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Bullous pemphigoid associated with acquired hemophilia A: A case report

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World Journal of Clinical Cases 2025年第4期13卷 28-33页

作者： Su-Ye Hu Meng-Can Li zi-Jia Hao Xu-Ya Chai Pei-Sai Li yang liu Li-Xia liu Ying Xu Pan-Pan yang Ling-E Li Department of Dermatology Shijiazhuang Traditional Chinese Medicine HospitalShijiazhuang 050051Hebei ProvinceChina Graduate School Hebei University of Traditional Chinese MedicineShijiazhuang 050051Hebei ProvinceChina Department of Pathology Shijiazhuang Traditional Chinese Medicine HospitalShijiazhuang 050051Hebei ProvinceChina

BACKGROUND Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disordercaused by circulating autoantibodies against factor Ⅷ (FⅧ). In approximately50% of the patients, the condition is associated with autoimmune diseases,cancers, medication use, pregnancy, and the post-partum period. Bullous pemphigoid(BP) is a chronic autoimmune subepidermal blistering disease associatedwith tissue-bound and circulating autoantibodies against BP antigens 180 (BP180)and 230 (BP230). AHA-associated BP has a high mortality rate;hence, the understandingof this disease must *** SUMMARY A 69-year-old man presented with erythema, blisters, blood blisters, and crustsaccompanied by severe pruritus for more than 20 days, and ecchymosis andswelling on his left upper arm for 3 days. Pathological examination revealed asubepidermal blister that contained eosinophils. Laboratory tests showed that theBP180 autoantibody levels had increased, isolated activated partial thromboplastintime was notably prolonged (115.6 s), and coagulation FⅧ activity wasextremely low (< 1.0%). Furthermore, the FⅧ inhibitor titer had greatlyincreased (59.2 Bethesda units). Therefore, the patient was diagnosed as having BP associated with AHA, prescribed 0.05% topical halometasone cream, and transferred to a higher-level hospitalfor effective treatment;however, he died after 2 *** AHA associated BP is rare, dangerous, and has a high mortality rate. Therefore, its timely diagnosis and effectivetreatment are necessary.

关键词： Bullous pemphigoid Acquired hemophilia A Acquired hemophilia A FactorⅧ Case report

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GdNi_2合金电弧放电合成钆富勒

应用化学 2005年第3期22卷 238-241页

作者：郑奎玲刘子阳朱妙琴任小元贵州师范大学理学院浙江大学化学系杭州310027

利用GdNi2合金为金属源电弧放电合成金属富勒烯,用甲苯提取和吡啶高温高压相结合的方法得到不同的提取液。用激光解吸质谱法分析提取液的富勒烯组成。研究了石墨棒中引入不同比例的金属钆对富勒烯和金属钆富勒烯生成产率的影响,优化了... 详细信息

利用GdNi2合金为金属源电弧放电合成金属富勒烯,用甲苯提取和吡啶高温高压相结合的方法得到不同的提取液。用激光解吸质谱法分析提取液的富勒烯组成。研究了石墨棒中引入不同比例的金属钆对富勒烯和金属钆富勒烯生成产率的影响,优化了电弧放电条件,在电流为 90A、电压为 25V、电极放电距离为8mm、He气压力为 40kPa的条件下,x(Gd)∶x(C) =1∶50时,金属富勒烯产率为 2 .54%。

关键词：金属富勒烯钆钆镍合金电弧放电合成

Formation and evolution of supercritical geofluid

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Science China Earth Sciences 2025年第1期68卷 39-51页

作者： Huaiwei NI Yilin XIAO Xiaolin XIONG Xiandong liu Chunxiao GAO Yi-Xiang CHEN Yunguo LI Wan-Cai LI Xuan GUO yang-yang WANG Dong-Bo TAN Li ZHANG State Key Laboratory of Lithospheric and Environmental Coevolution University of Science and Technology of China Deep Space Exploration Laboratory/School of Earth and Space Sciences University of Science and Technology of China Guangzhou Institute of Geochemistry Chinese Academy of Sciences School of Earth Sciences and Engineering Nanjing University State Key Lab of Superhard Materials Jilin University

In this work, we provide a comprehensive review on the formation, evolution, properties, and effects of supercritical geofluid. In Earth's interior, enhanced miscibility between H2O and silicate by the addition of special components or by the increase of pressure and temperature gives rise to supercritical geofluid with a significant amount of both H2O and silicate *** formation of supercritical geofluid in magmatic-hydrothermal systems, typified by pegmatite system, is governed by meltfluid critical curve. The formation of supercritical geofluid in metamorphic systems, typified by subducted slab, is governed by the second critical end point. Experimental results suggest that the presence of boron and fluorine in pegmatite system makes it possible to form supercritical geofluid at crustal depths, but the release of supercritical geofluid from subducted slab is withheld until almost 100 km depth. A major presence of both H2O and depolymerized structural units（monomers, dimers, etc.） endows supercritical geofluid with unique physical properties including low density, low elastic moduli, low viscosity, high diffusivity,and high electrical conductivity. Supercritical geofluid can effectively mobilize a variety of elements even including high field strength elements and heavy rare earth elements. The chemical signatures of supercritical geofluid can be inherited by metasomatized mantle and mantle-derived melts, and this could give an explanation of the oxidation of arc magmas. Phase separation of supercritical geofluid through the mechanism of spinodal decomposition leads to formation of a melt network. Multiphase fluid inclusions recovered from subduction zone rocks and pegmatites are possible relics of supercritical geofluid. Supercritical geofluid can cause electrical anomaly and low seismic velocity near the top of subducted slab, and can be linked with intermediate-focus earthquakes. Supercritical geofluid may have played a crucial role in the formation of pe

