中国語教科書に見られる不自然な点について

随着日中交流的深人，日本的汉语学习方兴未艾。据文部科学省2008年发表的统计，早在2006年， 日本开设汉语课程的大学就有596所，占总数的84% ，仅次于英语，已成为第二大外语。开设汉语课的高 中有813所，社会上办的各种汉语学习班更是不计其数。如此，汉语学习者大量增加。 在这个背景下，汉语教材的出版、发行百家争鸣，百花齐放，以适应各种学习者的需要，促进了汉 语学习。 但在这教材的百花缭乱中，难免良莠不齐，我们发现一些教材出现了作为教材不应该出现的不妥。 仅从近年我们使用的教材中发现了如下一些不妥 : 1. 语法解释不当：　2. 按照语法硬造出来的欠自然 的表达; 3. 时态错误　；　4. 语法练习欠严谨 ; 5. 注释有误 ; 6. 插图有误导 ; 7. 不理解背景; 8. 会话欠自然得体，等等问题。 対此，我们认为，教材是供人学习之范本，应该从语言、文化、学习对象、教学法等各个角度加以 严谨的审视，尽最大努力，提供最善文本，以尽量避免 "误人子弟" 处的出现。同时觉得，任课教师在 教材的这百花缭乱中，教材的选择使用应慎之再慎

221 Newborn-Screened Neonates with Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency: Findings from the Inborn Errors of Metabolism Collaborative

Introduction: There is limited understanding of relationships between genotype, phenotype and other conditions contributing to health in neonates with medium-chain acyl-coenzyme A dehydrogenase deficiency (MCADD) identified through newborn screening. Methods: Retrospective analysis of comprehensive data from a cohort of 221 newborn-screened subjects identified as affected with MCADD in the Inborn Errors of Metabolism-Information System (IBEM-IS), a long term follow-up database of the Inborn Errors of Metabolism Collaborative, was performed. Results: The average age at notification of first newborn screen results to primary care or metabolic providers was 7.45 days. The average octanoylcamitine (C8) value on first newborn screen was 11.2 mu mol/L (median 8.6, range 036-43.91). A higher C8 level correlated with an earlier first subspecialty visit. Subjects with low birth weight had significantly lower C8 values. Significantly higher C8 values were found in symptomatic newborns, in newborns with abnormal lab testing in addition to newborn screening and/or diagnostic tests, and in subjects homozygous for the c.985A\u3eG ACADM gene mutation or compound heterozygous for the c.985A\u3eG mutation and deletions or other known highly deleterious mutations. Subjects with neonatal symptoms, or neonatal abnormal labs, or neonatal triggers were more likely to have at least one copy of the severe c.985A\u3eG ACADM gene mutation. C8 and genotype category were significant predictors of the likelihood of having neonatal symptoms. Neonates with select triggers were more likely to have symptoms and laboratory abnormalities. Conclusions: This collaborative study is the first in the United States to describe health associations of a large cohort of newborn-screened neonates identified as affected with MCADD. The IBEM-IS has utility as a platform to better understand the characteristics of individuals with newborn-screened conditions and their follow-up interactions with the health system. (C) 2016 Elsevier Inc. All rights reserved

Measuring a Safety Culture: Critical Pathway or Academic Activity?

he Institute of Medicine (IOM) identified six core needs in a health care system, the first of which was safety. 1 Furthermore, several IOM committees and others have identified the creation of a “culture of safety ” as the key institutional requirement to achieve safe medical care. 1–3 In this issue of the journal, Modak et al. 4 present an instrument that may help measure the extent to which a patient safety culture exists in an ambulatory setting. While these authors and others have done considerable work on defining and measuring a culture of safety in the hospital setting, 5,6 few have tackled the difficult task of measuring a safety culture in the ambulatory arena within the US health care system. Even in the hospital setting, where there has been more effort, the development of a culture of safety within all US hospitals has been spotty and, for some safety advocates, too slow. 7 There are many potential reasons for the poor progress in developing a culture of safety: confusion about the difference between safety and quality, concerns that increasing safety will further erode profits, or perhaps simply a lack of attention by institutional leaders. Whatever the reasons for the slow pace of transformation across the nation’s 5,000-plus hospitals, it is likely that this transformation will be even more difficult to achieve in the much larger and more diverse ambulatory setting. Thus, it is important to define and measure an ambulatory culture of safety. It is also difficult, perhaps impossible, to change beliefs, attitudes, knowledge, or actions (all components of a “culture”) without some form of feedback. Therefore, a necessary step in creating a culture of safety is to develop tools to measure the components of that culture. For those individuals and institutions that wish to truly improve the safety of the care they deliver, the creation and testing of tools such as the Safety Attitudes Questionnaire-Ambulatory (SAQ-A) version is critical. Beliefs, attitudes, and knowledge do not always lend themselves to clear-cut end points. Thus, we can expect to see more than one safety culture measuremen

