bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Landscape of Mucopolysaccharidosis in Southern and Eastern European Countries: A Survey from 19 Specialistic Centers.
Authors: Tylki-Szymańska Anna , Almássy Zsuzsanna , Anastasiadou Violetta Christophidou , Avdjieva-Tzavella Daniela , Barisic Ingeborg , Cerkauskiene Rimante , Cuturilo Goran , Djiordjevic Maja , Gucev Zoran , Hlavata Anna , Kieć-Wilk Beata , Magner Martin , Pecin Ivan , Plaiasu Vasilica , Samardzic Mira , Zafeiriou Dimitrios , Zaganas Ioannis , Lampe Christina 
Published: November 2021

Abstract
Background: Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders caused by defects in genes coding for different lysosomal enzymes which degrade glycosaminoglycans. Impaired lysosomal degradation causes cell dysfunction leading to progressive multiorgan involvement, disabling consequences and poor life expectancy. Enzyme replacement therapy (ERT) is now available for most MPS types, offering beneficial effects on disease progression and improving quality of life of patients. The landscape of MPS in Europe is not completely described and studies on availability of treatment show that ERT is not adequately implemented, particularly in Southern and Eastern Europe. In this study we performed a survey analysis in main specialist centers in Southern and Eastern European countries, to outline the landscape of disease management in the region and understand ERT implementation, with particular reference to MPS IVA.Results: 19 experts from 14 Southern and Eastern European countries in total responded to the survey. Results outlined a picture of MPS management in the region, with a high number of MPS patients managed in the centers and a high level of care. MPS II was the most prevalent followed by MPS IVA, with a particular high number of adult patients. The study particularly focused on management and treatment of MPS IVA patients. Adherence to current European Guidelines for follow-up of MPS IVA patients is generally adequate, although some important assessments are reported as difficult due to the lack of MPS skilled specialists. Availability of ERT in Southern and Eastern European countries is generally in line with other European regions, even though regulatory, organizational and reimbursement constrains are demanding. Conclusions: The landscape of MPS in Southern and Eastern European countries is generally comparable to that of other European regions on the epidemiology, treatment accessibility and follow up difficulties. However, issues limiting ERT availability and reimbursement should be simplified, to start treatment as early as possible and make it available for more patients. Besides, educational programs dedicated to specialists should be implemented, particularly for pediatricians, clinical geneticists, surgeons, anesthesiologists and neurologists.
Identification and fine-mapping of a major QTL (qRtsc8-1) conferring resistance to maize tar spot complex and production markers validation in breeding lines
Authors: Ren Jiaojiao , Wu Penghao , Huestis Gordon M. , Zhang Ao , Qu Jingtao , Liu Yubo , Zheng Hongjian , Alakonya Amos , Dhliwayo Thanda , Olsen Michael Scott , Vicente Félix San , Prasanna Boddupalli Maruthi , Chen Jiafa , Zhang Xuecai 
Published: November 2021

Abstract
Tar spot complex (TSC) is a major foliar disease of maize in many Central and Latin American countries and leads to severe yield loss. To dissect the genetic architecture of TSC resistance, a genome-wide association study (GWAS) panel and a bi-parental doubled haploid population were used for GWAS and selective genotyping analysis, respectively. A total of 115 SNPs in bin 8.03 were detected by GWAS and three QTL in bins 6.05, 6.07, and 8.03 were detected by selective genotyping. The major QTL qRtsc8-1 located in bin 8.03 was detected by both analyses, it explained 14.97% of the phenotypic variance. To fine-map qRtsc8-1, the recombinant-derived progeny test was implemented. Recombinations in each generation were backcrossed, and the backcross progenies were genotyped with Kompetitive Allele Specific PCR (KASP) markers and phenotyped for TSC resistance individually. The significant tests for comparing the TSC resistance between the two classes of progenies with and without resistant alleles were used for fine-mapping. In BC5 generation, qRtsc8-1 was fine mapped in an interval of ~721 kb flanked by markers of KASP81160138 and KASP81881276. In this interval, the candidate genes GRMZM2G063511 and GRMZM2G073884 were identified, which encode an integral membrane protein-like and a leucine-rich repeat receptor-like protein kinase, respectively. Both genes are involved in maize disease resistance responses. Two production markers KASP81160138 and KASP81160155 were verified in 471 breeding lines. This study provides valuable information for cloning the resistance gene, it will also facilitate the routine implementation of marker-assisted selection in the breeding pipeline for improving TSC resistance.
Dosimetric Evaluation of a Set of Specifically Designed Grids for Treating Subcutaneous Superficial Tumor with 6 and 18 MeV Electron Beam External Radiotherapy
Authors: Entezari Kamran , Hashemi Bijan , Mahdavi Seied Rabi 
Published: November 2021

