肝豆状核变性（Wilson病）治疗策略的变革及思考

评析肝豆状核变性(WD)当前的治疗方法,主要有低铜饮食,铜的螯合剂青霉胺及曲恩汀,锌制剂和四硫钼酸胺,肝移植。介绍WD治疗策略的变革:不再提倡驱铜疗法以去除堆积的铜,而是使血液中的游离铜离子浓度常态化以逆转铜中毒,并结合2007年《柳叶刀》WD治疗专家共识进行思考。最后,展望未来对WD可能的根治疗法,即基因治疗、肝细胞移植及现在可施行的肝移植。     

论神经哲学的兴起

人类心身问题的研究一直是科学界关注的热点。近年来,国外学者将神经科学与哲学研究统一起来,集合两门学科的研究方法、综合双方的研究成果,以此来共同推进人类心身问题的研究,因此诞生了一门介于神经科学和哲学之间的独立学科,它有着学科交叉的研究领域和特殊的跨学科研究方法,这门学科就是神经哲学。     

Use of Functional Magnetic Resonance Imaging in the Early Identification of Alzheimer's Disease

A growing body of evidence suggests that a preclinical phase of Alzheimer's disease (AD) exists several years or more prior to the overt manifestation of clinical symptoms and is characterized by subtle neuropsychological and brain changes. Identification of individuals prior to the development of significant clinical symptoms is imperative in order to have the greatest treatment impact by maintaining cognitive abilities and preserving quality of life. Functional magnetic resonance imaging (fMRI) offers considerable promise as a non-invasive tool for detecting early functional brain changes in asymptomatic adults. In fact, evidence to date indicates that functional brain decline precedes structural decline in preclinical samples. Therefore, fMRI may offer the unique ability to capture the dynamic state of change in the degenerating brain. This review examines the clinical utility of blood oxygen level dependent (BOLD) fMRI in those at risk for AD as well as in early AD. We provide an overview of fMRI findings in at-risk groups by virtue of genetic susceptibility or mild cognitive decline followed by an appraisal of the methodological issues concerning the diagnostic usefulness of fMRI in early AD. We conclude with a discussion of future directions and propose that BOLD-fMRI in combination with cerebral blood flow or diffusion techniques will provide a more complete accounting of the neurovascular changes that occur in preclinical AD and thus improve our ability to reliably detect early brain changes prior to disease onset.     

Religiousness, Health, and Health Behavior in Public-Use Data of the National Center for Health Statistics

Studies of religiousness and health-related variables in large, population-based cross-sectional or, preferably, longitudinal studies, which are often prohibitively expensive, are important to complement findings from the more commonly performed studies. Inadequately known among social science researchers, the national health surveys of the Centers for Disease Control and Prevention’s National Center for Health Statistics (NCHS) offer large, high-quality data sets to the public at no or nominal cost and hence offer important opportunities for research in the area of religion and health, religion and reproductive behavior, sociology of religion and psychology of religion. This report provides an overview of the data sets and a bibliography of prior research using these data, which is intended to suggest how the data of NCHS may be further exploited by researchers of religiousness and health.

错误相关负波（ERN）在精神病理学研究中的应用

错误相关负波（error-related negativity, ERN）是由行为错误诱发的一种脑电波成分,最大峰值在错误反应之后的50ms左右,偶极子源定位于前扣带回（anterior cingulate cortex, ACC）附近。错误加工（error processing）的经典研究范式中出现的ERN成分可能反映了ACC具有错误检测、冲突监控、强化学习、情绪动机等功能。大量研究表明过度的和不足的错误相关脑活动（hyperactive and hypoactive error processing）可能分别与精神病理学的内化性和外化性障碍（internalizing and externalizing disorders）相关联。内化性和外化性障碍的内表型（endophenotype）的研究,还存在许多值得进一步探索的问题。     

前临床期痴呆的认知特征：当前研究进展和未来研究展望

在这篇文章中, 我将讨论从正常老化到痴呆, 这一通常被称为痴呆前临床阶段的认知功能的转变。研究表明, 阿尔茨海默症和血管性痴呆病人在临床确诊之前的几年中, 会出现明显的认知损伤。早期最突出的损伤存在于情节记忆、加工速度以及执行功能。这些功能性损伤与神经生物学研究证实的边缘系统和新皮层区存在多重损伤是相一致的。虽然早在临床诊段之前, 病人组和控制组的平均成绩存在巨大差异, 但同时这两组测试成绩的分布在很大程度上是重叠的。寻求降低这种重叠度的方法是未来研究的一个重要任务。这有可能通过将认知的和其他指标(如基于脑的、基因的、临床的、社会的)相结合构建预测模型来实现。此外, 在未来研究中以下三方面也是急需考虑问题: (a) 找出在 前临床期间认知功能急速下降的时间点: (b) 评估从前临床到临床诊断变化速率中存在的个体差异; (c) 确定特定因素与随后发生的痴呆之间的关联强度是如何随时间向临床确诊推进而逐渐变化的。     

再生障碍性贫血发病机制认识的发展与启示

再生障碍性贫血的发病机制已经突破了“种子”、“土壤”、“虫子”学说,并沿着“免疫异常”理论进入“自身免疫病”理论。同时这是一个科学思维付诸实践不断探索的过程。大量证据表明再生障碍性贫血是一种自身免疫病,它应该符合自身免疫病的共性,这一认识开阔了人们的视野,使人们能在更高的层面上考察原来的事物。     

终末期肾病患者替代治疗方法的临床决策

血液透析、腹膜透析和肾移植是终末期肾病患者替代治疗的主要方法,三种治疗方法各有优缺点。临床上应根据患者原发病因、替代治疗并发症、卫生经济学、生存质量、所在地医疗条件、非医疗因素等作出科学临床决策,最大限度延长患者生命,提高生存质量。     

浅谈心血管疾病预防的重要性和可操作性

心血管疾病是人类死亡的主要病因,其危险因素包括吸烟、血脂异常、糖尿病、高血压、肥胖等。目前我国医务工作者限于治疗疾病,忽视疾病预防,这种模式过于被动,需要高昂的医疗支出。如果不做好心血管疾病的预防工作,将会极大影响国民经济增长。因此改善生活方式、预防心血管疾病发生,形成疾病预防模式就显得尤为重要,并具有可操作性。