The presence of an additional X or Y chromosome (sex chromosome trisomies, SCT) is associated with an increased risk for neurodevelopmental difficulties, including socio-emotional problems, across... Show moreThe presence of an additional X or Y chromosome (sex chromosome trisomies, SCT) is associated with an increased risk for neurodevelopmental difficulties, including socio-emotional problems, across the life span. Studying emotion regulation in young children with SCT could signal deviations in emotional development that serve as risk markers to guide clinical care. This study explored the presence and variety of emotion regulation strategies in 75 SCT children and 81 population-based controls, aged 1–7 years, during a frustration-inducing event in which physiological (heart rate) and observational data (behavioral responses) were collected. Children with SCT were equally physiologically aroused by the event as compared to controls. However, they showed more emotion regulation difficulties in terms of behavior compared to controls that were not explicable in terms of differences in general intellectual functioning. Specifically, they had a more limited range of behavioral alternatives and tended to rely longer on inefficient strategies with increasing age. The field of practice should be made aware of these early risk findings regarding emotion regulation in SCT, which may potentially lay the foundation for later socio-emotional problems, given the significant impact of emotion regulation on child and adult mental health outcomes. The current results may help to design tailored interventions to reduce the impact of the additional sex chromosome on adaptive functioning, psychopathology, and quality of life. Show less
Lubbe, A. van der; Swaab, H.; Vermeiren, R.R.J.M.; Ester, W.A. 2022
Mothers of children with Autism Spectrum Disorder (ASD) often experience chronic stress and are at risk for adverse health. However, little is known about fathers, especially when their child is in... Show moreMothers of children with Autism Spectrum Disorder (ASD) often experience chronic stress and are at risk for adverse health. However, little is known about fathers, especially when their child is in early childhood. Parenting stress, eating behavior and physical health was evaluated in mothers (n = 48) and fathers (n = 43) of young children (3-7 years) with ASD by questionnaires and physical measurements. Mother's prevalence rates of obesity (39.1%), abdominal obesity (59.6%) and metabolic syndrome (21.6%) were higher than the norm. In fathers, the prevalence rate of clinical parenting stress (33%) was higher than the norm. Parenting stress was positively related to disinhibited eating in mothers, not in fathers. It is crucial to monitor stress and health of parents of children with ASD. Show less
BackgroundSex Chromosome Trisomies (SCTs; XXX, XXY, XYY) are genetic conditions that are associated with increased risk for neurodevelopmental problems and psychopathology. There is a great need... Show moreBackgroundSex Chromosome Trisomies (SCTs; XXX, XXY, XYY) are genetic conditions that are associated with increased risk for neurodevelopmental problems and psychopathology. There is a great need for early preventive intervention programs to optimize outcome, especially considering the increase in prenatal diagnoses due to recent advances in non-invasive prenatal screening. This study is the first to evaluate efficacy of a neurocognitive training in children with SCT. As social behavioral problems have been identified as among the key areas of vulnerability, it was targeted at improving a core aspect of social cognition, the understanding of social cues from facial expressions.MethodsParticipants were 24 children with SCT and 18 typically developing children, aged 4–8 years old. Children with SCT were assigned to a training (n = 13) or waiting list (no-training) group (n = 11). Children in the training group completed a neurocognitive training program (The Transporters), aimed to increase understanding of facial emotions. Participants were tested before and after the training on facial emotion recognition and Theory of Mind abilities (NEPSY-II), and on social orienting (eyetracking paradigm). The SCT no-training group and typically developing control group were also assessed twice with the same time interval without any training. Feasibility of the training was evaluated with the Social Validity Questionnaire filled out by the parents and by children's ratings on a Visual Analog Scale.ResultsThe SCT training group improved significantly more than the SCT no-training and TD no-training group on facial emotion recognition (large effect size; η2p = 0.28), performing comparable to typical controls after completing the training program. There were no training effects on ToM abilities and social orienting. Both children and parents expressed satisfaction with the feasibility of the training.ConclusionsThe significant improvement in facial emotion recognition, with large effect sizes, suggests that there are opportunities for positively supporting the development of social cognition in children with an extra X- or Y-chromosome, already at a very young age. This evidence based support is of great importance given the need for preventive and early training programs in children with SCT, aimed to minimize neurodevelopmental impact. Show less
