bluuebunny/crossref_metadata_2025_split huggingface dataset:
Diagnosis and management of Adenosine Deaminase 2 Deficiency children: the experience from China
Authors: Wang Wei , Zhang Tiannan , Zheng Wenjie , Zhong Linqing , Wang Lin , Li Ji , Liu Qian , Dong Yanqing , Song Hongmei 
Published: December 2020

Abstract
BACKGROUND: Deficiency of adenosine deaminase 2 (DADA2) is a rare autoinflammatory disease caused by mutations in the ADA2 gene. Few Chinese cases have been reported. We describe and compare the clinical features, genotypes, and treatments of Chinese DADA2 patients and non-Chinese patients.METHODS: Primary immunodeficiency disease panel or whole-exome sequencing was performed for suspected cases, and assays for adenosine deaminase 2 (ADA2) enzyme activity were also carried out for the patients and their parents. Case reports of Chinese and non-Chinese patients with DADA2 were searched in PubMed and Chinese national databases.RESULTS: Seven unrelated children from China with DADA2 were included in our study. Five were identified at Peking Union Medical College Hospital, and two had been reported previously (1 on PubMed and 1 in Chinese literature). Fourteen mutations in ADA2 were identified, 7 of which have not previously been reported in non-Chinese patients. Four children who underwent enzymatic analysis had lower ADA2 activity compared with their parents. Phenotypic manifestations included fever, skin symptoms, vasculitis, and neurologic involvement. Treatments varying from steroids, immunosuppressants, and tocilizumab, anti-TNF therapy and hematopoietic stem cell transplantation (HSCT) were effective depending on phenotype and severity.CONCLUSION: This study includes the largest number of Chinese DADA2 patients to date. We recommend the combination of enzymatic analysis with gene screening to confirm the diagnosis. Different genotypes were observed among Chinese DADA2 patients; most phenotypes were similar to those of non-Chinese DADA2 patients, except for growth retardation. Disease remission might not be achieved with anti-IL-6 therapy.
Differential mitochondrial proteomic analysis of A549 cells infected with avian influenza virus subtypes H5 and H9&nbsp;
Authors: Yang Yuting , Zhang Yun , Yang Changcheng , Fang Fang , Wang Ying , Chang Haiyan , Chen Ze , Chen Ping 
Published: December 2020

Abstract
Background: Both the highly pathogenic avian influenza (HPAI) H5N1 and low pathogenic avian influenza (LPAI) H9N2 viruses have been reported to cross species barriers to infect humans. H5N1 viruses can cause severe damage and a high mortality rate, but H9N2 viruses cannot. Our purpose was to use proteomics technology to study the differential expression of mitochondrial-related proteins caused by H5N1 and H9N2 virus infections. Methods: According to the determined viral infection titer, A549 cells were infected with 1 MOI (multiplicity of infection) virus, and the mitochondria were extracted after 24 h of incubation. The protein from lysed mitochondria was analyzed by the BCA method to determine the protein concentration, SDS-PAGE preliminary analysis, two-dimensional gel electrophoresis, and mass spectrometry. Differential protein spots were selected,, and Western blotting was performed to verify the proteomics results. The identified proteins were subjected to GO analysis for subcellular localization, KEGG analysis for functional classification and signaling pathways assessment, STRING analysis for functional protein association network construction. Results: In the 2-D gel electrophoresis analysis, 227 protein spots were detected in the H5N1-infected group, and 169 protein spots were detected in the H9N2-infected group. Protein spots further mass spectrometry identification and removal of redundancy, 32 differentially expressed proteins were identified. Compared with the H9N2 group, the H5N1-infected group had 16 upregulated mitochondrial proteins and 16 downregulated proteins. The differential expression of 70-kDa heat shock protein analogs, short-chain enoyl-CoA hydratase, malate dehydrogenase, and ATP synthase was verified by Western blot, and the results were consistent with the proteomics findings. Functional analysis indicated that these differentially expressed proteins were involved mainly in apoptosis and metabolism. Conclusions: Compared with their expression in the H9N2 group, the differential expression of eight mitochondrial proteins in H5N1 group led to host T cell activation, antigen presentation, stress response, ATP synthesis and cell apoptosis reduction, leading to higher pathogenicity of H5N1 than H9N2.
HDL-Cholesterol Efflux Capacity and Lipid Profile in Patients with Systemic Sclerosis.
Authors: Ferraz-Amaro Iván , Delgado-Frías Esmeralda , Hernández-Hernández Vanesa , Sánchez-Pérez Hiurma , de Armas-Rillo Laura , Armas-González Estefanía , Machado José David , Diaz-Gonzalez Federico 
Published: December 2020

