Patients with Chromosome 11q Deletions Are Characterized by Inborn Errors of Immunity Involving both B and T Lymphocytes.

Disorders of the long arm of chromosome 11 (11q) are rare and involve various chromosomal regions. Patients with 11q disorders, including Jacobsen syndrome, often present with a susceptibility for bacterial and prolonged viral and fungal infections partially explained by hypogammaglobulinemia. Additional T lymphocyte or granular neutrophil dysfunction may also be present. In order to evaluate infectious burden and immunological function in patients with 11q disorders, we studied a cohort of 14 patients with 11q deletions and duplications. Clinically, 12 patients exhibited prolonged and repetitive respiratory tract infections, frequently requiring (prophylactic) antibiotic treatment (n = 7), ear-tube placement (n = 9), or use of inhalers (n = 5). Complicated varicella infections (n = 5), chronic eczema (n = 6), warts (n = 2), and chronic fungal infections (n = 4) were reported. Six patients were on immunoglobulin replacement therapy. We observed a high prevalence of low B lymphocyte counts (n = 8), decreased T lymphocyte counts (n = 5) and abnormal T lymphocyte function (n = 12). Granulocyte function was abnormal in 29% without a clinical phenotype. Immunodeficiency was found in patients with terminal and interstitial 11q deletions and in one patient with terminal 11q duplication. Genetically, FLI1 and ETS1 are seen as causative for the immunodeficiency, but these genes were deleted nor duplicated in 4 of our 14 patients. Alternative candidate genes on 11q may have a role in immune dysregulation. In conclusion, we present evidence that inborn errors of immunity are present in patients with 11q disorders leading to clinically relevant infections. Therefore, broad immunological screening and necessary treatment is of importance in this patient group.

Attentional control and executive functioning in school-aged children: Linking self-regulation and parenting strategies.

Good parenting strategies can shape children's neurocognitive development, yet little is known about the nature of this relation in school-aged children and whether this association shifts with age. We aimed to investigate the relation between parenting strategies observed during a home visit and children's performance-based attentional control and executive functioning (N=98, aged 4-8years). Linear and curvilinear regression analyses showed that children of parents who were more supportive, were less intrusive, and asked more open-ended questions displayed better inhibitory control. In addition, children of parents who asked relatively more open-ended than closed-ended questions showed better performance on inhibition, working memory, and cognitive flexibility tasks. Curvilinear relations indicated the presence of an optimal amount of closed-ended and elaborative questions by parents-that is, not too few and not too many-which is linked to increased performance on attentional and inhibitory control in children. Higher parental intrusiveness and more frequent elaborative questioning were associated with decreased inhibitory control in younger children, whereas no such negative associations were present in older children. These results suggest that susceptibility to certain parenting strategies may shift with age. Our findings underscore the importance of adaptive parenting strategies to both the age and needs of school-aged children, which may positively affect their self-regulation skills.

Educating parents to improve parent-child interactions: Fostering the development of attentional control and executive functioning.

BACKGROUND: Parent-child interaction is essential in the development of attentional control (AC) and executive functioning (EF). Educating parents in AC and EF development may help them to respond more adaptively to their child's developmental needs. AIM: This study aimed to investigate whether parents can be educated to improve interactions with their child through a compact psycho-educational programme that focuses on fostering the development of AC and EF. SAMPLE: Parents and their children in a low-risk sample of four- to eight-year-olds were randomly assigned to either the educational programme condition (N = 34) or the control condition (N = 36). METHODS: Parental supportive presence and intrusiveness were observed during home visits, and children's performance-based AC and EF were assessed before and after the four-session programme. RESULT: Parents in the educational programme improved significantly in support ( η p 2  = .19) and intrusiveness ( η p 2  = .09) compared to controls. There was no short-term programme mediation effect on child AC and EF through parental support and intrusiveness. This study showed, however, that parents who improved after the educational programme had children who improved on AC and EF. CONCLUSION: Parent-child interaction can be enhanced in a low-risk sample of four- to eight-year-olds using a compact educational group programme within the school community. Future studies should aim at examining variations in programme responsiveness and assessing associations between parent-child interaction and AC and EF over time.

