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Haploinsufficiency of A20 (HA20): updates on the genetics, phenotype, pathogenesis and treatment

World Journal of Pediatrics 2020年第6期16卷 575-584页

作者： mei-ping Yu Xi-Sheng Xu Qing Zhou Natalie Deuitch mei-ping lu Department of Rheumatology Immunology and Allergy.Children's Hospital Zhejiang University School of MedicineHangzhou 310052China MOE Key Laboratory of Biosystems Homeostasis&Protection Life Sciences Institute.Zhejiang UniversityHangzhouChina Genetics Department Stanford UniversityCaliforniaUSA

Background A20,a protein encoded by the tumor necrosis factor alpha-induced protein 3 gene(TNFAIP3),plays a vital role in the negative regulation of inflammation and ***-of-function mutation in TNFAIP3 leads to a new described autoinflammatory disease-haploinsufficiency of A20(HA20).Since HA20 was first described in 2016,a number of new cases have been described in this literature,however,the disease and its pathogenesis are poorly *** review seeks to improve clinical recognition of this disorder,and promote both earlier diagnosis and initiation of targeted therapies to improve patients’*** We reviewed 26 papers about A20 and HA20,and we summarized genetic variants and clinical manifestations of a total of 61 reported patients from 26 families identified to have a genetic diagnosis of germline pathogenic variants in TNFAIP3/***,we discussed the pathogenesis and treatment of *** A total of 24 pathogenic variants of A20 had been *** was significant clinical heterogeneity,even among those with the same variants in *** to receiving a molecular diagnosis of HA20,patients had been diagnosed with Behcet's disease,rheumatoid arthritis,rheumatic fever,juvenile idiopathic arthritis,systemic lupus erythematosus,and even adult-onset Stills'*** patients with HA20 that presented with inflammatory signatures in NF-kB signaling were mostly responsive to *** HA20 is a monogenic autoinflammatory disease with highly variable clinical *** extensive heterogeneity makes it difficult to set a clinical diagnostic criteria,and genetic sequencing is necessary for a definitive diagnosis of HA20.

关键词： Autoinflammatory disease HA20 NF-κB signaling TNFAIP3 Treatment

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Juvenile dermatomyositis:advances in clinical presentation,myositis-specific antibodies and treatment

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World Journal of Pediatrics 2020年第1期16卷 31-43页

作者： Jian-Qiang Wu mei-ping lu Ann M.Reed Department of Rheumatology Immunology and Allergy Children's HospitalZhejiang University School of MedicineHangzhou 310052China Department of Pediatrics Division of Pediatric RheumatologyDuke University School of MedicineDurham 27710USA

Background Juvenile dermatomyositis(JDM)is a chronic autoimmune disease characteristic by inflammation of small vessels within the skin,muscle and vital *** the clinical features and treatment of JDM have not been fully *** sources Databases underwent through PubMed for articles about the clinical features,myositis-specific antibodiesof JDM and its treatment,and we selected publications written in English which were relevant to the topic of this *** Clinical features and myositis-specific antibodies may predict the severity and prognosis of *** the mortality rate has been lower with traditional treatments,such as corticosteroid,intravenous immunoglobulin,and diseasemodifying anti-rheumatic drugs such as methotrexate,their usages are *** biological therapies seem to be effective for refractory JDM patients,but more clinical trials are *** JDM is a sever disease of *** need to better understand recent advances of JDM in the context of clinical features including skin manifestations,muscle weakness and organ damage,myositis-specific antibodies and their associated outcomes and the treatment of disease.

关键词： Biologic agents Extramuscular manifestations Juvenile dermatomyositis Myositis autoantibodies

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Current status and recent advances on the use of ultrasonography in pediatric rheumatic diseases

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World Journal of Pediatrics 2020年第1期16卷 52-59页

作者： Li-Xia Zou mei-ping lu Lawrence Kwok Leung Jung Department of Rheumatology Immunology and Allergy Children's HospitalZhejiang University School of Medicine57 Zhugan LaneHangzhou 310003China Division of Rheumatology Children's National Medical Center in Washington D.C.111 Michigan Avenue NWWashingtonD.C.20010USA

