Medium-chain acyl CoA dehydrogenase deficiency: Its relationship to SIDS and the impact on genetic counseling

Sudden infant death syndrome (SIDS) is defined as the sudden and unexpected death of an apparently healthy infant under 1 year of age. Routine autopsies often provide few clues as to the cause of death and rarely include a biochemical evaluation. Genetic counseling for SIDS can be difficult as recurrence risks vary depending on the age at death and the number of deaths which have occurred in the family. Biochemical disorders may account for up to 5% of SIDS. Of the metabolic disorders known to be involved in SIDS, the most commonly found is medium-chain acyl CoA dehydrogenase deficiency (MCAD). MCAD is an autosomal recessive disorder of fatty acid oxidation which accounts for up to 1% of SIDS. For some families, the addition of a postmortem biochemical investigation can identify an unsuspected metabolic disorder as the cause of death. Once the diagnosis is established, accurate genetic counseling can then be provided. Metabolic testing of the surviving siblings of victims of sudden death, and the subsequent identification of those due to MCAD can prevent the tragedy of recurrent SIDS in some families. In addition, screening the survivors of an acute life threatening event (ALTE) may also prevent a recurrence.     

On the dynamics of dying

This study is concerned with dynamic processes that underly the rapid, degenerative changes associated with the “dying” stage of the multicellular organism's life cycle. The interaction between negative and positive feedback cycles is discussed: negative feedback cycles underly the superstability characteristic of health and illness. When negative feedback cycles fade in the dying phase of life, positive feedback cycles, previously held in check by the negative feedback cycles to which they had been coupled, rise explosively, driving physiologic variables from their normal values towards extremes. This results in the rapid downturn that we associate with dying—an accelerating disintegration terminating in death. A medical case history is analyzed.     

被动性社交网站使用与青少年体像烦恼的关系:体像比较和体像自我差异的中介作用

为探讨被动性社交网站使用和青少年体像烦恼的关系,基于社会比较理论和自我差异理论,采用被动性社交网站使用问卷、社交网站体像比较量表、理想体像问卷和体像烦恼量表,对789名初、高中生进行调查。结果表明：（1）被动性社交网站使用对青少年体像烦恼具有显著的正向预测作用;（2）体像比较和体像自我差异能在被动性社交网站使用和体像烦恼的关系中起中介作用;（3）在被动性社交网站使用与体像烦恼的关系中,女生的体像比较和体像自我差异起并行中介作用,而对男生而言,体像比较和体像自我差异起链式中介作用。研究结果表明被动性社交网站使用不仅能直接影响青少年的体像烦恼,还可通过体像比较和体像自我差异的间接作用对青少年体像烦恼产生影响,且中介路径存在性别差异。     

The Del Greco Assertive Behavior Inventory

Previous research in the area of assertiveness training has been examined with respect to the definitions of assertive, aggressive, and nonassertive behavior and the prominent assertion model. Logical inconsistencies have led to a refinement of the definitions of these behaviors, the inclusion of passive aggressive behavior, and the testing of a new two-dimensional model of assertion. The present study has developed a psychometric test to measure assertive, aggressive, nonassertive, and passive aggressive behavior in the college dormitory population. In addition, support was demonstrated for the validity of the two dimensional model of assertion. The instrument development was divided into four phases: item generation, item evaluation and revision, reliability testing and item analysis, and validity testing. Data analysis supported the two-dimensional model of assertion. In addition, an 86-item Del Greco Assertive Behavior Inventory designed for use with college dormitory students has weathered preliminary validity and reliability testing.     

老年人生命教育发展研究

老年人生命教育既包括生死教育,也包括生命健康教育。老龄社会为老年人生命教育发展提供难得的机遇,如老年人生命问题为其发展提出了现实要求,“以健康为中心”理念为其提供了理念基础,各种政策为其发展提供了依据。但是,由于我国社会传统中的重“生”观念、以“治疗为中心”的理念、教育体系不完备以及生命教育内容融入问题等,严重制约老年人生命教育开展。为此,应当以生活方式医学重构老年人的生命健康理念,并在个人、社会及政府三个层面探索相关措施以推进老年人生命教育。

     

“本己”抑或“他性”？——海德格尔与列维纳斯死亡观比较分析

海德格尔与列维纳斯都“直面”死亡问题,并对死亡的本性、自我与死亡的关系以及他人之死与自我的关系等问题做出充分回应。海德格尔认为死亡是始终“悬临”于此在生存之中的向终结存在的方式,死亡构成了此在最为本己的整体能在,并展现为主体“我-能”的意向性结构。列维纳斯却要打破生存论上的死亡“悬临”,消解主体的“我-能”,并将“向来属我”的死亡孤独转换为伦理学上“异质属他”的死亡责任,由此,死亡不再是此在为自身存在负责的方式而是为“他者”担负责任的伦理要求。

     

不给予或撤除生命维持干预与安乐死、消极安乐死的概念辨析

不给予或撤除生命维持干预是临床实践中常见且棘手的伦理学问题。该问题的讨论应建立在清晰的概念之上,但不给予或撤除生命维持干预常与安乐死、消极安乐死和尊严死等概念混淆,引发误解。重新定义安乐死和消极安乐死具有理论和现实的紧迫性。基于汤姆•比彻姆和阿诺德•戴维森提出的安乐死定义,提出消极安乐死的定义。基于该定义,认为不给予或撤除生命维持干预与消极安乐死为交叉关系。鉴于安乐死概念的争议及其富含的感情色彩,强调讨论生命维持干预的使用时应区分事实与价值,并建议慎用或弃用安乐死与消极安乐死。

     

临床工作者面对肿瘤患者照护需求的压力分析

在以实务为基础的研究上进行灵性多元观点的内容分析。对临床工作者进行焦点访谈,研究显示面对患者的死亡与未知的恐惧、患者的身心灵整合与情绪变化、家属的心理支持与情绪处理等,都会无意间增加临床工作者的压力困境。而当前存在的主要问题包括临床工作者认为灵性需求应该具备有意义的信念;临床工作者面对正负向情绪,透过修复自我的价值观与经验复制的分享,来协助家属适应与度过哀伤期;临床工作者并非仅是让患者得以善终,还必须着重改善照护质量。

     

社区护理病房中长期慢性病患者的照料服务机制——基于质性研究的方法

现有社区护理病房照料服务存在不能充分满足患者的深层需求、生活质量不佳的问题。访谈材料显示出由于照料者缺乏对“善终”概念的认知,患者、家属以及医护人员的三方关系中,患者处于权力的弱势地位,主体之间在讨论死亡相关问题上存在严重的脱节。患者的意愿从表达、传递、实施等环节中都存在阻碍,患者“善终”的需求无法实现。“以患者为中心”的照料理念应在现有照料服务中加以推广,通过提升社区照料质量来满足医学关系、家庭关系和患者自身利益的既定政策目标。

     

解蔽死亡的必要性、可行性与教育实践主体

医学实践和殡葬活动以其自身的方式对死亡产生了遮蔽效应, 解蔽死亡的必要性、可行性和教育优势主体分析仍旧需要医学和殡葬的承负。以医学实践、殡葬活动为代表的死亡遮蔽导致了严重的人文疾患, 解蔽死亡成为医学变革和殡葬改革的必要, 也是再认生命意义的必要。生死哲学的理论成果是解蔽死亡的保障, 医学实践和殡葬活动本身的革新要求是解蔽死亡的动力, 当代社会条件保障了解蔽死亡的可行。解蔽死亡是教育实践活动的表现形式, 医务工作者和殡葬工作者是该教育实践的优势主体。