The Coffin-Siris Syndrome (CSS) is a rare genetic disorder with many different potential symptoms and a broad neurocognitive and behavioral phenotype. The aim of the present study was to generate...Show moreThe Coffin-Siris Syndrome (CSS) is a rare genetic disorder with many different potential symptoms and a broad neurocognitive and behavioral phenotype. The aim of the present study was to generate more knowledge about this syndrome by investigating how the executive functions inhibition, emotion regulation and task-monitor predict the outcome of externalizing behavior in children with CSS. The sample included 26 children and adolescents between the biological ages of 4 and 17 years (M = 9.8, SD = 4.11), of whom 46.2% were boys (n = 12) and 53.8% were girls (n = 14) originating from the Netherlands (n = 25) and Belgium (n = 1). Inhibition, emotion regulation and task-monitor were the executive function-domains used as independent variables and externalizing behavior as dependent. The results show an above average level of problems with executive functions within children with CSS and indicate that a combination of the executive function problems is related to the extent of externalizing behavior (F(3,26) = 4.971, p = .015, R2 = .516, Radj2 = .412). A non-significant trend was found for emotion regulation problems in the prediction of externalizing behavior. This illustrates the variety of symptoms in CSS-patients and suggests that it is probably a combination of such symptoms that leads to further problems in socio-behavioral outcomes. The findings also suggest that targeting a combination executive functions in interventions might be beneficial to behavioral outcomes in CSS.Show less
