bluuebunny/crossref_metadata_2025_split huggingface dataset:
A Modified Progressive Supranuclear Palsy Rating Scale
Authors: Grötsch Marie‐Therese , Respondek Gesine , Colosimo Carlo , Compta Yaroslau , Corvol Jean Christophe , Ferreira Joaquim , Huber Meret Koroni , Klietz Martin , Krey Lea F.M. , Levin Johannes , Jecmenica‐Lukic Milica , Macías‐García Daniel , Meissner Wassilios G. , Mir Pablo , Morris Huw , Nilsson Christer , Rowe James B. , Seppi Klaus , Stamelou Maria , van Swieten John C. , Wenning Gregor , Del Ser Teodoro , Golbe Lawrence I. , Höglinger Günter U. ,   for the Describe PSP Study Group, the ProPSP Study Group, and the Movement Disorder Society–Endorsed PSP Study Group
Published: January 2021

ABSTRACTBackgroundThe Progressive Supranuclear Palsy Rating Scale is a prospectively validated physician‐rated measure of disease severity for progressive supranuclear palsy. We hypothesized that, according to experts' opinion, individual scores of items would differ in relevance for patients' quality of life, functionality in daily living, and mortality. Thus, changes in the score may not equate to clinically meaningful changes in the patient's status.ObjectiveThe aim of this work was to establish a condensed modified version of the scale focusing on meaningful disease milestones.MethodsSixteen movement disorders experts evaluated each scale item for its capacity to capture disease milestones (0 = no, 1 = moderate, 2 = severe milestone). Items not capturing severe milestones were eliminated. Remaining items were recalibrated in proportion to milestone severity by collapsing across response categories that yielded identical milestone severity grades. Items with low sensitivity to change were eliminated, based on power calculations using longitudinal 12‐month follow‐up data from 86 patients with possible or probable progressive supranuclear palsy.ResultsThe modified scale retained 14 items (yielding 0–2 points each). The items were rated as functionally relevant to disease milestones with comparable severity. The modified scale was sensitive to change over 6 and 12 months and of similar power for clinical trials of disease‐modifying therapy as the original scale (achieving 80% power for two‐sample t test to detect a 50% slowing with n = 41 and 25% slowing with n = 159 at 12 months).ConclusionsThe modified Progressive Supranuclear Palsy Rating Scale may serve as a clinimetrically sound scale to monitor disease progression in clinical trials and routine. © 2021 International Parkinson and Movement Disorder Society
A novel Xp11.22–22.33 deletion suggesting a possible mechanism of congenital cervical spinal muscular atrophy
Authors: Liu Jingwei , Wang Kelai , Li Baomin , Yang Xiaofan 
Published: January 2021

AbstractBackgroundCongenital cervical spinal muscular atrophy (CCSMA) is a rare, nonprogressive, neurogenic disorder characterized by symmetric arthrogryposis and motor deficits mainly confined to upper extremities. Since its first proposal by Darwish et al. 39 years ago, only few cases have ever been reported. Vascular insult to the anterior horn of cervical spinal cord during fetal development was speculated to be the cause, however, the exact pathogenesis is still not well understood.MethodsIn this study, whole‐exome sequencing (WES) and copy number variation (CNV) analysis were conducted on a definitive CCSMA patient, confirmed by the clinical manifestations and other supplementary examinations.ResultsOn physical examination, the patient was mainly characterized by symmetric, congenital, nonprogressive contractures, hypotonia, and muscle weakness mainly confined to the upper limbs, which were further supported by MRI and electromyography. Neuromuscular biopsy of the deltoid muscle demonstrated the type 1 myofiber predominance without any infiltration of inflammatory cells. The WES and CNV analysis unveiled a de novo Xp11.22–22.33 deletion. On further examination of the genes contained within this segment, we recognize UBA1 gene as the most likely pathogenic gene. Ubiquitin‐like modifier activating enzyme 1 is encoded by UBA1 gene (MIM 314370) located in Xp11.3 and is a critical protein that plays a vital role in ubiquitin‐proteasome system and autophagy. It is well documented that UBA1 gene mutation causes X‐linked infantile spinal muscular atrophy (XL‐SMA), which manifests phenotypes of arthrogryposis, hypotonia, and myopathic face. Type 2 XL‐SMA, which follows a nonprogressive and nonlethal course is very similar to the presentations of CCSMA.ConclusionThe phenotypic similarities between this CCSMA case and XL‐SMA prompt us to hypothesize a possible connection between UBA1 gene deficit and the pathogenesis of CCSMA. Our study is the first to demonstrate that CCSMA might have a genetic etiology, thus, expanding our insights into the underlying cause of CCSMA.
Straying and Life History of Adult Steelhead in a Small California Coastal Stream Revealed by Otolith Natural Tags and Genetic Stock Identification
Authors: Donohoe Christopher J. , Rundio David E. , Pearse Devon E. , Williams Thomas H. 
Published: January 2021

