bluuebunny/crossref_metadata_2025_split huggingface dataset:
Molecular Analysis of Cystic Fibrosis Patients in Hungary – An Update to the Mutational Spectrum/Molekularna Analiza Obolelih Od Cistične Fibroze U Mađarskoj – Dopune Spektru Mutacija
Authors: Ivády Gergely , Koczok Katalin , Madar Laszlo , Gombos Eva , Toth Izabella , Gyori Klaudia , Balogh István 
Published: August 2014

Summary
Background: In this study the authors present an update to the CFTR mutation profile in Hungary, utilizing data from a selected cohort of 45 cystic fibrosis (CF) patients from different regions of the country.
Methods: Depending on the preceding analysis, four different mutation detection methods were used. A commercial assay targeting the most common CF-causing mutations was performed as the first test followed by an allele specific PCR for CFTRdele2,3(21kb), Sanger sequencing and MLPA analysis of the coding region of the CFTR gene.
Results: In our recent study 27 different mutations were detected, including 2 novel ones (c.1037_1038insA and c.1394C>T). Besides F508del (c.1521_1523delCTT), the following mutations were found at a frequency of ≥ 4.0%: W1282X (c.3846G>A), N1303K (c.3909C>G), CFTRdele2,3(21kb) (c.54-5940_273+10250del21kb) and 2184insA (c.2052_2053insA). In addition, four mutations (G542X, Y1092X, 621+1G>T, and 2143delT) were found in more than one allele.
Conclusions: The updated database of Hungarian mutations not only enables to increase the efficiency of the existing diagnostic approach, but also provides a further refined basis for the introduction of the molecular newborn screening (NBS) program in Hungary.
The Delimitation of Urban Hinterlands Based on Transport Flows: A Case Study of Regional Capitals in The Czech Republic
Authors: Kraft Stanislav , Halás Marián , Vančura Michal 
Published: August 2014

Abstract
The delimitation of the urban hinterlands of the most important settlement centres in the Czech Republic, based on transportation flows, is presented in this paper. Transport flows are a very good indicator of complex spatial relations. Therefore, one can hypothesize that the delimited transport hinterlands are strongly associated with other types of urban hinterlands (e.g. commuting hinterlands). Transport regions of the Czech regional capitals are defined in the empirical section of this paper. These transport regions, supra-nodal territorial units of the Czech transport system, identify the main transport relations within the Czech road network and define the hinterlands of the main settlement centres. The metropolitan regions defined by transport relations are compared with regions of the Czech Republic based on commuting flows. There is a high concordance between the two regional delimitations.
Small-Scale Renewable Energy Systems in the Development of Distributed Generation in Poland
Authors: Chodkowska-Miszczuk Justyna 
Published: August 2014

Abstract
Small-scale renewable energy systems in the context of the development of distributed generation, are discussed for the case of Poland. A distributed energy system is efficient, reliable and environmentally friendly, and is one of the most recent trends in the development of the energy sector in Poland. One of the important dimensions of this process is the creation of micro- and small-power producers based on renewable, locally-available energy sources. It is clear that the development of small-scale renewable energy producers takes place in two ways. One of these is through small hydropower plants, which are the aftermath of hydropower development in areas traditionally associated with water use for energy purposes (northern and western Poland). The second is through other renewable energy sources, mainly biogas and solar energy and located primarily in southern Poland, in highly urbanized areas (e.g. Śląskie Voivodship). In conclusion, the development of small-scale renewable energy systems in Poland is regarded as a good option with respect to sustainable development.
A Curse of Coal? Exploring Unintended Regional Consequences of Coal Energy in The Czech Republic
Authors: Frantál Bohumil , Nováková Eva 
Published: August 2014

Abstract
Focusing on coal energy from a geographical perspective, the unintended regional consequences of coal mining and combustion in the Czech Republic are discussed and analysed in terms of the environmental injustice and resource curse theories. The explorative case study attempts to identify significant associations between the spatially uneven distribution of coal power plants and the environmental and socioeconomic characteristics and development trends of affected areas. The findings indicate that the coal industries have contributed to slightly above average incomes and pensions, and have provided households with some technical services such as district heating. However, these positive effects have come at high environmental and health costs paid by the local populations. Above average rates of unemployment, homelessness and crime indicate that the benefits have been unevenly distributed economically. A higher proportion of uneducated people and ethnic minorities in affected districts suggest that coal energy is environmentally unjust.
Petrological implications of the Early Mesozoic lamprophyre dikes and related Tarkhata syenites (SE Altai and NW Mongolia)
Authors: Vasyukova Elena A. , Borisenko Alexander S. 
Published: August 2014

