bluuebunny/crossref_metadata_2025_split huggingface dataset:
RESTORE: A Prospective Multinational Registry of Patients with Genetically Confirmed Spinal Muscular Atrophy - Rationale and Study Design
Authors: Finkel Richard S. , Day John W. , De Vivo Darryl C. , Kirschner Janbernd , Mercuri Eugenio , Muntoni Francesco , Shieh Perry B. , Tizzano Eduardo , Desguerre Isabelle , Quijano-Roy Susana , Saito Kayoko , Droege Marcus , Dabbous Omar , Khan Farid , Renault Lydie , Anderson Frederick A. , Servais Laurent 
Published: February 2020

Background: Dramatic improvements in spinal muscular atrophy (SMA) treatment have changed the prognosis for patients with this disease, leading to important new questions. Gathering representative, real-world data about the long-term efficacy and safety of emerging SMA interventions is essential to document their impact on patients and caregivers. Objectives: This registry will assess outcomes in patients with genetically confirmed SMA and provide information on the effectiveness and long-term safety of approved and emerging treatments. Design and Methods: RESTORE is a prospective, multicenter, multinational observational registry. Patients will be managed according to usual clinical practice. Both newly recruitedSMAtreatment centers and sites involved in existing SMA registries, including iSMAC, Treat-NMD, French SMA Assistance Publique- Hôpitaux de Paris (AP-HP), Cure-SMA, SMArtCARE, will be eligible to participate; de novo; sites already participating in another registry may be included via consortium agreements. Data from patients enrolled in partnering registries will be shared with the RESTORE Registry and data for newly diagnosed patients will be added upon enrollment. Patients will be enrolled over a 5-year period and followed for 15 years or until death. Assessments will include SMA history and treatment, pulmonary, nutritional, and motor milestones, healthcare resource utilization, work productivity, activity impairment, adverse events, quality of life, caregiver burden, and survival. Status: Recruitment started in September 2018. As of January 3, 2020, 64 patients were enrolled at 25 participating sites. Conclusions: The RESTORE Registry has begun recruiting recently diagnosed patients with genetically confirmed SMA, enabling assessment of both short- and long-term patient outcomes.
Fingolimod Profoundly Reduces Frequencies and Alters Subset Composition of Circulating T Follicular Helper Cells in Multiple Sclerosis Patients
Authors: Huber Johanna E. , Chang Yinshui , Meinl Ingrid , Kümpfel Tania , Meinl Edgar , Baumjohann Dirk 
Published: February 2020

Abstract
Fingolimod is an effective treatment for relapsing-remitting multiple sclerosis. It is well established that fingolimod, a modulator of the sphingosine-1-phosphate pathway, restrains the egress of CCR7+ lymphocytes from lymphatic tissues into the blood, thus resulting in reduced lymphocyte counts in peripheral blood. CXCR5+ T follicular helper (Tfh) cells provide help to B cells, are essential for the generation of potent Ab responses, and have been shown to be critically involved in the pathogenesis of several autoimmune diseases. Besides lymphoid tissue-resident Tfh cells, CXCR5+ circulating Tfh (cTfh) cells have been described in the blood, their numbers correlating with the magnitude of Tfh cells in lymphoid tissues. Although the effect of fingolimod on circulating lymphocyte subsets has been established, its effect on cTfh cells remains poorly understood. In this study, we found that although fingolimod strongly and disproportionally reduced cTfh cell frequencies, frequencies of activated cTfh cells were increased, and the composition of the cTfh cell pool was skewed toward a cTfh1 cell phenotype. The circulating T follicular regulatory cell subset and CXCR5+ CD8+ T cell frequencies were also strongly and disproportionally decreased after fingolimod treatment. In contrast, relative frequencies of CXCR5– memory Th cells as well as regulatory T and B cells were increased. In summary, these data provide new insights into fingolimod-induced compositional changes of lymphocyte populations in the blood, in particular cTfh cells, and thus contribute to a better understanding of the mechanism of action of fingolimod in multiple sclerosis patients.
Delayed sleep-wake phase disorder in a clinical population: gender and sub-population diferences
Authors: Reis Cátia , Paiva Teresa 
Published: February 2020