关键词： Supercritical geofluid Miscibility Subduction zone Pegmatite

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息肉状脉络膜血管病变抗血管内皮生长因子(VEGF)治疗后1年的视力预后与基线特征的相关性分析

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眼科新进展 2018年第5期38卷 440-443页

作者：刘子扬夏松杨景元陈有信中国医学科学院北京协和医学院北京协和医院眼科北京市100730

目的分析息肉状脉络膜血管病变(polypoidal choroidal vasculopathy,PCV)经过连续3个月的每月抗血管内皮生长因子(vescular endothelial growth factor,VEGF)治疗后1 a的视力预后与基线特征的相关性。方法回顾性队列研究。收集2015年7月... 详细信息

目的分析息肉状脉络膜血管病变(polypoidal choroidal vasculopathy,PCV)经过连续3个月的每月抗血管内皮生长因子(vescular endothelial growth factor,VEGF)治疗后1 a的视力预后与基线特征的相关性。方法回顾性队列研究。收集2015年7月至2016年12月于我院就诊的PCV患者44例(44眼),所有患者在基线时均行最佳矫正视力(bestcorrected visual acuity,BCVA)、眼压、眼底检查以及光学相干断层扫描、眼底荧光素血管造影和吲哚青绿血管造影检查。所有患者在确诊后均行连续3个月的每月1次抗VEGF(包括雷珠单抗和康柏西普)注射,随后按需治疗,记录第1次抗VEGF治疗后随访1 a的BCVA并与基线BCVA比较,依据BCVA变化分为BCVA提高组和未提高组,单因素和Logistic回归分析BCVA预后与基线特征的相关性。结果单因素分析结果显示,BCVA提高组比BCVA未提高组具有较短的发病时间、较小的病灶最大线性尺寸(P=0.045、0.037)。Logistic回归结果显示,脉络膜高渗透性和病灶最大线性尺寸是视力预后的独立相关因素(回归系数为0.963和0.001;P=0.010、0.012;比值比为0.083和1.002;95%的可信区间为0.013～0.549和1.001～1.004μm)。结论合并脉络膜血管高渗透性可能是经抗VEGF治疗的PCV患眼视力预后不佳的预测指标,同时病灶的最大线性尺寸和发病时间也与PCV经抗VEGF治疗后的BCVA预后相关。

关键词：息肉状脉络膜血管病变血管内皮生长因子最佳矫正视力脉络膜血管高渗透性

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型钢热轧润滑的研究

钢铁研究总院学报 1985年第2期 131-136页

作者：刘庆贺杨子

为延长轧辊使用寿命和提高轧机生产能力,在型钢轧机上应用工艺润滑是很必要的。本文讨论的是在φ326mm型钢轧机上轧制角钢时应用工艺润滑的方法和试验结果。试验表明,润滑轧制得到了以下效果:孔型磨损减少50％,轧辊使用寿命延长一倍,轧... 详细信息

为延长轧辊使用寿命和提高轧机生产能力,在型钢轧机上应用工艺润滑是很必要的。本文讨论的是在φ326mm型钢轧机上轧制角钢时应用工艺润滑的方法和试验结果。试验表明,润滑轧制得到了以下效果:孔型磨损减少50％,轧辊使用寿命延长一倍,轧辊重车时的消耗减少45.8％;由于轧辊表面的改善而轧制出表面质量好的角钢,角钢一边的厚度差改善57％;轧制负荷和轧制能耗分别降低23％和15％。

关键词：润滑轧制角钢厚度工艺润滑表面质量轧辊表面型钢轧机轧辊使用孔型磨损

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基于遥测数据的无人机横向气动参数辨识

飞行力学 2018年第4期36卷 88-91,96页

作者：羊雨璇高占宝刘紫阳李绪隆北京航空航天大学自动化科学与电气工程学院北京100191

针对某型无人机实际飞行中遥测数据不完备的问题,提供了相关参数的估计方法,并利用风速、地速、空速三者的矢量关系估计风场来修正气动参数值;针对横侧向模型辨识精度不高的问题,建立了一种新的辨识模型。辨识结果表明,所提方法提高了... 详细信息

针对某型无人机实际飞行中遥测数据不完备的问题,提供了相关参数的估计方法,并利用风速、地速、空速三者的矢量关系估计风场来修正气动参数值;针对横侧向模型辨识精度不高的问题,建立了一种新的辨识模型。辨识结果表明,所提方法提高了模型的准确度,即辨识精度,同时验证了方法在平飞段和爬升段的有效性。