Abstract
This paper presents new data derived from field sampling and from a thorough description of lamprophyres located in southeastern Altai and northwestern Mongolia in terms of their mineralogy, textures, and chemical composition. The swarms of alkaline mafic dikes in the area coexist with granosyenite-monzodiorite and gabbro-dolerite intrusions and spatially coincide with an ore district of Sb-Hg, Ag-Sb, Ni-Co-As, Cu-Mo-W, and CaF2 hydrothermal mineralization. All lamprophyres belong to the Early Mesozoic Chuya complex formed in an intracontinental enviroment. Their distribution and orientation is controlled by two large fault zones. The Chuya dikes were investigated at two localities, namely, Yustyd and South-Chuya. The Yustyd lamprophyres intrude Middle-Upper Devonian black shale of the Yustyd depression. At South Chuya, lamprophyres, together with the Tarkhata granosyenite-monzodiorite complex, are hosted by Cambrian and Ordovician metamorphic rocks of the South-Chuya Range. Ar-Ar (phlogopite) and U-Pb (SHRIMP, zircon) ages of the lamprophyre dikes indicate long and continuous period of the formation of the Chuya complex (250-235 Ma). Major- and trace-element compositions of the lamprophyres from both localities and of the syenite indicate their origin from the same magma source. The textures and structures of the lamprophyre and plutonic rocks, their mineral assemblages and the chemistry of the rock-forming minerals provide clues to the evolution of the parental alkaline mafic magma and fluid regime.
Megacrysts of kyanite from Baranec Mt., Western Tatra Mountains, Slovakia
Authors: Pyka Paulina , Szopa Krzysztof , Gawęda Aleksandra 
Published: August 2014

Abstract
Large crystals of kyanite (<15 cm in size) occur in quartz segregations in Paleozoic gneissses on Baranec Mt., Western Tatra Mountains, northern Slovakia. Blue kyanite crystals coexist with quartz and plagioclase. The kyanite contains inclusions of apatite, monazite. gamet, rutile and biotite and overgrowths of retrograde sillimanite. muscovite and biotite. The kyanite crystals are the largest found up to now in the Tatra crystalline massif or in the other Western Carpathians crystalline cores. Kyanite. with the co-existing mineral assemblage, is indicative of a HP stage duiing Hercynian metamorphism of the Western Tatra Mountains.
Digital Signal Processing Applied to the Modernization Of Polish Navy Sonars
Authors: Marszal Jacek 
Published: August 2014

Abstract
The article presents the equipment and digital signal processing methods used for modernizing the Polish Navy’s sonars. With the rapid advancement of electronic technologies and digital signal processing methods, electronic systems, including sonars, become obsolete very quickly. In the late 1990s a team of researchers of the Department of Marine Electronics Systems, Faculty of Electronics, Telecommunications and Informatics, Gdansk University of Technology, began work on modernizing existing sonar systems for the Polish Navy. As part of the effort, a methodology of sonar modernization was implemented involving a complete replacement of existing electronic components with newly designed ones by using bespoke systems and methods of digital signal processing. Large and expensive systems of ultrasound transducers and their dipping and stabilisation systems underwent necessary repairs but were otherwise left unchanged. As a result, between 2001 and 2014 the Gdansk University of Technology helped to modernize 30 sonars of different types.
A Multi-gene Analysis Reveals Multiple Highly Divergent Lineages of The Isopod Chelator Insignis (Hansen, 1916) South of Iceland
Authors: Brix Saskia , Svavarsson Jörundur , Leese Florian 
Published: August 2014

