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Long noncoding nuclear enriched abundant transcript 1_2 is a promising biomarker for childhood-onset systemic lupus erythematosus

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Pediatric Investigation 2024年第2期8卷 101-107页

作者： Shipeng li Xia Wang Xiaozhen Zhao Jianghong Deng Weiying Kuang Junmei Zhang Xiaohua Tan Chao li caifeng li Department of Rheumatology Beijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijingChina Center for Infectious Diseases Beijing Youan HospitalCapital Medical UniversityBeijing Key Laboratory for HIV/AIDS ResearchBeijingChina

Importance:Systemic lupus erythematosus(SLE)is a diffuse connective tissue disease with complex clinical manifestations and prolonged *** early diagnosis and condition monitoring of SLE are crucial to disease ***:To assess the diagnostic value of long noncoding RNA(lncRNA)nuclear enriched abundant transcript 1(NEAT1)in childhood-onset SLE(cSLE).Methods:Fifty-seven children diagnosed with SLE,40 children diagnosed with juvenile idiopathic arthritis(JIA),and 40 healthy children were *** blood samples from each patient were collected.A quantitative polymerase chain reaction was used to confirm the expression of lncNEAT1_1 and lncNEAT1_2 in peripheral *** among parameters were analyzed using the Mann-Whitney U test or independent sample ***:The expression of both lncNEAT1_1 and lncNEAT1_2 in patients with cSLE were significantly higher than that of healthy control and patients with *** operating characteristic curves revealed an area under the curve(AUC)of 0.633(95%confidence interval[CI],0.524-0.742;P=0.024)for lncNEAT1_*** AUC of lncNEAT1_2 was 0.812(95%CI,0.727-0.897;P<0.0001)to discriminate individuals with cSLE from health control and children with JIA with a sensitivity of 0.622 and a specificity of ***,lncNEAT1_2 expression was higher in patients with cSLE presenting with fever,lupus nephritis,elevated erythrocyte sedimentation rate,active disease activity,and decreased C3 level,compared with those without these ***,no similar correlation was observed for lncNEAT1_***:The expression of lncNEAT1_2 was significantly elevated in children with SLE,especially those with fever,renal involvement,and low C3 *** findings suggest that lncNEAT1_2 may represent a potential biomarker for cSLE.

关键词： Biomarker Children LncRNA SLE NEAT1

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认识儿童Ⅰ型干扰素通路病——早期识别,精准治疗,改善预后

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中国临床新医学 2024年第9期17卷 955-960页

作者：高凤乔李彩凤国家儿童医学中心首都医科大学附属北京儿童医院风湿科北京100045

Ⅰ型干扰素通路病是一类由单基因突变引起的自身炎症性疾病,其特征是Ⅰ型干扰素信号通路异常激活,导致广泛的全身性炎症反应。在日常自身免疫性疾病诊疗中,早期识别这类疾病至关重要。对于起病年龄小、多系统炎症表现及自身抗体阳性的患... 详细信息

Ⅰ型干扰素通路病是一类由单基因突变引起的自身炎症性疾病,其特征是Ⅰ型干扰素信号通路异常激活,导致广泛的全身性炎症反应。在日常自身免疫性疾病诊疗中,早期识别这类疾病至关重要。对于起病年龄小、多系统炎症表现及自身抗体阳性的患者,临床医师应高度警惕Ⅰ型干扰素通路病,必要时进行基因检测以尽早诊断。早期诊断,精准治疗,不仅有助于及时控制炎症,还能有效预防或减缓脏器损伤的进展,显著改善患者的长期预后。

关键词： Ⅰ型干扰素通路病儿童基因突变诊断靶向治疗自身炎症性疾病

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儿童系统性红斑狼疮合并血栓性微血管病的临床特点及预后分析

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中华实用儿科临床杂志 2024年第9期39卷 666-671页

作者：邓江红刘炫邑李士朋高凤乔邝伟英张俊梅檀晓华李超薛媛李彩凤国家儿童医学中心首都医科大学附属北京儿童医院风湿科首都医科大学风湿病学系北京100045

目的分析系统性红斑狼疮(SLE)合并血栓性微血管病(TMA)患儿的临床特征,并通过其治疗及随访情况明确患儿的临床结局及相关危险因素。方法单中心回顾性病例对照研究。选取2017年1月至2023年1月首都医科大学附属北京儿童医院确诊为SLE合并... 详细信息