Objective/Background Delayed sleep-wake phase disorder (DSWPD) is defined by a delay in the major sleep episode relative to desired or required sleep and wake times. The objectives of this study were to evaluate DSWPD in our population and to compare it with similar clinical data, to analyse gender differences, and to identify possible subpopulations based on circadian timing and alignment.
Patients/Methods 162 consecutive DSWPD patients from a sleep clinic with a median age of 35.5 (24.0) years, 85 (52.5%) males were studied. Patient data were obtained from a clinical interview composed of socio-demographic, life events, daily habits, consumptions, and comorbidities data; and from diaries, actimetry, melatonin and PSG T1. The Dim Light Melatonin Onset (DLMO) was used to define circadian alignment or misalignment.
Results In our DSWPD cohort, there were gender differences for different age groups (p=0.028). Men were more likely to be single and women more likely to be married (p=0.034). In students, school failure was higher for women (p<0.001); for workers, absenteeism was higher in women (p=0.001). In the circadian aligned (compared to misaligned group), DLMO was later (p<0.001), sleep onset time (p=0.046) was later, total sleep time (p=0.035), and number of sleep cycles (p=0.018) were lower, as measured using PSG T1.
Conclusions In this clinical population, DSWPD is more prevalent in young men and in middle age women, although with no overall significant differences between genders. There are two different phenotypes of DSWPD: circadian misaligned and circadian aligned. Depression is prevalent in both groups. Better definition, classification and diagnostic criteria for DSWPD are still needed, and targeted therapeutical intervention should be evaluated.
Electrodynamics of Magnetoelectric Media and Magnetoelectric Fields
Authors: Kamenetskii Eugene O. 
Published: February 2020

AbstractThe relationship between magnetoelectricity and electromagnetism is a subject of a strong interest and numerous discussions in microwave and optical wave physics and material sciences. The definition of the energy and momentum of the electromagnetic (EM) field in a magnetoelectric (ME) medium is not a trivial problem. The question of whether electromagnetism and magnetoelectricity can coexist without an extension of Maxwell's theory arises when the effects of EM energy propagation are studied and the group velocity of the waves in an ME medium is considered. The energy balance equation reveals unusual topological structure of fields in ME materials. Together with certain constraints on the constitutive parameters of a medium, definite constraints on the local field structure should be imposed. Analyzing the EM phenomena inside an ME material, the question “what kind of the near fields arising from a sample of such a material can be measured?” should be answered. The visualization of the ME states requires an experimental technique that is based on an effective coupling to the violation of spatial as well as temporal inversion symmetry. To observe the ME energy in a subwavelength region, it is necessary to assume the existence of first‐principle near fields—the ME fields. These are non‐Maxwellian near fields with specific properties of violation of spatial and temporal inversion symmetry. A particular interest to the ME fields arises in studies of metamaterials with “artificial‐atoms” ME elements.
Access to 1,3‐Dinitriles by Enantioselective Auto‐tandem Catalysis: Merging Allylic Cyanation with Asymmetric Hydrocyanation
Authors: Long Jinguo , Yu Rongrong , Gao Jihui , Fang Xianjie 
Published: February 2020

AbstractEnantioselective auto‐tandem catalysis represents a challenging yet highlight attractive topic in the field of asymmetric catalysis. In this context, we describe a dual catalytic cycle that merges allylic cyanation and asymmetric hydrocyanation. The one‐pot conversion of a broad array of allylic alcohols into their corresponding 1,3‐dinitriles proceeds in good yield with high enantioselectivity. The products are densely functionalized and can be easily transformed to chiral diamines, dinitriles, diesters, and piperidines. Mechanistic studies clearly support a novel sequential cyanation/hydrocyanation pathway.
Institutional pressures and the accounting and reporting of environmental liabilities
Authors: Negash Minga , Lemma Tesfaye T. 
Published: February 2020

