bluuebunny/crossref_metadata_2025 huggingface dataset:
The role of cooperative alliances in internationalization strategy
Authors: Morais Flávio , Franco Mário
Published: August 2018

Purpose

From the network approach, the purpose of this paper is to provide empirical evidence about the role of international cooperative alliances (CAs) in the decision of small- and medium-sized enterprises (SMEs) to internationalize and remain in the external market.

Design/methodology/approach

To fulfill the proposed objective, qualitative research was carried out through analyzing two SMEs (case studies) in the Portuguese textile sector. Data collection was based on structured interviews, direct observation and some documental analysis.

Findings

The results show that the analyzed SMEs internationalized immediately after their creation, with this decision not being influenced by CAs but rather by the founder’s role and above all by a saturated domestic market.

Practical implications

Alliances are determinant in subsequent developments in the foreign market, allowing SMEs to reach new markets, increase their market share and consolidate their name in the international market. Thus, managers/entrepreneurs should adopt strategies that stimulate the firm’s expansion in the external market, prioritizing the formation of CAs with international partners.

Originality/value

This study suggests that none of the dominant models can exclusively explain the internationalization process of the studied SMEs. Therefore, this research has implications for the recent stream of literature that argues for a holistic view of internationalization models, considering the arguments of all models instead of only one.
Impact of mentoring on practicing engineers: a meta-synthesis
Authors: Wong Silky S.K. , Cross Jennifer A. , Mueller Phillip S.
Published: August 2018

Purpose

This paper aims to present a literature review to examine the career development outcomes on practicing engineering novices who are being mentored at work, and factors of mentoring that contribute to those career development outcomes.

Design/methodology/approach

Through a meta-synthesis, the research premises and findings of 12 articles identified through a systematic search of nine databases were examined and synthesized, to identify the most common career development outcomes and the most influential factors contributing to these outcomes.

Findings

The most common contributing factors were found to be job characteristics, career development mentoring support, psychosocial mentoring support and mentoring methods. The most frequent career outcomes were career satisfaction and promotion.

Research limitations/implications

The sample consisted of only 12 articles; however, according to the guidelines of meta-synthesis and Critical Appraisal Skills Program (CASP), the sample size was ideal. Further, articles were all of high or very high quality, and, in all studies, multiple contributing factors were measured to investigate the relationships among those factors and career outcomes. The factors and outcomes can be considered by researchers as study variables in future studies.

Practical implications

Organizations and practitioners can use factors that influence mentoring outcomes and the mentoring outcomes found in this study to develop more effective mentoring programs.

Originality/value

To the authors’ knowledge, this is the first systematic review of prior research regarding mentoring for practicing engineers. This paper allows researchers and practitioners to identify key findings and trends in past works, recognize research gaps and propose future research directions.
Social learning constructs and employee learning performance in informal Web-based learning environments
Authors: Lee HyunKyung , Lee MyungGeun
Published: August 2018

Purpose

The purpose of this paper is to investigate the relationship between social learning constructs and perceived learning performance in corporate informal Web-based learning environments. The study aims at providing significant implications for corporate educators who have worked on designing social learning environments in the workplace.

Design/methodology/approach

To identify the casual relationship of the proposed research model, data collected from 523 South Koreans who were corporate employees and social media users were analyzed using structural equation modeling.

Findings

The results indicate that self-motivation, learning community and social media usage were significantly related to perceived learning performance. In addition, social media usage mediated the relationship between the other social learning constructs and the learning performance.

Originality/value

Given that corporate personnel typically gain job-related knowledge and skills through social learning, corporate educators need to provide learners with social learning environments that are conducive to self-motivation and learning community. Social media, when used as a learning tool, might not sufficiently improve learning performance without the help of other social learning constructs. Findings shed light on which social learning constructs are essential to effective social learning environment design in the workplace.
Understanding intellectual capital disclosure in online media Big Data
Authors: Ndou Valentina , Secundo Giustina , Dumay John , Gjevori Elvin
Published: August 2018

Purpose

Intellectual capital disclosure (ICD) in universities is gaining increasing attention, especially through the adoption of innovative technologies. Online media, as a relevant source of Big Data, is shifting ICD. The purpose of this paper is to explore how Big Data generated through online media, such as websites and platforms like Facebook, can be used as rich sources of data and viable disclosure channels for ICD in a university.

Design/methodology/approach

This is an exploratory case study, following the methodology in Yin (2014), that examines how online media data contributes to closing the ICD gap. The IC disclosed through different online media channels by a private university in Albania is analysed using Secundo et al.’s (2016) collective intelligence framework. The online data sources include the university’s website, Facebook page, periodic reports and statements outlining future goals.

Findings

What the authors discover in this research is that IC is an important part of how universities operate, and IC is communicated through social media, although unintentionally. However, this only serves to highlight the importance of IC, and if researchers want to discover IC and understand how it works in an organisation, they need to include social media and a prime resource for developing that understanding.

Research limitations/implications

Most importantly, the findings add to a growing consensus that ICD researchers, and researchers in other management and accounting disciplines, who traditionally rely on annual corporate social responsibility and other periodic reports, they need to change their medium of analysis because these reports no longer can be relied on to understand IC and its impact on an organisation.

