Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria
| Autor(a) principal: | |
|---|---|
| Data de Publicação: | 2021 |
| Outros Autores: | , , , , , , , , , , , , |
| Tipo de documento: | Artigo |
| Idioma: | eng |
| Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
| Texto Completo: | http://hdl.handle.net/10362/114280 |
Resumo: | This article was funded by the project NORTE-08-5369-FSE-000018, supported by Norte Portugal Regional Programme (Norte2020), under the PORTUGAL 2020 Partnership Agreement, through the European Regional Development Fund (ERDF) and by FCT—Fundação para a Ciência e a Tecnologia [UID/ BIM/04293/2013]. |
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Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuriaa clinical perspectiveAmino acidsCasein glycomacropeptideNutritional statusPhenylalaninePhenylketonuriaTyrosineGenetics(clinical)Pharmacology (medical)This article was funded by the project NORTE-08-5369-FSE-000018, supported by Norte Portugal Regional Programme (Norte2020), under the PORTUGAL 2020 Partnership Agreement, through the European Regional Development Fund (ERDF) and by FCT—Fundação para a Ciência e a Tecnologia [UID/ BIM/04293/2013].Background: In phenylketonuria (PKU), modified casein glycomacropeptide supplements (CGMP-AA) are used as an alternative to the traditional phenylalanine (Phe)-free L-amino acid supplements (L-AA). However, studies focusing on the long-term nutritional status of CGMP-AA are lacking. This retrospective study evaluated the long-term impact of CGMP-AA over a mean of 29 months in 11 patients with a mean age at CGMP-AA onset of 28 years (range 15–43) [8 females; 2 hyperphenylalaninaemia (HPA), 3 mild PKU, 3 classical PKU and 3 late-diagnosed]. Outcome measures included metabolic control, anthropometry, body composition and biochemical parameters. Results: CGMP-AA, providing 66% of protein equivalent intake from protein substitute, was associated with no significant change in blood Phe with CGMP-AA compared with baseline (562 ± 289 µmol/L vs 628 ± 317 µmol/L; p = 0.065). In contrast, blood tyrosine significantly increased on CGMP-AA (52.0 ± 19.2 μmol/L vs 61.4 ± 23.8 μmol/L; p = 0.027). Conclusions: Biochemical nutritional markers remained unchanged which is an encouraging finding in adults with PKU, many of whom are unable to maintain full adherence with nutritionally fortified protein substitutes. Longitudinal, prospective studies with larger sample sizes are necessary to fully understand the metabolic impact of using CGMP-AA in PKU.NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)RUNPena, Maria JoãoPinto, Alexde Almeida, Manuela Ferreirade Sousa Barbosa, CatarinaRamos, Paula CristinaRocha, SaraGuimas, ArlindoRibeiro, RosaMartins, EsmeraldaBandeira, AnabelaDias, Cláudia CamilaMacDonald, AnitaBorges, NunoRocha, Júlio César2021-03-22T23:28:16Z2021-03-132021-03-13T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10362/114280eng1750-1172PURE: 28644497https://doi.org/10.1186/s13023-021-01721-8info: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:RCAAP2024-03-11T04:56:55Zoai:run.unl.pt:10362/114280Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T03:42:28.983092Repositório 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 |
Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria a clinical perspective |
| title |
Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria |
| spellingShingle |
Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria Pena, Maria João Amino acids Casein glycomacropeptide Nutritional status Phenylalanine Phenylketonuria Tyrosine Genetics(clinical) Pharmacology (medical) |
| title_short |
Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria |
| title_full |
Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria |
| title_fullStr |
Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria |
| title_full_unstemmed |
Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria |
| title_sort |
Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria |
| author |
Pena, Maria João |
| author_facet |
Pena, Maria João Pinto, Alex de Almeida, Manuela Ferreira de Sousa Barbosa, Catarina Ramos, Paula Cristina Rocha, Sara Guimas, Arlindo Ribeiro, Rosa Martins, Esmeralda Bandeira, Anabela Dias, Cláudia Camila MacDonald, Anita Borges, Nuno Rocha, Júlio César |
| author_role |
author |
| author2 |
Pinto, Alex de Almeida, Manuela Ferreira de Sousa Barbosa, Catarina Ramos, Paula Cristina Rocha, Sara Guimas, Arlindo Ribeiro, Rosa Martins, Esmeralda Bandeira, Anabela 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) RUN |
| dc.contributor.author.fl_str_mv |
Pena, Maria João Pinto, Alex de Almeida, Manuela Ferreira de Sousa Barbosa, Catarina Ramos, Paula Cristina Rocha, Sara Guimas, Arlindo Ribeiro, Rosa Martins, Esmeralda Bandeira, Anabela Dias, Cláudia Camila MacDonald, Anita Borges, Nuno Rocha, Júlio César |
| dc.subject.por.fl_str_mv |
Amino acids Casein glycomacropeptide Nutritional status Phenylalanine Phenylketonuria Tyrosine Genetics(clinical) Pharmacology (medical) |
| topic |
Amino acids Casein glycomacropeptide Nutritional status Phenylalanine Phenylketonuria Tyrosine Genetics(clinical) Pharmacology (medical) |
| description |
This article was funded by the project NORTE-08-5369-FSE-000018, supported by Norte Portugal Regional Programme (Norte2020), under the PORTUGAL 2020 Partnership Agreement, through the European Regional Development Fund (ERDF) and by FCT—Fundação para a Ciência e a Tecnologia [UID/ BIM/04293/2013]. |
| publishDate |
2021 |
| dc.date.none.fl_str_mv |
2021-03-22T23:28:16Z 2021-03-13 2021-03-13T00:00:00Z |
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info:eu-repo/semantics/publishedVersion |
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info:eu-repo/semantics/article |
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article |
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publishedVersion |
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http://hdl.handle.net/10362/114280 |
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http://hdl.handle.net/10362/114280 |
| dc.language.iso.fl_str_mv |
eng |
| language |
eng |
| dc.relation.none.fl_str_mv |
1750-1172 PURE: 28644497 https://doi.org/10.1186/s13023-021-01721-8 |
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info:eu-repo/semantics/openAccess |
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openAccess |
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application/pdf |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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