bluuebunny/crossref_metadata_2025_split huggingface dataset:
Time to diagnosis of Duchenne muscular dystrophy remains unchanged: Findings from the Muscular Dystrophy Surveillance, Tracking, and Research Network, 2000‐2015
Authors: Thomas Shiny , Conway Kristin M. , Fapo Olushola , Street Natalie , Mathews Katherine D. , Mann Joshua R. , Romitti Paul A. , Soim Aida , Westfield Christina , Fox Deborah J. , Ciafaloni Emma ,   for the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet)
Published: March 2022

AbstractIntroduction/AimsWith current and anticipated disease‐modifying treatments, including gene therapy, an early diagnosis for Duchenne muscular dystrophy (DMD) is crucial to assure maximum benefit. In 2009, a study from the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet) showed an average diagnosis age of 5 years among males with DMD born from January 1, 1982 to December 31, 2000. Initiatives were implemented by the US Centers for Disease Control and Prevention (CDC) and patient organizations to reduce time to diagnosis. We conducted a follow‐up study in a surveillance cohort born after January 1, 2000 to determine whether there has been an improvement in time to diagnosis.MethodsWe assessed the age of diagnosis among males with DMD born from January 1, 2000 to December 31, 2015 using data collected by six US MD STARnet surveillance sites (Colorado, Iowa, western New York State, the Piedmont region of North Carolina, South Carolina, and Utah). The analytic cohort included 221 males with definite or probable DMD diagnosis without a documented family history. We computed frequency count and percentage for categorical variables, and mean, median, and standard deviation (SD) for continuous variables.ResultsThe mean [median] ages in years of diagnostic milestones were: first signs, 2.7 [2.0]; first creatine kinase (CK), 4.6 [4.6]; DNA/muscle biopsy testing, 4.9 [4.8]; and time from first signs to diagnostic confirmation, 2.2 [1.4].DiscussionThe time interval between first signs of DMD and diagnosis remains unchanged at 2.2 years. This results in lost opportunities for timely genetic counseling, implementation of standards of care, initiation of glucocorticoids, and participation in clinical trials.
Creatine kinase‐MM concentration in dried blood spots from newborns and implications for newborn screening for Duchenne muscular dystrophy
Authors: Park Sunju , Maloney Breanne , Caggana Michele , Tavakoli Norma P. 
Published: March 2022

AbstractIntroduction/AimsCreatine kinase‐MM (CK‐MM) is a marker of skeletal muscle damage. Detection of elevated levels of CK‐MM in newborns can enable an early suspicion of the diagnosis of Duchenne muscular dystrophy (DMD) before symptom onset. Our aim was to investigate CK‐MM levels in DMD‐affected and unaffected newborns using an immunoassay that measures CK‐MM concentration in dried blood spots collected for routine newborn screening.MethodsTo validate the assay in our laboratory, CK‐MM measurements and newborn demographic information were collected for 8584 de‐identified specimens and 15 confirmed DMD patients. After analyzing validation data, CK‐MM normal ranges were determined based on age of newborn at specimen collection. Subsequently, the assay was used to measure CK‐MM concentration in 26 135 newborns as part of a consented pilot study to screen for DMD in New York State. Mean and median levels of CK‐MM based on age of collection, in addition to the 2.5th, 50th, 97.5th, and 99.5th percentiles, were recalculated using the validation and screening data sets.ResultsMedian CK‐MM within 1 hour of birth was 109 ng/mL, rose to a high of 499 ng/mL at 25 hours of age, and then declined to 200 ng/mL at 2 days of life. The median continued to decline more slowly and then stabilized at approximately 40 ng/mL at 1 week of life.DiscussionBecause of the marked variability and elevated CK‐MM levels observed within the first days of life, it is important to set multiple CK‐MM age‐related cut‐offs when screening for DMD in newborns.
Improving the thermal properties of olive/bamboo fiber‐based epoxy hybrid composites
Authors: Rashid Bushra , Jawaid Mohammad , Fouad Hassan , Saba Naheed , Awad Sameer , Khalaf Eman , Sain Mohini 
Published: March 2022