目的分析系统性红斑狼疮(SLE)合并血栓性微血管病(TMA)患儿的临床特征,并通过其治疗及随访情况明确患儿的临床结局及相关危险因素。方法单中心回顾性病例对照研究。选取2017年1月至2023年1月首都医科大学附属北京儿童医院确诊为SLE合并TMA的患儿为TMA组,并选取SLE未合并TMA患儿作为对照组。根据TMA组患儿治疗后的预后情况进一步分为预后良好组及预后不良组。收集患儿年龄、性别、SLE疾病活动、诊断时及血栓时的临床表现、实验室检查、治疗策略和预后及随访结果。计数资料比较采用χ^(2)检验和Z检验。计量配对资料比较采用t检验。采用Fisher′s精确概率法比较2组分类变量间的差异。采用单因素Logistic回归分析对预后不良危险因素进行分析。结果TMA组29例,TMA发生率占SLE患儿的2.53%;对照组33例。诊断TMA年龄为13岁5个月(9岁1个月5 d至17岁4个月),常见临床表现依次为肾脏受累(28例,96.55%),血液系统受累(26例,89.66%),浆膜腔积液(17例,58.62%),皮疹(13例,44.82%),神经系统受累(12例,41.38%)。TMA组发生胸膜炎或心包炎、肾脏受累及神经系统受累多于对照组(17例比3例、28例比10例、12例比3例);TMA组的面部皮疹及关节炎表现均低于对照组(13例比25例、4例比17例),差异均有统计学意义(均P<0.05)。TMA组SLE疾病活动指数评分[(24.14±9.42)分]明显高于对照组[(10.18±9.42)分],差异有统计学意义(t=3.233,P<0.05)。TMA组患儿的血红蛋白水平、血小板、补体C3水平均明显低于对照组,双链DNA抗体、乳酸脱氢酶、D二聚体、尿素、肌酐、铁蛋白水平、尿蛋白定量均明显高于对照组,差异均有统计学意义(均P<0.05)。TMA组中5例ADAMTS13活性降低,5例补体C5b9明显增高。TMA组中共15例(51.72%)患儿行肾活检,其中13例合并肾TMA。TMA组中28例(96.6%)患儿接受激素治疗,17例患儿接受血浆置换,12例联合免疫抑制剂及生物制剂治疗,19例(65.5%)好转,10例(34.5%)因病情恶化放弃治疗。预后良好组的尿素水平及外周血破碎红细胞百分率明显低于预后不良组[(13.18±4.39)mmol/L比(21.16±10.14)mmol/L,t=2.975,P=0.006;8/17(47.06%)比7/7(100%),χ^(2)=5.929,P=0.015]。单因素Logistic回归分析显示,破碎红细胞、ADAMTS13活性及尿素均为预后不良的独立危险因素(均P<0.05)。结论对于中重度疾病活动的SLE患儿,尤其是以溶血性贫血、血小板减少、肾功能异常为突出表现者,需警惕合并TMA的风险,早期诊断、及早制定诊疗策略至关重要。

关键词：系统性红斑狼疮血栓性微血管病临床特征危险因素儿童

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IgA肾病患者使用肾素血管紧张素系统抑制剂的真实世界研究

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实用药物与临床 2024年第7期27卷 506-510页

作者：李彩凤林丽瑜黄英滕杰复旦大学附属中山医院厦门医院肾内科福建厦门361015 厦门市肾病专业质量控制中心福建厦门361015

目的调查根据指南及质控标准应使用肾素血管紧张素系统(RAS)抑制剂治疗的IgA肾病患者RAS抑制剂的实际使用率、用药剂量、治疗效果及未使用的原因。方法选择2019年1月至2023年6月在复旦大学附属中山医院厦门医院诊断为原发性IgA肾病且根... 详细信息

