuria
spellingShingle Protein intake and prevalence of overweight in patients with phenylketonuria
Rodrigues, Catarina
Natural protein
Overweight
Phenylalanine
Phenylketonuria
Protein intake
Endocrinology, Diabetes and Metabolism
Biochemistry
Molecular Biology
Genetics
Endocrinology
SDG 3 - Good Health and Well-being
title_short Protein intake and prevalence of overweight in patients with phenylketonuria
title_full Protein intake and prevalence of overweight in patients with phenylketonuria
title_fullStr Protein intake and prevalence of overweight in patients with phenylketonuria
title_full_unstemmed Protein intake and prevalence of overweight in patients with phenylketonuria
title_sort Protein intake and prevalence of overweight in patients with phenylketonuria
author Rodrigues, Catarina
author_facet Rodrigues, Catarina
Sousa Barbosa, Catarina
de Almeida, Manuela Ferreira
Bandeira, Anabela
Martins, Esmeralda
Rocha, Sara
Guimas, Arlindo
Ribeiro, Rosa
Soares, António
Moreira-Rosário, André
Dias, Cláudia Camila
MacDonald, Anita
Borges, Nuno
Rocha, Júlio César
author_role author
author2 Sousa Barbosa, Catarina
de Almeida, Manuela Ferreira
Bandeira, Anabela
Martins, Esmeralda
Rocha, Sara
Guimas, Arlindo
Ribeiro, Rosa
Soares, António
Moreira-Rosário, André
Dias, Cláudia Camila
MacDonald, Anita
Borges, Nuno
Rocha, Júlio César
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)
Comprehensive Health Research Centre (CHRC) - pólo NMS
Centro de Investigação em Tecnologias e Serviços de Saúde (CINTESIS)
RUN
dc.contributor.author.fl_str_mv Rodrigues, Catarina
Sousa Barbosa, Catarina
de Almeida, Manuela Ferreira
Bandeira, Anabela
Martins, Esmeralda
Rocha, Sara
Guimas, Arlindo
Ribeiro, Rosa
Soares, António
Moreira-Rosário, André
Dias, Cláudia Camila
MacDonald, Anita
Borges, Nuno
Rocha, Júlio César
dc.subject.por.fl_str_mv Natural protein
Overweight
Phenylalanine
Phenylketonuria
Protein intake
Endocrinology, Diabetes and Metabolism
Biochemistry
Molecular Biology
Genetics
Endocrinology
SDG 3 - Good Health and Well-being
topic Natural protein
Overweight
Phenylalanine
Phenylketonuria
Protein intake
Endocrinology, Diabetes and Metabolism
Biochemistry
Molecular Biology
Genetics
Endocrinology
SDG 3 - Good Health and Well-being
description Funding Information: This work was supported by an Individual Research Agreement between BioMarin Pharmaceutical Inc. and CINTESIS, R&D Unit (reference UIDB/4255/2020). The supporting source had no such involvement or restrictions regarding publication.Anita MacDonald received research funding and honoraria from Nutricia, Vitaflo International, and Merck Serono. She is a member of the Advisory Board entitled ELEMENT (Danone-Nutricia), and member of an Advisory Board for Arla, Applied Pharma Research and Vitaflo International. Catarina Rodrigues received honoraria as a speaker from PIAM. J\u00FAlio C\u00E9sar Rocha was a member of the European Nutritionist Expert Panel (Biomarin), the Advisory Board for Applied Pharma Research, Vitaflo, Synlogic, Biomarin, PTC Therapeutics and Nutricia, and received honoraria as speaker from APR, Merck Serono, Biomarin, Nutricia, Vitaflo, Cambrooke, PIAM, Lifediet and PTC Therapeutics. Funding Information: This work was supported by an Individual Research Agreement between BioMarin Pharmaceutical Inc. and CINTESIS , R&D Unit (reference UIDB/4255/2020 ). The supporting source had no such involvement or restrictions regarding publication. Publisher Copyright: © 2025
publishDate 2025
dc.date.none.fl_str_mv 2025-03-24T21:15:54Z
2025-04
2025-04-01T00:00:00Z
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/10362/181238
url http://hdl.handle.net/10362/181238
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 1096-7192
PURE: 113624794
https://doi.org/10.1016/j.ymgme.2025.109068
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:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
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instname_str FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
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reponame_str Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
collection Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
repository.name.fl_str_mv Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
repository.mail.fl_str_mv info@rcaap.pt
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