bluuebunny/crossref_metadata_2025_split huggingface dataset:
A cohort study on deficiency of ADA2 from China
Authors: Li Guomin , Han Xu , Wu Ye , Wang Wei , Tang Hong-xia , Lu Mei-ping , Tang Xue-mei , Lin Yi , Deng Fan , Yang Jun , Wang Xin-ning , Liu Cong-cong , Zheng Wen-jie , Wu Bing-bing , Zhou Fang , Luo Hong , Zhang Liang , Liu Hai-mei , Guan Wan-zhen , Wang Shi-hao , Tao Pan-feng , Jin Tai-jie , Fang Ran , Wu Yuan , Zhang Jie , Zhang yao , Zhang Tian-nan , Yin Wei , Guo Li , Tang Wen-jing , Chang Hong , Zhang Qiu-ye , Li Xiao-zhong , Li Jian-guo , Zhou Zhi-xuan , Yang Si-rui , Yang Kang-kang , Xu Hong , Song Hong-mei , Zhou Qing , Sun Li 
Published: July 2022

Abstract
Purpose: Deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive autoinflammatory disorder caused by biallelic loss-of-function mutations in ADA2, characterized by systemic inflammation, vasculitis, immunodeficiency, and/or hematologic abnormalities. Methods: A retrospective analysis of patients with DADA2 diagnosed by whole exome sequencing at 17 rheumatology centers across China was conducted. Clinical characteristics, laboratory findings, genotype and treatment responses were analyzed.Results Thirty patients with DADA2 were enrolled in this cohort between January 2015 and December 2021. Median age at onset was 4.3 years and the median age at diagnosis was 7.8 years. All but one patient presented during childhood and two subjects in the cohort died from DADA2-related complications. The patients presented with systemic inflammation (92.9%), vasculitis (86.7%), and hypogammaglobinemia (73.3%) while one patient with pure red cell aplasia (PRCA) and bone marrow failure (BMF) with variable cytopenia was observed in our cohort. Twenty-three (76.7%) patients received TNF inhibitors and all achieved clinical remission. Homozygous variants and compound heterozygous variants were identified in 5 and 25 patients, respective. Two patients received hematopoietic stem cell transplantation (HSCT), and both got remission. Among 39 unique variants in our cohort, six is novel.Conclusion: In order to establish the diagnosis early and prevent devastating clinical outcomes, genetic screening and/ or testing of adenosine deaminase enzymatic activity should be performed in patients with suspected clinical features. TNF inhibitors can be first line drug for patients with vasculitic DADA2. HSCT is an alternative choice for patients with refractory to TNF inhibitors or hematological disorders.
Computational Screening of Transition Metal-Doped CdS for Photocatalytic Hydrogen Production.
Authors: Vega Lourdes , Li Yuting , Bahamon Daniel , Sinnokrot Mutasem 
Published: July 2022

Abstract
A novel computational screening study of single transition metal (TM), TM-doped (TM@CdS), and dual TMs-doped (TM1-TM2@CdS) on CdS (110) surfaces via DFT calculations is presented, focusing on their stability and catalytic activity, searching for efficient photocatalysts for hydrogen production. Criteria based on key performance descriptors allowed to fine-tune the selection. Results indicate that TM dopants can reduce the energy band gap and enhance impurity d-states. Pt, Rh and Pd were found to be the best dopant in TM@CdS, since their |ΔGH| is 80% smaller compared to the pristine CdS surface. Moreover, TM1-TM2@CdS catalysts show better performance than TM@CdS for the hydrogen evolution reaction (HER) due to synergistic effects of the two TM, where Co-Pt, Pd-Pt and Co-Rh@CdS significantly reduced |ΔGH| to less than 0.1 eV. Results point out four promising novel catalysts (i.e., Co@CdS, Co-Pt@CdS, Co-Rh@CdS, Rh-Ag@CdS) with very good performance in HER, to be further explored in experimental studies.
USP22-FASN Axis Derepression by p53 Loss under Oxidative Stress Drives Lipogenesis and Colorectal Tumorigenesis
Authors: Han Zelong , Liu Ming , Zeng Kunlin , Chen Yanwen , Wang Li , Chen Xiaoxia , Luo Yaxin , Zeng Yu , Zhan Hongchao , Zhang Keqin , Zhu Xiaoxia , Liu Side , Luo Xiaobei , Zhou Aidong 
Published: July 2022

