bluuebunny/crossref_metadata_2025_split huggingface dataset:
Long‐term survival outcomes of patients with Niemann‐Pick disease type C receiving miglustat treatment: A large retrospective observational study
Authors: Patterson Marc C. , Garver William S. , Giugliani Robert , Imrie Jackie , Jahnova Helena , Meaney F John , Nadjar Yann , Vanier Marie T. , Moneuse Patrick , Morand Olivier , Rosenberg Daniel , Schwierin Barbara , Héron Benedicte 
Published: April 2020

AbstractMiglustat has been indicated for the treatment of Niemann‐Pick disease type C (NP‐C) since 2009. The aim of this observational study was to assess the effect of miglustat on long‐term survival of patients with NP‐C. Data for 789 patients from five large national cohorts and from the NPC Registry were collected and combined. Miglustat‐treated and untreated patients overall and within sub‐groups according to age‐at‐neurological‐onset, that is, early infantile‐onset (<2 years), late infantile‐onset (2 to <6 years), juvenile‐onset (6 to <15 years), and adolescent/adult‐onset (≥15 years) were analysed and compared. Survival was analysed from the time of first neurological manifestation (Neurological onset group, comprising 669 patients) and from diagnosis (Diagnosis group, comprising 590 patients) using a Cox proportional hazard model adjusted for various covariates. Overall, 384 (57.4%) patients in the Neurological onset group and 329 (55.8%) in the Diagnosis group were treated with miglustat. Miglustat treatment was associated with a significant reduction in risk of mortality in both groups (entire Neurological onset group, Hazard ratio [HR] = 0.51; entire Diagnosis group, HR = 0.44; both P < .001). The effect was observed consistently in all age‐at‐neurological‐onset sub‐groups (HRs = 0.3 to 0.7) and was statistically significant for late infantile‐onset patients in both groups (Neurological onset group, HR = 0.36, P < .05; Diagnosis group, HR = 0.32, P < .01), and juvenile‐onset patients in the Diagnosis group only (HR = 0.30, P < .05). Despite the limitations of the data that urge cautious interpretation, the findings are consistent with a beneficial effect of miglustat on survival in patients with NP‐C.
Development of a methodology to study typicity of PDO wines with professionals of the wine sector
Authors: Leriche Coline , Molinier Clara , Caillé Soline , Razungles Alain , Symoneaux Ronan , Coulon‐Leroy Cécile 
Published: April 2020

AbstractBACKGROUNDToday, many agricultural products claiming a link to their origin and typicity receive a warm welcome on the market. Nevertheless, the notion of typicity is blurred for consumers and needs to be objectified for communication purposes. This study aims at formalizing a methodology for studying typicity of terroirs, with PDO wines as an example, using a participatory approach with professionals of the wine sector from terroirs, using focus group and tasting. The vision of typicity of terroirs by professionals outside of these terroirs has been studied by a free word association task.RESULTSThis study allowed a clear distinguishing of the typicity of the studied terroirs. Professionals from terroirs identified the global conceptual typicity of their terroirs using various factors, mainly soil, geography and grape variety, whereas professionals outside terroirs expressed their visions of terroirs by reputation or sensory characteristics of wines. Tasting results showed a discrimination of wines based on their typicity and highlighted descriptors involved in sensory perceptual typicity for each studied terroir.CONCLUSIONSProfessionals from terroirs share a common vision of their typicity and identify more typicity factors than professionals outside terroirs. Sensory typicity has been highlighted for five of the six terroirs studied, according to the various descriptors. The study of two populations, from and outside terroirs, shows the gap between the typicities identified by professionals from terroirs and those perceived by professionals outside terroirs. © 2020 Society of Chemical Industry
Effect of different biopolymer‐based structured systems on the survival of probiotic strains during storage and in vitro digestion
Authors: Melchior Sofia , Marino Marilena , Innocente Nadia , Calligaris Sonia , Nicoli Maria Cristina 
Published: April 2020