Originality/value

Online media tools and the advent of Big Data have created new opportunities for universities to disclose their IC information to stakeholders in a timely manner and to gain relevant insights into their impact on the society. The originality of the paper resides in the contribution of Big Data to the ICD research stream.
Determining and prioritizing man-made threats in critical infrastructures
Authors: Jalali Farahani Gholamreza , Hosnavi Reza , Ataee Mohamad Hasan , Ghanbary Nasab Ali , Ataee Mohammad Ali
Published: August 2018

Purpose

The purpose of this paper is to determine and prioritize man-made threats that have the probability of occurring in civilian airports, as an important part of critical infrastructures in each country.

Design/methodology/approach

The paper is practical-developmental in terms of research type and its approach is descriptive. Desktop research methods and interviews (qualitative) have been used in identifying deliberate man-made threats that are faced by the civilian airports. Moreover, a questionnaire (quantitative) has been utilized to assess the civilian airports’ assets that are targets likely to be threatened and assess threats by determining and prioritizing.

Findings

The results of the paper show that the main threat faced by civilian airports is “Air and missile strikes.” An additional 15 threats endangering civilian airports that have been extracted and presented through integrating specialized literature were prioritized in the main components of civilian airports. This prioritization has been performed on the basis of the following five indicators: “Damage Severity,” “Threat Precedent,” “Target Attractiveness,” “Negative Consequences for the Enemy” and “Enemy Ability.”

Originality/value

This paper is extracted from master’s thesis that identifies and assesses the threats to civil airport, and prioritizes them, and the results were confirmed by the experts.
Self-emulsified waterborne epoxy hardener without acid neutralizers and its emulsifying and curing properties
Authors: Yin Hao , Wan Yun , Zhou Jinjun , Sun Dewen , Li Bo , Ran Qianping
Published: August 2018

Purpose

The purpose of this study is to prepare a new self-emulsified waterborne epoxy hardener which can emulsify the liquid epoxy resin in aqueous media without addition of acid neutralizers.

Design/methodology/approach

Two synthetic steps were adopted to prepare novel self-emulsified amine-epoxy adduct type hardener composition based on the reaction of a commercially available polyetheramine, bisphenol A epoxy resin and triethylene tetramine. The different factors affecting the synthesis of the waterborne epoxy hardener were explored by emulsifying and curing properties. The process and products were analyzed and confirmed by FIRT.

Findings

Compared to an introduction of polyether as a hydrophilic segment in previous literature, whose reaction required Lewis acid catalysts and strict control of the presence of water, the introduction of polyetheramine to obtain a novel self-emulsified waterborne epoxy hardener were concise and convenient by mere two amine-epoxy addition steps in this research. Moreover, the final product offered good film formation with a practical value of mechanical properties, glass transition temperature and water-resistant property.

Practical implications

The self-emulsified waterborne epoxy hardener can be used to prepare water-resistant waterborne coatings from liquid epoxy resins while also providing the option of zero VOC formulations.

Originality/value

Introduction of hydrophilic polyether amine to prepare self-emulsified waterborne epoxy hardener without acid neutralizers has not been systematically studied previously.
Tensile behavior of aloe vera fiber reinforced epoxy and polyester resin matrix composites
Authors: Jenarthanan M.P. , Marappan Karthikeyan
Published: August 2018

Purpose

This paper aims to investigate the tensile behavior of epoxy and polyester matrix composites reinforced with continuous and aligned aloe vera fibers.

Design/methodology/approach

Composites with different volume fractions (30, 40 and 50 Vol. %) were fabricated by laying the fibers in a steel mould and pouring liquid resin, either DEGEBA/TETA epoxy or methyl-ethyl ketone hardened orthophthalic polyester, under pressure of 3 MPa. The specimens were cured for 24 h at room temperature and then tested in a universal Instron testing machine, model 5582, at 298 K (25°C).

Findings

The fracture surface was analyzed by scanning electron microscopy (SEM) under an acceleration voltage of 15 kV. SEM fractography revealed a poor adhesion between both the epoxy and polyester matrices with the aloe vera fiber. The results showed that in both cases the introduction of aloe vera fibers had a minor effect on the matrix reinforcement.

Originality/value

Investigation and comparison of tensile behavior of epoxy and polyester matrix composites reinforced with continuous and aligned aloe vera fibers have not been attempted so far.
Effect of feeding regimen on the fatty acid profile of sheep bulk tank milk
Authors: Renes Erica , De la Fuente Fernando , Fernández Domingo , Tornadijo María Eugenia , Fresno José María
Published: August 2018

The quality of dairy products is affected by the fatty acid (FA) profile of the milk. The aim of this study was to determine whether the feeding regimen and lactation stage of the sheep produced a healthier FA profile of milk. The study was carried out on 30 commercial farms, and the feeding regimens studied were different grazing allowances (50%, G1; 25%, G2; 0%, G3). The variance explained by the lactation stage for the FAs was below 4.18%. The milk from the grazing allowance G1 (50% grazing time per day plus alfalfa silage and barley grains) showed a higher conjugated linoleic acid (1.16%) proportion, followed by the grazing allowances G2 (0.90%) and G3 (0.79%), which showed that grazing improved the nutritional quality of milk fat.
Environmental safety: Air pollution while using MIRUS™ for short‐term sedation in the ICU
Authors: Herzog‐Niescery Jennifer , Vogelsang Heike , Gude Philipp , Seipp Hans‐Martin , Uhl Waldemar , Weber Thomas P. , Bellgardt Martin
Published: August 2018

