Chronic glucocorticoid hypersecretion in Cushing’s syndrome exacerbates cognitive aging

Cumulative exposure to glucocorticoid hormones (GC) over the lifespan has been associated with cognitive impairment and may contribute to physical and cognitive degeneration in aging. The objective of the present study was to examine whether the pattern of cognitive deficits in patients with Cushing’s syndrome (CS), a disorder characterized by chronic exposure to elevated levels of glucocorticoids (GC), is similar to that observed in older individuals. Ten subjects with CS were compared to sex-, age-, and education-matched healthy controls and older subjects (age of CS subjects + 15 yr). All participants were administered tests to assess attention, visuospatial processing, learning and memory, reasoning, concept formation and verbal fluency. MANCOVAs with depression scores as covariate and polynomial contrasts revealed that the age-matched control group performed better than the CS and older subject groups in visual target detection, trail making test, stroop task, digit symbol substitution, block design, object assembly, visual reproduction, spatial memory and similarities. The CS and older subjects performed similarly on these tasks. Further, a principal component analysis revealed two significant factors, representing general cognitive function and verbal memory explaining 39.9% and 10.0% of the variance, respectively. Additional MANCOVAs with depression as a covariate revealed that CS and older control subjects showed impaired performance on general cognitive function compared to age-matched controls. These results suggest that hypersecretion of GCs has “aging-like” effects on cognitive performance in individuals with CS.     

Social Information Processing Skills in Children with Histories of Heavy Prenatal Alcohol Exposure

Based on caregiver report, children with prenatal alcohol exposure have difficulty with social functioning, but little is known about their social cognition. The current study assessed the social information processing patterns of school-age children with heavy prenatal alcohol exposure using a paradigm based on Crick and Dodge’s reformulated six-stage model. Fifty-two children (aged 7–11) with and without heavy prenatal alcohol exposure were tested using a structured interview measure of social information processing involving 18 videotaped vignettes of children in group entry and provocation situations. Alcohol-exposed children displayed maladaptive processing patterns on the goal, response generation, and response evaluation steps in group entry situations, and encoding, attribution, response evaluation, and enactment steps during provocation situations. Children with heavy prenatal alcohol exposure also had difficulty on the Test of Problem Solving, and performance correlated with social information processing measures. Such difficulties may lead to problems in social functioning and warrant early intervention.     

持续正压通气治疗睡眠呼吸暂停低通气综合征的接受意向调查

探讨影响持续气道内正压通气(CPAP)治疗阻塞性睡眠呼吸暂停低通气综合征(OSAHS)的接受性的非技术因素。设计OSAHS认知度和CPAP意向调查表、信赖他人量表,60例OSAHS患者经健康教育后,100例健康志愿者随机填表,结果做统计分析。两组中43.3%和5%基本了解OSAHS(P<0.01);10%和5%表示接受CPAP(P>0.05);85%和90%认为CPAP机价格高(P>0.05)。量表得分:两组得分分布近似,高分比例大,添加题90%和80%选择负性答案。目前公众对OSAHS认知差,对医生的信任度低,而CPAP机价格高,患者对CPAP接受性差。     

ALI/ARDS患者糖皮质激素的治疗效果和时机

急性肺损伤/急性呼吸窘迫综合征(ALI/ARDS)是临床各科经常遇到的急症,其发病机制主要是中性粒细胞向炎症部位的移动,释放出大量的炎性介质和细胞因子,引起肺泡毛细血管膜通透性增高,弥漫性肺损伤和蛋白性肺泡水肿液聚集等病理生理改变。临床表现为呼吸困难、呼吸窘迫、顽固的低氧血症,虽然使用机械通气和呼气末正压通气(PEEP)后,其病死率有所下降,但仍然维持在50%左右。就糖皮质激素的作用机制,对不同原因引起的ALI/ARDS治疗效果,使用时机作一综述,期望对ALI/ARDS治疗过程中糖皮质激素的使用予以规范。     

