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Pediatric Investigation 2024年第2期8卷 154-156页

作者： xiaojuan tian Changhong Ding Ming Liu Lifang Dai Zixing Xie Tie Fang Department of Neurology Neurological CenterBeijing Children’s HospitalCapital Medical UniversityNational Center For Children’s HealthBeijingChina Department of Functional Neurosurgery Neurological CenterBeijing Children’s HospitalCapital Medical UniversityNational Center For Children’s HealthBeijingChina

To the editor:Myoclonus-dystonia syndrome(MDS)(OMIM15990)is a heritable disorder characterized by early onset subcortical myoclonic jerks and/or less prominent *** leads to disability and is often refractory to medical treatment.1 Deep brain stimulation(DBS)for the treatment of refractory MDS has been proven effective in adults for reducing both myoclonic jerks and dystonia.1,2,3 Here,we describe the application of bilateral globus pallidus internus(GPi)DBS in a 5-year-old boy diagnosed with MDS.

关键词： globus editor Deep

甘肃兴隆山不同演替阶段群落土壤氮素矿化对温度的响应

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干旱区研究 2024年第6期41卷 984-997页

作者：唐维春刘小娥苏世平田晓娟唐庆童张婧甘肃农业大学林学院甘肃兰州730070 兴隆山国家级自然保护区管护中心甘肃兰州730100 甘肃农业大学理学院甘肃兰州730070

土壤氮素矿化是土壤中氮素循环的关键过程,而温度是影响土壤氮素矿化的最重要的因素之一,研究温度变化对不同演替阶段群落土壤氮素矿化特征的影响,对于明确陆地生态系统中土壤氮素循环过程具有重要意义。本研究以甘肃兴隆山不同演替阶... 详细信息

土壤氮素矿化是土壤中氮素循环的关键过程,而温度是影响土壤氮素矿化的最重要的因素之一,研究温度变化对不同演替阶段群落土壤氮素矿化特征的影响,对于明确陆地生态系统中土壤氮素循环过程具有重要意义。本研究以甘肃兴隆山不同演替阶段群落为研究对象,采用室内恒温好气培养法,研究不同演替阶段群落[草地、灌丛林、白桦林(Betula platyphylla forest)、青杄-白桦林(Picea wilsonii-Betula platyphylla forest)和青杄林(Picea wilsonii forest)]在不同温度(15℃、25℃和35℃)下的土壤氮素矿化特征。结果表明:(1)除草地的0~20 cm土层外,其余演替阶段群落土壤氮素矿化速率随着温度(15~35℃)的增加而增大,且不同演替阶段群落土壤氮素累积矿化量随着温度的增加而增加;(2)随着演替的正向推进,不同演替阶段群落土壤氮素矿化速率与累积矿化量均呈先升高后降低趋势,白桦林的土壤氮素矿化速率最大,分别均是草地、灌丛林、青杄-白桦林、青杄林的1.63倍、1.61倍、1.25倍、1.47倍;而青杄-白桦林的累积矿化量最高,分别是草地、灌丛林、白桦林、青杄林的0.68倍、0.72倍、0.84倍、0.97倍;(3)随着土壤深度的增加,土壤氮素矿化速率与累积矿化量均呈降低趋势,以0~20 cm土层的最大;(4)不同演替阶段群落在15℃培养和25℃培养下的温度敏感系数Q10均有显著差异(P0.05)。研究结果将为群落土壤的质量演变和土壤供氮能力的动态变化提供理论依据。

关键词：土壤氮素矿化温度演替阶段兴隆山甘肃

Surface contact behavior of functionally graded thermoelectric materials indented by a conducting punch

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Applied Mathematics and Mechanics(English Edition) 2021年第5期42卷 649-664页

作者： xiaojuan tian Yueting ZHOU Lihua WANG Shenghu DING School of Aerospace Engineering and Applied Mechanics Tongji UniversityShanghai 200092China School of Mathematics and Statistics Ningxia UniversityYinchuan 750021China

