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Effects of Normalized Difference Vegetation Index and Related Wavebands′ Characteristics on Detecting Spatial Heterogeneity Using Variogram-based Analysis

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Chinese Geographical Science 2012年第2期22卷 188-195页

作者： WEN Zhaofei ZHANG Ce ZHANG Shuqing ding changhong LIU Chunyue PAN Xin LI Huapeng SUN Yan Northeast Institute of Geography and Agroecology Chinese Academy of Sciences Changchun 130012 China Graduate University of Chinese Academy of Sciences Beij＇ing 100049 China Laboratory of Geographical Resources and Environmental Remote Sensing College of Geographical Sciences Harbin Normal University Harbin 150025 China Electrical and Electronic Teaching and Research Office Aviation University of Air Force Changehun 130022 China

Spatial heterogeneity is widely used in diverse applications, such as recognizing ecological process, guiding ecological restoration, managing land use, etc. Many researches have focused on the inherent scale multiplicity of spatial heterogeneity by using various environmental variables. How these variables affect their corresponding spatial heterogeneities, however, have received little attention. In this paper, we examined the effects of characteristics of normalized difference vegetation index （NDVI） and its related bands variable images, namely red and near infrared （NIR）, on their corresponding spatial heterogeneity detection based on variogram models. In a coastal wetland region, two groups of study sites with distinct fractal vegetation cover were tested and analyzed. The results show that： l） in high fractal vegetation cover （H-FVC） area, NDV! and NIR variables display a similar ability in detecting the spatial he- terogeneity caused by vegetation growing status structure; 2） in low fractal vegetation cover （L-FVC） area, the NIR and red variables outperform NDVI in the survey of soil spatial heterogeneity; and 3） generally, NIR variable is ubiquitously applicable for vegetation spatial heterogeneity investigation in different fractal vegetation covers. Moreover, as variable selection for remote sensing applications should fully take the characteristics of variables and the study object into account, the proposed variogram analysis method can make the variable selection objectively and scientifically, especially in studies related to spatial heterogeneity using remotely sensed data.

关键词： spatial variation spatial structure NDVI characteristic semivariogram model semivariogram analysis

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Neurophenotype and genetic analysis of children with Aicardi-Goutières syndrome in China

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儿科学研究(英文) 2024年第3期8卷 193-200页

作者： Zhang Shen Zhang Weihua ding changhong Ren Xiaotun Fang Fang Wu Yun Department of Neurology Beijing Children’s Hospital Capital Medical University National Center for Children’s Health Beijing China

Importance: Aicardi-Goutières syndrome (AGS) is a rare genetic disorder mainly affecting the central nervous system and autoimmunity. However, research on AGS among Chinese patients is ***: To summarize the neurologic phenotypes and genetic causes in pediatric AGS patients, providing insights for early recognition and diagnosis in the Chinese ***: Clinical features and neuroimaging results of the patients diagnosed with AGS from Beijing Children’s Hospital between January 2018 and January 2022 were collected. Whole exome sequencing was used for genetic ***: A total of 15 patients was included, all presenting with various neurological symptoms, including developmental delay (100%), motor skill impairment (100%), language disability (78.6%), dystonia (93.3%), microcephaly (73.3%), sleep disorders (26.7%), regression (20.0%), vessel disease (6.7%), and epilepsy (6.7%). Neuroimaging revealed intracranial calcification (86.7%), cerebral atrophy (73.3%), and leukodystrophy (73.3%). Seven genes were identified, withTREX1 being the most common (40.0%, 6/15), followed byIFIH1 (20.0%, 3/15). Variant c.294dupA (p.C99Mfs*3) was detected in four unrelated patients, accounting for 66.7% (4/6) patients with theTREX1 variant. A literature review showed thatTREX1 gene mutations in 35.6% (21/59) of AGS patients among the Chinese ***: Neurological symptoms are the most prevalent and severe presentation of AGS. Diagnosis may be considered when symptoms such as developmental delay, dystonia, microcephaly, brain calcification, and leukodystrophy ***1 mutations are predominant in the Chinese population.

