bluuebunny/crossref_metadata_2025_split huggingface dataset:
Association of the Systemic Lupus International Collaborating Clinics Frailty Index and Damage Accrual in Longstanding Systemic Lupus Erythematosus
Authors: Lima Kaitlin , Legge Alexandra , Hanly John G. , Lee Jungwha , Song Jing , Chung Anh , Ramsey‐Goldman Rosalind 
Published: September 2021

ObjectiveTo externally validate the Systemic Lupus International Collaborating Clinics Frailty Index (SLICC‐FI) in a prevalent systemic lupus erythematosus (SLE) cohort and to assess the ability of the SLICC‐FI to predict organ damage accrual among individuals with longstanding SLE.MethodsThis was a secondary analysis of data from the Study of Lupus Vascular and Bone Long‐Term Endpoints (SOLVABLE) cohort, which consists of adult women from the Chicago Lupus Database who met the 1997 revised American College of Rheumatology (ACR) classification criteria for SLE. There were 185 patients with SLE enrolled, of whom 149 patients were included in a 5‐year follow‐up analysis. The SLICC‐FI and SLICC/ACR Damage Index (SDI) scores were calculated at baseline and 5‐year follow‐up. Unadjusted and adjusted logistic regression models estimated the association of baseline SLICC‐FI scores (per 0.05 increase) with damage accrual at 5‐year follow‐up.ResultsAt enrollment the mean ± SD age of the 149 patients was 43.30 ± 10.15 years, the mean ± SD disease duration was 11.93 ± 8.46 years, and the mean ± SD SDI score was 1.64 ± 1.83. At baseline, the mean ± SD SLICC‐FI score was 0.18 ± 0.08, and 36% of participants were categorized as frail (SLICC‐FI score >0.21). In a model adjusted for age, race, and disease duration, each 0.05‐unit increase in the baseline SLICC‐FI score was associated with 28% higher odds of subsequent damage accrual (odds ratio 1.28, 95% confidence interval 1.01–1.63).ConclusionIn a prevalent cohort of women with established SLE, higher baseline SLICC‐FI scores were associated with a higher risk of subsequent damage accrual at 5‐year follow‐up.
Finite time adaptive fault‐tolerant controller based on neural networks for switched stochastic nonlinear systems with actuator failures and disturbance
Authors: He Qingzhi , Mu Xiaowu , Cheng Guifang 
Published: September 2021

SummaryThis article investigates the novel finite time adaptive neural fault‐tolerant controller (FTC) for strict‐feedback switched stochastic systems under arbitrary switching signals and takes into actuator failures including loss of effectiveness faults and bias faults consideration concurrently. Neural networks are utilized to approximate the unknown external disturbance and internal dynamics. On the basis of Itô differential equation and backstepping technique, an adaptive neural finite time FTC method is put forward. It is attested that the closed‐loop systems are semiglobal practical finite time stable in probability and the tracking effects are great. Finally, to further demonstrate the high efficiency of proposed control method, two simulation examples are given.
Formation and Cellular Impact of Cholesterol Crystals in Health and Disease
Authors: Baumer Yvonne , McCurdy Sara G. , Boisvert William A. 
Published: September 2021

AbstractCholesterol crystals (CCs) were first discovered in atherosclerotic plaque tissue in the early 1900 and have since been observed and implicated in many diseases and conditions, including myocardial infarction, abdominal aortic aneurism, kidney disease, ocular diseases, and even central nervous system anomalies. Despite the widespread involvement of CCs in many pathologies, the mechanisms involved in their formation and their role in various diseases are still not fully understood. Current knowledge concerning the formation of CCs, as well as the molecular pathways activated upon cellular exposure to CCs, will be explored in this review. As CC formation is tightly associated with lipid metabolism, the role of cellular lipid homeostasis in the formation of CCs is highlighted, including the role of lysosomes. In addition, cellular pathways and processes known to be affected by CCs are described. In particular, CC‐induced activation of the inflammasome and production of reactive oxygen species, along with the role of CCs in complement‐mediated inflammation is discussed. Moreover, the clinical manifestation of embolized CCs is described with a focus on renal and skin diseases associated with CC embolism. Lastly, potential therapeutic measures that target either the formation of CCs or their impact on different cell types and tissues are highlighted.
Giant Red‐Shifted Emission in (Sr,Ba)Y2O4:Eu2+ Phosphor Toward Broadband Near‐Infrared Luminescence
Authors: Yang Zhiyu , Zhao Yifei , Zhou Yayun , Qiao Jianwei , Chuang Yu‐Chun , Molokeev Maxim S. , Xia Zhiguo 
Published: September 2021