Abstract
Background: Conventional electron beam radiotherapy used for treating superficial cancer tumors suffers from the disadvantage of low skin sparing effect. Furthermore, increasing electron energy for treating deeper-seated tumors leads to significant increase of skin dose. To overcome this, various grids are recommended for electron beam radiotherapy of subcutaneous tumors. However, appropriate grids are required to be designed for decreasing skin dose while delivering uniform high doses to deep-seated superficial tumors. Our goal was to design, examine and propose appropriate grid(s) for optimum electron beam radiotherapy of subcutaneous tumors with the best skin sparing with 6 and 18 MeV energies.Materials and Methods: Relevant dosimetric characteristics were determined and analyzed for five grids manufactured from dry lead having various cavity diameters (1.5, 2.0, 2.5, 3.0, 3.5 cm) and shielded areas (0.3, 0.4, 0.5, 0.6, 0.7 cm) among the cavities but the same fraction of cavity/open (68%) and shielded/closed (38%) areas under the grid plates. Isodose distributions and dose profiles resulted from the grids were investigated using EDR2 films and MATLAB software. Results: The grids with 2 and 2.5 cm diameter cavities and 0.4 and 0.5 cm shielded areas were the most appropriate grids for 6 and 18 MeV radiotherapy, respectively. With these grids, the 100% PDDs (percentage depth doses) located at 1.25 and 2.5 cm for an open filed (without the grids) were moved down to 1.87 and 5.4 cm for 6 and 18 MeV energies, respectively. Furthermore, the proposed grids provided the least peak to valley dose variations hence the most uniform doses delivered at their relevant depths of treatment. Conclusions: To decrease the skin dose in 6 and 18 MeV electron beam radiotherapy of superficial subcutaneous tumors, various home-made grids were designed and investigated. The most appropriate grids (having 2 and 2.5 cm cavity diameters for 6 and 18 MeV, respectively) provided the optimum dose delivery for superficial subcutaneous tumors locating around 1.5 and 5 cm depth for 6 and 18 MeV energies. Our comprehensive study provides reliable results that could be considered and developed more for a wider range of MeV electron grid therapies in routine clinical practices.
Determinants of Market Orientation and Market Participation: Agricultural Commercialization in Central and North Gondar, Ethiopia
Authors: Koye Tigabu Dagnew , Mekie Taye Melese , Dessie Abebe Birara , Malede Tegegne Debas 
Published: November 2021

Abstract
In Ethiopia, agricultural transformation is faced with many challenges such as poor infrastructure especially in rural area where huge agricultural activities are carried out, poor institutional services, lack of awareness of farmers on value addition of goods and so on. To fill this knowledge gap, this study was aimed the determinants of market orientation and market participation in Central and North Gondar Rural Ethiopia separately. The data were collected a sample of 344 households selected using multistage purposive and random sampling techniques. Seemingly unrelated regression (SUR) and Tobit regression models were employed. The SUR model estimation indicated adult equivalent, chemical fertilizer users and tropical livestock unit (TLU) affect both market oriented cash and stable crops positively, while child dependency ratio, cultivated land, the distance to the market and road affect both market oriented cash and stable crops negatively. Level of education (grading), and irrigation users affect market oriented cash and stable crops positively, respectively. The empirical results of Tobit model show that cultivated land, land allocated to staples, off/non-farm income and irrigation user affect crop commercialization positively. Based on the findings, the study suggest that farmers should keep going to employ an additional off-farm income activities, improve rural urban roads, employ agricultural intensification, and the government should be supplied chemical fertilizer in sufficient amount and on time at reasonable price to improve farmers’ crop production.
A Nomogram to Predict Survival and Determine Necessity of Induction Chemotherapy for Patients With Stage T3-4N1 Nasopharyngeal Carcinoma
Authors: jiang yuting , chen kaihua , yang jie , liang zhongguo , qu song , li ling , zhu xiaodong 
Published: November 2021

