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30 October 2018 : Clinical Research  

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

Eduardo Vieira Neto12ABCDEF*, Heber de Souza Maia Filho3ACDE, Claudia Braga Monteiro4ACD, Lilian de Mattos Carvalho5BD, Thuane Silva da Cruz1BDF, Belisia Vasconcelos de Barros1BDF, Marcia Gonçalves Ribeiro1ABCDEF

DOI: 10.12659/MSM.909146

Med Sci Monit 2018; 24: CLR7759-7769


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 AND 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.

Keywords: Attention, Child Behavior, Cognition, Patient Compliance, Phenylketonurias



12 September 2022 : Editorial  

Editorial: Treatment with Dual Incretin Receptor Agonists to Maintain Normal Glucose Levels May Also Maintain Normal Weight and Control Metabolic Dysfunction-Associated Fatty Liver Disease (MAFLD)

Ana Luisa Ordóñez-Vázquez, Sofía Murúa Beltrán-Gall, Shreya C. Pal, Nahum Méndez-Sánchez
Liver Research Unit, Medica Sur Clinic Foundation, Mexico City, Mexico

DOI: 10.12659/MSM.938365

Med Sci Monit 2022; 28:e938365


30 September 2022 : Editorial  

Editorial: Rebound COVID-19 and Cessation of Antiviral Treatment for SARS-CoV-2 with Paxlovid and Molnupiravir

Med Sci Monit In Press; DOI: 10.12659/MSM.938532  

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Med Sci Monit In Press; DOI: 10.12659/MSM.938141  

12 September 2022 : Clinical Research  

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Med Sci Monit In Press; DOI: 10.12659/MSM.937741  

In Press

30 Sep 2022 : Editorial  

Editorial: Rebound COVID-19 and Cessation of Antiviral Treatment for SARS-CoV-2 with Paxlovid and Molnupiravir

Med Sci Monit In Press; DOI: 10.12659/MSM.938532  

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Med Sci Monit In Press; DOI: 10.12659/MSM.938091  

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Med Sci Monit In Press; DOI: 10.12659/MSM.937862  

29 Sep 2022 : Animal Research  

Effect of Antilogous Platelet-Rich Plasma on the Revascularization of Rabbit Prefabricated Flap

Med Sci Monit In Press; DOI: 10.12659/MSM.937718  

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Medical Science Monitor eISSN: 1643-3750
Medical Science Monitor eISSN: 1643-3750