169 resultados para Childhood myelodysplastic syndromes


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The purpose of this study was to examine differences in asthma management among families with a child who has moderate to severe asthma. Half of the 50 families chosen for study had participated in an intensive in-patient asthma treatment program and half had participated in an out-patient day camp. Two broad categories of outcome were examined - illness and self-management skills. Families who participated in the in-patient program exhibited a pattern of illness behaviours which indicated asthma symptoms were better managed in comparison to those families that participated in the out-patient program. It was also observed that children who participated in the in-patient program had a tendency to feel more positive about having asthma with the more self-management behaviours they practised. On the other hand, children from the out-patient program reported a more negative attitude about having asthma with the more self-management behaviours they practised.

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This study examined patterns in adaptation among parents with a child who had moderate to severe persistent asthma. Specifically, we were interested in examining the differences in adaptation between mothers and fathers in which it was hypothesized that gender effects would be obtained in patterns of coping. Eighty-four parents participated in the study, representing 37 intact families in which both parents were present and 13 single-parent families. Within intact families, mothers exhibited greater efforts than fathers in coping patterns including strategies to acquire social support outside the family, enhance self-worth, and decrease psychological tensions. When compared to mothers in single-parent families, mothers within intact families had a greater tendency to use coping patterns related to family integration and cooperation. Such findings demonstrate a need for additional support for mothers in their role in caring for the chronically ill child. The implications of these findings for clinical practice are discussed. Copyright 2002, Elsevier Science (USA). All rights reserved.

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To evaluate the relative effect of cyanosis, surgical interventions and family processes on neuropsychological and behavioural outcomes in 4-year-old survivors of serious congenital heart disease (CHD).

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Aims - To evaluate the outcome of filtering procedures supplemented with mitomycin C in children with glaucoma. Methods - All patients aged 17 or younger with glaucoma who underwent filtering surgery supplemented with mitomycin C at a tertiary care centre (n = 21) during a 5 year interval (1992 and 1996) were included. One eye for each patient was entered into the analysis. The postoperative intraocular pressure (IOP), use of antiglaucoma medications, clinical stability of glaucoma, complications, and visual acuity were retrospectively evaluated. Kaplan-Meier survival curves were used to estimate the probability of success. Results - At the time of surgery mean age was 5.7 (SD 5.0) years. The most common diagnoses were trabeculodysgenesis (n = 6) and aphakic glaucoma (n = 8). Mean IOP before surgery was 35.7 (10.5) mmHg. Average length of follow up was 18.6 (14.7) months. The probability of having IOP less than 21 mmHg with no antiglaucoma medications and with clinically stable glaucoma 1 year after surgery was 76.9% in phakic eyes (n = 13) and 0% in aphakic eyes (n = 8). A phakic patient with Sturge-Weber's syndrome had choroidal effusion after surgery that resolved spontaneously. In the aphakic group one patient had retinal detachment and another developed an encapsulated bleb. Visual acuity deteriorated in one patient. Conclusion - A guarded filtration procedure with mitomycin C is relatively successful in phakic children with glaucoma, but unsuccessful in aphakic ones.

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A systematic review was conducted of studies evaluating the effects of interventions aimed at reducing ethnic prejudice and discrimination in young children. Articles published between 1980 and 2010 and including children of 8 years and under were identified, harvested, and assessed for quality, both for the exposure/program as well as for the evaluation. In total, 32 studies (14 contact and 18 media or instruction) yielded 62 effects on attitudes and 59 effects on peer relations. An overall count of the positive (40%), non-significant (50%), and negative effects (10%) indicate a mixed picture. Overall, more attitude effects (55%) than peer relations effects (25%) were positive, and media/instruction (47%) was more successful than contact (36%). Most of the effects were observed with children from a majority ethnicity: 67% of the attitude effects were positive, and media/instruction and contact were equally effective at delivering these. Few differences were found as a function of the quality of the exposure and evaluation, but differences were found depending on the context of exposure (naturally occurring or experimental manipulation) and research design (random assignment or self-selection). In conclusion, the findings were more mixed than expected, though sufficiently strong studies exist to provide lessons for future research.


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The authors sought to determine whether the clinical manifestations of schizophrenia and other psychotic disorders are correlated in affected sibling pairs.