Background

MIRUS™ is a device for target‐controlled inhalational sedation in the ICU in combination with use of isoflurane, or sevoflurane, or desflurane. The feasibility of this device has recently been proven; however, ICU staff exposure may restrict its application. We investigated ICU ambient room pollution during daily work to estimate ICU personnel exposure while using MIRUS™.

Methods

This observational study assessed pollution levels around 15 adult surgical patients who received volatile anaesthetics‐based sedation for a median of 11 hours. Measurements were performed by photoacoustic gas monitoring in real‐time at different positions near the patient and in the personnel's breathing zone. Additionally, the impact of the Clean Air™ open reservoir scavenging system on volatile agent pollution was evaluated.

Results

Baseline concentrations [ppm] during intervention and rest periods were isoflurane = 0.58 ± 0.49, = 5.72; sevoflurane = 0.22 ± 0.20, = 7.93; and desflurane = 0.65 ± 0.57, = 6.65. Refilling MIRUS™ with liquid anaesthetic yielded gas concentrations of = 2.18 ± 1.48 ppm and = 13.03 ± 9.37 ppm in the personnel's breathing zone. Air pollution in the patient's room was approximately five times higher without a scavenging system.

Conclusion

Ambient room pollution was minimal in most cases, and the measured values were within or below the recommended exposure limits. Caution should be taken during refilling of the MIRUS™ system, as this was accompanied by higher pollution levels. The combined use of air‐conditioning and gas scavenging systems is strongly recommended.
Loss of periostin occurs in aging adipose tissue of mice and its genetic ablation impairs adipose tissue lipid metabolism
Authors: Graja Antonia , Garcia‐Carrizo Francisco , Jank Anne‐Marie , Gohlke Sabrina , Ambrosi Thomas H. , Jonas Wenke , Ussar Siegfried , Kern Matthias , Schürmann Annette , Aleksandrova Krasimira , Blüher Matthias , Schulz Tim J.
Published: August 2018

Remodeling of the extracellular matrix is a key component of the metabolic adaptations of adipose tissue in response to dietary and physiological challenges. Disruption of its integrity is a well‐known aspect of adipose tissue dysfunction, for instance, during aging and obesity. Adipocyte regeneration from a tissue‐resident pool of mesenchymal stem cells is part of normal tissue homeostasis. Among the pathophysiological consequences of adipogenic stem cell aging, characteristic changes in the secretory phenotype, which includes matrix‐modifying proteins, have been described. Here, we show that the expression of the matricellular protein periostin, a component of the extracellular matrix produced and secreted by adipose tissue‐resident interstitial cells, is markedly decreased in aged brown and white adipose tissue depots. Using a mouse model, we demonstrate that the adaptation of adipose tissue to adrenergic stimulation and high‐fat diet feeding is impaired in animals with systemic ablation of the gene encoding for periostin. Our data suggest that loss of periostin attenuates lipid metabolism in adipose tissue, thus recapitulating one aspect of age‐related metabolic dysfunction. In human white adipose tissue, periostin expression showed an unexpected positive correlation with age of study participants. This correlation, however, was no longer evident after adjusting for BMI or plasma lipid and liver function biomarkers. These findings taken together suggest that age‐related alterations of the adipose tissue extracellular matrix may contribute to the development of metabolic disease by negatively affecting nutrient homeostasis.
Customised growth charts in large‐for‐gestational‐age infants and the association with emergency caesarean section rate
Authors: Pritchard Natasha , Lindquist Anthea , Hiscock Richard , Diksha Prerna , Walker Susan P. , Permezel Michael
Published: August 2018

Background and Aim

Large‐for‐gestational‐age (LGA) infants are at increased risk of intrapartum complications. However, some infants classified as LGA may be appropriate‐for‐gestational‐age (AGA) if adjusted for maternal stature. We determined whether customisation of birthweight centiles by maternal height, or height and weight, improves the detection of LGA infants at risk of complications.

Methods

We conducted a retrospective analysis of 38 246 term, singleton nulliparous women. We compared population birthweight centiles to those customised by height, or height and weight for complications including intrapartum caesarean section, instrumental delivery, postpartum haemorrhage, anal sphincter injury and neonatal outcomes.

Results

Those considered LGA when customised for height but AGA by population centiles (LGA‐ht‐only) were at increased risk of intrapartum emergency caesarean section compared with infants AGA on all charts (AGA‐all); odds ratio (OR) 4.64, 95% CI 3.22–6.76. In contrast, infants considered LGA on population charts, but AGA when customised by height (LGA‐pop‐only) were not at increased risk compared to the AGA‐all group (OR 1.43, 95% CI 0.70–1.88). Infants classified as LGA‐ht‐only compared to LGA‐pop‐only remained at significantly higher risk after adjustment for potential confounders (aOR 3.27; 95% CI 2.02–5.31). No difference was seen for any other outcomes. No benefit was seen with customisation by both maternal height and weight.

