bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical Features of Lysosomal Acid Lipase Deficiency
Authors: Burton Barbara K. , Deegan Patrick B. , Enns Gregory M. , Guardamagna Ornella , Horslen Simon , Hovingh Gerard K. , Lobritto Steve J. , Malinova Vera , McLin Valerie A. , Raiman Julian , Di Rocco Maja , Santra Saikat , Sharma Reena , Sykut‐Cegielska Jolanta , Whitley Chester B. , Eckert Stephen , Valayannopoulos Vassili , Quinn Anthony G. 
Published: August 2015

AbstractObjective:The aim of this study was to characterize key clinical manifestations of lysosomal acid lipase deficiency (LAL D) in children and adults.Methods:Investigators reviewed medical records of LAL D patients ages ≥5 years, extracted historical data, and obtained prospective laboratory and imaging data on living patients to develop a longitudinal dataset.Results:A total of 49 patients were enrolled; 48 had confirmed LAL D. Mean age at first disease‐related abnormality was 9.0 years (range 0–42); mean age at diagnosis was 15.2 years (range 1–46). Twenty‐nine (60%) were male patients, and 27 (56%) were <20 years of age at the time of consent/assent. Serum transaminases were elevated in most patients with 458 of 499 (92%) of alanine aminotransferase values and 265 of 448 (59%) of aspartate aminotransferase values above the upper limit of normal. Most patients had elevated low‐density lipoprotein (64% patients) and total cholesterol (63%) at baseline despite most being on lipid‐lowering therapies, and 44% had high‐density lipoprotein levels below the lower limit of normal. More than half of the patients with liver biopsies (n = 31, mean age 13 years) had documented evidence of steatosis (87%) and/or fibrosis (52%). Imaging assessments revealed that the median liver volume was ~1.15 multiples of normal (MN) and median spleen volume was ~2.2 MN. Six (13%) patients had undergone a liver transplant (ages 9–43.5 years).Conclusion:This study provides the largest longitudinal case review of patients with LAL D and confirms that LAL D is predominantly a pediatric disease causing early and progressive hepatic dysfunction associated with dyslipidemia that often leads to liver failure and transplantation.
Decreased transcription factor binding levels nearby primate pseudogenes suggests regulatory degeneration
Authors: Douglas Gavin M. , Wilson Michael D. , Moses Alan M. 
Published: August 2015

AbstractCharacteristics of pseudogene degeneration at the coding level are well-known, such as a shift towards neutral rates of nonsynonymous substitutions and gain of frameshift mutations. In contrast, degeneration of pseudogene transcriptional regulation is not well understood. Here, we test two predictions of regulatory degeneration along the pseudogenized lineage: (1) decreased transcription factor binding and (2) accelerated evolution in putative cis-regulatory regions.We find evidence for decreased TF binding levels nearby two primate pseudogenes compared to functional liver genes. We also find evidence for pseudogene-lineage-specific relaxation of sequence constraint on a fragment of the promoter of the primate pseudogene urate oxidase (Uox) and a nearby cis-regulatory module (CRM). However, the majority of TF-bound sequences nearby pseudogenes do not show evidence for lineage-specific accelerated rates of evolution. We conclude that decreases in TF binding level could be a marker for regulatory degeneration, while sequence degeneration in most CRMs may be obscured by background rates of TF binding site turnover.
Motivating teachers’ commitment to change through transformational school leadership in Chinese urban upper secondary schools
Authors: Liu Peng 
Published: August 2015

