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Independent temporal lobe epilepsy in patients with tuberous sclerosis complex

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Pediatric Investigation 2022年第1期6卷 23-28页

作者： tinghong liu Jing Ding Shaohui Zhang Yangshuo Wang Jinshan Xu liu Yuan Feng Zhai Guojun Zhang Shuli Liang Department of Functional Neurosurgery Key Laboratory of Major Disease in ChildrenMinistry of EducationBeijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Department of Neurology Zhongshan HospitalFudan UniversityShanghaiChina Department of Neurosurgery Fourth Medical CenterPLA General HospitalBeijingChina

Tuberous sclerosis complex(TSC)is a rare disease that involves multiple organs,including the brain;approximately 80%-90%of TSC patients exhibit TSC-associated *** temporal lobe epilepsy(TLE),TSC-unrelated epilepsy,is particularly rare in patients with ***,we describe three patients with TSC with independent TLEs that were confirmed by stereo-electroencephalography(EEG),postoperative pathological findings,and seizure outcome at *** patients were retrospectively enrolled at two centers;their ictal epileptiform discharge onsets were determined using electrode contacts in the hippocampus during *** three patients underwent anterior temporal lobectomies and remained seizure-free at 1-5 years after *** pathological examinations confirmed hippocampal sclerosis in all three ***,postoperative intelligence quotient improvement was evident in one patient,while the quality of life was improved in two patients at 12 months after surgery.

关键词： Anterior temporal lobectomy Hippocampal sclerosis Temporal lobe epilepsy Tuberous sclerosis complex

A nonenzymatic electrochemical sensor for the detection of hydrogen peroxide in vitro and in vivo fibrosis models

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Chinese Chemical Letters 2024年第3期35卷 394-398页

作者： Hongyao liu Yan Yu Taixiong Xue Cailing Gan Yuting Xie Doudou Wang Peilin Li Zhiyong Qian tinghong Ye Department of Gastroenterology and Hepatology Sichuan University-University of Oxford Huaxi Joint Centre for Gastrointestinal Cancer and Frontiers Science Center for Disease-Related Molecular NetworkState Key Laboratory of BiotherapyWest China HospitalSichuan UniversityChengdu 610041China Department of Biotherapy Cancer Center and State Key Laboratory of BiotherapyWest China HospitalSichuan UniversityChengdu 610041China

Fibrosis occurs due to the excessive deposition of extracellular matrix caused by cell *** various types of tissue injury,the dysregulation of the internal response can eventually lead to the destruction of organ structure and *** is increasing evidence that oxidative stress,which is characterized by excessive production of hydrogen peroxide(H_(2)O_(2)),is an important cause of ***,we synthesized a biosensitive and efficient electrochemical H_(2)O_(2)sensor based on PtNi nanoparticle-doped N-reduced graphene oxide(PtNi-N-rGO)to detect H_(2)O_(2)released from transforming growth factorβ1(TGFβ1)-induced *** addition,the sensor could easily detect changes in H_(2)O_(2)in the lung and bronchoalveolar lavage fluid(BALF)of mice with pulmonary ***,the sensor could also detect H_(2)O_(2)in activated hepatic stellate cells and the liver of carbon tetrachloride(CCl_(4))-induced liver ***,the alterations in H_(2)O_(2)detected by the sensor were consistent with nicotinamide adenine dinucleotide phosphate oxidase 4(NOX4)protein expression and the staining results of pathological *** together,these results highlight the use of H_(2)O_(2)sensors for the rapid detection of fibrosis and facilitate the rapid evaluation of antifibrotic drug candidates.

