bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cardiovascular involvement in alpha-n-acetyl neuraminidase deficiency syndromes (sialidosis type I and II)
Authors: Prasanna Priyanka , Sriram Chenni S. , Rodriguez Sarah H. , Kohli Utkarsh 
Published: January 2021

AbstractSialidosis, a rare autosomal recessive disorder, is caused by a deficiency of NEU1 encoded enzyme alpha-N-acetyl neuraminidase. We report a premature male with neonatal-onset type II sialidosis which was associated with left ventricular dysfunction. The clinical presentation and subsequent progression which culminated in his untimely death at 16 months of age are succinctly described. Early-onset cardiovascular involvement as noted in this patient is not well characterised. The case report is supplemented by a comprehensive review of the determinants, characteristics, and the clinical course of cardiovascular involvement in this rare condition.
Changes in socio-economic inequality in nutritional status among children in EAG states, India
Authors: Kumar Rajeev , Paswan Balram 
Published: January 2021

AbstractObjective:The primary purpose of this study is to examine changes in socio-economic inequality in nutritional status (stunting and underweight) among children in Empowered Action Group (EAG) states.Design:The study is based on the most recent two wave’s cross-sectional data from the National Family Health Survey (NFHS) conducted in 2005–2006 (NFHS-3) and 2015–2016 (NFHS-4). The study used height-for-age (stunting) and weight-for-age (underweight) of children as anthropometric indicators.Setting:EAG states including Bihar, Chhattisgarh, Jharkhand, Madhya Pradesh, Odisha, Rajasthan, Uttarakhand and Uttar Pradesh in India.Participants:The study includes a total of 11 858 (NFHS-3) and 92 630 (NFHS-4) children under 5 years of age.Result:The socio-economic inequality in stunting remained unchanged in all EAG states. At the same time, the inequality in underweight decreased during 2005–2016. On decomposing, the factors contributing to socio-economic inequality revealed that household wealth index, mother’s education and mother’s nutritional status were the largest contributors to stunting (47 %, 24 % and 8 %) and underweight (51 %, 21 % and 16 %), respectively, in 2015–2016.Conclusion:The study concluded the socio-economic inequality in underweight among children under 5 years of age increased over the years in EAG states in India. Altogether, household wealth index, mother’s education and mother’s nutritional status contributed to nearly 80 % to inequality in stunting and 90 % to inequality in underweight in 2015–2016. Hence, efforts should be made to minimise the socio-economic inequality in the nutritional status of children, particularly in EAG states in India.
Resistance to bixlozone and clomazone in cross-resistant rigid ryegrass (Lolium rigidum) populations from southern Australia
Authors: Brunton David J. , Gill Gurjeet , Preston Christopher 
Published: January 2021

AbstractThree resistant (R) rigid ryegrass (Lolium rigidum Gaudin) populations from southern Australia (EP162, 375-14, and 198-15) with cross-resistance to thiocarbamate, chloroacetamide, and sulfonylisoxazoline herbicides displayed reduced sensitivity to the isoxazolidinone herbicides bixlozone and clomazone. Each of these R populations was exposed to two cycles of recurrent selection (RS) in which plants were treated with the field rate of bixlozone, survivors were bulk crossed, and seed was collected. After the first cycle of recurrent selection (RS1), the LD50 to bixlozone in population 198-15 increased to 17.5-fold compared with the S population and increased further to 26.9-fold after a second cycle of recurrent selection (RS2). The recurrent selection process also increased the level of resistance to bixlozone in populations EP162 and 375-14 (7.8- to 18.4-fold) compared with the S population. Phorate antagonized bixlozone and clomazone in SLR4 (34.6- and 28.1-fold increase in LD50) and both herbicides in populations EP162 (36.5- to 46.6-fold), 375-14 (71.4- to 73.9-fold), and 198-15 (86.4- to 91.5-fold) compared with the absence of phorate. The increase in LD50 of all L. rigidum RS populations when treated with phorate suggests a lack of herbicide activation is not the likely resistance mechanism to these herbicides. This research highlights the elevated risk of thiocarbamate-resistant L. rigidum populations to rapidly evolve resistance to the isoxazolidinone herbicides bixlozone and clomazone.
Segment‐Scale Seismicity of the Ultraslow Spreading Knipovich Ridge
Authors: Meier Michaela , Schlindwein Vera , Scholz John‐Robert , Geils Jonah , Schmidt‐Aursch Mechita C. , Krüger Frank , Czuba Wojciech , Janik Tomasz 
Published: January 2021

