BACKGROUND:

Studies conducted on mild intellectual disability (MID) in children are infrequent and the prevalence rates vary widely. This study aimed to estimate the prevalence of MID in children in a French county (Isère), to describe the clinical signs and associated comorbidities, and to specify the aetiologies of this disability.

METHODS:

The target population was comprised of the 15 100 children born in 1997 residing in Isère County, France, in 2008. Our goal was to find the children in this group with MID diagnosed between 9 and 13 years of age. MID was defined as an overall IQ score of between 50 and 69 [International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10)]; this definition was adjusted for the study by integrating confidence intervals so that the risk of IQ measurement relativity and possible discrepancy of scores could be taken into account. Children were identified through an administrative data source designed to assist disabled persons that contains health information, and an educational data source. Parents who agreed to let their children participate responded to an in-depth questionnaire on their child's medical and academic history. A genetic investigation was proposed for those children whose MID had an unknown aetiology.

RESULTS:

The preliminary selection included 267 children, resulting in a prevalence rate of 18 per 1000 (CI [15.6; 19.9]), within the expected mean. Of these 267 cases, 181 families agreed to participate in the study (68%). MID more often affected boys [male gender ratio = 1.4 (CI [1.2; 1.6])], low socioeconomic groups, and families with a history of intellectual disability. The clinical signs and comorbidities associated with MID were very frequent, with 54% spoken language disorders and 10% pervasive developmental disorder. Only 9% of the children had undergone a genetic investigation before the study. The known aetiology rate for MID was 19% among all the children who had had genetic tests performed.

CONCLUSION:

MID is an important public health issue based on its prevalence. The associated clinical signs and comorbidities may be warning signs of MID in case of learning difficulties. This study may help decision-makers to develop and organise screening and care for MID.

BACKGROUND:

Obesity is a major health problem in people with intellectual disabilities. It is also widely accepted that low-grade systemic inflammation associated to obesity plays a key role in the pathogenic mechanism of several disorders. Fortunately, physical activity has shown to improve inflammation in people with metabolic syndrome and type 2 diabetes. Accordingly, we assessed the influence of aerobic training on pro-inflammatory cytokines and acute phase proteins in women with Down syndrome.

METHODS:

To achieve this outcome, 20 premenopausal obese young women with Down syndrome volunteered for this study. Eleven were randomly assigned to the intervention group and performed a 10-week aerobic training programme, three sessions per week, consisting of a warm-up then a 30- to 40-min treadmill exercise at a work intensity of 55-65% of peak heart rate followed by a cooling-down period. The control group included nine age-, sex- and body mass index-matched women with Down syndrome. Fat mass percentage and fat distribution were measured. Plasmatic levels of tumour necrosis factor (TNF)-α, interleukin (IL)-6 and fibrinogen were assessed by commercial enzyme-linked immunosorbent assay kits. C-reactive protein (CRP) was assessed by nephelometry.

RESULTS:

Plasmatic levels of TNF-α (11.7 ± 1.6 vs. 9.2 ± 1.3 pg/ml; P = 0.022), IL-6 (8.2 ± 1.1 vs. 6.1 ± 0.9 pg/ml; P = 0.014) and high sensitive CRP (0.62 ± 0.11 vs. 0.53 ± 0.09 mg/dl; P = 0.009) were significantly reduced in the intervention group. Further, significant correlations between plasmatic and anthropometric parameters were found.

CONCLUSION:

A 10-week training programme reduced pro-inflammatory cytokines and acute phase proteins in obese young women with Down syndrome. Long-term, well-conducted studies are still required to determine whether correction of this low-grade inflammation improves clinical outcomes of women with trisomy 21.

BACKGROUND:

Children with 22q11.2 deletion syndrome (22q11DS) are at risk for social-behavioural and neurocognitive sequelae throughout development. The current study examined the impact of family environmental characteristics on social-behavioural and cognitive outcomes in this paediatric population.

METHOD:

Guardians of children with 22q11DS were recruited through two medical genetics clinics. Consenting guardians were asked to complete several questionnaires regarding their child's social, emotional and behavioural functioning, as well as family social environment and parenting styles. Children with 22q11DS were asked to undergo a cognitive assessment, including IQ and achievement testing, and measures of attention, executive function and memory.

RESULTS:

Modest associations were found between aspects of the family social environment and parenting styles with social-behavioural and cognitive/academic outcomes. Regression models indicated that physical punishment, socioeconomic status, parental control and family organisation significantly predicted social-behavioural and cognitive outcomes in children with 22q11DS.

