bluuebunny/crossref_metadata_2025_split huggingface dataset:
Severe course with lethal hepatocellular injury and skeletal muscular dysgenesis in a neonate with infantile liver failure syndrome type 1 caused by novel LARS1 mutations
Authors: Hirata Katsuya , Okamoto Nobuhiko , Ichikawa Chihiro , Inoue Shouta , Nozaki Masatoshi , Banno Kimihiko , Takenouchi Toshiki , Suzuki Hisato , Kosaki Kenjiro 
Published: December 2020

AbstractInfantile liver failure syndrome type 1 (ILFS1) is a recently recognized autosomal recessive disorder caused by deleterious mutations in the leucyl‐tRNA synthetase 1 gene (LARS1). The LARS1 enzyme is responsible for incorporation of the amino acid leucine during protein polypeptide synthesis. Individuals with LARS1 mutations typically show liver failure from infancy to early childhood during periods of illness or other physiological stress. While 25 patients from 15 families with ILFS1 have been reported in the literature, histological reports from autopsy findings are limited. We report here a premature male neonate who presented with severe intrauterine growth retardation, microcytic anemia, and fulminant liver failure, and who was a compound heterozygote for two novel deleterious mutations in LARS1. An autopsy showed fulminant hepatitis‐like hepatocellular injury and fibrogenesis in the liver and a lack of uniformity in skeletal muscle, accompanied by the disruption of striated muscle fibers. Striking dysgenesis in skeletal muscle detected in the present case indicates the effect of LARS1 functional deficiency on the musculature. Whole‐exome sequencing may be useful for neonates with unexplained early liver failure if extensive genetic and metabolic testing is inconclusive.
Violence in the first millenniumBCEEurasian steppe: Cranial trauma in three Turpan Basin populations from Xinjiang, China
Authors: Zhang Wenxin , Zhang Qun , McSweeney Kathleen , Han Tao , Man Xingyu , Yang Shiyu , Wang Long , Zhu Hong , Zhang Quanchao , Wang Qian 
Published: December 2020

AbstractObjectivesViolence affected daily life in prehistoric societies, especially at conflict zones where different peoples fought over resources and for other reasons. In this study, cranial trauma was analyzed to discuss the pattern of violence experienced by three Bronze to early Iron Age populations (1,000–100 BCE) that belonged to the Subeixi culture. These populations lived in the Turpan Basin, a conflict zone in the middle of the Eurasian Steppe.MethodsThe injuries on 129 complete crania unearthed from the Subeixi cemeteries were examined for crude prevalence rate (CPR), trauma type, time of occurrence, possible weapon, and direction of the blow. Thirty‐three injuries identified from poorly preserved crania were also included in the analyses except for the CPR. Data was also compared between the samples and with four other populations that had violence‐related backgrounds.ResultsOverall, 16.3% (21/129) of the individuals showed violence‐induced traumatic lesions. Results also indicated that most of the injuries were perimortem (81.6%), and that women and children were more involved in conflict than the other comparative populations. Wounds from weapons accounted for 42.1% of the identified cranial injuries. Distribution analysis suggested no dominant handedness of the attackers, and that blows came from all directions including the top (17.1%). Wounds caused by arrowheads and a special type of battle‐ax popular in middle and eastern Eurasian Steppe were also recognized.DiscussionA comprehensive analysis of the skeletal evidence, historical records, and archeological background would suggest that the raiding to be the most possible conflict pattern reflected by the samples. The attackers were likely to have been nomadic invaders from the steppe (such as the Xiongnu from historical records), who attacked the residents in the basin more likely for their resources rather than territory or labor force.
Fabrication and characterization of high flux poly(vinylidene fluoride) electrospun nanofibrous membrane using amphiphilic polyethylene‐block‐poly(ethylene glycol) copolymer
Authors: Gandhimathi Chinnasamy , Sundarrajan Subramanian , Matsuura Takeshi , Srinivasan Dinesh Kumar , Wei He , Xuecheng Dong , Ramakrishna Seeram 
Published: December 2020