Abstract
Overproduction of reactive oxygen species (ROS) and aberrant lipid metabolism are established hallmarks of cancers; however, the role of ROS in lipid synthesis during tumorigenesis is almost unknown. Herein, we show that ROS critically regulates lipid synthesis and thus controls colorectal tumorigenesis through a p53-dependent mechanism. In p53 wild-type colorectal cancers (CRCs), ROS-induced p53 expression represses the transcription of deubiquitinase USP22, which otherwise deubiquitinates and stabilizes Fatty Acid Synthase (FASN), and thus inhibits fatty acid synthesis and tumor growth. Whereas, in p53-deficient CRC cells, ROS-mediated inhibition of USP22 is relieved, leading to FASN stabilization, which thus promotes lipid synthesis and colorectal tumorigenesis. In human CRC specimens, the expression levels of USP22 and FASN are positively correlated and high USP22/FASN level predicts poor prognosis. Our study demonstrates that ROS critically controls lipid synthesis and tumorigenesis through the USP22-FASN axis in a p53-dependent manner, and that targeting the USP22-FASN axis may represent a potential strategy for the treatment of colorectal cancer.
Altered Neutrophil-to-Lymphocyte Ratio in Patients with Non-Affective First Episode Psychosis and its Relationship with Symptom Severity and Cognitive Impairment
Authors: Leung Kwan Keung , Wong Yip Chau , Shea Ka Sin , Chan Sheung Chun , Chang Wing Chung , Mo Yi Man Flora , Chan Sau Man Sandra 
Published: July 2022

Abstract
Signatures of immune dysregulation as clinical biomarker for psychosis have remained unclear. We aimed to compare the Neutrophil-to-lymphocyte ratio (NLR) of patients with acute non-affective first-episode psychosis (FEP) with healthy controls after accounting for emotional states, and explore their associations with symptom severity, onset profile and cognitive functions. The NLR was enumerated from complete blood count taken within a week of assessment. All FEP patients were assessed with Positive and Negative Syndrome Scale (PANSS) and the Clinician Global Impression-Severity (CGI-S) scores. Verbal memory and executive functions were assessed with the Cambridge Neuropsychological Test Automated Battery. Prevailing emotional state was measured with Beck Depression Inventory-II and Beck Anxiety Inventory. 27 eligible subjects screened out of 79 consecutive FEP patients with their age-/sex-matched controls were recruited. The FEP patients had an NLR of 1.886 higher than controls after accounting for emotional states. The NLR of FEP patients was positively associated with PANSS positive symptom, disorganization and excitation scores, as well as CGI-S scores. There was no significant correlation between NLR with duration of untreated psychosis and cognitive performances. These findings support using NLR as clinical biomarker in FEP, purporting further prospective study to measure NLR changes in the course of treatment.
Knowledge, Attitude and Practice of Animal Handlers towards Antibiotic Usage and Antimicrobial Resistance in Peninsular Malaysia
Authors: Chai Min Hian , Zaidi Aida Afiqah , Hashim Siti Noorfazira , Ariffin Siti Mariam Zainal , Choong Siew Shean , Lani Mohd Nizam , Aziz Nor Azlina Abdul , Roslan Farah Syahrain , Ghazali Mohd Faizal 
Published: July 2022