Evidence of the effectivity of play-based interventions in children with autism spectrum disorder (ASD) was evaluated by PRISMA-based literature study and a Risk of Bias (RoB) assessment. Many of... Show moreEvidence of the effectivity of play-based interventions in children with autism spectrum disorder (ASD) was evaluated by PRISMA-based literature study and a Risk of Bias (RoB) assessment. Many of the 32 eligible randomized controlled trials (RCT) reported improved social interaction, communication, daily functioning and play behaviour. They also reported decreased problem behaviour, better parental attunement and parent-child interaction. We assessed 25/32 of the RCTs with high RoB, mainly related to homogeneity of the study population, lack of power, and performance bias. We concluded with due care that the effectivity of play-based interventions differed across RCTs, most reported improvements are found in ASD symptoms, everyday functioning, and parental attunement. In future research, findings should be replicated, taking account of the RoB. Show less
Individuals with sex chromosome trisomies ([SCT], XXX, XXY, and XYY)) are at increased risk for neurodevelopmental problems, given that a significant portion of the sex chromosome genes impact... Show moreIndividuals with sex chromosome trisomies ([SCT], XXX, XXY, and XYY)) are at increased risk for neurodevelopmental problems, given that a significant portion of the sex chromosome genes impact brain functioning. An elevated risk for psychopathology has also been described, including attention deficit-hyperactivity disorder (ADHD). The present study aimed at identifying early markers of ADHD, providing the first investigation of ADHD symptomology in very young children with SCT. The variety, type, and severity of ADHD symptomology in 1-6-year-old children with SCT (n = 104) were compared with population-based controls (n = 101) using the strengths and weaknesses of ADHD symptoms and normal-behavior (SWAN) parent-report questionnaire. ADHD symptomology was significantly more prevalent in SCT and already present from toddlerhood on, compared to controls. ADHD inattention symptoms were significantly increased in all karyotypes (XXX, XXY, and XYY), boys with XYY also showed significantly more hyperactivity/impulsivity symptoms than controls. Inattentiveness was more pronounced with increasing age for SCT, in contrast to controls. Within the SCT group, 24% of the children had significantly elevated ADHD symptoms at a clinical level. Already from an early age on, SCT is associated with a risk for ADHD, suggesting that its neurodevelopmental risk lies anchored in early brain maturation. Studying this genetically vulnerable population allows for the prospective study of risk markers to facilitate early and preventive interventions. Show less
BackgroundDespite the increasing interest in cardiac autonomic nervous activity, the normal development is not fully understood. The main aim was to determine the maturation of different cardiac... Show moreBackgroundDespite the increasing interest in cardiac autonomic nervous activity, the normal development is not fully understood. The main aim was to determine the maturation of different cardiac sympathetic-(SNS) and parasympathetic nervous system (PNS) activity parameters in healthy patients aged 0.5 to 20 years. A second aim was to determine potential sex differences.Methods and ResultsFive studies covering the 0.5- to 20-year age range provided impedance- and electrocardiography recordings from which heart rate, different PNS-parameters (eg, respiratory sinus arrhythmia) and an SNS-parameter (pre-ejection period) were collected. Age trends were computed in the mean values across 12 age-bins and in the age-specific variances. Age was associated with changes in mean and variance of all parameters. PNS-activity followed a cubic trend, with an exponential increase from infancy, a plateau phase during middle childhood, followed by a decrease to adolescence. SNS-activity showed a more linear trend, with a gradual decrease from infancy to adolescence. Boys had higher SNS-activity at ages 11 to 15 years, while PNS-activity was higher at 5 and 11 to 12 years with the plateau level reached earlier in girls. Interindividual variation was high at all ages. Variance was reasonably stable for SNS- and the log-transformed PNS-parameters.ConclusionsCardiac PNS- and SNS-activity in childhood follows different maturational trajectories. Whereas PNS-activity shows a cubic trend with a plateau phase during middle childhood, SNS-activity shows a linear decrease from 0.5 to 20 years. Despite the large samples used, clinical use of the sex-specific centile and percentile normative values is modest in view of the large individual differences, even within narrow age bands. Show less