AbstractThe study explores the role of institutional forces in shaping corporate accounting and reporting for environmental liabilities practice within the context of South Africa. Drawing on the literature that conceptualizes accounting as a “social and institutional practice” and using Scott's institutional pillars approach, we qualitatively analyze data obtained through in‐depth interviews and documentary reviews and demonstrate that environmental liabilities reported on financial statements of South African companies may not reflect the full extent of environmental obligations stemming from their operations. The enabling environment for this dereliction of corporate responsibility is fostered by the following: (a) a mosaic of weakly enforced laws, acts, and regulations that constitutes the regulative pillar; (b) a cultural‐cognitive template that is still at an early stage of its development; and (c) a stronger normative pillar reinforced by relatively powerful actors who support the institutionalization of normative modes and conflicts and/or contradictions among actors who support the regulative institutions. Our findings suggest that companies in South Africa tend to adopt practices that foster production and disclosure of environmental information that comply with the normatively sanctioned scripts to maintain legitimacy with important stakeholders while decoupling these practices from the regulative prescripts that call for greater environmental accountability.
Graphene/Amorphous Carbon Restriction Structure for Stable and Long‐Lifespan Antimony Anode in Potassium‐Ion Batteries
Authors: Yang Xu , Zhang Rongyu , Xu Shifeng , Xu Dan , Ma Jia , Zhang Zhongyu , Yang Shu 
Published: February 2020

AbstractSb‐based materials have attracted much attention owing to their ability to undergo a multi‐electron alloy reaction with K+. However, there are still the serious problems of volume change and aggregation of particles, which lead to rapid capacity fading and a limited lifespan. In this work, a graphene/amorphous carbon restriction structure is proposed, in which the amorphous carbon layer on the surface of Sb nanoparticles can protect the particles from pulverization, and the graphene can buffer the volume change of the material. In addition, the conductive network formed by the dual carbon structure effectively improves the rate performance of the material. Thus, the material delivers a high capacity of 550 mA h g−1 at 100 mA g−1, a rate capability of 370 mA h g−1 at 2000 mA g−1, and a long lifespan of 350 cycles without significant capacity fading. The dual carbon strategy proposed offers a reference for the design of high‐performance anode materials.
Synthesis of the 8,19‐Epoxysteroid Eurysterol A
Authors: Taspinar Ömer , Wilczek Tobias , Erver Julian , Breugst Martin , Neudörfl Jörg‐Martin , Schmalz Hans‐Günther 
Published: February 2020

AbstractWe report the first chemical synthesis of eurysterol A, a cytotoxic and antifungal marine steroidal sulfate with a unique C8−C19 oxy‐bridged cholestane skeleton. After C19 hydroxylation of cholesteryl acetate, used as an inexpensive commercial starting material, the challenging oxidative functionalization of ring B was achieved by two different routes to set up a 5α‐hydroxy‐7‐en‐6‐one moiety. As a key step, an intramolecular oxa‐Michael addition was exploited to close the oxy‐bridge (8β,19‐epoxy unit). DFT calculations show this reversible transformation being exergonic by about −30 kJ mol−1. Along the optimized (scalable) synthetic sequence, the target natural product was obtained in only 11 steps in 5 % overall yield. In addition, an access to (isomeric) 7β,19‐epoxy steroids with a previously unknown pentacyclic ring system was discovered.
Coronary atherosclerosis in middle‐aged athletes: Current insights, burning questions, and future perspectives
Authors: Aengevaeren Vincent L. , Eijsvogels Thijs M. H. 
Published: February 2020

AbstractRegular exercise training is considered healthy as it reduces the risk of cardiovascular events and mortality. Nevertheless, athletes are not immune to the development of cardiovascular diseases and recent studies reported a higher prevalence of coronary artery calcifications and atherosclerotic plaques in athletes compared to less active controls. These observations have raised many questions among sport scientists, sports cardiologists, amateur athletes, and the general population. For example, Are athletes (not) immune for coronary atherosclerosis? How to assess coronary atherosclerosis in athletes? What about chalk (calcified plaque) and cheese (mixed plaque)? Does exercise intensity play a role? Are there sport‐related differences? Are there sex differences? Can sports medical evaluation detect coronary atherosclerosis? Do athletes get worried? Should athletes get worried? How should athletes with coronary atherosclerosis be managed? The goal of this review is to discuss the latest scientific insights and to answer these important questions. Furthermore, we will explore potential clinical implications and point out directions for further research.
Can temporal covariation and autocorrelation in demographic rates affect population dynamics in a raptor species?
Authors: Fay Rémi , Michler Stephanie , Laesser Jacques , Jeanmonod Jacques , Schaub Michael 
Published: February 2020

