Children with sex chromosome trisomy (SCT) are at increased risk for psychopathology, particularly within the social domain. This study investigates whether children with SCT exhibit elevated...Show moreChildren with sex chromosome trisomy (SCT) are at increased risk for psychopathology, particularly within the social domain. This study investigates whether children with SCT exhibit elevated autism traits compared to typically developing children, given the difficulties in social interaction and communication that are typical of autism spectrum disorders. The sample comprised 67 children with SCT (27 girls with XXX, 29 boys with XXY, 11 boys with XYY) and 70 typically developing children, aged 2.5 to 7.5 years. Autism traits were assessed using the Social Responsiveness Scale, Second edition (SRS-2). Statistical analyses revealed that children with SCT demonstrate more severe deficits in social responsiveness than typically developing children, scoring primarily in the subclinical range. Children with SCT scored significantly higher on all assessed autism aspects, with social awareness being the least affected compared to the control group. No differences were found across different karyotypes, suggesting a consistent elevation of autism traits across SCT genetic variants. Age had no effect on the severity of autism traits, implying stability across development. These insights enhance understanding of the specific needs and challenges of children with SCT in their social development, which is crucial for the development of effective support and intervention programs. Additionally, these findings underscore the importance of early detection and intervention for children with SCT, with autism traits serving as potential early indicators of SCT presence.Show less
This study explores the relationship between genotype, parental functioning, and executive function development in children with Sex Chromosome Trisomies (SCTs). Despite growing awareness of the...Show moreThis study explores the relationship between genotype, parental functioning, and executive function development in children with Sex Chromosome Trisomies (SCTs). Despite growing awareness of the importance of genetic research, knowledge about the behavioral and neurocognitive characteristics of SCTs remains limited, as most studies focus on medical aspects. Children with SCTs are at increased risk for suboptimal neurodevelopment, leading to intellectual and developmental challenges, including executive function issues. This current study indicates that children with SCTs have more problems with more complex executive functions like emotion regulation and cognitive flexibility compared to peers without SCTs, with borderline significant differences in the basal executive function inhibition. No significant differences were found between girls with Trisomy X (47, XXX) and boys with Klinefelter syndrome (47, XXY) in these domains, which suggests that the extra X-chromosome is associated with suboptimal development of executive functions, regardless of gender. This indicates that these domains of executive functioning are particularly vulnerable in this population. Parental sensitivity appears to play a role in the cognitive flexibility of children with SCTs, implying that interventions aimed at improving parent-child interactions may be beneficial for improving executive functions.Show less
This research study explores the differences in executive functioning (EF) between normally developing children and children with Sex Chromosome Trisomies (SCT) between 1 and 8 year old. Rather...Show moreThis research study explores the differences in executive functioning (EF) between normally developing children and children with Sex Chromosome Trisomies (SCT) between 1 and 8 year old. Rather than the typical karyotype XX or XY, people with SCT have three sex chromosomes, resulting in karyotype XXY, XYY or XXX. Executive functions can be defined as mental processes that allow people to control their actions. The BRIEF, BRIEF-P and MEFS were utilized to obtain information about the development of EF. A total of 147 participants between the ages of 2,8 and 7,6 years old took part in the study. 74 participants with SCT (Mage= 4,8, SD = 1,3) and 70 (Mage= 4,5, SD = 1,0) participants without SCT. Analysis methods used were independent samples t-tests, correlations and Fisher-Z transformations. It was found that there are differences in executive functioning as a whole between children with and without SCT. The differences in working memory, inhibition and cognitive flexibility are small. Bigger differences between children with and without SCT were found with regards to their emotional control and cognitive flexibility. The research concludes that developmental delay regarding executive functioning is visible in children with SCT before the age of 8 years old. When children mature these differences become more obvious as the gap between what is expected with regards to EF increases faster than children with SCT improve their EF skills. Additional research is needed in order to discover whether the EF profile found is unique for children with SCT and what implications this has. Future studies should also investigate the ways EF impacts children with SCT and which treatments yields the most benefits for them. Support with regards to the development of EF in children with SCT is crucial in order to enable them to maximize the possibilities in their lives.Show less
Research master thesis | Developmental Psychopathology in Education and Child Studies (research) (MSc)