Abstract
Objective. It is well established that patients with systemic sclerosis (SSc) have a disrupted lipid profile and an increased cardiovascular risk. Cholesterol efflux capacity (CEC), the ability of high-density lipoprotein (HDL)-cholesterol to accept cholesterol from macrophages has been linked to cardiovascular events. The aim of this study was to establish whether CEC and lipid profile were impaired in SSc patients respect to controls and whether these changes were associated with disease-related data. Methods. Cross-sectional study encompassed 188 individuals: 73 SSc patients and 115 controls. CEC, using an in vitro assay, and lipoprotein serum concentrations were assessed in patients and controls. A multivariable analysis was performed to study the differences in CEC between patients and controls, and if SSc-related data could explain such differences. Results. The multivariate analysis adjusted for demographic characteristics, cardiovascular risk factors and lipid-related molecules showed that total cholesterol (beta coefficient: -22 [95%CI -37- -7], p=0.004), triglycerides (beta coefficient: 24 [95%CI 2-47], p=0.033), lipoprotein A (beta coefficient: 22 [95%CI 2-43], p=0.033) and CEC (beta coefficient: -6 [95%CI -10- -2]%,p=0.002) were significantly differences between patients and controls. Skin thickness, as assessed by modified Rodnan skin score, was independently associated with a lower CEC (beta coefficient: -0.21 [95%CI -0.37- -0.05]%, p=0.011) after multivariable adjustment. Conclusion. SSc patients show an abnormal lipid profile with respect to controls including CEC. Skin thickness is independent and inversely associated with CEC in SSc patients.
Higher expression of monocyte chemotactic protein 1 in mild COVID-19 patients might be correlated&nbsp;with inhibition of&nbsp;Type I IFN signaling
Authors: Xi Xueyan , Guo Yang , Zhu Min , Wei Yuhui , Li Gang , Du Boyu , Wang Yunfu 
Published: December 2020

Abstract
Background: Chemokine levels in severe coronavirus disease 2019 (COVID-19) patients have been shown to be markedly elevated. But the role of chemokines in mild COVID-19 has not yet been established. According to the epidemiological statistics, most of the COVID-19 cases in Shiyan City, China, have been mild. The purpose of this study was to evaluate the level of chemokines in mild COVID-19 patients and explore the correlation between chemokines and host immune response. Methods: In this study, we used an enzyme-linked immunosorbent assay (ELISA) to detect serum levels of chemokines in COVID-19 patients in Shiyan City. Expression of chemokine receptors and of other signaling molecules was measured by real-time polymerase chain reaction (PCR). Results: We first demonstrated that COVID-19 patients, both sever and mild cases, are characterized by higher level of chemokines. Specifically, monocyte chemotactic protein 1 (MCP-1) is expressed at higher levels both in severe and mild cases of COVID-19. The receptor of MCP-1, C-C chemokine receptor type 2 (CCR2), was expressed at higher levels in mild COVID-19 patients. Finally, we observed a significant negative correlation between expression levels of interferon (IFN) regulatory factor 3 (IRF3) and serum levels of MCP-1 in mild COVID-19 patients. Conclusion: Higher expression of MCP-1 in mild COVID-19 patients might be correlated with inhibition of IFN signaling. The finding adds to our understanding of the immunopathological mechanisms of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and provides potential therapeutic targets and strategies.
The Discriminant Analysis Approach for Evaluating Effectiveness of Learning in an Instructor-Led Virtual Classroom
Authors: Magdalene Delighta Angeline D. , Ramasubramanian P. , Samuel Peter James I. 
Published: December 2020