Self-reported adverse childhood experiences and quality of life among children in the two last grades of Dutch elementary education.

BACKGROUND: Adverse Childhood Experiences (ACEs) may have a life-long impact on mental health and are related to physical disease, such as diabetes and cardiovascular diseases in adulthood. Research on ACEs suffers from recall bias when performed with adults. OBJECTIVE: To estimate the prevalence of ACEs and the interrelationships between ACEs as reported by children, and to determine the impact on their self-reported quality of life (QoL). Children's opinions on the ACE-Questionnaire were also obtained. METHOD: A cross-sectional study was conducted with a child version of the ACE-Questionnaire. This questionnaire assesses parental separation or divorce, physical and emotional child abuse and neglect, sexual violence, domestic violence, household substance abuse, psychological issues or suicide, and incarceration of a household member. QoL was measured with the Kidscreen-10. PARTICIPANTS AND SETTING: The questionnaire was completed by 644 children at a mean age of 11 years (range 9-13 years), in the two last grades of regular elementary schools, recruited throughout the Netherlands. RESULTS: Data were weighted by ethnicity to obtain a representative sample of children in Dutch elementary education. Of all children, 45.3% had one or more out of ten ACEs. Child maltreatment was experienced by 26.4%. ACEs often co-occurred. A higher number of ACEs correlated with a lower mean level of QoL (p <  0.001). Mean QoL was 8.5 points lower (Cohen's d = 0.8) in children who experienced child maltreatment. Children's opinions on the questionnaire were positive in 82.4%. CONCLUSION: Prevention of ACEs, professional training and trauma-focus in schools are urgently needed.

Linking Parenting and Social Competence in School-Aged Boys and Girls: Differential Socialization, Diathesis-Stress, or Differential Susceptibility?

Girls generally demonstrate superior skill levels in social competence compared to boys. The exact relations of parenting with these gender differences are currently unclear. Gender differences may occur due to exposure to different parenting strategies (differential socialization model) or due to a different impact of similar parenting strategies for boys and girls (differential susceptibility and diathesis-stress model). Objective: In this study we assessed both hypotheses using a multi-method multi-informant approach. We investigated (1) to what extent different parenting strategies mediate the relation between gender and social competence and (2) whether gender and age moderate the relation between parenting strategies and social competence. Design: Parenting strategies were observed during home visits and social competence was assessed using parent and teacher questionnaires and performance-based neurocognitive tasks (N = 98, aged 4 to 8). Results: (1) Parenting strategies did not mediate the relation between gender and social competence. (2) Gender moderated the association between parental questioning style and children's level of social competence: parents asking fewer questions was associated with poorer social cognitive skills in boys only. Parental supportive presence and intrusiveness were related to aspects of social competence irrespective of gender. Age moderated the relation between parenting and aspects of social competence, though in various (unexpected) directions. Conclusion: Our findings do not support the differential socialization hypothesis and provide partial evidence for a diathesis-stress model as an explanation for parental influence on gender differences in social competence.

Antisocial and delinquent behaviors in youths with mild or borderline disabilities.

Six types of antisocial and delinquent behaviors (e.g., property destruction and authority avoidance) were assessed in 526 youths (11 to 24 years of age) with mild to borderline intellectual disabilities and 1,030 11- to 18-year-olds without intellectual disabilities. Overall, 10% to 20% of youths with intellectual disabilities exhibited some type of antisocial and delinquent behavior, which were quite persistent over a 5-year period. Youths who exhibited one type of antisocial and delinquent behavior were likely to also exhibit other types of such behaviors. Being male, younger, and having behavioral problems particularly predicted these behaviors across a 5-year period. Overall, boys but not girls with intellectual disabilities exhibited antisocial and delinquent behaviors more often than peers without intellectual disabilities. Clinical implications and implications for future research are discussed.