AbstractElectrospun nanofiber membranes (ENM) made of polymers such as polysulfone and poly(vinylidene fluoride) (PVDF) have a much higher contact angle (CA) and also more hydrophobic when compared to the virgin polymers. For water treatment applications, membranes with hydrophilic nature are highly desirable in order to achieve high flux and less fouling potentials. Hence, in the present study, highly hydrophilic electrospun nanofiber membranes (ENMs) were prepared by blending PVDF polymer with amphiphilic polyethylene‐block‐poly (ethylene glycol) (PE‐b‐PEG) copolymer. Resulting amphiphilic ENMs were highly porous (77%–92%) and the breaking elongation of 140% with a young's modulus of 2.55 MPa was observed. When compared with the control PVDF membrane, PE‐b‐PEG blended ENMs revealed higher water permeation flux owing to the enrichment of the hydrophilic PEG segments at the membrane surface, which was confirmed by using X‐ray photoelectric spectroscopy and Energy‐dispersive spectroscopy measurements. When compared to the phase inversion process (CA of 97.3°) blended ENM had CA of 0°, which indicates that besides hydrophilic block copolymer segments, the nature of membrane formation also contributes its role in influencing the hydrophilicity of the membrane. This improved hydrophilicity in combination with larger pore sizes of the PVDF/ PE‐b‐PEG membranes have contributed to enhancement of pure water flux, protein solution permeability and water flux recovery, which can be applied potentially for water treatment applications.
Electromechanical deformation and failure of multilayered films
Authors: Zhang Ci , Zhu Lei , Olah Andrew , Baer Eric 
Published: December 2020

AbstractLayer thickness was found to have a significant effect on the irreversible electromechanical deformation and the failure mechanism in polycarbonate (PC)/poly (vinylidene fluoride) (PVDF) multilayered films when subjected to an electrical impulse in a DC needle‐plane configuration. Three distinct regions of behavior were observed. Region I comprised thick layer systems that exhibited only irreversible center deformation. The improvement to failure resistance compared to the monolithic films was attributed to the interphase between the two components. Region II films with an intermediate layer thickness showed both an irreversible center deformation and a treeing mechanism which were observed to simultaneously occur. The surface treeing mechanism, similar to the lightning treeing phenomena in nature, occurs only at impact rates. The tree morphology showed large amounts of plowing, indicating that this damage mechanism can dissipate a large amount of energy prior to electromechanical fracture of the film. Region III films comprise ultrathin layers in the nanoscale and showed no treeing. The unique interphase region between these ultrathin layers was estimated to be at least ten percent of the overall layered structure. These films behaved similar to monolithic materials with improved electromechanical failure characteristics. This work complements the enhanced dielectric performance of multilayer films observed in earlier investigations.
Comparison of Lesional Juvenile Myositis and Lupus Skin Reveals Overlapping Yet Unique Disease Pathophysiology
Authors: Turnier Jessica L. , Pachman Lauren M. , Lowe Lori , Tsoi Lam C. , Elhaj Sultan , Menon Rajasree , Amoruso Maria C. , Morgan Gabrielle A. , Gudjonsson Johann E. , Berthier Celine C. , Kahlenberg J. Michelle 
Published: December 2020

ObjectiveSkin inflammation heralds systemic disease in juvenile myositis, yet we lack an understanding of pathogenic mechanisms driving skin inflammation in this disease. We undertook this study to define cutaneous gene expression signatures in juvenile myositis and identify key genes and pathways that differentiate skin disease in juvenile myositis from childhood‐onset systemic lupus erythematosus (SLE).MethodsWe used formalin‐fixed paraffin‐embedded skin biopsy samples from 15 patients with juvenile myositis (9 lesional, 6 nonlesional), 5 patients with childhood‐onset SLE, and 8 controls to perform transcriptomic analysis and identify significantly differentially expressed genes (DEGs; q ≤ 5%) between patient groups. We used Ingenuity Pathway Analysis (IPA) to highlight enriched biologic pathways and validated DEGs by immunohistochemistry and quantitative real‐time polymerase chain reaction.ResultsComparison of lesional juvenile myositis to control samples revealed 221 DEGs, with the majority of up‐regulated genes representing interferon (IFN)–stimulated genes. CXCL10, CXCL9, and IFI44L represented the top 3 DEGs (fold change 23.2, 13.3, and 13.0, respectively; q < 0.0001). IPA revealed IFN signaling as the top canonical pathway. When compared to childhood‐onset SLE, lesional juvenile myositis skin shared a similar gene expression pattern, with only 28 unique DEGs, including FBLN2, CHKA, and SLURP1. Notably, patients with juvenile myositis who were positive for nuclear matrix protein 2 (NXP‐2) autoantibodies exhibited the strongest IFN signature and also demonstrated the most extensive Mx‐1 immunostaining, both in keratinocytes and perivascular regions.ConclusionLesional juvenile myositis skin demonstrates a striking IFN signature similar to that previously reported in juvenile myositis muscle and peripheral blood. Further investigation into the association of a higher IFN score with NXP‐2 autoantibodies may provide insight into disease endotypes and pathogenesis.
Cationic Nonporous Macrocyclic Organic Compounds for Multimedia Iodine Capture
Authors: Xu Xiao‐Qian , Cao Li‐Hui , Yang Yan , Bai Xiang‐Tian , Zhao Fang , He Zhen‐Hong , Yin Zheng , Ma Yang‐Min 
Published: December 2020