AbstractIn this work, thermal analysis of olive/bamboo fiber‐based epoxy hybrid composites was carried out. Three types of olive fibers, which are olive tree small branch (OTS), olive tree big branch (OTB), and olive tree leaves (OTL), along with bamboo fibers (B), were used to fabricate the composites. Thermal properties of hybrid composites were examined by the thermogravimetric analyzer (TGA), dynamic mechanical analyzer (DMA), and thermomechanical analyzer (TMA). It was found that the thermal stability improved with the incorporation of hybrid fibers in epoxy composites compared to pure fiber composites. Hybrid composite (OTS‐B) exhibited a lower residue (15.82%) whereas hybrid composites (OTB‐B and OTS‐B) show 54.65% and 54.53% weight loss at the maximum decomposition temperature. DMA results showed that the storage modulus and loss modulus reduced with hybrid fiber composites while the damping factor (tan delta) was increased. The storage modulus values of the pure composite sample (B) exhibited a higher increased (3150 MPa). In contrast, the pure composite sample (B) exhibited the highest loss modulus (337 MPa). From TMA analysis, OTL‐B hybrid composite presented a higher Tg and lower coefficient of thermal expansion. We concluded that finding from this work will strengthen attracting interpretation of utilization of two different fibers to fabricate hybrid composites for various lightweight purposes in a wide‐ranging choice of industrial applications such as biomedical tools, automobile, and construction fields. Additionally, a novel method can be used to develop hybrid biocomposites materials, which have potential applications in biomaterials and engineering areas.
Design and demonstration of a low‐field magnetic resonance imaging rhizotron for in‐field imaging of energy sorghum roots
Authors: Bagnall G. Cody , Altobelli Stephen A. , Conradi Mark S. , Fabich Hilary T. , Fukushima Eiichi , Koonjoo Neha , Kuethe Dean O. , Rooney William L. , Stupic Karl F. , Sveinsson Bragi , Weers Brock , Rajan Nithya , Rosen Matthew S. , Morgan Cristine L. S. 
Published: March 2022

AbstractRoot phenotyping provides critical information to plant breeders for developing varieties with improved drought tolerance, greater root biomass, and greater nutrient use efficiency. Phenotyping roots in the natural environment is important for understanding the effect of the soil environment on root genotypic expressions. The goal of this work was to design and test a field‐scale mobile low‐field magnetic resonance imaging (LF‐MRI) Rhizotron that produces actionable root phenotyping data. We demonstrated this novel technology for root visualization and quantification using a LF‐MRI Rhizotron operating at 47 mT with two soil types. The LF‐MRI Rhizotron weights 453 kg, with a height of 90 cm, a diameter of 28 cm and an imaging field of view of 28 cm × 28 cm. The unit was operated in a Belk clay (Entic Hapluderts) and Weswood silt loam (Udifluventic Halustepts) generating 2‐D and 3‐D image data sets. The 2‐D image data had a collection time of 16.5 min per image at an image resolution of 2.2 mm per pixel. The 3‐D data had a collection time of 13 h per image with a 2.2 × 2.2 × 2.2 mm voxel resolution. Low‐field magnetic resonance imaging worked well for visualizing roots in moderate to high clay soils, demonstrating the potential for this technology; however, the broad application of this platform is hampered due to the prohibitively long scanning time to obtain 3‐D images. By increasing the field strength, and therefore the signal‐to‐noise ratio, faster scan times can enable a more useful system for root phenotyping.
Respiratory management of spinal muscular atrophy type 1 patients treated with Nusinersen
Authors: Menard Joris , Seferian Andreea M , Fleurence Emmanuelle , Barzic Audrey , Binoche Alexandra , Labouret Géraldine , Coutier Laurianne , Vuillerot Carole , Bieleu Blaise M , Gomez Garcia de la Banda Marta , Corvol Harriet , Servais Laurent , Taytard Jessica 
Published: March 2022