Abstract
Background: Antimicrobial resistance (AMR) has become one of the most significant global public health threats. The lack of effective legislation and strict regulations on drug usage in many developing countries as well as self-prescription of antimicrobials at the maximum to animals by their owner have contributed to the rapid emergence of AMR worldwide. Thus, educating stakeholders about the threats of AMR is an important step in controlling the emergence of antibiotic resistance. As there is a wide cross-national difference in the antibiotic usage as well as knowledge level regarding AMR, it is crucial to continuously assess the knowledge, attitude, and practices of the key stakeholders concerning AMR to tailor intervention strategies to address this local issue. The present study aims to assess the knowledge, attitude and practice of Peninsular Malaysian animal handlers towards antibiotic usage practice and antibiotic resistance.Methods: A cross-sectional study was conducted from August 2019 to February 2022 among animal handlers in Peninsular Malaysia. A questionnaire containing a list of questions covering the socio-demographic, knowledge, attitude and practice sections on antibiotic usage and antibiotic resistance was distributed to animal handlers in Peninsular Malaysia via the online platform and face-to-face interviews.Results: A total of 963 animal handlers from several states of Peninsular Malaysia participated in this study. The respondents showed a moderate knowledge score level when being asked about antibiotics and antibiotic resistance. It appears that the majority of the respondents knew about antibiotics. However, the respondents were not well-aware of antibiotic resistance. In addition, the respondents showed low attitude and moderate practice levels when using antibiotics, which may lead to the emergence of antibiotic-resistant bacteria in the long term. Furthermore, it was revealed that the KAP awareness of the respondents from different age groups, ethnicity and educational backgrounds were significantly different. Male, older individuals (30 years and above), minority ethnics and non-university graduates were found to have lower KAP scores. Nonetheless, the knowledge level of the respondents was positively correlated with their attitude and practice scores. Conclusion: The animal handler respondents in Peninsular Malaysia showed moderate knowledge, attitude and practice levels towards antibiotic usage and antibiotic resistance.
Evidence-based consensus recommendations for skin care in healthy, full-term neonates in India
Authors: Gupta Piyush , Nagesh Karthik , Garg Pankaj , Thomas Jayakar , Suryawanshi Pradeep , Sethuraman Giridhar , Hazarika Rashna Dass , Verma Rahul , Kumar C Suresh , Kumari Shantha , Taneja Sunil , Chavhan Vaishali , Thakor Priti , Pandita Ankita 
Published: July 2022

Abstract
Background: Neonatal skin care practices that are often guided by personal experience and preferences result in substantial variation in the practices being followed across hospital settings. The objective of this consensus recommendations was to provide guidance to health care practitioners on evidence-based neonatal skin care practices for adoption in hospitals: from delivery to discharge.Methods: A Scientific Advisory Board meeting on “Evidence-based Neonatal Skin Care Practices and Protocols” was held in December 2020 with an expert panel comprising neonatologists, pediatricians, obstetricians and gynaecologists and a pediatric dermatologist were invited. A comprehensive literature search was performed using the PubMed and Google Scholar databases to source relevant evidence.Results: Recommendations were developed on critical aspects of skin care in healthy full-term neonates, including cleansing at birth, skin-to-skin care, cord care, diaper area care, initial and routine bathing, use of cleansers and emollients, and the criteria to choose appropriate skin care products. The newborn’s first bath should occur only once cardiorespiratory and thermal stability have been achieved, anywhere between 6 to 24 hours after birth. Bathing with water alone or water with a mild liquid cleanser demonstrated appropriate for newborn does not impact the developing skin barrier. The use of emollients to maintain and enhance skin barrier function was encouraged for neonates with risk factors that can affect skin barrier integrity. Skin assessment should become an integral part of routine neonatal care. Skin Care Advice with “Do’s and Don’ts” should be a part of the neonatal discharge checklist. The importance of rigorous quality control, high-quality clinical trials to evaluate the safety and efficacy of baby products, usage of products that contain only ingredients and which are formulated appropriately for newborn babies, and full label transparency for baby products were highlighted. The panel further identified gaps in the existing literature and discussed the scope for future research. Conclusions: The consensus recommendations may help standardize evidence-based skin care for healthy full-term neonates in Indian hospital settings. This may also improve the quality of care that the neonates receive in the hospital and facilitate improvement in overall neonatal health outcomes.
Salinity mediates the effects of groundwater and seasonal streamflow on the symbiotic nitrogen fixation of a dryland phreatophyte
Authors: Li Meimei , Petrie Matthew D. , Chen Hao , Zeng Fanjiang , Ahmed Zeeshan , Sun Xibin 
Published: July 2022