Abstract
BackgroundThe value of induction chemotherapy (IC) is still controversial for stage T3-4N1 nasopharyngeal carcinoma (NPC) patients receiving concurrent chemoradiotherapy (CCRT). Therefore, we established a nomogram to predict clinical outcome and explore the therapeutic efficiency of IC. MethodsOverall, 699 stage T3-4N1 NPC patients treated with CCRT with or without IC between January 2010 and December 2018 were examined. Overall survival (OS) was regarded the main endpoint. A nomogram was developed including prognostic variables selected by multivariate analysis. The Harrell Concordance Index (C-index), calibration curves, and time-dependent receiver operator characteristic (td-ROC) curves were performed to assess the predictive ability of the nomogram. All patients were divided into high- and low-risk groups based on the optimal cutoff of risk score to investigate the role of IC. Resultshe nomogram yielded C-index of C-index of 0.725 (95% CI: 0.672–0.778) in the training and 0.647 (95% CI: 0.580–0.742) in the validation cohort. Calibration curves for 3- and 5-year OS rate suggested a good association between the nomogram predicted and actual observed probabilities. Td-ROC analysis demonstrated good discriminatory ability. In the high-risk group, no statistically significant difference was observed between patients receiving IC + CCRT and those with CCRT alone. However, in the low-risk group, the applying of IC was associated with worse locoregional recurrence-free survival (LRRFS) and distant metastasis-free survival (DMFS). Conclusionse established and validated a nomogram for LA-NPC patients with N1 disease to predict OS and determine necessity of IC, which has satisfactory prognosis predicting ability and clinical practicability. The use of IC for stage T3-4N1 NPC patients should be considered carefully.
Environmental indicators combined with risk analysis to evaluate potential wildfire incidence on the Dadu Plateau in Taiwan
Authors: Lin Chao-Yuan , Shieh Pei-Ying , Wu Shao-Wei , Wang Po-Cheng , Chen Yung-Chau 
Published: November 2021

Abstract
Wildfire is a common disaster in the world, and it has a considerable impact on the safety of residents and ecological disturbance. Periodic wildfires are an urgent problem to be solved. This research uses big data from relevant departments to extract environmental indicators that affect wildfires, including satellite images, meteorological observations, and field surveys and establishes a risk model for the Spatio-temporal distribution of wildfires based on risk analysis. Previous studies using Differenced Normalized Burn Ratio (dNBR) to assess fire severity and distinguish wildfire ruins did not deal with the impact of atmospheric humidity on dNBR values. In this study, an adjustable fire threshold was developed to enable dNBR to improve the accuracy of identifying wildfire locations. Regarding the temporal distribution of wildfire risks, environmental vulnerability cannot specifically reflect the frequency of actual wildfires. If the hazard degree is introduced to calculate the wildfire risk, the coefficient of determination can be increased from 0.49 to 0.79. The verification of the village boundary zone depicts that the risk analysis can effectively show the temporal and spatial distribution of wildfire hotspots. On this basis, a village-level wildfire disaster prevention strategy can be formulated.
Notch1 Signaling Contributed To TLR4-Triggered NF-kB Activation In Macrophages
Authors: Li Li , Jin Jin-hua , Liu Han-ye , Ma Xiao-fei , Wang Dan-dan , Song Yi-lan , Wang Chong-yang , Jiang Jing-zhi , Yan Guang-hai , Qin Xiang-zheng , Li Liang-chang 
Published: November 2021

