bluuebunny/crossref_metadata_2025_split huggingface dataset:
Developments in evidence creation for treatments of inborn errors of metabolism
Authors: Stockler‐Ipsiroglu Sylvia , Potter Beth K. , Yuskiv Nataliya , Tingley Kylie , Patterson Marc , van Karnebeek Clara 
Published: September 2020

AbstractInborn errors of metabolism (IEM) represent the first group of genetic disorders, amenable to causal therapies. In addition to traditional medical diet and cofactor treatments, new treatment strategies such as enzyme replacement and small molecule therapies, solid organ transplantation, and cell‐and gene‐based therapies have become available. Inherent to the rare nature of the single conditions, generating high‐quality evidence for these treatments in clinical trials and under real‐world conditions has been challenging. Guidelines developed with standardized methodologies have contributed to improve the practice of care and long‐term clinical outcomes. Adaptive trial designs allow for changes in sample size, group allocation and trial duration as the trial proceeds. n‐of‐1 studies may be used in small sample sized when participants are clinically heterogeneous. Multicenter observational and registry‐based clinical trials are promoted via international research networks. Core outcome and standard data element sets will enhance comparative analysis of clinical trials and observational studies. Patient‐centered outcome‐research as well as patient‐led research initiatives will further accelerate the development of therapies for IEM.
Effectiveness and surface changes of different decontamination protocols at smooth and minimally rough titanium surfaces
Authors: Stuani Vitor T. , Kim David M. , Nagai Masazumi , Chen Chia‐Yu , Sant'Ana Adriana C.P. 
Published: September 2020

AbstractBackgroundThe objective of this study is to evaluate titanium decontamination after different protocols while assessing changes in surface roughness, chemical composition, and wettability.MethodsNinety‐six smooth (S) and 96 minimally rough (R) titanium microimplants were used. Pristine microimplants were reserved for negative control (S‐nC/R‐nC, n = 9), while the remaining microimplants were incubated in Escherichia coli culture. Non‐decontaminated microimplants were used as positive control (S‐pC/R‐pC, n = 3). The other microimplants were divided into seven different decontamination protocols (12 S/R per group): 24% EDTA, 2% chlorhexidine (CHL), gauze soaked in 2% chlorhexidine (GCHL), gauze soaked in ultrapure water (GMQ), scaling (SC), titanium brush (TiB), and implantoplasty (IP). Contaminated areas were assessed by scanning electron microscope images, chemical composition by energy dispersive X‐ray spectroscopy, wettability by meniscus technique, and roughness by an optical profiler.ResultsHigher residual bacteria were observed in R‐pC compared with S‐pC (P <0.0001). When comparing S and R with their respective pC groups, the best results were obtained with GCHL, SC, TiB, and IP, with no difference between these protocols (P >0.05). Changes in surface roughness were observed after all treatments, with S/R‐IP presenting the smoother and a less hydrophilic surface (P <0.05). Apart from IP protocol, all the other groups presented a more hydrophilic surface in R than in S microimplants (P <0.003). All decontamination protocols resulted in a lower percentage of superficial Ti when compared with S/R‐nC (P <0.002).ConclusionsAll decontamination protocols resulted in changes in roughness, wettability, and chemical composition, but GCHL, SC, TiB, an IP presented the best decontamination outcomes.
Comparison of the pharmacokinetic profiles of 13 phenolic acids and 6 triterpenes in normal and leukopenia rats after oral administration of Sanguisorba officinalis L. extract by LC‐MS/MS
Authors: Fan Linzi , Wang Xiaotong , Huang Jing , Gan Chunli , Jiang Shuang , Yang Xinrong , Yang Chunjuan , Yao Meicun 
Published: September 2020

