Adherence to Treatment of Phenylketonuria: A Study in Southern Brazilian Patients
Autor(a) principal: | |
---|---|
Data de Publicação: | 2015 |
Outros Autores: | , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Journal of Inborn Errors of Metabolism and Screening |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942015000100350 |
Resumo: | Abstract Introduction: Phenylketonuria (PKU) is caused by the deficient activity of phenylalanine hydroxylase. Aim: To identify the factors associated with treatment adherence among patients with PKU seen at a southern Brazil reference center. Methodology: A cross-sectional, outpatient-based study including 56 patients with PKU (median age, 12 years) for whom a Phe-restrict diet plus specific metabolic formula have been prescribed. Patients were considered adherent or nonadherent depending on the median phenylalanine concentration for the 12 months prior to study and target levels of phenylalanine for each age range (<13 years = ≤360 µmol/L; ≥13 years = ≤900 µmol/L). Data were collected through a review of patient’s medical records and a set of interviews with patients and their relatives. Results: Eighteen patients (32.1%; ≥13 years, 11) were classified as treatment adherent. Among all factors analyzed, only mental retardation, living with parents, and level of maternal education were associated with adherence to treatment. Conclusion: Our findings reinforce the importance of the family as promoting factor for treatment adherence. |
id |
IGPT-1_840d263bc4fcc2d52d0288ce3521a94c |
---|---|
oai_identifier_str |
oai:scielo:S2326-45942015000100350 |
network_acronym_str |
IGPT-1 |
network_name_str |
Journal of Inborn Errors of Metabolism and Screening |
repository_id_str |
|
spelling |
Adherence to Treatment of Phenylketonuria: A Study in Southern Brazilian Patientsphenylketonuriaadherence to treatmentinborn errors of metabolismAbstract Introduction: Phenylketonuria (PKU) is caused by the deficient activity of phenylalanine hydroxylase. Aim: To identify the factors associated with treatment adherence among patients with PKU seen at a southern Brazil reference center. Methodology: A cross-sectional, outpatient-based study including 56 patients with PKU (median age, 12 years) for whom a Phe-restrict diet plus specific metabolic formula have been prescribed. Patients were considered adherent or nonadherent depending on the median phenylalanine concentration for the 12 months prior to study and target levels of phenylalanine for each age range (<13 years = ≤360 µmol/L; ≥13 years = ≤900 µmol/L). Data were collected through a review of patient’s medical records and a set of interviews with patients and their relatives. Results: Eighteen patients (32.1%; ≥13 years, 11) were classified as treatment adherent. Among all factors analyzed, only mental retardation, living with parents, and level of maternal education were associated with adherence to treatment. Conclusion: Our findings reinforce the importance of the family as promoting factor for treatment adherence.Latin American Society Inborn Errors and Neonatal Screening (SLEIMPN); Instituto Genética para Todos (IGPT)2015-01-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942015000100350Journal of Inborn Errors of Metabolism and Screening v.3 2015reponame:Journal of Inborn Errors of Metabolism and Screeninginstname:Instituto Genética para Todos (IGPT)instacron:IGPT10.1177/2326409815579861info:eu-repo/semantics/openAccessVieira,Tatiane AlvesNalin,TatiéleKrug,Bárbara CorreaBittar,Camila MatzenbacherOliveira Netto,Cristina BrinckmannSchwartz,Ida Vanessa Doederleineng2019-06-17T00:00:00Zoai:scielo:S2326-45942015000100350Revistahttp://jiems-journal.org/ONGhttps://old.scielo.br/oai/scielo-oai.phpjiems@jiems-journal.org||rgiugliani@hcpa.edu.br2326-45942326-4594opendoar:2019-06-17T00:00Journal of Inborn Errors of Metabolism and Screening - Instituto Genética para Todos (IGPT)false |
dc.title.none.fl_str_mv |
Adherence to Treatment of Phenylketonuria: A Study in Southern Brazilian Patients |