The contact problem for thermoelectric materials with functionally graded properties is *** material properties,such as the electric conductivity,the thermal conductivity,the shear modulus,and the thermal expansion coefficient,vary in an exponential *** the Fourier transform technique,the electro-thermoelastic problems are transformed into three sets of singular integral equations which are solved numerically in terms of the unknown normal electric current density,the normal energy flux,and the contact ***,the complex homogeneous solutions of the displacement fields caused by the gradient parameters are simplified with the help of Euler’s *** addressing the non-linearity excited by thermoelectric effects,the particular solutions of the displacement fields can be *** effects of various combinations of material gradient parameters and thermoelectric loads on the contact behaviors of thermoelectric materials are *** results give a deep insight into the contact damage mechanism of functionally graded thermoelectric materials(FGTEMs).

关键词： thermoelectric material functionally graded property conducting punch conjugate complex root energy flux contact pressure

Cryo-electron microscopy reconstructions of two types of wild rabbit hemorrhagic disease viruses characterized the structural features of Lagovirus

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Protein & Cell 2010年第1期1卷 48-58页

作者： Zhongjun Hu xiaojuan tian Yujia Zhai Wei Xu Dong Zheng Fei Sun National Laboratory of Biomacromolecules Institute of BiophysicsChinese Academy of SciencesBeijing 100101China Department of Biochemistry and Molecular Biology College of Life SciencesBeijing Normal UniversityBeijing 100875China

Rabbit hemorrhagic disease was described in China in 1984 and can cause hemorrhagic necrosis of the liver within two or three days after *** etiological agent,rabbit hemorrhagic disease virus(RHDV),belongs to the Lagovirus genus in the Caliciviridae *** to other calicivirus,such as rNV and SMSV,the structure of Lagovirus members is not well *** this report,structures of two types of wild RHDV particles,the intact virion and the core-like particle(CLP),were reconstructed by cryo-electron microscopy at 11Åand 17Å,*** is the first time the 3D structure of wild caliciviruses CLP has been provided,and the 3D structure of intact RHDV virion is the highest resolution structure in *** of the intact virion and CLP structures clearly indicated that CLP was produced from the intact virion with the protrusion *** contrast with the crystal structures of recombinant Norovirus and San Miguel sea lion virus,the capsomers of RHDV virion exhibited unique structural features and assembly *** P1 and P2 subdomains have interactions inside the AB capsomer,while only P2 subdomains have interaction inside CC *** pseudo atomic models of RHDV capsomers were constructed by homology modeling and density map fitting,and the rotation of RHDV VP60 P domain with respect to its S domain,compared with SMSV,was ***,our cryo-electron microscopic studies of RHDV provide close insight into the structure of Lagovirus,which is important for functional analysis and better vaccine development in the future.

关键词： rabbit hemorrhagic disease virus calicivirus Lagovirus core-like particle cryo-electron microscopy reconstruction

儿童眼球阵挛-肌阵挛-共济失调综合征临床及预后分析

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中华儿科杂志 2024年第3期62卷 256-261页

作者：周季卓秀伟金眉段超张炜华任长红巩帅田小娟丁昌红任晓暾李久伟国家儿童医学中心、首都医科大学附属北京儿童医院神经内科北京100045 国家儿童医学中心首都医科大学附属北京儿童医院肿瘤内科北京100045

目的总结儿童眼球阵挛-肌阵挛-共济失调综合征(OMAS)患儿的临床及预后特征。方法对2015年6月至2023年6月于北京儿童医院神经内科住院的46例OMAS患儿进行回顾性病例分析。并在2023年6至8月对患儿进行集中的互联网诊疗或电话访视。收集患... 详细信息