Abstract
Straying has been difficult to study directly in natural steelhead Oncorhynchus mykiss populations. We analyzed an opportunistic sample of seven adult steelhead from a small basin on the Big Sur coast of California to determine their life history traits, including whether they were strays. Otolith natural tags (87Sr/86Sr) and genetic stock identification indicated that all seven adults were strays from at least six different sources. Three adults strayed from nearby streams (&lt;72 km) on the Big Sur coast, while three strayed from distant sources, including the Klamath River (680 km to the north); the source of one stray could not be identified. Six strays were progeny of steelhead mothers, but one was the offspring of a nonanadromous mother. Six were female and one was male, and all that could be genotyped were homozygous (n = 4) or heterozygous (n = 2) for the anadromy-associated haplotype in a migration-associated genomic region. While the opportunistic nature and small size of the sample prevents us from inferring the rate of straying into the basin, our study nonetheless demonstrates that steelhead may stray across greater distances than generally has been appreciated and that nonanadromous females can produce anadromous offspring that stray, thus providing connectivity among basins.
Wet‐chemistry approach for the synthesis of single phase ferromagnetic Co3C nanoparticle
Authors: Shen Xianfeng , Ma Chenwei , Suo HaiYun , Zhang Tianfu , Yan Lai , Huang Lichun , Zhou Jianqiang , Wen Xiaodong , Li Yongwang , Yang Yong 
Published: January 2021

AbstractCobalt carbides nanoparticles have great potential as novel materials in magnetic fields. However, it is difficult to synthesize single phase cobalt carbides, especially Co3C. In this paper, we propose a one‐pot wet‐chemistry approach to synthesize single phase ferromagnetic Co3C without using any alkali as nucleation solvent. The particle size of Co3C nanoparticle is about 3 nm. The magnetic properties of Co3C nanoparticles are measured at 300 K from –30 to 30 kOe. Compared with Co2C, Co3C exhibits a large saturation magnetization of 46.1 emu g–1 and a high coercivity of 1.5 kOe. Small amount of carbon and small particle size of the Co3C nanoparticle are believed to contribute to large magnetization and high coercivity. Magnetic measurements also reveal the spin glass behavior caused by the small Co3C nanoparticle at the spin freezing temperature of 43 K. This work provides a facile method to synthesize single phase Co3C nanoparticle which is applicable in magnetic field.
Detection and evaluation of the fibers' deposition parameters during wet filament winding
Authors: Hopmann Christian , Magura Nadine , Rozo Lopez Nicolas , Schneider Daniel , Fischer Kai 
Published: January 2021

AbstractWet filament winding technology has been extensively used for the manufacture of rotationally symmetric parts made of fiber‐reinforced plastics (FRP). As the design and modeling of FRP‐parts require numerous assumptions, deviations between calculated and achieved mechanical properties are expected. One aspect that contributes to this discrepancy is the assumption of a homogeneous rectangular cross‐section of the fiber‐band. In this work, the fiber‐band geometry in a wet‐winding process of carbon fiber cylinders is analyzed. An Infrared‐optical system for the detection of the fiber bandwidth and winding‐angle is implemented. The influence of the winding speed and the resin temperature is analyzed. An image processing algorithm for the automatic measurement of the fiber's bandwidth and winding‐angle is developed. Manual and adaptive gamma corrections are implemented to improve image quality. A parameter study for the suitable selection of image processing parameters is performed.
Pseudomonas aeruginosa colonization in cystic fibrosis: Impact on neutrophil functions and cytokine secretion capacity
Authors: Aslanhan Umit , Cakir Erkan , Pur Ozyigit Leyla , Kucuksezer Umut Can , Gelmez Yusuf Metin , Yuksel Mine , Deniz Gunnur , Cetin Aktas Esin 
Published: January 2021