Vermeulen, M.C.M.; Heijden, K.B. van der; Kocevska, D.; Treur, J.L.; Huppertz, C.; Beijsterveldt, C.E.M. van; ... ; Bartels, M. 2021
Children with SCT have an increased risk of suboptimal neurodevelopment. Previous studieshave shown an elevated risk for neurobehavioral problems in individuals with SCT.However, not much is known... Show moreChildren with SCT have an increased risk of suboptimal neurodevelopment. Previous studieshave shown an elevated risk for neurobehavioral problems in individuals with SCT.However, not much is known about neurobehavioral problems in very young children;knowledge that could help with early identification of children at risk for suboptimal development,and that could help establish targets for early intervention. This study addressedthe question of what the behavioral profile of children with SCT aged 1–5 yearslooks like. In total, 182 children aged 1–5 years participated in this study (NSCT=87,Nnonclinical controls = 95). Recruitment and assessment took place in the Netherlands and theUnited States. The SCT group was recruited through prospective follow-up (50%), informationseeking parents (31%), and clinical referral (18%). Behavioral profiles were assessedwith the child behavior checklist and the ages-and-stages social–emotional questionnaire.Levels of parent-rated problem behavior were higher in children with SCT. Difficultieswith overall social–emotional functioning were already present in 1-year-olds,and elevated scores were persistent across the full age range. Affective and pervasivedevelopmental behaviors were seen in late toddlerhood and prominent at preschoolage. Anxiety, attention deficit, and oppositional defiant behaviors were seen in preschool-aged children. Within this cross-sectional study, the developmental trajectoryof affective, pervasive developmental, and oppositional defiant behaviors seemed tobe different for SCT children than nonclinical controls.Collectively, these results demonstrate the importance of behavioral screening forbehavioral problems in routine clinical care for children with SCT from a young age.Social–emotional problems may require special attention, as these problems seem mostprominent, showing increased risk across the full age range, and with these problemsoccurring regardless of the timing of diagnosis, and across all three SCT karyotypes. Show less
Noten, M.M.P.G.; Heijden, K.B. van der; Huijbregts, S.C.J.; Goozen, S.H.M. van; Swaab, H. 2020
Impaired empathy has been associated with aggression in children, adolescents and adults, but results have been contradictory for the preschool period. Impaired inhibitory control also increases... Show moreImpaired empathy has been associated with aggression in children, adolescents and adults, but results have been contradictory for the preschool period. Impaired inhibitory control also increases the risk of aggression, and possibly moderates empathy-aggression associations. The current study investigated whether empathy and inhibitory control are associated with aggression in toddlerhood. Furthermore, we aimed to clarify the role of inhibitory control in empathy and aggression, specifically, whether inhibitory control moderates the association between empathy and aggression. During a laboratory visit at age 30 months (N = 103), maternal reports of physical aggression were obtained and child inhibitory control was examined using a gift delay task. Empathy was examined by obtaining behavioral observations and recording physiological responses (heart rate response and respiratory sinus arrhythmia response) to an empathy-eliciting event (i.e., simulated distress). Reduced inhibitory control was associated with more aggression. Behavioral and physiological indicators of empathy were not associated with aggression. Hierarchical regression analyses revealed an interaction effect of heart rate response to distress simulation with inhibitory control in the prediction of aggression. Post hoc analyses indicated a negative association between heart rate response and aggression when inhibitory control was high, but a positive association was found in toddlers who demonstrated low inhibitory control. These results suggest that children are less aggressive when they have both high levels of empathy and inhibitory control. Therefore, both empathy and inhibition are important targets for interventions aiming to reduce or prevent aggression at a young age. Show less
Dijkhuis, R.; De Sonneville, L.; Ziermans, T.; Swaab, H. 2020