Conclusion

Women with an infant classified as AGA on population centiles but LGA when customised for height are at increased risk of intrapartum caesarean section. This is a population unrecognised in current practice. Fetal growth should be customised for maternal height when making assessments regarding the LGA infant.
A critical analysis of technology‐enhanced learning design frameworks
Authors: Bower Matt , Vlachopoulos Panos
Published: August 2018

Numerous models have been developed to help teachers efficiently and effectively design learning opportunities using new and emerging technologies. However, the literature to date makes little reference to the variation that exists within the models and frameworks as far as their scope, context, epistemological and pedagogical underpinnings and so on. In this paper, we critically contrast models of technology‐enhanced learning design in order to support educator selection of models, as well as to derive an overarching understanding of how learning design models may differ ontologically. A total of 21 models were selected from a systematic search of the technology‐enhanced learning design research literature. Findings indicated that technology‐enhanced learning design models can be differentiated according to whether they constitute a conceptual framework or a procedural method, their epistemological and pedagogical underpinnings, the level of granularity of the model, the extent to which contextual elements are considered, whether interactions between teachers and students are integral, whether guidance for selecting technologies is included and whether any sort of evaluation of the model has been conducted. The utility of each of these dimensions in terms of supporting technology‐enhanced learning design is considered, and the value more broadly of learning design models is critically discussed.
Dimerised heterobifunctional reactive dyes. Part 1: characterisation using quadrupole time‐of‐flight mass spectrometry
Authors: Sultana Nadia , Liu Yixin , Szymczyk Malgorzata , Freeman Harold S. , Vinueza Nelson R.
Published: August 2018

As part of an approach to enhancing the efficiency of reactive dye adsorption on cellulosic fibres at low electrolyte levels, commercially available dyes were dimerised using hexamethylenediamine (HMDA) as a linking group. A key component of this work involved using high‐resolution mass spectrometry (HRMS) to characterise a group of polysulphonated heterobifunctional monochlorotriazine/vinyl sulphone reactive dyes (CI Reactive Yellow 176, CI Reactive Red 239, CI Reactive Blue 221, CI Reactive Red 194 and CI Reactive Blue 222) and their dimeric analogs. In this respect, dimeric dye ions of differently charged states were observed using HRMS‐negative electrospray ionisation in combination with quadrupole time‐of‐flight mass spectrometry. For example, HRMS showed that the HMDA‐linked reaction products were mixtures of the target (unhydrolysed) dimers, hydrolysed dimers, monoreacted products and hydrolysed unreacted dyes, with CI Reactive Yellow 176 and CI Reactive Red 194 producing the desired unhydrolysed dimers.
Men and women respond differently to rapid weight loss: Metabolic outcomes of a multi‐centre intervention study after a low‐energy diet in 2500 overweight, individuals with pre‐diabetes (PREVIEW)
Authors: Christensen Pia , Meinert Larsen Thomas , Westerterp‐Plantenga Margriet , Macdonald Ian , Martinez J. Alfredo , Handjiev Svetoslav , Poppitt Sally , Hansen Sylvia , Ritz Christian , Astrup Arne , Pastor‐Sanz Laura , Sandø‐Pedersen Finn , Pietiläinen Kirsi H. , Sundvall Jouko , Drummen Mathijs , Taylor Moira A. , Navas‐Carretero Santiago , Handjieva‐Darlenska Teodora , Brodie Shannon , Silvestre Marta P. , Huttunen‐Lenz Maija , Brand‐Miller Jennie , Fogelholm Mikael , Raben Anne
Published: August 2018

Aims

The PREVIEW lifestyle intervention study (ClinicalTrials.gov Identifier: NCT01777893) is, to date, the largest, multinational study concerning prevention of type‐2 diabetes. We hypothesized that the initial, fixed low‐energy diet (LED) would induce different metabolic outcomes in men vs women.

Materials and methods

All participants followed a LED (3.4 MJ/810 kcal/daily) for 8 weeks (Cambridge Weight Plan). Participants were recruited from 8 sites in Europe, Australia and New Zealand. Those eligible for inclusion were overweight (BMI ≥ 25 kg/m2) individuals with pre‐diabetes according to ADA‐criteria. Outcomes of interest included changes in insulin resistance, fat mass (FM), fat‐free mass (FFM) and metabolic syndrome Z‐score.

Results

In total, 2224 individuals (1504 women, 720 men) attended the baseline visit and 2020 (90.8%) completed the follow‐up visit. Following the LED, weight loss was 16% greater in men than in women (11.8% vs 10.3%, respectively) but improvements in insulin resistance were similar. HOMA‐IR decreased by 1.50 ± 0.15 in men and by 1.35 ± 0.15 in women (ns). After adjusting for differences in weight loss, men had larger reductions in metabolic syndrome Z‐score, C‐peptide, FM and heart rate, while women had larger reductions in HDL cholesterol, FFM, hip circumference and pulse pressure. Following the LED, 35% of participants of both genders had reverted to normo‐glycaemia.