AbstractBackgroundChronic colonization with Pseudomonas (P.) aeruginosa worsens the prognosis of cystic fibrosis (CF) patients. This study aims to analyze the functional properties of neutrophils in CF patients with P. aeruginosa colonization.MethodsPatients with CF (n = 16) were grouped by positivity of P. aeruginosa in sputum culture, as positive (P.+) or negative (P.−), then compared with age and sex matched healthy controls (n = 8). Adhesion molecules, apoptotic index, intracellular CAP‐18, interleukin 8 (IL‐8), and tumor necrosis factor α (TNF‐α) levels of neutrophils, following P. aeruginosa and lipopolysaccharides (LPS) stimulation, were analyzed by flow cytometry. IL‐1β, IL‐6, TNF‐α, and IL‐17 plasma levels were determined by Luminex.ResultsPatients with CF had increased phagocytosis of Escherichia coli and P. aeruginosa, upregulated oxidative burst and chemotaxis. Increased neutrophil apoptosis was noted in CF patients. In unstimulated conditions, higher levels of CD16+TNF‐α+ and CD16+IL‐8+ neutrophils were determined, whereas bacteria and LPS stimulation significantly decreased secretion of CAP‐18 from CD16+ neutrophils of CF patients. Plasma levels of IL‐1β, TNF‐α and IL‐17 in P.+ patients were higher than in P.− group.ConclusionOur findings confirm inadequate neutrophil defense towards pathogens in CF. A significant difference in migration, phagocytosis, oxidative burst, percentage of IL‐8 producing neutrophils, IL‐1β, TNF‐α, and IL‐17 secretions were noted among CF patients according to their colonization status, which might induce a further destructive effect on airways, resulting in an unfavorable prognosis for children with CF who also have colonization.
Screening of depression and anxiety in adolescents with cystic fibrosis and caregivers in Turkey by PHQ‐9 and GAD‐7 questionnaires
Authors: Mursaloğlu H. Hakan , Yılmaz Yeğit Cansu , Ergenekon Almala P. , Gökdemir Yasemin , Eralp Ela E. , Karakoç Fazilet , Nasr Samya Z. , Karadağ Bulent T. 
Published: January 2021

AbstractBackgroundDepression and anxiety symptoms in patients with cystic fibrosis (CF) and their caregivers are 2–3 times higher than in the normal population. This study aims to evaluate the frequency and severity of depression and anxiety symptoms and to determine possible risk factors in CF patients and their mother and/or fathers at Marmara University CF center.MethodsThe study included 132 CF patients who were followed up at our CF center. Patient Health Questionnaire (PHQ‐9) and the Generalized Anxiety Disorder Questionnaire (GAD‐7) were used to screen depression and anxiety. The questionnaires were completed by 50 CF patients (aged 12–17 years) and 132 parents of patients (aged 0–17 years).ResultsWhile moderate to severe depressive symptoms were seen in 26% of patients, 33.7% of mothers and 14.6% of fathers; moderate to severe anxiety were present in 18%, 21.8% and 8.5%, respectively. None of the demographic characteristics was identified as a predictor of depression or anxiety. GAD‐7 scores have shown a higher prevalence of anxiety in mothers of patients with chronic methicillin‐resistant Staphylococcus aureus (p = .034). Additionally, hospitalization in the last 12 months was significantly correlated with higher PHQ‐9 scores in fathers (p = .043). Analysis of patients' adherence to medical treatment and airway clearance showed higher depression and anxiety in mothers of the nonadherent group (p = .002).ConclusionDepression and anxiety were common in CF patients and their parents. These results illustrate the importance of depression/anxiety screening and psychosocial support for the CF patient and their parents.
Outcomes of repeat sweat testing in cystic fibrosis newborn screen positive infants
Authors: Bauer Sarah E. , Wesson Melissa , Oles Sylwia K. , Ren Clement L. 
Published: January 2021