Background Ultrasonography has become a useful tool in the clinical rheumatology settings in the last two decades,but its use has only recently been explored by pediatric *** aim of this article is to review the literature on the current status and recent advances on the use of ultrasound in pediatric rheumatic *** sources We have retrieved and reviewed the relevant articles from MEDLINE/PubMed databases published so far,on the applications of ultrasound in juvenile idiopathic arthritis(JIA),systemic lupus erythematosus,dermatomyositis,enthesitis,Sjogren's syndrome,and other rheumatic *** addition,articles on novel ultrasound imaging technology of potential use in pediatric rheumatology are also *** In JIA,ultrasound can be used to detect subclinical synovitis,to improve the classification of patients in JIA subtypes,to capture early articular damage,to monitor treatment response,and to guide intraarticular *** is also considered useful in other rheumatic disorders for the evaluation of musculoskeletal symptoms,assessment of parotid gland pathology,and measurement of skin thickness and *** ultrasound techniques developed to augment the functionality of ultrasonography may also be applicable in pediatric rheumatic *** Ultrasound shows great promise in the assessment and management of children with rheumatologic ***,standardization and validation of ultrasound in healthy children and in patients with rheumatic diseases are still needed.

关键词： Dermatomyositis Juvenile arthritis lupus Pediatric rheumatic diseases Sonoelastography Ultrasound

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Immunological pathogenesis and treatment of systemic lupus erythematosus

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World Journal of Pediatrics 2020年第1期16卷 19-30页

作者： lu Pan mei-ping lu Jing-Hua Wang Meng Xu Si-Rui Yang Department of Pediatric Rheumatology and Allergy The First HospitalJilin UniversityChangchunChina Department of Allergy Immunology and Rheumatology Children's HospitalZhejiang University School of MedicineHangzhouChina

Background Systemic lupus erythematosis(SLE)is a complex and clinically heterogeneous autoimmune disease.A variety of immunological defects contribute to SLE,including dysregulated innate and adaptive immune response.A clearer understanding of the mechanisms driving disease pathogenesis combined with recent advances in medical science is predicted to enable accelerated progress towards improved SLE-personalized approaches to *** aim of this review was to clarify the immunological pathogenesis and treatment of *** sources Literature reviews and original research articles were collected from database,including PubMed and *** articles about SLE were *** Breakdown of self-tolerance is the main pathogenesis of *** innate and adaptive immune networks are interlinked with each other through cytokines,complements,immune complexes and kinases of the intracellular *** targeted at possible targets of immunity have been assessed in clinical *** of them did not show better safety and efficacy than traditional ***,novel targeting treatments are still being *** Dysregulated immune response plays a critical role in SLE,including innate immunity and adaptive *** agents that aim to specifically target abnormal immune processes were assessing and may bring new hope to SLE patients.

关键词： Immunological pathogenesis Systemic lupus erythematosis Treatment

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Prevalence of Epstein-Barr virus infection and characteristics of lymphocyte subsets in newly onset juvenile dermatomyositis

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World Journal of Pediatrics 2021年第2期17卷 205-209页

作者： Qi Zheng Kun Zhu Cai-Na Gao Yi-ping Xu mei-ping lu Department of Rheumatology Immunology and AllergyChildren’s HospitalZhejiang University School of Medicine57#Zhu Gan RoadHangzhou 310000China Department of Pathology Children's HospitalZhejiang University School of MedicineHangzhouChina

Background The underlying etiology of juvenile dermatomyositis(JDM)is unknown.T cell deficiency as well as Epstein-Barr virus(EBV)infection had been suspected to be involved in the pathogenesis,but it has been poorly evaluated in JDM *** This study described the traits of T and B lymphocyte subsets in newly onset JDM patients and the incidence of EBV infection in JDM patients compared with match *** developed JDM patients from 2014 to 2018 were included in the *** with different markers(CD3+,CD3+CD4+,CD3+CD8+,CD3-CD19+and CD3-CD16+CD56+)were tested with flow cytometry in the first admission or after 6 months of *** analysis was conducted to compare the EBV infection in the group of JDM patients and *** We observed that JDM patients had higher positive rate of Epstein-Barr nuclear antigen-immunoglobulin G(IgG)(Plusions The level of CD3-CD16+CD56+cells may associate with the clinical course of *** may act as an environmental factor predisposing patients to the development of JDM.

