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Behavioral and Emotional Problems in Early-Treated Brazilian Children and Adolescents with Phenylketonuria

BACKGROUND: Phenylketonuria (PKU) is an inborn error of metabolism caused by mutations in the phenylalanine hydroxylase (PAH) gene. When untreated, PKU leads to a significant intellectual deficiency. Although early initiation of dietary therapy allows normal cognitive development, low adherence to t...

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Autores principales: Neto, Eduardo Vieira, de Souza Maia Filho, Heber, Monteiro, Claudia Braga, de Mattos Carvalho, Lilian, da Cruz, Thuane Silva, de Barros, Belisia Vasconcelos, Ribeiro, Márcia Gonçalves
Formato: Online Artículo Texto
Lenguaje:English
Publicado: International Scientific Literature, Inc. 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6354646/
https://www.ncbi.nlm.nih.gov/pubmed/30375370
http://dx.doi.org/10.12659/MSM.909146
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author Neto, Eduardo Vieira
de Souza Maia Filho, Heber
Monteiro, Claudia Braga
de Mattos Carvalho, Lilian
da Cruz, Thuane Silva
de Barros, Belisia Vasconcelos
Ribeiro, Márcia Gonçalves
author_facet Neto, Eduardo Vieira
de Souza Maia Filho, Heber
Monteiro, Claudia Braga
de Mattos Carvalho, Lilian
da Cruz, Thuane Silva
de Barros, Belisia Vasconcelos
Ribeiro, Márcia Gonçalves
author_sort Neto, Eduardo Vieira
collection PubMed
description BACKGROUND: Phenylketonuria (PKU) is an inborn error of metabolism caused by mutations in the phenylalanine hydroxylase (PAH) gene. When untreated, PKU leads to a significant intellectual deficiency. Although early initiation of dietary therapy allows normal cognitive development, low adherence to treatment may result in neuropsychological deficits, including attention problems. This study was performed to evaluate emotional and behavioral problems in early-treated children and adolescents with PKU using the Child Behavior Checklist – CBCL/6–18 answered by parents. MATERIAL/METHODS: The study included 36 PKU patients. The mean scores of internalizing, externalizing, and total problems, syndrome scales, and DSM-IV-oriented scales of patients were compared with those of controls. An analysis to evaluate the importance of adherence to treatment and presence of intellectual disability was also performed. RESULTS: There were no significant differences between patients and controls for almost all CBCL/6–18 scales, with the exception of the Attention Problem Scale – CBCL-APS. The mean (±SD) of the CBCL-APS scores of patients (7.86±5.33) was considerably higher than the mean of the controls (6.07±4.37; p=0.016), but not different from the mean of a matched control subsample (6.69±4.46; p=0.316). The difference between the mean of the scores of DSM-IV/ADHD scale of patients (6.72±4.07) and controls (5.73±3.56; p=0.102) was not significant. Non-adherence to treatment and intellectual disability had a negative impact on both CBCL-APS and DSM-IV/ADHD scale scores. CONCLUSIONS: Our findings indicate a significant prevalence of parents’ complaints of attention problems and hyperactivity in non-adherent to treatment and intellectually low performing patients with PKU.
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spelling pubmed-63546462019-02-15 Behavioral and Emotional Problems in Early-Treated Brazilian Children and Adolescents with Phenylketonuria Neto, Eduardo Vieira de Souza Maia Filho, Heber Monteiro, Claudia Braga de Mattos Carvalho, Lilian da Cruz, Thuane Silva de Barros, Belisia Vasconcelos Ribeiro, Márcia Gonçalves Med Sci Monit Clinical Research BACKGROUND: Phenylketonuria (PKU) is an inborn error of metabolism caused by mutations in the phenylalanine hydroxylase (PAH) gene. When untreated, PKU leads to a significant intellectual deficiency. Although early initiation of dietary therapy allows normal cognitive development, low adherence to treatment may result in neuropsychological deficits, including attention problems. This study was performed to evaluate emotional and behavioral problems in early-treated children and adolescents with PKU using the Child Behavior Checklist – CBCL/6–18 answered by parents. MATERIAL/METHODS: The study included 36 PKU patients. The mean scores of internalizing, externalizing, and total problems, syndrome scales, and DSM-IV-oriented scales of patients were compared with those of controls. An analysis to evaluate the importance of adherence to treatment and presence of intellectual disability was also performed. RESULTS: There were no significant differences between patients and controls for almost all CBCL/6–18 scales, with the exception of the Attention Problem Scale – CBCL-APS. The mean (±SD) of the CBCL-APS scores of patients (7.86±5.33) was considerably higher than the mean of the controls (6.07±4.37; p=0.016), but not different from the mean of a matched control subsample (6.69±4.46; p=0.316). The difference between the mean of the scores of DSM-IV/ADHD scale of patients (6.72±4.07) and controls (5.73±3.56; p=0.102) was not significant. Non-adherence to treatment and intellectual disability had a negative impact on both CBCL-APS and DSM-IV/ADHD scale scores. CONCLUSIONS: Our findings indicate a significant prevalence of parents’ complaints of attention problems and hyperactivity in non-adherent to treatment and intellectually low performing patients with PKU. International Scientific Literature, Inc. 2018-10-30 /pmc/articles/PMC6354646/ /pubmed/30375370 http://dx.doi.org/10.12659/MSM.909146 Text en © Med Sci Monit, 2018 This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/) )
spellingShingle Clinical Research
Neto, Eduardo Vieira
de Souza Maia Filho, Heber
Monteiro, Claudia Braga
de Mattos Carvalho, Lilian
da Cruz, Thuane Silva
de Barros, Belisia Vasconcelos
Ribeiro, Márcia Gonçalves
Behavioral and Emotional Problems in Early-Treated Brazilian Children and Adolescents with Phenylketonuria
title Behavioral and Emotional Problems in Early-Treated Brazilian Children and Adolescents with Phenylketonuria
title_full Behavioral and Emotional Problems in Early-Treated Brazilian Children and Adolescents with Phenylketonuria
title_fullStr Behavioral and Emotional Problems in Early-Treated Brazilian Children and Adolescents with Phenylketonuria
title_full_unstemmed Behavioral and Emotional Problems in Early-Treated Brazilian Children and Adolescents with Phenylketonuria
title_short Behavioral and Emotional Problems in Early-Treated Brazilian Children and Adolescents with Phenylketonuria
title_sort behavioral and emotional problems in early-treated brazilian children and adolescents with phenylketonuria
topic Clinical Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6354646/
https://www.ncbi.nlm.nih.gov/pubmed/30375370
http://dx.doi.org/10.12659/MSM.909146
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