目的总结儿童眼球阵挛-肌阵挛-共济失调综合征(OMAS)患儿的临床及预后特征。方法对2015年6月至2023年6月于北京儿童医院神经内科住院的46例OMAS患儿进行回顾性病例分析。并在2023年6至8月对患儿进行集中的互联网诊疗或电话访视。收集患儿住院和门诊随访期间的病例资料,包括临床表现、辅助检查、治疗及预后情况等。根据患儿是否合并肿瘤分为两组,组间比较采用χ^(2)检验或Mann-Whitney U检验。使用单因素Logistic回归分析OMAS复发和预后相关因素。结果46例患儿中男25例,起病年龄1.5(1.2,2.4)岁。26例(57%)患儿病程中确诊神经母细胞瘤,无患儿高危。36例(78%)患儿随访≥6个月,这36例患儿均使用糖皮质激素、丙种球蛋白和(或)促肾上腺皮质激素作为一线治疗,其中9例(25%)使用二线治疗≥3个月(包括利妥昔单抗和环磷酰胺),17例(47%)使用神经母细胞瘤相关的化疗;随访4.2(2.2,5.5)年,10例(28%)患儿出现OMAS复发。36例患儿末次随访时Mitchell and Pike OMS评分量表评分为0.5(0,2.0)分,7例(19%)认知轻度落后于同龄儿,6例(17%)重度落后。仅1例患儿在随访中出现肿瘤复发。未合并肿瘤组起病前疫苗接种或感染病史较合并肿瘤组更多见[55%(11/20)比23%(6/26),χ^(2)=4.95,P=0.026],首发症状肌阵挛出现更频繁[40%(8/20)比4%(1/26),χ^(2)=7.23,P=0.007]。Logistic单因素回归分析发现,合并肿瘤组比未合并肿瘤组更少出现复发[OR=0.19(0.04~0.93),P=0.041],病程6个月内使用二线治疗或化疗预后更好[OR=11.64(1.27~106.72),P=0.030]。结论儿童OMAS多于幼儿期起病,约半数合并神经母细胞瘤。合并OMAS的神经母细胞瘤通常危险分级低,预后好。对比合并和未合并肿瘤的OMAS患儿,后者起病前感染或接种疫苗诱因更常见,首发症状肌阵挛更多见。早期加用二线治疗与OMAS更好的预后相关。

关键词：神经母细胞瘤预后眼球阵挛-肌阵挛综合征

胆红素总量/白蛋白比值、听力筛查及MRI-T_(1)WI影像对新生儿急性胆红素脑病的诊断价值

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中外医学研究 2024年第4期22卷 143-148页

作者：王粉王燕顾圆王倩倩陈志云田小娟太仓市第一人民医院江苏太仓215400

目的:探究胆红素总量/白蛋白比值(B/A)、听力筛查及磁共振成像-T_(1)加权成像(MRI-T_(1)WI)对新生儿急性胆红素脑病(ABE)的诊断价值。方法:选取2013年6月—2023年6月于太仓市第一人民医院接受治疗的136例新生儿高胆红素血症(NHB)患儿作... 详细信息

目的:探究胆红素总量/白蛋白比值(B/A)、听力筛查及磁共振成像-T_(1)加权成像(MRI-T_(1)WI)对新生儿急性胆红素脑病(ABE)的诊断价值。方法:选取2013年6月—2023年6月于太仓市第一人民医院接受治疗的136例新生儿高胆红素血症(NHB)患儿作为研究对象,根据是否并发ABE分为并发组(n=42)和对照组(n=94)。对比两组的临床资料、B/A水平、听力筛查结果、苍白球T_(1)WI信号强度,对单因素分析有意义的指标进行多因素logistic回归分析,采用ROC曲线分析其对新生儿ABE的诊断价值。结果:并发组总血清胆红素(TSB)峰值、B/A水平均高于对照组,差异有统计学意义(P<0.05)。并发组初筛听力异常率为38.10%,复筛听力异常率为35.71%,均高于对照组的18.09%、8.51%,差异有统计学意义(P<0.05)。并发组苍白球与额叶脑皮质T_(1)WI信号强度比值(G/Gr)水平高于对照组,差异有统计学意义(P<0.05)。logistic回归分析显示,B/A、听力异常和G/Gr水平表达均为影响NHB患儿并发ABE的独立危险因素,差异有统计学意义(P<0.05)。ROC曲线分析显示,B/A、G/Gr、听力异常及三者联合检测NHB患儿并发ABE的AUC值分别为0.773、0.652、0.754、0.863,有较高诊断价值,且联合诊断价值最高(P<0.05)。结论:ABE患儿中B/A、听力异常和G/Gr表达水平明显升高,三者单独和联合检测NHB患儿并发ABE均有较高诊断价值,其中三者联合诊断效果最佳。