Purpose– The purpose of this paper is to examine the effects of transformational school leadership on teachers’ commitment to change and the effects of organizational and teachers’ factors on teachers’ perception of transformational school leadership in the Chinese urban upper secondary school context.Design/methodology/approach– The paper mainly uses quantitative methods to explore the relationships between different constructs. The author asks: to what extent can transformational school leadership practices in the urban upper secondary schools of a particular Chinese city explain the variation in teachers’ commitment to change during curriculum reform? What are the effects of organizational and teachers’ factors on teachers’ perceptions of transformational school leadership?Findings– The results of multiple regression analysis showed that the effect of transformational school leadership was moderate when transformational school leadership and teachers’ commitment to change were treated as single variables. Four dimensions of transformational leadership practice together explained the moderate effects on four dimensions of teachers’ commitment to change, among which the effect of managing the instructional program was the most prominent. The results of multiple regression analysis also revealed that variables like culture, strategy, environment, and teachers’ age had significant relationships with teachers’ perceptions of transformational school leadership. Culture, environment, strategy, structure, and teachers’ factors such as age and grade taught had moderate effects on different dimensions of teachers’ perceptions of transformational school leadership.Originality/value– This study is one of the first to explore the effects of transformational school leadership on teachers’ commitment to change in the Chinese urban upper secondary school context. The findings contribute to educational management in China and similar contexts, and this study advances knowledge and furthers the understandings of the transferability of theories to different contexts.
The development of entrepreneurial potential among higher education students
Authors: Varamäki Elina , Joensuu Sanna , Tornikoski Erno , Viljamaa Anmari 
Published: August 2015

Purpose
– The purpose of this paper is to increase the understanding of how entrepreneurial potential is developed among young people. Changes in individuals’ entrepreneurial intentions and the antecedents of intentions are investigated, as well as the impact of entrepreneurship education on the changes.

Design/methodology/approach
– Ajzen’s Theory of Planned Behavior (TPB) is applied. Longitudinal data from 197 HE students, in their first and third year of studies, is examined using path analysis.

Findings
– Overall the entrepreneurial intentions of HE students decreased over time. Intentions decreased particularly for those with high initial level of intentions, whereas the group with increasing intentions rose from low to neutral level of intentions. Changes in attitudes and perceived behavioral control have a significant positive impact. Versatile entrepreneurship courses have direct effect on changes in attitudes. Changes in attitudes have a dual role, as they influence change in both intentions and perceived behavioral control. The developed model explains 19 percent of the variance among women and 28 percent among men, suggesting gender differences in development of intentions.

Research limitations/implications
– Only one way of developing entrepreneurial potential in young people (i.e. education) is covered. The empirical sample is limited to one university.

Practical implications
– The results suggest versatile methods of entrepreneurship education are more effective in developing intentions than perhaps purely active modes. Gender differences should be considered when designing interventions to foster entrepreneurial potential.

Originality/value
– The research confirms with longitudinal individual-level data the applicability of TPB on entrepreneurial intentions and demonstrates the mediated impact of versatile entrepreneurship courses on changes in intentions.
Revisiting control aversion: the role of financiers in connecting entrepreneurs to the marketplace
Authors: Silver Lars , Lundahl Nicolaus , Berggren Björn 
Published: August 2015

Purpose
– The purpose of this paper is to investigate the effects of small business entrepreneurs’ relinquishment of control aversion and the impact of their interaction with external financiers on market connection.

Design/methodology/approach
– Questionnaires were sent to the chief executive officers of small businesses in the manufacturing and professional services sectors. A total of 459 valid responses were analyzed in a structural equation model.

Findings
– The attitude of small business entrepreneurs in relying on financiers’ advice is marked by control aversion. This fear of losing control creates information asymmetry, which in itself leads to decreased financing opportunities for small business entrepreneurs. The results of the study suggest that small firms seeking the aid of financiers will be provided with substantial additional information about the market. Issues pertaining to supply seem to be less relevant than those relating to demand, thus indicating that greater focus should be placed on the investment readiness of small businesses.

