BackgroundGene mutations within the RAS-MAPK signaling cascade result in Noonan syndrome (NS), neurofibromatosis type 1 (NF1), and related disorders. Recent research has documented an increased risk for social difficulties and features of autism spectrum disorder (ASD) among children with these conditions. Despite this emerging evidence, the neuropsychological characteristics associated with social skills deficits are not well understood, particularly for children with NS.MethodsParents of children with NS (n = 39), NF1 (n = 39), and unaffected siblings (n = 32) between the ages of 8 and 16 years were administered well-validated caregiver questionnaires assessing their child’s social skills, language abilities, attention-deficit hyperactivity disorder (ADHD) symptoms and anxiety.ResultsWith respect to overall social skills, average ratings of children in both clinical groups were similar, and indicated weaker social skills compared to unaffected siblings. Although ratings of social skills were outside of normal limits for more than four in ten children within the clinical groups, most of the deficits were mild/moderate. Fifteen percent of the children with NS and 5% of the children with NF1 were rated as having severe social skills impairment (< − 2SD). Independent of diagnosis, having fewer ADHD symptoms or better social-pragmatic language skills was predictive of stronger social skills.ConclusionsAmidst efforts to support social skill development among children and adolescents with RASopathies, neuropsychological correlates such as social language competence, attention, and behavioral self-regulation could be important targets of intervention.
While neurofibromatosis type 1 (NF1) and Noonan syndrome (NS) are clinically distinct genetic syndromes, they have overlapping features because they are caused by pathogenic variants in genes encoding molecules within the Ras-mitogen-activated protein kinase signaling pathway. Increased risk for emotional and behavioral challenges has been reported in both children and adults with these syndromes. The current study examined parent-report and self-report measures of emotional functioning among children with NF1 and NS as compared to their unaffected siblings. Parents and children with NS (n = 39), NF1 (n = 39), and their siblings without a genetic condition (n = 32) completed well-validated clinical symptom rating scales. Results from parent questionnaires indicated greater symptomatology on scales measuring internalizing behaviors and symptoms of attention deficit hyperactivity disorder (ADHD) in both syndrome groups as compared with unaffected children. Frequency and severity of emotional and behavioral symptoms were remarkably similar across the two clinical groups. Symptoms of depression and anxiety were higher in children who were also rated as meeting symptom criteria for ADHD. While self-report ratings by children generally correlated with parent ratings, symptom severity was less pronounced. Among unaffected siblings, parent ratings indicated higher than expected levels of anxiety. Study findings may assist with guiding family-based interventions to address emotional challenges. K E Y W O R D Sanxiety, depression, emotional function, neurofibromatosis type 1, Noonan syndrome, RASopathies
Objective This study examined the possible moderating role of social support on the association between child externalizing behavior and parenting stress in a cross‐sectional sample of typically developing toddlers and their mothers. Background Child externalizing behavior problems often emerge during the toddler years and are associated with increased parenting stress. Family stress theory posits that resources (e.g., social support) may buffer the effects of stressors such as child behavior concerns. However, limited empirical research has examined the impact of social support on parenting stress in parents of typically developing toddlers. Method The sample included 1,036 mother–toddler dyads and was representative of the broader region in which the research took place (59% African American, 58% married/cohabitating, 53% with high school diploma). General linear models were performed. Results The interaction term was not significant; therefore, a moderator model was not supported. However, the final model demonstrated that child behavior problems were associated with higher levels of parenting stress; and lower levels of both amount of support and satisfaction with social support were associated with additional increases in parenting stress. Conclusion Results suggest that parents who have low levels of social support and a toddler with higher levels of externalizing behaviors are at particular risk for experiencing elevated levels of parenting stress. Implications Given that externalizing behaviors are common during the toddler years, future research is needed to better understand how social support benefits parents of toddlers and to develop new or modify existing interventions to increase social support and reduce parenting stress.
Adolescents with autism spectrum disorder (ASD) are vulnerable to declines in social connections and an increase in depression, anxiety, and other co-occurring conditions. This study introduces a novel intervention that matches adolescents and adults with ASD in one-to-one mentoring relationships in an afterschool setting and examines its social validity. In this single-group, mixed-method pilot study, participants included seven adolescent mentees (14–18 years old; 100% male), seven adult mentors (19–33 years old; 71% male), and eight parents of mentees. A combination of project-specific and standardized assessments was used to describe the participants’ perceptions of the program and to assess well-being, self-concept, and social-emotional and behavioral outcomes. Results showed high uptake, program satisfaction, positive ratings of mentoring relationship quality, and desirable pre- to post-test change on several targeted outcomes. This study provides preliminary evidence to support the applicability and utility of a mentoring program for adolescents with ASD by adults with ASD
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