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This study sought to determine whether 80-lead body surface potential mapping (BSPM) would improve detection of acute myocardial infarction (AMI) and occluded culprit artery in patients presenting with ST-segment depression (STD) only on 12-lead ECG.

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The operant learning theory account of behaviors of clinical significance in people with intellectual disability (ID) has dominated the field for nearly 50 years. However, in the last two decades, there has been a substantial increase in published research that describes the behavioral phenotypes of genetic disorders and shows that behaviors such as self-injury and aggression are more common in some syndromes than might be expected given group characteristics. These cross-syndrome differences in prevalence warrant explanation, not least because this observation challenges an exclusively operant learning theory account. To explore this possible conflict between theoretical account and empirical observation, we describe the genetic cause and physical, social, cognitive and behavioral phenotypes of four disorders associated with ID (Angleman, Cornelia de Lange, Prader-Willi and Smith-Magenis syndromes) and focus on the behaviors of clinical significance in each syndrome. For each syndrome we then describe a model of the interactions between physical characteristics, cognitive and motivational endophenotypes and environmental factors (including operant reinforcement) to account for the resultant behavioral phenotype. In each syndrome it is possible to identify pathways from gene to physical phenotype to cognitive or motivational endophenotype to behavior to environment and back to behavior. We identify the implications of these models for responsive and early intervention and the challenges for research in this area. We identify a pressing need for meaningful dialog between different disciplines to construct better informed models that can incorporate all relevant and robust empirical evidence.

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The behavioural phenotypes of Prader-Willi (PWS) and Fragile-X (FraX) syndromes both comprise repetitive behaviours with differences between the profiles. In this study we investigated the context and antecedents to the repetitive behaviours and the association with other behavioural phenotypic characteristics in order to generate testable hypotheses regarding the cause of the behaviours.

The parents or carers of 46 children with PWS (mean age 14.1 years; 20 girls), and 33 boys with FraX (mean age 13.11 years) were interviewed about their children's repetitive behaviour in a semi-structured format.

Children showed negative emotional behaviour (PWS: 87.0%; FraX: 79.4%) and repetitive questions (PWS: 78.3%; FraX: 73.5%) following changes in routine or expectations. Significantly more temper outbursts were reported to follow changes in children with PWS (89.1%) compared with boys with FraX (41.2%) (chi(2) = 20.93; P <0.001). Anxiety that was frequently associated with repetitive and self-injurious behaviours in boys with FraX, followed changes in significantly more boys with FraX (76.5%) compared with children with PWS (6.5%) (chi(2) = 43.19, P <0.001).

On the basis of these reports and existing literature, we hypothesise that decreases in predictability are aversive to children with PWS and FraX. We also hypothesise that these children have a propensity to show a syndrome-related pattern of behaviour (temper outbursts in PWS and displays of anxiety in FraX) when an event in the environment has this aversive property. We hypothesise that questions may be reinforcing to children in their own right by increasing the predictability of the environment. We outline how a specific cognitive deficit in the endophenotypes associated with both PWS and FraX could be investigated as a potential explanation for the hypothesised aversive properties of decreased predictability.

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Northern Ireland is in the early stages of transition from conflict, but progress is regularly affected by political and public discontent. A divided landscape, segregated and under-resourced communities are enduring legacies of ‘the Conflict’. Yet the political will to tackle social and community division, consult with and support communities has been lacking. Grounded in six communities most affected by poverty and the Conflict this paper illustrates the difficulties, tensions and contradictions experienced during transition and how, in the process of ‘change’, children and young people have been silenced, marginalised and
demonised.

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There are strong links between childhood trauma and the risk of violence (Ford et al., 2007). Despite evidence that people with psychotic disorders are at a higher risk of violence than the general population (Witt et al., 2013) there have been few studies that have examined the trauma-violence link in this population (Spidel et al., 2010). This study explored the association between a history of childhood trauma (abuse, neglect and conflict-related trauma) and the risk of violence in adults with psychotic disorders. The strongest associations with the risk of violence were found for sexual abuse (r = .32, p < .05) and the impact of community conflict (r = .32, p < .05). An accumulative effect of trauma was found using a hierarchical regression (adjusted R2 = .14, F(2,37) = 4.23, p < .05). There are implications for applying models of violence to psychosis, risk assessment and treatment of people with psychotic disorders as well as informing trauma models and protective factors for children in conflict-affected regions.