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Clinical heterogeneity and five phenotypes identified in pediatric Behçet’s syndrome: a cohort study from Shanghai Behçet’s syndrome database

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World Journal of Pediatrics 2024年第8期20卷 801-808页

作者： Dan Hu Chun-Hui She Hua-Fang Bao Jun Zou jian-Fei Cai Jing‑Fen Ye Yan Shen Hai‑Fen Ma Dan Luo jian-long guan Department of Rheumatology and Immunology Huadong Hospital Affiliated to Fudan University221 Yan’an West Road200040ShanghaiChina

Objectives Behçet’s syndrome(BS)is a rare disease of unknown etiology,with limited reports especially in pediatric *** clinical characteristics and phenotypes of pediatric BS as a highly heterogeneous variable vessel vasculitis were investigated in this *** A cross-sectional study was conducted to compare clinical variables and descriptive characteristics of BS by age of onset and *** analysis was then performed to identify the phenotypes of pediatric *** A total of 2082 BS patients were included in this study,1834 adults and 248 *** with adult-onset BS,pediatric BS had a higher incidence of folliculitis[relative risks(RR)and 95%confidence interval(CI)1.3(1.0–1.5)],uveitis of the left eye[RR and 95%CI 2.3(1.0–5.0)],intestinal ulcer complications[RR and 95%CI 2.1(1.1–4.2)],pericarditis[RR and 95%CI 2.5(1.0–6.2)],and psychiatric disorders[RR and 95%CI 2.8(1.0–7.9)],while the incidence of thrombocytopenia was lower[RR 0.2(0.1–1.0)].Among pediatric BS,females had more genital ulcers,while males were more likely to have skin lesions,panuveitis,vascular involvement,venous lesions,cardiac involvement,and aortic *** analysis classified pediatric BS into five clusters(C1–C5):C1(n=61,24.6%)showed gastrointestinal(GI)involvement;C2(n=44,17.7%)was the central nervous system(CNS)type where 23 cases overlapped joint involvement;in C3(n=35,14.1%),all patients presented with arthritis or arthralgia;all patients in C4(n=29,11.7%)manifested ocular involvement,with a few patients overlapping with GI involvement or joint damage;C5(n=79,31.9%)was the mucocutaneous type,presenting both oral ulcers,genital ulcers,and skin *** The clinical features of pediatric and adult BS differ *** and female pediatric BS also have a distinct *** phenotypes including GI,CNS,joint,ocular,and mucocutaneous types were identified for pediatric BS.

关键词： Behget's syndrome Clinical manifestation Cluster analysis-Pediatric Phenotype

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Reply to "Comment to Deep vein thrombosis induced by vasculitis in the Behcet's syndrome"

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Chinese Medical Journal 2019年第4期132卷 503-504页

作者： Yong Chen jian-long guan Department of Rheumatology and Immunology Huadong Hospital Affiliated to Fudan UniversityShanghai 200040China Integrated Hospital of Traditional Chinese Medicine Southern Medical UniversityGuangzhouGuangdong 510330China

We appreciate the attention from all the authors of the manuscript entitled "Comment to Deep Vein Thrombosis Induced by Vasculitis in The Behcet's Syndrome".As Behcet's disease belong to one type of vacuities,there is no need to repeat the term as "Vasculitis in The Behcet's Syndrome"and this type of thrombosis is regarded as inflammation related thrombosis,so inflammation is the mechanism,while vasculitis is a diagnostic term,so I recommend of change into Deep Vein Thrombosis Induced by vascular inflammation in patients with Behcet's Syndrome.

关键词： manuscript entitled The Behcet

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Endoscopic findings of gastrointestinal involvement in Chinese patients with Behcet's disease

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World Journal of Gastroenterology 2014年第45期20卷 17171-17178页

作者： Jun Zou Yan Shen Da-Nian Ji Song-Bai Zheng jian-long guan Division of Immunology and Rheumatology Shanghai Key Laboratory of Clinical Geriatric Medicine Huadong Hospital Research Center on Aging and Medicine Fudan University Center of Gastrointestinal Endoscopy Huadong Hospital Fudan University Division of Gastroenterology Huadong Hospital Fudan University

AIM:To report the incidence,clinical features and outcomes of gastrointestinal(GI)involvement in Behcet’s disease(BD).METHODS:A total of 168 consecutive patients with BD were screened and upper and lower GI endoscopies were performed in 148 *** hundred age-and sex-matched controls were enrolled for ***:Fifty-two(35.1%)patients had GI *** a mean follow-up of 10 mo,ileocecal ulcers had been confirmed in 20 patients,including active ulcer(s)in 18 patients,but no ileocecal ulceration was found in *** symptoms were present in 14 patients with active ulcer(s),while 4 patients with smaller ulcer were *** features of ileocecalulcer were:a single ulcer(50%),larger than 1 cm in diameter(72.2%),and round/oval or volcano-type in shape(83.3%).Compared with patients without GI involvement,less ocular lesions,lower levels of albumin,erythrocyte count and hemoglobin,and higher levels of C-reactive protein and erythrocyte sedimentation rate were confirmed in the intestinal BD *** patients had esophageal ulcers in the BD group but no case in *** other endoscopic findings were similar between the two *** prevalence of Helicobacter pylori infection was similar in both *** patients received an immunomodulator and responded ***:GI lesions commonly occur in Chinese BD *** most frequently involved area is the ileocecal *** ulcer might be a rare but unique lesion.

