Miracle-dz/UniFAR-FTUs huggingface dataset:
Clinical and Laboratory Features of Acute Porphyria: A Study of 36 Subjects in a Chinese Tertiary Referral Center

Porphyria is a group of eight metabolic disorders characterized by defects in heme biosynthesis. The presentation of porphyria is highly variable, and the symptoms are nonspecific, which accounts in part for delays in establishing a diagnosis. In this study, we report the characteristics of 36 Chinese acute porphyria patients. Most of them were female (33/36), and the median age was 25.3 years (range 18–45 years). The most frequent presenting symptom was abdominal pain (32/36). Hyponatremia was the most common electrolyte abnormality (29/36), and the serum sodium concentration was significantly negatively correlated with convulsion (p = 0.00). Genetic testing provided a precise diagnosis of the patients. Genetic analysis of the porphobilinogen deaminase (PBGD) gene was performed for 10 subjects. Of them, 9 were found to harbor a mutation in the PBGD gene, proving a diagnosis of acute intermittent porphyria, and, in 1 case, a novel Cys209Term mutation was found.
Viable wikis: struggle for life in the wikisphere

Wikis are collaborative platforms enabling collective elaboration of knowledge, the most famous and possibly the most successful thereof being the Wikipedia. There are currently plenty of other active open-access wikis, with varying success: some recruit many users and achieve sustainability, while others strive to attract sufficient active contributors, irrespective of the topic of the wiki. We make an exploratory investigation of some factors likely to account for these various destinies (such as distinct policies, norms, user incentives, technical and structural features), examining the demographics of a portion of the wikisphere. We underline the intertwining of population and content dynamics and emphasize the existence of different periods of development of a wiki-based community, from bootstrapping by founders with a pre-established set of rules, to more stable regimes where constant enrollment and training of new users balances out the occasional departure of more advanced users.
Productive herpes simplex virus in brain of elderly normal subjects and Alzheimer's disease patients

It was previously shown that herpes simplex virus type 1 (HSV1) DNA resides latently in a high proportion of aged brains and that in carriers of the type 4 allele of the apolipoprotein E gene (APOE‐ε4), it confers a strong risk of Alzheimer's disease. It was suggested that initial entry of brain by HSV1 and any subsequent reactivation(s) would cause a type of limited encephalitis, the resulting damage being more harmful in APOE‐ε4 carriers. Reactivation(s) would induce synthesis of intrathecal antibodies; these are long‐lived after herpes simplex encephalitis so they were sought in cerebrospinal fluid (CSF) of Alzheimer's disease patients and age‐matched normal subjects. Intrathecal antibodies to human herpesvirus 6 (HHV6) were also sought as DNA of this virus has been detected previously in a high proportion of Alzheimer's disease brains. Antibody indices for HSV and HHV6 were measured using indirect ELISA for IgG antibody, and single radial immunodiffusion was used for albumin, in serum and CSF. A raised antibody index (>1.5) indicative of virus‐specific intrathecal HSV1 IgG synthesis was found in 14/27 (52%) Alzheimer's disease patients and 9/13 (69%) age‐matched normals (difference non‐significant). A raised antibody index to HHV6 was detected in 22% of the Alzheimer's disease patients and in no normals, so presumably this virus either did not reactivate in brain or it elicited only short‐lived intrathecal antibodies. The HSV1 results confirm the original PCR findings that show the presence of HSV1 DNA sequences in many elderly brains, and indicate also that the whole functional HSV1 genome is present, and that the virus has replicated. J. Med. Virol. 75:300–306, 2005. © 2004 Wiley‐Liss, Inc.
European standards for mobile communications: the tense relationship between standards and intellectual property rights

this article starts by discussing the nature of European formal telecommunications standards. A new classification of the different types of standards is proposed, and the instutional framework for formal standards in Europe is described. The next section deals with the relevant property rights. We distinguish the strategeis for three different phases in the standardisation process. The next section explains how the instutional field reacted to possible problems of IPRs within telecommunication standard. The subjects of the two final sections, firm strategy and the standardisation bodies' policy, are illustrated in the GSM case.
Optimal Dissent in Organizations