AbstractUltraslow spreading ridges form the slowest divergent plate boundaries and exhibit distinct spreading processes in volcanically active magmatic sections and intervening amagmatic sections. Local seismicity studies of ultraslow spreading ridges until now cover only parts of segments and give insight into spreading processes at confined locations. Here, we present a microseismicity data set that allows to study spreading processes on the scale of entire segments. Our network of 26 ocean bottom seismometers covered around 160 km along axis of the ultraslow spreading Knipovich Ridge in the Greenland Sea and recorded earthquakes for a period of about 1 year. We find seismicity varying distinctly along‐axis. The maximum earthquake depths shallow over distances of 70 km toward the Logachev volcanic center. Here, swarm activity occurs in an otherwise aseismic zone. Melts may thus be guided along the subparallel topography of the lithosphere‐asthenosphere boundary toward major volcanic centers explaining the uneven along‐axis melt distribution typical for ultraslow ridges. Absence of shallow seismicity in the upper 8 km of the lithosphere with a band of deep seismicity underneath offsets presumably melt‐poor regions from magma richer sections. Aseismic deformation in these regions may indicate weakening of mantle rocks by alteration. We do not find obvious indications for major detachment faulting that characterizes magma‐poor spreading at some ultraslow spreading segments. The highly oblique spreading of Knipovich Ridge may be the reason for a fine‐scale segmentation of the seismic activity with zones of weak seismicity possibly indicating transform motion on short obliquely oriented faults.
Echocardiographic assessment of fetal cardiac function in the uterine artery ligation rat model of IUGR
Authors: Dai Yichen , Zhao Dan , Chen Ching Kit , Yap Choon Hwai 
Published: January 2021

Abstract
Background
Intrauterine growth restriction (IUGR) leads to cardiac dysfunction and adverse remodeling of the fetal heart, as well as a higher risk of postnatal cardiovascular diseases. The rat model of IUGR, via uterine artery ligation, is a popular model but its cardiac sequelae is not well investigated. Here, we performed an echocardiographic evaluation of its cardiac function to determine how well it can represent the disease in humans.

Methods
Unilateral uterine artery ligation was performed at embryonic day 17 (E17) and echocardiography was performed at E19 and E20.

Results
Growth-restricted fetuses were significantly smaller and lighter, and had an higher placenta-to-fetus weight ratio. Growth-restricted fetal hearts had reduced wall thickness-to-diameter ratio, indicating left ventricular (LV) dilatation, and they had elevated trans-mitral and trans-tricuspid E/A ratios and reduced left and right ventricular fractional shortening (FS), suggesting systolic and diastolic dysfunction. These were similar to human IUGR fetuses. However, growth-restricted rat fetuses did not demonstrate head-sparing effect, displayed a lower LV myocardial performance index, and ventricular outflow velocities were not significantly reduced, which were dissimilar to human IUGR fetuses.

Conclusions
Despite the differences, our results suggest that this IUGR model has significant cardiac dysfunction, and could be a suitable model for studying IUGR cardiovascular physiology.

Impact

Animal models of IUGR are useful, but their fetal cardiac function is not well studied, and it is unclear if they can represent human IUGR fetuses.

We performed an echocardiographic assessment of the heart function of a fetal rat model of IUGR, created via maternal uterine artery ligation.

Similar to humans, the model displayed LV dilatation, elevated E/A ratios, and reduced FS.

Different from humans, the model displayed reduced MPI, and no significant outflow velocity reduction.

Despite differences with humans, this rat model still displayed cardiac dysfunction and is suitable for studying IUGR cardiovascular physiology.
The use of hyperpolarised 13C-MRI in clinical body imaging to probe cancer metabolism
Authors: Woitek Ramona , Gallagher Ferdia A. 
Published: January 2021

AbstractMetabolic reprogramming is one of the hallmarks of cancer and includes the Warburg effect, which is exhibited by many tumours. This can be exploited by positron emission tomography (PET) as part of routine clinical cancer imaging. However, an emerging and alternative method to detect altered metabolism is carbon-13 magnetic resonance imaging (MRI) following injection of hyperpolarised [1-13C]pyruvate. The technique increases the signal-to-noise ratio for the detection of hyperpolarised13C-labelled metabolites by several orders of magnitude and facilitates the dynamic, noninvasive imaging of the exchange of13C-pyruvate to13C-lactate over time. The method has produced promising preclinical results in the area of oncology and is currently being explored in human imaging studies. The first translational studies have demonstrated the safety and feasibility of the technique in patients with prostate, renal, breast and pancreatic cancer, as well as revealing a successful response to treatment in breast and prostate cancer patients at an earlier stage than multiparametric MRI. This review will focus on the strengths of the technique and its applications in the area of oncological body MRI including noninvasive characterisation of disease aggressiveness, mapping of tumour heterogeneity, and early response assessment. A comparison of hyperpolarised13C-MRI with state-of-the-art multiparametric MRI is likely to reveal the unique additional information and applications offered by the technique.
Dupilumab-associated ocular surface disease: presentation, management and long-term sequelae
Authors: Popiela Magdalena Z. , Barbara Ramez , Turnbull Andrew M. J. , Corden Emma , Martinez-Falero Beatriz Suarez , O’Driscoll Daniel , Ardern-Jones Michael R. , Hossain Parwez N. 
Published: January 2021