CONCLUSION:

Characteristics of the family social environment and parenting approaches appear to be associated with functional outcomes of children with 22q11DS. Understanding the impact of environmental variables on developmental outcomes can be useful in determining more effective targets for intervention. This will be important in order to improve the quality of life of individuals affected by 22q11DS.

BACKGROUND:

The developmental disorder Williams syndrome (WS) is characterised by a distinctive cognitive profile and an intriguing social phenotype. Individuals with the disorder are often highly social engaging with familiar and unfamiliar people and once in an interaction they often show subtle abnormalities of social behaviour. Atypically increased approach to unfamiliar people is widely reported in the existing literature for both children and adults. Parents frequently report interactions with unfamiliar people as a major concern.

METHODS:

In this study we aimed to evaluate 'stranger danger' awareness using a video vignette task with individuals who had WS. When linked to other components of the WS phenotype (e.g. reduced intellectual ability, increased social approach) an awareness of stranger danger is particularly important.

RESULTS:

Qualitative and quantitative data showed that young people with WS have difficulties making judgements about whether or not to trust and engage in conversation with unfamiliar people. Qualitative data showed that individuals with WS often suggested that they would engage in an interaction with an unfamiliar person.

CONCLUSIONS:

The findings have substantial implications for the safety of young people with the disorder and emphasise the need for intervention regarding this behaviour.

PURPOSE:

The purpose of this study was to examine the psychometric properties and the theoretical structure of the Parenting Stress Index-short form (PSI-SF) with Jordanian parents of children with autistic disorder.

METHODS:

Using a cross-sectional design for data collection, the convenience sample of the study was composed of 184 Jordanian parents of children with autistic disorder. The factor structure for the PSI-SF was examined using confirmatory and exploratory factor analyses.

RESULTS:

We found that the modified three-factor model (30 items) fits the data significantly better than the 36-item model. The results showed that the 12 items of the Parental Distress sub-scale support the original scale structure. However, items in the Parent-Child Dysfunctional Interaction and Difficult Child sub-scales did not show stability in their structure. The results in this study showed that the PSI-SF in its 30-item model has endorsed the necessary validity of the scale with parents of children with autistic disorder. The study provides information on the effects of Arab culture on the validity of PSI-SF.

CONCLUSION:

It is recommended to use the new factors structure of the PSI-SF with the 30 items in the studies that intend to examine the stress among parents with children with autistic disorder in the Arab world.

BACKGROUND:

Aggressive behaviour is associated with certain personality traits in both the general population and among individuals with mental health problems, but little attention has been paid to the relationship between aggressive behaviour and personality among individuals with intellectual disabilities (ID). The aim of this study was to circumscribe personality profiles associated with aggressive behaviour among individuals with ID.

METHOD:

In this cross-sectional study of 296 adults with mild or moderate ID, information on mental health, personality and aggressive behaviour was gathered through structured interviews with the ID participants and their case manager, and a review of client files.

RESULTS:

The results of the Reiss Profile were submitted to hierarchical cluster analysis method. Subsequently, the distribution of aggressive behaviour, sociodemographic characteristics and clinical characteristics across personality profiles was analysed. The analyses yielded seven distinct personality profiles in relation to patterns of aggressive behaviour: Pacifists, Socials, Confidents, Altruists, Conformists, Emotionals and Asocials.

CONCLUSION:

The identification of distinct personality profiles sheds light on the risk factors for aggressive behaviour, and suggests new approaches to improving diagnostic and intervention strategies.

BACKGROUND:

Down syndrome (DS) affects not only children but also their families. Much remains to be learned about factors that influence how families of children with DS function, especially families in non-Western populations. The purpose of this cross-sectional, correlational study was to examine how family demographics, family demands and social support relate to family functioning as well as the potential mediating effect of social support on the relationship between family demands and family functioning in Taiwanese families of children with DS.

METHOD:

One hundred and fifty-five parents (80 mothers and 75 fathers) from 83 families independently completed mailed questionnaires. Data were analysed using a principal component analysis and mixed linear modelling.

RESULTS:

Families having older children with DS, greater parental education, higher family income, fewer family demands and greater social support contributed to healthier family functioning. Social support partially mediated the effects of family demands on family functioning.

