检索结果-南通市图书馆

Clinical and Genetic Characteristics of Alagille Syndrome in Adults

Journal of Clinical and Translational Hepatology 2023年第1期11卷 156-162页

作者： Jianguo Li Haicong Wu Shuru Chen jiahui pang Heping Wang Xinhua Li Weiqiang Gan Department of Infectious Diseases The Third Affiliated Hospital of Sun Yat-sen UniversityGuangzhouGuangdongChina Guangdong Key Laboratory of Liver Disease Research The Third Affiliated Hospital of Sun Yat-sen UniversityGuangzhouGuangdongChina Department of Hepatobiliary Medicine 900th Hospital of Joint Logistics Support ForceFuzong Clinical Medical College of Fujian Medical UniversityFuzhouFujianChina

Background and Aims:Alagille syndrome(AGS)is an autosomal dominant multisystem disorder caused by mutations in the JAG1 and NOTCH2 genes.AGS has been rarely reported in adult patients,mainly because its characteristics in adults are subtle.The study aimed to improve the understanding of adult AGS by a descriptive case series.Methods:Eight adults diagnosed with AGS at our hospital between June 2016 and June 2019 were included in the study.Clinical data,biochemical results,imaging results,liver histopathology,and genetic testing were analyzed.Results:Three female and five male patients with a median age of 24.5 years at the time of diagnosis were included in the analysis.The clinical manifestations were adult-onset(62.5%,5/8),cholestasis(50%,4/8),butterfly vertebrae(62.5%,5/8),systolic murmurs(12.5%,1/8),typical facies(12.5%,1/8),posterior embryotoxon,and renal abnormalities(0/8).Genetic sequencing showed that all patients had mutations,with four occurring in the JAG1 gene and four in the NOTCH2 gene.Six were substitution mutations,one was a deletion mutation,and one was a splicing mutation.Five had been previously reported;but the others,one JAG1 mutation and two NOTCH2 mutations were unique and are reported here for the first time.Conclusions:The clinical manifestations highlighted by the current diagnostic criteria for most adults with AGS are atypical.Those who do not meet the criteria but are highly suspicious of having AGS need further evaluation,especially genetic testing.

关键词： Alagille syndrome Clinical features JAG1 NOTCH2 Adult

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

The relationship between zinc deficiency and infectious complications in patients with hepatitis B virus-related acute-on-chronic liver failure

引用

Gastroenterology Report 2022年第1期10卷 552-558页

作者： Xinhua Li Lu Wang Na He Yeqiong Zhang jiahui pang Heping Wang Meng Yu Yongyu Mei Liang Peng Wenxiong Xu Department of Infectious Diseases The Third Affiliated Hospital of Sun Yat-sen UniversityGuangzhouGuangdongP.R.China Guangdong Key Laboratory of Liver Disease Research The Third Affiliated Hospital of Sun Yat-sen UniversityGuangzhouGuangdongP.R.China Key Laboratory of Tropical Disease Control(Sun Yat-sen University) Ministry of EducationGuangzhouGuangdongP.R.China Department of Diagnostics Second School of Clinical MedicineBinzhou Medical UniversityYantaiShandongP.R.China

Background The prevalence of zinc deficiency is high in patients with chronic liver disease,but few studies have hitherto explored the relationship between the serum zinc level and hepatitis B virus(HBV)-related acute-on-chronic liver failure(ACLF).This study aimed to assess the association between zinc deficiency and infectious complications,and model for end-stage liver disease(MELD)score in patients with HBV-related ACLF.Methods Patients with HBV-related ACLF from the Department of Infectious Diseases of the Third Affiliated Hospital of Sun Yat-sen University(Guangzhou,China)between January 2019 and December 2019 were retrospectively analysed in this study.Their demographic,clinical,and laboratory data were retrieved from the hospital information system and analysed.The Student’s t-test was used for normally distributed continuous variables between two groups and the Chi-square test was used for categorical data.Univariate and multivariate logistic regression analyses were applied to identify independent parameters.Results A total of 284 patients were included in this study,including 205 liver cirrhosis and 79 non-cirrhosis patients.The proportion of patients with zinc deficiency was the highest(84.5%),followed by subclinical zinc deficiency(14.1%)and normal zinc level(1.4%).Patients in the zinc deficiency group had a higher MELD score than the subclinical zinc deficiency or normal zinc group(P=0.021).Age,total bilirubin,and serum zinc level were independent factors for infection(P_(s)<0.05).The serum zinc level in patients without complications at admission was significantly higher than that in patients with complications(P=0.004).Moreover,the serum zinc level in patients with prothrombin time activity(PTA)of<20%was significantly lower than that in patients with 20%≤PTA<30%(P=0.007)and that in patients with 30%≤PTA<40%(P<0.001).Conclusions Zinc deficiency is common in patients with HBV-related ACLF.Zinc deficiency is closely associated with infectious complications and MELD score in patients with HBV-related