Conclusions

An 8‐week LED induced different effects in women than in men. These findings are clinically important and suggest gender‐specific changes after weight loss. It is important to investigate whether the greater decreases in FFM, hip circumference and HDL cholesterol in women after rapid weight loss compromise weight loss maintenance and future cardiovascular health.
The price of the poor's words: social relations and the economics of deposing for one's ‘betters’ in early modern England
Authors: Taylor Hillary
Published: August 2018

Historians of early modern England are aware that the legal testimony of poor, dependent, and subordinated individuals was regarded with suspicion. Contemporaries believed that labouring people would provide false evidence in return for ‘gifts’ or ‘rewards’. To what extent did such assumptions accurately reflect the processes whereby such witnesses came to depose for their ‘betters’? This article uses sixteenth‐ and early seventeenth‐century perjury and subornation suits from the court of Star Chamber to reconstruct labouring people's experiences and understandings of the politics of testimony. In explicating the structural and material factors that could militate against their deposing, override their reservations about doing so, and colour the contents of the depositions they gave, it makes two broader contributions to our understanding of the period. On the one hand, it presents a markedly more pessimistic account of the social relations involved in the increase in litigation. On the other, it reappraises a category of source—depositions—that historians have long regarded as providing singularly privileged access to the expressions of social groups that left little trace in the historical record.
Forecasting China's industrial output using a spatial Bayesian vector autoregressive model
Authors: Lacombe Donald J. , Michieka Nyakundi M.
Published: August 2018

This paper forecasts cement, steel, and TV production in China's top industrial provinces using a Bayesian prior that incorporates both time and spatial dependence as proposed by LeSage and Cashell (2015). Results indicate that growth in cement production will increase following the 3‐year slump experienced between 2013 and 2016 in the five provinces in our sample. Average growth rates for steel production between 2017 and 2018 are similar to those experienced in 1999 and 2008. Our findings indicate that forecast accuracy for TV production demonstrate the superior forecasting characteristics of the hybrid prior.
Mammalian embryos show metabolic plasticity toward the surrounding environment during neural tube closure
Authors: Miyazawa Hidenobu , Yamamoto Masamichi , Yamaguchi Yoshifumi , Miura Masayuki
Published: August 2018

Developing embryos rewire energy metabolism for developmental processes. However, little is known about how metabolic rewiring is coupled with development in a spatiotemporal manner. Here, we show that mammalian embryos display plasticity of glucose metabolism in response to the extracellular environment at the neural tube closure (NTC) stage, when the intrauterine environment changes upon placentation. To study how embryos modulate their metabolic state upon environmental change, we analyzed the steady‐state level of ATP upon exposure to extrauterine environments using both an enzymatic assay and a genetically encoded ATP sensor. Upon environmental changes, NTC‐stage embryos exhibited increased ATP content, whereas embryos before and after NTC did not. The increased ATP in the NTC‐stage embryos seemed to depend on glycolysis. Intriguingly, an increase in mitochondrial membrane potential (ΔΨm) was also observed in the neural ectoderm (NE) and the neural plate border of the non‐neural ectoderm (NNE) region. This implies that glycolysis can be coupled with the TCA cycle in the NE and the neural plate border depending on environmental context. Disrupting ΔΨm inhibited folding of the cranial neural plate. Thus, we propose that embryos tune metabolic plasticity to enable coupling of glucose metabolism with the extracellular environment at the NTC stage.
Review of molecular mechanisms at distal Xq28 leading to balanced or unbalanced genomic rearrangements and their phenotypic impacts on hemophilia
Authors: Lannoy N. , Hermans C.
Published: August 2018

The distal Xq28 region is very gene‐rich, comprising a relatively large number of low‐copy repeats (LCRs) predisposing to genomic rearrangements. The best‐known rearrangement at this locus is the F8 intron 22 inversion, responsible for up to 45% of severe hemophilia A (HA) cases. An additional inversion of intron 1 of F8 has more recently been described, affecting 2%‐5% of patients with severe HA. These “balanced” rearrangements are mediated by intrachromosomal homologous recombination between inversely oriented LCRs located in intron 1 or 22 and other extragenic copies positioned more telomerically outside the F8 gene. The successive innovations of semi‐quantitative technologies like multiplex ligation‐dependent probe amplification (MLPA) and array comparative genomic hybridization (array CGH) have rendered it possible to highlight a significant number of “unbalanced” rearrangements associated or not with these inversions. Some rearrangements are generated by the non‐allelic homologous recombination (NAHR) pathway between directly oriented LCRs. Others are probably the result of unequal crossing‐over or U‐loop exchanges during female meiosis. This review sought to provide an overview of the mechanisms underlying rearrangements at the distal Xq28 locus and discuss their clinical impacts other than HA, such as risks of developing high inhibitor levels and spontaneous abortion, as well as other pathologies like cardiovascular disease or potentially X‐linked intellectual disease.
Value of prophylaxis vs on‐demand treatment: Application of a value framework in hemophilia
Authors: Nugent Diane , O'Mahony Brian , Dolan Gerry ,   on behalf of the International Haemophilia Access Strategy Council
Published: August 2018

Introduction

Therapeutic advances over the past 30 years have led to longer life expectancy and improved quality of life (QOL) for persons with hemophilia. Access to innovative therapy may be compromised if treatment decisions are driven solely by cost. New strategies are needed to assess true therapeutic values, along with financial cost, as physicians, policymakers, payers and manufacturers work together to improve patient care.