Abstract
Objectives
To determine the presenting features of ocular surface disease in patients with atopic dermatitis (AD) treated with dupilumab at a tertiary, university hospital. To establish the need for treatment of dupilumab-associated ocular surface disease and report any long-term effects on the ocular surface.

Methods
A retrospective analysis of consecutive patients treated with dupilumab for AD between January 2017 and August 2019 was undertaken. Data were collected on demographics, incidence and type of ocular disease features, natural history and treatment.

Results
A total of 50% (14/28) patients developed ocular symptoms with a mean time of onset of 6.75 (±6.1) weeks from starting dupilumab. Of these, 69% (9/13) were diagnosed with conjunctivitis associated with cicatrisation in two patients and periorbital skin changes in four. Of these nine, four had prior history of atopic keratoconjunctivitis. All were treated with topical steroids; two required additional ciclosporin drops. In all, 67% (6/9) patients went on to have on-going ocular inflammation requiring maintenance drops at a mean of 16 (±6.9) months of follow-up. All patients had improvement in their AD severity; only one patient discontinued dupilumab due to ocular side effects.

Conclusion
The rate of dupilumab-associated ocular surface disease was 32%. Periorbital skin changes and conjunctival cicatrisation were noted in association with conjunctivitis. Ocular surface disease improved on topical steroids and ciclosporin but 67% of patients needed on-going treatment. Close liaison with an ophthalmologist should be considered in those patients who develop conjunctivitis or have a past history of severe ocular surface disease.
Genome-wide association study identifies risk loci for progressive chronic lymphocytic leukemia
Authors: Lin Wei-Yu , Fordham Sarah E. , Sunter Nicola , Elstob Claire , Rahman Thahira , Willmore Elaine , Shepherd Colin , Strathdee Gordon , Mainou-Fowler Tryfonia , Piddock Rachel , Mearns Hannah , Barrow Timothy , Houlston Richard S. , Marr Helen , Wallis Jonathan , Summerfield Geoffrey , Marshall Scott , Pettitt Andrew , Pepper Christopher , Fegan Christopher , Forconi Francesco , Dyer Martin J. S. , Jayne Sandrine , Sellors April , Schuh Anna , Robbe Pauline , Oscier David , Bailey James , Rais Syed , Bentley Alison , Cawkwell Lynn , Evans Paul , Hillmen Peter , Pratt Guy , Allsup David J. , Allan James M. 
Published: January 2021

AbstractPrognostication in patients with chronic lymphocytic leukemia (CLL) is challenging due to heterogeneity in clinical course. We hypothesize that constitutional genetic variation affects disease progression and could aid prognostication. Pooling data from seven studies incorporating 842 cases identifies two genomic locations associated with time from diagnosis to treatment, including 10q26.13 (rs736456, hazard ratio (HR) = 1.78, 95% confidence interval (CI) = 1.47–2.15; P = 2.71 × 10−9) and 6p (rs3778076, HR = 1.99, 95% CI = 1.55–2.55; P = 5.08 × 10−8), which are particularly powerful prognostic markers in patients with early stage CLL otherwise characterized by low-risk features. Expression quantitative trait loci analysis identifies putative functional genes implicated in modulating B-cell receptor or innate immune responses, key pathways in CLL pathogenesis. In this work we identify rs736456 and rs3778076 as prognostic in CLL, demonstrating that disease progression is determined by constitutional genetic variation as well as known somatic drivers.
Measuring the frequency chirp of extreme-ultraviolet free-electron laser pulses by transient absorption spectroscopy
Authors: Ding Thomas , Rebholz Marc , Aufleger Lennart , Hartmann Maximilian , Stooß Veit , Magunia Alexander , Birk Paul , Borisova Gergana Dimitrova , Wachs David , da Costa Castanheira Carina , Rupprecht Patrick , Mi Yonghao , Attar Andrew R. , Gaumnitz Thomas , Loh Zhi-Heng , Roling Sebastian , Butz Marco , Zacharias Helmut , Düsterer Stefan , Treusch Rolf , Eislage Arvid , Cavaletto Stefano M. , Ott Christian , Pfeifer Thomas 
Published: January 2021