AbstractBACKGROUNDThis study aimed to evaluate the protective effect of different biopolymer systems on the viability of two probiotics (Lactobacillus rhamnosus and Streptococcus thermophilus) during storage and in vitro digestion. Methylcellulose (MC), sodium alginate (SA), and whey protein (WP)‐based structures were designed and characterized in terms of pH, rheological properties, and visual appearance.RESULTSThe results highlighted that the WP‐system ensured probiotic protection during both storage and in vitro digestion. This result was attributed to a combined effect of the physical barrier offered by the protein gel network and whey proteins as a nutrient for microbes. On the other hand, surprisingly, the viscous methylcellulose‐based system was able to guarantee good microbial viability during storage. However, this was not confirmed during in vitro digestion. The opposite results were obtained for sodium alginate beads.CONCLUSIONThe results suggest that the capacity of a polymeric structure to protect probiotic bacteria is a combination of structural organization and system formulation. © 2020 Society of Chemical Industry
Fetal Anomaly Detection in Pregnancies With Pregestational Diabetes
Authors: Martin Robert B. , Duryea Elaine L. , Mcintire Donald D. , Twickler Diane M. , Dashe Jodi S. 
Published: April 2020

ObjectivesTo evaluate fetal anomaly detection in pregnancies with pregestational diabetes, according to the gestational age at the time of specialized sonography, use of follow‐up sonography, maternal body mass index, and organ system(s) involved.MethodsWomen with pregestational diabetes who received prenatal care and delivered a live‐born or stillborn neonate at our hospital from October 2011 through April 2017 were ascertained. We included all pregnancies with at least 1 confirmed structural anomaly (EUROCAT classification) who had detailed sonography at 18 weeks’ gestation or later. We analyzed detection of anomalous fetuses at the initial detailed sonogram and, if no abnormality was identified, during any follow‐up sonograms. Statistical analyses were performed with the χ2 test and Mantel‐Haenszel χ2 test for trend.ResultsSeventy‐two anomalous neonates (72 of 1060 [6.8%]) were born. Overall detection was 55 of 72 (76%); 49 of 72 (68%) were detected at the initial detailed sonogram, compared to 6 of 15 (40%) of follow‐up examinations (P = .04). Recognition at the initial or follow‐up examination was not dependent on gestational age or body mass index category (all P > .05). Of individual organ system anomalies, 67 of 89 (75%) were identified. Detection exceeded 85% for central nervous system, genitourinary, and musculoskeletal abnormalities and 43% for craniofacial anomalies. Sixty‐five percent of cardiac anomalies were detected, and 14 of 17 (82%) requiring specialized care in the immediate neonatal period were recognized.ConclusionsApproximately three‐fourths of anomalous fetuses were identified, with greater detection at the initial detailed examination. Fetuses with central nervous system, genitourinary, musculoskeletal abnormalities and those with cardiac anomalies requiring specialized cardiac care were more likely to come to attention.
Comparison of short‐period daytime convective rainfall accumulations with total column precipitable water: Derivation of an operational forecasting technique
Authors: Young Martin , Lamb Daniel , Lane Andrew , Lattimore Chris 
Published: April 2020

AbstractA major forecasting challenge is predicting the likely intensity of rainfall in convective situations in which intense short‐period rainfall can lead to surface water flooding. The study demonstrates a useful correlation between precipitable water and 1 hr gauge‐derived rainfall accumulations in situations with deep instability and relatively slack flow in depth. The correlation forms the basis of a forecasting technique, independent of numerical weather prediction (NWP) model forecasts of rainfall, being applied at the UK's Met Office Flood Forecasting Centre (FFC).
PEG‐PLA‐PCL based electrospun yarns with curcumin control release property as suture
Authors: Sharifisamani Elahe , Mousazadegan Fatemeh , Bagherzadeh Roohollah , Latifi Masoud 
Published: April 2020