关键词： Epstein-barr virus Juvenile dermatomyositis Lymphocytes

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Feature Matching via Topology-Aware Graph Interaction Model

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IEEE/CAA Journal of Automatica Sinica 2024年第1期11卷 113-130页

作者： Yifan lu Jiayi Ma Xiaoguang mei Jun Huang Xiao-ping Zhang the Electronic Information School Wuhan UniversityWuhan 430072China IEEE Tsinghua Berkeley Shenzhen Institute Shenzhen 518071China the Department of Electrical Computer and Biomedical EngineeringToronto Metropolitan UniversityToronto ON M5B 2K3Canada

Feature matching plays a key role in computer vision. However, due to the limitations of the descriptors, the putative matches are inevitably contaminated by massive *** paper attempts to tackle the outlier filtering problem from two aspects. First, a robust and efficient graph interaction model,is proposed, with the assumption that matches are correlated with each other rather than independently distributed. To this end, we construct a graph based on the local relationships of matches and formulate the outlier filtering task as a binary labeling energy minimization problem, where the pairwise term encodes the interaction between matches. We further show that this formulation can be solved globally by graph cut algorithm. Our new formulation always improves the performance of previous localitybased method without noticeable deterioration in processing time,adding a few milliseconds. Second, to construct a better graph structure, a robust and geometrically meaningful topology-aware relationship is developed to capture the topology relationship between matches. The two components in sum lead to topology interaction matching(TIM), an effective and efficient method for outlier filtering. Extensive experiments on several large and diverse datasets for multiple vision tasks including general feature matching, as well as relative pose estimation, homography and fundamental matrix estimation, loop-closure detection, and multi-modal image matching, demonstrate that our TIM is more competitive than current state-of-the-art methods, in terms of generality, efficiency, and effectiveness. The source code is publicly available at http://***/Yifanlu2000/TIM.

关键词： Feature matching graph cut outlier filtering topology preserving

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Clinical and laboratory features,treatment,and outcomes of macrophage activation syndrome in 80 children:a multi-center study in China

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World Journal of Pediatrics 2020年第1期16卷 89-98页

作者： Li-Xia Zou Yun Zhu Li Sun Hui-Hui Ma Si-Rui Yang Hua-Song Zeng Ji-Hong Xiao Hai-Guo Yu Li Guo Yi-ping Xu mei-ping lu Department of Rheumatology Immunology and Allergy Children's HospitalZhejiang University School of Medicine57 Zhugan LaneHangzhou 310003China Department of Nephrology and Immunology Children's Hospital of Soochow University92 Zhongnan StreetSuzhou 215003China Department of Rheumatology Children's Hospital of Fudan University399 Wanyuan RoadShanghai 201102China Department of Rheumatology and Immunology Children's Hospital of Nanjing Medical University72 Guangzhou RoadNanjing 210008China Department of Pediatric Rheumatology and Allergy The First Hospital of Jilin University71 Xinming StreetChangchun 130021China Pediatric Allergy Immunology and Rheumatology DepartmentGuangzhou Children's HospitalGuangzhou Women and Children's Medical CenterGuangzhou Medical University318 Renminzhong RoadGuangzhou 510120China Department of Pediatric The First Affiliated Hospital of Xiamen University55 Zhenhai RoadXiamen 361003China

Background Macrophage activation syndrome(MAS)is a major cause of morbidity and mortality in pediatric *** aimed to further understand the clinical features,treatment,and outcome of MAS in *** A multi-center cohort study was performed in seven hospitals in China from 2012 to *** patients with MAS were enrolled,including 53 cases with systemic juvenile idiopathic arthritis(SJIA-MAS),10 cases of Kawasaki disease(KD-MAS),and 17 cases of connective tissue disease(CTD-MAS).The clinical and laboratory data were collected before(pre-),at onset,and during full-blown stages of *** compared the data among the SJIA-MAS,KD-MAS,and CTD-MAS *** 51.2%of patients developed MAS when the underlying disease was first *** patients with SJIA,22.6%(12/53)were found to have hypotension before the onset of *** patients were also found to have significantly increased aspartate aminotransferase(AST)and lactate dehydrogenase(LDH),as well as decreased albumin(Plusions About half of MAS occurred when the underlying autoimmune diseases(SJIA,KD,and CTD)were

关键词： Connective tissue disease Kawasaki disease Macrophage activation syndrome Systemic juvenile idiopathic arthritis

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Predictors of Adverse Pregnancy Outcomes Following Traumatic Injuries

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Current Medical Science 2024年第3期44卷 642-647页

作者： Wan-rong lu ping WU Gong SONG mei-qi GU Zhe XU Li HE National Clinical Research Center for Gynecology and Obstetrics Cancer Biology Research CenterTongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan430030China Department of Traumatic Surgery Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan430030China

