Are motivational deficits part of the behavioural phenotype of Down syndrome?

Motivational deficits are generally accepted to be part of the behavioural phenotype associated with Down syndrome (DS). A motivational profile comprising low or inconsistent levels of task persistence, avoidance of challenging activities and over-dependence on adult direction has been described. However, comparisons are usually made between children with DS and those who are developing typically, without the inclusion of samples with intellectual disability (ID) from aetiologies other than DS. Such comparisons are needed to determine the extent to which motivational deficits are specific to DS, as opposed to being a feature of ID generally. Methods: The current study collected data about the personality-motivation profiles of children in three groups matched for mental age. They consisted of 80 typically developing (TD)3–7 year old children, 62 children with DS aged 7–15 years, and 54 children with moderate ID aged 7–15 years. Parents completed the 37-item EZ-Personality Questionnaire (EZPQ; Zigler et al., 2002), a measure of personality-motivational functioning. Results: There were significant differences between TD children and those with ID on all EZPQ scales. In most respects children with DS did not differ significantly from others with moderate ID, although they were rated as having greater expectancy of success and fewer negative reactions. Conclusion: The finding that children with DS are less motivated than TD children of the same mental age is consistent with previous studies in which parents have rated motivation. It seems, however, that motivation difficulties are associated with ID more generally, rather than being specific to those with DS. The study raises questions about phenotypic versus experiential effects on motivation for children with ID.

Investigating the change in three dimensional deformity for idiopathic scoliosis using axially loaded MRI

Background: Adolescent idiopathic scoliosis is a complex three-dimensional deformity, involving a lateral deformity in the coronal plane and axial rotation of the vertebrae in the transverse plane. Gravitational loading plays an important biomechanical role in governing the coronal deformity, however, less is known about how they influence the axial deformity. This study investigates the change in three-dimensional deformity of a series of scoliosis patients due to compressive axial loading. Methods: Magnetic resonance imaging scans were obtained and coronal deformity (measured using the coronal Cobb angle) and axial rotations measured for a group of 18 scoliosis patients (Mean major Cobb angle was 43.4 o). Each patient was scanned in an unloaded and loaded condition while compressive loads equivalent to 50% body mass were applied using a custom developed compressive device. Findings: The mean increase in major Cobb angle due to compressive loading was 7.4 o (SD 3.5 o). The most axially rotated vertebra was observed at the apex of the structural curve and the largest average intravertebral rotations were observed toward the limits of the coronal deformity. A level-wise comparison showed no significant difference between the average loaded and unloaded vertebral axial rotations (intra-observer error = 2.56 o) or intravertebral rotations at each spinal level. Interpretation: This study suggests that the biomechanical effects of axial loading primarily influence the coronal deformity, with no significant change in vertebral axial rotation or intravertebral rotation observed between the unloaded and loaded condition. However, the magnitude of changes in vertebral rotation with compressive loading may have been too small to detect given the resolution of the current technique.

Risk of metabolic syndrome among children living in metropolitan Kuala Lumpur: A case control study

Background With the increasing prevalence of childhood obesity, the metabolic syndrome has been studied among children in many countries but not in Malaysia. Hence, this study aimed to compare metabolic risk factors between overweight/obese and normal weight children and to determine the influence of gender and ethnicity on the metabolic syndrome among school children aged 9-12 years in Kuala Lumpur and its metropolitan suburbs. Methods A case control study was conducted among 402 children, comprising 193 normal-weight and 209 overweight/obese. Weight, height, waist circumference (WC) and body composition were measured, and WHO (2007) growth reference was used to categorise children into the two weight groups. Blood pressure (BP) was taken, and blood was drawn after an overnight fast to determine fasting blood glucose (FBG) and full lipid profile, including triglycerides (TG), high-density lipoprotein cholesterol (HDL-C), low-density lipoprotein cholesterol (LDL-C) and total cholesterol (TC). International Diabetes Federation (2007) criteria for children were used to identify metabolic syndrome. Results Participants comprised 60.9% (n = 245) Malay, 30.9% (n = 124) Chinese and 8.2% (n = 33) Indian. Overweight/obese children showed significantly poorer biochemical profile, higher body fat percentage and anthropometric characteristics compared to the normal-weight group. Among the metabolic risk factors, WC ≥90th percentile was found to have the highest odds (OR = 189.0; 95%CI 70.8, 504.8), followed by HDL-C≤1.03 mmol/L (OR = 5.0; 95%CI 2.4, 11.1) and high BP (OR = 4.2; 95%CI 1.3, 18.7). Metabolic syndrome was found in 5.3% of the overweight/obese children but none of the normal-weight children (p < 0.01). Overweight/obese children had higher odds (OR = 16.3; 95%CI 2.2, 461.1) of developing the metabolic syndrome compared to normal-weight children. Binary logistic regression showed no significant association between age, gender and family history of communicable diseases with the metabolic syndrome. However, for ethnicity, Indians were found to have higher odds (OR = 5.5; 95%CI 1.5, 20.5) compared to Malays, with Chinese children (OR = 0.3; 95%CI 0.0, 2.7) having the lowest odds. Conclusions We conclude that being overweight or obese poses a greater risk of developing the metabolic syndrome among children. Indian ethnicity is at higher risk compared to their counterparts of the same age. Hence, primary intervention strategies are required to prevent this problem from escalating.