AbstractThis work presents an interesting method using an electrospinning process to fabricate suture yarns loaded with curcumin to achieve reasonable mechanical properties as well as tunable drug release behavior. Different structures including different yarn counts and twists as well as core‐sheath structures were used to adjust drug release properties along with improving the yarn's mechanical properties. The core parts were made of polycaprolactone and the sheath parts were made of polyethylene glycol, polylactic acid, and polycaprolactone. Drugs can be incorporated in both parts based on the required condition and application. Electrospun yarns were compared using both structural properties and their drug release profiles as metrics. The results of comparing drug release profiles of six electrospun yarns with different yarn counts and twists showed that yarns with finer fiber diameters in the core part have more drug release as well as more initial release. Overall evaluations showed that core‐sheath drugloaded yarn with appropriate physical and mechanical properties can be a useful material as a drug delivery system to the site of damaged tissue. It can also be concluded that the amount and duration of drug release can be controlled using the structural parameters of electrospun yarns as an engineering tool for designing suture yarns with required properties.
How to convert green supply chain integration efforts into green innovation: A perspective of knowledge‐based view
Authors: Kong Ting , Feng Taiwen , Huang Yufei , Cai Junya 
Published: April 2020

AbstractAlthough the importance of green supply chain integration (GSCI) in facilitating green innovation has been recognized, most existing literature constructs a direct link for the GSCI‐green innovation relationship. How firms convert their GSCI efforts into green innovation is still unclear. By collapsing GSCI into three dimensions (i.e., green supplier integration, green internal integration, and green customer integration), this study explores an indirect GSCI‐green innovation link in which knowledge exchange and knowledge combination are deemed as mediators. We employ survey data from 237 Chinese manufacturers and structural equation modeling techniques to test research hypotheses. Our findings support the major argument that the impact of GSCI on green innovation is indirect. Specifically, green internal integration impacts green product and process innovation via both knowledge exchange and knowledge combination; while green supplier integration affects green product and process innovation indirectly only via knowledge combination and green customers integration affects green product and process innovation indirectly only via knowledge exchange. This study advances our understanding on the GSCI‐green innovation relationship.
Bi2O3@mSiO2 as an Environmentally Benign and Sustainable Solid Acid Catalyst for Benzoylation of Aromatics: Impact of Silica Encapsulation on Catalyst Leaching and Reaction Synergy
Authors: Jain Prateek U. , Samant Shriniwas D. 
Published: April 2020

AbstractLeaching of active species is one of the major causes of catalyst deactivation. The accumulation of this leached species as chemical waste can be detrimental to the environment. In this study, a series of environmentally benign core‐shell catalysts of type Bi2O3@mSiO2 were synthesized to address these issues. The catalysts, varying in silica content and shell thickness, were characterized and evaluated for Friedel‐Crafts benzoylation. Amongst them, Bi‐4 (containing 71.8 % Bi2O3), having shell thickness of ∼42 nm, showed the best response under optimized conditions. Textural analysis of Bi‐4 confirmed the mesoporous nature of shell, with average pore diameter of ∼40 Å and surface area of 62 m2/g. During the reaction, the Bi2O3 core was converted to BiOCl, which was easily recycled back to its original form. No leaching or any loss in catalytic activity was observed for the recycled catalyst during reuse. Thus, an inexpensive, reusable and environmentally benign catalyst is proposed.
An Approximate Method to Simulate Post-Seismic Deformations in a Realistic Earth Model
Authors: Tang He , Dong Jie , Sun Wenke 
Published: April 2020

AbstractThe geodetic observations of static deformations, including gravity perturbations and displacement fields due to huge earthquakes, are understood and explained using recent dislocation theories. Due to multiple possible mechanisms for the post-seismic phase of earthquakes, the dominant mechanism may change at different spatiotemporal ranges for different earthquake types. Accurate forward and inverse modeling of post-seismic deformations is valuable and needed information for geoscience communities. The existing methods for calculating gravitational viscoelastic relaxation can be improved or simplified to make them more suitable for more realistic Earth models and/or to overcome the poor convergence performance and/or overflow risks during numerical calculations. In this study, a simple and effective method for calculating the post-seismic relaxation deformations is proposed. This method is different from previous methods, such as the normal mode summation and rectangle integration methods. The proposed method consists of a rational functional approximation of the integral kernel and a transformation of the numerical inverse Laplace transform (NILT) into an alternating series summation using the residual theorem. Then the intrinsic oscillation and overflow risks are thoroughly suppressed. The accuracy of the calculated Green’s functions can be easily controlled by choosing a suitable parameter. In addition, the proposed method also has applicability in different Earth models with linear rheological profiles.
Curriculum and Teacher Education Reforms in Finland That Support the Development of Competences for the Twenty-First Century
Authors: Lavonen Jari 
Published: April 2020