We model an organization as a two-agent hierarchy: an informed Decision Maker in charge of selecting projects and a (possibly) uninformed Implementer in charge of their execution. Both have intrinsic preferences over projects. This paper models the costs and benefits of divergence between their preferences, that is, dissent within the organization. Dissent is useful to (1) foster the use of objective (and sometimes private) information in decision making and (2) give credibility to the Decision Maker's choices. However, dissent comes at the cost of hurting the Implementer's intrinsic motivation, thereby impairing organizational efficiency. We show that dissent can be optimal, in particular, when information is useful and uncertainty is high. Moreover, dissent remains an optimal organizational form even when Implementers can choose their employer or when Decision Makers have real authority over hiring decisions.
Production and Perception of Thai Lexical Tone and Intonation in Children

Previous developmental studies suggested that in tonal languages, both lexical tone and intonation are mastered very early in children. Unlike intonation which generally signals speaker’s attitudes and emotions, lexical tone distinguishes words. Focusing on fundamental frequency (F0) and pitch as main acoustic and perceptual cues, this work explores Thai children’s production and perception of five lexical tones (Experiments 1 and 2) and those of affective utterances (Experiments 3 and 4). Experiment 1 investigates the production of five lexical tones by comparing F0 measures and contours taken from recordings of 15 Thai children aged 2–3;11, 4–5;11 and 6–7;11. In Experiment 2, lexical tone identification of 75 Thai young children aged 2–3;11, 4–5;11 and 6–7;11 was compared using a 2-choice picture identification task. The findings suggested that at the age of 2–3, Thai children were able to produce and differentiate the five lexical tones, but they did not achieve mastery of these contrasts until the later age. In Experiment 3, Thai children’s production of affective utterances (happy–sad and interested–bored) was examined. F0 measures were taken from recordings of 12 Thai children aged 5–7;11 and 8–10;11. Finally, in Experiment 4, sensitivity to the affective intonations in 40 children aged 5;0 –7;11 and 8;0–10;11 was investigated with a 2-choice picture identification task. The results suggested that by the age of 5 Thai children’s ability to produce and their sensitivity to affective intonations were already developed and mastered.
Pupil Imitation of a Rewarding Teacher's Verbal Behavior.

DOCUMENT RESUME SP 003 849 Friedman, Philip; Bowers, Norman D. Pupil Imitation of a Rewarding Teacher's Verbal Behavior. (69] 14p. EDRS Price MF-$00 25 HC-$0.80 Behavior Patterns, *Classroom Observation Techniques, Elementary School Students, *Imitation, Interaction Process Analysis, Preschool Children, Reinforcement, *Student Behavior, Student Teacher Relationship, *Teacher Influencer *Verbal Development Patterns of teacher and student verbal behavior within a framework of reinforcement theory were analyzed through the use of a modified form of the Observation Schedule and Record 4V (OSCAR). The study examined the extent to which the rewarding teacher's classroom discourse served as a model for the verbal style of a student among his peers. Data were initially collected from 30 groups; 10 preschool, 10 kindergarten, and 10 first grade classrooms. From etch of these levels, six classes showing the highest frequency of teacher reinforcing verbal behavior were selected, and designated as the "rewarding teacher" group. Four pupils (two boys, two girls) were chosen at random from each class to form the final sample (N=72) . The verbal statements of these pupils were scored using five scales of imitative behavior developed from the OSCAR protocols. The data were analyzed using a 2 x 3 factoral design (sex x grade levels). Female pupils imitated significantly more than males on four of the five verbal categories. In three of the analyses grade level differences were located, and for only one analysis was there a significant interaction. Three findings are of importance in demonstrating the potential of the OSCAR technique for measuring constructs such as imitation and for measuring pupil as well as teacher behavior. (Included is a 22-item list of references.) (Author) Pupil Imitation of a Rewarding Teacher's Verbal Behavior Philip Friedman and Norman D. Bowers Northwestern University U.S. DEPARTMENT OF HEALTH, EDUCATION & WELFARE OFFICE OF EDUCATION THIS DOCUMENT HAS BEEN REPRODUCED EXACTLY AS RECEIVED FROM THE PERSON OR ORGANIZATION ORIGINATING IT. POINTS OF VIEW OR OPINIONS STATED DO NOT NECESSARILY REPRESENT OFFICIAL OFFICE OF EDUCATION POSITION OR POLICY.
Gold nanoparticles regulate the blimp1/pax5 pathway and enhance antibody secretion in B-cells