Development of a context model to prioritize drug safety alerts in CPOE systems

Background: Computerized physician order entry systems (CPOE) can reduce the number of medication errors and adverse drug events (ADEs) in healthcare institutions. Unfortunately, they tend to produce a large number of partly irrelevant alerts, in turn leading to alert overload and causing alert fatigue. The objective of this work is to identify factors that can be used to prioritize and present alerts depending on the 'context' of a clinical situation. Methods: We used a combination of literature searches and expert interviews to identify and validate the possible context factors. The internal validation of the context factors was performed by calculating the inter-rater agreement of two researcher's classification of 33 relevant articles. Results: We developed a context model containing 20 factors. We grouped these context factors into three categories: characteristics of the patient or case (e. g. clinical status of the patient); characteristics of the organizational unit or user (e. g. professional experience of the user); and alert characteristics (e. g. severity of the effect). The internal validation resulted in nearly perfect agreement (Cohen's Kappa value of 0.97). Conclusion: To our knowledge, this is the first structured attempt to develop a comprehensive context model for prioritizing drug safety alerts in CPOE systems. The outcome of this work can be used to develop future tailored drug safety alerting in CPOE systems

Long-term follow-up of IPEX syndrome patients after different therapeutic strategies : an international multicenter retrospective study

Background: Immunodysregulation polyendocrinopathy enteropathy x-linked(IPEX) syndrome is a monogenic autoimmune disease caused by FOXP3 mutations. Because it is a rare disease, the natural history and response to treatments, including allogeneic hematopoietic stem cell transplantation (HSCT) and immunosuppression (IS), have not been thoroughly examined. Objective: This analysis sought to evaluate disease onset, progression, and long-term outcome of the 2 main treatments in long-term IPEX survivors. Methods: Clinical histories of 96 patients with a genetically proven IPEX syndrome were collected from 38 institutions worldwide and retrospectively analyzed. To investigate possible factors suitable to predict the outcome, an organ involvement (OI) scoring system was developed. Results: We confirm neonatal onset with enteropathy, type 1 diabetes, and eczema. In addition, we found less common manifestations in delayed onset patients or during disease evolution. There is no correlation between the site of mutation and the disease course or outcome, and the same genotype can present with variable phenotypes. HSCT patients (n = 58) had a median follow-up of 2.7 years (range, 1 week-15 years). Patients receiving chronic IS (n 5 34) had a median follow-up of 4 years (range, 2 months-25 years). The overall survival after HSCT was 73.2% (95% CI, 59.4-83.0) and after IS was 65.1% (95% CI, 62.8-95.8). The pretreatment OI score was the only significant predictor of overall survival after transplant (P = .035) but not under IS. Conclusions: Patients receiving chronic IS were hampered by disease recurrence or complications, impacting long-term.disease-free survival. When performed in patients with a low OI score, HSCT resulted in disease resolution with better quality of life, independent of age, donor source, or conditioning regimen

A prospective study on the natural history of patients with profound combined immunodeficiency: An interim analysis

BACKGROUND: Absent T-cell immunity resulting in life-threatening infections provides a clear rationale for hematopoetic stem cell transplantation (HSCT) in patients with severe combined immunodeficiency (SCID). Combined immunodeficiencies (CIDs) and "atypical" SCID show reduced, not absent T-cell immunity. If associated with infections or autoimmunity, they represent profound combined immunodeficiency (P-CID), for which outcome data are insufficient for unambiguous early transplant decisions. // OBJECTIVES: We sought to compare natural histories of severity-matched patients with/without subsequent transplantation and to determine whether immunologic and/or clinical parameters may be predictive for outcome. // METHODS: In this prospective and retrospective observational study, we recruited nontransplanted patients with P-CID aged 1 to 16 years to compare natural histories of severity-matched patients with/without subsequent transplantation and to determine whether immunologic and/or clinical parameters may be predictive for outcome. // RESULTS: A total of 51 patients were recruited (median age, 9.6 years). Thirteen of 51 had a genetic diagnosis of "atypical" SCID and 14 of 51 of CID. About half of the patients had less than 10% naive T cells, reduced/absent T-cell proliferation, and at least 1 significant clinical event/year, demonstrating their profound immunodeficiency. Nineteen patients (37%) underwent transplantation within 1 year of enrolment, and 5 of 51 patients died. Analysis of the HSCT decisions revealed the anticipated heterogeneity, favoring an ongoing prospective matched-pair analysis of patients with similar disease severity with or without transplantation. Importantly, so far neither the genetic diagnosis nor basic measurements of T-cell immunity were good predictors of disease evolution. // CONCLUSIONS: The P-CID study for the first time characterizes a group of patients with nontypical SCID T-cell deficiencies from a therapeutic perspective. Because genetic and basic T-cell parameters provide limited guidance, prospective data from this study will be a helpful resource for guiding the difficult HSCT decisions in patients with P-CID

Appendix 5: Asynchronous Device Support's errors

this report. 99% of these code are standard tk programming and describing these code is out of the purpose of this report. The 1% left is the Channel Access's extension of the tk version used at CEBAF (the three commands mention above) and the `blt' tk's extension (allowing to create histograms). Location: server (task under VxWorks) which is willing to establish communication with an arbitrary number of clients. Channel access client services are available on both OPIs and IOCs. A client can communicate with an arbitrary number of servers thru the LAN (ethernet). Several Channel Access tools are available (cau, etc...) mainly for debugging purpose. Access to the record's fields (the I/O output data from the device) from an OPI (Hp9000) is just then a matter of how to communicate with the Channel Access application. At CEBAF, the main concern in slow controls is developing a Graphical User Interface (GUI) using tools such as Medm and Tcl/tk. On the site of CEBAF, these applications already include Channel Access interfacing