AbstractIntroductionThe recent development of disease‐modifying treatments in spinal muscular atrophy (SMA) type 1 shifted these patients' management from palliative to proactive. The aim of this study was to assess patients' nocturnal gas exchanges before noninvasive ventilation (NIV) initiation and their clinical evolution to determine if capnia is a good criterion to decide when to introduce respiratory support.Patients and MethodsThis multicentric retrospective study reports the respiratory management and evolution of 17 SMA type 1 children (10 females) for whom treatment with Nusinersen was initiated between 2016 and 2018.ResultsMedian [interquartile range—IQR] age at diagnosis and at first Nusinersen injection was of 4 [3;8] and 4 [3;9] months, respectively. Patients were followed during 38 [24;44] months. Thirteen (76%) patients were started on NIV at a median [IQR] age of 12 [9;18] months. Repeated hospitalizations and intensive care unit admissions were needed for 11 of them. Blood gas and nocturnal gas exchange recordings performed before NIV initiation were always normal. 9/13 X‐ray performed before NIV showed atelectasis and/or acute lower respiratory tract infections. There was a significant decrease in the total number of hospital admissions between the first and second year of treatment (p = 0.04).ConclusionThis study shows that patients do not present with nocturnal hypoventilation before respiratory decompensations and NIV initiation, and suggests that a delay in NIV initiation might result in respiratory complications. There is a need for disease‐centered guidelines for the respiratory management of these patients, including NIV indications.
Impact of climate change on water availability in Marsyangdi river basin, Nepal
Authors: Mudbhari Dipak , Kansal Mitthan Lal , Kalura Praveen 
Published: March 2022

AbstractThe Hindu Kush Himalaya region in Nepal is considered hydrologically vulnerable and fragile, and is likely to face adverse impacts due to climate change. Many rivers originate in this region, which are the lifeline for the millions of riparian people. The Marsyangdi river basin is the hub of hydroelectric power generation. The main objective of this study is to assess the impact of climate change in water availability by using the Soil and Water Assessment Tool (SWAT) model. Future climate projections were derived from three regional climate models (RCMs), namely ACCESS1‐CCAM (ACCESS) CNRM‐CM5‐CCAM (CNRM), and MPI‐ESM‐LR‐CSIRO‐CCAM under two representative concentration pathways, RCP 4.5 and RCP 8.5. These projections were bias‐corrected against the observed historical data, and simulation of impact was assessed using the SWAT model. This study reveals an increase in maximum temperature by 1.4°C by the 2040s (2031–50) and 2.6°C by the 2070s (2061–80) and in minimum temperature by 1.8 and 2.9°C by the 2040s and 2070s, respectively. The future projected rainfall shows a declining trend in all the models. The maximum increase in annual rainfall in the CNRM model is about 31.1% for RCP 8.5 during the 2040s and the MPI RCP 8.5 model show a minimum reduction of about 19.6% in the 2070s. In all three models the average annual rainfall is expected to increase by 10.2 and 10.7% by the 2040s and 2070s respectively. The reduction in streamflow in 2031–2050 is maximally about 19% in the CNRM RCP 8.5 and minimally about 6.3% in the MPI RCP 8.5. For the period 2061–2080, the CNRM RCP 8.5 shows a maximum decrease in streamflow of about 18.9% and the MPI RCP 8.5 shows a minimum reduction in streamflow of about 2.6%. This study will give significant insight to the planners for sustainable and integrated management of water resources and for the operation and design of hydropower projects in the basin.
Monitoring fin and blue whales in the lower St. Lawrence Seaway with onshore seismometers
Authors: Plourde Alexandre P. , Nedimović Mladen R. 
Published: March 2022

AbstractThe Lower St. Lawrence Seaway (LSLS), in eastern Canada, is an important habitat for several species of endangered baleen whale. As we seek to reduce the hazards that these endangered species face from human activity, there is increasing demand for detailed knowledge of their habitat use. Only a sparse network of hydrophones exists in the LSLS to remotely observe whales. However, there is also a network of onshore seismometers, designed to monitor earthquakes, that have sufficiently high sample rates to record fin and blue whale calls. We present a simple method for detecting band‐limited, regularly repeating calls, such as the 20 Hz calls of fin and blue whales, and apply the method to build a catalog of fin and blue whale detections at 14 onshore seismometers across the LSLS, over approximately a 4‐year period. The resulting catalog contains over 600 000 fin whale calls and almost 60 000 blue whale calls. Individual calls are rarely detected at more than one seismometer. Fin whale calls recorded onshore often consist of multiple seismic phases arriving as a ∼2 sequence. Onshore seismometers provide a valuable, previously unused source of data for monitoring baleen whales. However, in the LSLS, the current seismometer network cannot provide high‐precision whale tracking alone, so a denser deployment of onshore and/or offshore seismometers is required.
Superior Dehydrogenation Performance of α‐AlH3 Catalyzed by Li3N: Realizing 8.0 wt.% Capacity at 100 °C
Authors: Zhao Shaolei , Liang Long , Liu Baozhong , Wang Limin , Liang Fei 
Published: March 2022