急性肺损伤与急性呼吸窘迫综合征的诊断与治疗策略

急性肺损伤（ALI）和急性呼吸窘迫综合征（ARDS）是多个危险因素直接或间接引起肺部损伤的连续病理过程,以表现低氧血症为特征,ARDS是ALI严重末期的表现。低流量、呼气末正压（PEEP）保护性通气的措施可明显改善氧和,减轻机械通气肺损伤,降低病死率,明显优于传统通气方式。     

Differences in visual orienting between persons with Down or fragile X syndrome

The voluntary and reflexive orienting abilities of persons with Down syndrome and fragile X syndrome, at average MA levels of approximately 4 and 7 years, were compared with an RT task. Reflexive orienting abilities appeared to develop in accordance with MA for the participants with Down syndrome but not for those with fragile X syndrome. However, both groups showed delayed voluntary orienting. The group differences in reflexive orienting at the low MA level reinforce the practice of separating etiologies and highlight the contribution of rudimentary attentional processes in the study of individuals with mental retardation.     

Is Theory of Mind Understanding Impaired in Males with Fragile X Syndrome?

Males with fragile X syndrome (FXS) have difficulties with social interaction and many show autistic features. This study examined whether the social deficits characteristic of FXS are associated with theory of mind difficulties. Two groups of boys with FXS participated: a group with few autistic features and a group with many autistic features. An intellectual disability control group also participated. In addition to using standard theory of mind tasks, new techniques were used that were able to separate out the various processing demands of the task (e.g., memory, inhibitory control). Overall, the findings indicate that both groups of boys with FXS have difficulty with theory of mind tasks compared to an intellectual disability control group. However, both groups with FXS also performed worse on comparison trials that required working memory but not theory of mind. Theory of mind difficulties are likely to be an important aspect of the FXS clinical profile, but are most likely the result from a more basic difficulty with working memory.     

An Opportunity for Genetic Counseling Intervention: Depression in Parents of Individuals with Proteus Syndrome

Depression is common, affecting 2–5% of the general population. Parental depression can confound adjustment to, and caring for, a child with a genetic condition. As part of a study on psychosocial issues of parents caring for children with Proteus syndrome, 31 parents (20 mothers and 11 fathers) completed a depression screening tool, the Beck Depression Inventory. Approximately 23% (4/20 mothers and 3/11 fathers) scored positive on the tool. Pessimism, sense of failure, general lack of satisfaction, sense of punishment, self-dislike, social withdrawal, indecisiveness, work inhibition, somatic preoccupation, and loss of libido were reported more frequently by the group of parents with positive screen results than those with normal results. These data suggest that symptoms of depression may be prevailing among parents of individuals with Proteus syndrome. Because effective interventions for depression are readily available, genetic counselors working with families affected with rare, overgrowth disorders should specifically assess parents for physical and affective symptoms of depression and refer them for appropriate clinical treatment.     

系统生物学引导阿尔茨海默病治疗新策略

阿尔茨海默病(AD)是一种发生于中老年期的退行性脑神经变性疾病,临床上以进行性智能衰退,伴有人格改变为主要特征.其发病过程涉及许多途径和靶点.现代医学认为脑内胆碱能递质功能紊乱、β-淀粉样蛋白沉积、tau-蛋白过磷酸化、炎症反应以及基因突变等在其发病过程中起着重要作用.治疗方法从胆碱酯酶抑制剂、神经递质受体阻滞剂到补气活血、益精填髓、化痰开窍等中医药疗法不等.但抑制剂、阻滞剂等药物阻滞疗法,从单一环节阻断发病途径,忽略了AD发病的整体复杂性.中医辨证论治从整体观念出发,在宏观上治疗AD,对AD的发病缺乏微观深入研究.AD治疗需要借助于“系统生物学”理论的指导.中西医结合为AD的治疗提供了较为科学、有效的途径和发展空间.