AbstractNear‐infrared (NIR) light‐emitting diodes (LEDs) light sources are desirable in photonic, optoelectronic, and biological applications. However, developing broadband red and NIR‐emitting phosphors with good thermal stability is always a challenge. Herein, the synthesis of Eu2+‐activated SrY2O4 red phosphor with high photoluminescence quantum efficiency and broad emission band ranging from 540 to 770 nm and peaking at 620 nm under 450 nm excitation is designed. Sr/Ba substitution in SrY2O4:Eu2+ has been further utilized to achieve tunable emission by modifying the local environment, which facilitates the giant red‐shifted emission from 620 to 773 nm while maintaining the outstanding thermal stability of SrY2O4:Eu2+. The NIR emission is attributed to the enhanced Stokes shift and crystal field strength originated from the local structural distortions of [Y1/Eu1O6] and [Y2/Eu2O6]. The investigation in charge distribution around Y/Eu provides additional insight into increasing covalency to tune the emission toward the NIR region. As‐fabricated NIR phosphor‐converted LEDs demonstration shows its potential in night‐vision technologies. This study reveals the NIR luminescence mechanism of Eu2+ in oxide‐based hosts and provides a design principle for exploiting Eu2+‐doped NIR phosphors with good thermal stability.
Spin‐Printing of Liquid Crystal Polymer into Recyclable and Strong All‐Fiber Materials
Authors: Gantenbein Silvan , Mascolo Chiara , Houriet Caroline , Zboray Robert , Neels Antonia , Masania Kunal , Studart André R. 
Published: September 2021

AbstractFiber‐reinforced polymers are widely used as lightweight materials in aircraft, automobiles, wind turbine blades, and sports products. Despite the beneficial weight reduction achieved in such applications, these composites often suffer from poor recyclability and limited geometries. 3D printing of liquid crystal polymers into complex‐shaped all‐fiber materials is a promising approach to tackle these issues and thus increase the sustainability of current lightweight structures. Here, we report a spin‐printing technology for the manufacturing of recyclable and strong all‐fiber lightweight materials. All‐fiber architectures are created by combining thick print lines and thin spun fibers as reinforcing elements in bespoke orientations. Through controlled extrusion experiments and theoretical analyses, we systematically study the spinning process and establish criteria for the generation of thin fibers and laminates with unprecedented mechanical properties. The potential of the technology is further illustrated by creating complex structures with unique all‐fiber architectures and mechanical performance.
Self‐Polymerization of Monomer and Induced Interactions with Perovskite for Highly Performed and Stable Perovskite Solar Cells
Authors: Ma Ruiman , Zheng Jiawei , Tian Yu , Li Can , Lyu Benzheng , Lu Linyang , Su Zhenhuang , Chen Li , Gao Xingyu , Tang Jian‐Xin , Choy Wallace C. H. 
Published: September 2021

AbstractWhile there is promising achievement in terms of the power conversion efficiency (PCE) of perovskite solar cells (PSCs), long‐term stability has been considered the main obstacle for their practical application. In this work, the authors demonstrate the small monomer 2‐(dimethylamino) ethyl methacrylate (DMAEMA) with unsaturated carboxylic acid ester bond in the antisolvent for perovskite formation to improve the PCE and stability. The results show that DMAEMA is self‐polymerized and uniformly distributed in the film, contributing to the improved crystallinity of the perovskites. Equally important, it is found that there are newly established interactions of Pb2+ and DMAEMA, and iodine and ternary amine between DMAEMA and perovskites, which improves the uniformity of the lead (II) iodide vertical distribution along with the films and thus phase stability, as well as largely decreases defects density in the film. Overall, the inverted PSCs with DMAEMA exhibit a open‐circuit voltage of 1.10 V, short‐circuit current of 23.86 mA cm−2, fill factor of 0.82, and finally PCE reaches 21.52%. Meanwhile, the PSC stability is significantly improved due to the inhibition of the formation of iodine, reduction of the uncoordinated Pb2+, and suppression of phase segregation.
Highly Efficient Green‐Emitting Phosphor BaZnAl10O17:Mn2+ with Ultra‐Narrow Band and Extremely Low Thermal Quenching for Wide Color Gamut LCD Backlights
Authors: Li Haoran , Liang Yujun , Liu Shiqi , Zhang Weilun , Bi Yanying , Gong Yuming , Lei Wen 
Published: September 2021

