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| 1 | 帕博利珠单抗单用或与放疗联用治疗转移性非小细胞肺癌:两个随机试验的汇总分析显示文摘背景放疗可以提高整个机体对免疫治疗的应答。在Ⅱ期PEMBRO-RT研究和Ⅰ/Ⅱ期MDACC研究中,患有转移性非小细胞肺癌(NSCLC)的患者被随机分配入组,接受免疫治疗(帕博利珠单抗)+放疗联合疗法,或免疫治疗单一疗法。当上述2个研究单独分析时,联合疗法组显示出潜在获益。由于每个研究的样本量较小,缓解率和结局并未显示出统计学意义,然而却有显著的临床获益。因此,本研究进行汇总分析,来判断放疗是否会改善转移性NSCLC患者的免疫治疗应答。方法PEMBRO-RT和MDACC研究纳入标准:患者年龄≥18岁,患有转移性NSCLC,且有≥1处未经放疗照射的病灶,以便进行射野外应答监测。PEMBRO-RT研究纳入曾接受过化疗患者,MDACC研究纳入曾接受过治疗或新诊断患者。2个研究中的患者均未接受过免疫治疗。在PEMBRO-RT研究中患者被等比例随机分配入组,并根据吸烟状态进行分层(分为<10年组和≥10年组)。MDACC研究的患者根据放疗计划可行性被等比例随机分配入2个受试组。由于联合治疗组的干预本质,每个研究中的放疗均不适用盲法。在2个研究中,不论是否进行放疗,均静脉滴入帕博利珠单抗(每3周200 mg)。在PEMBRO-RT研究中,在放疗(24 Gy 3次分割照射)结束后1周给予第1剂帕博利珠单抗。在MDACC研究中,在第1次放疗(50 Gy 4次分割照射或45 Gy 15次分割照射)同时给予帕博利珠单抗。仅检测未经照射病灶的应答。本研究的终点为最佳射野外(远隔)应答率(ARR)、最佳射野外疾病控制率(ACR)、12周时ARR、12周时ACR、无进展生存期(PFS)和总生存期(OS)。2个研究的意向治疗(ITT)人群均纳入分析。PEMBRO-RT研究(NCT02492568)和MDACC研究(NCT02444741)均在ClinicalTrials.gov上注册。发现纳入148例患者,76例接受帕博利珠单抗治疗,72例接受帕博利珠单抗+放疗治疗。所有患者随访时间中位数为33个月[四分位距(IQR):32.4~33.6]。148例患者中124例(84%)组织学特征为非鳞癌,111例(75%)患者曾经接受过化疗。组间没有基线特征差异,包括PD-L1表达状态和转移灶体积。最常见的照射部位为肺转移灶(39%,28/72)、胸腔内淋巴结(21%,15/72)和非原发灶(17%,12/72)。帕博利珠单抗组和联合治疗组的最佳ARR分别为19.7%(15/76)和41.7%(30/72),OR=2.96,95%CI:1.42~6.20,P=0.0039;最佳ACR分别为43.4%(33/76)和65.3%(47/72),OR=2.51,95%CI:1.28~4.91,P=0.0071;PFS中位数分别为4.4(IQR:2.9~5.9)和9.0个月(IQR:6.8~11.2),HR=0.67,95%CI:0.45~0.99,P=0.045;OS中位数分别为8.7(IQR:6.4~11.0)和19.2个月(IQR:14.6~23.8),OR=0.67,95%CI:0.54~0.84,P=0.0004。在汇总分析中没有发现新的安全问题。解读帕博利珠单抗免疫疗法+放疗显著提高转移性NSCLC患者的应答和改善治疗结局。这些结果需要在三期临床试验中进行验证。 | 陈大卫(翻译) 于金明(校对) Willemijn S M E Theelen Vivek Verma Brian P Hobbs Heike M U Peulen Joachim G J V Aerts Idris Bahce Anna Larissa N Niemeijer Joe Y Chang Patricia M de Groot Quynh-Nhu Nguyen Nathan I Comeaux George R Simon Ferdinandos Skoulidis Steven H Lin Kewen He Roshal Patel John Heymach Paul Baas James W Welsh | 2021 | 中华肿瘤防治杂志2021，28，24： | 49 |
| 2 | 帕博利珠单抗治疗伴脑转移NSCLC患者的一项非随机、开放Ⅱ期试验的长期随访结果和生物标志物分析显示文摘背景与目的我们开展了一项帕博利珠单抗用于伴未治疗脑转移的非小细胞肺癌(non-small cell lung cancer,NSCLC)或黑色素瘤患者的疗效和安全性的II期试验,旨在评估程序性死亡受体1(programmed cell death 1,PD-1)抑制剂在中枢神经系统(central nervous system,CNS)中的疗效。中期结果已发表,现报道对NSCLC队列的更新分析结果。方法这是一项开放性、单中心、II期试验。纳入标准:年龄≥18岁,诊断为晚期NSCLC并伴有≥1个5 mm-20 mm脑转移病灶,既往从未治疗或之前放疗后进展,无神经系统症状,不需要激素治疗且美国东部肿瘤协作组(Eastern Cooperative Oncology Group,ECOG)<2分。患者每2周接受一次帕博利珠单抗(10 mg/kg)治疗。队列1为程序性死亡配体1(programmed cell death ligand 1,PD-L1)≥1%的患者,队列2为PD-L1<1%或未评估的患者。主要终点是脑转移患者缓解比例。所有经治患者均纳入疗效与安全性终点的分析。该研究已结束入组,并于Clinicaltrials.gov登记注册,注册号为NCT02085070。结果2014年3月31日-2018年5月21日,共42例患者接受治疗。中位随访时间为8.3个月(IQR:4.5个月-26.2个月)。队列1的37例患者中11例有脑转移缓解[29.7%(95%CI:15.9%-47.0%)]。队列2未观察到缓解。治疗相关的3级-4级不良事件(adverse events,AEs)包括2例肺炎、1例全身症状、1例结肠炎、1例肾上腺皮质功能不全、1例高血糖症和1例低钾血症。6例(14%)患者发生了治疗相关的严重不良事件,包括肺炎、急性肾损伤、低钾血症和肾上腺皮质功能不全。没有观察到治疗相关死亡病例。结论帕博利珠单抗治疗PD-L1≥1%的NSCLC伴脑转移患者有效,且对所有纳入的未经治疗的脑转移患者安全。需要进一步探索免疫治疗用于NSCLC合并CNS转移。 | Sarah B GOLDBERG Kurt A SCHALPER Scott N GETTINGER Amit MAHAJAN Roy S HERBST Anne C CHANG Rogerio LILENBAUM Frederick H WILSON Sacit Bulent OMAY James B YU Lucia JILAVEANU Thuy TRAN Kira PAVLIK Elin ROWEN Heather GERRSH Annette KOMLO Richa GUPTA Hailey WYATT Matthew RIBEIRO Yuval KLUGER Geyu ZHOU Wei WEI Veronica L CHANG Harriet M KLUGER 董晓荣(翻译/校对) | 2021 | 中国肺癌杂志2021，24，9： | 34 |