Abstract
The effective learning requires putting down various associations of new ideas to old ones to integrate some innovative thoughts. The learners must change the associations among the things they already know, or even reject some long-held attitude about the world. The choice to the essential reformation is to deform the new information to fit their old ideas or to reject the new information entirely. Learners come to the classroom with their own ideas, some may be correct and some may not be, concerning roughly each topic they are expected to come across. If their perception and misunderstanding are unnoticed or discharged out of control, it affects the learning of a learner. The learners must be encouraged to build up new observation by seeing how such observation helps them make better sense of the world. The objective of this research paper is to put down the fundamentals of learning that promotes effective learning in an instructor-led virtual classroom and to analyze the learners’ learning performance using the Discriminant Analysis, a data mining technique. The Discriminant Analysis uses statistically significant determinants to predict learners’ learning in a classroom.
Sugar Substitutes and Sweeteners in Confectionery Technology
Authors: Reznichenko Irina , Shcheglov Mikhail 
Published: December 2020

Introduction. Targeted diabetic confectionery for people with carbohydrate metabolism impairment includes sugar substitutes or sweeteners instead of sugar. At the same time, modified carbohydrate composition should guarantee high sensory characteristics of the finished product, production technology and the raw materials being the main quality factors. Certain safety requirements are imposed on sweeteners and sugar substitutes, and their daily intake is regulated. Sweeteners and sugar substitutes have various technological and functional properties that are taken into account when developing specialized confectionery products. In this regard, the search for substitutes of non-sugar nature is a relevant matter. The research objective was to analyze, systematize, and generalize international and domestic experience in the use of sweeteners and sugar substitutes in the technology of confectionery products.
Study objects and methods. The authors analyzed related scientific articles and legislative documents over the past ten years. The materials were obtained from Elibrary, Scimago Journal Country Rank, Scopus, and Scielo. The methods included analysis, systematization, and generalization.
Results and discussion. Technological exclusion of sugar from formulations of flour and sugar confectionery is a difficult task. Each of the currently known sweeteners and sugar substitutes has its advantages and disadvantages. The authors analyzed their specific features, technological properties, and consumption rates. They identified sweeteners that are widely used in technologies of sugar and flour confectionery products and do not affect the sensory characteristics of the finished product.
Conclusion. The range of sweeteners and sugar substitutes in confectionery technology is limited. The domestic and foreign experience of using sweeteners showed that stevioside and sorbitol are used both as sweeteners and in flour confectionery. Isomalt and lactitol proved effective in chocolate production, if used under optimal technological conditions. New scientifically based formulations and technologies for targeted confectionery for people with impaired carbohydrate metabolism and further research on the use of sweeteners and sugar substitutes will satisfy the increasing demand for these products and expand the range of healthy foods.
New Functional Formulations for Dry Seafood Concentrates and their Properties
Authors: Bogdanov Valery , Simdiankin Andrei , Pankina Anna , Mostovoi Vadim 
Published: December 2020

Introduction. Nutrition has a significant impact on human health, performance, well-being, and lifespan. The research objective was to develop formulations for functional compositions of seafood cryoconcentrates.
Study objects and methods. The study featured concentrates obtained by cryotechnology from carcasses of Pacific squid (Todarodes pacificus), muscular shell of cucumaria (Cucumaria japonica), scallop mantle (Mizuhopecten yessoensis), octopus skin (Octopus dofleini), milt of Pacific herring (Pacific herring), and Japanese kelp (Laminaria japonica), as well as their multicomponent mixes. The content of biologically active substances was determined by the spectrophotometric method. The safety and biological value were biotested on Tetrahymena pyriformis.
Results and discussion. The dry seafood concentrates had a high content of protein, minerals, and biologically active components, i.e. hexosamines, triterpene glycosides, carotenoids, nucleic acids, etc. The seafood cryoconcentrate mixes were divided into fortifying, restoring, immunomodulating, and mineral. Their chemical composition varied greatly, depending on the functional aim of the mix. For instance, the protein content varied from 28.8 to 57.6%, lipids – from 3.1 to 6.9%, carbohydrates – from 15.0 to 35.9%, minerals – from 10.9 to 22.9%, and hexosamines – from 2.2 to 5.6%. The experiments made it possible to substantiate the composition of the dry mixes. The compilation principle was based on the chemical profile of the raw material, as well as on the choice of biologically active substances. All the dry seafood concentrates showed a high relative biological value. However, the values differed, depending on the type of raw material: from 65.5% for the scallop mantle sample to 86.3% for the octopus skin sample. The difference could be explained by the chemical composition of the cryopowders and their availability to ciliate enzymes.
Conclusion. The research resulted in formulations and functional mixes of dry seafood concentrates obtained by cryotechnology. The dry seafood concentrates can be used both independently and as functional compositions. The developed mixes demonstrated rather high content of animal proteins, minerals, and biologically active substance, e.g. hexosamines, triterpene glycosides, carotenoids, nucleic acids, etc. Their functional compositions proved to have excellent sensory properties; they are biologically safe for human consumption and have a high relative biological value.
Correction of the Carbohydrate Composition of Raw Materials for Microbial Transformation Based on Microbial Consortia
Authors: Kondratenko Vladimir , Posokina Natalia , Lyalina Ol’ga , Kolokolova Anastasiay , Glazkov Sergey 
Published: December 2020

