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

Detalhes bibliográficos
Autor(a) principal: Ilgaz, Fatma
Data de Publicação: 2019
Outros Autores: Pinto, Alex, Gökmen-Özel, Hülya, Rocha, Júlio César, van Dam, Esther, Ahring, Kirsten, Bélanger-Quintana, Amaya, Dokoupil, Katharina, Karabulut, Erdem, MacDonald, Anita
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10400.16/2426
Resumo: There 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.
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spelling Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysisphenylketonuriahyperphenylalaninemiagrowthanthropometricsweightheightz-scoresThere 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.MDPIRepositório Científico do Centro Hospitalar Universitário de Santo AntónioIlgaz, FatmaPinto, AlexGökmen-Özel, HülyaRocha, Júlio Césarvan Dam, EstherAhring, KirstenBélanger-Quintana, AmayaDokoupil, KatharinaKarabulut, ErdemMacDonald, Anita2020-07-14T14:43:18Z2019-09-032019-09-03T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.16/2426engIlgaz 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/nu110920702072-664310.3390/nu11092070info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-10-20T11:00:40Zoai:repositorio.chporto.pt:10400.16/2426Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T20:38:37.335906Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis
title Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis
spellingShingle Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis
Ilgaz, Fatma
phenylketonuria
hyperphenylalaninemia
growth
anthropometrics
weight
height
z-scores
title_short Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis
title_full Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis
title_fullStr Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis
title_full_unstemmed Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis
title_sort Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis
author Ilgaz, Fatma
author_facet Ilgaz, Fatma
Pinto, Alex
Gökmen-Özel, Hülya
Rocha, Júlio César
van Dam, Esther
Ahring, Kirsten
Bélanger-Quintana, Amaya
Dokoupil, Katharina
Karabulut, Erdem
MacDonald, Anita
author_role author
author2 Pinto, Alex
Gökmen-Özel, Hülya
Rocha, Júlio César
van Dam, Esther
Ahring, Kirsten
Bélanger-Quintana, Amaya
Dokoupil, Katharina
Karabulut, Erdem
MacDonald, Anita
author2_role author
author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Repositório Científico do Centro Hospitalar Universitário de Santo António
dc.contributor.author.fl_str_mv Ilgaz, Fatma
Pinto, Alex
Gökmen-Özel, Hülya
Rocha, Júlio César
van Dam, Esther
Ahring, Kirsten
Bélanger-Quintana, Amaya
Dokoupil, Katharina
Karabulut, Erdem
MacDonald, Anita
dc.subject.por.fl_str_mv phenylketonuria
hyperphenylalaninemia
growth
anthropometrics
weight
height
z-scores
topic phenylketonuria
hyperphenylalaninemia
growth
anthropometrics
weight
height
z-scores
description There 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.
publishDate 2019
dc.date.none.fl_str_mv 2019-09-03
2019-09-03T00:00:00Z
2020-07-14T14:43:18Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://hdl.handle.net/10400.16/2426
url http://hdl.handle.net/10400.16/2426
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv Ilgaz 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/nu11092070
2072-6643
10.3390/nu11092070
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv MDPI
publisher.none.fl_str_mv MDPI
dc.source.none.fl_str_mv reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
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reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
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