Abstract
Purpose
Biological nitrogen fixation of deep-rooted legumes is a major pathway of nitrogen (N) inputs to drylands ecosystems. Water sources including groundwater and streamflow are vital to the growth of deep-rooted legumes in drylands, but it is unclear how their different availability and biochemical properties may influence plant N fixation.
Methods
We established two intersecting sampling transects at the southern fringe of the Taklimakan Desert to study the effects of the groundwater depth and distance to a seasonally active river channel on the N fixation rate of Alhagi sparsifolia, a dominant phreatophytic legume species. We quantified the N fixation rate of A. sparsifolia using the 15N natural abundance method, and related variation in N fixation to water source use and soil biochemical properties along the two transects.
Results
The N fixation rate of A. sparsifolia decreased with groundwater depth, and increased with distance from the river channel. Instead of variation in water source or nutrient concentrations, we found that gradients in salinity formed by groundwater flow and streamflow were most associated with the spatial pattern in N fixation of A. sparsifolia.
Conclusions
In many dryland regions, water availability controls the spatial variability of symbiotic N fixation along hydrological gradients. Our study system provides a contrasting condition where salinity most strongly regulates N fixation. In addition to understanding the important role of variation in water source and nutrient availability, our study suggests that the impact of soil salinity in mediating the N fixation of dryland ecosystems is deserving of increased attention.
Several characterizations of third degree semiclassical linear forms of class two appearing via cubic decomposition
Authors: Khalfallah Mohamed 
Published: July 2022

Abstract
In this work we consider orthogonal polynomials via cubic decomposition in the framework of the third degree semiclassical class. Based on their third degree character, we give a complete description, by using the formal Stieltjes function and the moments, of semiclassical linear forms of class two arising from the cubic decomposition W3n(x) = Pn(x3); n ≥ 0. We focus our attention on the link between these forms and the strict third degree classical forms Vqk,l= J (k + q/3; l -q/3), q ∈{1,2},k,l ∈Z with k+ l≥ -1. All of them are rational transformations of the Jacobi form V = J (-2/3;-1=3).
Barley metallothionein isoforms, MT2b2 and MT4, differentially respond to photohormones in barley aleurone layer and their recombinant forms show different affinity for binding to zinc and cadmium
Authors: Pourjalali Zahra , Shahpiri Azar , Golkar Pooran 
Published: July 2022

Abstract
Metallothioneins (MTs) are metal-binding proteins that have important roles in the homeostasis of heavy metals. In this study, the two MT genes was studied in response to phytohormones using the barley aleurone layer as a kind of model system. The aleurone layer was isolated from barley embryo-less half grains and was incubated for 24 h with different phytohormones. Based on the results the genes encoding HvMT2b2 and HvMT4 were down-regulated through gibberellic acid (GA), while they were and up-regulated through salicylic acid (SA). Despite this, these two genes were differentially expressed to other hormones. Furthermore, the proteins HvMT2b2 and HvMT4 were heterologous expressed as GST-fusion proteins in E. coli. The HvMT4 and HvMT2b2 heterologous expression in E. coli gives rise to 10- and 3-fold improvements in the accumulation capacity for Zn2+, respectively. Whereas the transgenic E. coli strain that expresses HvMT2b2 could accumulate Cd2+ three-fold higher than control. The expression of HvMT4 did not affect the accumulation of Cd2+. HvMT4 which is known as seed-specific isoform seems to be able to bind to Zn2+ with good affinity and cannot bind Cd2+. In comparison, HvMT2b2 was able to bind both Zn2+ and Cd2+. Therefore HvMT4 could serve a noteworthy role in zinc storage in barley seeds. The expression of HvMT4 is induced by SA 30-fold, concerning the untreated aleurone layer. Such results could provide good insights for the assessment of the effects of phytohormones in the molecular mechanism involved in essential metal storage in cereal seeds.
Palifermin Compared to Supersaturated Calcium Phosphate Rinse in Prevention of Severe Oral Mucositis after Stem Cell Transplantation
Authors: Hadid Tarik , Al-Katib Ayad , Binongo Jose , Berteotti Gina M. , Fazal Salman , Rossetti James M. , Lister John 
Published: July 2022