关键词： Behcet's disease Gastrointestinal involve-ment Ulcer Endoscopy Case-controlled

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Is Warfarin Indicated in Behcet＇s Syndrome Associated with hrombosis？ Reflections on Treatment and Death of a Patient

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Chinese Medical Journal 2018年第1期131卷 105-106页

作者： Yong Chen jian-long guan Department of Rheumatology and Immunology Huadong Hospital Affiliated to Fudan University Shanghai 200040 China

A 37-year-old male patient was admitted to Huadong Hospital with diagnosis of Behcet＇s syndrome （BS） for 5 years and deep vein thrombosis （DVT） for 2 months. The patient complained of recurrent oral ulceration, approximately five to seven times per year starting 5 years before presentation. He had a history of genital ulceration one to two times every year. The patient did not follow a strict treatment regimen.

关键词： Warfarin Reflections on Treatment

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Metformin Might Inhibit Virus through Increasing Insulin Sensitivity

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Chinese Medical Journal 2018年第3期131卷 376-377页

作者： Yong Chen Feng Gu jian-long guan Department of Rheumatology and Immunology Huadong Hospital Affiliated with Fudan University Shanghai 200040 China MOE and MOH Key Laboratory of Medical Molecular Virology School of Basic Medicine Shanghai Medical Coege Fudan University Shanghai 325444 China

To the Editor：Metformin is believed to be the most widely used medication for diabetes as well as the first-line medication for the treatment of type 2 diabetes,especially in patients who are *** is described as a magic medicine currently due to its broad range of potential benefits for polycystic ovary syndrome,cardiovascular disease,and various cancers and even prolonged ***,limited in vitro and in vivo research suggests that metformin may have inhibitory effect on virus,especially hepatitis C virus （HCV）.Besides,it would be amazing if metformin possess antivirus function in hepatitis B virus （HBV）,human immunodeficiency virus （HIV）,etc.,to benefits virus-infected patients from other aspects of mechanism.

关键词： HEPATITIS-C

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Surgical Intervention for Behcet＇s Disease with Aorta Aneurysm and Pseudoaneurysm： Opposite Outcomes in Two Cases

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Chinese Medical Journal 2017年第20期130卷 2503-2505页

作者： Yong Chen Jia-Shen Cui jian-Fei Cai Jun Zou jian-long guan Department of Rheumatology and Immunology Huadong Hospital Affiliated with Fudan University Shanghai 200040 China Department of Vascular Surgery Huadong Hospital Affiliated with Fudan University Shanghai 200040 China

Behcet＇s disease （BD） is a chronic, relapsing autoimmune disorder characterized by oral and genital ulcerations with uveitis, and additional clinical manifestations in multiple organ systems. The occurrence of vasc... 详细信息

关键词： Aorta Aneurysm Behcet's Disease Pseudoaneurysm, Surgery

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TBK1:a new target for overcoming cancer immunotherapy resistance

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Science China(Life Sciences) 2024年第1期67卷 217-218页

作者： Jizhong guan long Zhang Fangfang Zhou Institutes of Biology and Medical Science Soochow UniversitySuzhou 215123China MOE Laboratory of Biosystems Homeostasis and Protection and Innovation Center for Cell Signaling Network Life Sciences InstituteZhejiang UniversityHangzhou 310058China

In a recent study published in Nature,Sun et al.(2023)discovered that targeting TANK-binding kinase 1(TBK1)can successfully overcome resistance to immune checkpoint blockade(ICB)treatment and the study revealed its potential *** authors provided evidence that the disruption of TBK1 signal enhances the blockade of programmed cell death protein-1(PD-1)and promotes anti-tumor immunity in the tumor microenvironment(TME).Tumor cells rely on Janus kinase(JAK)and signal transducer as well as activator of transcription(STAT)signals to elicit an immune response by perceiving tumor necrosis factorα(TNF-α)and interferon-γ(IFN-γ),resulting in receptor-interacting protein kinase(RIPK)-caspase-mediated cell death in tumor cells(Figure 1).