| 3 | 血管周围脂肪组织中的Bmal1通过血管紧张素原转录调控管理静息期血压显示文摘血管周围脂肪组织(perivascular adipose tissue,PVAT)围绕血管,构成了在生理条件下保持血管张力所需的脉管系统的独特的功能整体层。然而,关于PVAT与血压调节之间的关系的资料很少,包括其对昼夜血压变化的潜在作用。 | Chang L Xiong W Zhao X Fan Y Guo Y Garcia-Barrio M Zhang J Jiang Z Lin JD Chen YE 刘青 叶鹏 | 2018 | 中华高血压杂志2018，26，2： | 23 |
| 4 | Upper mantle anisotropy and crust-mantle deformation pattern beneath the Chinese mainland显示文摘Over the past 10 years,the number of broadband seismic stations in China has increased significantly.The broadband seismic records contain information about shear-wave splitting which plays an important role in revealing the upper mantle anisotropy in the Chinese mainland.Based on teleseismic SKS and SKKS phases recorded in the seismic stations,we used the analytical method of minimum transverse energy to determine the fast wave polarization direction and delay time of shear-wave splitting.We also collected results of shear-wave splitting in China and the surrounding regions from previously published papers.From the combined dataset we formed a shear-wave splitting dataset containing 1020 parameter pairs.These splitting parameters reveal the complexity of the upper mantle anisotropy image.Our statistical analysis indicates stronger upper mantle anisotropy in the Chinese mainland,with an average shear-wave time delay of 0.95 s;the anisotropy in the western region is slightly larger(1.01 s)than in the eastern region(0.92 s).On a larger scale,the SKS splitting and surface deformation data in the Tibetan Plateau and the Tianshan region jointly support the lithospheric deformation mode,i.e.the crust-lithospheric mantle coherent deformation.In eastern China,the average fast-wave direction is approximately parallel to the direction of the absolute plate motion;thus,the upper mantle anisotropy can be attributed to the asthenospheric flow.The area from the Ordos block to the Sichuan Basin in central China is the transition zone of deformation modes between the east and the west regions,where the anisotropy images are more complicated,exhibiting'fossil'anisotropy and/or two-layer anisotropy.The collision between the Indian Plate and the Eurasian Plate is the main factor of upper mantle anisotropy in the western region of the Chinese mainland,while the upper mantle anisotropy in the eastern region is related to the subduction of the Pacific Plate and the Philippine Sea Plate beneath the Eurasian Plate. | WANG ChunYong CHANG LiJun DING ZhiFeng LIU QiongLin LIAO WuLin Lucy M FLESCH | 2014 | Science China Earth Sciences2014，57，1： | 18 |
| 5 | Bis(7)-tacrine attenuates beta amyloid-induced neuronal apoptosis by regulating L-type calcium channels显示文摘 | Fu H Li W Lao Y Luo J Lee NT Kan KK Tsang HW Tsim KW Pang Y Li Z Chang DC Li M Han Y | 2006 | 中国生物学文摘2006，20，10： | 10 |
| 6 | Diabetes mellitus as a risk factor for gastrointestinal cancer among American veterans显示文摘AIM:To assess the risk of biliary and pancreatic cancers in a large cohort of patients with type 2 diabetes mellitus(DM) .METHODS:Eligibility for this study included patients with type 2 DM(ICD-9 code 250.0) who were discharged from Department of Veteran Affairs hospitals between 1990 and 2000.Non-matched control patients without DM were selected from the same patient treatment files during the same period.Demographic information included age,sex and race.Secondary diagnoses included known risk factors based on their ICD-9 codes.By multivariate logistic regression,the