Short form of the developmental behaviour checklist.

A 24-item short form of the 96-item Developmental Behaviour Checklist was developed to provide a brief measure of Total Behaviour Problem Score for research purposes. The short form Developmental Behaviour Checklist (DBC-P24) was chosen for low bias and high precision from among 100 randomly selected item sets. The DBC-P24 was developed from epidemiological data in the first three waves of the Australian Child to Adult Development study, and cross validated for groups with autism, fragile X, Prader-Willi, and Williams in this longitudinal study and in cross sectional Dutch, English, and Finnish samples of young people with intellectual disability. The DBC-P24 has low bias and high precision in cross-validation samples and achieves high sensitivity and specificity to full DBC-P based caseness decisions.

Cognitive, Parent and Teacher Rating Measures of Executive Functioning: Shared and Unique Influences on School Achievement.

Very little is known about the relative influence of cognitive performance-based executive functioning (EF) measures and behavioral EF ratings in explaining differences in children's school achievement. This study examined the shared and unique influence of these different EF measures on math and spelling outcome for a sample of 84 first and second graders. Parents and teachers completed the Behavior Rating Inventory of Executive Function (BRIEF), and children were tested with computer-based performance tests from the Amsterdam Neuropsychological Tasks (ANT). Mixed-model hierarchical regression analyses, including intelligence level and age, showed that cognitive performance and teacher's ratings of working memory and shifting concurrently explained differences in spelling. However, teacher's behavioral EF ratings did not explain any additional variance in math outcome above cognitive EF performance. Parent's behavioral EF ratings did not add any unique information for either outcome measure. This study provides support for the ecological validity of performance- and teacher rating-based EF measures, and shows that both measures could have a complementary role in identifying EF processes underlying spelling achievement problems. The early identification of strengths and weaknesses of a child's working memory and shifting capabilities, might help teachers to broaden their range of remedial intervention options to optimize school achievement.

Self-reports on mental health problems of youth with moderate to borderline intellectual disabilities.

OBJECTIVE: To determine the extent to which the Youth Self-Report (YSR) can be used to assess emotional and behavioral problems in adolescents with intellectual disabilities (IDs). METHOD: In 2003, 281 11- to 18-year-olds with IDs (IQ > or =48) completed the YSR in an interview, and in 1993, 1,047 non-ID adolescents completed the YSR themselves. Parents completed the Child Behavior Checklist (CBCL). The ID sample was split into lower (IQ 48-69) and higher (IQ > or =70) IQ groups. Cronbach's alpha values of the YSR scales and (intraclass) correlation coefficients between and within YSR and CBCL scale scores were calculated to determine parent-adolescent agreement and YSR construct validity, which were compared between samples. Mean YSR scale scores were compared between adolescents with ID with and without psychiatric symptoms. RESULTS: Cronbach's alpha, parent-adolescent agreement, and indications of construct validity were about similar in all samples, although discriminant validity was somewhat weaker in the lower IQ group. Mean scale scores were 1.5 to 2.0 times higher for ID adolescents with psychiatric symptoms. CONCLUSIONS: The YSR seems applicable in youth with an IQ > or =48. Further research is needed to refine and confirm these findings and the factor structure of the YSR in adolescents with ID and to differentiate between adolescents with moderate and mild IDs.

Help-seeking process of parents for psychopathology in youth with moderate to borderline intellectual disabilities.

OBJECTIVE: To study the help-seeking process of parents for emotional or behavioral problems in their child with borderline to moderate intellectual disabilities. METHOD: In 2003, in a special education-based sample of 522 youths (ages 10-18 years, response = 77.9%), we studied the parents' perception of their child's problems, their subsequent felt need for professional help, actual help-seeking, and the factors possibly related to taking these steps. RESULTS: Even when parents indicated their child's emotional or behavioral functioning as "neither good nor bad," in about 70%, these problems were present according to standardized measures. Of the 213 parents (40.8%) who perceived problems, 70.6% felt a need, and 55.2% of these parents subsequently sought professional help. Parents more often sought help when their child had problems of anxiety and depression, experienced negative life events, and when parents perceived child psychopathology before the past year. Reported barriers to seeking help predominantly related to parents' evaluation of the severity of these problems and wanting to solve the problems themselves. CONCLUSIONS: Clinicians and other service providers should address parents' concerns regarding their child's emotional/behavioral functioning and treatment seeking. Also, they should provide information on treatment options and on signs and potential negative prospects of their child's problems.

