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An understanding of the role of inherited cancer predisposition syndromes in pediatric tumor diagnoses continues to develop as more information is learned through the application of genomic technology. Identifying patients and their relatives at an increased risk for developing cancer is an important step in the care of this patient population. The purpose of this review is to highlight various tumor types that arise in the pediatric population and the cancer predisposition syndromes associated with those tumors. The review serves as a guide for recognizing genes and conditions to consider when a pediatric cancer referral presents to the genetics clinic.  相似文献   
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Recent evidence suggests those with autism may be generally impaired in visual motion perception. To examine this, we investigated both coherent and biological motion processing in adolescents with autism employing both psychophysical and fMRI methods. Those with autism performed as well as matched controls during coherent motion perception but had significantly higher thresholds for biological motion perception. The autism group showed reduced posterior Superior Temporal Sulcus (pSTS), parietal and frontal activity during a biological motion task while showing similar levels of activity in MT+/V5 during both coherent and biological motion trials. Activity in MT+/V5 was predictive of individual coherent motion thresholds in both groups. Activity in dorsolateral prefrontal cortex (DLPFC) and pSTS was predictive of biological motion thresholds in control participants but not in those with autism. Notably, however, activity in DLPFC was negatively related to autism symptom severity. These results suggest that impairments in higher-order social or attentional networks may underlie visual motion deficits observed in autism.  相似文献   
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This study aimed to characterize affective functioning in families of youth at high familial risk for depression, with particular attention to features of affective functioning that appear to be critical to adaptive functioning but have been underrepresented in prior research including: positive and negative affect across multiple contexts, individual and transactional processes, and affective flexibility. Interactions among early adolescents (ages 9–14) and their mothers were coded for affective behaviors across both positive and negative contexts. Primary analyses compared never-depressed youth at high (n = 44) and low (n = 57) familial risk for depression. The high risk group showed a relatively consistent pattern for low positive affect across negative and positive contexts at both the individual and transactional level. In contrast to prior studies focusing on negative contexts that did not support disruptions in negative affect among high risk youth, the data from this study suggest variability by context (i.e. increased negativity in a positive, but not negative, context), and individual vs. transactional processes (e.g., negative escalation). Findings are discussed in concert with attention to affect flexibility, contextual and transactional factors.  相似文献   
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Objective: Chronic lymphocytic leukaemia (CLL) is the most prevalent adult leukaemia and is incurable. The course and treatment of CLL is unique and characterised by repeated cycles of treatment, stable disease and relapse. Utilising a Self-Regulatory Model framework, we examined the relationship between patients’ illness perceptions and cancer-specific stress, depressive symptoms and fatigue. Our aim was to test illness perceptions as predictors of these outcomes when variance due to disease and treatment variables was controlled.

Design: Data were collected on 147 patients with relapsed/refractory CLL as they entered a phase II clinical trial of an investigational medication at a university affiliated, National Cancer Institute designated comprehensive cancer center.

Main outcome measures: Cancer-specific stress, depressive symptoms and fatigue interference.

Result:. Hierarchical multiple regression was used. Consequences and emotional representation were related to all outcomes (ps?p?p?Conclusion: Illness perceptions are related to cancer-specific stress, depressive symptoms and fatigue interference in relapsed/refractory CLL. Interventions targeted at restructuring maladaptive illness perceptions may have clinical benefit in this population.  相似文献   
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Chimpanzees provide help to unrelated individuals in a broad range of situations. The pattern of helping within pairs suggests that contingent reciprocity may have been an important mechanism in the evolution of altruism in chimpanzees. However, correlational analyses of the cumulative pattern of interactions over time do not demonstrate that helping is contingent upon previous acts of altruism, as required by the theory of reciprocal altruism. Experimental studies provide a controlled approach to examine the importance of contingency in helping interactions. In this study, we evaluated whether chimpanzees would be more likely to provide food to a social partner from their home group if their partner had previously provided food for them. The chimpanzees manipulated a barpull apparatus in which actors could deliver rewards either to themselves and their partners or only to themselves. Our findings indicate that the chimpanzees’ responses were not consistently influenced by the behavior of their partners in previous rounds. Only one of the 11 dyads that we tested demonstrated positive reciprocity. We conclude that contingent reciprocity does not spontaneously arise in experimental settings, despite the fact that patterns of behavior in the field indicate that individuals cooperate preferentially with reciprocating partners.  相似文献   
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Two studies examined relations between children’s anger regulation and externalizing behavior utilizing community samples. The first study included 58 children from preschool through second grade and examined concurrent associations between regulation and externalizing behavior. The second study included 40 preschool and kindergarten children and examined regulation and externalizing behavior 2 years later. In both studies, anger reactivity and regulation strategies were assessed during home visits using the same observational task. In study 1, teachers completed a problem behavior checklist shortly after the home visit. In study 2, teachers completed a problem behavior checklist 2 years later. Results indicate that the dysregulation of anger is associated with externalizing problems concurrently and two years later. Findings also suggest that attention refocusing may be a successful strategy for reducing anger and externalizing problems among highly reactive children.  相似文献   
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