Introduction. Fermentative processing of plant raw materials is traditionally carried out using native (epiphytic) microflora, which is located on the surface and represented by lactic acid microorganisms. During this process, the carbohydrates in the raw material are metabolized into lactic acid. This process does not always result in optimal product quality as the raw material often lacks carbohydrates, the optimal conditions for the development of the target microflora are hard to achieve, the microflora might be inhibited by contaminants, etc. Lactic acid microbial consortia can act as a good alternative to spontaneous fermentation of cabbage as this method creates good conditions for the microbial synergistic interaction. Such fermentation process can be controlled by adjusting the carbohydrate composition of the substrate. The research objective was to develop an analytical approach to determine the minimum required degree of change in the native carbohydrate composition of substrate that would ensure the synergy of lactic acid microorganisms.
Study objects and methods. The fermentation process was performed using white cabbage of Slava variety and such strains of lactic acid microorganisms as Lactobacillus casei VCM 536, Lactobacillus plantarum VCM B-578, and Lactobacillus brevis VCM B-1309, as well as their paired consortia. The raw material was subjected to grinding, and the epiphytic microflora was removed to create optimal conditions for the development of the lactic acid microflora.
Results and discussion. The study made it possible to define the dynamics of carbohydrate fermentation in white cabbage by various strains of lactic bacteria and their paired consortia during processing. Mathematical models helped to describe the dynamics of glucose and fructose fermentation. The experiment also demonstrated the changes that occurred in the interaction within the paired consortia during fermentation. The paper introduces a new approach to determining the minimum required degree of change in the native carbohydrate composition required to ensure synergy of lactic acid microorganisms in paired consortia.
Conclusion. The research defined the necessary amounts of carbohydrate needed to shift the integral factor of mutual influence towards sustainable synergy for three paired consortia. Consortium L. brevis + L. plantarum + 3.65 g/100 g of fructose proved to be the optimal variant for industrial production of sauerkraut from white cabbage of Slava variety. The developed approach can improve the existing industrial technologies of fermentation and create new ones.
Hand hygiene innovation for low income households in India
Authors: Gibson Walter T. , Trevaskis Helen , Raman R. V. , Chauhan Ayush , George Babitha , Singh Neha , Roongta Akshay , Krishnaswamy Rikta , Andrade Nalini , Curtis Valerie 
Published: December 2020

Abstract
Our objective was to explore and prototype new products to facilitate improved hand hygiene after defecation by overcoming the constraints associated with soap use in low income settings, specifically urban slums in India. We used a systematic user-centred innovation process which led to the design of two products with user appeal ready for market trials, and one further concept ready for feasibility testing and further product development.
Adequacy assessment of an urban drainage system considering future land use and climate change scenario
Authors: Afrin S. , Islam M. M. , Rahman M. M. 
Published: December 2020

