Social functioning and behaviour in Mucopolysaccharidosis IH [Hurlers Syndrome]

Background: Mucopolysaccharidosis type IH (MPS-IH) [Hurlers Syndrome] is a developmental genetic disorder characterised by severe physical symptoms and cognitive decline. This study aimed to investigate the behavioural phenotype of MPS-IH treated by haematopoietic cell transplantation, focusing on social functioning and sleep. Parental stress was also measured. Methods: Participants were 22 children with MPS-IH (mean age 9 years 1 month), of whom 10 were male (45%). Parents completed the Social Responsiveness Scale (SRS), Child Behaviour Checklist (CBCL), Children’s Sleep Habit Questionnaire and Parent Stress Index, Short Form (PSI-SF). Results: Twenty-three per cent of children with MPS-IH scored in the severe range of the SRS, suggesting significant difficulties in social functioning. Children with MPS-IH were more than 30 times more likely to receive scores in the severe range than typically developing children. Thirty-six per cent scored in the mild-to-moderate range, suggesting milder, but marked, difficulties in social interaction. Although children with MPS-IH did not show significantly higher rates of internalising, externalising or total behaviour problems than the normative sample, they received scores that were significantly higher on social, thought and attention problems and rule-breaking behaviour, and all the competence areas of the CBCL. Parents of children with MPS-IH did not score significantly higher on parental stress than parents in a normative sample. Conclusions: Parents of children with MPS-IH rate their children as having problems with social functioning and various areas of competence more frequently than previously thought, with implications for clinical support

Brief Report of Preliminary Outcomes of an Emotion Regulation Intervention for Children with Autism Spectrum Disorder

DOI: 10.1007/s10803-015-2446-1Children with autism spectrum disorder (ASD) often present with comorbid psychopathology including problems with emotion regulation. The goal of the present research was to investigate the feasibility of a multicomponent manualized cognitive behavior therapy treatment program for improving emotion regulation in youth with ASD 8 to 12 years of age. Thirteen males and their parents participated in the intervention, reporting high satisfaction with the activities and program overall, and attending all sessions. Preliminary outcomes regarding emotion regulation and psychopathology, and feasibility of the intervention, are summarized and discussed.Chair in Autism Spectrum Disorders Treatment and Care Research (#RN284208; Canadian Institutes of Health Research in partnership with NeuroDevNet, Sinneave Family Foundation, CASDA, Autism Speaks Canada and Health Canada) and seed funding from the Spectrum of Hope Autism Foundatio

Intensive intervention for children and adolescents with autism in a community setting in Italy: a single-group longitudinal study

<p>Abstract</p> <p>Background</p> <p>Previous studies have shown favourable results with intensive behavioural treatment for children with autism: evidence has emerged that treatment can be successfully implemented in a community setting and in adolescent participants. The aim of this study was to describe the 2-year adaptive functioning outcome of children and adolescents with autism treated intensively within the context of special autism centres, as well as to evaluate family satisfaction with the activity of the centres.</p> <p>Methods</p> <p>Sixty participants with autism (20 females and 40 males, aged between 4 and 18 years) attending the semi-residential rehabilitation centres for autism located in the Abruzzo region (Central Italy) were followed up and their adaptive functioning was evaluated both at baseline and after one and two years using the Vineland Adaptive Behaviour Scales (VABS). Parents' satisfaction with the service was evaluated using the Orbetello Satisfaction Scale for Children and Adolescent Mental Health.</p> <p>Results</p> <p>The increase in VABS scores was significant on several domains in the different gender and age categories. It is worth noting that male children had improved a great deal (roughly, an effect size >0.20) in the domains of communication, daily living and motor skills (effect sizes 0.34, 0.45 and 0.27 respectively) whereas in male adolescents, a notable increase in VABS scores was recorded in the domain of socialization only (effect size 0.23). On the other hand, adaptive behaviour in female children increased in the domains of socialization and motor skills (effect sizes 0.27 and 0.42 respectively) whereas in female adolescents, good results were achieved in the domains of daily living, socialization and motor skills (effect sizes 0.22, 0.26 and 0.20 respectively).</p> <p>The level of satisfaction of users of the service over time was found to be substantial, even when they had recently started the program.</p> <p>Conclusions</p> <p>Our results support the implementation of special autism treatment community centres, based on a parent co-directed rehabilitative, intensive and early intervention. Further experimental research designed to document the effectiveness of services provided to children and adolescents with autism in the community is recommended.</p

