Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis
Autor(a) principal: | |
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Data de Publicação: | 2019 |
Outros Autores: | , , , , , , , , |
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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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 |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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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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1799133647089958912 |