DSM-IV disorders in children with borderline to moderate intellectual disability. I: prevalence and impact.

OBJECTIVE: To assess the prevalence, comorbidity, and impact of DSM-IV disorders in 7- to 20-year-olds with intellectual disability. METHOD: A total of 474 children (response 86.8%) were randomly selected from a sample of students from Dutch schools for the intellectually disabled. Parents completed the anxiety, mood, and disruptive disorder modules of the Diagnostic Interview Schedule for Children. RESULTS: A total of 21.9% of the children met the DSM-IV symptom criteria for anxiety disorder, 4.4% for mood disorder, and 25.1% for disruptive disorder. Similar prevalence rates were found for children who screened positive or negative for pervasive developmental disorder. More than half of the children meeting the criteria for a DSM-IV disorder were severely impaired in everyday functioning, and about 37% had a comorbid disorder. Children with multiple disorders were more likely to be impaired across various areas of everyday functioning. Almost 27% of the diagnosed children received mental health care in the last year. Comorbidity and impairment in everyday functioning increased the likelihood of referral. CONCLUSIONS: Most disorders can be observed in intellectually disabled children. Impairment and comorbidity are high. The finding that less than one third of the children with a psychiatric disorder receive mental health care deserves attention.

DSM-IV disorders in children with borderline to moderate intellectual disability. II: child and family predictors.

OBJECTIVE: To identify child and family factors that predict DSM-IV disorders in children with intellectual disability. METHOD: In 1997, a total of 968 6- to 18-year-olds were randomly selected from Dutch schools for intellectual disability (response 69.3%). Parents completed the Child Behavior Checklist, Developmental Behavior Checklist, Vineland Screener, and instruments addressing their child's physical health, family functioning, and parental mental health. One year later, parents of 474 children, randomly selected from the 1997 participants (response 86.8%), completed the anxiety, mood, and disruptive disorder modules of the Diagnostic Interview Schedule for Children-IV. RESULTS: Both child and family factors were significantly related to DSM-IV outcome 1 year later. Social incompetence, inadequate daily living skills, child health problems, negative life events, emotional and behavioral problems, and parental mental health problems were the strongest predictors of DSM-IV disorders 1 year later. After correcting for the level of behavioral problems in the previous year, the first four factors proved to be significant risk factors for DSM-IV outcome. CONCLUSIONS: These factors can improve the identification of children at risk and point to topics that need attention in diagnostic and intervention procedures.

Assessing emotional and behavioral problems in children with intellectual disability: revisiting the factor structure of the developmental behavior checklist.

The objective of the reported study was to reassess the factor structure of the Developmental Behaviour Checklist (DBC) in a large cross-cultural sample representing all levels of intellectual disability. Parent and teacher DBC ratings on a combined sample of 1536 Dutch and Australian children and adolescents (ages 3-22) with mild to profound intellectual disability were used. Principal components analyses produced five subscales: Disruptive/Antisocial, Self-Absorbed. Communication Disturbance, Anxiety, and Social Relating, explaining 43.7% of the total variance. Internal consistencies of these subscales ranged from .66 to .91. The revised factor structure of the DBC appears to be an improved and useful tool for assessing emotional and behavioral problems in children with intellectual disabilities.

Developmental course of psychopathology in youths with and without intellectual disabilities.