Aim

To provide an evidence‐based assessment of the value of prophylaxis vs on‐demand therapy for hemophilia, based on a widely recognized three‐tiered value framework approach for assessing a range of therapeutic interventions.

Methods

Data from six randomized clinical trials (ESPRIT, Joint Outcomes Study, SPINART, LEOPOLD II, ADVATE and POTTER) and four observational studies comparing primary and secondary prophylaxis vs on‐demand therapy were applied to a hemophilia value framework.

Results

Both primary and secondary prophylaxis showed advantages in Tier 1 “Degree of health/recovery” outcomes, including measures of bleeding, musculoskeletal complications, pain, function/activity and QOL. Tier 2 “Process of Recovery” outcomes, also favoured prophylaxis, including measures of recovery time, return to normal activities, orthopaedic intervention and venous access. In Tier 3 “Sustainability of Health Recovery,” measures of breakthrough bleeds, joint preservation, sustained productivity and QOL showed significant improvement with prophylaxis.

Conclusion

The hemophilia value framework affirmed value of primary and secondary prophylaxis vs on‐demand therapy, with clinical benefit demonstrated in all three tiers. This analysis also demonstrates clinical utility of the value framework process in the determination of optimal and cost‐effective hemophilia care for all stakeholders.
Impact of target joint and FVIII inhibitor οn bone properties in children with haemophilia A: A peripheral quantitative computed tomography study
Authors: Xafaki Panagiota , Balanika Alexia , Pergantou Helen , Papakonstantinou Olympia , Platokouki Helen
Published: August 2018

Background

Haemophilic children are prone to low bone mass accrual.

Objective

To assess bone properties in haemophilic children, using peripheral quantitative computed tomography (pQCT) and to correlate findings with clinical data.

Subjects/Methods

Peripheral quantitative computed tomography scan of both radii and tibiae were performed in 31 haemophilic A children (severe 24, mean age 11.2 years). Seven subjects had a history of inhibitors. Five children had an upper extremity target‐joint and 12 had at least one lower extremity target‐joint. The following parameters were measured: trabecular, total and cortical bone density and content (TBD, ToBD, CBD, TbC, CC), strength‐stress index (SSI), and tibial cross‐sectional area (CA), outer and inner bone contour length (PERI, ENDO) and cortical thickness (CTHC).

Results

Mean right radius TBD was significantly higher than the left one (P = 0.015). In subjects with arm target‐joint, radius TBD was significantly lower in the target than in non‐target arm (186.6 ± 60.4 vs 218.6 ± 39.8, P = 0.032). Left arm target‐joint subjects had significantly lower left radius TBD in comparison to subjects without arm target‐joint (155.4 ± 50.3 vs 215.7 ± 37.9, P = 0.019). There were no similar differences in leg target‐joint. Bone quality and geometry parameters in cortical compartment were significantly lesser in inhibitor group, with statistically significant side‐to‐side differences for legs and arms and left side predominance.

Conclusion

In children with haemophilia A and a history of target‐joint and/or FVIII inhibitor, abnormalities may occur in the long bones as were revealed by pQCT, where low trabecular density and weak cortical bone quality in upper and lower extremities, respectively, were confirmed.
A will and a way to fund medicines for rare diseases: the story of human growth hormone replacement for adults with growth hormone deficiency
Authors: Lipworth Wendy , Ambler Geoffrey , Burt Morton G. , Fairchild Jan , Inder Warrick J. , Werther George , Ho Ken
Published: August 2018

Growth hormone (GH) replacement therapy was recently recommended by the Pharmaceutical Benefits Advisory Committee (PBAC) for listing on the Pharmaceutical Benefits Scheme for adults with severe GH deficiency and impaired quality of life. This approval was significant for two reasons. First, the application was initiated and coordinated by a health professional working group, who prepared a ‘public interest’ submission to PBAC. Second, it resulted in a recommendation to subsidise therapy for a rare disease after two prior rejections on the basis of uncertainty about efficacy and cost effectiveness. There are important lessons to learn about the power of professional groups to drive health policy and attain funding for rare diseases.
Medial medullary stroke due to neurosyphilis in a newly diagnosed HIV‐positive man
Authors: Katelaris Anthea L. , Janson Sonja , Ramachandran Prashanth , Spencer Emma
Published: August 2018

This case report presents the clinical record of a 37‐year‐old man who presented with a dense right hemiplegia, found to be caused by a left medial medullary stroke. The cause of the stroke was unclear, and bacterial endocarditis was initially suspected. However, he was ultimately found to have neurosyphilis on a background of undiagnosed human immunodeficiency virus and was treated with benzylpenicillin. This case report reviews the diagnosis of neurosyphilis and highlights the importance of considering neurosyphilis as a rare but important cause of stroke, especially given the increasing prevalence of syphilis in Australia.
Rescue haploidentical peripheral blood stem cell transplantation for engraftment failure: a single‐centre case series
Authors: Kliman David , Bilmon Ian , Kwan John , Blyth Emily , Micklethwaite Ken , Panicker Shyam , Bhattacharyya Abir , Deren Stephanie , Antonenas Vicki , Huang Gillian , Gottlieb David
Published: August 2018

