Children with Klinefelter may face difficulties in their later life when it comes to social emotional development and behavior problems (Bruining et al., 2009). This could lead to behavior- and...Show moreChildren with Klinefelter may face difficulties in their later life when it comes to social emotional development and behavior problems (Bruining et al., 2009). This could lead to behavior- and emotion regulation problems including anxiety. During a stressful life event it is crucial to be able to control/use cognitive emotion regulation strategies (Thompson, 1994). If this fails, it can lead to behavior- or social development problems. Although separate results from previous studies suggest a link between anxiety, regulation problems and stressful life events, this possible coherence has not been studied yet for Klinefelter children. Method: To investigate this, 95 children in the age of 1-7 years (of which 51 children with Klinefelter) and their parents participated in a series of questionnaires (Child Behavior Checklist, Children’s Behavior-/Early Childhood Behavior-/VMG Life Events Questionnaire). Results: Children with Klinefelter were found to be more anxious and showed a less expressive internal regulation state compared to the control group, according to their parents. According to the CBQ/ECBQ Reactivity/Soothability subscale results, children with Klinefelter showed less expressive (blunted) emotions when they were in pain, upset or experienced an exciting activity/event. In addition, stressful life events turned out to be a predictor for anxious depressed behavior in children with Klinefelter. There was also no interaction found for the degree of stressful life events and anxious/depressed or reactivity/soothability behavior within the Klinefelter- or control group. Conclusion: Children with Klinefelter experience more anxiety/depressed behavior and less expressive internal regulation state than children without Klinefelter. An association between stressful life events and anxiety/depressed behavior is found within children with Klinefelter, but there is no evidence found for a coherence between stressful life events and regulation problems. Furthermore, no interaction was found between the degree of stressful life events and anxious/depressed or reactivity/soothability behavior within the Klinefelter- or control group.Show less