AbstractNarrow‐band green‐emitting phosphors have attracted increasing attention for designing wide color gamut devices with high quality. However, the development of highly efficient and thermally stable green phosphor with narrow band is still a significant challenge. Herein, a novel green narrow‐band phosphor, BaZnAl10O17:Mn2+ (BZAO:Mn2+) is developed owing to the weak coupling interaction of Mn ions in the highly symmetric BZAO with rigid structure. Upon 450 nm excitation, BZAO:0.20Mn2+ shows an intense green emission peaking at 516 nm with a full width at half maximum of 31 nm, a color purity of 78%, and an internal quantum efficiency of 85.59%. Moreover, when temperature rises to 423 K the emission peak intensity still maintains 95% of that at 298 K, and the color and brightness present no obvious degradation, indicating an extremely indistinctive thermal quenching behavior. The fabricated light emitting diode (LED) device exhibits bright white light with corrected color temperature (CCT) of 6732 K and its color gamut (CG) value reaches as wide as 110% National Television System Committee (NTSC) standard. These results demonstrate BZAO:0.20Mn2+ can be considered as a potential efficient phosphor with green narrow‐band emission for wide color gamut backlight.
Design of Superhydrophobic Surfaces for Stable Perovskite Solar Cells with Reducing Lead Leakage
Authors: Zhang Hua , Li Kang , Sun Man , Wang Fanglin , Wang Huan , Jen Alex K.‐Y. 
Published: September 2021

AbstractEnvironment‐related degradation and lead leakage in perovskite solar cells have posed a big challenge for their commercialization. Here, design of superhydrophobic surfaces is demonstrated as an effective strategy toward these issues, in which thiol‐functionalized perfluoroalkyl molecules are employed to chemically modify the lead halide perovskite film and metal electrode via a vapor‐assisted self‐assembly process. Due to the van der Waals forces, the generation of self‐assembly monolayer prefers to pack in a dense way, resulting in the formation of a closest‐packed, crystalline‐like molecular array. This dense array is endowed with a low‐surface‐energy chemistry that can not only enhance the water and oxygen resistance of the completed device but also reduce the defect density on the perovskite surfaces. These merits eventually boost the efficiency of inverted perovskite solar cells up to 21.79% along with a substantially improved long‐term stability. More importantly, the thiol‐functionalized superhydrophobic array can immobilize most of the undercoordinated lead ions on the perovskite surfaces by metal‐thiol coordination effect, which results in suppressing the lead leakage from the water‐soluble lead halide perovskites. Therefore, an avenue is pointed out here to fabricate stable perovskite solar cells with reducing lead leakage, representing a substantial step toward practical applications.
Judge it by its shape: a pollinator‐blind approach reveals convergence in petal shape and infers pollination modes in the genus Erythrina
Authors: Bilbao Gonzalo , Bruneau Anne , Joly Simon 
Published: September 2021

AbstractPremisePollinators are thought to exert selective pressures on plants, mediating the evolution of convergent floral shape often recognized as pollination syndromes. However, little is known about the accuracy of using petal shape for inferring convergence in pollination mode without a priori pollination information. Here we studied the genus Erythrina L. as a test case to assess whether ornithophyllous pollination modes (hummingbirds, passerines, sunbirds, or mixed pollination) can be inferred based on the evolutionary analysis of petal shape.MethodsWe characterized the two‐dimensional dissected shape of standard, keel, and wing petals from 106 Erythrina species using geometric morphometrics and reconstructed a phylogenetic tree of 83 Erythrina species based on plastid trnL‐F and nuclear ribosomal ITS sequences. We then used two phylogenetic comparative methods based on Ornstein–Uhlenbeck models, SURFACE and l1OU, to infer distinct morphological groups using petal shape and identify instances of convergent evolution. The effectiveness of these methods was evaluated by comparing the groups inferred to known pollinators.ResultsWe found significant petal shape differences between hummingbird‐ and passerine‐pollinated Erythrina species. Our analyses also revealed that petal combinations generally provided better inferences of pollinator types than individual petals and that the method and optimization criterion can affect the results.ConclusionsWe show that model‐based approaches using petal shape can detect convergent evolution of floral shape and relatively accurately infer pollination modes in Erythrina. The inference power of the keel petals argues for a deeper investigation of their role in the pollination biology of Erythrina and other bird‐pollinated legumes.
Evidence for human‐caused founder effect in populations ofSolanum jamesiifound at archaeological sites: I. Breeding experiments and the geography of sexual reproduction
Authors: Pavlik Bruce M. , Baker Margaret , Bamberg John , del Rio Alfonso , Kinder David , Louderback Lisbeth A. 
Published: September 2021

