Human severe combined immune deficiency (SCID) is the most serious inherited immunological deficit. Recent work has revealed defects in the predominant pathway for double‐strand break repair called nonhomologous DNA end joining, or NHEJ. Progress in the biochemistry and genetics of NHEJ and of human SCID has proven to be synergistic between these two fields in a manner that covers the range from biochemical etiology to considerations about possible gene therapy for the B− SCID patients. BioEssays 25:1061–1070, 2003. © 2003 Wiley (...) Periodicals, Inc. (shrink)

Studying compliance, in terms of the business responses to legal rules, is notoriously difficult. This paper focuses on the difficulty of capturing the behavioral response itself, rather than on difficulties in explaining compliance and isolating particular factors of influence on it. The paper argues that existing approaches to capture such compliance, using surveys and governmental data, run the risk of failing to capture compliance as it occurs in the reality of day-to-day business responses to the law. It does so by (...) means of a unique ethnographic approach to study compliance. Drawing from data of deep participant observation about responses to legal rules in two small businesses, the paper finds that in this context there is Compliance Dynamism. This means that compliance varies for different rules, it varies over time, and businesses learn from one response to the law to the next on a daily basis. Compliance is also situational, and there is an Indirect Observer Effect, where the way compliance is measured, especially when using data derived from inspections, shapes what compliance is observed and what is not. Therefore, compliance should be captured not as a singular state but as a string of reiterative processes that occur in their situational context. And this fundamentally challenges most existing methods to capture compliance and thus our understanding of what compliance occurs and what may shape it. In its conclusion, the paper draws out the implications this has for studies that seek to find simple and usable findings about compliance. (shrink)

ObjectiveTo confirm the effects of a transtheoretical model based on multidimensional life management on healthy behavior in patients with polycystic ovary syndrome.MethodsIn total, eighty eligible patients were recruited from March 2021 to June 2021 and randomly assigned to either the intervention or control groups. Outcome measures include health-promoting behavior, self-efficacy, anthropometrics, and the number of unplanned outpatient admissions. Data were collected at baseline, 3, and 6 months after the intervention. The chi-square test, rank-sum test, t-test, and repeated measurement analysis of (...) variance were used to analyze the data.ResultsIn total, sixty-six participants completed the study: 35 participants in the intervention group and 31 participants in the control group. After 6 months of intervention, the healthy behavior level of patients with PCOS increased from moderate [health-promoting lifestyle profile -II score of 105.37 ± 12.57] to good in the intervention group; and there was no change observed in the control group. In addition, the total self-efficacy score, PCOS-related unplanned outpatient admissions, waist circumference, and body mass index were found to have a significant difference in the intervention group at 6 months. Meanwhile, repeated measures analysis of variance showed a significantly greater improvement in health-promoting behavior and self-efficacy over time in the intervention group than in the control group.ConclusionThe transtheoretical model based on multidimensional life management positively affected healthy behavior, self-efficacy, the number of unplanned outpatient admissions, and anthropometrics in patients with PCOS.Clinical Trial Registrationwww.chictr.org.cn, ChiCTR2000034572. (shrink)

作者对当前的改革以及与此有关的马克思主义理论、党的方针政策、城乡经济、思想修养、人生道德、文化教育、体育艺术、民情反映等问题作了种种思考。.

本书评介了普列汉诺夫及其美学思想,尤其是从艺术的起源、艺术与社会心理等方面讨论了普列汉诺夫的观点,提出许多新的见解。.

Da natureza afetiva da forma na obra de arte.--Forma e personalidade.--Panorama da pintura moderna.

School administrators are mandated to take the instructional leadership roles. On this premise, a study assessed the extent of instructional leadership practices of public elementary school administrators in El Salvador City Division, Philippines. Also, it explored their actual practices, challenges encountered, and the ways they overcome the challenges in practicing instructional leadership. It employed a mixed-method research design. It administered the adopted assessment tool on instructional leadership to 15 school administrators and 12 of them were involved in the individual interviews. (...) This was conducted between the last quarter of 2019 and the first quarter of 2020. Descriptive statistics were used to describe the extent of instructional leadership practices. Also, it analyzed the actual practices, the challenges encountered, and the ways of overcoming these challenges using thematic narrative analysis. Results revealed that public school administrators have always practiced the four domains or strands of instructional leadership to a very high extent. Providing the technical assistance, conducting clinical supervision, and innovating teaching and learning emerged as themes of their actual practices. These administrators had encountered challenges in dealing with teachers’ attitudes, conflicting schedules and activities, and teachers’ resistance to changes. They overcome the challenges by trying to meet the competency standards, adapting and modifying the existing programs, contextualizing teaching and learning, and inculcating the value and benefits of class observation. Looking at the findings from the lens of deliberate practice theory, it was concluded that school administrators have indicated they have acquired knowledge and a high level of understanding of their instructional leadership roles. But despite of this, they still met challenges and have tried their best to manage them. This study presented some doable and practical recommendations to the Department of Education (DepEd) and concerned offices which may benefit both the internal and external stakeholders of the schools. (shrink)