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Background and objective: Sex Chromosome Trisomy (SCT) refers to having an extra X or Y chromosome (XXX, XXY, XYY), which occurs in approximately 1-2 of 1000 children. In this study, social...Show moreBackground and objective: Sex Chromosome Trisomy (SCT) refers to having an extra X or Y chromosome (XXX, XXY, XYY), which occurs in approximately 1-2 of 1000 children. In this study, social attention, Theory of Mind (ToM) and neurobehavioral outcomes are investigated in preschool aged children with SCT compared to typically developing controls. Furthermore, the study aims to explore with a longitudinal design whether social attention and ToM in preschool aged children with SCT can be predictive of neurobehavioral outcomes one year later. Methods: In this study 71 children with SCT (aged 3-7) and 71 typically developing age matched controls participated. The Social Interaction Scanning (SIS) eye tracking paradigm was used to investigate social attention. ToM skills were measured with the ToM subtest of the Developmental NEuroPSYchological Assessment, second edition (NEPSY-II). Neurobehavioral outcomes were measured with the Social Responsiveness Scale, second edition (SRS-2), Strengths and Weaknesses of ADHD and Normal behavior (SWAN) and the Child Behavior Check List (CBCL) parental questionnaires respectively. Results: Results of the current study show a vulnerability for children with SCT in the domain of social attention to faces and eyes and ToM compared to typically developing controls. Findings are more pronounced for children aged 5-7 years old. Furthermore, children with SCT appear at increased risk for poorer neurobehavioral outcomes. Finally, results of the current study suggest that ToM may have a unique predictive value for social impairment over time, specifically in the domain of social communication and interaction. Secondly, social attention may have a unique predictive value for ADHD symptoms over time. Conclusions: Results indicate that screening of social cognitive functions (around the age of 5-7) and monitoring of neurodevelopmental pathways is important for clinical practice in SCT. In future research the efficacy of early tailored interventions targeting social cognitive skills in SCT can be investigated, to positively influence developmental outcomes of children with SCT.Show less
Research master thesis | Developmental Psychopathology in Education and Child Studies (research) (MSc)
closed access
One in 650-1000 individuals has sex chromosome trisomy (SCT), being born with an extra X or Y chromosome (XXX; XXY; XYY). SCT comes with various developmental risks. The present study was designed...Show moreOne in 650-1000 individuals has sex chromosome trisomy (SCT), being born with an extra X or Y chromosome (XXX; XXY; XYY). SCT comes with various developmental risks. The present study was designed to evaluate the relation between parental factors (parenting skills (parental reflective functioning, sensitivity, and intrusiveness) and family functioning) and the social cognitive skills and its rate of development of children with SCT. Literature suggests that parenting skills and family functioning influence social cognitive development in typical children, but this has not been researched in children with SCT. Included in this study were 106 children with SCT and 102 controls (age M = 3.65, SD = 1.78 range = .98-7.66). A free play task, the parental developmental interview, and a questionnaire were used for evaluating parental factors, and the social cognitive skills of the child were measured using the ASIEP-3. Children with SCT showed significant impairments in social cognitive skills while the rate of development of social cognitive skills proved identical to controls. Parental sensitivity, intrusiveness, and reflective functioning were similar across groups and were found to not significantly influence social cognitive development. Family functioning was found to be positively related to the social cognitive skills of SCT children. However, family functioning did not influence the rate of social cognitive development of either SCT or control groups. The findings imply other factors to be of more importance in the social cognitive development of children with SCT at the average ages of 1-2 years and 3-6 years. It is recommended to research other factors, namely brain maturation and timing of diagnosis. Further knowledge about which factors stimulate the development of the social cognitive abilities of children with SCT, to what extent and at what age, is of importance as this may help to develop and personalize prevention and intervention programs.Show less
Children with sex chromosome trisomy’s (SCT) are more vulnerable to developing behavioral, neurocognitive and social-emotional functioning problems and have an increased risk of psychopathology....Show moreChildren with sex chromosome trisomy’s (SCT) are more vulnerable to developing behavioral, neurocognitive and social-emotional functioning problems and have an increased risk of psychopathology. This study investigates through group comparisons to what extent the timing of diagnosis (prenatal/postnatal) influences the type and degree of problem behavior of children aged 1-7 years. In total, 93 children (71 prenatal and 22 postnatal) with an extra X or Y chromosome and 102 non-clinical controls and their parents participated in the study. Types of problem behavior were assessed using the Child Behavior Checklist (CBCL) and a medical history questionnaire. The results indicate that children with SCT generally show more total and internalizing problem behavior than typically developing children, but not significantly more externalizing problem behavior. When it comes to specific problem behaviors, SCT children generally have a shorter attention span, are more easily distracted, tend to be more withdrawn and isolated, worry and experience anxieties and are more sensitive to stimuli and moodiness. The results show that no differences are found in total, internalizing and externalizing problem behavior between children with a prenatal and postnatal diagnosis. However, children with a postnatal diagnosis generally worry more excessively, experience anxiety and are often more irritable compared to children with a prenatal diagnosis of SCT. Finally, the results show that temper tantrums/oppositional behavior, short span of attention/distractibility and excessive worry and fear within the clinical group increases with age. In conclusion, this suggests that it is important to bring the possible consequences of SCT to the attention of medical care providers and parents, so that these children do not worry excessively, experience anxiety and are more irritable (anymore). This proves that there is a need for early interventions in children with SCT, to ensure the quality of life and to reduce the risk of mental health problems.Show less