Graft failure affects approximately 5% of allogeneic stem cell transplants, with a poor prognosis. Salvage second allogeneic stem cell transplantation (alloSCT2) is limited by high rates of transplant‐related mortality from infection and graft‐versus‐host disease. We report on five adult patients receiving rescue alloSCT2 using haploidentical peripheral blood stem cells. All patients achieved neutrophil engraftment, two subsequently died from sepsis and disease relapse, respectively. Three patients remain alive up to 2 years post‐transplant. We suggest consideration of haploidentical alloSCT2 for patients with graft failure.
Ghettos, banlieues – is the difference disappearing?
Authors: Rey Henri
Published: August 2018

This article considers the use of references to the American ghettos in the history of the French working‐class suburbs ‐ the “banlieues”. When construction work first began on the new housing developments, concerns were voiced about the scale and isolation of their apartment blocks, in contrast to the model of individual houses. With the pauperisation of a section of the working classes and growth of the immigrant population, successive periods of rioting and the prevalence of an economy of drug dealing and other illicit trades among an element of the youth in these neighbourhoods, over a quarter of whom are unemployed, the comparison between ghettos and banlieues gained credibility. Also influential was public policy targeting the French banlieues, which reflected the dogma of republican equality, and fierce opposition to communalism. However, a section of the inhabitants of these neighbourhoods have gradually developed a subjective feeling of being separate from the rest of society and imprisoned in a ghetto with its own codes and laws, for example in the treatment of women. So the clear distinction established 20 years ago between the “black belt” and the 𠄌red belt” (Wacquant 2006) has greatly reduced.
What enhances the right to information? A cross‐national examination of legal, technological, and administrative influences
Authors: Nam Taewoo
Published: August 2018

This study examines the influence of legal arrangements (freedom of information legislation), technological advancements (e‐government), and administrative capacity (government effectiveness) on the right to information with a cross‐country view. To that end, it uses various global indicators. A regression‐based analysis reveals that legal guarantees and e‐government do not significantly influence the extent to which the right to information is realised. Freedom of information legislation can offer legal guarantees for the right to information; however, the outcomes in terms of the right to information have not yet been reached for many new adopters of the legislation. While the contribution of e‐government to the right to information is also insignificant, a government's administrative capacity is pivotal to achieving the right to information.
An Investigation into Uncovering and Understanding Tacit Knowledge in a First‐Year Design Studio Environment
Authors: van Kampen Saskia
Published: August 2018

Tacit knowledge, intuitive responses, empirical research – how can design elevate itself as a discipline when its main forms of knowledge gathering and generation are still believed to be less valuable than those in traditional academia? Beginning design students must learn to put words to their actions, to understand their processes and to be critical and reflective throughout all stages of a project. By guiding students into developing rigorous iterative processes that combine theoretical research, empirical research, questioning and reflection students will no longer need to hide behind the lure of the enigma of intuition. The introduction of writing in the studio classroom is one method that can help students see the relationship between the various stages of their creative process and articulate the reasons behind the decisions they make. Experiential learning techniques that combine writing, making, research and reflection will help students shift their personal frames of reference and thus create a more critically engaged student body.
Using Thematic Analysis to Facilitate Meaning‐Making in Practice‐Led Art and Design Research
Authors: Lin Fabia Ling‐Yuan
Published: August 2018

This article argues that thematic analysis could aid artist‐researchers in enhancing the arguments they construct in their fields of interest as they move through research projects. It discusses some of the distinctive features and obstacles of practice‐led research in art and design and relates them to the use of thematic analysis. Artistic research often includes acts of interpretation, where the artist‐researcher reflects on his or her artistic work process and the context of the artefacts created during the process. A systematic thematic approach is important for the whole process, because it supports the separate work periods of artmaking and conceptualising and binds them to the study. In its theoretical freedom, thematic analysis provides a flexible and useful research tool that can bridge the two realms of knowing in practice‐led research, potentially providing a rich and synthesised account of the creative experience. The benefits of thematic analysis will be demonstrated by examining two examples.
Disclosure of Product System Models in Life Cycle Assessment: Achieving Transparency and Privacy
Authors: Kuczenski Brandon
Published: August 2018

Many of the challenges facing knowledge synthesis from life cycle assessment (LCA) studies stem from the inability of study authors and readers to formally agree on the structure and content of the product system models used to perform LCA computations. This article presents a framework for formally disclosing the foreground of an LCA study in a way that permits the computations to be inspected, verified, and reproduced by a reader, provided that the reader has access to the same life cycle inventory and impact characterization resources as the author. The framework can also be used to partition a study into public and private portions, allowing both portions to be critically reviewed but omitting the private information from the disclosure. A disclosure is made up of six components, including three lists of entities in the model and three sparse matrices describing their interconnections. The entity lists make reference to previously‐published resources, including background inventory databases and characterized elementary flows, and the disclosure framework requires both author and reader to agree on the meaning of each of these references. The framework contributes to ongoing efforts within and beyond industrial ecology to improve the reproducibility and verifiability of scholarly works, and if implemented, plots a course toward distributed, platform‐independent computation and validation of LCA results.
Group Prenatal Visits: Maternal and Neonatal Health Outcomes
Authors: Groskaufmanis Lauren , Brunner Huber Larissa R. , Vick Tara
Published: August 2018

