Behavioral, social and school functioning in children with Pompe disease
| Autor: | Stephanie Austin, Mihaela Stefanescu, Gail A. Spiridigliozzi, Priya S. Kishnani, Aditi Korlimarla |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
Conners-3
Conners 3rd Edition Parent CBCL Disease Screening for behavior problems Standard score Emotional functioning 03 medical and health sciences 0302 clinical medicine Endocrinology CBCL Child Behavior Checklist Social functioning ADHD attention-deficit/hyperactivity disorder Genetics IEP Individualized Education Program Child Behavior Checklist lcsh:QH301-705.5 Molecular Biology Behavior checklists lcsh:R5-920 0303 health sciences Descriptive statistics GAA acid alpha-glucosidase 030305 genetics & heredity IPD infantile Pompe disease Enzyme replacement therapy Checklist School functioning ERT enzyme replacement therapy lcsh:Biology (General) Children with Pompe disease lcsh:Medicine (General) LOPD late-onset Pompe disease SD standard deviation Psychology 030217 neurology & neurosurgery Research Paper BRIEF2 Behavior Rating Inventory of Executive Function-Second Edition Clinical psychology |
| Zdroj: | Molecular Genetics and Metabolism Reports Molecular Genetics and Metabolism Reports, Vol 25, Iss, Pp 100635-(2020) |
| ISSN: | 2214-4269 |
| DOI: | 10.1016/j.ymgmr.2020.100635 |
| Popis: | Purpose To improve our understanding of the behavioral, social, and emotional functioning of children and adolescents with Pompe disease. Method Parents/guardians of 21 children (age 5-18y) with infantile (IPD) or late-onset (LOPD) Pompe disease on long-term enzyme replacement therapy completed three standardized checklists regarding their child's behavior: the Child Behavior Checklist (CBCL), Conners 3 Parent (Conners-3), Behavior Rating Inventory of Executive Function-2 (BRIEF2), and a survey of their child's educational services. Results Descriptive statistics were used to summarize the findings for each behavior checklist. Age standard scores from each checklist were reported for the IPD (n = 17, 9 females, mean age = 9y, 4 mo; SD = 3y, 8mo) and LOPD (n = 4, 1 female; mean = 11y, 2mo; SD = 2y, 1mo) groups. The majority of children with Pompe exhibited age-appropriate behavior and emotional functioning on these standardized checklists. However, negative mood symptoms, learning problems, decreased participation in structured social activities, and attentional difficulties were more frequently reported in children with IPD in comparison to same-aged peers. Parents of children with LOPD reported fewer problematic behaviors but endorsed negative mood symptoms and difficulties with peer relations. Most children received accommodations in regular education classrooms at school. Conclusions These standardized behavior checklists are useful screening tools for the early identification and treatment of behavior, emotional, and social concerns in children with Pompe disease. Highlights • Parents of children with Pompe disease completed standardized behavior checklists. • Most children with Pompe exhibited age appropriate behavior and emotional functioning. • Negative mood, learning, and attentional problems reported in infantile Pompe group. • Negative mood and concerns about peer interactions reported in late-onset Pompe group. • Most children with Pompe attend and succeed at school, with classroom accommodations. |
| Databáze: | OpenAIRE |
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