Pathogenic variations in the RAS-MAPK cellular signaling system cause RASopathies, which are genetic disorders. Neurofibromatosis type 1, Noonan syndrome, cardiofaciocutaneous syndrome, and Costello syndrome are among the syndromes linked to a wide range of medical and behavioral health issues. Even though social problems and autism spectrum disorder (ASD) have a higher probability of co-occurring, little research has evaluated distinct aspects of social behavior in these disorders. It’s also unclear whether children with RASopathies have different underlying cognitive traits that lead to social competence and socially empathic (“prosocial”) behaviors than children with nonsyndromic (i.e., idiopathic) ASD. Caregivers of preschool and school-aged children with RASopathies (n=202) or idiopathic ASD (n=109) contributed demographic, medical, and developmental information about their child, including psychiatric comorbidities, in the cross-sectional, survey-based investigation. Caregivers completed standardized rating scales to assess social competence and empathic conduct, as well as signs of hyperactivity/inattention and emotional difficulties, for children who could communicate verbally. Children with RASopathies were judged as having better resilience in the domain of empathy relative to their overall social competence than children with idiopathic ASD. In these 2 groups, similarities and differences in psychological characteristics that influenced social behavior emerged. For both groups, improved communication skills and fewer hyperactive-impulsive behaviors were linked to enhanced empathy and social competence. For children with RASopathies, more emotional problems were related to reduced social competence and higher empathy for children with idiopathic ASD. In comparing children with idiopathic ASD, children with RASopathy with a co-occurring ASD diagnosis exhibited more significant socially sympathetic behaviors. The research implied that, unlike a behaviorally defined illness, the development of social behavior in children with RASopathies involves a different pattern of strengths and deficits (idiopathic ASD). Identification of areas of resilience, as well as behavioral and social difficulties, will allow for more focused intervention.

 

Source:link.springer.com/article/10.1186/s11689-021-09414-w

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