occurrence of biliary and pancreatic cancer was compared between case subjects with DM and controls without DM.RESULTS:A total of 1172496 case and control subjects were analyzed.The mean age for study and control subjects was 65.8 ± 11.3 and 64.8 ± 12.6 years,respectively.The frequency of pancreatic cancer in subjects with DM was increased(0.9%) in comparison to control subjects(0.3%) with an OR of 3.22(95% CI:3.03-3.42) .The incidence of gallbladder andextrahepatic biliary cancers was increased by twofold in diabetic patients when compared to controls.The OR and 95% CI were 2.20(1.56-3.00) and 2.10(1.61-2.53) ,respectively.CONCLUSION:Our study demonstrated that patients with DM have a threefold increased risk for developing pancreatic cancer and a twofold risk for developing biliary cancer. | M Mazen Jamal Eugene J Yoon Kenneth J Vega Mehrtash Hashemzadeh Kenneth J Chang | 2009 | World Journal of Gastroenterology2009，15，42： | 10 |
| 7 | Lattice Boltzmann Flux Solver:An Efficient Approach for Numerical Simulation of Fluid Flows显示文摘A lattice Boltzmann flux solver(LBFS)is presented for simulation of fluid flows.Like the conventional computational fluid dynamics(CFD)solvers,the new solver also applies the finite volume method to discretize the governing differential equations,but the numerical flux at the cell interface is not evaluated by the smooth function approximation or Riemann solvers.Instead,it is evaluated from local solution of lattice Boltzmann equation(LBE)at cell interface.Two versions of LBFS are presented in this paper.One is to locally apply one-dimensional compressible lattice Boltzmann(LB)model along the normal direction to the cell interface for simulation of compressible inviscid flows with shock waves.The other is to locally apply multi-dimensional LB model at cell interface for simulation of incompressible viscous and inviscid flows.The present solver removes the drawbacks of conventional lattice Boltzmann method(LBM)such as limitation to uniform mesh,tie-up of mesh spacing and time interval,limitation to viscous flows.Numerical examples show that the present solver can be well applied to simulate fluid flows with non-uniform mesh and curved boundary. | Shu Chang Wang Y Yang L M Wu J | 2014 | Transactions of Nanjing University of Aeronautics and Astronautics2014，31，1： | 7 |
| 8 | Osteoprotegerin: A Novel Secreted Protein Involved in the Regulation of Bone Density显示文摘 | W.S Simonet D.L Lacey C.R Dunstan M Kelley M.-S Chang R Lüthy H.Q Nguyen S Wooden L Bennett T Boone G Shimamoto M DeRose R Elliott A Colombero H.-L Tan G Trail J Sullivan E Davy N Bucay L Renshaw-Gegg T.M Hughes D Hill W Pattison P Campbell S Sander G Van | 1997 | Cell1997，，2： | 6 |
| 9 | It is safe to use transdermal glyceryl trinitrate to lower blood pressure in patients with acute ischaemic stroke with carotid stenosis显示文摘Background There is concern that blood pressure(BP)lowering in acute stroke may compromise cerebral perfusion and worsen outcome in the presence of carotid stenosis.We assessed the effect of glyceryl trinitrate(GTN)in patients with carotid stenosis using data from the Efficacy of Nitric Oxide in Stroke(ENOS)Trial.Methods ENOS randomised 4011 patients with acute stroke and raised systolic BP(140-220 mm Hg)to transdermal GTN or no GTN within 48 