AbstractA selective, accurate, and efficient liquid chromatography‐tandem mass spectrometry method was developed for the simultaneous determination of 13 phenolic acids. Additionally, for more comprehensively determining the chemical constituents in Sanguisorba officinalis L. extract, a previously developed method was employed for the simultaneous determination of six triterpenes. Thus, two methods were used to ensure the comprehensiveness and reliability of this study. Based on these methods, the pharmacokinetic profiles of the 13 phenolic acids and 6 triterpenes in normal and leukopenia rats after oral administration of S. officinalis L. extract were compared for the first time in the present study. Quantitative detection of the 13 phenolic acids and 6 triterpenes was performed using the multiple reaction monitoring mode with the electrospray ion source in negative and positive electrospray ionization, respectively. Chromatographic separation was performed on an Agilent Eclipse Plus C18 RRHD column (50 × 2.1 mm, 1.8 µm) using gradient elution with a mobile phase composed of methanol–0.1% aqueous formic acid. The pharmacokinetic results demonstrated that the pharmacokinetic characteristics of the 19 analytes in leukopenia rats differed significantly from those determined in normal rats, which could provide a helpful reference for the clinical application of S. officinalis L. in the prevention and treatment of leucopenia.
Supraglottic Carcinoma in Intravenous Opioid Drug Abusers: A Distinct Disease with Improved Survival
Authors: Yaniv Dan , Reuven Yonatan , Lahav Yonatan , Cohen Oded , Hamzany Yaniv , Moore Assaf , Rapana Olga G. , Argaman Natan , Halperin Doron , Popovtzer Aron , Bachar Gideon , Shoffel‐Havakuk Hagit 
Published: September 2020

Objectives/HypothesisRecent evidence indicates an increased prevalence of intravenous opioid drug abusers (IVDAs) among supraglottic squamous cell carcinoma (SG‐SCC) patients. This study investigates whether the clinical course of SG‐SCC in IVDA differs from SG‐SCC in non‐IVDA.Study DesignA retrospective case–control study conducted in a in two tertiary referral centers.MethodsThis case–control study compares IVDA with non‐IVDA patients diagnosed and treated for SG‐SCC in between 2005 and 2018. Disease‐free survival (DFS) and overall survival (OS) were calculated using the Kaplan–Meier estimator. Adjusted odds ratios (ORs) for mortality were calculated using multivariant analyses.ResultsA total of 124 patients were included; 21% (26) were IVDA, and 79% (98) were non‐IVDA. Age at diagnosis in the IVDA group versus the non‐IVDA group was 53 and 66 years, respectively (P = .001). Nevertheless, the age hazard ratio for OS was calculated and found to have minimal to no effect, 1.05 (95% Cl: 1.025–1.076). Otherwise, the two groups were comparable regarding demographics, other risk factors (i.e., gender, smoking, and alcohol), and comorbidities status, as well as the comparable stage at diagnosis, histologic grading, and treatment modalities. Although the DFS was comparable in both groups, the 5‐year OS was 55% in the IVDA group compared with 34% among the non‐IVDA patients (P = .04). In multivariant analyses for mortality, positive IVDA history was found to be protective, adjusted OR: 0.263 (95% CI: 0.081–0.854). Similarly, within the subgroup of 100 patients with advanced‐stage disease (III and IV), the adjusted OR was 0.118 (95% CI: 0.028–0.495).ConclusionsSG‐SCC in IVDA patients has a distinct clinical course, presenting at a younger age, and may have improved prognosis.Level of EvidenceNA Laryngoscope, 131:E1190–E1197, 2021
A novel GABRB3 variant in Dravet syndrome: Case report and literature review
Authors: Pavone Piero , Pappalardo Xena Giada , Marino Simona D. , Sciuto Laura , Corsello Giovanni , Ruggieri Martino , Parano Enrico , Piccione Maria , Falsaperla Raffaele 
Published: September 2020

AbstractBackgroundMutations in GABRB3 have been identified in subjects with different types of epilepsy and epileptic syndromes, including West syndrome (WS), Dravet syndrome (DS), Lennox‐Gastaut syndrome (LGS), myoclonic‐atonic epilepsy (MAE), and others.Methods and resultsWe herewith report on a girl affected by DS, who has been followed from infancy to the current age of 18 years. Next‐generation sequencing (NGS)‐based genetic testing for multigene analysis of neurodevelopmental disorders identified two likely de novo pathogenic mutations, a missense variant in GABRB3 gene (c.842 C>T; p.Thr281IIe) and a nonsense variant found in BBS4 gene (c.883 C>T; p.Arg295Ter).ConclusionA likely relationship between the novel GABRB3 gene variant and the clinical manifestations presented by the girl is proposed. Previously, one case of DS and two of DS‐like linked with GABRB3 mutations have been reported. To the best of our knowledge, this is the first report of DS associated with this novel variant. A literature review of clinical cases with various types of epileptic encephalopathies (EEs) related to GABRB3 mutations is reported.
Stimulation of Gastric Transit Function Driven by Hydrolyzed Casein Increases Small Intestinal Carbohydrate Availability and Its Microbial Metabolism
Authors: Shen Junhua , Mu Chunlong , Wang Huisong , Huang Zan , Yu Kaifan , Zoetendal Erwin G , Zhu Weiyun 
Published: September 2020