AbstractHigh-intensity ultrashort pulses at extreme ultraviolet (XUV) and x-ray photon energies, delivered by state-of-the-art free-electron lasers (FELs), are revolutionizing the field of ultrafast spectroscopy. For crossing the next frontiers of research, precise, reliable and practical photonic tools for the spectro-temporal characterization of the pulses are becoming steadily more important. Here, we experimentally demonstrate a technique for the direct measurement of the frequency chirp of extreme-ultraviolet free-electron laser pulses based on fundamental nonlinear optics. It is implemented in XUV-only pump-probe transient-absorption geometry and provides in-situ information on the time-energy structure of FEL pulses. Using a rate-equation model for the time-dependent absorbance changes of an ionized neon target, we show how the frequency chirp can be directly extracted and quantified from measured data. Since the method does not rely on an additional external field, we expect a widespread implementation at FELs benefiting multiple science fields by in-situ on-target measurement and optimization of FEL-pulse properties.
Dynamics of genome architecture and chromatin function during human B cell differentiation and neoplastic transformation
Authors: Vilarrasa-Blasi Roser , Soler-Vila Paula , Verdaguer-Dot Núria , Russiñol Núria , Di Stefano Marco , Chapaprieta Vicente , Clot Guillem , Farabella Irene , Cuscó Pol , Kulis Marta , Agirre Xabier , Prosper Felipe , Beekman Renée , Beà Silvia , Colomer Dolors , Stunnenberg Hendrik G. , Gut Ivo , Campo Elias , Marti-Renom Marc A. , Martin-Subero José Ignacio 
Published: January 2021

AbstractTo investigate the three-dimensional (3D) genome architecture across normal B cell differentiation and in neoplastic cells from different subtypes of chronic lymphocytic leukemia and mantle cell lymphoma patients, here we integrate in situ Hi-C and nine additional omics layers. Beyond conventional active (A) and inactive (B) compartments, we uncover a highly-dynamic intermediate compartment enriched in poised and polycomb-repressed chromatin. During B cell development, 28% of the compartments change, mostly involving a widespread chromatin activation from naive to germinal center B cells and a reversal to the naive state upon further maturation into memory B cells. B cell neoplasms are characterized by both entity and subtype-specific alterations in 3D genome organization, including large chromatin blocks spanning key disease-specific genes. This study indicates that 3D genome interactions are extensively modulated during normal B cell differentiation and that the genome of B cell neoplasias acquires a tumor-specific 3D genome architecture.
A small molecule compound with an indole moiety inhibits the main protease of SARS-CoV-2 and blocks virus replication
Authors: Hattori Shin-ichiro , Higashi-Kuwata Nobuyo , Hayashi Hironori , Allu Srinivasa Rao , Raghavaiah Jakka , Bulut Haydar , Das Debananda , Anson Brandon J. , Lendy Emma K. , Takamatsu Yuki , Takamune Nobutoki , Kishimoto Naoki , Murayama Kazutaka , Hasegawa Kazuya , Li Mi , Davis David A. , Kodama Eiichi N. , Yarchoan Robert , Wlodawer Alexander , Misumi Shogo , Mesecar Andrew D. , Ghosh Arun K. , Mitsuya Hiroaki 
Published: January 2021

AbstractExcept remdesivir, no specific antivirals for SARS-CoV-2 infection are currently available. Here, we characterize two small-molecule-compounds, named GRL-1720 and 5h, containing an indoline and indole moiety, respectively, which target the SARS-CoV-2 main protease (Mpro). We use VeroE6 cell-based assays with RNA-qPCR, cytopathic assays, and immunocytochemistry and show both compounds to block the infectivity of SARS-CoV-2 with EC50 values of 15 ± 4 and 4.2 ± 0.7 μM for GRL-1720 and 5h, respectively. Remdesivir permitted viral breakthrough at high concentrations; however, compound 5h completely blocks SARS-CoV-2 infection in vitro without viral breakthrough or detectable cytotoxicity. Combination of 5h and remdesivir exhibits synergism against SARS-CoV-2. Additional X-ray structural analysis show that 5h forms a covalent bond with Mpro and makes polar interactions with multiple active site amino acid residues. The present data suggest that 5h might serve as a lead Mpro inhibitor for the development of therapeutics for SARS-CoV-2 infection.
Phenylalkylammonium passivation enables perovskite light emitting diodes with record high-radiance operational lifetime: the chain length matters
Authors: Guo Yuwei , Apergi Sofia , Li Nan , Chen Mengyu , Yin Chunyang , Yuan Zhongcheng , Gao Feng , Xie Fangyan , Brocks Geert , Tao Shuxia , Zhao Ni 
Published: January 2021

