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 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.

Adolescents Emotional State and Behavioral and Dietary Habit Changes during Isolation Due to the COVID-19 Pandemic.

INTRODUCTION: The coronavirus disease 2019 (COVID-19) was recognized as a pandemic by the World Health Organization on March 11, 2020. As an infectious disease with no specific treatment, several measures have been established to minimize the outbreak of this disease, including social isolation. OBJECTIVE: To evaluate the behavior of adolescents during the isolation period. METHODS: This is a cross-sectional descriptive study conducted at the Adolescent Health Studies Center. Data were obtained from a questionnaire prepared on Google Forms, sent by a multiplatform instant messaging application, and analyzed using the Stata 14 software. RESULTS: A total of 208 adolescents with a mean age of 15.3 years (SD ± 1.8) answered the questionnaire, 57.7% were female. About 93.3% of adolescents said they were in isolation with a changed routine, 67.3% increased their food consumption, 86.5% were inactive, and 58.7% reported screen time over 8 h/d. There was an association between anxiety and increased food consumption (odds ratio: 3.9; CI 95% 2-7.5; p = 0.00), sleeping difficulty (odds ratio: 3.6; CI 95% 1.9-6.8; p = 0.00), and conflicting family relationship (odds ratio: 5.7; CI 95% 1.6-7.8; p = 0.01). CONCLUSION: The study revealed that social isolation due to an infectious disease was associated with several effects on the behavior and eating behavior of adolescents, which need to be acknowledged to encourage them to lead a healthy lifestyle after the COVID-19 confinement.