关键词： Fibrosis H_(2)O_(2) Electrochemical sensor NOX4 Nintedanib

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高频振荡脑电图在癫痫外科中的应用

中华神经医学杂志 2020年第7期19卷 649-653页

作者：梁树立刘婷红国家儿童医学中心首都医科大学附属北京儿童医院功能神经外科100045

高频脑电图是相对于常规脑电图(EEG)而言,指80 Hz以上的脑电信号。1989年首先在大鼠癫痫模型上记录到高频放电,1992年Fisher等首次在癫痫患者的皮质记录到高频电活动,此后应用微电极在动物癫痫模型及癫痫患者的颅内记录到高频放电,并命... 详细信息

高频脑电图是相对于常规脑电图(EEG)而言,指80 Hz以上的脑电信号。1989年首先在大鼠癫痫模型上记录到高频放电,1992年Fisher等首次在癫痫患者的皮质记录到高频电活动,此后应用微电极在动物癫痫模型及癫痫患者的颅内记录到高频放电,并命名为高频振荡(high frequency oscillations,HFOs)[1-2]。

关键词：脑电图高频振荡癫痫

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迷走神经刺激治疗Dravet综合征一例

罕见病研究 2022年第3期1卷 318-324页

作者：刘婷红袁柳徐金山王旸烁梁树立国家儿童医学中心、首都医科大学附属北京儿童医院功能神经外科北京100045

Dravet综合征是一种儿童期起病的癫痫综合征,表现为药物难治性癫痫,并常出现癫痫持续状态,随病程进展出现认知和语言损害。抗癫痫发作药物治疗和切除性癫痫手术治疗对Dravet综合征治疗效果差,神经调控手术,特别是迷走神经刺激是有效、... 详细信息

Dravet综合征是一种儿童期起病的癫痫综合征,表现为药物难治性癫痫,并常出现癫痫持续状态,随病程进展出现认知和语言损害。抗癫痫发作药物治疗和切除性癫痫手术治疗对Dravet综合征治疗效果差,神经调控手术,特别是迷走神经刺激是有效、可行的治疗方案。现报道迷走神经刺激治疗Dravet综合征病例一例,一年随访癫痫发作减少大于50%。同时,进行了相关文献复习和总结,共收集已报道的迷走神经刺激术治疗Dravet病例141例,总体癫痫控制有效率为53􀆰9%。

关键词： Dravet综合征肌阵挛发作癫痫发育性癫痫性脑病迷走神经刺激

KMT2B基因变异致儿童肌张力障碍28型11例临床与遗传学分析

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中华实用儿科临床杂志 2022年第15期37卷 1146-1150页

作者：代丽芳丁昌红方铁谢自行刘婷红张炜华王晓慧任晓暾刘明田小娟吴沪生方方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功能神经外科北京100045

目的总结KMT2B基因变异致肌张力障碍28型(DYT28)患儿的临床资料,以提高临床医师对该病的认识。方法回顾性总结分析2018年3月至2021年1月首都医科大学附属北京儿童医院神经内科确诊的11例KMT2B基因变异致DYT28患儿的临床表现、治疗及基... 详细信息

目的总结KMT2B基因变异致肌张力障碍28型(DYT28)患儿的临床资料,以提高临床医师对该病的认识。方法回顾性总结分析2018年3月至2021年1月首都医科大学附属北京儿童医院神经内科确诊的11例KMT2B基因变异致DYT28患儿的临床表现、治疗及基因变异谱,并随访。结果 11例患儿中,男8例,女3例;起病年龄1个月~6岁。11例患儿均隐匿起病,表现为肌张力障碍症状,8例为全身型,3例为多灶型;7例以单/双侧下肢足尖行走起病,4例以构音障碍、饮水呛咳或干呕起病。随病情进展,11例均有咽喉肌受累,10例双下肢受累,8例双上肢受累。6例合并其他运动障碍症状。10例伴不同程度身材矮小、小头畸形、小下颌、骨骼畸形、智力障碍、内分泌异常和睡眠障碍。仅1例头颅磁共振成像异常。共发现KMT2B基因变异11个(移码变异8个、整码变异1个、错义变异2个),其中4个为未见报道的新变异。对全部患儿进行随访,随访年龄1岁7个月~17岁9个月,1例未治疗,3例药物治疗运动症状轻度改善,1例药物治疗尿便障碍症状消失,6例药物治疗无效,其中5例行脑深部电刺激术治疗,肌张力障碍症状均有明显改善,2例尿便障碍患儿术后尿便控制良好。患儿Burke-Fahn-Marsden肌张力障碍运动评分量表运动评分改善率为55.8%~90.7%,功能评分改善率为14.8%~69.6%。结论 KMT2B基因变异致DYT28是儿童早发遗传性肌张力障碍的常见类型,肌张力障碍症状由局灶型逐渐进展至全身型,以下肢和咽喉肌受累为著,需关注尿、便障碍症状。肌张力障碍症状轻微患儿药物治疗有效,症状严重者药物治疗疗效不佳,需予脑深部电刺激术治疗,可显著改善肌张力障碍症状。

