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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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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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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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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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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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A guide to human microbiome research: study design, sample collection, and bioinformatics analysis

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Chinese Medical Journal 2020年第15期133卷 1844-1855页

作者： Xu-Bo Qian Tong Chen Yi-ping Xu Lei Chen Fu-Xiang Sun mei-ping lu Yong-Xin Liu Department of Rheumatology Immunology&Allergy Children's HospitalZhejiang University School of MedicineHangzhou 310003Zhejiang ProvinceChina National Resource Center for Chinese Materia Medica China Academy of Chinese Medical SciencesBeijing 100700China Department of Vascular Surgery Fu Xing HospitalCapital Medical UniversityBeijing 100045China EHBI0 Gene Technology(Beijing)Co. LtdBeijing 100009China Institute of Genetics and Developmental Biology Chinese Academy of SciencesBeijing 100101China CAS Center for Excellence in Biotic Interactions University of Chinese Academy of SciencesBeijing 100049China

The purpose of this review is to provide medical researchers,especially those without a bioinformatics background,with an easy-to-understand summary of the concepts and technologies used in microbiome ***,we define primary concepts such as microbiota,microbiome,and ***,we discuss study design schemes,the methods of sample size calculation,and the methods for improving the reliability of *** emphasize the importance of negative and positive controls in this ***,we discuss statistical analysis methods used in microbiome research,focusing on problems with multiple comparisons and ways to compare β-diversity between ***,we provide step-by-step pipelines for bioinformatics *** summary,the meticulous study design is a key step to obtaining meaningful results,and appropriate statistical methods are important for accurate interpretation of microbiome *** step-by-step pipelines provide researchers with insights into newly developed bioinformatics analysis methods.

关键词： Microbiome Microbiota Study design Statistical analysis Sample size Bioinformatics analysis Pipeline

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Nitric oxide inhalation inhibits inducible nitric oxide synthase but not nitrotyrosine formation and cell apoptosis in rat lungs with meconiuminduced injury

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Acta Pharmacologica Sinica 2005年第9期26卷 1123-1129页

作者： mei-ping lu Li-zhong DU~2 Wei-zhong GU Xiang-xiang CHEN Neonatal Intensive Care Unit,Department of Pediatrics,Children’s Hospital,Zhejiang University School of Medicine,Hangzhou 310003,China Department of Pediatrics Neonatal Intensive Care Unit Children's Hospital Zhejiang University School of Medicine Hangzhou China

Aim:To investigate the effects of inhaled nitric oxide(NO)on pulmonaryinflammation,apoptosis,peroxidation and protein nitration in a rat model of acutelung injury(ALI)induced by ***:Twenty-four healthy maleSprague-Dawley rats were randomly devided into 3 groups(n=8):meconium-induced ALl with intratracheal instillation of 1 mL/kg saline(Mec/saline group),continuous inhalation of NO at 20 μL/L.(Mec/iNO),and the control group(control).Electromicroscopic examination was used to determine the extent of *** was used to detect DNA fragmentation in pulmonary apoptoticcells,expressed as the apoptosis index(AI).Western blotting was used to detectpulmonary inducible NO synthase(iNOS)***-PCR was used to detectinterleukin(IL)-1β mRNA *** count in bronchoalveolar lavage(BAL),myeloperoxidase(MPO)activity,as well as malondialdehyde(MDA)andnitrotyrosine formation,the markers of toxic NO-superoxide pathway in rat lungparenchyma specimens,were also ***:Expression of iNOS pro-tein and IL- 113 mRNA were increased significantly in the Mec/saline group(bothPlunginjury score,pulmonary AI,MDA level and nitrotyrosine formation were alsoincreased significantly(all Plung injury score were also decreased significantly(Plungs from inflammatory injury,but does not decreaseepithelial apoptosis or lung nitrotyrosine formati

关键词： inhaled nitric oxide meconium acute lung injury nitrotyrosine apoptosis

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Applied anatomy of the submental island flap and its clinical application in the repair of defects following hypopharyngeal carcinoma resection

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World Journal of Otorhinolaryngology-Head and Neck Surgery 2015年第1期1卷 -页

作者： Xi Chen Han Zhou Yong-Jie Zhang Lin Yin mei-ping lu Guang-Qian Xing Zi-ping Lin Department of Otorhinolaryngology The First Affiliated Hospital Nanjing Medical University Nanjing 210029 China Department of Human Anatomy School of Basic Medical Sciences Nanjing Medical University Nanjing 210029 China Department of Stomatology The First Affiliated Hospital Nanjing Medical University Nanjing 210029 China

Objective: To explore the feasibility of the submental island flap in the repair of hypopharyngeal ***: We collected wet specimens of fresh cadaveric heads from the Han Chinese adult population for applied anatomy of the submental island flap, and followed five patients with pyriform sinus carcinoma after reconstruction surgery using submental island ***: We found that the average length and width of the submental island flaps were (65.20 ± 11.69) mm and (46.70 ± 6.59) mm, *** skin flap in all five patients survived after surgery, and tracheal tubes and gastric tubes were removed 7-36 days after *** were followed up for 24-42 months, pharyngeal flaps grew well, and speech and swallowing functions were ***: The submental island flap is a preferred material for the repair of hypopharyngeal defects after hypopharyngeal carcinoma resection, because of good blood supply, easy harvesting, and high survival rate.

关键词： Submental island flap Submental artery Submental vein Hypopharyngeal neoplasms Reconstructive surgical procedures

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胺基CO_2开关型表面活性剂的合成与性能研究

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日用化学工业 2017年第7期47卷 369-373页

作者：张鹏梅平赖璐长江大学化学与环境工程学院湖北荆州434023 非常规油气湖北省协同创新中心湖北武汉430100

以溴代十四烷和二乙烯三胺为原料,合成了表面活性剂十四烷基二乙烯三胺(C_(14)DETA)。采用红外光谱和核磁共振氢谱对C_(14)DETA进行了结构表征。通过研究其电导率可逆、pH可逆和与SDS协同作用的开启,证明了C_(14)DETA具有良好的CO_2开... 详细信息

以溴代十四烷和二乙烯三胺为原料,合成了表面活性剂十四烷基二乙烯三胺(C_(14)DETA)。采用红外光谱和核磁共振氢谱对C_(14)DETA进行了结构表征。通过研究其电导率可逆、pH可逆和与SDS协同作用的开启,证明了C_(14)DETA具有良好的CO_2开关性能。测定了十四烷基二乙烯三胺碳酸氢盐(C_(14)DETA-CO_2)和SDS/C_(14)DETA-CO_2复配体系的临界胶束浓度,分别为1.0×10^(-2)和1.0×10^(-4)mol/L。通过测定C_(14)DETA-CO_2对正癸烷/水体系的乳化性能,证明了C_(14)DETA-CO_2具有良好的乳化性能,所制得乳状液为W/O型,其较佳乳化条件为:C_(14)DETA-CO_2的质量分数4%,油水体积比1∶1或1∶2。

关键词： CO2开关型表面活性剂表征临界胶束浓度性能

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