A randomised, feasibility trial of a tele-health intervention for Acute Coronary Syndrome patients with depression ('MoodCare')

Background Coronary heart disease (CHD) and depression are leading causes of disease burden globally and the two often co-exist. Depression is common after Myocardial Infarction (MI) and it has been estimated that 15-35% of patients experience depressive symptoms. Co-morbid depression can impair health related quality of life (HRQOL), decrease medication adherence and appropriate utilisation of health services, lead to increased morbidity and suicide risk, and is associated with poorer CHD risk factor profiles and reduced survival. We aim to determine the feasibility of conducting a randomised, multi-centre trial designed to compare a tele-health program (MoodCare) for depression and CHD secondary prevention, with Usual Care (UC). Methods Over 1600 patients admitted after index admission for Acute Coronary Syndrome (ACS) are being screened for depression at six metropolitan hospitals in the Australian states of Victoria and Queensland. Consenting participants are then contacted at two weeks post-discharge for baseline assessment. One hundred eligible participants are to be randomised to an intervention or a usual medical care control group (50 per group). The intervention consists of up to 10 × 30-40 minute structured telephone sessions, delivered by registered psychologists, commencing within two weeks of baseline screening. The intervention focuses on depression management, lifestyle factors (physical activity, healthy eating, smoking cessation, alcohol intake), medication adherence and managing co-morbidities. Data collection occurs at baseline (Time 1), 6 months (post-intervention) (Time 2), 12 months (Time 3) and 24 months follow-up for longer term effects (Time 4). We are comparing depression (Cardiac Depression Scale [CDS]) and HRQOL (Short Form-12 [SF-12]) scores between treatment and UC groups, assessing the feasibility of the program through patient acceptability and exploring long term maintenance effects. A cost-effectiveness analysis of the costs and outcomes for patients in the intervention and control groups is being conducted from the perspective of health care costs to the government. Discussion This manuscript presents the protocol for a randomised, multi-centre trial to evaluate the feasibility of a tele-based depression management and CHD secondary prevention program for ACS patients. The results of this trial will provide valuable new information about potential psychological and wellbeing benefits, cost-effectiveness and acceptability of an innovative tele-based depression management and secondary prevention program for CHD patients experiencing depression.

Child characteristics associated with delay of gratification in children with Down syndrome

Aim: Children with Down syndrome have been identified as having difficulty delaying gratification when compared to mental age matched children who are developing typically. This study investigated the association between individual characteristics hypopthesized to be associated with ability to delay as well as the strategies children used in a waiting task. Method: Thirty-two children with Down syndrome and 50 typically developing children matched for mental age completed the tasks. Observations of their behaviour while waiting were video-recorded for later analysis. In addition, parents completed questionnaires with respect to their child’s personality and behaviour. Results: Children with Down syndrome were significantly less able to delay gratification than the comparison group. Different patterns of association were found for the two groups between the observational and questionnaire measures and delay time. Conclusions: Children with Down syndrome have greater difficulty delaying gratification than would be predicted on the basis of their mental age. The contributions to delay appear to differ from those for typically developing children and these differences need to be considered when planning interventions for developing this skill

Strategy use of children with Down syndrome in a delay of gratification situation

Background: The capacity to delay gratification has been shown to be a very important developmental task for children who are developing typically. There is evidence that children with Down syndrome have more difficulty with a delay of gratification task than typically developing children of the same mental age. This study focused on the strategies children with Down syndrome use while in a delay of gratification situation to ascertain if these contribute to the differences in delay times from those of typically developing children. Method: Thirty-two children with Down syndrome (15 females) and 50 typically developing children participated in the study. Children with Down syndrome had a mental age, as measured by the Stanford-Binet IV, between 36 and 66 months (M = 45.66). The typically developing children had a mean chronological age of 45.76 months. Children participated in a delay of gratification task where they were offered two or one small treats and asked which they preferred. They were then told that they could have the two treats if they waited for the researcher to return (an undisclosed time of 15 min). If they did not want to wait any longer they could call the researcher back but then they could have only one treat. Twenty-two of the children with Down syndrome and 43 of the typically developing children demonstrated understanding of the task and their data are included here. Sessions were videotaped for later analysis. Results: There were significant differences in the mean waiting times of the two groups. The mean of the waiting times for children with Down syndrome was 181.32 s (SD = 347.62) and was 440.21 s (SD = 377.59) for the typically developing children. Eighteen percent of the group with Down syndrome waited for the researcher to return in comparison to 35% of the typically developing group. Sixty-four percent of children with Down syndrome called the researcher back and the remainder (18%) violated. In the typically developing group 37% called the researcher back and 28% violated. The mean waiting time for the group of children with Down syndrome who called the researcher back was 24 s. Examination of strategy use in this group was therefore very limited. There appeared to be quite similar strategy use across the groups who waited the full 15 min. Conclusions: These results confirm the difficulty children with Down syndrome have in delaying gratification. Teaching strategies for waiting, using information drawn from the behaviours of children who are developing typically may be a useful undertaking. Examination of other contributors to delay ability (e.g., language skills) is also likely to be helpful in understanding the difficulties demonstrated in delaying gratification.