关键词：急性胆红素脑病新生儿胆红素总量/白蛋白比值听力筛查磁共振成像-T_(1)加权成像

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TRIO基因相关儿童智力发育障碍的临床特点和基因分析

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中华儿科杂志 2024年第11期62卷 1071-1075页

作者：田小娟王晓慧任晓暾贾天明张广宇国家儿童医学中心首都医科大学附属北京儿童医院神经内科北京郑州大学第三附属医院儿科郑州

目的总结TRIO基因相关儿童智力发育障碍(MRD)的临床表现和基因特点。方法病例系列研究, 回顾性收集首都医科大学附属北京儿童医院神经内科及郑州大学第三附属医院儿科2019年8月至2024年3月就诊的9例TRIO基因相关MRD患儿病例资料, 包括... 详细信息

目的总结TRIO基因相关儿童智力发育障碍(MRD)的临床表现和基因特点。方法病例系列研究, 回顾性收集首都医科大学附属北京儿童医院神经内科及郑州大学第三附属医院儿科2019年8月至2024年3月就诊的9例TRIO基因相关MRD患儿病例资料, 包括性别、年龄、智力及运动发育、外观、癫痫发作、头颅影像学和基因结果, 总结其临床特点及基因型表型特点。结果 9例患儿中男6例、女3例, 4例患儿符合MRD63型表型, 表现为中重度发育落后伴大头畸形;5例患儿符合MRD44型表型, 表现为轻中度发育落后伴小头畸形。伴有明显特殊面容(枕部扁平、眉毛粗重、连眉、耳朵大、手指短小、肤白发黄和斜视等)5例, 伴有癫痫发作2例(肌阵挛发作和失神发作各1例), 伴有喂养困难4例, 伴有先天性白内障、先天性心脏病、反复感染、眼底改变(漆裂纹样改变)各1例。9例患儿携带的TRIO基因变异均为新生变异, 包括8个变异位点, 其中错义变异7个、移码变异1个, 分别为c.3232C>T/p.R1078W(2例)、c.3920A>G/p.Y1307C、c.4112A>T/p.H1371L、c.4283G>T/p.R1428L、c.4394A>G/p.N1465S、c.6041T>C/p.I2014T、c.6821G>A/p.R2274H、c.7027delC/p.Q2343Sfs*70。2例变异位点位于Spectrin结构域, 4例位于鸟嘌呤核苷酸交换因子(GEF)D1结构域, 2例位于GEFD2结构域, 1例(移码变异)位于PH2-SH3结构域。移码变异者表现为失神发作和轻度发育落后, 表型最轻。肌阵挛发作癫痫患儿加用丙戊酸联合左乙拉西坦发作控制, 失神癫痫患儿加用丙戊酸联合拉莫三嗪发作控制。9例患儿均进行积极康复训练, 发育缓慢进步。结论 TRIO基因相关儿童MRD表现为不同程度的发育落后, 多伴有大头或小头畸形, 特殊面容, 伴或不伴癫痫发作;变异类型以错义变异为主, 错义变异大多集中于Spectrin结构域和GEFD结构域, p.R1078W可能为相对热点变异, 移码变异者表型相对较轻。

关键词：发育障碍小头畸形大头畸形面容畸形基因, TRIO

Structure Regulation of Electric Double Layer via Hydrogen Bonding Effect to Realize High-Stability Lithium-Metal Batteries

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Energy & Environmental Materials 2024年第3期7卷 72-83页

作者： Sheng Liu Chaozhu Shu Yu Yan Dayue Du Longfei Ren Ting Zeng xiaojuan Wen Haoyang Xu Xinxiang Wang Guilei tian Ying Zeng College of Materials and Chemistry&Chemical Engineering Chengdu University of Technology1#DongsanluErxianqiaoChengdu 610059China Institute of Fundamental and Frontier Sciences University of Electronic Science and Technology of ChinaChengdu 611731China State Key Laboratory of Polymer Materials Engineering Polymer Research InstituteSichuan UniversityChengdu 610065China