AbstractPremisePlant domestication can be detected when transport, use, and manipulation of propagules impact reproductive functionality, especially in species with self‐incompatible breeding systems.MethodsEvidence for human‐caused founder effect in the Four Corners potato (Solanum jamesiiTorr.) was examined by conducting 526 controlled matings between archaeological and non‐archaeological populations from field‐collected tubers grown in a greenhouse. Specimens from 24 major herbaria and collection records from >160 populations were examined to determine which produced fruits.ResultsArchaeological populations did not produce any fruits when self‐crossed or outcrossed between individuals from the same source. A weak ability to self‐ or outcross within populations was observed in non‐archaeological populations. Outcrossing between archaeological and non‐archaeological populations, however, produced fully formed, seed‐containing fruits, especially with a non‐archaeological pollen source. Fruit formation was observed in 51 of 162 occurrences, with minimal evidence of constraint by monsoonal drought, lack of pollinators, or spatial separation of suitable partners. Some archaeological populations (especially those along ancient trade routes) had records of fruit production (Chaco Canyon), while others (those in northern Arizona, western Colorado, and southern Utah) did not.ConclusionsThe present study suggests that archaeological populations could have different origins at different times—some descending directly from large gene pools to the south and others derived from gardens already established around occupations. The latter experienced a chain of founder events, which presumably would further reduce genetic diversity and mating capability. Consequently, some archaeological populations lack the genetic ability to sexually reproduce, likely as the result of human‐caused founder effect.
Dispersal evolution in temporally variable environments: implications for plant range dynamics
Authors: Oldfather Meagan F. , Van Den Elzen Courtney L. , Heffernan Patrick M. , Emery Nancy C. 
Published: September 2021

AbstractDispersal—the movement of an individual from the site of birth to a different site for reproduction—is an ecological and evolutionary driver of species ranges that shapes patterns of colonization, connectivity, gene flow, and adaptation. In plants, the traits that influence dispersal often vary within and among species, are heritable, and evolve in response to the fitness consequences of moving through heterogeneous landscapes. Spatial and temporal variation in the quality and quantity of habitat are important sources of selection on dispersal strategies across species ranges. While recent reviews have evaluated the interactions between spatial variation in habitat and dispersal dynamics, the extent to which geographic variation in temporal variability can also shape range‐wide patterns in dispersal traits has not been synthesized. In this paper, we summarize key predictions from metapopulation models that evaluate how dispersal evolves in response to spatial and temporal habitat variability. Next, we compile empirical data that quantify temporal variability in plant demography and patterns of dispersal trait variation across species ranges to evaluate the hypothesis that higher temporal variability favors increased dispersal at plant range limits. We found some suggestive evidence supporting this hypothesis while more generally identifying a major gap in empirical work evaluating plant metapopulation dynamics across species ranges and geographic variation in dispersal traits. To address this gap, we propose several future research directions that would advance our understanding of the interplay between spatiotemporal variability and dispersal trait variation in shaping the dynamics of current and future species ranges.
Expanding on the phenotypic spectrum ofWoodhouse‐Sakatisyndrome due to founder pathogenic variant inDCAF17: Report of 58 additional patients from Qatar and literature review
Authors: Ali Rehab , Al‐Dewik Nader , Mohammed Shayma , Elfituri Mahmud , Agouba Sahar , Musa Sara , Mahmoud Laila , Almulla Mariam , El‐Akouri Karen , Mohd Howaida , Bux Reem , Almulla Hajer , Othman Amna , Al‐Mesaifri Fatma , Shahbeck Noora , Al‐Muriekhi Mariam , Khalifa Amal , Al‐Sulaiman Reem , Ben‐Omran Tawfeg 
Published: September 2021