关键词：遥测数据参数估计风场修正横侧向气动参数辨识

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论加强多党合作与提高中国共产党的执政能力

广西社会主义学院学报 2009年第6期20卷 26-29页

作者：郑毅刘洋子桂林电子科技大学广西桂林541001

加强党的执政能力建设,是坚持和巩固党的执政地位的需要,也是时代赋予党的使命。多党合作制度作为处理政党关系的重要政治制度,是提高党的执政能力的重要基础、重要资源和保证。因此,应通过加强多党合作来提高党的执政能力。

关键词：党的执政能力多党合作关系探索

AAV2-PDE6B restores retinal structure and function in the retinal degeneration 10 mouse model of retinitis pigmentosa by promoting phototransduction and inhibiting apoptosis

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Neural Regeneration Research 2025年第8期20卷 2408-2419页

作者： Ruiqi Qiu Mingzhu yang Xiuxiu Jin Jingyang liu Weiping Wang Xiaoli Zhang Jinfeng Han Bo Lei Henan Eye Hospital Henan Provincial People’s HospitalPeople’s Hospital of Zhengzhou UniversityZhengzhouHenan ProvinceChina Eye Institute Henan Academy of Innovations in Medical ScienceZhengzhouHenan ProvinceChina Branch of National Clinical Research Center for Ocular Disease Henan Provincial People’s HospitalZhengzhouHenan ProvinceChina Academy of Medical Science Zhengzhou UniversityZhengzhouHenan ProvinceChina

Retinitis pigmentosa is a group of inherited diseases that lead to retinal degeneration and photoreceptor cell ***,there is no effective treatment for retinitis pigmentosa caused by PDE6B ***-associated virus(AAV)-mediated gene therapy is a promising strategy for treating retinitis *** aim of this study was to explore the molecular mechanisms by which AAV2-PDE6B rescues retinal *** do this,we injected retinal degeneration 10(rd10)mice subretinally with AAV2-PDE6B and assessed the therapeutic effects on retinal function and structure using dark-and light-adapted electroretinogram,optical coherence tomography,and ***-independent acquisition-mass spectrometry-based proteomic analysis was conducted to investigate protein expression levels and pathway enrichment,and the results from this analysis were verified by real-time polymerase chain reaction and western ***2-PDE6B injection significantly upregulated PDE6βexpression,preserved electroretinogram responses,and preserved outer nuclear layer thickness in rd10 *** expressed proteins between wild-type and rd10 mice were closely related to visual perception,and treating rd10 mice with AAV2-PDE6B restored differentially expressed protein expression to levels similar to those seen in wild-type *** Encyclopedia of Genes and Genome analysis showed that the differentially expressed proteins whose expression was most significantly altered by AAV2-PDE6B injection were enriched in phototransduction ***,the phototransductionrelated proteins Pde6α,Rom1,Rho,Aldh1a1,and Rbp1 exhibited opposite expression patterns in rd10 mice with or without AAV2-PDE6B ***,Bax/Bcl-2,p-ERK/ERK,and p-c-Fos/c-Fos expression levels decreased in rd10 mice following AAV2-PDE6B *** data suggest that AAV2-PDE6B-mediated gene therapy promotes phototransduction and inhibits apoptosis by inhibiting the ERK signaling pathway and upr

关键词： apoptosis AAV2-PDE6B ERK1/2 gene therapy phototransduction proteomics rd10 retinitis pigmentosa

Distinct MLT asymmetry of auroral kilometric radiation observed by the FAST satellite

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Earth and Planetary Physics 2025年第1期9卷 188-193页

作者： JiaWen Tang Sai Zhang FuLiang Xiao HongMing yang Si liu YiHua He Chang yang YuYue Jin ZhouKun Deng Ping Li Archie James Johnston School of Physics and Electronic Sciences Changsha University of Science and TechnologyChangsha 410114China Hunan Province Higher Education Key Laboratory of Modeling and Monitoring on the Near-Earth Electromagnetic Environments Changsha University of Science and TechnologyChangsha 410114China School of Electrical and Information Engineering Changsha University of Science and TechnologyChangsha 410114China

Auroral Kilometric Radiation (AKR) is a common radio emission,which can contribute to the magnetosphere-ionosphereatmosphere co u *** emissions have been observed in all magnetic planet magnetospheres of the solar *** this study,using observations from the FAST satellite from 30 August 1996 to 9 September 2001,the distribution of AKR in altitude=500-4500 km and invariant latitude (|ILAT|)=60°-80°has been analyzed.63045 AKR samples have been identified with~48%(52%) samples on the dayside (nightside).Of considerable interest,there is a distinct MLT asymmetry with the high occurrence rate in MLT=05-08 and 18-22(02-05 and 12-17) in the northern (southern) *** distinct MLT asymmetry is associated with the direction of Bxof the interplaneta ry magnetic *** addition,the occurrence rate on the nightside clearly increases as the AE^(*) index *** study further enriches the information and understanding of AKR in the magnetosphere as well as other similar radio emissions.

关键词： asymmetry auroral kilometric radiation interplanetary magnetic field