Introduction

Most studies evaluating the effect of group prenatal care on maternal and neonatal health outcomes assess the CenteringPregnancy model, which follows a set structure and educational curriculum. Group prenatal visits (GPVs) bring together pregnant patients for visits that include education and a health evaluation. GPVs represent a more flexible method of delivering group prenatal care, compared with CenteringPregnancy. Our study sought to determine whether GPV participation affects maternal and neonatal health outcomes.

Methods

The Myers Park Obstetrics and Gynecology Clinic is located in Charlotte, North Carolina, and serves a racially, ethnically, and socioeconomically diverse population. GPVs were offered at the clinic between July 2014 and July 2015. Retrospective data were collected for women who obtained prenatal care, either GPV or individual care, during that period. Demographic, birth, and postpartum data were extracted from the electronic health record. GPV participants were categorized by the percent of prenatal visits that were GPVs (limited GPV: <30% of visits as GPV; moderate GPV: ≥30% of visits as GPV). Logistic regression models were created to assess the effect of GPV participation on low birth weight, preterm birth, cesarean birth, and postpartum visit attendance.

Results

There were 355 study participants (GPV n = 78, individual care n = 277). Among GPV participants, 52.6% were classified as limited GPV, and 47.4% were classified as moderate GPV. The adjusted analysis showed limited‐GPV patients had lower odds of postpartum visit attendance, compared with individual‐care patients (odds ratio, 0.48; 95% CI, 0.24‐0.94). Neither the unadjusted nor adjusted models demonstrated a statistically significant association between GPV participation and low birth weight, preterm birth, or cesarean birth.

Discussion

GPVs for prenatal care can be implemented without negative effects on maternal or neonatal health. However, fidelity to a more comprehensive model of group prenatal care may be necessary to achieve health outcome improvements.
Development and validation of microsatellite markers for Karnal bunt (Tilletia indica) and loose smut (Ustilago segetum tritici) of wheat from related fungal species
Authors: Sharma Pradeep , Shefali  , Pandey Bharati , Muthusamy Senthilkumar K. , Kumar Satish , Saharan Mahender Singh , Kumar Sudheer , Singroha Garima , Sharma Indu , Singh Gyanendra Pratap
Published: August 2018

Simple sequence repeats (SSRs) are preferred molecular markers because of their abundance, robustness, high reproducibility, high efficiency in detecting variation and suitability for high‐throughput analysis. In this study, an attempt was made to mine and analyse the SSRs from the genomes of two seed‐borne fungal pathogens, viz Ustilago maydis, which causes common smut of maize, and Tilletia horrida, the cause of rice kernel smut. After elimination of redundant sequences, 2,703 SSR loci of U. maydis were identified. Of the remaining SSRS, 44.5% accounted for di‐nucleotide repeats followed by 29.8% and 2.7% tri‐ and tetranucleotide repeats, respectively. Similarly, 2,638 SSR loci were identified in T. horrida, of which 20.2% were di‐nucleotide, 50.4% tri‐ and 20.5% tetra‐nucleotide repeats. A set of 65 SSRs designed from each fungus were validated, which yielded 23 polymorphic SSRs from Ustilago and 21 from Tilletia. These polymorphic SSR loci were also successfully cross‐amplified with the Ustilago segetum tritici and Tilletia indica. Principal coordinate analysis of SSR data clustered isolates according to their respective species. These newly developed and validated microsatellite markers may have immediate applications for detection of genetic variability and in population studies of bunt and smut of wheat and other related host plants. Moreover, this is first comprehensive report on molecular markers suitable for variability studies in wheat seed‐borne pathogens.
Noncolloquial Arabic in Tunisian Children With Autism Spectrum Disorder: A Possible Instance of Language Acquisition in a Noninteractive Context
Authors: Kissine Mikhail , Luffin Xavier , Aiad Fethia , Bourourou Rym , Deliens Gaétane , Gaddour Naoufel
Published: August 2018

We have documented the significant presence of spontaneous and productive use of Modern Standard Arabic (MSA) in the speech of five Tunisian boys with autism, an unusual phenomenon. In typical development, MSA is not fully acquired before the late school years. The Arabic language in Tunisia is in a state of diglossia, and (unlike the colloquial Tunisian Arabic variety) MSA is virtually never used in everyday conversation. Television programs broadcast across the Arabic‐speaking world constitute the most important source of MSA for preschool children. Typically developing children require active social interaction to develop language, but some children with autism may use television and cartoons as noninteractional input to acquire language. This study highlighted the existence of a noninteractional language‐learning strategy that may partly compensate for the sociopragmatic deficits that characterize autism.