AbstractThis chapter analyzes how learning twenty-first century competences has been implemented in the Finnish educational context through the enactment of national and local level curricula and the design of a teacher education development program in a decentralized education system, in which teachers, schools, municipalities, and universities have high autonomy. The curricula and development program emphasize learning twenty-first century competences. Both were designed in collaboration with Finnish teachers and teacher educators, representatives from the Ministry of Education and Culture, the Association of Finnish Local and Regional Authorities, the Teacher’s Union, the Student’s Unions, and the Principal Association. The major actions taken to implement these changes included piloting, seminars and conferences, having different support and local level collaborations, and networking. According to recent evaluations, both endeavors – the development of national and local level curricula and a teacher education development program – have resulted in progress towards implementing twenty-first century competences in schools and for teacher education.
Curriculum and Educational Reforms in Portugal: An Analysis on Why and How Students’ Knowledge and Skills Improved
Authors: Crato Nuno 
Published: April 2020

AbstractBy the turn of the century, following the dismal first results in TIMSS and PISA, the Portuguese educational system was at a crossroads. It was clear that students were not attaining minimal levels of proficiency in reading, math, science, and other basic subjects. The system needed a deep reshaping, and so changes were made. By the time the last PISA and TIMSS international large-scale surveys’ results were released in 2015, Portugal registered a quantum leap: in PISA, student achievement was above the OECD average and in TIMSS, 4th graders had higher scores in Mathematics than several usually high-performing countries, including Finland. How was this possible? To understand what happened, we need to look at what Portugal has done in the last 10–15 years. Although many different ministers from different ideological standpoints made different reforms, there is a common thread to most changes: they paid increased attention to results. This proved to be a powerful thrust for improvement, backed up by experienced teachers. However, this general thrust assumed many concrete different aspects and promoted different reforms. During the 2011–2015 period, these reforms went further and were very clear, intentional, and explicit: a clear curriculum, increased school autonomy, students’ regular assessment, vocational paths, flexibility. All this helped to prepare youngsters for an active, productive, and responsible life in the twenty-first century.
What Does It Mean?
Authors: Willison John 
Published: April 2020

AbstractThis chapter is titled ‘What does it mean?’ and provokes a consideration of seminal and recent learning theories and what they mean for contemporary educational practices including Machine Teaching and teacher action research in light of the MELT. Placing learning theories on the MELTcontinuum of learning autonomyis very practical for teacher understanding of theories and for application to classrooms and online learning.
β-1,3-Glucanase production as an anti-fungal enzyme by phylogenetically different strains of the genus Clostridium isolated from anoxic soil that underwent biological disinfestation
Authors: Ueki Atsuko , Takehara Toshiaki , Ishioka Gen , Kaku Nobuo , Ueki Katsuji 
Published: April 2020