目的调查根据指南及质控标准应使用肾素血管紧张素系统(RAS)抑制剂治疗的IgA肾病患者RAS抑制剂的实际使用率、用药剂量、治疗效果及未使用的原因。方法选择2019年1月至2023年6月在复旦大学附属中山医院厦门医院诊断为原发性IgA肾病且根据指南和质控标准有RAS抑制剂使用适应证而无禁忌证[尿蛋白定量≥0.5 g/d且肾小球滤过率(eGFR)≥30 ml/min、无肾动脉狭窄或低血压]的患者154例,将其中使用RAS抑制剂且随访资料完整的患者122例,根据是否使用糖皮质激素分为RAS抑制剂联合激素组(52例)、RAS抑制剂组(70例),收集患者的临床资料、肾脏病理及实验室指标,记录患者RAS抑制剂及糖皮质激素使用情况,随访尿蛋白、血清肌酐、eGFR、钾及血压等变化情况并进行统计分析。结果154例患者中有132例使用RAS抑制剂治疗,RAS抑制剂的使用率为85.7%,87.1%的患者使用了标准剂量,3.8%的患者使用了0.5倍剂量,9.1%的患者使用了双倍剂量。无论是否联用糖皮质激素,RAS抑制剂治疗后,患者尿蛋白排泄均较治疗前下降[0.30(0.15,0.66)g/d vs.1.83(1.21,2.37)g/d,0.33(0.20,0.61)g/d vs.1.05(0.70,1.63)g/d](P<0.05),eGFR维持稳定。未使用RAS抑制剂的患者22例,其中10例因轻中度肾功能不全未用药,5例因合并急性肾损伤(AKI)未使用,4例因基础血压<100/60 mmHg未使用,1例因高钾血症未使用。结论RAS抑制剂可减少IgA肾病患者尿蛋白排泄,大部分IgA肾病患者能遵循指南使用RAS抑制剂治疗,未用药的主要原因是轻中度肾功能不全和基础血压偏低,严密监测肾功能和血压可能有助于提高RAS抑制剂的使用率。

关键词： IgA肾病肾素血管紧张素系统抑制剂蛋白尿肾小球滤过率质量控制真实世界研究

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Effcacy-oriented compatibility for Tianma(Rhizoma Gastrodiae),Yanlingcao(Trillium tschonoskii Maxim) and Bingpian(Borneolum Syntheticum) on improving cerebral ischemia stroke by network pharmacology and serum pharmacological methods

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Journal of Traditional Chinese Medicine 2022年第3期42卷 408-416页

作者： li Zhiyong ZHU Na li Jianliang FENG liang JIANG Yanyan li caifeng liN ling HUANG Xiulan Postdoctoral mobile station of environmental biology Faculty of Life Science and TechnologyKunming University of Science and TechnologyKunmingYunnan 650500China Center for Analytical Chemistry of Chinese Materia Medica Institute of Chinese Materia MedicaChina Academy of Chinese Medical SciencesBeijing 100700China Pharmacological Department School of PharmacyMinzu University of ChinaBeijing100081China Aerosol preparation research center Institute of Chinese Materia MedicaChina Academy of Chinese Medical SciencesBeijing 100700China School of Traditional Chinese Pharmacy China Pharmaceutical UniversityNanjingJiangsu 211198China Department of Chinese Materia Medica Chemistry School of Chinese Materia MedicaBeijing University of Chinese MedicineBeijing102488China Academician work station Jiangxi University of Traditional Chinese MedicineNanchangJiangxi330004China

OBJECTIVE:To evaluate the compatibility of Tianma(Rhizoma Gastrodiae,TM),Yanlingcao(Trillium tschonoskii Maxim,YLC)and Bingpian(Borneolum Syntheticum,BP),and their efficacy in the treatment of cerebral ischemic ***:Network pharmacology was used to determine the compatibility of TM,YLC,and BP,and their potential *** middle cerebral artery occlusion(MCAO)rat model was used to evaluate the curative effect of the six combinations of TM,YLC,and BP(TZB1-TZB6)on cerebral ischemia,by using the weight matching method to *** potential component changes of TM and YLC in the blood and brains of rats were analyzed using ultra performance liquid chromatography-mass ***,molecular docking linked the results of animal experiments and network pharmacology,determining the potential component contributors of TM and YLC to treating ischemic ***:TZB reduced the cerebral infarct volume and protected the nerve cells in MCAO *** components of TM and YLC were also identified in the blood and brain homogenate,and BP can facilitate the entry of the components of TM and YLC into the blood and ***,pennogenin,and gastrodin induced effective binding activities with adenosine receptor ***:We investigate an approach that improves the means of folk prescription combined with multi technology that maybe promote the transformation of Chinese medicinal prescription into component-based Chinese medicine.