Abstract
Dhaka, the capital of Bangladesh, has been experiencing severe water-logging and urban flooding in the last few decades. In this paper, we estimate the peak storm runoff of Hatirjheel-Begunbari canal – the largest drainage system of the city – under different operational, land use and climate scenarios (2013, 2025 and 2040). Our method includes digital elevation model (DEM) reconditioning, watershed delineation, and development of future land use scenario. We apply HEC-RAS to check the adequacy of Begunbari canal cross-sections to carry peak runoff for the scenarios considered here. The Hatirjheel-Begunbari system is found to drain stormwater from ∼25% of the city. Within the system, built-up areas are increasing linearly by 0.8 Km2/year, whereas water body and wetlands are decreasing exponentially, which might increase the runoff coefficient by 11% in 2040 relative to 2013. Climate-induced change in rainfall intensity along with land-use change show three times higher runoff in 2040 than in 2013. Around 58% of canal cross-sections appear to be overflown at both banks while carrying a 5-year return period peak runoff under the 2013 scenario. For future scenarios, all sections seem to cause an overflow, which is alarming.
Green synthesis of Quercus coccifera hydrochar in subcritical water medium and evaluation of its adsorption performance for BR18 dye
Authors: Saleh Mohammed , Bilici Zeynep , Ozay Yasin , Yabalak Erdal , Yalvac Mutlu , Dizge Nadir 
Published: December 2020

Abstract
In this study, we investigated the production conditions of Quercus coccifera hydrochar, which is an inexpensive and easy available adsorbent, for the adsorption of Basic Red 18 (BR18) azo dye. The hydrochar was produced in the eco-friendly subcritical water medium (SWM). The effects of the pH (2–10), adsorbent size (45–106 μm), adsorbent dose (0.5–1.5 g/L), dye concentration (40–455 mg/L), and contact time (5–120 min) were studied via optimization experiments. The optimum conditions were pH 10, particle size of 45 μm, particle amount of 1.5 g/L, dye concentration of 455 mg/L, and 60 min. The removal efficiency increased sharply for the first 5 min; after that the removal efficiency reached a steady state at 60 min, with a maximum removal of 88.7%. The kinetic studies for the adsorption of BR18 dye in aqueous solution using hydrochar showed pseudo-second-order kinetics. The Langmuir and Freundlich isotherm models were used to explain the relationship between adsorbent and adsorbate, and Freundlich isotherm was the most suitable model because of its high regression coefficient (R2) value. The intraparticle diffusion model was used to determine the adsorption mechanism of BR18 onto Q. coccifera acorn hydrochar. Desorption studies were also carried out using different types of acid and different molarities.
An Asynchronous, Mobile Text-Based Platform (XatJove Anoia) for Providing Health Services to Teenagers: Protocol for a Quasiexperimental Study
Authors: Sauch Valmaña Glòria , Vidal-Alaball Josep , Garcia Furió Victoria , Testoni Giorgia , Espelt Albert , Exposito Katarin , Saigí-Rubió Francesc , Carré Núria , Sanz Ikuska , Vicens Victor 
Published: December 2020

Background
Due to the COVID-19 pandemic, it is more essential than ever to implement protective measures in primary care centers to ensure patients’ safety. This protocol describes a quasiexperimental study on the use of a mobile chat platform as a clinical consultation tool for adolescents and primary health care physicians.

Objective
The purpose of the quasiexperimental study is to demonstrate that the use of mobile phones and messaging apps increases the number of health consultations. The study will be performed as part of the Health and School program in the Anoia region.

Methods
The quasiexperimental study will compare the number of face-to-face consultations to the number of consultations conducted on XatJove Anoia, as part of the Health in Schools program in the Anoia region. The study will involve the use of a new communication platform (ie, XatJove Anoia) for health care professionals and adolescents, and data on the number of face-to-face consultations will be collected as part of the same program in another region. Data will be collected from secondary schools during the academic year 2020-2021. Statistical analyses will be performed on the data that users will enter in the registration form. These data will be collected by means of a questionnaire, which will be submitted once the questionnaire is closed. The questionnaire will consist of multiple-choice questions, which will allow numerical values to be assigned to various responses in order to carry out statistical analyses.

Results
The study is projected to start at the beginning of November 2020 and finish in June 2021, which is when data analysis is expected to start.

Conclusions
The results of the quasiexperimental study may assist in the development and planning of school health programs.

Trial Registration
ClinicalTrials.gov NCT04562350; https://clinicaltrials.gov/ct2/show/NCT04562350.

International Registered Report Identifier (IRRID)
PRR1-10.2196/25062