Evaluation of effectiveness and outcome of PKU screening and management in the State of Sergipe, Brazil
Autor(a) principal: | |
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Data de Publicação: | 2014 |
Outros Autores: | , , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UFS |
Texto Completo: | https://ri.ufs.br/handle/riufs/1752 |
Resumo: | Objectives: Phenylketonuria (PKU) was the first inherited metabolic disease known to cause mental retardation for which a newborn screening program (NBS) was developed. The objective of this study was to evaluate the effectiveness of PKU NBS and the management of cases in the northeastern Brazilian state of Sergipe (SE). Materials and methods: We reviewed the phenylalanine concentrations in filter-paper collected from the heel (PKUneo) of 43,449 newborns; blood concentrations obtained by venipuncture in the subjects with abnormal PKUneo; the children’s age at several phases of the program, the incidence of the disease from January 2007 to June 2008; and metabolic control of the patients. Results: The coverage of NBS/SE was 78.93%. The children’s age was 10 ± 7 days at PKUneo collection. Twelve children were recalled based on the PKUneo cutoff value at 28 ± 13 days. From these, the concentrations of phenylalanine collected by venipuncture were normal in five children. The incidence of hyperphenylalaninemia was 1/43,449, and of PKU was 1/8,690 (5 cases). One suspected subject died. Another death occurred in the cohort, in a confirmed PKU case. PKU treatment began within 51 ± 12 days of life. In the four patients under dietary phenylalanine restriction, metabolic control was often difficult. Conclusions: PKU NBS/SE has satisfactory coverage and adequate cutoff for recalling patients and diagnosis, but the onset of treatment is delayed, and follow-up metabolic control is frequently inadequate._________________________________________________________________________________________ RESUMO: Objetivos: A fenilcetonúria (PKU) foi a primeira causa metabólica hereditária de retardamento mental para a qual foi desenvolvido um programa de triagem em recém-nascidos (NBS). O objetivo deste estudo foi avaliar a eficácia do NBS para a PKU e o manejo dos casos em Sergipe (SE), Brasil. Materiais e métodos: Revisamos as concentrações de fenilalanina no filtro de papel coletado do calcanhar (PKUneo) de 43.449 recém-nascidos, suas concentrações de sangue obtidas por punção venosa em indivíduos com PKUneo anormal, a idade das crianças em diversas fases do programa, a incidência da doença no período de janeiro de 2007 a junho de 2008 e o controle metabólico dos pacientes. Resultados: A cobertura da NBS/SE foi de 78,93%. A idade das crianças era de 10 ± 7 dias na coleta de PKUneo. Doze crianças foram reconvocadas com base no ponto de corte de PKUneo aos 28 ± 13 dias de idade. Destas, as concentrações de fenilalanina por venipunctura foram normais em cinco. A incidência da hiperfenilalaninemia foi 1/43.449 e de PKU foi 1/8.690 (5 casos), e um indivíduo suspeito foi a óbito. Outro óbito ocorreu na coorte em um caso de PKU confirmado. O tratamento para a PKU começou com 51 ± 12 dias. Nos quatro pacientes sob restrição de fenilalanina alimentar, o controle metabólico foi frequentemente difícil. Conclusões: PKU NBS/SE apresenta uma cobertura satisfatória e ponto de corte adequado para reconvocação e diagnóstico, mas o início do tratamento é atrasado e o controle no seguimento é frequentemente inadequado. |
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Ramalho, Antônio Roberto de OliveiraRamalho, Roberto Jose RabeloOliveira, Carla Raquel PereiraMagalhães, Marta Maria Galvão de SousaSantos, Elenilde GomesMatos, Diana O.Oliveira, Mario C. P.Oliveira, André L. P.Oliveira, Manuel Herminio de AguiarSarmento, Polyana Maria Palmeira2016-04-11T12:06:45Z2016-04-11T12:06:45Z2014-02RAMALHO, A. R. O. et al. Evaluation of effectiveness and outcome of PKU screening and management in the State of Sergipe, Brazil. Arquivos Brasileiros de Endocrinologia & Metabologia, São Paulo, v. 58, n. 1, fev. 2014. Disponível em: <http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27302014000100062&lng=en&nrm=iso&tlng=en>. Acesso em: 11 abr. 2016.1677-9487https://ri.ufs.br/handle/riufs/1752Creative Commons Attribution Non-Commercial LicenseObjectives: Phenylketonuria (PKU) was the first inherited metabolic disease known to cause mental retardation for which a newborn screening program (NBS) was developed. The objective of this study was to evaluate the effectiveness of PKU NBS and the management of cases in the northeastern Brazilian state of Sergipe (SE). Materials and methods: We reviewed the phenylalanine concentrations in filter-paper collected from the heel (PKUneo) of 43,449 newborns; blood concentrations obtained by venipuncture in the subjects with abnormal PKUneo; the children’s age at several phases of the program, the incidence of the disease from January 2007 to June 2008; and metabolic control of the patients. Results: The coverage of NBS/SE was 78.93%. The children’s age was 10 ± 7 days at PKUneo collection. Twelve children were recalled based on the PKUneo cutoff value at 28 ± 13 days. From these, the concentrations of phenylalanine collected by venipuncture were normal in five children. The incidence of hyperphenylalaninemia was 1/43,449, and of PKU was 1/8,690 (5 cases). One suspected subject died. Another death occurred in the cohort, in a confirmed PKU case. PKU treatment began within 51 ± 12 days of life. In the four patients under dietary phenylalanine restriction, metabolic control was often difficult. Conclusions: PKU NBS/SE has satisfactory coverage and adequate cutoff for recalling patients and diagnosis, but the onset of treatment is delayed, and follow-up metabolic control is frequently inadequate._________________________________________________________________________________________ RESUMO: Objetivos: A fenilcetonúria (PKU) foi a primeira causa metabólica hereditária de retardamento mental para a qual foi desenvolvido um programa de triagem em recém-nascidos (NBS). O objetivo deste estudo foi avaliar a eficácia do NBS para a PKU e o manejo dos casos em Sergipe (SE), Brasil. Materiais e métodos: Revisamos as concentrações de fenilalanina no filtro de papel coletado do calcanhar (PKUneo) de 43.449 recém-nascidos, suas concentrações de sangue obtidas por punção venosa em indivíduos com PKUneo anormal, a idade das crianças em diversas fases do programa, a incidência da doença no período de janeiro de 2007 a junho de 2008 e o controle metabólico dos pacientes. Resultados: A cobertura da NBS/SE foi de 78,93%. A idade das crianças era de 10 ± 7 dias na coleta de PKUneo. Doze crianças foram reconvocadas com base no ponto de corte de PKUneo aos 28 ± 13 dias de idade. Destas, as concentrações de fenilalanina por venipunctura foram normais em cinco. A incidência da hiperfenilalaninemia foi 1/43.449 e de PKU foi 1/8.690 (5 casos), e um indivíduo suspeito foi a óbito. Outro óbito ocorreu na coorte em um caso de PKU confirmado. O tratamento para a PKU começou com 51 ± 12 dias. Nos quatro pacientes sob restrição de fenilalanina alimentar, o controle metabólico foi frequentemente difícil. Conclusões: PKU NBS/SE apresenta uma cobertura satisfatória e ponto de corte adequado para reconvocação e diagnóstico, mas o início do tratamento é atrasado e o controle no seguimento é frequentemente inadequado.Sociedade Brasileira de Endocrinologia e MetabologiaPhenylketonuriaScreening neonatalPhenylalanineCoverageIncidenceFenilcetonúriaTriagem neonatalFenilalaninaCoberturaIncidênciaEvaluation of effectiveness and outcome of PKU screening and management in the State of Sergipe, BrazilAvaliação da eficiência e desfecho da triagem e manejo da PKU no Estado de Sergipe, Brasilinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleengreponame:Repositório Institucional da UFSinstname:Universidade Federal de Sergipe (UFS)instacron:UFSinfo:eu-repo/semantics/openAccessTHUMBNAILPKUManagementSergipe.pdf.jpgPKUManagementSergipe.pdf.jpgGenerated