AbstractBackgroundInfants with a positive cystic fibrosis (CF) newborn screen, only one identified CFTR mutation (NBS+/1 mut), and an initial intermediate sweat chloride (30–59 mmol/L) should have repeat sweat chloride testing (SCT). However, the outcome of repeat SCT and the relationship between initial sweat Cl and subsequent CF diagnosis have not been reported.ObjectiveThe objective of this study was to analyze the outcomes of repeat SCT and subsequent CF diagnosis in NBS+/1 mut infants based on their initial sweat chloride concentration.MethodsWe retrospectively identified all infants born in Indiana from 2007 to 2017 with NBS+/1 mut and initial SCT in the intermediate range. For each infant, we recorded the initial and repeat SCT results and/or a final CF diagnosis.ResultsFrom 2007 through 2017 there were 2822 NBS+/1 mut infants of which 2613 (82%) had at least one SCT result. No infants with an initial SCT of 30–39 mmol/L were subsequently diagnosed with CF. Of the 31 infants with an initial SCT of 40–49 mmol/L, only 1 was subsequently diagnosed with CF. In contrast, 61% of those with SCTs of 50–59 mmol/L were later diagnosed with CF.ConclusionThese results suggest that infants with a positive NBS for CF and one CFTR mutationwhose initial sweat chloride concentration is 50–59 mmol/L need to be monitored more closely forCF with strong consideration for earlier repeat SCTs and immediate genotyping
Designed leucine‐rich repeat proteins bind two muramyl dipeptide ligands
Authors: Kim Christina S. , Brown Anne M. , Grove Tijana Z. , Etzkorn Felicia A. 
Published: January 2021

AbstractDesigned protein receptors hold diagnostic and therapeutic promise. We now report the design of five consensus leucine‐rich repeat proteins (CLRR4–8) based on the LRR domain of nucleotide‐binding oligomerization domain (NOD)‐like receptors involved in the innate immune system. The CLRRs bind muramyl dipeptide (MDP), a bacterial cell wall component, with micromolar affinity. The overall Kd app values ranged from 1.0 to 57 μM as measured by fluorescence quenching experiments. Biphasic fluorescence quenching curves were observed in all CLRRs, with higher affinity Kd1 values ranging from 0.04 to 4.5 μM, and lower affinity Kd2 values ranging from 3.1 to 227 μM. These biphasic binding curves, along with the docking studies of MDP binding to CLRR4, suggest that at least two MDPs bind to each protein. Previously, only single MDP binding was reported. This high‐capacity binding of MDP promises small, soluble, stable CLRR scaffolds as candidates for the future design of pathogen biosensors.
Numerical prediction of tropical cyclogenesis part I: Evaluation of model performance
Authors: Liang Mei , Chan Johnny C. L. , Xu Jianjun , Yamaguchi Munehiko 
Published: January 2021

AbstractThis is a two‐part study investigating the numerical prediction of tropical cyclogenesis. This paper (Part I) presents a comprehensive statistical assessment of the performance of the high‐resolution European Centre for Medium‐Range Weather Forecasts (ECMWF) deterministic forecast in predicting tropical cyclone (TC) genesis over the western North Pacific (WNP) between 2007 and 2018 using The International Grand Global Ensemble (TIGGE) data. Each genesis forecast at each lead time is classified into one of these categories: well predicted (WP), early formation (EF), late formation (LF), or failed prediction (FP). Based on the synoptic patterns at the actual genesis time, the genesis of a particular TC is grouped into one of five flow patterns: monsoon shear line (SL), monsoon confluence region (CR), monsoon gyre (GY), easterly wave (EW), and preexisting tropical cyclone (PTC). Any case that does not fall in one of these patterns is labeled as unclassified flow pattern (UCF). Overall, the prediction skill of SL cases is the highest, followed by CR, GY, and PTC, while the EW and UCF patterns have the lowest skill. No significant improvement in the model performance is found during the 12 years, probably partly because of the low percentage of cases with the SL pattern in the later years. The prediction skills of TC genesis in May, June, September, and October are the highest, while those in July, August, and November are the lowest. TCs that form further to the south are easier to predict in the SL pattern. In Part II, we will examine the model predictions of the dynamic and thermodynamic variables in the WP cases to identify the physical processes responsible for TC genesis.
Prediction of multiple soil fertility parameters using VisNIR spectroscopy and PXRF spectrometry
Authors: Liu Ya , Wang Changkun , Xiao Chenchao , Shang Kun , Zhang Yan , Pan Xianzhang 
Published: January 2021