AbstractThe eurybathic isopod speciesChelator insignisshows a wide distribution south of Iceland. We analysed 51 specimens from shelf (213–305 m depth), slope (885–891 m and 1380–1390 m depth) and deep-sea habitats (2750 m) south of Iceland with different DNA markers. A fragment of the mitochondrial cytochrome c oxidase subunit I gene (COI) was studied for 47 specimens, 16S was studied for 36 specimens, and a fragment for the 18S rRNA gene could be amplified for 11 specimens. For the COI data, specimens clustered into five distinct lineages each separated by ≥20% uncorrected pairwise distances. Both the mitochondrial 16S and the nuclear 18S sequence data further support this deep divergence, suggesting the presence of overlooked species inside the nominalC. insignis. Populations on the shelf occurring east and west of the Reykjanes Ridge were genetically identical suggesting that this ridge is not a barrier to gene flow. However, populations from different depth ranges differed substantially. Our multi-gene analysis suggests that the newly found species likely have more narrow vertical distribution ranges and highlights a possible role of bathymetry in speciation processes.
Discovery of Swimming Males of Paratanaoidea (Tanaidacea)
Authors: Błażewicz-Paszkowycz Magdalena , Jennings Robert M. , Jeskulke Karen , Brix Saskia 
Published: August 2014

AbstractIn Tanaidacea morphological identification of male individuals to the species level is complicated by two factors: the presence of multiple male stages/instars confuse the assessment of sexual stage while strong sexual dimorphism within several families obscures the morphological affinities of undescribed males to described females. Males of Paratanaoidea are often morphologically quite different from females and have not been discovered for most genera so far, which has led to the assumption that some tanaidaceans might have parthenogenetic reproduction or simply have undeveloped secondary sex traits. As a part ofthe IceAGE project (Icelandic marine Animals: Genetics and Ecology), with the support of molecular methods, the first evidence for the existence of highly dimorphic (swimming) males in four families of the superfamily Paratanaoidea (Agathotanaidae, Cryptocopidae, Akanthophoreidae, and Typhlotanaidae) is presented. This study suggests that these males might be the next instars after juvenile or preparatory males, which are morphologically similar to females. It has been assumed that “juvenile” males with a restricted ability for swimming (e.g., undeveloped pleopods) have matured testes, are capable of reproduction, and mate with females nearby, while swimming males can mate with distant females. Our explanation of the dimorphism in Tanaidomorpha lies in the fact that males of some species (e.g.,Nototanais) retain the same lifestyle or niche as the females, so secondary traits improve their ability to guard females and successfully mate. Males of other species that have moved into a regime (niche) different than that of the female have acquired complex morphological changes (e.g.,Typhlotanais).
Comparison of 3-Dimensional Shoulder Complex Kinematics in Individuals With and Without Shoulder Pain, Part 1: Sternoclavicular, Acromioclavicular, and Scapulothoracic Joints
Authors: Lawrence Rebekah L. , Braman Jonathan P. , LaPrade Robert F. , Ludewig Paula M. 
Published: August 2014

Study Design Cross-sectional. Objectives To compare sternoclavicular, acromioclavicular, and scapulothoracic joint motion between symptomatic and asymptomatic individuals during shoulder motion performed in 3 planes of humerothoracic elevation. Background Differences in scapulothoracic kinematics are associated with shoulder pain. Several studies have measured these differences using surface sensors, but the results of this technique may be affected by skin-motion artifact. Furthermore, previous studies have not included the simultaneous measurement of sternoclavicular and acromioclavicular joint motion. Methods Transcortical bone pins were inserted into the clavicle, scapula, and humerus of 12 asymptomatic and 10 symptomatic individuals for direct, bone-fixed tracking using electromagnetic sensors. Angular positions for the sternoclavicular, acromioclavicular, and scapulothoracic joints were measured during shoulder flexion, abduction, and scapular plane abduction. Results Differences between groups were found for sternoclavicular and scapulothoracic joint positions. Symptomatic individuals consistently demonstrated less sternoclavicular posterior rotation, regardless of angle, phase, or plane of shoulder motion. Symptomatic individuals also demonstrated less scapulothoracic upward rotation at 30° and 60° of humerothoracic elevation during shoulder abduction and scapular plane abduction. Conclusion The results of this study show that differences in shoulder complex kinematics exist between symptomatic and asymptomatic individuals. However, the magnitude of these differences was small, and the resulting clinical implications are not yet fully understood. The biomechanical coupling of the sternoclavicular and acromioclavicular joints requires further research to better understand scapulothoracic movement deviations and to improve manual therapy and exercise-based physical therapy interventions. J Orthop Sports Phys Ther 2014;44(9):636–645. Epub 7 August 2014. doi:10.2519/jospt.2014.5339
Drug utilization study in orthopaedic units: Antibiotics prescribed in hospital out-patients in Dhaka, Bangladesh
Authors: Bithi Seheli Sejuti , Khan Md. Minhazur Rahman , Khan Ahsan Ullah 
Published: August 2014