Nanoparticles are potential threats to human health and the environment; however, their medical applications as drug carriers targeting cancer cells bring hope to contemporary cancer therapy. As a model drug carrier, gold nanoparticles (GNPs) have been investigated extensively for in vivo toxicity. The effect of GNPs on the immune system, however, has rarely been examined. Antibody-secreting cells were treated with GNPs with diameters ranging from 2 to 50 nm. The GNPs enhanced IgG secretion in a size-dependent manner, with a peak of efficacy at 10 nm. The immune-stimulatory effect reached a maximum at 12 h after treatment but returned to control levels 24 h after treatment. This enhancing effect was validated ex vivo using B-cells isolated from mouse spleen. Evidence from RT-PCR and western blot experiments indicates that GNP-treatment upregulated B-lymphocyte-induced maturation protein 1 (blimp1) and downregulated paired box 5 (pax5). Immunostaining for blimp1 and pax5 in B-cells confirmed that the GNPs stimulated IgG secretion through the blimp1/pax5 pathway. The immunization of mice using peptide-conjugated GNPs indicated that the GNPs were capable of enhancing humoral immunity in a size-dependent manner. This effect was consistent with the bio-distribution of the GNPs in mouse spleen. In conclusion, in vitro, ex vivo, and in vivo evidence supports our hypothesis that GNPs enhance humoral immunity in mouse. The effect on the immune system should be taken into account if nanoparticles are used as carriers for drug delivery. In addition to their toxicity, the immune-stimulatory activity of nanoparticles could play an important role in human health and could have an environmental impact.
Voices of endangerment: : A language ideological debate on the Swedish language

This thesis is concerned with three language debates that reached their most crucial peaks in Sweden at the beginning of the twenty-first century: (i) the debate on the promotion of the Swedish language, (ii) the debate on language testing for citizenship, and (iii) the debate on mother tongue instruction. The main scope of the thesis is to take a theoretically multi-pronged approach to these debates trying to shed light on the following aspects: Why did such debates emerge when they did? Which discourses were available in those specific historical moments? Who are the social actors that intervened in these debates? What is at stake for them? What do they claim? What systems of values, ideas and beliefs – i.e. ideologies – underlie such claims? What are the effects in terms of identities, objects of political intervention, commonsensical knowledge and authority that these discourses and ideologies produce? Taking Sweden as a case in point, the thesis adds to the existing literature another example of how language debates are the manifestation of conflicts between different language ideologies that struggle for hegemony, thus attempting to impose one specific way of envisaging the management of a nation-state in a time of globalisation. In their outer and most patent facets, these struggles deal with the relationships between languages in today’s Sweden, and how the state, through legislation, should – or should not – regulate such relationships in order to (re)produce some kind of linguistic order. However, the thesis also illustrates that when social actors appeal to a linguistic order, they not only draw boundaries between different languages in a given society, but they also bring into existence a social world in which the speakers of those languages come to occupy specific social positions. These linguistic and social hierarchies, in turn, are imbricated in an often implicit moral regime of what counts as good or bad, acceptable or taboo in that society.
Spatial patterns of phylogenetic diversity

Ecologists and conservation biologists have historically used species–area and distance–decay relationships as tools to predict the spatial distribution of biodiversity and the impact of habitat loss on biodiversity. These tools treat each species as evolutionarily equivalent, yet the importance of species' evolutionary history in their ecology and conservation is becoming increasingly evident. Here, we provide theoretical predictions for phylogenetic analogues of the species–area and distance–decay relationships. We use a random model of community assembly and a spatially explicit flora dataset collected in four Mediterranean-type regions to provide theoretical predictions for the increase in phylogenetic diversity – the total phylogenetic branch-length separating a set of species – with increasing area and the decay in phylogenetic similarity with geographic separation. These developments may ultimately provide insights into the evolution and assembly of biological communities, and guide the selection of protected areas.
Immunization with R mutants of S. Minnesota. I. Protection against challenge with heterologous gram-negative bacilli.