关键词： hepatitis B virus acute-on-chronic liver failure mortality zinc complications

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

A novel nomogram based on routine clinical indicators for screening for Wilson's disease

引用

Liver Research 2023年第1期7卷 82-89页

作者： jiahui pang Shuru Chen Weiqiang Gan Guofang Tang Yusheng Jie Zhanyi Li Yutian Chong Youming Chen Jiao Gong Xinhua Li Yongyu Mei Department of Infectious Diseases The Third Affiliated Hospital of Sun Yat-sen UniversityGuangzhouGuangdongChina Guangzhou Eighth People's Hospital Guangzhou Medical UniversityGuangzhouGuangdongChina Department of Laboratory Medicine The Third Affiliated Hospital of Sun Yat-sen UniversityGuangzhouGuangdongChina Department of Infectious Diseases National Regional Medical Center for Infectious DiseasesKashgar Hospital Affiliated to Sun Yat-sen UniversityKashgarXinjiangChina

Background and aims:There is currently no single model for predicting Wilson's disease(WD).We aimed to create a nomogram using daily clinical parameters to improve the accuracy of WD diagnosis in patients with abnormal liver function.Methods:Between July 2016 and December 2020,we identified 90 WD patients with abnormal liver function who had homozygous or compound heterozygous mutations in the ATP7B gene.The control group included 128 patients with similar liver function but no WD during the same time period.To create a nomogram,we screened potential predictive variables using the least absolute shrinkage and selection operator model and multivariate logistic regression.Results:We developed a nomogram for screening for WD based on six predictive factors:serum copper,direct bilirubin,uric acid,cholinesterase,prealbumin,and reticulocyte percentage.In the training cohort,the area under curve(AUC)of the nomogram reached 0.967(95%confidence interval(CI)0.946e0.988),while the area under the precision-recall curve was 0.961.Based on the optimal cutpoint of 213.55,our nomogram performed well,with a sensitivity of 96%and a specificity of 87%.In the validation cohort,the AUC of the nomogram was as high as 0.991(95%CI 0.970e1.000).Conclusions:We developed a nomogram that can predict the risk of WD prior to the detection of serum ceruloplasmin or urinary copper,greatly increasing screening efficiency for patients with abnormal liver function.

关键词： Wilson's disease(WD) Diagnosis Nomogram Probabilistic model Predictor

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

家校合作对初中生非认知能力的影响研究——基于中国教育追踪调查数据分析

引用

基础教育参考 2024年第6期 65-80页

作者：庞佳慧徐丹诚首都师范大学教育学院北京100048 中国医学科学院北京协和医学院北京100730

教师和家长是影响学生发展的核心角色,加强教师和家长之间的合作,对促进学生的全面发展具有重要意义。文章基于2013—2014年及2014—2015年中国教育追踪调查数据,使用多层线性模型,分析了家校合作对初中生的非认知能力的影响。研究结果... 详细信息

教师和家长是影响学生发展的核心角色,加强教师和家长之间的合作,对促进学生的全面发展具有重要意义。文章基于2013—2014年及2014—2015年中国教育追踪调查数据,使用多层线性模型,分析了家校合作对初中生的非认知能力的影响。研究结果发现,家校合作对初中生的非认知能力存在显著正向影响,且对学校位于城乡接合部和乡镇农村、家庭经济地位处于中下阶层的初中生影响更大。基于此,建议推动家校合作提质增效,打破家校合作现实困境;保证家校合作公平渠道,推动弱势家庭融入家校合作;拓宽农村家校合作资源,增强农村家庭家校合作意愿。