Originality/value
– This study emphasizes the importance of the role of attitudes among SMEs in understanding capital market failure and credit rationing.
Entrepreneurship and poetry: analyzing an aesthetic dimension
Authors: Smith Robert 
Published: August 2015

Purpose– The purpose of this paper is to investigate the aesthetic dimension of entrepreneur poems. The notion of the entrepreneur as storyteller, and the entrepreneur story as cultural genres have become so firmly entrenched in the collective social consciousness that little consideration is given to the existence of other narrative genres, such as business poetry as expressions, or manifestations of enterprising behaviour and indeed identities. Poetry, like art, possesses aesthetic dimensions which make it difficult to theorize and analyze. Indeed, as a genre, poetry seldom features as a heuristic device for better understanding entrepreneurial behaviour or learning. This is surprising because poetry in particular is a wonderfully creative and expressive narrative medium and accordingly, many entrepreneurs engage in writing poetry as a form of creative expression.Design/methodology/approach– In this study the author considers the entrepreneur as poet and from a reading of the literatures of entrepreneurship and aesthetics develops an aesthetic framework for analysing entrepreneur poetry which is used to analyze six poems written by entrepreneurs or about entrepreneurs.Findings– That poetry has value in terms of entrepreneurial learning because of its atheoretical nature it permits listeners to experience the emotion and passion of lived entrepreneurial experiences and to relive these vicariously. In particular entrepreneur poems are a variant form of entrepreneur story devoid of the usual cliché.Research limitations/implications– There are obvious limitations to the study in that the analysis of six poems can merely scratch the surface and that aesthetic analysis is by its very nature subjective and open to interpretation. The study opens up possibilities for further research into entrepreneur poems, the aesthetics of other non-standard entrepreneur narratives and consideration of the aesthetic elements of entrepreneurshipper se. Poetics and aesthetics are areas of narrative understanding ripe for further empirical research.Originality/value– The paper is original in terms of creating an aesthetic framework used to analyze entrepreneur poems. Indeed, little consideration had previously been given to the topic.
Using great works of literature in an interdisciplinary, political economy framework
Authors: Mearman Andrew , Snow Steve G. 
Published: August 2015

Purpose
– The purpose of this paper is to contribute to the effectiveness of teaching in economics.

Design/methodology/approach
– This paper provides as a case study, the authors’ reflections on a first-year “learning community” at a US liberal arts college, which combined courses on political science and economics to explore issues of political economy, including poverty and social mobility.

Findings
– The authors argue that, compared with standard delivery, using literature allows increased student access to and, thus, greater engagement with certain theoretical concepts. More specifically, literature can open avenues for critical thought, and challenge pre-existing views, when it illuminates controversial questions without providing obvious answers. It is this open-ended aspect of literature that seems to provide the best opportunity for increased student engagement and critical thinking.

Research limitations/implications
– The arguments in this paper are based on authors’ reflections and would benefit from other empirical analysis. This method of teaching only offers pedagogical opportunities, and learning gains do not occur automatically. Several features of the course design and assessment, and the interaction between disciplines, made the use of literature more likely to be successful.

Practical implications
– These authors also reflect more broadly on their use of literature in other courses, and provide some suggestions for specific works that might be used in further teaching and research.

Originality/value
– This paper contributes to current debate on teaching practice in economics, and will be of use to those seeking to challenge student viewpoints in a subtle yet effective manner.
Ideographic use of economic terms
Authors: Avsar Rojhat Berdan 
Published: August 2015

Purpose– The purpose of this paper is to challenge the claim that economics is of neutral value and unveil common value judgments underlying the standard policy positions in economics. These value judgments are communicated through the economic lexicon.Design/methodology/approach– The author uses discourse analysis and focuses on certain authoritative economic terms, most of which are metaphors, functioning like arguments. The author calls such terms as “deadweight loss” ideographs in the sense McGee (1980) used the term.Findings– Economic language is not neutral. Certain terms that are treated as common sense mask the normative commitments to which economists often are subscribed, consciously or not.Originality/value– The author treats economics as a particular welfare ideology whose normative commitments are communicated by its vocabulary. The critical approach used here is not common in economics. The author argues that implicit biases built in the discipline are reinforced by the particular economic-language awareness, which is vital to maintaining economics as a pluralist discipline.
Eating words: a discourse historical analysis of the public debate over India’s 2013 National Food Security Act
Authors: Kotwal Ashok , Power Kate 
Published: August 2015