Thumbnailimage/jpeg1688https://ri.ufs.br/jspui/bitstream/riufs/1752/4/PKUManagementSergipe.pdf.jpgcc661030065fe475e02b78609825457aMD54ORIGINALPKUManagementSergipe.pdfPKUManagementSergipe.pdfapplication/pdf104729https://ri.ufs.br/jspui/bitstream/riufs/1752/1/PKUManagementSergipe.pdfe02a75c34e7e4e61c39c1ae46daf81eeMD51LICENSElicense.txtlicense.txttext/plain; charset=utf-81748https://ri.ufs.br/jspui/bitstream/riufs/1752/2/license.txt8a4605be74aa9ea9d79846c1fba20a33MD52TEXTPKUManagementSergipe.pdf.txtPKUManagementSergipe.pdf.txtExtracted texttext/plain26540https://ri.ufs.br/jspui/bitstream/riufs/1752/3/PKUManagementSergipe.pdf.txtfa3e6a7e1e807f81595d55549f7a485aMD53riufs/17522016-04-12 02:00:13.734oai:ufs.br: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Repositório InstitucionalPUBhttps://ri.ufs.br/oai/requestrepositorio@academico.ufs.bropendoar:2016-04-12T05:00:13Repositório Institucional da UFS - Universidade Federal de Sergipe (UFS)false |
dc.title.pt_BR.fl_str_mv |
Evaluation of effectiveness and outcome of PKU screening and management in the State of Sergipe, Brazil |
dc.title.alternative.pt_BR.fl_str_mv |
Avaliação da eficiência e desfecho da triagem e manejo da PKU no Estado de Sergipe, Brasil |
title |
Evaluation of effectiveness and outcome of PKU screening and management in the State of Sergipe, Brazil |
spellingShingle |
Evaluation of effectiveness and outcome of PKU screening and management in the State of Sergipe, Brazil Ramalho, Antônio Roberto de Oliveira Phenylketonuria Screening neonatal Phenylalanine Coverage Incidence Fenilcetonúria Triagem neonatal Fenilalanina Cobertura Incidência |
title_short |
Evaluation of effectiveness and outcome of PKU screening and management in the State of Sergipe, Brazil |
title_full |
Evaluation of effectiveness and outcome of PKU screening and management in the State of Sergipe, Brazil |
title_fullStr |
Evaluation of effectiveness and outcome of PKU screening and management in the State of Sergipe, Brazil |
title_full_unstemmed |
Evaluation of effectiveness and outcome of PKU screening and management in the State of Sergipe, Brazil |
title_sort |
Evaluation of effectiveness and outcome of PKU screening and management in the State of Sergipe, Brazil |
author |
Ramalho, Antônio Roberto de Oliveira |
author_facet |
Ramalho, Antônio Roberto de Oliveira Ramalho, Roberto Jose Rabelo Oliveira, Carla Raquel Pereira Magalhães, Marta Maria Galvão de Sousa Santos, Elenilde Gomes Matos, Diana O. Oliveira, Mario C. P. Oliveira, André L. P. Oliveira, Manuel Herminio de Aguiar Sarmento, Polyana Maria Palmeira |
author_role |
author |
author2 |
Ramalho, Roberto Jose Rabelo Oliveira, Carla Raquel Pereira Magalhães, Marta Maria Galvão de Sousa Santos, Elenilde Gomes Matos, Diana O. Oliveira, Mario C. P. Oliveira, André L. P. Oliveira, Manuel Herminio de Aguiar Sarmento, Polyana Maria Palmeira |
author2_role |
author author author author author author author author author |
dc.contributor.author.fl_str_mv |
Ramalho, Antônio Roberto de Oliveira Ramalho, Roberto Jose Rabelo Oliveira, Carla Raquel Pereira Magalhães, Marta Maria Galvão de Sousa Santos, Elenilde Gomes Matos, Diana O. Oliveira, Mario C. P. Oliveira, André L. P. Oliveira, Manuel Herminio de Aguiar Sarmento, Polyana Maria Palmeira |
dc.subject.por.fl_str_mv |
Phenylketonuria Screening neonatal Phenylalanine Coverage Incidence Fenilcetonúria Triagem neonatal Fenilalanina Cobertura Incidência |
topic |
Phenylketonuria Screening neonatal Phenylalanine Coverage Incidence Fenilcetonúria Triagem neonatal Fenilalanina Cobertura Incidência |
description |
Objectives: Phenylketonuria (PKU) was the first inherited metabolic disease known to cause mental retardation for which a newborn screening program (NBS) was developed. The objective of this study was to evaluate the effectiveness of PKU NBS and the management of cases in the northeastern Brazilian state of Sergipe (SE). Materials and methods: We reviewed the phenylalanine concentrations in filter-paper collected from the heel (PKUneo) of 43,449 newborns; blood concentrations obtained by venipuncture in the subjects with abnormal PKUneo; the children’s age at several phases of the program, the incidence of the disease from January 2007 to June 2008; and metabolic control of the patients. Results: The coverage of NBS/SE was 