关键词：肌张力障碍28型 KMT2B基因基因变异脑深部电刺激

过渡金属掺杂的g-GaN吸附Cl_(2)和CO气体分子的第一性原理研究

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无机化学学报 2024年第6期40卷 1189-1200页

作者：田双林高廷红刘玉涛陈茜谢泉肖清泉梁永超贵州大学大数据与信息工程学院新型光电子材料与技术研究所贵阳550025

基于密度泛函理论的第一性原理计算,系统研究了类石墨烯氮化镓(g-GaN)和掺杂过渡金属原子(TM)的g-GaN对Cl_(2)和CO气体分子的吸附行为。结果表明,Cl_(2)和CO在本征g-GaN上的吸附均为物理吸附,2个体系的吸附能均为正值,表明体系不稳定。... 详细信息

基于密度泛函理论的第一性原理计算,系统研究了类石墨烯氮化镓(g-GaN)和掺杂过渡金属原子(TM)的g-GaN对Cl_(2)和CO气体分子的吸附行为。结果表明,Cl_(2)和CO在本征g-GaN上的吸附均为物理吸附,2个体系的吸附能均为正值,表明体系不稳定。相反,Cl_(2)和CO在Fe和Co掺杂的g-GaN上吸附时的吸附能为负值,且吸附能较小,表明吸附体系稳定。通过分析态密度、电荷密度差和能带结构等性质,可以得出结论:过渡金属原子的引入能有效增强气体分子与g-GaN之间的相互作用。

关键词：气体传感类石墨烯GaN 吸附过渡金属掺杂

Elevation gradient distribution of indices of tree population in a montane forest:The role of leaf traits and the environment

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Forest Ecosystems 2022年第1期9卷 124-130页

作者： Zuhua Wang Rong Zheng Lilin Yang tinghong Tan Haibo Li Min liu College of A&F Engineering and Planning Tongren UniversityTongren 554300GuizhouChina Guizhou Provincial Key Laboratory for Biodiversity Conservation and Utilization in the Fanjing Mountain Region Tongren UniversityTongren 554300GuizhouChina College of Life and Technology Inner Mongolia Normal UniversityHohhot 010022Inner MongoliaChina National Nature Reserve Administration of Fanjing Mountain Tongren 554400GuizhouChina

Background:To disentangle the controls on species distribution in the context of climate change is a central element in proposed strategies to maintain species ***,previous studies have focused mainly on the roles of abiotic factors(e.g.,climate and soil properties),with much less attention given to the roles of biotic factors such as functional ***,we measured eight leaf traits for 240 individual trees of 53 species and analyzed the variation in traits and population composition indices and their relationships with soil properties,climate factors,and leaf ***:The tree density,frequency and species importance values of the overall species and saplings significantly increased with increasing elevation,while the same indices(except for species frequency)of adults did not significantly *** largest percentage of variation of species importance value(greater than 50%)was explained by climate,but leaf traits played a critical role in driving elevation distribution patterns of both saplings and adults;the abundance of saplings significantly increased with elevation,with increased leaf carbon contents,while the abundance of adults did not change in accordance with a nutrient conservation strategy associated with the leaf economic ***:Our results suggest that the elevation gradient distribution of woody plant species is dependent on tree size and that local atmospheric humidity and leaf traits cause considerable variation in species distribution along subtropical mountain *** provide evidence of which leaf traits play a key role in the elevation gradient distribution of different sizes of woody tree species.