AbstractPerovskite light emitting diodes suffer from poor operational stability, exhibiting a rapid decay of external quantum efficiency within minutes to hours after turn-on. To address this issue, we explore surface treatment of perovskite films with phenylalkylammonium iodide molecules of varying alkyl chain lengths. Combining experimental characterization and theoretical modelling, we show that these molecules stabilize the perovskite through suppression of iodide ion migration. The stabilization effect is enhanced with increasing chain length due to the stronger binding of the molecules with the perovskite surface, as well as the increased steric hindrance to reconfiguration for accommodating ion migration. The passivation also reduces the surface defects, resulting in a high radiance and delayed roll-off of external quantum efficiency. Using the optimized passivation molecule, phenylpropylammonium iodide, we achieve devices with an efficiency of 17.5%, a radiance of 1282.8 W sr−1 m−2 and a record T50 half-lifetime of 130 h under 100 mA cm−2.
LPA signaling acts as a cell-extrinsic mechanism to initiate cilia disassembly and promote neurogenesis
Authors: Hu Huai-Bin , Song Zeng-Qing , Song Guang-Ping , Li Sen , Tu Hai-Qing , Wu Min , Zhang Yu-Cheng , Yuan Jin-Feng , Li Ting-Ting , Li Pei-Yao , Xu Yu-Ling , Shen Xiao-Lin , Han Qiu-Ying , Li Ai-Ling , Zhou Tao , Chun Jerold , Zhang Xue-Min , Li Hui-Yan 
Published: January 2021

AbstractDynamic assembly and disassembly of primary cilia controls embryonic development and tissue homeostasis. Dysregulation of ciliogenesis causes human developmental diseases termed ciliopathies. Cell-intrinsic regulatory mechanisms of cilia disassembly have been well-studied. The extracellular cues controlling cilia disassembly remain elusive, however. Here, we show that lysophosphatidic acid (LPA), a multifunctional bioactive phospholipid, acts as a physiological extracellular factor to initiate cilia disassembly and promote neurogenesis. Through systematic analysis of serum components, we identify a small molecular—LPA as the major driver of cilia disassembly. Genetic inactivation and pharmacological inhibition of LPA receptor 1 (LPAR1) abrogate cilia disassembly triggered by serum. The LPA-LPAR-G-protein pathway promotes the transcription and phosphorylation of cilia disassembly factors-Aurora A, through activating the transcription coactivators YAP/TAZ and calcium/CaM pathway, respectively. Deletion of Lpar1 in mice causes abnormally elongated cilia and decreased proliferation in neural progenitor cells, thereby resulting in defective neurogenesis. Collectively, our findings establish LPA as a physiological initiator of cilia disassembly and suggest targeting the metabolism of LPA and the LPA pathway as potential therapies for diseases with dysfunctional ciliogenesis.
Observing the quantum Cheshire cat effect with noninvasive weak measurement
Authors: Kim Yosep , Im Dong-Gil , Kim Yong-Su , Han Sang-Wook , Moon Sung , Kim Yoon-Ho , Cho Young-Wook 
Published: January 2021

AbstractOne of the common conceptions of nature, typically derived from the experiences with classical systems, is that attributes of the matter coexist with the substance. In the quantum regime, however, the quantum particle itself and its physical property may be in spatial separation, known as the quantum Cheshire cat effect. While there have been several reports to date on the observation of the quantum Cheshire cat effect, all such experiments are based on first-order interferometry and destructive projection measurement, thus allowing simple interpretation due to measurement-induced disturbance and also subject to trivial interpretation based on classical waves. In this work, we report an experimental observation of the quantum Cheshire cat effect with noninvasive weak quantum measurement as originally proposed. The use of the weak-measurement probe has allowed us to identify the location of the single photon and that of the disembodied polarization state in a quantum interferometer. The weak-measurement probe based on two-photon interference makes our observation unable to be explained by classical physics. We furthermore elucidate the quantum Cheshire cat effect as quantum interference of the transition amplitudes for the photon and the polarization state which are directly obtained from the measurement outcomes or the weak values. Our work not only reveals the true quantum nature of Cheshire cat effect but also sheds light on a comprehensive understanding for the counter-intuitive quantum phenomena.