Purpose– This paper aims to provide a situated critical discourse analysis of the public debate around India’s 2013 National Food Security Act (NFSA), describing its rhetorical characteristics and the context within which it has taken place.Design/methodology/approach– Using Wodak’s (2001) Discourse Historical Approach (DHA), the authors examine media coverage of the NFSA, attending to perspectivization, intensification and mitigation and representational and argumentational strategies. The authors also consider this coverage in light of its intratextual, intertextual, situational and wider socio-political and economic contexts. The corpus consists of 29 English-language Indian newspaper and magazine articles, published in print and online between 2011 and 2014.Findings– This paper explains the rhetorical purchase of the term “food security” in contemporary Indian public policy debates by comparing the leftist, right wing and centrist arguments.Research limitations/implications– Owing to the detailed qualitative analysis presented here, the corpus is necessarily limited in size. Newspaper articles contributed by one of the authors were omitted from the study.Originality/value– The DHA claims to be an interdisciplinary framework, but relatively few studies involve true cross-disciplinary research. By contrast, this study relies on close collaboration by scholars active in economics and applied linguistics – thus, demonstrating both the potential for, and the value of, working coherently across academic disciplines. Also, unlike most DHA studies, which interrogate dominant discourses, this paper compares diverse discourses competing for influence.
Distracted driving impairs police patrol officer driving performance
Authors: James Stephen M. 
Published: August 2015

Purpose
– Most US states exempt police officers from restrictive distracted laws, and most agencies require officers to use mobile data computers while driving. The purpose of this paper is to examine the impact of a text-based distraction task on officer driving performance.

Design/methodology/approach
– Experienced police patrol officers (n=80) participated in controlled laboratory experiments during which they drove a high-fidelity driving simulator on four separate occasions; twice immediately following five consecutive 10:40 hour patrol shifts (fatigued condition) and again 72 hours after completing the last shift in a cycle (rested condition). In each condition, officers drove identical, counterbalanced 15-minute courses with and without distraction tasks. The research used a within- and between-subjects design.

Findings
– A generalized linear mixed-model analysis of driving performance showed that officers’ distracted driving performance had significantly greater lane deviation (F=88.58, df=1,308, p
<
0.001), instances of unintentionally leaving assigned driving lane (F=64.76, df=1,308, p
<
0.001), and braking latency (F=200.82, df=1,308, p
<
0.001) than during non-distracted drives. These measures are leading indicators for collision risk.

Research limitations/implications
– Simulated driving tasks presented were generally less challenging than patrol driving and likely underestimate the impact of distraction on police driving.

Originality/value
– Police officers appear to drive significantly worse while distracted, and their routine experience with using text-based communication devices while driving does not mitigate the risks associated with doing so. Study results suggest that policing organizations should modify policies, practices, training, and technologies to reduce the impact of distraction on officers’ driving. Failing to do so exposes officers and the communities they serve to unnecessary hazards and legal liabilities.
Office‐Based Tools and Primary Care Visit Communication, Length, and Preventive Service Delivery
Authors: Lafata Jennifer Elston , Shay L. Aubree , Brown Richard , Street Richard L. 
Published: August 2015