Antibiotics are generally prescribed for both as prophylactically (before orthopaedics surgery etc.) or to treat ongoing infection (like septic arthritis, osteomyelitis etc.) in the orthopaedics department. But if antibiotics are not use rationally then there will be increase chances of resistance of bacteria as also as deteriorate the patients disease condition which ultimately increase the total cost of treatment. The goal of this study was to see the antibiotics utilization pattern. A prospective cross-sectional, multicentre drug utilization study was conducted for a period of 3 months to evaluate the pattern of antibiotics use in orthopaedic unit of various hospitals of Dhaka City, Bangladesh. A total of 498 prescriptions were studied; in which 160 prescriptions contained mono-antibiotic therapy (51.79%) and 147 prescriptions contained poly-antibiotics therapy (48.21%) and the beta-lactam antibiotics were most commonly prescribed (81.68%). Mono-antibiotics prescriptions were the common pattern than combination and brand name has been prescribed frequently than generic. To minimize cost, it is advisable to promote prescription of generic drugs. Also justifying efforts are needed to improve appropriateness of antimicrobial therapy and minimize the development of antimicrobial resistance. Hence the results of the study showed that there is a considerable scope for improvement in the prescription pattern.DOI: http://dx.doi.org/10.3329/icpj.v3i9.19896 International Current Pharmaceutical Journal, August 2014, 3(9): 318-321
Open badges for education: what are the implications at the intersection of open systems and badging?
Authors: Ahn June , Pellicone Anthony , Butler Brian S. 
Published: August 2014

Badges have garnered great interest among scholars of digital media and learning. In addition, widespread initiatives such as Mozilla’s Open Badge Framework expand the potential of badging into the realm of open education. In this paper, we explicate the concept of open badges. We highlight some of the ways that researchers have examined badges as part of educational practice and also highlight the different definitions of open-ness that are employed in popular and scholarly thought. By considering badges from three different perspectives (motivation, pedagogy, and credential) and the concept of openness from three different perspectives (production, access and appropriation) we develop a framework to consider the tensions where these competing conceptions meet. This explication illuminates how the ideas of open and badges intersect, and clarifies situations where these concepts come into direct conflict or mutually enhance each other. Our analysis pinpoints and elucidates particular areas where research is needed to better understand the complex phenomenon of open badges, and also offers design considerations for developers, educators, and organizations that are actively involved in open badges.Keywords: open education; gamification; learning; credentials; badges; education reform(Published: 8 August 2014)Citation: Research in Learning Technology 2014, 22: 23563 -http://dx.doi.org/10.3402/rlt.v22.23563
Cost-Effectiveness of Boceprevir Co-Administration versus Pegylated Interferon-α2b and Ribavirin Only for Patients with Hepatitis C Genotype 1 in Singapore
Authors: Dan Yock Young , Ferrante Shannon A , Elbasha Elamin H , Hsu Tun-Ying 
Published: August 2014