hours of onset.Those on prestroke antihypertensives were also randomised to stop or continue their medication for 7 days.The primary outcome was the modified Rankin Scale(mRS)at day 90.Ipsilateral carotid stenosis was split:<30%;30-<50%;50-<70%;≥70%.Data are ORs with 95%CIs adjusted for baseline prognostic factors.results 2023(60.5%)ischaemic stroke participants had carotid imaging.As compared with<30%,≥70%ipsilateral stenosis was associated with an unfavourable shift in mRS(worse outcome)at 90 days(OR 1.88,95%CI 1.44 to 2.44,p<0.001).Those with≥70%stenosis who received GTN versus no GTN had a favourable shift in mRS(OR 0.56,95%CI 0.34 to 0.93,p=0.024).In those with 50-<70%stenosis,continuing versus stopping prestroke antihypertensives was associated with worse disability,mood,quality of life and cognition at 90 days.Clinical outcomes did not differ across bilateral stenosis groups.Conclusions Following ischaemic stroke,severe ipsilateral carotid stenosis is associated with worse functional outcome at 90 days.GTN appears safe in ipsilateral or bilateral carotid stenosis,and might improve outcome in severe ipsilateral carotid stenosis. | Jason P Appleton Lisa J Woodhouse Andrew Belcher Daniel Bereczki Eivind Berge Valeria Caso Hui Meng Chang Hanne K Christensen Ronan Collins John Gommans Ann C Laska George Ntaios Serefnur Ozturk Gillian M Sare Szabolcs Szatmari Yongjun Wang Joanna M Wardlaw Nikola Sprigg Philip M Bath for the ENOS investigators | 2019 | Stroke & Vascular Neurology2019，4，1： | 6 |
| 10 | Age and menopausal status are important factors influencing the serum human epididymis secretory protein 4 level:a prospective cross-sectional study in healthy Chinese people显示文摘Background::Human epididymis secretory protein 4(HE4)is a new ovarian cancer biomarker.The factors influencing HE4 levels are not clear,and the reference data in China are limited.Here,we aim to evaluate the effects of menopause and age on HE4 levels and to provide a possible reference value for HE4 in healthy Chinese people.Methods::A total of 2493 healthy females aged 40 years or older were recruited from March 2013 to March 2017 with the cooperation of four medical institutions across Beijing,China.The serum levels of HE4 and cancer antigen 125(CA125)were measured by enzyme-linked immunosorbent assay.The Wilcoxon rank-sum test of variance and a stratified analysis were used to analyze the relationships among age,menopausal status,and levels of HE4 or CA125.Confidence intervals(5%-95%)were determined for reference ranges in different populations.Results::There was a statistically significant difference in median HE4 levels between the post-menopausal(n=2168)and premenopausal groups(n=325)(36.46 vs.24.04 pmol/L,Z=-14.41,P<0.001).HE4 increased significantly with age in the post-menopausal groups(H=408.18,P<0.001)but not in the pre-menopausal subjects(Z=-0.43,P=0.67).The upper 95th percentile of HE4 levels were 44.63 pmol/L for pre-menopausal women,78.17 pmol/L for post-menopausal women,and 73.3 pmol/L for all women.In the post-menopausal population,the HE4 reference ranges were 13.15 to 47.31,14.31 to 58.04,17.06 to 73.51,24.50 to 115.25,and 35.71 to 212.37 pmol/L for different age groups from forty divided by decade.The CA125 level was affected mainly by menopausal status and not age.Conclusions::Menopausal status and age were both important factors influencing the level of HE4,and age affected HE4 levels mainly in post-menopausal women.The HE4 level was higher in the post-menopausal population than in the pre-menopausal population and increased with age. | Hong-Yan Cheng Lin Zeng Xue Ye Rui-Qiong M a Zhi-Jian Tang Hong-Ling Chu Yi-M ing Zhao Li-Rong Zhu Yu-Nong Gao Xiao-Hong Chang Heng Cui | 2020 | Chinese Medical Journal2020，，11： | 6 |
