Modeling Family Dynamics in Children with Fragile X Syndrome

Few studies have examined the impact of children with genetic disorders and their unaffected siblings on family functioning. In this study, the reciprocal causal links between problem behaviors and maternal distress were investigated in 150 families containing a child with fragile X syndrome (FXS) and an unaffected sibling. Both children's behavior problems appeared to have strong, direct effects on maternal distress, but maternal distress did not appear to have any reciprocal causal effects on either child's behavior problems. Surprisingly, there were no significant differences in the effects of the two children's behavior problems on maternal distress. These data suggest that the problem behaviors of children with FXS, as well as their unaffected siblings, can have a substantial and additive impact on maternal depression and anxiety. Future research efforts should employ longitudinal research designs to confirm these findings.     

Spoken and gestural production in a naming task by young children with Down syndrome

Lexical production in children with Down syndrome (DS) was investigated by examining spoken naming accuracy and the use of spontaneous gestures in a picture naming task. Fifteen children with DS (range 3.8-8.3 years) were compared to typically developing children (TD), matched for chronological age and developmental age (range 2.6-4.3 years). Relative to TD children, children with DS were less accurate in speech (producing a greater number of unintelligible answers), yet they produced more gestures overall and of these a significantly higher percentage of iconic gestures. Furthermore, the iconic gestures produced by children with DS accompanied by incorrect or no speech often expressed a concept similar to that of the target word, suggesting deeper conceptual knowledge relative to that expressed only in speech.     

Fetal androgens in the early treated adrenogenital syndrome of 46 XX hermaphroditism: Influence on assertive and aggressive types of behavior

Fifteen hermaphroditic females with the adrenogenital syndrome received corrective, androgen-suppressing therapy with cortisone from birth onward. They were studied psychologically in late adolescence and adulthood, with particular attention to a possible long-term effect of fetal androgenization on aggressive manifestations. No such effect was evident. The girls were tomboyish in the manner previously reported for the adrenogenital syndrome.     

社会支持缓解高互依自我个体的急性心理应激反应

高互依自我构念个体以社会角色来定义自己, 更看重自己的社会关系; 社会评价威胁是诱发个体社会心理应激反应的重要情境因素, 然而高互依自我构念个体在心理性应激情境中是否会表现出更高的应激反应, 以及社会支持是否会缓解高互依自我构念个体的急性心理应激反应还不可知。本研究以特里尔社会应激测试为范式, 以主观应激报告、心率和唾液皮质醇作为应激指标, 使用自我构念问卷测量个体互依自我构念水平, 将健康大学生被试随机分配到自我支持启动组和社会支持启动组。结果发现, 互依自我构念水平正向预测个体在应激情境中的皮质醇水平增加量; 同时, 相比于自我支持启动, 高互依自我构念的个体在经历社会支持后有更低的唾液皮质醇变化量。研究结果提示, 社会支持对高互依自我构念个体的急性心理应激反应起到了良好的缓解作用。     

重型肝炎治疗方案的理性选择

针对我国重型肝炎治疗现状,对重型肝炎常用的以及部分有争议的治疗药物和方法进行分析和讨论,就如何选择治疗方案提出医疗原则,认为应采取内科治疗-人工肝-肝移植的综合治疗模式,结合患者的治疗愿望及经济承受力,理性地选择治疗方案,以期达到合理、有效和经济的目的。     

Symptom patterns in late luteal-phase dysphoric disorder

The symptom patterns of 180 women with prospectively confirmed late luteal-phase dysphoric disorder (LLPDD) were examined using a careful application of factor and cluster analytic techniques. Factor analysis of premenstrual change scores on 33 common premenstrual syndrome symptoms yielded four orthogonal factors that were consistent across two sets of menstrual cycle data. These were a negative affect dimension with concomitant behavioral changes, physical symptoms, agitation, and positive arousal. Cluster analysis of factor scores grouped patients into one of five symptom patterns, the most common of which is a general distress cluster, characterized by symptoms across all dimensions. The LLPDD symptom groups identified are remarkably consistent with those of earlier studies using both normative and clinic samples, and consideration of symptom pattern differences in future research may greatly increase our understanding of this disorder.     

People and objects affect the quality of vocalizations in infants with down syndrome

The vocalizations of eight infants with Down syndrome were recorded longitudinally in relation to different social and non-social contexts. The infants were observed biweekly from 8 to 24 weeks and monthly up to 40 weeks. At each visit the infants were presented with their mother, a female stranger, and a rattle puppet, each alternately active and passive. Each condition lasted 60 sec. The results showed that by 4 months of age, the infants produced different types of vocal sounds in relation to environmental contexts. They produced significantly more melodic (speechlike) sounds, vocalic (non-speechlike) sounds, and emotional (crying, laughing and fussing) sounds when facing people than objects. By 6 months of age, these utterances began to be distinguished between mother and female stranger and active and passive adults. However, within the communicative context the overall amount of vocalic (non-speechlike) sounds produced was larger than the amount of melodic (speechlike) sounds. It is suggested that this low output of melodic sounds in the overall vocal production of these infants may adversely affect the development of more appropriate vocal behaviour.     

SLEEP DEPRIVATION,ALLERGY SYMPTOMS,AND NEGATIVELY REINFORCED PROBLEM BEHAVIOR

We studied the relation between the presence versus the absence of sleep deprivation or allergy symptoms and the rate and function of problem behavior. Three students whose problem behavior was negatively reinforced by escape from instruction were studied across several weeks using analogue functional analyses. Our results indicated that the extraexperimental events were associated with (a) termination of instruction functioning as a negative reinforcer, (b) increased rates of negatively reinforced problem behavior, or (c) increased rates of problem behavior across all conditions.     

EFFECTS OF EXTINCTION ALONE AND EXTINCTION PLUS FUNCTIONAL COMMUNICATION TRAINING ON COVARIATION OF PROBLEM BEHAVIORS

In this investigation, extinction (EXT) was applied alone or in combination with functional communication training (FCT) to less severe topographies of problem behavior while more severe topographies continued to be reinforced. EXT alone decreased less severe and increased more severe topographies of problem behavior (i.e., response covariation), whereas EXT with FCT reduced all topographies of problem behavior to near-zero levels.     

Cowden Syndrome

Cowden syndrome is an autosomal dominant inherited cancer syndrome characterized by multiple hamartomas which may develop in any organ. Mucocutaneous papillomas and trichilemmomas are hallmarks of the syndrome. Affected individuals are at high risk of developing both benign and malignant disease of the thyroid and breast. Epithelial thyroid carcinoma occurs in 3–10% of affected individuals while women have a 25–50% lifetime risk of developing adenocarcinoma of the breast. Because of the cancer risk, it is imperative that health care providers recognize Cowden syndrome. The susceptibility gene has been localized to 10q22-23 and preliminary studies demonstrate no genetic heterogeneity.