78.93%. The children’s age was 10 ± 7 days at PKUneo collection. Twelve children were recalled based on the PKUneo cutoff value at 28 ± 13 days. From these, the concentrations of phenylalanine collected by venipuncture were normal in five children. The incidence of hyperphenylalaninemia was 1/43,449, and of PKU was 1/8,690 (5 cases). One suspected subject died. Another death occurred in the cohort, in a confirmed PKU case. PKU treatment began within 51 ± 12 days of life. In the four patients under dietary phenylalanine restriction, metabolic control was often difficult. Conclusions: PKU NBS/SE has satisfactory coverage and adequate cutoff for recalling patients and diagnosis, but the onset of treatment is delayed, and follow-up metabolic control is frequently inadequate._________________________________________________________________________________________ RESUMO: Objetivos: A fenilcetonúria (PKU) foi a primeira causa metabólica hereditária de retardamento mental para a qual foi desenvolvido um programa de triagem em recém-nascidos (NBS). O objetivo deste estudo foi avaliar a eficácia do NBS para a PKU e o manejo dos casos em Sergipe (SE), Brasil. Materiais e métodos: Revisamos as concentrações de fenilalanina no filtro de papel coletado do calcanhar (PKUneo) de 43.449 recém-nascidos, suas concentrações de sangue obtidas por punção venosa em indivíduos com PKUneo anormal, a idade das crianças em diversas fases do programa, a incidência da doença no período de janeiro de 2007 a junho de 2008 e o controle metabólico dos pacientes. Resultados: A cobertura da NBS/SE foi de 78,93%. A idade das crianças era de 10 ± 7 dias na coleta de PKUneo. Doze crianças foram reconvocadas com base no ponto de corte de PKUneo aos 28 ± 13 dias de idade. Destas, as concentrações de fenilalanina por venipunctura foram normais em cinco. A incidência da hiperfenilalaninemia foi 1/43.449 e de PKU foi 1/8.690 (5 casos), e um indivíduo suspeito foi a óbito. Outro óbito ocorreu na coorte em um caso de PKU confirmado. O tratamento para a PKU começou com 51 ± 12 dias. Nos quatro pacientes sob restrição de fenilalanina alimentar, o controle metabólico foi frequentemente difícil. Conclusões: PKU NBS/SE apresenta uma cobertura satisfatória e ponto de corte adequado para reconvocação e diagnóstico, mas o início do tratamento é atrasado e o controle no seguimento é frequentemente inadequado. |
publishDate |
2014 |
dc.date.issued.fl_str_mv |
2014-02 |
dc.date.accessioned.fl_str_mv |
2016-04-11T12:06:45Z |
dc.date.available.fl_str_mv |
2016-04-11T12:06:45Z |
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.citation.fl_str_mv |
RAMALHO, A. R. O. et al. Evaluation of effectiveness and outcome of PKU screening and management in the State of Sergipe, Brazil. Arquivos Brasileiros de Endocrinologia & Metabologia, São Paulo, v. 58, n. 1, fev. 2014. Disponível em: <http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27302014000100062&lng=en&nrm=iso&tlng=en>. Acesso em: 11 abr. 2016. |
dc.identifier.uri.fl_str_mv |
https://ri.ufs.br/handle/riufs/1752 |
dc.identifier.issn.none.fl_str_mv |
1677-9487 |
dc.identifier.license.pt_BR.fl_str_mv |
Creative Commons Attribution Non-Commercial License |
identifier_str_mv |
RAMALHO, A. R. O. et al. Evaluation of effectiveness and outcome of PKU screening and management in the State of Sergipe, Brazil. Arquivos Brasileiros de Endocrinologia & Metabologia, São Paulo, v. 58, n. 1, fev. 2014. Disponível em: <http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27302014000100062&lng=en&nrm=iso&tlng=en>. Acesso em: 11 abr. 2016. 1677-9487 Creative Commons Attribution Non-Commercial License |
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https://ri.ufs.br/handle/riufs/1752 |
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eng |
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openAccess |
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Sociedade Brasileira de Endocrinologia e Metabologia |
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Sociedade Brasileira de Endocrinologia e Metabologia |
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