A retrospective study of eating and psychosocial problems in patients with hepatic glycogen storage diseases and idiopathic ketotic hypoglycemia: Towards a standard set of patient-reported outcome measures.

There is a paucity in literature on eating and psychosocial problems in patients with hepatic glycogen storage disease (GSD) and idiopathic ketotic hypoglycemia (IKH), problems that can greatly affect quality of life. This is a monocentre, retrospective, observational mixed method study of patients with hepatic GSD or IKH treated at the Beatrix Children's Hospital Groningen, who had been referred to SeysCentra, a specialist centre for the treatment of eating problems. Additionally, a systematic literature review has been performed to identify instruments to quantify patient-reported outcome measures of psychosocial problems in hepatic GSD patients. Sixteen patients from 12 families were included with ages ranging between 3 and 24 years. Five out of sixteen patients were diagnosed with Avoidant/Restrictive Food Intake Disorder and six patients showed characteristics of this disorder. Fourteen patients experienced sleeping problems, and 11 out of 12 parent couples experienced stress about the illness of their child. We subsequently identified 26 instruments to quantify patient-reported outcome measures for GSD patients. This study demonstrates that GSD patients can develop Avoidant/Restrictive Food Intake Disorder influencing quality of life at multiple domains. The identification of instruments to assess psychosocial wellbeing is an important step towards a standard set of patient-reported outcome measures.

Training Teachers to Implement Classroom Pivotal Response Teaching during Small-group Instruction: A Pilot Study.

Background: Providing effective education to students with autism spectrum disorder (ASD) poses a significant challenge to educators. Although several evidence-based practices (EBPs) have been developed, few have been systematically implemented in educational settings. Pivotal response treatment (PRT) is a naturalistic behavioral intervention that has been adapted for implementation in the school context. Methods: This pilot study used a concurrent multiple baseline design across seven teachers and students with ASD to examine the effectiveness of teacher training in classroom pivotal response teaching (CPRT) on teacher fidelity of implementation during small-group instruction and students' communication skills and maladaptive behaviors in schools for special education in the Netherlands. Results: Results indicated no replicated effect of CPRT training on teachers' fidelity of implementation or children's communications skills and maladaptive behavior, although teachers reported high satisfaction with the CPRT training. Discussion: Implications for clinical practice and directions for future research are discussed.

Pivotal response treatment: A study into the relationship between therapist characteristics and fidelity of implementation.

LAY ABSTRACT: Pivotal response treatment is a naturalistic behavioral intervention that teaches pivotal skills to children with autism spectrum disorder to produce widespread gains in other skills. Although most children with autism spectrum disorder benefit from pivotal response treatment, intervention outcomes vary considerably among children. Fidelity of intervention implementation (i.e. the extent to which an intervention is implemented as intended) may affect intervention outcomes. In this study, we studied the relationship between fidelity of pivotal response treatment implementation and therapist characteristics, such as therapist personality, therapist-child relationship, therapist attitude toward evidence-based practices, and therapist experience. We also explored whether a child's age and autism symptom severity were related to pivotal response treatment fidelity. Participants were 41 pivotal response treatment therapists who videotaped three pivotal response treatment sessions and completed four questionnaires to measure therapist characteristics. This study found that therapists' openness to innovation and their experience with pivotal response treatment predicted fidelity of implementation. Therapist personality, therapist-child relationship, and child characteristics were not related to pivotal response treatment fidelity. The results of this study emphasize that it is important (1) to target therapists' attitudes toward innovation prior to or during training in pivotal response treatment and (2) to provide therapists with ongoing supervision and feedback after training to increase fidelity of implementation and thus to improve intervention outcomes for children with autism spectrum disorder.

Effectiveness of Parent Education in Pivotal Response Treatment on Pivotal and Collateral Responses.

In two studies, we investigated the effectiveness of parent education in Pivotal Response Treatment (PRT) on parent-created opportunities and spontaneous child initiations in two community-based treatment facilities for children with autism spectrum disorder (ASD). Changes in parental stress and self-efficacy were explored. Participants were 26 parents and their children who participated in group (Study 1) or individual (Study 2) parent education in PRT. Results indicated that group-based parent education resulted in moderate increases in opportunities, functional initiations, and empathic social initiations. Furthermore, parental stress reduced and self-efficacy increased. Individual parent education resulted in large increases in opportunities and functional initiations, but parental stress and self-efficacy did not change. Implications for clinical practice and directions for future research are discussed.