Many students with autism spectrum disorders (ASDs) attending higher education drop out prematurely. The predictive value of self-reported daily executive functioning (EF) and (cognitive)... Show moreMany students with autism spectrum disorders (ASDs) attending higher education drop out prematurely. The predictive value of self-reported daily executive functioning (EF) and (cognitive) performance-based EF (mental flexibility and working memory) for academic progress was evaluated in 54 young adults with ASD (Mage = 22.5, SD = 2.4, 72% male). Regression analyses showed that autism symptom severity explained 12% of variance in academic progress, which was raised to 36% by adding self-reported daily EF, and to 25% by adding performance-based EF. It is suggested that EF is a candidate marker for academic progress in higher education students with ASD and a candidate target for early intervention. Show less
Dijkhuis, R.; Sonneville, L. de; Ziermans, T.; Staal, W.; Swaab, H. 2020
Sex chromosome trisomies (SCT) are among the most common chromosomal duplicationsin humans. Due to recent technological advances in non-invasive screening, SCTcan already be detected during... Show moreSex chromosome trisomies (SCT) are among the most common chromosomal duplicationsin humans. Due to recent technological advances in non-invasive screening, SCTcan already be detected during pregnancy. This calls for more knowledge about thedevelopment of (young) children with SCT. This review focused on neurocognitivefunctioning of children with SCT between 0 and 18 years, on domains of global intellectualfunctioning, language, executive functioning, and social cognition, in order toidentify targets that could benefit from early treatment.Online databases were used to identify peer-reviewed scientific articles using specificsearch terms. In total 18 studies were included. When applicable, effect sizes werecalculated to indicate clinical significance.Results of the reviewed studies show that although traditionally, the focus has been onlanguage and intelligence (IQ) in this population, recent studies suggest that executivefunctioning and social cognition may also be significantly affected already in childhood.These findings suggest that neuropsychological screening of children diagnosed withSCT should be extended, to also include executive functioning and social cognition.Knowledge about these neurocognitive risks is important to improve clinical care andhelp identify targets for early support and intervention programs to accommodatefor the needs of individuals with SCT. Show less
Abbing, A.C.; De Sonneville, L.; Baars, E.; Bourne, D.; Swaab, H. 2019
ObjectivesTo explore possible working mechanisms of anxiety reduction in women with anxiety disorders, treated with art therapy (AT).MethodsA RCT comparing AT versus waiting list (WL) condition on... Show moreObjectivesTo explore possible working mechanisms of anxiety reduction in women with anxiety disorders, treated with art therapy (AT).MethodsA RCT comparing AT versus waiting list (WL) condition on aspects of self-regulation. Stress regulation (heart rate and heart rate variability) and executive functioning (daily behavioural and cognitive performance aspects of executive functioning (EF)) were evaluated in a pre-post design. Participants were women, aged 18–65 years with moderate to severe anxiety symptoms.ResultsEffectiveness of AT compared to WL was demonstrated in a higher resting HRV post treatment, improvements in aspects of self-reported daily EF (emotion control, working memory, plan/organize and task monitor), but not in cognitive performance of EF, stress responsiveness and down regulation of stress. The decrease in anxiety level was associated with improvements in self-reported daily EF.ConclusionsAT improves resting HRV and aspects of EF, the latter was associated with art therapy-related anxiety reduction Show less
Adrichem, D.S. van; Huijbregts, S.C.J.; Heijden, K.B. van der; Goozen, S.H.M. van; Swaab, H. 2019
Parental beliefs, parenting behavior, and precursors of theory of mind have been related uniquely to each other and to early aggression, but have not yet been studied simultaneously. The present... Show moreParental beliefs, parenting behavior, and precursors of theory of mind have been related uniquely to each other and to early aggression, but have not yet been studied simultaneously. The present study combined these risk factors in the prediction of aggression during toddlerhood using a sample of 152 mother-child dyads. At 20 months, mothers' parental beliefs (parental self-efficacy and perceived parental impact) were examined with the Parental Cognitions and Conduct Toward the Infant Scale. Maternal parenting behavior (sensitivity, intrusiveness, and successful positive engagement) was observed during free play and teaching tasks, and children's precursors of theory of mind were assessed using a visual perspectives task and an imitation task. At 30 months, child aggression was examined using the Child Behavior Checklist. A regression analysis indicated that lower parental self-efficacy and lower imitation skills predicted more aggressive behavior. When estimating the indirect effects using bootstrapping, a final model was found indicating that lower perceived parental impact was related to less successful positive engagement, which, in turn, was associated with children's poorer imitation abilities which predicted more aggressive behavior. It can be concluded that aggression during toddlerhood is predicted significantly by interrelated parental beliefs, parenting behavior, and children's early social cognitive abilities. Show less