Abstract
Purpose: Oral mucositis (OM) is a common, debilitating complication of conditioning regimens for hematopoietic stem cell transplantation (HSCT). Supersaturated calcium phosphate rinse (SCPR) and palifermin have shown efficacy in preventing severe OM. However, whether their efficacy differs is unknown. We aimed to compare the efficacy of SCPR and palifermin in HSCT patients receiving myeloablative conditioning. Methods: A comprehensive review of our institutional database was performed to identify patients who received myeloablative-conditioning therapy over 5 years. Most patients received Fludarabine, Busulfan and total body irradiation (FBT). Results: We identified 26 patients who received SCPR and 122 patients who received palifermin for OM prophylaxis. The prevalence of World Health Organization (WHO) grade 3 or 4 OM was significantly lower in the palifermin group (57% vs 100%, p=0.01). In addition, the palifermin group had lower WHO grade 4 OM (22% vs 62%, p=0.0006). The overall prevalence of OM was not significantly different between the two groups (86% for palifermin group vs 100% for SCPR arm, p=0.15). Subgroup analyses demonstrated improved outcomes with palifermin, regardless of age, sex, disease status, donor type, and primary diagnosis. Conclusion: As compared to SCPR, the use of palifermin is associated reduced severity of OM in HSCT patients receiving radiotherapy-based myeloablative conditioning.
Factors affecting knowledge of autism spectrum disorder among pediatric residents in eastern China: A cross-sectional study
Authors: Song Chao , Wu Lingling , Hong Yunxia , Chen Xiaoyang , Zhu Zhiwei 
Published: July 2022

Abstract
Background: There is a global increase in the prevalence of autism spectrum disorder (ASD). Early identification of ASD in children and intervention are key aspects in the management of ASD. However, early identification is partly dependent on knowledge on ASD among pediatricians. This study analyzed the extent of ASD knowledge and its underlying factors among pediatric residents in eastern China, to provide a reference for medical education reforms. Methods: The study employed the Knowledge about Childhood Autism among Health Workers questionnaire. A total of 138 pediatric residents participated in the survey. Descriptive statistics were used to describe demographic characteristics and the four domains of the questionnaire. Univariate analysis was employed to assess impacts of the demographic characteristics on the questionnaire scores. On the other hand, multivariate regression analysis was used to analyze the correlation between the participants’ demographic characteristics and the questionnaire scores. Results: The average ASD cognitive score of 138 respondents was 13.38 ± 4.48. The ASD cognitive scores in female pediatric residents were higher compared to that in males (p<0.05). Residents who had obtained professional doctor qualification certificate were more than those without professional doctor qualification certificate (p<0.05). The ASD knowledge in the group which did not have rotation in both departments was lower than in the group which had rotation in both departments (p<0.05) as well as the group that had rotation in developmental and behavioral pediatrics department only (p<0.05). Our multivariate linear regression model demonstrated significant statistical differences (p<0.05), and showed that gender and systematic exposure to ASD knowledge had significant effects on cognitive scores (p<0.05). Conclusions: Most participants had relatively low levels of awareness and knowledge about ASD, especially on ASD comorbidities. Women, systematic learning of ASD knowledge in medical school, successful passing of the physician examination, and rotation in the developmental and behavioral pediatrics (DBP) department significantly influence the levels of ASD awareness and knowledge. It is, therefore, important to strengthen ASD education in medical students at the university level and make rotation in the DBP department a requisite for pediatric trainees.
Toughening modification of polylactic acid by long-chain hyperbranched polymers containing polycaprolactone end groups
Authors: Tie Rui , Huang Yansong , Jin Yujuan , Sun Jianjian , Tian Huafeng , Lei Xiao , Li Gongcheng , Men Shuang 
Published: July 2022

Abstract
The hyperbranched polyester were synthesized by the "one-step method" and grafted with caprolactone (ε-CL) to obtain long-chain hyperbranched polymers (LCHBPs-Cl). LCHBPs-Cl was then melt blended with PLA to study its effect on the toughness of PLA. Compared to the pure PLA, the most significant toughening effect was observed when the addition amount of LCHBPs-Cl was 2.0phr. The tensile strength was increased by 69.21% from 37MPa to 62.61 MPa, the impact strength was increased from 14.88kJ to 18.50kJ. The scanning electron microscope (SEM) images showed that the impact section of the blends was changed from smooth to rough, the brittle-ductile fracture transition occurred, and a large number of white wire drawing phenomena were produced. Due to the formation of topological entanglement and cohesive entanglement between the long-chain substituents of LCHBPs-Cl and PLA molecular chains, the plastic deformation of PLA was successfully improved.