Brief report:effects of sensory sensitivity and intolerance of uncertainty on anxiety in mothers of children with autism spectrum disorder

This study examined the relations between anxiety and individual characteristics of sensory sensitivity (SS) and intolerance of uncertainty (IU) in mothers of children with ASD. The mothers of 50 children completed the Hospital Anxiety and Depression Scale, the Highly Sensitive Person Scale and the IU Scale. Anxiety was associated with both SS and IU and IU was also associated with SS. Mediation analyses showed direct effects between anxiety and both IU and SS but a significant indirect effect was found only in the model in which IU mediated between SS. This is the first study to characterize the nature of the IU and SS interrelation in predicting levels of anxiety

Anxiety Levels in Children with Autism Spectrum Disorder:A Meta-Analysis

The aim of the current study was to meta-analytically examine whether anxiety levels in children with autism spectrum disorders (ASD) are elevated. A total of 83 articles were selected from a systematic literature search and were included in the meta-analyses. Results demonstrated that children with ASD had higher anxiety levels compared to typically developing children, and this difference increased with IQ. Youth with ASD also tended to have higher anxiety levels compared to clinically referred children, and this difference increased with age. Children with ASD had higher anxiety levels compared to youth with externalizing or developmental problems, but not when compared to youth with internalizing problems. The study findings highlight the importance of more research in order to fully understand the nature and development of anxiety in children with ASD. More specifically, the results suggest that especially high-functioning adolescents with ASD may be at risk for developing anxiety disorders. Therefore, it seems important to carefully follow and monitor children with ASD transcending to adolescenc

Comparisons of the factor structure and measurement invariance of the Spence children’s anxiety scale - parent version in children with autism spectrum disorder and typically developing anxious children

The Spence Children’s Anxiety Scale - Parent version (SCAS-P) is often used to assess anxiety in children with autism spectrum disorder (ASD), however, little is known about the validity of the tool in this population. The aim of this study was to determine whether the SCAS-P has the same factorial validity in a sample of young people with ASD (n=285), compared to a sample of typically developing young people with anxiety disorders (n=224). Poor model fit with all of the six hypothesised models precluded invariance testing. Exploratory factor analysis indicated that different anxiety phenomenology characterises the two samples. The findings suggest that cross-group comparisons between ASD and anxious samples based on the SCAS-P scores may not always be appropriat

Emotion Coregulation in Mother-Child Dyads: A Dynamic Systems Analysis of Children with and without Autism Spectrum Disorder

Few studies have investigated patterns of emotion coregulation in families of children with Autism Spectrum Disorder (ASD) or contrasted the ways in which their emotion coregulation patterns differ from families of typically developing (TD) children. To address this gap, we used a dynamic systems approach to compare flexible structure and emotional content of coregulation between mothers and children (3-7&nbsp;years) with ASD (n&nbsp;=&nbsp;47) and TD children (n&nbsp;=&nbsp;26). Mother-child play interactions in the home were videotaped and emotion-engagement states were coded in micro-level 5-s intervals based on behavioral and affective expressions. Analyses indicated that mother-child dyads in the ASD group spent more time than dyads in the TD group in mismatched emotion-engagement states (e.g., child negative/mother positive), and children with ASD spent more time than TD children engaged exclusively with objects. Mother-child dyads in the TD group stayed longer in mutual positive engagement states. Compared to dyads in the TD group, mother-child dyads in the ASD group exhibited greater flexibility (i.e., a wider range of emotional-engagement states, more frequent changes in states, and less time in each state). These findings suggest that mothers and their children with ASD do not sustain dyadic positive engagement patterns in a low-stress environment. Findings confirmed the preference of children with ASD for objects over social partners, even when they are at home with their mothers, and elucidated a challenging mother-child interactional style. Results have implications for mother-child interventions aimed at regulating negative emotional states and sustaining positive ones in families raising children with ASD