Human biochemical genetics： an insight into inborn errors of metabolism

Inborn errors of metabolism （IEM） include a broad spectrum of defects of various gene products that affect intermediary metabolism in the body. Studying the molecular and biochemical mechanisms of those inherited disorder, systematically summarizing the disease phenotype and natural history, providing diagnostic rationale and methodology and treatment strategy comprise the context of human biochemical genetics. This session focused on： （1） manifestations of representative metabolic disorders; （2） the emergent technology and application of newborn screening of metabolic disorders using tandem mass spectrometry; （3） principles of managing IEM; （4） the concept of carrier testing aiming prevention. Early detection of patients with IEM allows early intervention and more options for treatment.     

Values education as holistic development for all sectors: researching for effective pedagogy

The paper argues that values education has moved from being associated most heavily with the religious agenda of faith schools to being central to updated research insights into effective pedagogy. As such, it represents a vital approach to education in any school setting. The paper draws on an array of values education research and practice in making the case but centres especially on findings from a number of recent publicly funded projects in Australia with which the authors have been associated. Of special importance is evidence from the Values Education Good Practice Schools Project and the Project to Test and Measure the Impact of Values Education on Student Effects and School Ambience that provide both anecdotal and empirical evidence that high quality values education contributes to holistic educational development, including academic advancement, of students across all school sectors.     

Deaf and hearing students' morphological knowledge applied to printed English

The study examined the ability of deaf and hearing students at the college and middle school levels to discern and apply knowledge of printed word morphology. There were 70 deaf and 58 hearing participants. A two-part paper-and-pencil test of morphological knowledge examined subjects' ability to (a) perceive segmentation of morphemes within printed words and (b) recognize meanings associated with various printed morphemes. The hearing college students performed best on every dependent measure of the two-part test. The deaf college students scored significantly lower than the hearing college students but similarly to the hearing middle school students. Deaf middle school students consistently scored the lowest on both parts of the test. While all students' performance declined as the difficulty of the morphemic content increased within both tasks, the decline was greatest among middle school deaf students. Although segmentation and semantic analysis skills necessary to morphographic decoding were apparent in the deaf students, their mastery levels fell significantly below those of the hearing subjects.     

期刊评价：影响因子及其相关指标

在西部大开发环境下，联系陕西省科技期刊的现状，研究了陕西省科技期刊的发展策略，首次提出“寓发展于服务中、中陕西省科技期刊与西部大开发的结合点。指出陕西省科技期刊发展的基本原则是坚持两个效益辩证统一，发展的出发点是服务西部大开发。     

The effects of substrate and fluid provision on thermoregulatory and metabolic responses to prolonged exercise in a hot environment

A high ambient temperature reduces the capacity to perform prolonged exercise. Total carbohydrate oxidation is less, and thus glycogen depletion is not limiting. Fluid ingestion in the heat should, therefore, focus on maintenance of hydration status rather than on substrate provision. Six healthy males cycled to exhaustion at 60% of maximum oxygen consumption (VO 2max ) with no drink, ingestion of a 15% carbohydrate-electrolyte drink (1.45 - 0.29 litres) or ingestion of a 2% carbohydrate-electrolyte drink (3.12 - 0.47 litres). The ambient temperature was 30.2 - 0.6°C (mean - s ), with a relative humidity of 71 - 1% and an air speed of approximately 0.7 m.s -1 on all trials. Weighted mean skin temperature, rectal temperature and heart rate were recorded and venous samples drawn for determination of plasma volume changes, blood metabolites, serum electrolytes and osmolality. Expired gas was collected to estimate rates of fuel oxidation. Exercise capacity was significantly ( P ? 0.05) different in all trials. The median (range) time to exhaustion was 70.9 min (39.4-97.4 min) in the no-drink trial, 84.0 min (62.7-145 min) in the 15% carbohydrate trial and 118 min (82.6-168 min) in the 2% carbohydrate trial. The 15% carbohydrate drink resulted in significantly ( P ? 0.05) elevated blood glucose and total carbohydrate oxidation compared with the no-drink trial. The 2% carbohydrate drink restored plasma volume to pre-exercise values by the end of exercise. No differences were observed in other thermoregulatory or cardiorespiratory responses between trials. These results suggest that fluid replacement with a large volume of a dilute carbohydrate drink is beneficial during exercise in the heat, but the precise mechanisms for the improved exercise capacity are unclear.     

Contextual factors that sustain innovative pedagogical practice using technology: an international study

Pedagogical innovation—whether involving technology or not—is shaped by a complex interaction of the innovation with contextual factors such as school and school district policy, leadership, cultural norms and values, teacher attitudes and skills, and student characteristics. This study examined school and classroom contexts in which pedagogical innovations employing technology were successfully sustained. Data were obtained from 59 cases drawn from the Second Information Technology in Education Study—Module 2, a project that examined 174 cases of innovative pedagogical practice in schools in 28 countries. An explanatory model of sustainability was derived from a qualitative analysis of the cases using grounded theory techniques. Essential conditions for the sustainability of classroom innovation were teacher and student support of the innovation, teacher perceived value of the innovation, teacher professional development, and principal approval. Contributing factors for sustainability were supportive plans and policies, funding, innovation champions, and internal and external recognition and support. An interactive website that allows for searching of SITES-M2 case reports on various attributes such as sustainability, transferability, level of school, and evidence of supportive policies and plans is available at http://sitesm2.org.     

Explaining organizational knowledge creation with a knowledge maturing model

Social media challenge knowledge management because of encouraging conversations, networking and participation in more distributed, diverse and dynamic ways of knowledge development and increasingly important individuals’ interests driving them. Hence, we need to understand the complex relationships between different qualities of knowledge developed in informal and formal processes as well as for overcoming misalignments in routines, tools and infrastructures supporting organizational knowledge creation. This paper contributes a maturation perspective towards explaining organizational knowledge creation and presents a knowledge maturing model, which is grounded in organizational practice and validated with qualitative and quantitative empirical and design studies. The results describe how characteristics of knowledge and support by IT change between phases of knowledge maturing. Our findings confirm theories of organizational knowledge creation with respect to expanding scopes from individuals through communities to organizations moving from interest-driven knowledge exploration in informal contexts to goal-driven knowledge exploitation in formal contexts. The maturation perspective adds to our understanding that organizational knowledge creation is not simply a continuous process. Phases that emphasize changeability alternate with phases concerned with stability. Knowledge develops in contexts that need to switch multiple times between opening up for new knowledge and filtering relevant knowledge and between de- and re-contextualization.     

Shifting the Perspective of the Research Librarian: A CoResearch Paradigm

This article proposes the coresearch engagement model (CoRE), which addresses how the academic research librarian can become a coresearch partner with any patron. The CoRE model is an interpersonal process and is founded upon the concepts of mutual respect, collaboration, reciprocity, and empowerment. Grounded in Howard Gardner’s multiple intelligences theory, Malcolm Knowles’ andragogical model, and guidelines from the Reference and User Services Association, CoRE has the potential to result in engagement for the librarian and patron. The CoRE model can be utilized in many ways including self-awareness and development for those who need and want to expand their abilities as a research librarian. It provides a theoretical framework and a knowledge base, in addition to giving a context for further development by expanding, comparing the related theories for further research, and implementing various applications suggested by this article.