Recent studies have demonstrated that the basal core structure of the lipopolysaccharides of most Gram-negative bacilli are of similar or identical chemical structure. The current investigations evaluate the protective effect of immunization with these shared antigens against challenge with two heterologous Gram-negative bacilli, K. pneumoniae and E. coli 107. Active and passive immunization of mice with the Ra, Rb, Rc and Rd 1 mutants of S. minnesota failed to protect against challenge with K. pneumoniae . In contrast, active and passive immunization with the Rd 2 and Re mutants of S. minnesota afforded significant protection against challenge with K. pneumoniae . Similarly, active immunization with Re and passive immunization with Ra, Rc, Rd 2 and Re provided significant protection against challenge with E. coli 107. In all instances, immunization with Re afforded greater protection than any of the other rough mutants of S. minnesota . Active immunization with the Re mutant was considerably less effective than type specific immunization. Immunization with Re increased the LD 50 of E. coli 107 by 10-fold and K. pneumoniae by 100-fold over that of controls, while type specific immunization increased the LD 50 to these organisms by greater than 100-fold and 10,000-fold respectively. The specificity of the protection induced was demonstrated by the loss of the protective activity of rabbit antiserum to Re after adsorption of the serum with dead Re mutants.
Effect of temperature on spiking patterns of neocortical layer 2/3 and layer 6 pyramidal neurons

The spiking patterns of neocortical pyramidal neurons are shaped by the conductances in their apical dendrites. We have previously shown that the spiking patterns of layer 5 pyramidal neurons change with temperature, probably because temperature modulates the electrical coupling between somatic and dendritic compartments. Here we determine whether temperature has similar effects on the spiking patterns of layer 2/3 and layer 6 pyramidal neurons in acute slices of mouse primary motor cortex. In both cell types, decreasing temperature led to more irregular spiking patterns. Our results indicate that a decrease in spiking regularity with decreasing temperature, probably mediated by increased electrical coupling between soma and dendrites, is common to all pyramidal neurons in motor cortex.
Limits to arbitrage in electricity markets

As in most commodities markets, deregulated electricity markets allow the participation of purely financial (virtual) traders to enhance informational and productive efficiency. The presence of financial players is expected, among other things, to help eliminate predictable pricing gaps between forward and spot prices, which may arise in the presence of market power and are linked to productive inefficiency. However, we find that the impact of financial players on reducing pricing gaps has been limited, even using credibly exogenous variation in financial activity to address potential exogeneity. A forward premium persists. We show that financial traders effect on the premium was limited by two barriers. First, arbitrageurs do not have unlimited access to capital. Trading was reduced during the financial crisis, when capital availability was restricted. The second is regulation, as high transaction costs imposed by the regulator restricted arbitrage. Moreover, during this period we observe that some financial players appear to be betting in exactly the opposite direction of the pricing gap, sustaining large losses while doing so. We find evidence consistent with participants using forward market bids to affect congestion and thus increase the value of their Financial Transmission Rights (FTR), i.e. these financial players incur losses with one financial instrument to make larger profits with another, introducing artificial congestion to the system.
Research and research methods for youth practitioners