Objective After traumatic injury in pregnant women,providing timely and appropriate management for high-risk patients is crucial for both pregnant women and *** study aimed to identify risk factors that predict adverse pregnancy outcomes after traumatic *** A retrospective cohort study including 317 pregnant patients who experienced trauma was *** collected data included general demographics,injury mechanisms and adverse pregnancy *** were divided into two subgroups based on the absence or presence of trauma-related adverse pregnancy *** and multivariate logistic regressions were conducted to estimate the associations between clinical variables and adverse pregnancy *** A total of 41(12.93%)patients experienced adverse pregnancy outcomes within the first 24 h *** study revealed that age>35 years(OR=14.995,95%CI:5.024–44.755,P<0.001),third trimester trauma(OR=3.878,95%CI:1.343–11.204,P=0.012),abdominal pain(OR=3.032,95%CI:1.221–7.527,P=0.017),vaginal bleeding(OR=3.226,95%CI:1.093–9.523,P=0.034),positive scan in focused assessment with sonography for trauma(FAST)positive(OR=8.496,95%CI:2.825–25.555,P<0.001),9≤injury severity score(ISS)<16(OR=3.039,95%CI:1.046–8.835,P=0.041)and ISS≥16(OR=5.553,95%CI:1.387–22.225,P=0.015)increased the probability of posttraumatic adverse pregnancy *** age,gestational age at delivery,vaginal bleeding and positive FAST results were risk factors for abnormal *** Advanced maternal age,third trimester,and positive FAST results should alert multidisciplinary trauma teams to closely monitor patients to prevent adverse pregnancy outcomes.

关键词： adverse pregnancy outcomes predictive factors abnormal delivery trauma

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Study on carbon emission calculation method and carbon reduction indexes of urban building stock

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Science China(Technological Sciences) 2024年第9期67卷 2706-2726页

作者： YANG Liu ZHANG Nan lu mei luO ZhiXing DOU mei State Key Laboratory of Green Building Xi’an University of Architecture and TechnologyXi’an 710055China School of Architecture Xi’an University of Architecture and TechnologyXi’an 710055China School of Language Literature and LawXi’an University of Architecture and TechnologyXi’an 710055China School of Management Xi’an University of Architecture and TechnologyXi’an 710055China China Southwest Architectural Design and Research Institute Corp.Ltd. Chengdu 610041China

Carbon emissions associated with buildings are a major source of urban emissions. To put forward the methods and strategies to curb carbon emissions from urban building stock, it is not only necessary to establish a carbon emission calculation method for fine statistical analysis, but also to evaluate carbon emissions of urban planning schemes with applicable indexes. Currently,researches mainly focus on carbon emissions of individual buildings. When expanded to urban building stock, the calculation faces the lack of basic data, inadequate spatial analysis and unspecific carbon reduction indexes. Therefore, this study proposes a bottom-up calculation method for urban building stock, conducts spatial analysis based on carbon balance of urban grids, reveals the coupling mechanism between urban carbon reduction indexes and grid carbon emissions, and systematically establishes a carbon-reduction-oriented urban planning method that comprises calculation, analysis and evaluation, which is applied to Xi'an,China. This study provides a theoretical reference for cities to formulate carbon reduction targets and implement planning strategies by evaluating and predicting carbon emissions from urban building stock.

关键词： carbon emissions urban building stock calculation method carbon reduction index

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Self-assembled all-oxides three-phase vertically aligned nanocomposite thin film with multifunctionality

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Nano Research 2024年第9期17卷 8226-8232页

作者： Jijie Huang Yuan Fang ping lu Juanjuan lu Haiyan Wang School of Materials Shenzhen Campus of Sun Yat-sen UniversityShenzhen 518107China School of Materials Engineering Purdue UniversityWest LafayetteIN 47907USA Sandia National Laboratories AlbuquerqueNew Mexico 87185USA School of Electrical and Computer Engineering Purdue UniversityWest LafayetteIN 47907USA

Multi-phase vertically aligned nanocomposite(MP-VAN)thin films represent a promising avenue for achieving complex multifunctionality,exploring novel interfacial phenomena,and enabling complex metamaterial designs and *** this study,a novel self-assembled all-oxides three-phase VAN system was conceptualized and fabricated utilizing pulsed laser deposition(PLD)with a single composite *** microstructural analysis reveals the presence of three distinct phases:LiNbO_(3),CeO_(2-x),and LiNbCe_(1-x)O_(y)within the MP-VAN ***,ferroelectric,dielectric,optical anisotropy,and magnetic properties were systematically investigated to showcase the multifunctionality inherent in these *** work presents a pioneering approach to designing and realizing MP-VAN systems,and opens up opportunities for tailoring the complex three-dimensional(3D)physical properties and property coupling of VAN films towards diverse device applications.

关键词： thin film three-phase vertically aligned nanocomposite multifunctionality ferroelectricity optical anisotropy

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