关键词： Aicardi-Goutières syndrome Developmental delay Leukodystrophy Neurophenotype Whole exome sequencing

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The clinical and genetic characteristics in children with mitochondrial disease in China

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Science China(Life Sciences) 2017年第7期60卷 746-757页

作者： Fang Fang Zhimei Liu Hezhi Fang Jian Wu Danmin Shen Suzhen Sun changhong ding Tongli Han Yun Wu Junlan Lv Lei Yang Shufang Li Jianxin Lv Ying Shen Department of Neurology Beijing Children 's Hospital Capital Medical University National Center for Children's Health Beijing 100045 China Inspection Medical School Wenzhou Medical College Wenzhou 325035 China Translational Medical Center The General Hospital of the Chinese People's Liberation Army Beijing 100853 China Department of Neurology Children's Hospital of Hebei Province Hebei Medical University Shijiazhuang 050030 China

Mitochondrial disease was a clinically and genetically heterogeneous group of diseases, thus the diagnosis was very difficult to clinicians. Our objective was to analyze clinical and genetic characteristics of children with mitochondrial disease in China. We tested 141 candidate patients who have been suspected of mitochondrial disorders by using targeted next-generation sequencing(NGS), and summarized the clinical and genetic data of gene confirmed cases from Neurology Department, Beijing Children's Hospital, Capital Medical University from October 2012 to January 2015. In our study, 40 cases of gene confirmed mitochondrial disease including eight kinds of mitochondrial disease, among which Leigh syndrome was identified to be the most common type, followed by mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes(MELAS). The age-of-onset varies among mitochondrial disease, but early onset was common. All of 40 cases were gene confirmed, among which 25 cases(62.5%)with mitochondrial DNA(mtDNA) mutation, and 15 cases(37.5%) with nuclear DNA(nDNA) mutation. M.3243A>G(n=7)accounts for a large proportion of mtDNA mutation. The nDNA mutations include SURF1(n=7),PDHA1(n=2),and NDUFV1,NDUFAF6, SUCLA2, SUCLG1, RRM2 B, and C12orf65, respectively.

关键词： mitochondrial disease targeted next generation sequencing clinical features gene

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Bilateral globus pallidus internus-deep brain stimulation in a 5-year-old boy with SGCE-related myoclonus dystonia syndrome

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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

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Achieving ultralow leakage current in Schottky-MIS cascode anode lateral field-effect diode based on AlGaN/GaN HEMT

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Science China(Information Sciences) 2025年第1期68卷 379-385页

作者： Fangzhou WANG changhong GAO Guojian ding Cheng YU Zhuocheng WANG Xiaohui WANG Qi FENG Ping YU Peng ZUO Wanjun CHEN Yang WANG Haiqiang JIA Hong CHEN Bo ZHANG Zeheng WANG Songshan Lake Materials Laboratory School of Microelectronics Southern University of Science and Technology State Key Laboratory of Electronic Thin Films and Integrated Devices University of Electronic Science and Technology of China Manufacturing Commonwealth Scientific and Industrial Research Organisation

In this paper,we design and fabricate a Schottky-metal-insulator-semiconductor（MIS） cascode anode GaN lateral field-effect diode（CA-LFED） to achieve ultralow reverse leakage current(ILEAK).The device based on AlGaN/GaN highelectron-mobility-transistor（HEMT） includes a normally-off MIS-controlled channel that is cascoded with a high barrier height Schottky *** reverse bias,the high electric-field is effectively prevented by the normally-off MIS-controlled channel *** with the high barrier height Schottky contact,this feature significantly suppresses the *** by the device fabrication,the CA-LFED with high breakdown voltage（BV）>600 V shows an ultralow ILEAKof 3.6×10-9A/mm as well as a low forward voltage drop（VF） of 2.2 *** performance suggests that the CA-LFED can be a promising candidate for ultralow ILEAKand better VF-ILEAKtrade-off GaN power diode applications.