AbstractTheoretical studies suggest that temporal covariation among and temporal autocorrelation within demographic rates are important features of population dynamics. Yet, empirical studies have rarely focused on temporal covariation and autocorrelation limiting our understanding of these patterns in natural populations. This lack of knowledge restrains our ability to fully understand population dynamics and to make reliable population forecasts. In order to fill this gap, we used a long‐term monitoring (15 years) of a kestrel Falco tinnunculus population to investigate covariation and autocorrelation in survival and reproduction at the population level and their impact on population dynamics. Using Bayesian joint analyses, we found support for positive covariation between survival and reproduction, but weak autocorrelation through time. This positive covariation was stronger in juveniles compared with adults. As expected for a specialized predator, we found that the reproductive performance was strongly related to an index of vole abundance explaining 86% of the temporal variation. This very strong relationship suggests that the temporally variable prey abundance may drive the positive covariation between survival and reproduction in this kestrel population. Simulations suggested that the observed effect size of covariation could be strong enough to affect population dynamics. More generally, positive covariation and autocorrelation have a destabilizing effect increasing substantially the temporal variability of population size.
The functional interplay of lncRNA EGOT and HuR regulates hypoxia‐induced autophagy in renal tubular cells
Authors: Wang I‐Kuan , Palanisamy Kalaiselvi , Sun Kuo‐Ting , Yu Shao‐Hua , Yu Tung‐Min , Li Ching‐Hao , Lin Feng‐Yen , Chou An‐Kuo , Wang Guei‐Jane , Chen Kuen‐Bao , Li Chi‐Yuan 
Published: February 2020

AbstractAutophagy, an important cellular homeostatic mechanism regulates cell survival under stress and protects against acute kidney injury. However, the role of long noncoding RNA (lncRNA) in autophagy regulation in renal tubular cells (HK‐2) is unclear. The study was aimed to understand the importance of lncRNA in hypoxia‐induced autophagy in HK‐2 cells. LncRNA eosinophil granule ontogeny transcript (EGOT) was identified as autophagy‐associated lncRNA under hypoxia. The lncRNA EGOT expression was significantly downregulated in renal tubular cells during hypoxia‐induced autophagy. Gain‐ and loss‐of‐EGOT functional studies revealed that EGOT overexpression reduced autophagy by downregulation of ATG7, ATG16L1, LC3II expressions and LC 3 puncta while EGOT knockdown reversed the suppression of autophagy. Importantly, RNA‐binding protein, (ELAVL1)/Hu antigen R (HuR) binds and stabilizes the EGOT expression under normoxia and ATG7/16L1 expressions under hypoxia. Furthermore, HuR mediated stabilization of ATG7/16L1 expressions under hypoxia causes a decline in EGOT levels and thereby promotes autophagy. Altogether, the study first reveals the functional interplay of lncRNA EGOT and HuR on the posttranscriptional regulation of the ATG7/16L1 expressions. Thus, the HuR/EGOT/ATG7/16L1 axis is crucial for hypoxia‐induced autophagy in renal tubular cells.
Twitter as a tool for social movement: An analysis of feminist activism on social media communities
Authors: Li Manyu , Turki Nadia , Izaguirre Cassandra R. , DeMahy Chloe , Thibodeaux Brooklyn Labery , Gage Taylor 
Published: February 2020

AbstractIn recent years, social media has been widely used as a tool for feminist social movements, addressing social problems such as sexual assault traumatization. This research aims at understanding how social media users utilized Twitter to describe traumatic sexual assault experiences and reasons victims chose not to disclose their experiences (Study 1), and how users became a part of the digital activism (i.e., social media movement against sexual assault) to increase social actions (Study 2). Tweets using the hashtag #WhyIDidntReport and #MeToo were extracted. Thematic analyses were used to analyze tweets across the two studies. Results from Study 1 revealed that social media victims who self‐disclosed their victimization stories often reported having serious psychological impacts, a sense of helplessness, and issues with the police. Study 2 further uncovered that social media users engaged in hashtag activism through discussing views on relevant political and social issues, sharing resources to help sexual assault victims, and promoting social actions (e.g., protests, voting).
Partial omission of bleomycin for early‐stage Hodgkin lymphoma patients treated with combined modality therapy: Does incomplete ABVD lead to inferior outcomes?
Authors: Gunther Jillian R. , Pinnix Chelsea C. , Glober Gordon R. , Christopherson Kaitlin M. , Fang Penny , Lee Hun Ju , Ahmed Sairah , Steiner Raphael E. , Nair Ranjit , Strati Paolo , Neelapu Sattva S. , Nastoupil Loretta J. , Dabaja Bouthaina S. 
Published: February 2020