关键词： infarction,middle cerebral artery brain ischemia molecular docking simulation Adenosine receptor a1 network pharmacology

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新型耐温耐盐微生物多糖性能及驱油能力

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应用与环境生物学报 2024年第4期30卷 686-692页

作者：刘涛胡婧李彩风岳胜辉宋永亭汪卫东曹功泽束青林中国石化胜利油田分公司石油工程技术研究院微生物采油研究所东营257000

高温高盐油藏水驱过程中平面和纵向非均质性加剧,导致水淹水窜,注水利用率低,现有的聚合物体系不能完全满足开发需求.研究发酵得到了新型耐温耐盐微生物多糖,利用不同方法解析明确微生物多糖的结构特征,确定为一种新型微生物多糖.该微... 详细信息

高温高盐油藏水驱过程中平面和纵向非均质性加剧,导致水淹水窜,注水利用率低,现有的聚合物体系不能完全满足开发需求.研究发酵得到了新型耐温耐盐微生物多糖,利用不同方法解析明确微生物多糖的结构特征,确定为一种新型微生物多糖.该微生物多糖在高温90℃、高盐50000 mg/L条件下放置60 d后保持了高的黏弹特性,能够满足高温高盐油藏的需求.不同渗透率胶结岩心注入性表明微生物多糖在渗透率>300×10^(-3)μm^(2)的岩心不同位点均能有效建立驱替压差,微生物多糖能够有效运移到油藏深部,扩大在油藏中的波及范围;岩心驱油试验表明在空白岩心的基础上提高驱替效率13.2%,含水最大降幅18.4%.本研究得到的微生物多糖体系为高温高盐油藏提高采收率提供了一条可行的技术途径.(图12表6参30)

关键词：高温高盐油藏微生物多糖黏弹性运移驱替效率

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Gene mutations and clinical phenotypes in Chinese children with Blau syndrome

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Science China(life Sciences) 2017年第7期60卷 758-762页

作者： caifeng li Junmei Zhang Shipeng li Tongxin Han Weiying Kuang Yifang Zhou Jianghong Deng Xiaohua Tan Department of Rheumatology and Immunology Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing 100045 China

The mutations of CARD15 gene and clinical features of Chinese patients with Blau syndrome were analyzed. We identified10 missense mutations, out of which five were new: R334 L, E383 D, R471 C, C495 R and D512 F. The rest of them, R334 W,R334Q, G481 D, M513 T and R587 C, have been reported previously. Among all the mutations, R334 W, R334 Q and C495 R had the highest frequency. Blau syndrome was found at early age after birth. It began with lepidic rash and symmetric polyarthritis and was phenotypically characterized by typical rash, arthritis, iridocyclitis and arteritis. Cardiac involvement was also found in Blau syndrome. In addition to nerve deafness, renal involvement, osteochondroma and central nervous system involvement were also found in our patients. Therefore, Chinese children with Blau syndrome have unique gene mutations and complicated clinical phenotypes. Pathologic examination and CARD15 mutation testing should be considered for diagnosis as early as possible for suspected patients.

关键词： Blau syndrome genetic mutation clinical phenotype

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An Extracellular Oligopeptide Permease May Be a Potential Virulence Factor of Vibrio harveyi

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Journal of Ocean University of China 2011年第4期10卷 343-350页

作者： HE Qingfang CHEN Jixiang li caifeng Department of Marine Biology College of Marine Life SciencesOcean University of ChinaQingdao 266003P.R.China Oil Production Technology Research Institute Shengli Oilfield Company of SINOPECDongying 257000P.R.China

An oligopeptide permease A(OppA)was purified from the extracellular product of Vibrio harveyi *** molecular weight of the purified protein was estimated to be 58 kDa on *** purified protein showed phospholipase C activity at the optimal values of temperature 50℃ and pH *** enzymatic activity decreased when the temperature increased to 40℃.The N-terminal sequence of the purified protein was determined as ADVPAGTKLA,which is similar to that of *** OppA pre-cursor gene was cloned from the genome of *** *** gene consisted of 1665 base pairs and encoded a 554 amino acid polypeptide,which showed a high similarity to those of the OppAs of *** and other Vibrio *** gene was subcloned into pET-28a(+)and expressed in Escherichia *** expressed recombinant protein was purified by Ni-NTA metal affinity *** expressed recombinant protein showed a 58 kDa band on SDS-PAGE and exhibited phospholipase C activity with the optima of temperature 50℃ and pH *** purified protein was toxic to the flounder gill *** OppA mutant of *** SF-1 was constructed by homologous *** mutant strain was less virulent when it was intraperitoneally inoculated to zebra fish,with the LD50 of 5.46×105 CFU fish-1,compared to 3.11×104 CFU fish-1 of the wild-type strain,which indicated that the OppA might play an important role in the pathogenicity of ***.