AbstractGastrointestinal (GI) functions affect gut nutrient flow and microbial metabolism. Dietary peptides modulate GI functions and improve small intestinal health, but the mechanism remains elusive. This study aims to investigate whether dietary peptides affect small intestinal microbial metabolism, and the underlying mechanisms. An ileal‐cannulated pig model is adopted to explore the relationship between gut nutrient flow and microbial metabolism after treatment with hydrolyzed casein (peptides) or intact casein (Control)‐based diet. The results demonstrate that hydrolyzed casein enhances microbial carbohydrate metabolism with higher Streptococcus abundance and higher lactate level in the ileum. Meanwhile, hydrolyzed casein increases ileal flows of nutrients, especially carbohydrate, leading to a higher carbohydrate availability in ileal digesta. To unveil the mechanisms, it is found that the hydrolyzed casein enhances the ghrelin signal and improves development of interstitial cells of Cajal and muscular layer in gastric corpus, indicating the enhanced upper GI transit function. In addition, hydrolyzed casein improves small intestinal health, as indicated by higher villus heights and luminal lactate concentrations in the jejunum and ileum. In conclusion, hydrolyzed casein stimulates upper GI transit function, enhances gut nutrient flow, and increases small intestinal carbohydrate availability and its microbial metabolism, which favor the small intestinal health.
Printed planar tunable composite right/left‐handed leaky‐wave antenna based on a tunable polymer‐BST substrate
Authors: Hajisaeid Ehsan , Deshmukh Shruti , Burbine Stephen J. , Keaney Erin E. , Lepont Claire , Herndon Mary , Barry Carol , Mead Joey , Akyurtlu Alkim 
Published: September 2020

AbstractThis article presents a printed tunable right/left‐handed leaky wave antenna (LWA) on a new flexible, tunable low density polyethylene‐barium strontium titanate composite substrate using an aerosol jet printer. The new substrate had a dielectric constant of 16 and tunability of 3.5% at f = 10 GHz. Interdigital capacitors (IDC) were utilized in the leaky wave antenna design not only to act as shunt capacitor in CRLH transmission line design but also as tunability element to implement tuning capability of the LDPE‐BST substrate for antenna beam steerability for a fixed operated frequency in X‐band frequency range. Up to 15° beam steering was achieved by applying DC voltage through the printed leaky wave antenna.
Monitoring of the sedimentation kinetics of vaccine adjuvants using water proton NMR relaxation
Authors: Taraban Marc B. , Yu Yihua Bruce 
Published: September 2020

AbstractSuspensions of solid particles find applications in many areas—mining, waste treatment, and in pharmaceutical formulations. Pharmaceutical suspensions include aluminum‐adjuvanted vaccines are widely administered to millions of people worldwide annually. Hence, the stability parameters of such suspensions, for example, sedimentation rate and the compactness of the formed sediments, are of great interest to achieve the most optimal and stable formulations. Unlike currently used analytical techniques involving visual observations and/or monitoring of several optical properties using specialized glassware, water proton nuclear magnetic resonance (wNMR) used in this work allows one to analyze samples in their original sealed container regardless of its opacity and/or labeling. It was demonstrated that the water proton transverse relaxation rate could be used to monitor in real time the sedimentation process of two widely used aluminum adjuvants—Alhydrogel® and Adju‐Phos®. Using wNMR, we obtained valuable information on the sedimentation rate, dynamics of the supernatant and sediment formation, and the sedimentation volume ratio (SVR) reflecting the compactness of the formed sediment. Results on SVR from wNMR were verified by caliper measurements. Verification of the sedimentation rate results from wNMR by other analytical techniques is challenging due to differences in the measured attributes and even units of the reported rate. Nonetheless, our results demonstrate the practical applicability of wNMR as an analytical tool to study pharmaceutical suspensions, for example, aluminum‐adjuvanted vaccines, to provide higher quality and more efficient vaccines. Such analyses could be carried out in the original container of a suspension drug product to study its colloidal stability and to monitor its quality over time without compromising product integrity.
Randomized probabilistic approach for parametric uncertainties in unmanned helicopters
Authors: Singh Rupam , Bhushan Bharat 
Published: September 2020