Objectives The aim of this study is to evaluate if language and executive functioning deficits in individuals with the 47,XXY chromosomal pattern contribute to emotion regulation problems and... Show moreObjectives The aim of this study is to evaluate if language and executive functioning deficits in individuals with the 47,XXY chromosomal pattern contribute to emotion regulation problems and related symptoms of psychopathology. Methods A group of 26 adult men with 47,XXY completed measures of cognitive emotion regulation strategies, neurocognitive functioning, and symptoms of psychopathology. Results Atypical emotion regulation strategies were found in the XXY group, with increased expression of emotions (69%), avoiding (65%), distraction seeking (54%), and passive coping (54%). More difficulties in mental flexibility and attention regulation, and speeded responding were associated with more pronounced emotion expression (emotional outbursts). Emotion regulation problems were associated with symptoms of anxiety, depression, thought problems, and hostility. Conclusion This study has identified emotion regulation as a potential target for treatment and intervention, with a specific focus on executive functions in the management of emotions in individuals with 47,XXY. Show less
Noten, M.M.P.G.; Heijden, K.B. van der; Huijbregts, S.C.J.; Goozen, S.H.M. van; Swaab, H. 2019
Although emotional responses are theorized to be important in the development of empathy, findings regarding the prediction of early empathic behavior by infant behavioral and physiological... Show moreAlthough emotional responses are theorized to be important in the development of empathy, findings regarding the prediction of early empathic behavior by infant behavioral and physiological responses are mixed. This study examined whether behavioral and physiological responses to mild emotional challenge (still face paradigm and car seat task) in 118 infants at age 6 months predicted empathic distress and empathic concern in response to an empathy-evoking task (i.e, experimenter's distress simulation) at age 20 months. Correlation analyses, corrected for sex and baseline levels of physiological arousal, showed that stronger physiological and behavioral responses to emotional challenge at age 6 months were positively related to observed empathic distress, but not empathic concern, at age 20 months. Linear regression analyses indicated that physiological and behavioral responses to challenge at 6 months independently predicted empathic distress at 20 months, which suggests an important role for both physiological and behavioral emotional responses in empathy development. In addition, curvilinear regression analyses showed quadratic associations between behavioral responses at 6 months, and empathic distress and empathic concern at 20 months, which indicates that moderate levels of behavioral responsivity predict the highest levels of empathic distress and empathic concern. Show less
Adrichem, D.S. van; Huijbregts, S.C.J.; Heijden, K.B. van der; Goozen, S.H.M. van; Swaab, H. 2019
Prenatal risk and a lack of inhibitory control have consistently been related to the development of physical aggression in older children. This study examined whether inhibitory control mediated... Show morePrenatal risk and a lack of inhibitory control have consistently been related to the development of physical aggression in older children. This study examined whether inhibitory control mediated the relation between prenatal risk and aggression in infants and toddlers. The role of gender in this mediation model was also examined. The sample consisted of 161 mother-child dyads (83 boys). A prenatal cumulative risk score was created from a number of well-established risk factors including maternal psychopathology, substance use, and social and socioeconomic disadvantages. At 12 months, children performed an inhibitory control task. Physical aggression was assessed through maternal reports at 12 and 20 months of age. Results showed that higher prenatal risk was associated with more physical aggression. Inhibitory control mediated this association at both 12 and 20 months: higher prenatal risk was related to lower inhibitory control, which in turn led to higher aggression. At 20 months, gender moderated the mediation effect: the mediating role of inhibitory control was only found for girls. These results suggest that even before 2 years of age, inhibitory control is an important construct involved in the relation between prenatal risk and physical aggression. Show less