Rigorous research is crucial to effective work with young people and increasingly youth practitioners need to be able to develop, review and evidence their work using a variety of research and assessment tools. This text equips students and practitioners with a thorough understanding of research design, practice and dissemination, as well as approaches to evidence-based practice. A clear practice framework informs the book, outlining the significance of research to youth work, especially in relation to designing and developing services for young people. Research and Research Methods for Youth Practitioners: Analyses the research/practitioner role Explores the ethical context of research in youth work Offers a thorough analysis of key methodological questions in research in practice Provides a guide to data collection and analysis Presents five principal research strategies for youth work: ethnographic work and visual methods; interviewing and evaluation; surveys and evaluation; the use of secondary data and documentary analysis; and researching virtual and online settings Discusses the implications of research for work with young people as well as its dissemination. Written by experienced researchers and practitioner-researchers, each chapter in this accessible textbook includes an overview, a critical discussion of the pros and cons of the particular method or approach, a case study, a practice-based task, a summary and suggestions for further reading. This textbook is invaluable for student and practising youth workers. It is also a useful reference for other practitioners working with young people.
Association between the androgen levels and erectile function, cognitive functions and hypogonadism symptoms in aging males

Purpose: Aging in men is characterized by a moderate decrease in plasma testosterone (T) levels. However, the association between partial androgen deficiency of the aging male and clinical symptoms and the ideal screening test are controversial. In this study, we investigated the association between the androgen levels and erectile function, cognitive functions and hypogonadism symptoms in aging males. Materials and methods: We investigated the association between total (TT), calculated free (FT) and bioavailable (BT) testosterone, and various clinical and laboratory parameters in 103 healthy males, 50–80 years old. Biochemical assessment was done after overnight fasting. Questionnaires were used to test for hypogonadism symptoms, erectile and cognitive functions. Results: TT levels were not correlated with aging in this study. However, FT and BT were found to decrease with age due to rising sex hormone binding globulin. TT levels were strongly correlated with FT and BT levels (respectively p = 0.0001, p = 0.0001). TT, FT and BT were only correlated with cognitive functions (p = 0.012, p = 0.004, p = 0.02 respectively). There was no correlation between TT, FT and BT levels and erectile function and hypogonadism symptoms. Conclusion: T values in our study sample did not correlate with clinical signs and symptoms of hypogonadism. Thus, according to our data, symptoms in the aging male should not be indiscriminately assigned to a decrease in TT, FT or BT levels.
Meaning in action : interpretation and dialogue in policy analysis

This accessible book gives academics, graduate students, and researchers a comprehensive overview of the vast, varied, and often confusing landscape of interpretive policy analysis. It is both theoretically informed and clear and jargon-free as it discusses the specific strengths and weaknesses of different interpretive approaches--all with a practical orientation towards doing policy analysis
Movement programmes as a means to learning readiness

Movement is a prerequisite for learning readiness. There continues to be growing research that movement and bodywork improve brain function and learning. Learning readiness requires the effective functioning of lower level systems in order for the higher level systems to perform adequately. The aim of this study was to determine whether a specifically designed movement programme, which includes activities for developing the sensory-motor system, can help the educator to improve the child’s learning readiness. The systems specified in this study include the vestibular, proprioceptive, tactile, visual and auditory systems. A one-group pre-test/post-test design was selected to obtain data before the implementation of a movement programme regarding Grade 2 learners (N=14) who experienced barriers to learning, as well as to determine the impact of the movement programme. After applying the t-test in the different subtests, no significant differences between the pre-test and post-test were found. However, the significant results that were obtained must be considered with caution because of the size of the sample.
Business Cycles in the Maghreb: Does Trade Matter?

In this paper, we test the Frankel & Rose hypothesis of Optimum Currency Area for the Maghreb countries by demonstrating how the co-movements of outputs would respond to a trade integration process. In particular, by using panel analysis over the period 1980~2010, we evaluate if a monetary integration project across these countries is endogenous. Our main result suggests that while trade intensity may help to harmonize business cycles, the magnitude of this harmonization is lower for the Maghreb countries than for industrial countries. This result is robust even when we take into consideration many control variables including intra-industry trade, economic diversification, and financial integration. Therefore, we suggest that an acceleration of trade linkage as well as product diversification should take place prior to any move towards monetary union across the Maghreb countries.