Adherence to Treatment of Phenylketonuria: A Study in Southern Brazilian Patients
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.
Main Authors: | , , , , , |
---|---|
Format: | Digital revista |
Language: | English |
Published: |
Latin American Society Inborn Errors and Neonatal Screening (SLEIMPN); Instituto Genética para Todos (IGPT)
2015
|
Online Access: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942015000100350 |
Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
id |
oai:scielo:S2326-45942015000100350 |
---|---|
record_format |
ojs |
spelling |
oai:scielo:S2326-459420150001003502019-06-17Adherence to Treatment of Phenylketonuria: A Study in Southern Brazilian PatientsVieira,Tatiane AlvesNalin,TatiéleKrug,Bárbara CorreaBittar,Camila MatzenbacherOliveira Netto,Cristina BrinckmannSchwartz,Ida Vanessa Doederlein phenylketonuria adherence to treatment inborn errors of metabolism 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.info:eu-repo/semantics/openAccessLatin American Society Inborn Errors and Neonatal Screening (SLEIMPN); Instituto Genética para Todos (IGPT)Journal of Inborn Errors of Metabolism and Screening v.3 20152015-01-01info:eu-repo/semantics/articletext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942015000100350en10.1177/2326409815579861 |
institution |
SCIELO |
collection |
OJS |
country |
Brasil |
countrycode |
BR |
component |
Revista |
access |
En linea |
databasecode |
rev-scielo-br |
tag |
revista |
region |
America del Sur |
libraryname |
SciELO |
language |
English |
format |
Digital |
author |
Vieira,Tatiane Alves Nalin,Tatiéle Krug,Bárbara Correa Bittar,Camila Matzenbacher Oliveira Netto,Cristina Brinckmann Schwartz,Ida Vanessa Doederlein |
spellingShingle |
Vieira,Tatiane Alves Nalin,Tatiéle Krug,Bárbara Correa Bittar,Camila Matzenbacher Oliveira Netto,Cristina Brinckmann Schwartz,Ida Vanessa Doederlein Adherence to Treatment of Phenylketonuria: A Study in Southern Brazilian Patients |
author_facet |
Vieira,Tatiane Alves Nalin,Tatiéle Krug,Bárbara Correa Bittar,Camila Matzenbacher Oliveira Netto,Cristina Brinckmann Schwartz,Ida Vanessa Doederlein |
author_sort |
Vieira,Tatiane Alves |
title |
Adherence to Treatment of Phenylketonuria: A Study in Southern Brazilian Patients |
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 |
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. |
publisher |
Latin American Society Inborn Errors and Neonatal Screening (SLEIMPN); Instituto Genética para Todos (IGPT) |
publishDate |
2015 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942015000100350 |
work_keys_str_mv |
AT vieiratatianealves adherencetotreatmentofphenylketonuriaastudyinsouthernbrazilianpatients AT nalintatiele adherencetotreatmentofphenylketonuriaastudyinsouthernbrazilianpatients AT krugbarbaracorrea adherencetotreatmentofphenylketonuriaastudyinsouthernbrazilianpatients AT bittarcamilamatzenbacher adherencetotreatmentofphenylketonuriaastudyinsouthernbrazilianpatients AT oliveiranettocristinabrinckmann adherencetotreatmentofphenylketonuriaastudyinsouthernbrazilianpatients AT schwartzidavanessadoederlein adherencetotreatmentofphenylketonuriaastudyinsouthernbrazilianpatients |
_version_ |
1756441173362212864 |