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The Effect of Glycomacropeptide versus Amino Acids on Phenylalanine and Tyrosine Variability over 24 Hours in Children with PKU: A Randomized Controlled Trial

2019-02-28Journal article Open access
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dc.contributor.authorDaly, Anne
dc.contributor.authorEvans, Sharon
dc.contributor.authorChahal, Satnam
dc.contributor.authorSantra, Saikat
dc.contributor.authorPinto, Alex
dc.contributor.authorGingell, Cerys
dc.contributor.authorRocha, Júlio César
dc.contributor.authorvan Spronsen, Francjan
dc.contributor.authorJackson, Richard
dc.contributor.authorMacDonald, Anita
dc.date.accessioned2020-07-14T14:36:46Z
dc.date.available2020-07-14T14:36:46Z
dc.date.issued2019-02-28
dc.description.abstractIntroduction: In phenylketonuria (PKU), evidence suggests that casein glycomacropeptide supplemented with rate-limiting amino acids (CGMP-AA) is associated with better protein utilisation and less blood phenylalanine (Phe) variability. Aim: To study the impact of CGMP-AA on blood Phe variability using 3 different dietary regimens in children with PKU. Methods: This was a 6-week randomised controlled cross-over study comparing CGMP-AA vs. Phe-free l-amino acids (l-AA) assessing blood Phe and tyrosine (Tyr) variability over 24 h in 19 children (7 boys) with PKU, with a median age of 10 years (6⁻16). Subjects were randomised to 3 dietary regimens: (1) R1, CGMP-AA and usual dietary Phe (CGMP + Phe); (2) R2, CGMP-AA - Phe content of CGMP-AA from usual diet (CGMP - Phe); and (3) R3, l-AA and usual dietary Phe. Each regimen was administered for 14 days. Over the last 48 h on days 13 and 14, blood spots were collected every 4 h at 08 h, 12 h, 16 h, 20 h, 24 h, and 04 h. Isocaloric intake and the same meal plan and protein substitute dosage at standardised times were maintained when blood spots were collected. Results: Eighteen children completed the study. Median Phe concentrations over 24 h for each group were (range) R1, 290 (30⁻580), R2, 220 (10⁻670), R3, 165 (10⁻640) μmol/L. R1 vs. R2 and R1 vs. R3 p < 0.0001; R2 vs. R3 p = 0.0009. There was a significant difference in median Phe at each time point between R1 vs. R2, p = 0.0027 and R1 vs. R3, p < 0.0001, but not between any time points for R2 vs. R3. Tyr was significantly higher in both R1 and R2 [70 (20⁻240 μmol/L] compared to R3 [60 (10⁻200) μmol/L]. In children < 12 years, blood Phe remained in the target range (120⁻360 μmol/L), over 24 h, for 75% of the time in R1, 72% in R2 and 64% in R3; for children aged ≥ 12 years, blood Phe was in target range (120⁻600 μmol/L) in R1 and R2 for 100% of the time, but 64% in R3. Conclusions: The residual Phe in CGMP-AA increased blood Phe concentration in children. CGMP-AA appears to give less blood Phe variability compared to l-AA, but this effect may be masked by the increased blood Phe concentrations associated with its Phe contribution. Reducing dietary Phe intake to compensate for CGMP-AA Phe content may help.pt_PT
dc.description.sponsorshipThis research study was partly funded by Birmingham Children’s Hospital Research and Development Department and Vitaflo International Ltd., Liverpoolpt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationDaly A, Evans S, Chahal S, et al. The Effect of Glycomacropeptide versus Amino Acids on Phenylalanine and Tyrosine Variability over 24 Hours in Children with PKU: A Randomized Controlled Trial. Nutrients. 2019;11(3):520. Published 2019 Feb 28. doi:10.3390/nu11030520pt_PT
dc.identifier.doi10.3390/nu11030520pt_PT
dc.identifier.issn2072-6643
dc.identifier.issn2072-6643
dc.identifier.urihttp://hdl.handle.net/10400.16/2425
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherMDPIpt_PT
dc.relation.publisherversionhttps://www.mdpi.com/2072-6643/11/3/520pt_PT
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/pt_PT
dc.subjectglycomacropeptidept_PT
dc.subjectphenylalaninept_PT
dc.subjectphenylketonuriapt_PT
dc.subjectphenylalanine variabilitypt_PT
dc.subjectamino acidspt_PT
dc.subjecttyrosinept_PT
dc.titleThe Effect of Glycomacropeptide versus Amino Acids on Phenylalanine and Tyrosine Variability over 24 Hours in Children with PKU: A Randomized Controlled Trialpt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlaceSwitzerlandpt_PT
oaire.citation.issue3pt_PT
oaire.citation.startPage520pt_PT
oaire.citation.titleNutrientspt_PT
oaire.citation.volume11pt_PT
person.familyNameEvans
person.familyNameRocha
person.givenNameSharon
person.givenNameJúlio César
person.identifier.ciencia-idED11-107D-ABFB
person.identifier.orcid0000-0002-7654-3621
person.identifier.orcid0000-0002-4977-8345
person.identifier.ridK-5399-2013
person.identifier.scopus-author-id35196853000
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublicationdc274f7c-9873-44a1-9c19-222f9a1605c8
relation.isAuthorOfPublication2d020da8-ada9-4090-bef1-0ceae77f3fda
relation.isAuthorOfPublication.latestForDiscoverydc274f7c-9873-44a1-9c19-222f9a1605c8

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