AbstractOver the past two decades, progress in chemistry has generated various types of porous materials for removing iodine (129I or 131I) that can be formed during nuclear energy generation or nuclear waste storage. However, most studies for iodine capture are based on the weak host‐guest interactions of the porous materials. Here, we present two cationic nonporous macrocyclic organic compounds, namely, MOC‐1 and MOC‐2, in which 6I‐ and 8I− were as counter anions, for highly efficient iodine capture. MOC‐1 and MOC‐2 were formed by reacting 1,1′‐diamino‐4,4′‐bipyridylium di‐iodide with 1,2‐diformylbenzene or 1,3‐diformylbenzene, respectively. The presence of a large number of I− anions results in high I2 affinity with uptake capacities up to 2.15 g ⋅ g−1 for MOC‐1 and 2.25 g ⋅ g−1 for MOC‐2.
Baroque Science, Experimental Art? Jusepe de Ribera and other Neapolitan Sceptics
Authors: Sapir Itay 
Published: December 2020

AbstractCurrent attempts by historians of science to revise the narrative of the Scientific Revolution by using the concept of the Baroque have important implications for art history. Correspondences between baroque art and baroque science gain new complexity when the rational, epistemologically optimistic image of the New Science is put in doubt. Rather than a method of objective observation, early seventeenth‐century science and art share an acceptance of the constructed nature of reality, of human epistemological limitations and of the role of passions in the observation of the world. While Caravaggio has revolutionised art precisely through his interest in questions of knowledge and sensorial perception and by his subversive transformation of Renaissance epistemological values and ideals, this article concentrates on the work of Jusepe de Ribera, who made the senses and their shortcomings a major theme of his pictorial research. Ribera's epistemology is examined in the context of contemporary Neapolitan philosophy and science. Through the confrontation of some of the Spagnoletto's paintings with the work of figures such as Giovanni Battista della Porta, Federico Cesi and particularly Colantonio Stigliola, it becomes clear that early modern Neapolitan faith in rational knowledge was more ambiguous than is sometimes assumed.
Proposal of a laboratory course dedicated to the generation of protein molecular weight standards for sodium dodecyl sulfate‐polyacrylamide gel electrophoresis
Authors: Bubis José 
Published: December 2020

AbstractProtein molecular weight standards are routinely employed in sodium dodecyl sulfate‐polyacrylamide gel electrophoresis (SDS‐PAGE) to estimate the apparent sizes of unknown proteins within a sample. For training students, a laboratory course based on the production of marker proteins is proposed. Following fractionation by column chromatography, a series of purified proteins and mixtures of proteins were combined to generate various sets of unstained, prestained and fluorescently labeled molecular weight ladders for SDS‐PAGE. The produced material can be used in successive laboratory practices as an alternative to commercial protein markers.
Economic assessment of continuous processing for manufacturing of biotherapeutics
Authors: Gupta Paridhi , Kateja Nikhil , Mishra Somesh , Kaur Harmeet , Rathore Anurag S. 
Published: December 2020