Offers the first focused study of the shifei debates of the Warring States period in ancient China and challenges the imposition of Western conceptual categories onto these debates. In recent decades, a growing concern in studies in Chinese intellectual history is that Chinese classics have been forced into systems of classification prevalent in Western philosophy and thus imperceptibly transformed into examples that echo Western philosophy. Lin Ma and Jaap van Brakel offer a methodology to counter this approach, and illustrate their (...) method by carrying out a transcultural inquiry into the complexities involved in understanding shi and fei and their cognate phrases in the Warring States texts, the Zhuangzi in particular. The authors discuss important features of Zhuangzi’s stance with regard to language-meaning, knowledge-doubt, questioning, equalizing, and his well-known deconstruction of the discourse in ancient China on shifei. Ma and van Brakel suggest that shi and fei apply to both descriptive and prescriptive languages and do not presuppose any fact/value dichotomy, and thus cannot be translated as either true/false or right/wrong. Instead, shi and feican be grasped in terms of a pre-philosophical notion of fitting. Ma and van Brakel also highlight Zhuangzi’s idea of “walking-two-roads” as the most significant component of his stance. In addition, they argue that all of Zhuangzi’s positive recommendations are presented in a language whose meaning is not fixed and that every stance he is committed to remains subject to fundamental questioning as a way of life. (shrink)

本书分上、下两编,上编对各种语言形式语词、句子、篇章与各种思维形式概念、判断、推理、论证作系统地对比研究;下编并对几门语言逻辑分支问题逻辑、模态逻辑、时态逻辑进行比较系统的介绍。.

马克昌(1926~ ),武汉大学法学院教授博士研究生导师,中国法学会常务理事,中国法学会刑法学研究会副总干事.

Since March 14, 2020, Spanish citizens have been confined to their homes due to the impact of the COVID-19 pandemic. Participating in musical activities has been associated with reduced anxiety and increased subjective wellbeing. The aim of this study is to analyze how Spanish citizens used music during the lockdown period. We also study perceptions of the impact music has in everyday life, in particular examining the respondents’ insights into the effects of listening to music in situations of isolation. The (...) study was conducted using the MUSIVID19 questionnaire administered to a total of 1868 Spanish citizens. The results indicate that during lockdown, respondents perceived an increase in the time they devoted to musical activities such as listening, singing, dancing or playing an instrument. The participants also reported using music to cope with the lockdown, finding that it helped them to relax, escape, raise their mood or keep them company. The findings suggest an improvement in their perception of the value of music in personal and social wellbeing during the lockdown. However, the study reveals significant differences in the use and perceptions of music according to respondents’ personal situations. Age and feelings of vulnerability may lead to more conservative uses of musical practice and to more moderate perceptions of the positive values of music. (shrink)

The Global Financial Crisis of 2007–2009 exacerbated two distinct concerns about the independence of central banks: a concern about legitimacy and a concern about economic justice. This paper explores the legitimacy of independent central banks from the perspective of these two concerns, by presenting two distinct models of central banking and their different claims to political legitimacy and distributive justice. I argue primarily that we should avoid construing central bank independence in binary terms, such that central banks either are, or (...) are not, independent. I will argue that we should instead construe central bank independence in scalar terms, so that independence admits of degree, thus allowing us to develop an account of independence in which central banks can retain it to the extent necessary for economic efficiency, while meeting reasonable concerns regarding democratic legitimacy and economic justice. (shrink)

al-Juzʼ al-awwal. Dirāsāt fī al-manṭiq wa-falsafat al-ʻulūm -- al-juzʼ al-thānī. Dirāsāt fī al-manhaj wa-al-ʻilm al-ʻArabī.

Integration und Desintegration sind ein zentrales Thema der Geisteswissenschaften. Auf makropolitischer Ebene gilt das besondere Interesse dem Entstehen und Vergehen von Staaten, entscheiden sich doch hiermit existenzielle Fragen des Seins einer politischen Gemeinschaft. Auf mikropolitischer Ebene geht es um die Frage, inwieweit dem Bürger "aufgegeben" ist, sich in Gesellschaft und Staat zu integrieren. Supranationalisierung und Globalisierung lassen die Kraft des Nationalstaates schwinden. Dem Übergang der Moderne in die Postmoderne entspricht der Übergang von der Welt der Staaten mit ihren durch Grenzen (...) abgeschlossenen Territorien zur Welt der Kontinente, Gemeinschaften und Netzwerke. Doch gibt es viele Stimmen, die meinen, der Staat sei auf absehbare Zeit nicht wegzudenken und als politische Gemeinschaft zu schützen.Werner Mäder ist anderer Auffassung: "Deutschland ist kein Staat mehr..." lautet sein Befund. Der Autor zieht Parallelen zu Hegels Schrift "Kritik der Verfassung Deutschlands" aus dem Jahre 1801 und seinen Kriterien des Staates. Nach Mäder ist das Grundgesetz nur noch eine "Gedankenverfassung", seine fundamentalen Voraussetzungen Staat, Souveränität, Nation, Volk und Demokratie substanziell ausgezehrt. Für Europa bedeutet dies, sich mit eigener Identität selbst zu behaupten, um den Souveränitätsverlust seiner Länder auszugleichen und auf eine neue Multipolarität der internationalen Beziehungen hinzuarbeiten. (shrink)

本书是在总结作者近年来发表的论文的基础上写成的。全书共分三部分,第一部分是矩阵对策上的计策理论;第二部分是连续对策上的计策理论;第三部分是有限人对策上的计策理论.

本书从宇宙太空、万物之始、原子物理、人体生命、社会结构等多维度、多层面剖解世界与人生,辨物析理。.