Protein intake and prevalence of overweight in patients with phenylketonuria
| Main Author: | |
|---|---|
| Publication Date: | 2025 |
| Other Authors: | , , , , , , , , , , , , |
| 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 |
| id |
RCAP_8a30fc707c0ee7d68be267bbc6eb7be8 |
|---|---|
| oai_identifier_str |
oai:run.unl.pt:10362/181238 |
| network_acronym_str |
RCAP |
| network_name_str |
Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) |
| repository_id_str |
https://opendoar.ac.uk/repository/7160 |
| 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 instacron:RCAAP |
| instname_str |
FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia |
| instacron_str |
RCAAP |
| institution |
RCAAP |
| 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 |
| _version_ |
1833602126561935360 |