AbstractThe high dehydrogenation temperature of aluminum hydride (AlH3) has always been an obstacle to its application as a portable hydrogen source. To solve this problem, lithium nitride is introduced into the aluminum hydride system as a catalyst to optimize the dehydrogenation drastically, which reduces the initial dehydrogenation temperature from 140.0 to 66.8 °C, and provides a stable hydrogen capacity of 8.24, 6.18, and 5.75 wt.% at 100, 90, and 80 °C within 120 min by adjusting the mass fraction of lithium nitride. Approximately 8.0 wt.% hydrogen can be released within 15 min at 100 °C for the sample of 10 wt.% doping. Moderate dehydrogenation temperature slows down the inevitable self‐dehydrogenation process during the ball‐milling process, and the enhanced kinetics at lower temperature shows the possibility of application in the fuel cell.
Enzymatically Modified Cassava Starch as a Stabilizer for Fermented Dairy Beverages
Authors: Lopez‐Ochoa Juan David , Cadena‐Chamorro Edith , Ciro‐Velasquez Héctor , Rodríguez‐Sandoval Eduardo 
Published: March 2022

AbstractGrowing interest among consumers regarding sustainable food production has led to innovations in the use of cleaner additives and manufacturing methods for the food industry, such as the enzymatic modification of starches. The effect of enzymatic modification (pullulanase and amyloglucosidase) on the pasting and thermal properties of cassava starch and the quality properties of fermented dairy beverages are analyzed at the beginning and end of a 20‐day storage period and compared to beverages with chemically modified starch, native cassava starch (NAT), without the addition of starch (control), and a commercial beverage. Amyloglucosidase‐modified starch shows the highest degree of long‐term retrogradation, which can also be perceived in the quality of beverages during storage. Beverages prepared with pullulanase‐modified starch have the highest viscosity (503.56 mPa s) among the evaluated beverages and a lower syneresis (29.18 g/100 g) than control and NAT beverages. The addition of enzymatically modified cassava starch led to a significant increase in the general acceptability of the beverages compared to the control treatment. The results show that pullulanase‐modified starches have the potential to replace chemically modified starches in the production of fermented dairy beverages, enabling the development of products that meet the requirements of the natural foods market.
Regulation of mitophagy through HIF‐1α/miR‐140‐5p/PARKIN axis in acute kidney injury
Authors: Zhang Qin , Bian Zhi Xiang , Song Yanan , Wang Xiangxiang , Zhang Haili , Ren Qifang , Chen Shunjie 
Published: March 2022

AbstractMitochondria homeostasis plays an important role in acute kidney injury (AKI). In this study, we aimed at identifying the mechanism of mitophagy regulation in AKI. Activation of mitophagy after ischemic kidney injury was visualized with increased expression of LC3, PINK1, PARKIN expression and with a subsequent decline in p62 levels. Immuohistochemistry staining showed higher LC3 levels in ischemic kidney injury mice. Further, differential expression of PARKIN targeting miRNAs revealed that miR‐140‐5p was significantly downregulated followed by ischemic kidney injury. miR‐140‐5p mimics suppressed PARKIN expressions and their mitochondrial translocation. Further, miR‐140‐5p mimics under hypoxia prevented mitophagosome formation. These effects on hypoxia‐induced PARKIN expression and LC3/TOMM20 levels were reversed by antagomiR miR‐140‐5p treatment. Dual‐luciferase reporter assay revealed that miR‐140‐5p had significant interaction with 3'UTR of PARKIN. Our findings show that HIF‐1α is bound to miR‐140‐5p promoter and down regulates its expression and thereby promotes mitophagy process under hypoxic conditions. These results cumulatively show that HIF‐1α regulates mitophagy during AKI through the regulation of miR‐140‐5p/PARKIN axis.
Presence of multidrug resistant bacteria in Hurricane Ida overtop sediments in Southeast Louisiana, USA
Authors: Naquin Ethan , Boopathy Raj 
Published: March 2022