关键词： Vibrio harveyi oligopeptide permease phospholipase C pathogenicity

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Correlations between the North China Craton and the Indian Shield:Constraints from regional metallogeny

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Geoscience Frontiers 2015年第6期6卷 861-873页

作者： caifeng li Dongyue Chen Jianping Chen Xizhen Chen Xingchen Yang M.A.Aboelnour School of Earth Sciences and Resources China University of Geosciences(Beijing) Institute of High and New Techniques Applied to Land Resources China University of Geosciences(Beijing) Institute of Water Resources and Hydropower Wuhan University Institute of Geomechanics Chinese Academy of Geological Sciences

The correlation between the North China Craton（NCC） and the Indian Shield（IND） has been a hot topic in recent years,On the basis of ore deposit databases,the NCC and IND have shown broad similarity in metallogenesis from the middle Archaean to the Mesoproterozoic,The two blocks both have three major metallogenic systems：（1） the Archaean BIF metallogenic system;（2） the Paleoproterozoic Cu-Pb-Zn metallogenic system;and（3） the Mesoproterozoic Fe-Pb-Zn system,In the north margin of the NCC and the west margin of the IND,the Archaean BIF-Au-Cu-Pb-Zn deposits had the same petrogenesis and host rocks,the Paleoproterozoic Cu-Pb-Zn deposits were controlled by active belts,and the Mesoproterozoic Fe-Pb-Zn deposits were mainly related to multi-stage rifting,Matching regional mineralization patterns and geological features has established the continental assembly referred to as ＂NCWI＂,an acronym for the north margin of the NCC（NC） and the west margin of the IND（WI） during the middle Archaean to the Mesoproterozoic,In this assembly,the available geological and metallogenic data from the Eastern Block and active belts of NC fit those from the Dharwar craton and the Aravalli-DelhiVindhyan belt of WI,respectively,Moreover,the depositional model and environment of Paleoproterozoic metasedimentary manganese deposits in NCWI implied that the assembly may be located at low latitudes,where the conditions were favorable for dissolving ice and precipitating manganese deposits,

关键词： North China Craton Indian shield Metallogeny Archaean Correlations Mesoproterozoic

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Gene mutations and clinical phenotypes in 15 Chinese children with cryopyrin-associated periodic syndrome(CAPS)

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Science China(life Sciences) 2017年第12期60卷 1436-1444页

作者： caifeng li Xiaohua Tan Junmei Zhang Shipeng li Wenxiu Mo Tongxin Han Weiying Kuang Yifang Zhou Jianghong Deng Department of Rheumatology and Immunology Beijing Children's HospitalCapital Medical UniversityNational Center for Children"s HealthBeijing 100045China

The aim of our study is to explore the features of clinical manifestations and genetic mutations in Chinese CAPS patients. Fifteen confirmed patients with CAPS were enrolled. The onset time ranges from 2 days after birth to 6 years and 1 month. Recurrent urticaria rash(93.3%) with fever(100%) were two dominant characteristics of these patients that were presented as either acute or chronic process. Systemic involvements were found in all patients except for one with only rash and fever. The top three symptoms were fever(100%), rash(93.3%) and myalgia(76%). Other clinical manifestations include arthritis(11 cases), lung involvement(seven cases), optical dysfunction(seven cases), nerve deafness(six cases), nervous system involvement(five cases), hepatomegaly, splenomegaly and lymphadenectasis(five cases). Also, four patients had heart involvement and one patient suffered kidney involvement. The laboratory inflammation index such as leukocyte counts, platelet counts, erythrocyte sedimentation rate(ESR), C-reactive protein(CRP), serum amyloid A(SAA) and fibrinogen(FIB) increased significantly at initial stage, but decreased after therapy. As for gene mutation detection, Twelve out of 15 patients were confirmed with mutation in NLRP3, including 11 mutant site: c1789 A>G, c.1703 T>A, c.913 G>A, c.1710 G>C, c.1057 G >T, c.2335 C>T, c.932 T>C,c.296 G>C, c.663 C>T, t.1702 T>A, 299 G>A. Mutation sites c.1703 T>A, c.2335 C>T, c.296 G>C, c.663 C>T, and 299 G>A were newly identified. The association between gene mutation and clinical manifestation shows that D305 N was highly associated with severe organs involvements, and therefore, the time of therapy and regimen were critical for the prognosis of disease. As the largest cohort study of Chinese CAPS patients, we confirmed that all patients presented typical clinical manifestations, identified five new mutation sites on NLRP3 and analyzed the correlation between the genotypes and phenotypes. We also raise concerns for one case

关键词： CAPS genetic mutation clinical phenotTpe NLRP3

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