Abstract
Background: Macrophages substantially shape the development, progression, and complications of inflammation-driven diseases. Although numerous researches support a critical role for Notch signaling in most inflammatory diseases, there is limited data on the role of Notch signaling in TLR4-induced macrophage activation and the interaction of Notch signaling with other signaling pathways(e.g., the NF-kB pathway during inflammation) in macrophages. Methods and Results: In this study, we confirmed that stimulation with TLR4 ligand LPS up-regulates Notch1 expression in RAW264.7 monocyte/macrophage-like cell line. LPS also induced the expression of Notch target genes Notch1 and Hes1 mRNA in macrophages, suggesting that TLR4 signaling enhances Notch pathway activation. The upregulation of Notch1, NICD, and Hes1 protein by LPS treatment was inhibited by the Notch1 inhibitor of DAPT. The increase of TNF-a, IL-6, and IL-1b induced by LPS was inhibited by DAPT while jagged1, the Notch1 ligand, rescued them. Furthermore, the suppression of Notch signaling by DAPT up-regulated CYLD expression but down-regulated TRAF6, IKKa/bphosphorylation, and subsequently phosphorylation and degradation of IκB-α, indicating the inhibition of TLR4-triggered NF-kB activation by DAPT. Interestingly, DAPT showed no inhibitory effect on the increase of MyD88 expression induced by LPS in our study. Conclusions: Our study shows that the stimulation of macrophages via the TLR4 signaling cascade triggers the activation of Notch1 signaling, which regulates the expression patterns of genes involved in pro-inflammatory responses through by activating NF-kB. It may be dependent on the CYLD-TRAF6-IKK pathway. The Notch1 signaling may be considered as a potential therapeutic target against infectious and inflammatory driven diseases.
SULF2 Is a Prognostic Biomarker and Correlated with Tumor Associated Macrophages in Gastric Cancer
Authors: Wang Tong-Bo , Li Ze-Feng , Zhang Xiao-Jie , Sun Chong-Yuan , Fei He , Chen Ying-Tai , Guo Chun-Guang , Zhao Dong-Bing 
Published: November 2021

Abstract
Background: Recently, the mutual effects of tumor cells and the tumor immune microenvironment have been identified as key factors in promoting cancer progression. Sulfatase 2 (SULF2) encodes an extracellular endoglucosamine‑6-sulfatase, which could remodel the highly sulfated domains of heparan sulfate. The abnormal expression of SULF2 is reported to play an important role in the carcinogenesis of many kinds of cancer. However, the prognostic value of SULF2 and its correlation with immune cell infiltration in gastric cancer (GC) remain unclear. Results: SULF2 expression was significantly increased in GC compared with gastric normal tissue, especially in the advanced stage GC. In addition, high SULF2 expression significantly predicted an unfavorable prognosis in GC patients (overall survival P=0.0074), particularly who had metastatic lymph nodes. Besides, pathway analyses of SULF2 in GC revealed SULF2 may take part in extracellular structure organization, cell-cell adhesion and proteoglycans in cancer, etc. Importantly, the expression level of SULF2 was found to be positively correlated with the infiltration levels of tumor associated macrophages (TAMs). Moreover, SULF2 expression in GC positively correlated with expression of several immune cell markers, including monocyte markers, TAMs markers and programmed cell death 1 ligand 1 (PD-L1), suggesting its role in regulating tumor immunity.Conclusion: This study identified distinct expression and prognostic values of SULF2 in GC using public databases. Significantly, our findings shed light on the role of SULF2 in GC progression and provided an underlying mechanism that SULF2 expression might modulate tumor immunity by regulating the infiltration of TAMs in GC.
Preparation of Dual Z-Scheme Bi2MoO6/ZnSnO3/ZnO Heterostructure Photocatalyst for Efficient Visible Light Degradation of Organic Pollutants
Authors: Gao Yuanyingxue , Li Li , Zu Wenlin , Sun Yingru , Guan Jiahui , Cao Yanzhen , Yu Haixia , Zhang Wenzhi 
Published: November 2021