The interfacial chemistry of solid electrolyte interphases(SEI)on lithium(Li)electrode is directly determined by the structural chemistry of the electric double layer(EDL)at the ***,a strategy for regulating the structural chemistry of EDL via the introduction of intermolecular hydrogen bonds has been proposed(p-hydroxybenzoic acid(pHA)is selected as proof-of-concept).According to the molecular dynamics(MD)simulation and density functional theory(DFT)calculation results,the existence of hydrogen bonds realizes the anion structural rearrangement in the EDL,reduces the lowest unoccupied molecular orbital(LUMO)energy level of anions in the EDL,and the number of free solvent molecules,which promotes the formation of inorganic species-enriched SEI and eventually achieves the dendrite-free Li *** on this strategy,Li‖Cu cells can stably run over 185 cycles with an accumulated active Li loss of only 2.27 mAh cm^(-2),and the long-term cycle stability of Li‖Li cells is increased to 1200 *** addition,the full cell pairing with the commercial LiFePO_(4)(LFP)cathodes exhibits stable cycling performance at 1C,with a capacity retention close to 90%after 200 cycles.

关键词： electric double layer electrolyte additives intermolecular hydrogen bonds Li metal batteries p-Hydroxybenzoic acid

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论商周时期的玉石容器

中国国家博物馆馆刊 2015年第7期 71-83页

作者：田小娟陕西历史博物馆西安710061

商周玉石容器在大型墓葬偶有发现,与同时期的容器在形制、纹饰、尺寸大小上极为相近。本文就玉石器皿的器型、纹饰、年代等问题,结合墓葬出土的青铜器、陶器等进行类比,认为玉石容器与青铜礼器的相似性主要表现在簋、盘、豆、觯这几种... 详细信息

商周玉石容器在大型墓葬偶有发现,与同时期的容器在形制、纹饰、尺寸大小上极为相近。本文就玉石器皿的器型、纹饰、年代等问题,结合墓葬出土的青铜器、陶器等进行类比,认为玉石容器与青铜礼器的相似性主要表现在簋、盘、豆、觯这几种器型上,纹饰方面也尽量模仿青铜器但较为简单,但也有器物仿陶器。商代的玉石器皿以实用器为主,主要出土于殷墟,西周则主要发现于周原地区,均为把玩的小器皿,而非实用器。

关键词：商周玉石容器造型纹饰

Dip2a regulates stress susceptibility in the basolateral amygdala

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Neural Regeneration Research 2025年第6期20卷 1735-1748页

作者： Jing Li Zixuan He Weitai Chai Meng tian Huali Yu Xiaoxiao He xiaojuan Zhu Key Laboratory of Molecular Epigenetics Ministry of Education Institute of Genetics and CytologyNortheast Normal UniversityChangchunJilin ProvinceChina

Dysregulation of neurotransmitter metabolism in the central nervous system contributes to mood disorders such as depression, anxiety, and post–traumatic stress disorder. Monoamines and amino acids are important types of neurotransmitters. Our previous results have shown that disco-interacting protein 2 homolog A(Dip2a) knockout mice exhibit brain development disorders and abnormal amino acid metabolism in serum. This suggests that DIP2A is involved in the metabolism of amino acid–associated neurotransmitters. Therefore, we performed targeted neurotransmitter metabolomics analysis and found that Dip2a deficiency caused abnormal metabolism of tryptophan and thyroxine in the basolateral amygdala and medial prefrontal cortex. In addition, acute restraint stress induced a decrease in 5-hydroxytryptamine in the basolateral amygdala. Additionally, Dip2a was abundantly expressed in excitatory neurons of the basolateral amygdala, and deletion of Dip2a in these neurons resulted in hopelessness-like behavior in the tail suspension test. Altogether, these findings demonstrate that DIP2A in the basolateral amygdala may be involved in the regulation of stress susceptibility. This provides critical evidence implicating a role of DIP2A in affective disorders.

关键词： 5-hydroxytryptamine acute restraint stress basolateral amygdala CaMKII neurons DIP2A metabolomics neurotransmitters principal component analysis stress susceptibility tryptophan