AbstractBiological (or reductive) soil disinfestation (BSD or RSD) is a bioremediation process to control soil-borne plant pathogens using activities of indigenous bacteria in the soil. Three obligate anaerobic bacterial strains (TW1, TW10, and TB10), which were isolated from anoxic soil subjected to BSD treatments, were examined for their abilities to produce anti-fungal enzymes. All strains were affiliated with the different lineages of the genus Clostridium. The three strains decomposed β-1,3-glucans (curdlan and laminarin), and β-1,3-glucanase activities were detected from their culture supernatants with these glucans. The three strains also produced the enzyme with wheat bran as a growth substrate and killed the Fusarium pathogen (Fusarium oxysporum f. sp. spinaciae) in the anaerobic co-incubation conditions. Observation by fluorescence microscopy of the pathogen cells showed that the three strains had degraded the fungal cells in different manners upon co-incubation with wheat bran. When the three strains were cultivated with the dead cells or the cell wall samples prepared from the Fusarium pathogen, strain TW1 utilized these materials as easily decomposable substrates by releasing β-1,3-glucanase. When observed by fluorescence microscopy, it appeared that strain TW1 degraded the mycelial cell wall nearly thoroughly, with the septa remaining as undecomposed luminous rings. In contrast, the other two strains decomposed neither the dead cells nor the cell wall samples directly. The results indicate that the various anaerobic bacteria proliferated in the soil under the BSD treatments should play key roles as an organized bacterial community to eliminate fungal pathogens, namely by release of anti-fungal enzymes with different properties.

Key points•Three clostridial strains isolated from BSD-treated soils produced β-1,3-glucanase.•All strains killed the Fusarium pathogen in the anaerobic co-incubation conditions.•One of the strains produced β-1,3-glucanase with the fungal cell wall as a substrate.•The strain degraded the cell wall almost completely, except for the mycelial septa.
ENSO’s impacts on the tropical Indian and Atlantic Oceans via tropical atmospheric processes: observations versus CMIP5 simulations
Authors: He Shan , Yu Jin-Yi , Yang Song , Fang Shih-Wei 
Published: April 2020

AbstractThis study compares the impacts of the El Niño–Southern Oscillation (ENSO) on sea surface temperatures (SSTs) in the tropical North Atlantic Ocean and the tropical Indian Ocean during 1958–2004. It is found that the tropical atmospheric processes mediating the ENSO impacts are different between the two oceans for two reasons. First, the ENSO-induced anomalous Walker circulation is more extensive over the Atlantic than over the Indian Ocean. As a result, the atmospheric bridge (AB) mechanism is the major contributor to the differences in ENSO teleconnections between the two oceans. Secondly, SSTs in the tropical North Atlantic are under a greater control of the atmospheric thermal forcing than those in the tropical Indian Ocean. Due to these different controls, the tropospheric temperature (TT) mechanism also contributes to the different ENSO teleconnections. When compared with the observations, the mean of thirty-seven models from the Coupled Model Intercomparison Project Phase 5 overestimates the ENSO-induced SST response in the tropical Indian Ocean but underestimates the response in the tropical North Atlantic. The overestimation is brought about by a westward extension of ENSO SST anomalies in the models, which causes the AB mechanism to produce an overly strong impact on Indian Ocean SSTs. On the other hand, the underestimation is caused by a weaker-than-observed sensitivity in the simulated Atlantic SSTs to the thermal forcing produced by the TT mechanism.
Establishment and functional characterization of a murine primary Sertoli cell line deficient of connexin43
Authors: Gerber Jonathan , Rode Kristina , Hambruch Nina , Langeheine Marion , Schnepel Nadine , Brehm Ralph 
Published: April 2020

AbstractThe Sertoli cell (SC) specific connexin43 (Cx43) knockout (SCCx43KO) mouse line is ideal to gain insight into the mechanistic gap junction formation in SC and the seminiferous epithelium. A method for developing primary SC cultures from these mice was established, validated and successfully characterized via polymerase chain reaction, immunohistochemistry, immunofluorescence (IF), and Western blots (WB). It was evident that both knockout (KO) and wild-type (WT) primary cell cultures were similar in morphology. These highly pure SC cultures were subjected to cell proliferation assays indicating no notable proliferation in cultures of both genotypes. Measurements of cell monolayer integrity indicated significant increases in transepithelial electrical resistance and consequently in tight junction expression of the KO cultures. Using semi-quantitative WB and IF, tight junction protein claudin-11 was analyzed. These results support a role for Cx43 in regulating blood-testis barrier (BTB) function, composition, and dynamics in vitro. Thus, the SC deficient Cx43 cell cultures may provide a valuable in vitro tool for a better understanding of the mechanistic role of Cx43 in spermatogenesis and BTB assembly.