AbstractWoodhouse‐Sakati syndrome (WSS) is a rare autosomal recessive neuroendocrine and ectodermal disorder caused by variants in theDCAF17gene. In Qatar, the c.436delC variant has been reported as a possible founder pathogenic variant with striking phenotypic heterogeneity. In this retrospective study, we report on the clinical and molecular characteristics of additional 58 additional Qatari patients with WSS and compare them to international counterparts' findings. A total of 58 patients with WSS from 32 consanguineous families were identified. Ectodermal and endocrine (primary hypogonadism) manifestations were the most common presentations (100%), followed by diabetes mellitus (46%) and hypothyroidism (36%). Neurological manifestations were overlapping among patients with intellectual disability (ID) being the most common (75%), followed by sensorineural hearing loss (43%) and both ID and aggressive behavior (10%). Distinctive facial features were noted in all patients and extrapyramidal manifestations were uncommon (8.6%). This study is the largest to date on Qatari patients with WSS and highlights the high incidence and clinical heterogeneity of WSS in Qatar due to a founder variant c.436delC in theDCAF17gene. Early suspicion of WSS among Qatari patients with hypogonadism and ID, even in the absence of other manifestations, would shorten the diagnostic odyssey, guide early and appropriate management, and avoid potential complications.
Extending the clinical phenotype of SPTAN1: From DEE5 to migraine, epilepsy, and subependymal heterotopias without intellectual disability
Authors: Marco Hernández Ana Victoria , Caro Alfonso , Montoya Filardi Alejandro , Tomás Vila Miguel , Monfort Sandra , Beseler Soto Beatriz , Nieto‐Barceló Juan José , Martínez Francisco 
Published: September 2021

AbstractMutations in SPTAN1 gene, encoding the nonerythrocyte αII‐spectrin, are responsible for a severe developmental and epileptic encephalopathy (DEE5) and a wide spectrum of neurodevelopmental disorders, as epilepsy with or without intellectual disability (ID) or ID with cerebellar syndrome. A certain genotype–phenotype correlation has been proposed according to the type and location of the mutation. Herein, we report three novel cases with de novo SPTAN1 mutations, one of them associated to a mild phenotype not previously described. They range from (1) severe developmental encephalopathy with ataxia and a mild cerebellar atrophy, without epilepsy; (2) moderate intellectual disability, severe language delay, ataxia and tremor; (3) normal intelligence, chronic migraine, and generalized tonic–clonic seizures. Remarkably, all these patients showed brain MRI abnormalities, being of special interest the subependymal heterotopias detected in the latter patient. Thus we extend the SPTAN1‐related phenotypic spectrum, both in its radiological and clinical involvement. Furthermore, after systematic analysis of all the patients so far reported, we noted an excess of male versus female patients (20:9, p = 0.04), more pronounced among the milder phenotypes. Consequently, some protection factor might be suspected among female carriers, which if confirmed should be considered when establishing the pathogenicity of milder genetic variants in this gene.
Hydroxychloroquine for Primary Progressive Multiple Sclerosis
Authors: Koch Marcus W. , Kaur Sharanjit , Sage Kayla , Kim Janet , Levesque‐Roy Myriam , Cerchiaro Graziela , Yong Voon Wee , Cutter Gary R. , Metz Luanne M. 
Published: September 2021

ObjectivePrimary progressive multiple sclerosis (PPMS) does not respond well to immunomodulatory or immunosuppressive treatment. Chronic activation of microglia has been implicated in the pathophysiology of PPMS. The antimalarial drug hydroxychloroquine (HCQ) reduces the activity of human microglia and has neuroprotective effects in vitro.MethodsWe conducted a single‐arm, phase II futility trial of 200 mg oral HCQ twice daily for 18 months. In an effort to investigate disability worsening in the absence of overt focal inflammation, we excluded participants with contrast enhancing lesions on a screening magnetic resonance imaging (MRI). The primary end point was ≥20% worsening on the timed 25‐foot walk measured between 6 and 18 months of follow‐up.ResultsBased on original trial data, 40% of the cohort were expected to worsen. We used a Simon 2‐stage design to compare a null hypothesis of 40% of the cohort worsening against the one‐sided alternative of 20%. Using a 5% type 1 error rate and 80% power, HCQ treatment would be deemed successful if fewer than 10 of 35 participants experienced clinically significant worsening. The study met its primary end point, as only 8 of 35 participants worsened between 6 and 18 months. HCQ was overall well‐tolerated, with adverse events in 82% and serious adverse events in 12% of participants. All serious adverse events were unlikely related to HCQ use.InterpretationHCQ treatment was associated with reduced disability worsening in people with PPMS. HCQ is a promising treatment candidate in PPMS and should be investigated further in randomized controlled clinical trials. ANN NEUROL 2021;90:940–948