| 11 | Bupivacaine causes cytotoxicity in mouse C2C12 myoblast cells: involvement of ERK and Akt signaling pathways显示文摘 | Joseph M MAURICE Yan GAN Fan-xin MA Yong-chang CHANG Michael HIBNER Yao HUANG | 2010 | Acta Pharmacologica Sinica2010，31，4： | 5 |
| 12 | A FINITE-ELEMENT ANALYSIS OF ELECTROMAGNETIC SHEET METAL-EXPANSION PROCESS显示文摘1INTRODUCTIONElectromagneticforming,esentialybeingakindofapplicationofimpulseintensivecurenttechniqueinmetalworking,dealswith... | Huang, SY Chang, ZH Wang, ZR Wang, LF Yang, M | 1998 | 中国有色金属学会会刊:英文版1998，8，3： | 4 |
| 13 | Role of observation of live cases done by Japanese experts in the acquisition of ESD skills by a western endoscopist显示文摘AIM:To evaluate the role of observation of experts performing endoscopic submucosal dissection(ESD)in the acquisition of ESD skills.METHODS:This prospective study is documenting the learning curve of one Western endoscopist.The study consisted of three periods.In the first period(preobservation),the trainee performed ESDs in animal models in his home institution in the United States.The second period(observation)consisted of visit to Japan and observation of live ESD cases done by experts.The observation of cases occurred over a 5-wk period.During the third period(post-observation),the trainee performed ESD in animal models in a similar fashion as in the first period.Three animal models were used:live40-50 kg Yorkshire pig,explanted pig stomach model,and explanted pig rectum model.The outcomes from the ESDs done in the animal models before and after observation of live human cases(main study intervention)were compared.Statistical analysis of the data included:Fisher’s exact test to compare distributions of a categorical variable,Wilcoxon rank sum test to compare distributions of a continuous variable between the two groups(pre-observation and post-observation),and Kruskal-Wallis test to evaluate the impact of lesion location and type of model(ex-vivo vs live pig)on lesion removal time.RESULTS:The trainee performed 38 ESDs in animal model(29 pre-observation/9 post-observation).The removal times post-observation were significantly shorter than those pre-observation(32.7±15.0 min vs 63.5±9.8 min,P<0.001).To minimize the impact of improving physician skill,the 9 lesions post-observation were compared to the last 9 lesions pre-observation and the removal times remained significantly shorter(32.7±15.0 min vs 61.0±7.4 min,P=0.0011).Regression analysis showed that ESD observation significantly reduced removal time when controlling for the sequence of lesion removal(P=0.025).Furthermore,it was also noted a trend towards decrease in failure to remove lesions and decrease in complications after the period of observation.This study did not find a significant difference in the time needed to remove lesions in different animal models.This