AbstractVisible and near‐infrared (VisNIR) spectroscopy and portable X‐ray fluorescence (PXRF) spectrometry are successful in estimating soil attributes. However, it is still necessary to investigate their performance in estimating soil fertility properties. In this study, for the soil fertility properties of soil organic C (SOC), available N, total P, available P, total K, and available K, the performance of using VisNIR and PXRF individually and combined, three data fusion methods (concatenation, model fusion and model averaging), and two modeling methods was evaluated. The results showed that combining VisNIR and PXRF did not substantially improve the prediction accuracy of SOC, total P, total K and available K. Using VisNIR alone could predict them well, achieving the best prediction in total K (R2= .90, ratio of performance to interquartile distance [RPIQ] = 5.08, and residual prediction deviation [RPD] = 3.13), followed by SOC (R2= .77, RPIQ = 2.05, and RPD = 2.10), total P (R2= .63, RPIQ = 2.35, and RPD = 1.64), and available K (R2= .53, RPIQ = 2.04, and RPD = 1.41). Both available N and available P were poorly estimated by all the methods proposed in this study. Although PXRF alone could predict total P, total K, and available K with acceptable accuracy, it did not perform well on SOC (R2< .32, RPIQ < 1.16, and RPD < 1.19). Hence, for the precision application of organic and NPK fertilizers, VisNIR is supposed to be the optimal way to rapidly obtain soil information.
Probing the Thermal Stability of (3‐Mercaptopropyl)‐trimethoxysilane‐Protected Au25 Clusters by In Situ Transmission Electron Microscopy
Authors: Sudheeshkumar Veeranmaril , Soong Charles , Dogel Stas , Scott Robert W. J. 
Published: January 2021

AbstractHigh‐surface‐area gold catalysts are promising catalysts for a number of selective oxidation and reduction reactions but typically suffer catalyst deactivation at higher temperatures. The major reason for catalyst deactivation is sintering, which can be triggered via two mechanisms: particle migration and coalescence, and Ostwald ripening. Herein, a direct method to synthesize Au25 clusters stabilized with 3‐mercaptopropyltrimethoxysilane (MPTS) ligands is discussed. The sintering of Au25(MPTS)18 clusters on mesoporous silica (SBA‐15) is monitored by using an environmental in situ transmission electron microscopy (TEM) technique. Results show that agglomeration of smaller particles is accelerated by increased mobility of particles during heat treatment, while growth of immobile particles occurs via diffusion of atomic species from smaller particles. The mobility of the Au clusters can be alleviated by fabricating overlayers of silica around the clusters. The resulting materials show tremendous sinter‐resistance at temperatures up to 650 °C as shown by in situ TEM and extended X‐ray absorption fine structure analysis.
Phylogeny of Anisopappus with species circumscriptions revisited (Asteraceae: Athroismeae)
Authors: Bengtson Annika , Osborne Jo , Anderberg Arne A. 
Published: January 2021

AbstractAnisopappus (Asteraceae: Athroismeae) is a genus with its main distribution in Africa (one species also in Asia), currently considered to include around 21 species. A molecular phylogenetic study of Anisopappus is presented for the first time, based on plastid (ndhF, trnL‐trnF, trnQ‐rps16) and nuclear (ETS, ITS) data. Anisopappus is confirmed to be monophyletic, and species interrelationships are resolved. The results differ from earlier treatments based on morphology, and the phylogenetic analyses reveal a need for changes in species circumscriptions as compared to those of the most recent treatment. Consequently, many taxa currently treated as synonyms are here shown to represent separate species indicating that the genus includes well over 40 species. Distribution patterns now emerge where several clades are found to consist of species restricted to a particular geographical region. The Anisopappus of Madagascar, many of which were earlier placed in synonymy with species found on the African continent, are here shown to be endemic, and the results reveal a need for further studies of that group.
Qualitative study of the influence of horse‐owner relationship during some key events within a horse's lifetime
Authors: Clough Harriet , Roshier Mandy , England Gary , Burford John , Freeman Sarah 
Published: January 2021

AbstractBackground: There is a lack of research exploring how the horse‐human relationship influences horse owners' decision‐making. The aim of this study was to investigate how an owner's relationship with their horse affects decisions around key events during their horse's lifetime.Methods: An online survey and semi‐structured telephone interviews were conducted with horse‐owners. The online survey asked horse owners about their experiences of decision‐making and their relationship with their horse and identified interview participants. Two semi‐structured interviews were conducted with 11 horse owners on their experience of purchasing and euthanasia of horses. Interviews were recorded and transcribed, and thematic analysis was performed.Results: There were 938 responses to the online survey. The majority of owners considered their horse to be a member of the family (92.9%, 808/870). The mean amount spent annually on their horse was £9215 (range £324–14,240). Seven themes were identified in the interviews including mismatching of horse and rider; financial, time, welfare and personal obligations involved in horse ownership; shared‐decision making; the horse's quality of life; guilt and responsibility grief surrounding euthanasia decisions.Conclusion: This study highlighted the strong owner‐horse relationship, the significant obligations involved in horse ownership and the emotional impact of euthanasia decisions.