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Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis

2019-09-03Journal article Open access
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dc.contributor.authorIlgaz, Fatma
dc.contributor.authorPinto, Alex
dc.contributor.authorGökmen-Özel, Hülya
dc.contributor.authorRocha, Júlio César
dc.contributor.authorvan Dam, Esther
dc.contributor.authorAhring, Kirsten
dc.contributor.authorBélanger-Quintana, Amaya
dc.contributor.authorDokoupil, Katharina
dc.contributor.authorKarabulut, Erdem
dc.contributor.authorMacDonald, Anita
dc.date.accessioned2020-07-14T14:43:18Z
dc.date.available2020-07-14T14:43:18Z
dc.date.issued2019-09-03
dc.description.abstractThere is an ongoing debate regarding the impact of phenylketonuria (PKU) and its treatment on growth. To date, evidence from studies is inconsistent, and data on the whole developmental period is limited. The primary aim of this systematic review was to investigate the effects of a phenylalanine (Phe)-restricted diet on long-term growth in patients with PKU. Four electronic databases were searched for articles published until September 2018. A total of 887 results were found, but only 13 articles met eligibility criteria. Only three studies had an adequate methodology for meta-analysis. Although the results indicate normal growth at birth and during infancy, children with PKU were significantly shorter and had lower weight for age than reference populations during the first four years of life. Impaired linear growth was observed until the end of adolescence in PKU. In contrast, growth impairment was not reported in patients with mild hyperphenylalaninemia, not requiring dietary restriction. Current evidence indicates that even with advances in dietary treatments, "optimal" growth outcomes are not attained in PKU. The majority of studies include children born before 1990s, so further research is needed to show the effects of recent dietary practices on growth in PKU.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationIlgaz F, Pinto A, Gökmen-Özel H, et al. Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis. Nutrients. 2019;11(9):2070. Published 2019 Sep 3. doi:10.3390/nu11092070pt_PT
dc.identifier.doi10.3390/nu11092070pt_PT
dc.identifier.issn2072-6643
dc.identifier.issn2072-6643
dc.identifier.urihttp://hdl.handle.net/10400.16/2426
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherMDPIpt_PT
dc.relation.publisherversionhttps://www.mdpi.com/2072-6643/11/9/2070pt_PT
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/pt_PT
dc.subjectphenylketonuriapt_PT
dc.subjecthyperphenylalaninemiapt_PT
dc.subjectgrowthpt_PT
dc.subjectanthropometricspt_PT
dc.subjectweightpt_PT
dc.subjectheightpt_PT
dc.subjectz-scorespt_PT
dc.titleLong-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysispt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlaceSwitzerlandpt_PT
oaire.citation.issue9pt_PT
oaire.citation.startPage2070pt_PT
oaire.citation.titleNutrientspt_PT
oaire.citation.volume11pt_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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