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Glycomacropeptide: long-term use and impact on blood phenylalanine, growth and nutritional status in children with PKU

2019-02-15Journal article Open access
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dc.contributor.authorDaly, A
dc.contributor.authorEvans, S
dc.contributor.authorChahal, S
dc.contributor.authorSantra, S
dc.contributor.authorPinto, A
dc.contributor.authorJackson, R
dc.contributor.authorGingell, C
dc.contributor.authorRocha, Júlio César
dc.contributor.authorVan Spronsen, F J
dc.contributor.authorMacDonald, A
dc.date.accessioned2020-08-17T14:18:35Z
dc.date.available2020-08-17T14:18:35Z
dc.date.issued2019-02-15
dc.description.abstractIn phenylketonuria, casein glycomacropeptide (CGMP) requires modification with the addition of some essential and semi essential amino acids to ensure suitability as a protein substitute. The optimal amount and ratio of additional amino acids is undefined. Aim: A longitudinal, parallel, controlled study over 12 months evaluating a CGMP (CGMP-AA2) formulation compared with phenylalanine-free L-amino acid supplements (L-AA) on blood Phe, Tyr, Phe:Tyr ratio, biochemical nutritional status and growth in children with PKU. The CGMP-AA2 contained 36 mg Phe per 20 g protein equivalent. Methods: Children with PKU, with a median age of 9.2 y (5-16y) were divided into 2 groups: 29 were given CGMP-AA2, 19 remained on Phe-free L-AA. The CGMP-AA2 formula gradually replaced L-AA, providing blood Phe concentrations were maintained within target range. Median blood Phe, Tyr, Phe:Tyr ratio and anthropometry, were compared within and between the two groups at baseline, 26 and 52 weeks. Nutritional biochemistry was studied at baseline and 26 weeks only. Results: At the end of 52 weeks only 48% of subjects were able to completely use CGMP-AA2 as their single source of protein substitute. At 52 weeks CGMP-AA2 provided a median of 75% (30-100) of the total protein substitute with the remainder being given as L-AA. Within the CGMP-AA2 group, blood Phe increased significantly between baseline and 52 weeks: [baseline to 26 weeks; baseline Phe 270 μmol/L (170-430); 26 weeks, Phe 300 μmol/L (125-485) p = 0.06; baseline to 52 weeks: baseline, Phe 270 μmol/L (170-430), 52 weeks Phe 300 μmol/L (200-490), p < 0.001)]. However, there were no differences between the CGMP-AA2 and L-AA group for Phe, Tyr, Phe:Tyr ratio or anthropometry at any of the three measured time points. Within the CGMP-AA2 group only weight (p = 0.0001) and BMI z scores (p = 0.0001) increased significantly between baseline to 52 weeks. Whole blood and plasma selenium were significantly higher (whole blood selenium [p = 0.0002]; plasma selenium [p = 0.0007]) at 26 weeks in the CGMP-AA2 group compared L-AA. No differences were observed within the L-AA group for any of the nutritional markers. Conclusions: CGMP-AA increases blood Phe concentrations and so it can only be used partly to contribute to protein substitute in some children with PKU. CGMP-AA should be carefully introduced in children with PKU and close monitoring of blood Phe control is essential.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationDaly A, Evans S, Chahal S, et al. Glycomacropeptide: long-term use and impact on blood phenylalanine, growth and nutritional status in children with PKU. Orphanet J Rare Dis. 2019;14(1):44. Published 2019 Feb 15. doi:10.1186/s13023-019-1011-ypt_PT
dc.identifier.doi10.1186/s13023-019-1011-ypt_PT
dc.identifier.issn1750-1172
dc.identifier.urihttp://hdl.handle.net/10400.16/2435
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherBMCpt_PT
dc.relation.publisherversionhttps://ojrd.biomedcentral.com/track/pdf/10.1186/s13023-019-1011-ypt_PT
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/pt_PT
dc.subjectGlycomacropeptidept_PT
dc.subjectLarge neutral amino acidspt_PT
dc.subjectPhenylalaninept_PT
dc.subjectPhenylketonuriapt_PT
dc.subjectProtein substitutept_PT
dc.titleGlycomacropeptide: long-term use and impact on blood phenylalanine, growth and nutritional status in children with PKUpt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlaceEnglandpt_PT
oaire.citation.issue1pt_PT
oaire.citation.startPage44pt_PT
oaire.citation.titleOrphanet journal of rare diseasespt_PT
oaire.citation.volume14pt_PT
person.familyNameRocha
person.givenNameJúlio César
person.identifier.ciencia-idED11-107D-ABFB
person.identifier.orcid0000-0002-4977-8345
person.identifier.ridK-5399-2013
person.identifier.scopus-author-id35196853000
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublication2d020da8-ada9-4090-bef1-0ceae77f3fda
relation.isAuthorOfPublication.latestForDiscovery2d020da8-ada9-4090-bef1-0ceae77f3fda

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