关键词： Elevation gradient Species distribution Leaf traits Climate change Leaf economic spectrum Mt.Fanjingshan

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广东野生猪笼草的群落学与土壤养分特征

热带林业 2023年第3期51卷 9-15页

作者：叶冬梅余恩萍龚军凡刘庭宏邱志敏曹洪麟王峥峰中山市自然保护地管护中心广东中山528400 中国科学院华南植物园广东广州510650 中国科学院大学北京101400

对广东省内野生猪笼草集中分布的4个地方进行了群落调查及土壤养分分析。结果表明,广东省内野生猪笼草群落热带性质明显,主要分布在相对湿度较高的向阳山坡上,属沼生植被类型,其种群数量较少,群落多样性较低;生长环境的土壤偏酸性,有机... 详细信息

对广东省内野生猪笼草集中分布的4个地方进行了群落调查及土壤养分分析。结果表明,广东省内野生猪笼草群落热带性质明显,主要分布在相对湿度较高的向阳山坡上,属沼生植被类型,其种群数量较少,群落多样性较低;生长环境的土壤偏酸性,有机质、氮、钾含量都较高,全磷含量较低,沼生性质明显。分析猪笼草的群落学与土壤养分特征,可为其保护和引种栽培提供重要的科学依据。

关键词：猪笼草群落学特征土壤养分特征

GYPA、CD68和TNF-α蛋白鉴别水中晚期尸体生前死后伤的作用研究

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中国法医学杂志 2024年第2期39卷 196-202页

作者：向明亮王廷宏刘勤进王陶罗涛张伟章丽霞邓小冬刘云川北医学院基础医学与法医学院法医病理教研室四川南充637000 川北医学院医学影像四川省重点实验室四川南充637000

目的探讨血型糖蛋白A(Glycophorin A,GYPA)、CD68和肿瘤坏死因子-α(Tumor necrosis factor-α,TNF-α)蛋白在水中晚期尸体生前伤和死后伤的表达情况,为在水中晚期生前死后伤鉴定筛选标志物。方法18只SD大鼠分为对照组(n=6)、生前挫伤... 详细信息

目的探讨血型糖蛋白A(Glycophorin A,GYPA)、CD68和肿瘤坏死因子-α(Tumor necrosis factor-α,TNF-α)蛋白在水中晚期尸体生前伤和死后伤的表达情况,为在水中晚期生前死后伤鉴定筛选标志物。方法18只SD大鼠分为对照组(n=6)、生前挫伤浸没组(n=6)和死后挫伤浸没组(n=6),生前挫伤浸没组采用机械失重技术建立SD大鼠右后肢挫伤模型,死后挫伤浸没组是SD大鼠处死后用机械失重技术建立右后肢挫伤模型,对照组不予致伤处理,所有大鼠均浸没72 h。肉眼观察及HE染色观察生前伤和死后伤的情况;免疫组织化学染色(Immunohistochemistry,IHC)和蛋白质免疫印迹(Western blot)检测GYPA、CD68及TNF-α蛋白在挫伤组织表达情况;随后在实际案例中予以验证。结果肉眼观察和HE染色均无法较好地鉴别浸没72 h大鼠的生前伤和死后伤。免疫组织化学染色发现生前挫伤浸没组软组织GYPA表达呈阳性,CD68在炎性细胞膜呈阳性表达,TNF-α在胞质表达呈弱阳性,GYPA、CD68和TNF-α蛋白表达均显著高于死后挫伤浸没组和对照组(P<0.05)。GYPA、CD68和TNF-α蛋白在鉴别浸没尸体生前伤和死后伤实际案例中亦有相似结果。结论GYPA、CD68和TNF-α蛋白有望成为水中浸没尸体生前伤和死后伤鉴别的分子指标之一。

关键词：法医病理学生前伤死后伤血型糖蛋白A 肿瘤坏死因子-α CD68