SummaryParametric uncertainties and the problems associated with the design of robust state feedback control tends to be a major concern for achieving stable operation of nonlinear systems. To overcome these drawbacks, this article provides a perspective on a randomized algorithm based probabilistic control for nonlinear systems. The control approach aims at development of randomized algorithms for control of unmanned helicopters which are subjected to uncertainties. A controller gain is synthesized by developing a robust probabilistic approach using sequential randomized algorithm with the two degree of freedom helicopter system. Subsequently, the stability and reachability for the action of developed controller is validated with the help of Lyapunov asymptotic stability and reach tube analysis, and the corresponding results were analyzed. A comparative assessment of simulation and real‐time control results of projected approaches on helicopter model are presented for validation of the controller response.
Ultrastructure of cell trafficking pathways and coronavirus: how to recognise the wolf amongst the sheep
Authors: Neil Desley , Moran Linda , Horsfield Catherine , Curtis Elizabeth , Swann Olivia , Barclay Wendy , Hanley Brian , Hollinshead Michael , Roufosse Candice 
Published: September 2020

AbstractThe severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) pandemic has resulted in an urgent need to understand the pathophysiology of SARS‐CoV‐2 infection, to assist in the identification of treatment strategies. Viral tissue tropism is an active area of investigation, one approach to which is identification of virus within tissues by electron microscopy of post‐mortem and surgical specimens. Most diagnostic histopathologists have limited understanding of the ultrastructural features of normal cell trafficking pathways, which can resemble intra‐ and extracellular coronavirus; in addition, viral replication pathways make use of these trafficking pathways. Herein, we review these pathways and their ultrastructural appearances, with emphasis on structures which may be confused with coronavirus. In particular, we draw attention to the fact that, when using routine fixation and processing, the typical ‘crown’ that characterises a coronavirus is not readily identified on intracellular virions, which are located in membrane‐bound vacuoles. In addition, the viral nucleocapsid is seen as black dots within the virion and is more discriminatory in differentiating virions from other cellular structures. The identification of the viral replication organelle, a collection of membranous structures (convoluted membranes) seen at a relatively low scanning power, may help to draw attention to infected cells, which can be sparse. © 2020 The Authors. The Journal of Pathology published by John Wiley & Sons, Ltd. on behalf of The Pathological Society of Great Britain and Ireland.
Advancing reliability assessments of photovoltaic modules and materials using combined‐accelerated stress testing
Authors: Owen‐Bellini Michael , Hacke Peter , Miller David C. , Kempe Michael D. , Spataru Sergiu , Tanahashi Tadanori , Mitterhofer Stefan , Jankovec Marko , Topič Marko 
Published: September 2020

AbstractPreviously undiscovered failure modes in photovoltaic (PV) modules continue to emerge in field installations despite passing protocols for design qualification and quality assurance. Failure to detect these modes prior to widespread use could be attributed to the limitations of present‐day standard accelerated stress tests (ASTs), which are primarily designed to identify known degradation or failure modes at the time of development by applying simultaneous or sequential stress factors (usually two at most). Here, we introduce an accelerated testing method known as the combined‐accelerated stress test (C‐AST), which simultaneously combines multiple stress factors of the natural environment. Simultaneous combination of multiple stress factors allows for improved identification of failure modes with better ability to detect modes not known a priori. A demonstration experiment was conducted that reproduced the field‐observed cracking of polyamide‐ (PA‐) and polyvinylidene fluoride (PVDF)–based backsheet films, a failure mode that was not detected by current design qualification and quality assurance testing requirements. In this work, a two‐phase testing protocol was implemented. The first cycle (“Tropical”) is a predominantly high‐humidity and high‐temperature test designed to replicate harsh tropical climates. The second cycle (“Multi‐season”) was designed to replicate drier and more temperate conditions found in continental or desert climates. Testing was conducted on 2 × 2‐cell crystalline‐silicon cell miniature modules constructed with both ultraviolet (UV)–transmitting and UV‐blocking encapsulants. Cracking failures were observed within a cumulative 120 days of the Tropical condition for one of the PA‐based backsheets and after 84 days of Tropical cycle followed by 42 days of the Multi‐season cycle for the PVDF‐based backsheet, which are both consistent with failures seen in fielded modules. In addition to backsheet cracking, degradation modes were observed including solder/interconnect fatigue, various light‐induced degradation modes, backsheet delamination, discoloration, corrosion, and cell cracking. The ability to simultaneously apply multiple stress factors may allow many of the test sequences within the standardized design qualification procedure to be performed using a single test setup.
Hydrogen bond mediated self‐assembly of two diblock copolymers
Authors: Anderson Eric R. , Daga Vikram K. , Gido Samuel P. , Watkins James J. 
Published: September 2020