关键词： TBK1 immunity

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Expression of FLICE-inhibitory Protein in Synovial Tissue and Its Association with Synovial Inflammation in Juvenile Idiopathic Arthritis

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Chinese Medical Sciences Journal 2010年第1期25卷 20-26页

作者： Feng-xia Wu Li-jun Wu Xiong-yan Luo Ming-hui Yang Zhong Tang Chuan-mei Xie Jing-guo Zhou jian-long guan Guo-hua Yuan Institute of Rheumatology and Immunology Affiliated Hospital of North Sichuan Medical College Nanchong Sichuan 637000 China Department of Rheumatology People＇s Hosptial of Xinjiang Uygur Autonomus Region Urumchi 830001 China Department of Rheumatology and Immunology Changhai Hospital Second Military Medical University Shanghai 200433 China

Objective To examine the expression of FLICE-inhibitory protein (FLIP) in juvenile idiopathic arthritis (JIA) and analyze its correlation with synovial inflammation. Methods The expression of FLIP was assessed in 11 JIA and 3 normal synovial tissue samples by reverse transcription-polymerase chain reaction (RT-PCR) and immunohistochemistry. The cell types expressing FLIP were further characterized,and the correlation of FLIP expression with the degree of synovial inflammation,as well as the activity of caspase 8 was then analyzed. Results RT-PCR revealed the expression of FLIP mRNA in all 11 JIA samples,but not in 3 normal synovial tissues. In JIA,FLIP expression could be found in both the lining and sublining layers,mainly in the macrophage-like cells. Moreover,the expression of FLIP in JIA synovial tissues was positively correlated with the degree of synovial inflammation (r=0.563,P<0.05). Conclusion The expression of antiapoptotic FLIP in JIA synovial tissue and its correlation to accumulation of inflammatory cells in synovial tissue suggests that FLIP potentially extends the lifespan of synovial cells and thus contributes to the progression of joint destruction.

关键词： juvenile idiopathic arthritis FLICE-inhibitory protein caspase 8

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Effect of Mechanical Stress in Combination with Verapamil on Levels of Aggrecan and ADAMTS-5 mRNAs and Proteins in Human Osteoarthritic Chondrocyte/Agarose Constructs

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Chinese Medical Journal 2018年第18期131卷 2229-2231页

作者： Dan Luo Yan Shen jian-Qiang Lyu Yong-Qian Fan Dong-Hui Huang Wei-long Lin Hai-Min Shen Hu-Ji Xu jian-long guan Department of Rheumatology Huadong Hospital Fudan University Shanghai 200040 China Key Laboratory of Exercise and Health Sciences of Ministry of Education School of Kinesiology Shanghai University of Sport Shanghai 200438 China Department of Orthopedic Surgery Huadong Hospital Fudan University Shanghai 200040 China Department of Rheumatology Changzheng Hospital Second Military Medical University Shanghai 200003 China Department of Rheumatology Shanghai Key Laboratory of Clinical Geriatric Medicine Huadong Hospital Fudan University Shanghai 200040 China

To the Editor： Osteoarthritis （OA） has the highest prevalence and economic impact among arthritic maladies and is the most common cause of long-term disability among individuals over 65 years of age. OA is characterized by the degeneration of articular cartilage and the loss of cartilage matrix in affected joints. It is widely accepted that an imbalance between the biosynthesis and the degradation of extracellular matrix （ECM） occurs in OA cartilage.

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Non-monotonic behavior of jam probability and stretchedexponential distribution in pedestrian counterflow

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Chinese Physics B 2024年第9期33卷 259-266页

作者： Ze-Hao Chen Zhi-Xi Wu jian-Yue guan College of Physical Science and Technology Guangxi Normal UniversityGuilin 541004China Lanzhou Center for Theoretical Physics Key Laboratory of Theoretical Physics of Gansu Provinceand Key Laboratory for Quantum Theory and Applications of the Ministry of EducationLanzhou UniversityLanzhou 730000China

We adopt a floor field cellular automata model to study the statistical properties of bidirectional pedestrian flow movingin a straight corridor. We introduce a game-theoretic framework to deal with the conflict of multiple pedestrians tryingto move to the same target location. By means of computer simulations, we show that the complementary cumulative distributionof the time interval between two consecutive pedestrians leaving the corridor can be fitted by a stretched exponentialdistribution, and surprisingly, the statistical properties of the two types of pedestrian flows are affected differently by theflow ratio, i.e., the ratio of the pedestrians walking toward different directions. We also find that the jam probability exhibitsa non-monotonic behavior with the flow ratio, where the worst performance arises at an intermediate flow ratio of around0.2. Our simulation results are consistent with some empirical observations, which suggest that the peculiar characteristicsof the pedestrians may attributed to the anticipation mechanism of collision avoidance.

关键词： traffic and crowd dynamics evolutionary game theory

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