Background Patients infected with chronic HCV genotype 1 experience liver complications as the disease progresses. This study aims to project the long-term reduction of liver complications and cost-effectiveness of treatment strategies, including co-administrating boceprevir (BOC) with pegylated interferon-α2b (PEG-IFN) and ribavirin compared with standard of care (SOC) of PEG-IFN and ribavirin only. Methods A Markov model was created to estimate the expected costs and quality-adjusted life-years (QALYs) associated with treatment strategies outlined in the BOC package insert in Singapore. Patient characteristics were from pivotal trials, the transition probabilities and QALYs were estimated from publications, and the pharmaceutical and health status costs were obtained from a public hospital in Singapore. The threshold of cost-effectiveness was chosen as 65,000 SGD for this study. Results For treatment-naive patients, BOC is highly cost-effective compared with SOC (179 SGD/QALY) and cost-saving for patients who have failed prior treatment, due to higher QALYs from better sustained virological response (SVR) and lower costs from avoidance of complications. Sub-group analyses show that BOC is cost-effective for non-cirrhotic treatment-experienced patients and null responders. It out-performs SOC for treatment-naive non-cirrhotic and cirrhotic patients who have failed prior treatment. Even after adjusting for higher prevalence of favourable IL28B genotype in Asians, BOC is cost-effective compared with SOC. Only untreated cirrhotic patients showed inconclusive cost-effectiveness for BOC. Conclusions Compared with SOC, BOC prevents more HCV liver complications from HCV genotype 1, particularly in patients who have failed previous SOC. Improved SVR and shortened duration of treatment result in BOC being potentially cost-saving or cost-effective in an Asian population.
Safety, Pharmacokinetics and Pharmacodynamics of the Oral Toll-Like Receptor 7 Agonist GS-9620 in Treatment-Naive Patients with Chronic Hepatitis C
Authors: Lawitz Eric , Gruener Daniel , Marbury Thomas , Hill John , Webster Lynn , Hassman David , Nguyen Anh-Hoa , Pflanz Stefan , Mogalian Erik , Gaggar Anuj , Massetto Benedetta , Subramanian G Mani , McHutchison John G , Jacobson Ira M , Freilich Bradley , Rodriguez-Torres Maribel 
Published: August 2014

Background GS-9620 is a potent oral agonist of toll-like receptor 7, a key modulator of the innate immune response. In healthy volunteers, low doses of GS-9620 (2, 4 and 6 mg) induced significant expression of peripheral interferon-stimulated-gene (ISG) mRNA in the absence of detectable serum interferon-α and systemic adverse events (AEs). We evaluated the safety, pharmacokinetics and pharmacodynamics of GS-9620 in treatment-naive patients chronically infected with HCV genotype 1. Methods In this double-blind, placebo-controlled study, 51 patients were randomized 5:1 (active:placebo) to receive either a single dose or two once-weekly doses of GS-9620 at four dose levels (0.3, 1, 2 and 4 mg) or placebo. Pharmacodynamic assessments included peripheral ISG15 mRNA expression, serum interferon-α and interferon-γ-inducible protein (IP)-10 levels and HCV RNA quantification. Results GS-9620 was well-tolerated at all doses. Most AEs were mild or moderate in severity. GS-9620 exhibited dose-linear pharmacokinetics with a median half-life in plasma of 18 h. Transient, dose-dependent ISG15 induction was observed at 1, 2 and 4 mg, with peak mean fold change within 48 h followed by a decline to baseline levels within 7 days of dosing. Serum interferon-α induction post-baseline was detected in 16.7% (8/48) of patients. No clinically significant reductions in HCV RNA were observed. Conclusions GS-9620 was safe, well-tolerated and biologically active in patients with HCV infection. Induction of ISG15 occurred in the absence of detectable serum interferon-α or systemic AEs in most patients, supporting a pre-systemic mechanism of action. ClinicalTrials.gov identifier: NCT01591668.
AS PROFUNDEZAS VAMPIROTÊUTICAS DO PÓS-HUMANISMO DE VILÉM FLUSSER
Authors: Da Silva Junior José Geraldo 
Published: August 2014

Resumo A partir de um minucioso trabalho de pesquisa da obra do pensador tcheco Vilém Flusser que viveu por mais de 30 anos no Brasil e do pós-humanismo, tema ainda pouco estudado pela academia brasileira, os pesquisadores Erick Felinto e Lúcia Santaella sugerem a leitura da obra do autor do clássico "Filosofia da Caixa Preta – Ensaios para uma futura filosofia da caixa preta" em convergência com as questões do pós-humanismo e da teoria da mídia alemã. Palavras-chave: Pós-humanismo, Teoria da mídia alemã, Vilém Flusser. Abstract The brazilians researchers Erick Felinto and Lucia Santaella after a detailed research on post-humanism, subject still little studied by the brazilian academia, and the complex work of philosopher Vilém Flusser, suggest a reading of his work on convergence with the issues of post-humanism and German Media Theory. Keywords: Posthumanism; German Media Theory; Vilém Flusser.