AbstractTwo chemically dissimilar diblock copolymers, polybutadiene‐b‐poly(acrylic acid), PBd‐b‐PAA (Mw = 5.8–4 kg mol−1) and poly(styrene)‐b‐poly(ethylene oxide), PS‐b‐PEO (Mw = 9–5 kg mol−1) were blended in an effort to achieve morphologies typical of triblock copolymers. Blend compatibility was achieved by the hydrogen bond driven association of the PAA block of one diblock with the PEO block of the other. Small angle X‐ray scattering was used to determine the morphologies of the compositions, which were further investigated using transmission electron microscopy and selective staining techniques. The crystallinity of the PEO block was determined by differential scanning calorimetry. The hydrogen bond interactions between PEO and PAA yielded a complex triblock lamellar morphology of the form PS‐b‐(PEO/PAA)‐b‐PBd‐b‐(PEO/PAA). This morphology was stable when crystallization of PEO was suppressed by sufficient interaction with PAA.
Comparison of robotic‐assisted versus conventional unicompartmental knee arthroplasty for the treatment of single compartment knee osteoarthritis: A meta‐analysis
Authors: Zhang Pei , Xu Keteng , Zhang Jiale , Chen Pengtao , Fang Yongchao , Wang Jingcheng 
Published: September 2020

AbstractBackgroundThe robotic‐assisted unicompartmental knee arthroplasty (UKA) is proposed to improve the accuracy of component positioning.MethodsWe conducted a literature search in Medline, Embase, Web of Science and the Cochrane Library until April 2020.ResultsOur meta‐analysis included 10 articles, involving 1231 knees. Our meta‐analysis demonstrated that the robotic group had significantly better results in outliers of limb alignment (p < 0.001) and outliers of tibial alignment (p < 0.001). No statistical differences were found in the American Knee Society Score (p = 0.63), range of motion (p = 0.93), pain (p = 0.27), rate of revisions (p = 0.73) and rate of complications (p = 0.67).ConclusionsRobotic‐assisted UKA has better component position accuracy compared with conventional UKA. But there was no significant difference in clinical results. In order to further evaluate the utility of robotic‐assisted UKA, long‐term follow‐up randomized controlled trials (RCTs) are needed, as well as studies to evaluate the correlation between postoperative alignment and long‐term clinical results.
Investigation of immune cells on elimination of pulmonary‐Infected COVID‐19 and important role of innate immunity, phagocytes
Authors: Farshi Esmaeil , Kasmapur Bahram , Arad Anya 
Published: September 2020

SummaryWe identified types of immune cells that contribute to clearing COVID‐19 during the acute phase of the infection in mouse model and human. Our results suggest that both innate and adaptive immune responses are essential for controlling COVID‐19 infection. Mild infection report of children by COVID‐19 comparing adults' infection causes conclusion of higher resistance of immune system of children comparing adults. Our results show innate immune system including phagocytes contribute severely to the elimination of COVID‐19 in both mouse model and human. Our results also show the elimination of COVID‐19 required the activation of B cells by CD4+ T cells. CD4+ T cells play an important role in elimination of COVID‐19 in primary effection. We measured IgM and IgG in all patients including adults and kids (human) and found IgM and IgG in kids patients are much higher than other adults patients. It causes production of much more natural antibodies in kids' bodies to protect them against COVID‐19 that shows reason of mild effection of kids comparing adults. Our observations have important ramifications for the development of novel vaccination and medicine strategies to alleviate COVID‐19. The most important result is for producing any vaccine for COVID‐19, increasing and producing these factors must be included: (a) Phagocytes (IgM and IgG), (b) T Cells, and (c) White Cells.