AbstractOn August 29, 2021, Hurricane Ida made landfall as a category 4 hurricane, bringing a torrent of wind, rain, and storm surge to Southeast Louisiana. This hurricane brought several feet of sediment into the residential areas of southeast Louisiana affecting homes and businesses. The sediment contains industrial waste and sewage sludge. A study was conducted to find the presence of antibiotic‐resistant bacteria (ARB) in the sediments. The results of this study show the presence of human polyomavirus BK in the sediments indicating the presence of human feces. The sediment also showed high levels of antibiotics such as tetracycline and sulfonamide. Presence of many multidrug resistant bacteria were found in the sediments along with the presence of various antibiotic resistance genes (ARGs) such as sul1 and sul2 for sulfonamide resistant and tetA gene for resistant to tetracycline. The heavy metal analysis revealed an elevated presence of heavy metal contamination.
Synthesis, Crystal and Electronic Structures, and Nonlinear Optical Properties of Y4Si3S12
Authors: Bardelli Stefano , Ye Zhengyang , Wang Fei , Zhang Bingbing , Wang Jian 
Published: March 2022

AbstractA new acentric sulfide Y4Si3S12 was grown by a high temperature vapor transport reaction. The crystal structure of Y4Si3S12 was determined by single crystal X‐ray diffraction. Y4Si3S12 is isostructural to La4Ge3S12. The three dimensional structure of Y4Si3S12 is constructed by [Y1S7] augmented triangular prisms, [Y2S6] triangular prisms and [SiS4] tetrahedra through sharing vertices and edges. Y4Si3S12 is revealed as an indirect bandgap semiconductor with a calculated bandgap of 2.1 eV, which is close to 2.5(1) eV experimentally measured by UV‐Vis. The Y−S interactions and Si−S interactions are predicated to be strong ionic bonds and covalent bonds, respectively, by electron localization function coupled with crystal orbital Hamilton population calculations. Y4Si3S12 is verified by DFT calculations to have moderate birefringence, with incident 1900 nm laser, Δn=0.09. DFT calculations also predicted that Y4Si3S12 possesses moderate second harmonic generation response with χ111=15.03 pm/V. The nonlinear optical properties of Y4Si3S12 are mainly contributed by Y−S interactions revealed by DFT calculations.
Titanium Superoxide for the Oxidation of Amines: Synthesis of bis(3‐nitro‐1H‐1,2,4‐triazol‐5‐yl)methane and its Metal Salts
Authors: Creegan Shannon E. , Zeller Matthias , Byrd Edward F. C. , Piercey Davin G. 
Published: March 2022

AbstractBis(3‐nitro‐1H‐1,2,4‐triazol‐5‐yl)methane (BNTM) was synthesized in 79 % yield via the oxidation of 5,5'‐diamino‐3,3’‐methylene‐1H‐1,2,4‐triazole (DABTM) with titanium superoxide in a water and hydrogen peroxide solution. BNTM synthesized by this method was characterized via low‐resolution mass spectrometry (LRMS), nuclear magnetic resonance spectroscopy (NMR), infrared spectroscopy (IR), elemental analysis, and single crystal X‐ray crystallography for comparison with BNTM produced by traditional routes. The cesium, lithium, potassium, rubidium, sodium, and strontium salts of BNTM were characterized for their thermal stabilities and mechanical sensitives. Each of the salts possessed multiple hydrates, the loss of the associated water can clearly be seen by thermal gravimetric analysis (TGA). All the salts were less sensitive to impact than neutral BNTM and, except for the sodium and cesium salts, were less sensitive to friction. Single crystal X‐ray crystallography was utilized to analyze the crystal structures obtained for the cesium, strontium, and sodium salts.
The Framing of Gambling and the Commercial Determinants of Harm: Challenges for Regulation in the UK
Authors: Reith Gerda , Wardle Heather 
Published: March 2022

AbstractThis article argues that the framing of gambling is crucial for how it is dealt with at every level; from legislative, regulatory and commercial practice to the terms of media and civic debate. Whoever frames the debate has power over the ways that we can and cannot think about gambling, as well as what we can do about it.We take the example of Britain as a case study in which, despite recent repeated calls for gambling to be regarded as a public health issue, it continues to be framed primarily in terms of economic activity and consumerism. We argue that this framing is the product of a particular political-economic model and that it is embedded in legislation and regulatory practice. We go on to describe the commercial landscape of gambling that has been produced by this framework as one which produces harm. As such, we make the point that framing is a key component of the commercial determinants of harm in gambling. The final section of our paper considers the various forms of political and commercial influence that infiltrate and shape the framing of gambling in Britain. This work was funded by Wellcome Trust through a Humanities and Social Sciences Fellowship to Heather Wardle (grant number: 200306).