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The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis

dc.contributor.authorPena, M.
dc.contributor.authorPinto, A.
dc.contributor.authorDaly, A.
dc.contributor.authorMacDonald, A.
dc.contributor.authorAzevedo, L.
dc.contributor.authorRocha, J.
dc.contributor.authorBorges, N.
dc.date.accessioned2020-02-03T13:05:15Z
dc.date.available2020-02-03T13:05:15Z
dc.date.issued2018-11-18
dc.description.abstractIn phenylketonuria (PKU), synthetic protein derived from L-amino acids (AAs) is essential in a low-phenylalanine (Phe) diet. Glycomacropeptide (GMP), an intact protein, is very low in Phe in its native form. It has been modified and adapted for PKU to provide an alternative protein source through supplementation with rate-limiting amino acids (GMP-AAs), although it still contains residual Phe. This review aims to systematically evaluate published intervention studies on the use of GMP-AAs in PKU by considering its impact on blood Phe control (primary aim) and changes in tyrosine control, nutritional biomarkers, and patient acceptability or palatability (secondary aims). Four electronic databases were searched for articles published from 2007 to June 2018. Of the 274 studies identified, only eight were included. Bias risk was assessed and a quality appraisal of the body of evidence was completed. A meta-analysis was performed with two studies with adequate comparable methodology which showed no differences between GMP-AAs and AAs for any of the interventions analysed. This work underlines the scarcity and nature of studies with GMP-AAs interventions. All were short-term with small sample sizes. There is a need for better-designed studies to provide the best evidence-based recommendations.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationPena MJ, Pinto A, Daly A, et al. The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis. Nutrients. 2018;10(11):1794. Published 2018 Nov 18.pt_PT
dc.identifier.doi10.3390/nu10111794pt_PT
dc.identifier.eissn2072-6643
dc.identifier.issn2072-6643
dc.identifier.urihttp://hdl.handle.net/10400.16/2308
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherMDPIpt_PT
dc.relation.publisherversionhttps://www.mdpi.com/2072-6643/10/11/1794pt_PT
dc.subjectacceptabilitypt_PT
dc.subjectamino acidspt_PT
dc.subjectglycomacropeptidept_PT
dc.subjectmetabolic controlpt_PT
dc.subjectnutritional biomarkerspt_PT
dc.subjectphenylalaninept_PT
dc.subjectphenylketonuriapt_PT
dc.titleThe Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysispt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlaceSwitzerlandpt_PT
oaire.citation.issue11pt_PT
oaire.citation.startPage1794pt_PT
oaire.citation.titleNutrientspt_PT
oaire.citation.volume10pt_PT
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT

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