title |
Adherence to Treatment of Phenylketonuria: A Study in Southern Brazilian Patients |
spellingShingle |
Adherence to Treatment of Phenylketonuria: A Study in Southern Brazilian Patients Vieira,Tatiane Alves phenylketonuria adherence to treatment inborn errors of metabolism |
title_short |
Adherence to Treatment of Phenylketonuria: A Study in Southern Brazilian Patients |
title_full |
Adherence to Treatment of Phenylketonuria: A Study in Southern Brazilian Patients |
title_fullStr |
Adherence to Treatment of Phenylketonuria: A Study in Southern Brazilian Patients |
title_full_unstemmed |
Adherence to Treatment of Phenylketonuria: A Study in Southern Brazilian Patients |
title_sort |
Adherence to Treatment of Phenylketonuria: A Study in Southern Brazilian Patients |
author |
Vieira,Tatiane Alves |
author_facet |
Vieira,Tatiane Alves Nalin,Tatiéle Krug,Bárbara Correa Bittar,Camila Matzenbacher Oliveira Netto,Cristina Brinckmann Schwartz,Ida Vanessa Doederlein |
author_role |
author |
author2 |
Nalin,Tatiéle Krug,Bárbara Correa Bittar,Camila Matzenbacher Oliveira Netto,Cristina Brinckmann Schwartz,Ida Vanessa Doederlein |
author2_role |
author author author author author |
dc.contributor.author.fl_str_mv |
Vieira,Tatiane Alves Nalin,Tatiéle Krug,Bárbara Correa Bittar,Camila Matzenbacher Oliveira Netto,Cristina Brinckmann Schwartz,Ida Vanessa Doederlein |
dc.subject.por.fl_str_mv |
phenylketonuria adherence to treatment inborn errors of metabolism |
topic |
phenylketonuria adherence to treatment inborn errors of metabolism |
description |
Abstract Introduction: Phenylketonuria (PKU) is caused by the deficient activity of phenylalanine hydroxylase. Aim: To identify the factors associated with treatment adherence among patients with PKU seen at a southern Brazil reference center. Methodology: A cross-sectional, outpatient-based study including 56 patients with PKU (median age, 12 years) for whom a Phe-restrict diet plus specific metabolic formula have been prescribed. Patients were considered adherent or nonadherent depending on the median phenylalanine concentration for the 12 months prior to study and target levels of phenylalanine for each age range (<13 years = ≤360 µmol/L; ≥13 years = ≤900 µmol/L). Data were collected through a review of patient’s medical records and a set of interviews with patients and their relatives. Results: Eighteen patients (32.1%; ≥13 years, 11) were classified as treatment adherent. Among all factors analyzed, only mental retardation, living with parents, and level of maternal education were associated with adherence to treatment. Conclusion: Our findings reinforce the importance of the family as promoting factor for treatment adherence. |
publishDate |
2015 |
dc.date.none.fl_str_mv |
2015-01-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942015000100350 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942015000100350 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1177/2326409815579861 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Latin American Society Inborn Errors and Neonatal Screening (SLEIMPN); Instituto Genética para Todos (IGPT) |
publisher.none.fl_str_mv |
Latin American Society Inborn Errors and Neonatal Screening (SLEIMPN); Instituto Genética para Todos (IGPT) |
dc.source.none.fl_str_mv |
Journal of Inborn Errors of Metabolism and Screening v.3 2015 reponame:Journal of Inborn Errors of Metabolism and Screening instname:Instituto Genética para Todos (IGPT) instacron:IGPT |
instname_str |
Instituto Genética para Todos (IGPT) |
instacron_str |
IGPT |
institution |
IGPT |
reponame_str |
Journal of Inborn Errors of Metabolism and Screening |
collection |
Journal of Inborn Errors of Metabolism and Screening |
repository.name.fl_str_mv |
Journal of Inborn Errors of Metabolism and Screening - Instituto Genética para Todos (IGPT) |
repository.mail.fl_str_mv |
jiems@jiems-journal.org||rgiugliani@hcpa.edu.br |
_version_ |
1754732519885897728 |