finding could have important implications in designing training programs due to the substantial difference in cost between live animal and explanted organ models.The main limitation of this study is that it reflects the experience of a single endoscopist.CONCLUSION:Observation of experts performing ESD over short period of time can significantly contribute to the acquisition of ESD skills. | Peter V Draganov Myron Chang Roxana M Coman Mihir S Wagh Qi An Takuji Gotoda | 2014 | World Journal of Gastroenterology2014，20，16： | 3 |
| 14 | New universal current-mode filter with single input and three output using only five current conveyors显示文摘 | Chang C M | 1993 | Eletronics letters1993，29，： | 3 |
| 15 | Sedation-risk-free colonoscopy for minimizing the burden of colorectal cancer screening显示文摘Unsedated colonoscopy is available worldwide,but is not a routine option in the United States(US).We conducted a literature review supplemented by our experience and expert commentaries to provide data to support the use of unsedated colonoscopy for colorectal cancer screening.Medline data from 1966 to 2009 were searched to identify relevant articles on the subject.Data were summarized and co-authors provided critiques as well as accounts of unsedated colonoscopy for screening and surveillance.Diagnostic colonoscopy was initially dev eloped as an unsedated procedure.Procedure-re lated discomfort led to wide adoption of sedation in the US,although unsedated colonoscopy remains the usual practice elsewhere.The increased use of colonoscopy for colorectal cancer screening in healthy,asymptomatic individuals suggests a reass-essment of the burden of sedation in colonoscopy for screening is appropriate in the US for lowering costs and minimizing complications for patients.A water method developed to minimize discomfort has shown promise to enhance outcomes of unsedated colonoscopy.The use of scheduled,unsedated colono scopy in the US appears to be feasible for colorectal cancer screening.Studies to assess its applicability in diverse practice settings deserve to be conducted and supported. | Felix W Leung Abdulrahman M Aljebreen Emilio Brocchi Eugene B Chang Wei-Chih Liao Takeshi Mizukami Melvin Schapiro Konstantinos Triantafyllou | 2010 | World Journal of Gastrointestinal Endoscopy2010，2，3： | 3 |
| 16 | Antagonism of miR-33 in mice promotes reverse cholesterol transport and regression of atherosclerosis显示文摘 | Rayner Katey J Sheedy Frederick J Esau Christine C Hussain Farah N Temel Ryan E Parathath Saj van Gils Janine M Rayner Alistair J Chang Aaron N Suarez Yajaira Fernandez-Hernando Carlos Fisher Edward A Moore Kathryn J | 2011 | Journal of Clinical Investigation2011，，7： | 2 |
| 17 | Densities and P-x-y diagrams for carbo dioxide dissolution in methanol,ethano, and acetone mixtures显示文摘 | Chang C J Day C Y Ko C M | 1997 | Fluid Phase Equilib1997，131，： | 2 |