AbstractContinuous processing offers a promising approach to revolutionize biotherapeutics manufacturing as reflected in recent years. The current study offers a comparative economic assessment of batch and continuous processing for the production of biotherapeutic products. Granulocyte‐colony stimulating factor (GCSF), a protein expressed in E. coli, and an IgG1 monoclonal antibody, were chosen as representatives of microbial and mammalian derived products for this assessment. Economic indicators—cost of goods (COGs), net present value (NPV), and payback time have been estimated for the assessment. For the case of GCSF, conversion from batch to integrated continuous manufacturing induced a $COGs/g reduction of 83% and 73% at clinical and commercial scales, respectively. For the case of mAb therapeutic, a 68% and 35% reduction in $COGs/g on translation from batch to continuous process was projected for clinical and commercial scales, respectively. Upstream mAb titer was also found to have a significant impact on the process economics. With increasing mAb titer, the $COG/g decreases in both operating modes. With titer increasing from 2 to 8 g/L, the $COG/g of batch process was reduced by 53%, and that of the continuous process was reduced by 63%. Cost savings in both the cases were attributed to increased productivity, efficient equipment and facility utilization, smaller facility footprint, and reduction in utilization of consumables like resin media and buffers actualized by the continuous processing platform. The current study quantifies the economic benefits associated with continuous processing and highlights its potential in reducing the manufacturing cost of biotherapeutics.
Prediction of adolescent pelvis development using femoral head and acetabulum growth in a longitudinal radiographic study
Authors: Keller Nicole B. , Liu Raymond W. 
Published: December 2020

AbstractIntroductionAdolescent total hip arthroplasty (THA) is guarded partially due unclarity as to when pediatric hip joints reach full development. This study sought to identify when maximum acetabular and femoral head diameters are achieved with relation to chronological and skeletal age.Materials and MethodsConsecutive femoral head and acetabular diameters were measured in a random sample of 54 female and 78 male subjects, which were queried from a historical collection of annual radiographs of children. Femoral head and triradiate physeal plates were scored according to Oxford bone parameters.ResultsAt 14 years females had femoral diameters of 4.16 +/− 0.23 cm and acetabular diameters of 5.15 +/− 0.30 cm. At 16 years males had femoral diameters of 4.85 +/− 0.30 cm and acetabular diameters of 5.90 +/− 0.35 cm. In the year following maximal femoral Oxford scores, no significant change was seen in femur and acetabulum diameters in females and in femur diameters in males. In the year following maximal acetabular Oxford scores, there was a significant increase in both femur and acetabulum diameters in both females and males.ConclusionsFemales on average reach maximum pelvis maturity at 14 years and males reach maximum pelvis maturity at 16 years. A closed femoral head was found to be a good marker of full hip growth, while a closed triradiate was not. This study provides anatomical data for surgeons to consider in assessing risk factors of THA failure in adolescents.
Percutaneous axillary access: A call to arms
Authors: Seto Arnold H. , Baran David A. 
Published: December 2020

Key Points
The axillary artery is a useful alternative access site for large‐bore interventions as it is rarely atherosclerotic.
This systematic review of percutaneous large‐bore axillary artery access suggests that it is as safe and effective as open surgical access.
The Society of Cardiovascular Angiography and Interventions (SCAI) Expert Consensus Statement will soon be convened to provide best practice recommendations on transaxillary access including technique, training, and safety.
Prenatal characterization of a novel inverted SMAD2 duplication by mate pair sequencing in a fetus with dextrocardia
Authors: Zepeda‐Mendoza Cinthya J. , Essendrup Anna , Smoley Stephanie A. , Johnson Sarah H. , Hoppman Nicole L , Vasmatzis George , Jackson Daniel L. , Kearney Hutton M. , Baughn Linda B. 
Published: December 2020

AbstractThis case report underlines the importance of molecular characterization of genomic duplications and other structural variants in the prenatal setting to guide clinical interpretation, genetic counseling, and perinatal medical care.
A power optimization model for the long‐term planning scenarios: Case study of Mexico's power system decarbonization
Authors: Martínez‐Quintana Marco Antonio , Martín‐del‐Campo Cecilia , Cruz‐Mendoza Guadalupe 
Published: December 2020