关键词： Schottky-MIS cascode anode lateral field-effect diode (LFED) ultralow reverse leakage current ($I_{\rm{LEAK}}$) forward drop and reverse leakage trade-off AlGaN/GaN HEMTs

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CACNA1A基因相关神经系统疾病谱研究进展

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中华实用儿科临床杂志 2022年第10期37卷 787-790页

作者：刘明丁昌红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

CACNA1A基因编码电压门控钙通道的孔道形成蛋白, 在中枢神经系统具有重要功能, 该基因突变可导致多种神经系统疾病, 包括家族性偏瘫型偏头痛1型、脊髓小脑共济失调6型、发作性共济失调2型和婴儿早发癫痫性脑病42型, 少数患者可见上述表... 详细信息

CACNA1A基因编码电压门控钙通道的孔道形成蛋白, 在中枢神经系统具有重要功能, 该基因突变可导致多种神经系统疾病, 包括家族性偏瘫型偏头痛1型、脊髓小脑共济失调6型、发作性共济失调2型和婴儿早发癫痫性脑病42型, 少数患者可见上述表型的重叠。现综述总结上述疾病表型的临床特点及CACNA1A基因的变异特点。

关键词：偏瘫型偏头痛脊髓小脑共济失调发作性共济失调癫痫性脑病

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Clinical phenotype features and genetic etiologies of 38 children with progressive myoclonic epilepsy

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Acta Epileptologica 2020年第1期2卷 111-125页

作者： Jing Zhang Ying Yang Xueyang Niu Jiaoyang Chen Wei Sun changhong ding Lifang Dai Liping Zhang Qi Zeng Yi Chen Xiaojuan Tian Xiaoling Yang Taoyun Ji Zhixian Yang Yanling Yang Yuwu Jiang Yuehua Zhang Department of Pediatrics Peking University First HospitalNo.1 of Xian Men StreetXicheng DistrictBeijing 100034China. Department of Neurology Xuanwu HospitalCapital Medical UniversityNo.45 of Changchun StreetXicheng DistrictBeijing 100034China. Department of Neurology Beijing Children’s HospitalCapital Medical UniversityNo.56 of South Lishi RoadXicheng DistrictBeijing 100045China. Department of Pediatrics Xuanwu HospitalCapital Medical UniversityNo.45 of Changchun StreetXicheng DistrictBeijing 100034China.

Background:Progressive myoclonic epilepsy(PME)is a group of neurodegenerative diseases with genetic heterogeneity and phenotypic similarities,and many cases remain unknown of the genetic *** study is aim to summarize the clinical features and study the genetic causes of PME ***:Sanger sequencing of the target gene,Next Generation Sequencing(NGS)panels of epilepsy,trio-based Whole Exome Sequencing(WES)and detection of cytosine-adenine-guanine(CAG)repeat number were used to investigate the genetic causes of PME ***:Thirty-eight children with PME whose seizure onset age ranged from 3 months to 12 years were collected from February 2012 to November 2019 in three hospitals in Beijing,*** seizure types included myoclonic seizures(n=38),focal seizures(n=19),generalized tonic-clonie seizure(GTCS)(n=13),absence seizures(n=4),atonic seizures(n=3),epileptic spasms(n=2)and tonic seizures(n=1).Twenty-seven cases were sporadic and 11 had family members *** PME-related genes were identified in 30 out of 38(78.9%)patients who had either recessively inherited or de novo heterozygous *** these 30 cases,there were 12 cases(31.6%)of neuronal ceroid lipofuscinoses(the causing gene contains TPP1,PPT1,CLN5,CLN6 and MFSD8),two cases of sialidosis(the causing gene is NEU1),two cases of neuronopathic Gaucher disease(the causing gene is GBA),one case of spinal muscular atrophy-progressive myoclonic epilepsy(the causing gene is ASAH1),four cases of KCNC1 mutation-related PME,four cases of KCTD7 mutation-related PME,two cases of TBC1D24 mutation-related PME,one case of GOSR2 related PME,and two of dentatorubral-pallidoluysian atrophy(the causing gene is ATN1).In total,13 PME genes were identified in our *** etiology was not clear in eight ***:PME is a group of clinically and genetically heterogeneous *** diagnosis was clear in 78.9%of PME *** of genetic testing methods could inc

关键词： Progressive myoclonic epilepsy Genotype Phenotype

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Chinese patients with ***756 mutations of ATP1A3: Clinical manifestations, treatment, and follow-up

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Pediatric Investigation 2022年第1期6卷 5-10页

作者： Weihua Zhang Jiuwei Li Xiuwei Zhuo Ji Zhou Weixing Feng Shuai Gong Xiaotun Ren changhong ding Tongli Han Fang Fang Department of Neurology Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina

Importance:The phenotypes of ATP1A3 gene mutations are *** encephalopathy with cerebellar ataxia and fever-induced paroxysmal weakness and encephalopathy(FIPWE)are considered non-classical phenotypes caused by ***756 mutations of ***:To summarize the clinical manifestations,treatment,and followup of Chinese patients with ***756 mutations of ***:We analyzed the clinical features,treatment,and genotypes of eight children with ***756 mutations of ATP1A3 who were treated in Beijing Children’s Hospital from January 2014 to December ***:Eight patients(six boys and two girls)were included;seven had been misdiagnosed with *** age of onset ranged from 0.8 to 4.5 *** patients had encephalopathy and had at least one episode of *** ataxia was present in nine *** splenial lesions of the corpus callosum were found in two patients in the acute *** types of heterozygous ATP1A3 mutations were found:c.2267G>T(p.R756L)(patient 3[P3]),c.2266C>T(p.R756C)(P2 and P4),and c.2267G>A(p.R756H)(P1,P5,P6,P7,and P8).Six mutations were de novo;two mutations were *** patients with p.R756C and one patient(P7)with p.R756H had four episodes of severe ataxia as the main ***,in the other three episodes,limb weakness was more prominent than ataxia.P5 with p.R756H exhibited overlap with FIPWE and rapid-onset ***:Acute encephalopathy followed by febrile disease was characteristic of the disease in patients with ***756 mutations of ***,the weakness and ataxia were *** crossover and overlap were observed among these patients.

关键词： ATP1A3 Mutation Encephalopathy Fever

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磷酸甘油酸脱氢酶缺乏1例

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中华儿科杂志 2023年第7期61卷 653-655页

作者：张晓燕丁昌红任淑红首都医科大学附属北京儿童医院保定医院神经内科保定071000 国家儿童医学中心首都医科大学附属北京儿童院神经内科北京100045

患儿男,10月龄,因"点头拥抱样动作2个月"就诊。主要表现为智力及运动发育迟缓,婴儿痉挛症和小头畸形。全外显子基因测序提示PHGDH基因c.946-2A>G和c.766G>A,为复合杂合变异,诊断为磷酸甘油酸脱氢酶缺乏,该病临床症状缺乏特异性,按癫痫... 详细信息

患儿男,10月龄,因"点头拥抱样动作2个月"就诊。主要表现为智力及运动发育迟缓,婴儿痉挛症和小头畸形。全外显子基因测序提示PHGDH基因c.946-2A>G和c.766G>A,为复合杂合变异,诊断为磷酸甘油酸脱氢酶缺乏,该病临床症状缺乏特异性,按癫痫发作类型选择抗癫痫药物治疗可以部分有效控制发作。

关键词：磷酸甘油酸小头畸形癫痫发作类型运动发育迟缓婴儿痉挛症基因测序临床症状控制发作

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转录组学技术在人参皂苷生物合成途径研究中的应用进展

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食品与发酵工业 2022年第16期48卷 318-325页

作者：季宏与丁常宏于丹都晓伟黑龙江中医药大学药学院黑龙江哈尔滨150040

转录组学技术是挖掘新基因和阐明人参皂苷生物合成分子机理的重要工具,通过转录组学研究可以从细胞整体水平上探究基因表达情况及调控规律,对于促进人参皂苷生物合成,提高人参药用植物种质资源质量具有深远意义。该文在对转录组学研究... 详细信息

转录组学技术是挖掘新基因和阐明人参皂苷生物合成分子机理的重要工具,通过转录组学研究可以从细胞整体水平上探究基因表达情况及调控规律,对于促进人参皂苷生物合成,提高人参药用植物种质资源质量具有深远意义。该文在对转录组学研究方法及新技术回顾总结的基础上,从人参皂苷合成反应的3个阶段层面,阐述了转录组学技术在挖掘人参皂苷生物合成途径中候选基因的应用,对目前人参转录组学研究存在的问题及未来研究方向进行了讨论,采用转录组前沿技术及多组学联用技术手段,获得人参综合全面的生物学信息,更能深入研究影响人参皂苷合成的相关基因,以期为全面了解人参皂苷生物合成机制及调控网络提供借鉴和参考。

关键词：人参转录组学人参皂苷生物合成基因

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