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Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective

2021-02Journal article Open access
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dc.contributor.authorPena, Maria João
dc.contributor.authorPinto, Alex
dc.contributor.authorde Almeida, Manuela Ferreira
dc.contributor.authorde Sousa Barbosa, Catarina
dc.contributor.authorRamos, Paula Cristina
dc.contributor.authorRocha, Sara
dc.contributor.authorGuimas, Arlindo
dc.contributor.authorRibeiro, Rosa
dc.contributor.authorMartins, Esmeralda
dc.contributor.authorBandeira, Anabela
dc.contributor.authorDias, Cláudia Camila
dc.contributor.authorMacDonald, Anita
dc.contributor.authorBorges, Nuno
dc.contributor.authorRocha, Júlio César
dc.date.accessioned2023-10-30T16:08:24Z
dc.date.available2023-10-30T16:08:24Z
dc.date.issued2021-02
dc.description.abstractBackground: 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.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationPena MJ, Pinto A, de Almeida MF, et al. Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective. Orphanet J Rare Dis. 2021;16(1):84. doi:10.1186/s13023-021-01721-8pt_PT
dc.identifier.doi10.1186/s13023-021-01721-8pt_PT
dc.identifier.issn1750-1172
dc.identifier.urihttp://hdl.handle.net/10400.16/2861
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.relationInstitute for Research and Innovation in Health Sciences
dc.relation.publisherversionhttps://ojrd.biomedcentral.com/articles/10.1186/s13023-021-01721-8pt_PT
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/pt_PT
dc.subjectAmino acidspt_PT
dc.subjectCasein glycomacropeptidept_PT
dc.subjectNutritional statuspt_PT
dc.subjectPhenylalaninept_PT
dc.subjectPhenylketonuriapt_PT
dc.subjectTyrosinept_PT
dc.titleContinuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspectivept_PT
dc.typejournal article
dspace.entity.typePublication
oaire.awardTitleInstitute for Research and Innovation in Health Sciences
oaire.awardURIinfo:eu-repo/grantAgreement/FCT/6817 - DCRRNI ID/UID%2FBIM%2F04293%2F2013/PT
oaire.citation.conferencePlaceEnglandpt_PT
oaire.citation.issue1pt_PT
oaire.citation.startPage84pt_PT
oaire.citation.titleOrphanet Journal of Rare Diseasespt_PT
oaire.citation.volume16pt_PT
oaire.fundingStream6817 - DCRRNI ID
person.familyNameGuimas
person.familyNameMartins
person.familyNameBandeira
person.familyNameRocha
person.givenNameArlindo
person.givenNameEsmeralda
person.givenNameAnabela Oliveira
person.givenNameJúlio César
person.identifier.ciencia-idBF1C-DAE5-FAEA
person.identifier.ciencia-id7D12-C53D-288E
person.identifier.ciencia-idED11-107D-ABFB
person.identifier.orcid0000-0001-5631-8948
person.identifier.orcid0000-0002-9247-9391
person.identifier.orcid0000-0003-1203-8180
person.identifier.orcid0000-0002-4977-8345
person.identifier.ridK-5399-2013
person.identifier.scopus-author-id35196853000
project.funder.identifierhttp://doi.org/10.13039/501100001871
project.funder.nameFundação para a Ciência e a Tecnologia
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
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