Parenting satisfaction and self-efficacy : a longitudinal study of mothers of children with Down Syndrome

Continuities and changes in parenting sense of competence were examined for mothers of children with Down syndrome from early childhood to adolescence. The sample comprised 25 mothers whose child with Down syndrome was aged 4-6 years in the first phase of the study, and 11-15 years at the second time point. Maternal satisfaction with parenting increased over time, but there were no changes in parenting self-efficacy. Scores on these measures were no different from those reported in a normative sample of mothers of typically developing children, suggesting that the challenges of parenting a child with Down syndrome do not impact significantly on parenting sense of competence during the early childhood and adolescent periods. There were some significant relationships of maternal sense of competence with child characteristics and self-reported parenting style, mostly in the expected direction.

Contributors to life satisfaction in parents of an adult child with Down syndrome

Aim: This study investigated: (1) concurrent relationships between measures of family life and parental satisfaction with life in parents of an adult with Down syndrome and (2) influence of early family functioning on current parental satisfaction. Method: Sixty-two families were interviewed using a semi-structured interview, and responded to a series of questionnaires related to family functioning when their child with Down syndrome was between 7 and 15 years. Fifteen years later parents were asked to provide data on their current situation, including mental health, and satisfaction and difficulties with respect to care-giving in relation to their adult child. Results: Over half the families provided data to the second phase of the study. Life circumstances were appreciably worse for a small group of families than had been the case 15 years previously; however, these changes were generally unrelated to their parenting role. Overall, parents reported experiencing satisfaction from their care-giving role and did not report high levels of difficulties emanating from this role. Conclusions: Most parents demonstrated good levels of personal functioning, although there was a small group for whom this was not the case. Earlier functioning did not make a strong contribution to current levels of life satisfaction.

Secondary curve behavior in Lenke Type 1C adolescent idiopathic scoliosis after thoracoscopic selective anterior thoracic fusion

Study Design. Analysis of a case series of 24 Lenke 1C adolescent idiopathic scoliosis (AIS) patients receiving selective thoracoscopic anterior scoliosis correction. Objective. To report the behaviour of the compensatory lumbar curve in a group of Lenke IC AIS patients following thoracoscopic anterior scoliosis correction, and to compare the results of this study with previously published data. Summary of Background Data. Several prior studies have reported spontaneous lumbar curve correction for both anterior and posterior selective fusion in Lenke 1C/King-Moe II patients; however to our knowledge no previous studies have reported outcomes of thoracoscopic anterior correction for this curve type. Methods. All AIS patients with a curve classification of Lenke 1C and a minimum of 24 months follow-up were retrieved from a consecutive series of 190 AIS patients who underwent thoracoscopic anterior instrumented fusion. Cobb angles of the major curve, instrumented levels, compensatory lumbar curve, and T5-T12 kyphosis were recorded, as well as coronal spinal balance, T1 tilt angle and shoulder balance. All radiographic parameters were measured before surgery and at 2, 6, 12 and 24 months after surgery. Results. Twenty-four female patients with right thoracic curves had a mean thoracic Cobb angle of 53.0° before surgery, decreasing to 24.9° two years after surgery. The mean lumbar compensatory Cobb angle was 43.5° before surgery, spontaneously correcting to 25.4° two years after surgery, indicating balance between the thoracic and lumbar scoliotic curves. The lumbar correction achieved (41.8%) compares favourably to previous studies. Conclusions. Selective thoracoscopic anterior fusion allows spontaneous lumbar curve correction and achieves coronal balance of main thoracic and compensatory lumbar curves, good cosmesis and patient satisfaction. Correction and balance are maintained 24 months after surgery.

The battered child syndrome : changes in the law and child advocacy

In 1962, Dr C. Henry Kempe and his colleagues published the single most important article written to date about child maltreatment: The Battered-Child Syndrome. This chapter analyses the threefold nature of what these authors achieved: clearly identifing the medical evidence of severe child physical abuse and naming it as a syndrome; identifying the medical profession's resistance to its identification; and then translating their scholarship into advocacy for social and legal change. The chapter also traces some of the effects of Kempe's work, including the nature and effect of the subsequent introduction of mandatory reporting laws in the USA and internationally.