Abstract
An important means of achieving efficient charge separation and improving photocatalytic activity is the construction of heterostructures. In this study, the Bi2MoO6/ZnSnO3/ZnO heterostructure photocatalyst was synthesized by the hydrothermal method. The synthesized samples were carefully examined by X-ray photoelectron spectroscopy (XPS), X-ray diffraction (XRD), scanning electron microscope (SEM), high-resolution transmission electron microscopy (HR-TEM), photoluminescence (PL), and other analytical techniques. Meanwhile, the photocatalytic performance was further evaluated by multi-mode photocatalytic degradation with crystal violet (CV). The results show that the composite material has a relatively homogeneous cubic structure in size and shape. In the cubic structure, a heterogeneous structure exists between Bi2MoO6, ZnSnO3 and ZnO. Simultaneously, the dramatic changes in physical morphology, such as the specific surface area and particle size of the composites, led to a unique set of properties, such as a significant climb in light absorption properties and superior photocatalytic activity. In addition, the Bi2MoO6/ZnSnO3/ZnO composite material shows lower photoluminescence intensity, smaller arc radius, and stronger photocurrent response compared to ZnO, Bi2MoO6 and ZnSnO3/ZnO. Meanwhile, Bi2MoO6/ZnSnO3/ZnO shows higher photocatalytic efficiency for crystal violet (CV) and tetracycline hydrochloride (TC) and maintains good stability after 3 cycles of photodegradation experiments. Based on experimental results, the existence of heterojunctions between ZnO, ZnSnO3 and Bi2MoO6 and the possible photocatalytic mechanism for the degradation of crystal violet by dual Z-scheme composites are proposed. In conclusion, this study provides a feasible strategy for the photocatalytic degradation of organic pollutants by introducing ZnSnO3 and Bi2MoO6 to successfully construct composite catalysts with dual Z-scheme heterostructures.
Developing Co-management for Conservation and Local Development in China’s National Parks: Findings from Focus Groups in the Sanjiangyuan Region
Authors: Ma Ting , Swallow Brent , Foggin J. Marc , Zhong Linsheng , Sang Weiguo 
Published: November 2021

Abstract
Environmental protection in China has progressed significantly in the past decades, including introduction of more collaborative approaches in the management of protected areas and the establishment of a new national park system. Many milestones have been achieved. While such developments are driven largely by national and global goals, the people who are most affected are those who reside in the protected landscapes. A range of strategies have been proposed and tried in relation to local development, with many important lessons learned, yet little has been heard to date directly from the community stakeholders themselves. In this study we report on feedback and recommendations received from focus groups in vicinity of China’s first national park, Sanjiangyuan, regarding lived experiences of “community co-management” by Tibetan herders and local officials. Overall, the most recent National Park model is deemed successful, albeit with some notable perceived limitations. Focus group participants recommend more balanced compensation opportunities including for communities living outside but in close proximity to the paranagement and health care) and establishing a more effective compensation or insurance system to offset econok, eased restrictions on ecotourism, provision of public services for communities in the park (especially waste mmic losses due to wildlife damage.
Association Between Type 2 Diabetes and Classification of Periodontal Disease Severity in Japanese Men and Women: A Cross-Sectional Study
Authors: Dewake Nanae , Iwasaki Yukiko , Taguchi Akira , Udagawa Nobuyuki , Yoshinari Nobuo 
Published: November 2021

Abstract
Background: To evaluate the association between type 2 diabetes and periodontal disease severity using the rate of alveolar bone loss (ABL) and high-sensitivity C-reactive protein (hs-CRP) value as indices. Methods: In this cross-sectional study of 372 subjects (mean age ± SD, 53.2 ± 11.8 years) from a Japanese hospital, we measured ABL and number of teeth on either panoramic radiographs or intraoral dental radiographs of all teeth. A trial classification of periodontal disease was defined as follows: I (ABL < 25%, clinically mild); II (ABL ≥ 25%, <35%, moderate); III (ABL ≥ 35%, severe); and A (hs-CRP < 440 ng/ml, mild inflammation); B (hs-CRP ≥440 ng/ml, <1,020 ng/ml, moderate inflammation); and C (hs-CRP ≥ 1,020 ng/ml, severe inflammation). Periodontal disease severity was classified into nine groups by combining ABL and hs-CRP. Results: Forty-eight subjects had type 2 diabetes; 324 did not. Univariate analysis showed that type 2 diabetes was significantly associated with age, sex, body mass index, number of teeth, ABL, hs-CRP, and periodontal disease severity. Multivariate analysis showed significant associations between type 2 diabetes and the groups with high severity of periodontal disease. In receiver operating characteristic (ROC) curve analysis predicting the presence of diabetes, area under the ROC curve was 0.762 (95%CI = 0.688 - 0.835) for ABL, and 0.709 (95%CI = 0.635 - 0.784) for hs-CRP, which was significant. Conclusions: This study showed that diabetes could be associated with a periodontal disease severity classification using the combination of ABL and hs-CRP.