AbstractClassical Hodgkin lymphoma (HL) patients achieve excellent outcomes; therefore, treatment de‐escalation strategies to spare toxicity have been prioritized. In a large randomized trial of early‐stage HL patients, omission of chemotherapeutic agents including bleomycin from the standard ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) regimen was not found to be noninferior; however, the effect of partial omission is unknown. We investigated the effect of bleomycin omission on outcome for 150 early‐stage HL patients. At 8 years, freedom from relapse was 99% for both patients who received complete or incomplete bleomycin, which is reassuring for patients requiring bleomycin omission due to toxicity.
Hind foot drumming: Morphofunctional analysis of the hind limb osteology in three species of African mole‐rats (Bathyergidae)
Authors: Sahd Lauren , Bennett Nigel C. , Kotzé Sanet H. 
Published: February 2020

AbstractHind foot drumming is a form of seismic signaling that plays a vital role in the communication of several Bathyergidae species. Hind foot drumming is initiated by the rapid movement of the whole hind limb by flexion and extension of the hip and knee. This study aimed to determine if morphological adaptations of the hind limb osteology were measurable using established morphometric analyses in two drumming (Bathyergus suillus and Georychus capensis) and one non‐drumming (Cryptomys hottentotus natalensis) African mole‐rat species. Forty‐three linear measurements of the hind limb were taken in 48 limbs (n = 16 limbs per species) and 32 indices were calculated. Mixed model analysis of variance was used to compare the three species and sexes within a species. Thirteen indices had significant differences between species. Eleven indices had significant differences between sexes within a species. Significant differences between the drumming (B. suillus and G. capensis) and the non‐drumming species were observed in three indices. The femoral greater trochanter was relatively shorter in the drumming species compared to the non‐drumming species, which is proposed to allow for increased hip joint mobility, thereby permitting drummers to move their limbs at the rapid speed required to generate seismic signals. Furthermore, the small in‐lever (shorter greater trochanter) may increase the velocity of limb motion. The robust tibias in the drumming species, as indicated by the tibial robustness index, are likely to counter the additional biomechanical load caused by the muscles involved in hind foot drumming. The relatively small hind feet seen in the drumming species allows for reduced limb weight needed for the rapid extension and flexion motion required during hind foot drumming. The significant differences reflected in the hind limb osteological indices between B. suillus and G. capensis and the non‐drumming species are indicative of adaptations for hind foot drumming.
Kinetics of water‐extractable zinc release from seaweed (Fucus serratus) as soil amendment
Authors: Oluwadare David A. , Carney Helen E. , Sarker Mosharraf H. , Ennis Christopher J. 
Published: February 2020

AbstractSoil fertilization with trace‐metal rich organic fertilizers such as Fucus serratus seaweed may be an effective way to combat micronutrient deficiency. In this study the kinetics of zinc release from Fucus serratus seaweed was investigated in a packed soil column leaching experiment over 1,776 h. The release of zinc from control (soil only) and treatment (soil + seaweed; equivalent zinc application rate of 1.42 kg ha−1) columns, measured by ICP‐MS, demonstrated two distinct release stages. The cumulative zinc release data for each phase were fitted to five kinetic models: zero order, first order, Elovich, power function and parabolic diffusion. In the first stage (0–400 hours) the release of zinc from both control and treatment was best described by a parabolic rate law, indicating release of zinc from a soluble soil reservoir. In the second stage (400–1,776 h) zinc release followed a zero order rate law indicative of slow release from an essentially insoluble reservoir. The modelled difference between the amount of zinc released from treatment and control columns in stage 1 (230 ± 11 µg) represented the total amount of zinc added via seaweed. The parabolic rate constant for seaweed zinc release was 12.09 µg g−1 h−0.5. In summary, the addition of F. serratus to soil is a viable source of labile zinc and a low cost agronomic option for mitigating zinc deficiency in soils.