BackgroundThe use of physician office‐based tools such as electronic health records (EHRs), health risk appraisal (HRA) instruments, and written patient reminder lists is encouraged to support efficient, high‐quality, patient‐centered care. We evaluate the association of exam room use of EHRs, HRA instruments, and self‐generated written patient reminder lists with patient–physician communication behaviors, recommended preventive health service delivery, and visit length.Research MethodsObservational study of 485 office visits with 64 primary care physicians practicing in a health system serving the Detroit metropolitan area. Study data were obtained from patient surveys, direct observation, office visit audio‐recordings, and automated health system records. Outcome measures included visit length in minutes, patient use of active communication behaviors, physician use of supportive talk and partnership‐building communication behaviors, and percentage of delivered guideline‐recommended preventive health services for which patients are eligible and due. Simultaneous linear regression models were used to evaluate associations between tool use and outcomes. Adjusted models controlled for patient characteristics, physician characteristics, characteristics of the relationship between the patient and physician, and characteristics of the environment in which the visit took place.ResultsPrior to adjusting for other factors, visits in which the EHR was used on average were significantly (p < .05) longer (27.6 vs. 23.8 minutes) and contained fewer preventive services for which patients were eligible and due (56.5 percent vs. 62.7 percent) compared to those without EHR use. Patient written reminder lists were also significantly associated with longer visits (30.0 vs. 26.5 minutes), and less use of physician communication behaviors facilitating patient involvement (2.1 vs. 2.6 occurrences), but more use of active patient communication behaviors (4.4 vs. 2.6). Likewise, HRA use was significantly associated with increased preventive services delivery (62.1 percent vs. 57.0 percent). All relationships remained significant (p > .05) in adjusted models with the exception of that between HRA use and preventive service delivery.Dissemination and Implementation ImplicationsOffice‐based tools intended to facilitate the implementation of desired primary care practice redesign are associated with both positive and negative cost and quality outcomes. Findings highlight the need for monitoring both intended and unintended consequences of office‐based tools commonly used in primary care practice redesign.
When Children are the Least Vulnerable to False Memories: A True Report or a Case of Autosuggestion?
Authors: Brackmann Nathalie , Otgaar Henry , Sauerland Melanie , Jelicic Marko 
Published: August 2015

AbstractIn this case report, a legal case revolving around the reliability of statements given by a 6‐year‐old girl is described. She claimed to have witnessed her mother being murdered by her father. Two psychological experts provided diametrically opposed opinions about the reliability of her statements. One expert, a clinician, opined that the girl's statements were based on autosuggestion whereas the other expert, a memory researcher, stated that autosuggestion was unlikely to have played a role. This case and the analysis of the experts' opinions illustrate what may happen when experts in court are unaware of the recent literature on (false) memory. That is, recent studies show that autosuggestion is less likely to occur in young children than in older children and adults. The current case stresses the importance and implications of relying on memory experts in cases concerning the reliability of eyewitness statements.
Using admixture analysis to examine birth‐cohort effects on age at onset of bipolar disorder
Authors: Golmard J.‐L. , Scott J. , Etain B. , Preisig M. , Aubry J.‐M. , Henry C. , Jamain S. , Azorin J.‐M. , Leboyer M. , Bellivier F. 
Published: August 2015

ObjectiveIt is suggested that age at onset (AAO) of bipolar I disorder (BP‐I) is decreasing. We tested for a birth‐cohort effect on AAO using admixture analysis.MethodA clinical sample of 3896 BP‐I cases was analysed using two approaches: (i) in a subsample with untruncated AAO × birth year distribution (n = 1865), we compared the best‐fitting model for the observed AAO in patients born ≤1960 and >1960, (ii) to control for potential confounders, two separate subsamples born ≤1960 and >1960 were matched for age at interview (n = 250), and a further admixture analysis was undertaken.ResultsThe two approaches indicated that the proportion of cases in the early AAO category was significantly greater in cases born >1960; manic onsets were also more frequent in the early onset BP‐I cases born >1960.ConclusionThe decrease in AAO of BP‐I in recent birth‐cohorts appears to be associated with an increase in the proportion of cases in the early onset subgroup; not with a decrease in the mean AAO in each putative subgroup. This could indicate temporal changes in exposure to risk factors for mania.