AbstractMexico is committed to reducing its CO2 emissions according to the Paris Agreement. A relevant effort must be made for the analysis of Mexico's electric energy system towards a progressive decarbonization with a larger participation of intermittent renewable energies. The analysis of power planning scenarios, with different assumptions on costs, emissions, and intermittent performance of the power generating technologies, is needed to make sustainable decisions in the transition toward a cleaner power sector. Tools for energy modelling are required to develop and analyze scenarios with minimum costs subject to environmental constraints. The purpose of the article is to explain the modelling approach of a novel and flexible power planning tool, which is based on a well‐known linear programming optimization method combined with a computing strategy to optimize time consumed for reading, processing calculations, and writing the huge number of economic and technical parameters required for the hourly power dispatch in complex interconnected electric systems. The time consumed has been optimized by means of a binary matrix that activates the input and use of only the data needed for the solution of the problem. The paper describes the MC model and demonstrates some of its analytical capabilities through a Mexican case study with a least cost scenario and two decarbonization scenarios of the power interconnected system for the period from 2020‐2050.
History of nitroxide mediated polymerization in Canada
Authors: Marić Milan 
Published: December 2020

AbstractNitroxide mediated polymerization (NMP), sometimes known as stable free radical polymerization (SFRP), is considered one of the main types of reversible deactivation radical polymerization (RDRP) that have emerged as a major advance in polymer synthesis in the past 30 years. This review examines NMP's development from a uniquely Canadian perspective. Inspired by reports of a reversible equilibrium between the TEMPO persistent radical and a growing polymeric radical chain, researchers at the Xerox Research Centre of Canada (XRCC) reported radical polymerizations of styrene with hallmarks typically associated with living polymerizations: linear degree of polymerization of with conversion, narrow molecular weight distributions, and ability to re‐initiate the chain end with additional monomer. This effort expanded to polymerizations in dispersed aqueous media (eg, miniemulsion) and to polymerizations directed for various architectures (block, graft, star), but NMP was ultimately generally limited to styrenic monomers. Consequently, NMP trailed other RDRP methods, such as atom transfer radical polymerization (ATRP) and reversible addition fragmentation transfer polymerization (RAFT), due to its limited monomer choice. With the advent of second‐generation alkoxyamine initiators, however, the range of monomers polymerizable in a controlled manner greatly expanded to include acrylates, acrylamides, and eventually methacrylates (under certain conditions), providing renewed impetus towards using NMP. Consequently, Canadian researchers applied these new alkoxyamines for more versatile polymers, resulting in products such as stimuli‐responsive polymers, CO2‐switchable latexes, bio‐hybrid composites, organic photovoltaic materials, and photolithographic materials. The chronological progression of the developments in NMP from various Canadian laboratories highlights these achievements in new polymeric materials.
CO2 reforming of methane over the growth of hierarchical Ni nanosheets/Al2O3‐MgO synthesized via the ammonia vapour diffusion impregnation
Authors: Dharmasaroja Nichthima , Ratana Tanakorn , Tungkamani Sabaithip , Sornchamni Thana , Simakov David S. A. , Phongaksorn Monrudee 
Published: December 2020

AbstractA novel ammonia vapour diffusion‐assisted impregnation technique was developed to synthesize the Al2O3‐MgO‐supported hierarchical Ni nanosheets. The resulting catalysts with different times for ammonia vapour treatments (at 12, 18, and 20 hours) were prepared to investigate the growth of Ni nanosheets on the catalyst surface. All catalysts were tested for CO2 reforming of methane and a comprehensive characterization study was conducted by XRD, N2 adsorption‐desorption, H2‐TPD, H2‐TPR, CO2‐TPD, and TGA. The Ni nanosheets were obtained using the ammonia vapour treatment for 20 hours, improving the selectivity toward H2 generation without a lower CH4 conversion. When compared to the reference catalyst prepared by a conventional impregnation method, the H2/CO ratio for CO2 reforming of the methane process was enhanced by 0.35. Additionally, the carbon deposition was reduced by half using hierarchical Ni nanosheets for the CO2 reforming of methane at 620°C for 20 hours. The mechanism of this improvement was achieved by the increase in medium basicity associated with a strong metal‐support interaction that promotes the CO2 activation‐dissociation pathways, preventing carbon formation and inhibiting the reverse water gas shift.