Relationships between challenging behavior and gastrointestinal symptoms, sleep problems, and internalizing and externalizing symptoms in children and adolescents with Angelman syndrome.

BACKGROUND: Angelman syndrome (AS), is a rare genetic disorder. This study investigated the relationship between parent-reported comorbid symptoms including gastrointestinal symptoms, sleep problems, internalizing symptoms, and behavior problems in children and adolescents with AS. METHOD: Parents of 98 children and adolescents with AS completed the Gastrointestinal Symptom Inventory, Children's Sleep Habits Questionnaire, Child Behavior Checklist, Social Communication Questionnaire, and the Behavior Problem Inventory-Short Form. Data were analyzed using descriptive statistics, Pearson's correlation coefficients, and hierarchical multiple regressions. RESULTS: There was a high frequency of GI symptoms (99%), sleep problems (95.9%), challenging behavior (98%), internalizing symptoms (38%), and 72.4% of children and adolescents presented with ASD symptoms. Self-injurious behavior (SIB), aggressive/destructive behavior, and the frequency of stereotyped behavior positively correlated with GI symptoms and sleep problems and it was moderately negatively associated with age. Internalizing symptoms and age were positively associated with SIB. Aggression was significantly related to gender, but not the presence of ASD symptoms. CONCLUSIONS: Findings highlight the relationships between comorbid conditions. They may lead to a deeper understanding of how comorbidities present in children and adolescents with Angelman Syndrome.

Transition Program from School to Employment in Youths with Intellectual Disability: Evaluation of the Irish Pilot Study E-IDEAS.

AIM: The purpose of this pilot was to evaluate the effectiveness of the E-IDEAS curriculum, specifically designed for workplace inclusion of youths with intellectual disabilities (ID) and aimed to transfer social, communication, independent living and employment skills. METHOD: The curriculum was attended by 5 participants, and it was provided concurrently with five different work-placements across a period of six months. Two assessment tools were used to demonstrate the acquisition of such skills and an evidence-base improvement of their quality of life. Pre and post-intervention standardized assessments were also taken for measuring the improvement in quality of life (through the San Martín Scale) and adaptive behavior (through the Vineland-II Adaptive Behavior Scale). RESULTS: The data collected showed increases in the acquisition of such skills. Evidence of maintenance and generalization were also demonstrated. CONCLUSION: Implications for practice and further research are discussed.

Relationship between parent-reported gastrointestinal symptoms, sleep problems, autism spectrum disorder symptoms, and behavior problems in children and adolescents with 22q11.2 deletion syndrome.

BACKGROUND: 22q11.2 deletion syndrome (22q) is a chromosome disorder, where a segment of chromosome 22, located at q11.2, is missing. This study aims to investigate the relationship between a number of parent-reported comorbid conditions including gastrointestinal symptoms, sleep problems, autism spectrum disorder (ASD) symptoms and behavior problems in children and adolescents with 22q deletion syndrome. METHOD: The Gastrointestinal Symptom Inventory, Children's Sleep Habits Questionnaire, Behavior Problem Inventory-Short Form and the Social Communication Questionnaire were completed by parents of 149 children and adolescents aged 3-18 years with a diagnosis of 22q. RESULTS: A series of correlations and hierarchical multiple regressions were conducted to examine the relationships between GI symptoms, sleep problems and behavior problems in children and adolescents with 22q deletion syndrome. A significant moderate relationship was found between GI symptoms and sleep problems. Gender and ASD symptoms predicted GI symptoms. Significant small relationships were found between GI symptoms and self-injurious behavior. Significant small to moderate relationships were found between sleep problems and self-injurious behavior, aggressive/destructive behavior, and sterotyped behavior. Sleep problems predicted challenging behavior. CONCLUSIONS: This research demonstrated the importance of studying the relationship between comorbidities, including gastrointestinal symptoms, sleep problems, and behavior problems and how they shape the phenotype of 22q deletion syndrome.

A Self-Assessment Framework for Inclusive Schools Supporting Assistive Technology Users.

In order to support schools to assess their performance in supporting children with disabilities in their ICT and ICT-AT needs, a self-assessment framework was developed by a task force of partners and associate partners of the ENTELIS project. The self-assessment tool aims to help educational establishments that welcome learners with disabilities to assess their current outcomes and to plan improvements in supporting these students in increasing digital literacy and developing digital skills. This includes the use of mainstream Information and Communication Technology (ICT) and specially designed digital Assistive Technologies (ICT-AT). This can only successfully happen if schools fully embrace an inclusive approach to education. In this paper the authors describe the development of the framework and the further steps for its use.