CONCLUSIONS:

Family demographics, family demands and social support appear to be important factors that may play a critical role in how Taiwanese families respond to the birth of a child with DS. Care of children with DS and their families is likely to be more effective if professionals working with these families are aware of factors that contribute to healthy family functioning.

BACKGROUND:

Although students with mild intellectual disability (MID) present unique educational needs and considerations, in research and in practice, they are sometimes aggregated with students with learning disabilities and emotional disorders and considered mild disabilities or aggregated with students with moderate/severe intellectual disability and labelled as intellectual disability.

METHOD:

This study is a secondary analysis of the NLTS2 data to understand the immediate (i.e. within 2 years) and longer-term outcomes (i.e. within 4 years, within 6 years and within 8 years) of secondary students with MID. Frequency distributions and a significant test were conducted to analyse data from the NLTS2.

RESULTS:

Students with MID struggled with postschool success when considering employment, postsecondary education, and independent living. Across the span of time since graduation (i.e. within 2 years, within 4 years, within 6 years, and within 8 years), a lack of consistent pattern existed, in general, for these students with regards to outcomes. Students did not necessarily improve or decline in their outcomes the longer they were out of school.

CONCLUSIONS:

The postschool outcome data warrant critical examination of the factors contributing to the poor outcomes. The field needs to systematically understand what schools can control with regards to improved outcomes for students with MID - particularly employment regardless of the length of time out of school and independent living as the time since school exit increases - and then implement such practices.

BACKGROUND:

People with intellectual disability (ID) and epilepsy are more likely to die prematurely than the general population. A significant number of deaths in people with epilepsy may be potentially preventable through better seizure control, regular monitoring and raising awareness among patients and carers. The aim of this project was to study mortality from sudden unexpected death in epilepsy (SUDEP) in adults with ID.

METHODS:

All adults (≥20 years old) living in Leicester city, Leicestershire and Rutland, UK, with ID between 1993 and 2010 were identified using the Leicestershire Intellectual Disability Register database. People with and without ID who died during the same period were identified using death certificate data from the Office for National Statistics (ONS). Deaths from probable and definite SUDEP were identified. Additional information on adults with ID who had died from probable or definite SUDEP was obtained from case notes and post-mortem reports, where available. Cases of probable and definite SUDEP in adults with ID were compared with the general population using standardised mortality ratios (SMRs).

RESULTS:

A total of 898 adults with ID had died over the 18-year study period. Of these, 244 deaths (27%) occurred in people with ID who had a diagnosis of epilepsy. Twenty-six people with ID died from probable or definite SUDEP, which was the second most common cause of death among adults with ID and epilepsy. All-cause specific SMRs were 2.2 [95% confidence interval (CI): 2.0-2.4] and 2.8 (95% CI: 2.5-3.1) for men and women with ID respectively. SMRs were 3.2 (95% CI: 2.7-3.8) and 5.6 (95% CI: 4.6-6.7) for men and women with epilepsy and ID respectively. During the same study period, 83 adults without ID had died of probable or definite SUDEP. The SMRs for SUDEP in patients with ID were 37.6 for men (95% CI: 21.9-60.2) and 52.0 for women (95% CI: 23.8-98.8). We found that in the majority of ID cases there was little detailed documentation on the circumstances surrounding deaths, no communication with patients/carers about risk of SUDEP and an absence of post-mortem reports or carers' referral for bereavement counselling.

CONCLUSION:

The authors believe that a comprehensive risk management under a multiagency/multidisciplinary framework should be undertaken for all adults with ID and epilepsy in day-to-day clinical practice to reduce mortality in people with ID.

BACKGROUND:

Several cross-sectional studies have shown an association between exposure to life events and psychological problems in adults with intellectual disability (ID). To establish life events as a risk factor, prospective designs are needed.

METHODS:

Support staff informants provided data on the psychological problems of 68 adults with ID and their recent exposure to life events. Using data collected on the same sample 3.5 to 4 years earlier, prospective analysis of the relationships between life events exposure and psychological problems over time was explored.

RESULTS:

Hierarchical linear regression analyses demonstrated a contribution of life events to the prediction of later psychological problems after controlling for earlier psychological problems. Exploratory analyses showed that the relationship between life events and psychological problems might be unidirectional, and non-spurious; remaining present once the impact of other correlates of psychological problems was controlled.

CONCLUSIONS:

These data offer support for the status of life events (with a negative valence) as a risk factor for psychological problems in adults with ID. To establish life events as a causal risk factor, research is needed to examine the mechanisms via which life events have their impact on psychological well-being.