BACKGROUND: We aimed to describe similarities and differences in the developmental course of psychopathology between children with and without intellectual disabilities (ID). METHODS: Multilevel growth curve analysis was used to analyse the developmental course of psychopathology, using the Child Behavior Checklist (CBCL), in two longitudinal multiple-birth-cohort samples of 6- to 18-year-old children with ID (N = 978) and without ID (N = 2,047) using three repeated measurements across a 6-year period. RESULTS: Children with ID showed a higher level of problem behaviours across all ages compared to children without ID. A significant difference between the samples in the developmental courses was found for Aggressive Behaviour and Attention Problems, where children with ID showed a significantly larger decrease. Gender differences in the development of psychopathology were similar in both samples, except for Social Problems where males with ID showed a larger decrease in problem behaviour across time than females with ID and males and females without ID. CONCLUSIONS: Results indicate that children with ID continue to show a greater risk for psychopathology compared to typically developing children, although this higher risk is less pronounced at age 18 than it is at age 6 for Aggressive Behaviour. Contrary to our expectations, the developmental course of psychopathology in children with ID was quite similar from age 6 to 18 compared to children without ID. The normative developmental trajectories of psychopathology in children with ID, presented here, can serve as a yardstick against which development of childhood psychopathology can be detected as deviant.

Developmental trajectories of depressive symptoms from early childhood to late adolescence: gender differences and adult outcome.

BACKGROUND: Limited information is available on gender differences and young-adult poor outcome in children and adolescents following distinct developmental trajectories of depressive symptoms. METHODS: Parent information on depressive symptoms of 4- to 18-year-olds from an ongoing Dutch community-based longitudinal multiple-cohort study (N = 2,076) was used to estimate trajectories from semi-parametric mixture models. The identified trajectories were used to predict depressive problems, general mental health problems, referral to mental health care, and educational attainment in young adulthood. RESULTS: In both genders six distinct developmental trajectories were identified. Gender differences existed not only in level, but also in shape and timing of onset of depressive problems. Only in girls was a chronic trajectory of early childhood-onset depression identified. In both boys and girls a group with increasing levels of depressive symptoms was identified that reached a high level around adolescence, although boys showed an earlier onset. Two decreasing trajectories were found in boys, one reaching normative levels of depressive symptoms around late childhood and one around mid-adolescence, while none was found for girls. Individuals who followed elevated trajectories during their whole childhood or starting at adolescence had significantly more depressive and other mental health problems in young adulthood compared to those who followed normative trajectories. Boys in these elevated trajectories showed lower educational attainment, while girls were more likely to have been referred to mental health care. CONCLUSIONS: This study shows the value of estimating growth-mixture models separately for boys and girls. Girls with early childhood or adolescence-onset depressive problems and boys with depressive problems during childhood or starting in adolescence are especially at risk for poor outcome as young adults and should be considered candidates for intervention.

Emotional and behavioral problems in children and adolescents with and without intellectual disability.

BACKGROUND: The main objective of this study was to assess and compare the prevalence of a wide range of emotional and behavioral problems in children with and without intellectual disability (ID). METHODS: We studied 1,041 non-residential children randomly selected from special schools for educable (IQ 60 to 80) and trainable (IQ 30 to 60) children without severe additional physical or sensory impairments, and compared them to 1,855 children randomly selected from the general population (both ages 6 to 18). Parents completed the Child Behavior Checklist (CBCL), and teachers the Teacher's Report Form (TRF). RESULTS: Controlling for sex, age, and socioeconomic status, we found that both educable and trainable children had significantly higher mean scores on all CBCL and TRF scales than children without ID, except for trainable children on the scales Anxious/Depressed and Somatic Complaints. Almost 50% of children with ID had a Total Problem score in the deviant range compared to about 18% in children without ID. Compared to children without ID, the most prominent problem behaviors of educable children were Social Problems, Attention Problems, and Aggressive Behavior, and trainable children had an increased risk for Social Problems, Attention Problems, Withdrawn and Thought Problems. CONCLUSIONS: Elevated scale scores reflected differences between children with and without ID over a broad range of items, and not solely on items more likely to be related to developmental delay. Therefore, problem areas covered by the items in these scales deserve special attention in the mental health care of children with ID.