Increasing Opportunities for Question-Asking in School-Aged Children with Autism Spectrum Disorder: Effectiveness of Staff Training in Pivotal Response Treatment.

Deficits in question-asking are common in children with autism spectrum disorder (ASD). Furthermore, their opportunities to self-initiate questions are often hindered by directive behavior of their conversation partners. This study assessed the effectiveness of staff training in pivotal response treatment (PRT) on staff member-created opportunities and self-initiated questions of school-aged children with ASD. Generalization and maintenance were also assessed. Participants were 14 staff members and children with ASD attending an inpatient treatment facility. Data showed that PRT resulted in significant increases in both staff member-created opportunities and child-initiated questions. Generalization to group situations and collateral changes in children's language, pragmatic, and adaptive skills, and maladaptive behaviors did not occur. Implications for clinical practice and directions for future research are discussed.

Improving Collaborative Play Between Children with Autism Spectrum Disorders and Their Siblings: The Effectiveness of a Robot-Mediated Intervention Based on Lego® Therapy.

The aim of the study was to investigate the effectiveness of a brief robot-mediated intervention based on Lego(®) therapy on improving collaborative behaviors (i.e., interaction initiations, responses, and play together) between children with ASD and their siblings during play sessions, in a therapeutic setting. A concurrent multiple baseline design across three child-sibling pairs was in effect. The robot-intervention resulted in no statistically significant changes in collaborative behaviors of the children with ASD. Despite limited effectiveness of the intervention, this study provides several practical implications and directions for future research.

Promoting question-asking in school-aged children with autism spectrum disorders: effectiveness of a robot intervention compared to a human-trainer intervention.

OBJECTIVE: The purpose of the present study was to investigate the effectiveness of an applied behaviour analysis (ABA)-based intervention conducted by a robot compared to an ABA-based intervention conducted by a human trainer in promoting self-initiated questions in children with autism spectrum disorder (ASD). METHODS: Data were collected in a combined crossover multiple baseline design across participants. Six children were randomly assigned to two experimental groups. RESULTS: Results revealed that the number of self-initiated questions for both experimental groups increased between baseline and the first intervention and was maintained during follow-up. The high number of self-initiated questions during follow-up indicates that both groups maintained this skill. CONCLUSIONS: The interventions conducted by a robot and a human trainer were both effective in promoting self-initiated questions in children with ASD. No conclusion with regard to the differential effectiveness of both interventions could be drawn. Implications of the results and directions for future research are discussed.

Reactive/proactive aggression and affective/cognitive empathy in children with ASD.

The main aim of this study was to examine the extent to which affective and cognitive empathy were associated with reactive and proactive aggression, and whether these associations differed between children with an Autism Spectrum Disorder (ASD) and typically developing (TD) children. The study included 133 children (67 ASD, 66 TD, Mage=139 months), who filled out self-report questionnaires. The main findings showed that the association between reactive aggression and affective empathy was negative in TD children, but positive in children with ASD. The outcomes support the idea that a combination of poor emotion regulation and impaired understanding of others' emotions is associated with aggressive behavior in children with ASD.

Staff training effective in increasing learning opportunities for school-aged children with autism spectrum disorders.

OBJECTIVE: This study examined the effectiveness of instruction and video feedback on staff's ABA skills during one-to-one play situations and initiations of children with autism spectrum disorder (ASD). METHODS: Data were collected within a multiple baseline design across 5 dyads. A continuous 20 s interval recording system was used to record motivation, creating opportunities, prompting and reinforcement of staff and child initiations. Training included instruction, consisting of instructions, video examples and role-plays. After this, a 4-h delayed video feedback condition started. RESULTS: Three staff members created significantly more learning opportunities during post-instruction and a significant increase occurred during video feedback for one staff member. Initiatives increased significantly in two children during post-instruction. During follow-up, three children showed unprompted initiatives. The mean percentage of spontaneous initiations increased during follow-up. CONCLUSION: The findings provide support for training staff in a clinical setting to create learning opportunities, which also may result in concomittant improvement in child initiations.