| 18 | Analysis of SSRs Uncovers Hierarchical Structure and Genetic Diversity in Chinese Soybean Landraces显示文摘For clarifying the hierarchical patterns of population structure of soybean landraces in China, the seven clusters previously identified using Bayesian clustering of 1 504 soybean landraces based on SSR markers genotyping data were further analyzed. Using the largest value of ΔK, these landraces could be split into 20 sub-clusters, which was supported by highly significant pairwise Fst-values and generally in accordance with the geographic origin and sowing types. The autumn-sowing types ended up in one distinct sub-cluster from the otherwise summer-sowing type, where the autumn-sowing types are most likely derived from. The division into 20 sub-clusters explained 7.3% of the genetic variation, next to 9.7% present among the seven clusters, 81.1% residing among landraces within sub-clusters, and 1.9% within the landraces. The distribution pattern of genetic diversity among the sub-clusters of each cluster was uneven, with two HSuM sub-clusters (Central China) and some South China sub-clusters showing significantly higher level of genetic diversity. | LI Ying-hui Marinus J M Smulders CHANG Ru-zhen QIU Li-juan | 2010 | Agricultural Sciences in China2010，9，12： | 2 |
| 19 | Intracellular Hmgb1 Inhibits Inflammatory Nucleosome Release and Limits Acute Pancreatitis in Mice显示文摘 | Rui Kang Qiuhong Zhang Wen Hou Zhenwen Yan Ruochan Chen Jillian Bonaroti Preeti Bansal Timothy R. Billiar Allan Tsung Qingde Wang David L. Bartlett David C. Whitcomb Eugene B. Chang Xiaorong Zhu Haichao Wang Ben Lu Kevin J. Tracey Lizhi Cao Xue-Gong Fan M | 2013 | Gastroenterology2013，，： | 2 |
| 20 | Comprehensive review of autoantibodies in patients with hyper-IgM syndrome显示文摘Hyper-immunoglobulin M syndrome is an X-linked primary immunodeficiency disease caused by mutations in the CD40 ligand gene.The CD40 ligand has been recently highlighted as playing a key role in the pathogenesis of primary biliary cholangitis.In the present study,we assessed an extensive set of serum autoantibodies in a series of well-defined patients with hyper-immunoglobulin M syndrome.Serum,liver-related and liver-not-related autoantibodies IgG,IgM and IgA were tested by ELISA and standard indirect immunofluorescence in HEp-2 cells in 13 Tunisian patients(8 males and 5 females,aged 1–12 years)with hyper-immunoglobulin M syndrome during 1995–2012 and,as controls,21 age-and gender-matched blood donors.The level of IgM antibody against MIT3 was significantly higher in patients than in controls(35.8 vs 10.7,P=0.002).Half of the hyperimmunoglobulin M syndrome patients were found to be anti-MIT3 IgM positive vs none of the controls(Po0.0001).Twenty-three percent of patients were found to be anti-sp100 antibody positive vs only 0.05%of controls.By immunofluorescence,92.3%of patients were MIT3 IgM positive vs none of the controls.In conclusion,the IgM class of anti-MIT3 antibodies was shown to be present by both ELISA and immunofluorescence in most of the patients with hyper-immunoglobulin M syndrome.The presence of the hallmark of primary biliary cholangitis,a disease where the CD40 ligand is a key player,in an immunodeficiency disease caused by mutations in the CD40 ligand gene is very intriguing and opens new scenarios in understanding the immune pathogenesis of primary biliary cholangitis. | Mohamed-Ridha Barbouche Qubo Chen Marco Carbone Imen Ben-Mustapha Zakera Shums Mehdi Trifa Federica Malinverno Francesca Bernuzzi Haiyan Zhang Nourhen Agrebi Gary L Norman Christopher Chang M Eric Gershwin Pietro Invernizzi | 2018 | Cellular & Molecular Immunology2018，15，6： | 2 |
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