关键词：家校合作非认知能力多层线性模型基础教育初中生

来源：

维普期刊数据库

同方期刊数据库博看期刊评论

在线全文

学校读者我要写书评

暂无评论

社会工作者的边界生产:形式与逻辑--以海南省A市某社会工作组织为例

引用

社会工作与管理 2024年第2期24卷 69-78页

作者：庞嘉慧张朝华祁菲暨南大学人文学院广东珠海519070

以海南省A市某社会工作组织为例,探索社会工作者这一情感劳动者是如何通过划分边界来获得积极的工作体验的。研究发现,社会工作者通常采用深层表演的策略破除边界,和案主建立亲密关系,以建构职业认同。而在社会工作组织这一场域内,社会... 详细信息

以海南省A市某社会工作组织为例,探索社会工作者这一情感劳动者是如何通过划分边界来获得积极的工作体验的。研究发现,社会工作者通常采用深层表演的策略破除边界,和案主建立亲密关系,以建构职业认同。而在社会工作组织这一场域内,社会工作者则团结起来,形成情感共同体,采用边界清晰化的方式来对待过度的行政干预。在与基层政府及其代理部门的互动中,社会工作者则进行表层表演,以在模糊的边界中维持自身工作的平衡。应将情感劳动纳入社会工作者的边界生产策略,关注社会工作者在跨越不同边界时的情感策略及其深层逻辑。

关键词：社会组织情感策略边界生产边界建构

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Seawater temperature and salinity sensing based on in-fiber Michelson-Fabry-Perrot hybrid interferometer employing frequency domain decomposition method

引用

Optoelectronics Letters 2024年第2期20卷 70-75页

作者： WANG jiahui WEI Xian ZHU Yan SHEN Yue pang Lipeng DUAN Shaoxiang Institute of Modern Optics Tianjin Key Laboratory of Micro-scale Optical Information Science and TechnologyNankai UniversityTianjin 300350China Foreign Cooperation Projects Department of PetroChina Dagang Oilfield Company Tianjin 300280China The 58th Research Institute of China Electronics Technology Group Corporation Wuxi 214035China Southern Marine Science and Engineering Guangdong Laboratory Zhuhai 519000China1

We propose an in-fiber Michelson-Fabry-Perrot(M-FP)hybrid interferometer for the simultaneous measurement of seawater temperature and salinity.The sensor head consists of two parallel hetero Fabry-Perot(FP)cavities fabricated on the end face of the twin core fiber(TCF).A fiber fusion taper is used to split and recouple the light in the two cores.In this case,the Vernier effect can be obtained which can greatly enhance the sensitivity and solve the problem of temperature cross-sensitivity.Different from the traditional demodulation method based on envelop detection,we employed frequency domain decomposition method(FDDM)to demodulate the sensing signal.The simulation results indicate that the proposed sensor has high sensitivity to salinity and temperature.Thanks to the merits of high sensitivity,ease of fabrication and small footprint,the proposed seawater temperature and salinity sensor would have potential applications in marine science,food industry and ocean ranching.

关键词： temperature salinity ocean

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Hierarchical Spinning of Janus Textiles with Anisotropic Wettability for Wound Healing

引用

Research 2024年第1期2023卷 97-105页

作者： Han Zhang Lingyu Sun jiahui Guo Yuanjin Zhao Department of Rheumatology and Immunology Nanjing Drum Tower HospitalSchool of Biological Science and Medical EngineeringSoutheast UniversityNanjingJiangsu 210096China Oujiang Laboratory(Zhejiang Lab for Regenerative Medicine Vision and Brain Health)Wenzhou InstituteUniversity of Chinese Academy of SciencesWenzhouZhejiang 325001China

Wound healing and tissue repair are recognized as basic human health problems worldwide.Attempts to accelerate the reparative process are focused on developing functional wound dressings.Herein,we present novel Janus textiles with anisotropic wettability from hierarchical microfluidic spinning for wound healing.The hydrophilic hydrogel microfibers from microfluidics are woven into textiles for freeze-drying treatment,followed by the deposition of electrostatic spinning nanofibers composed of hydrophobic polylactic acid(PLA)and silver nanoparticles.The electrospun nanofiber layer can be well coupled with the hydrogel microfiber layer to generate Janus textiles with anisotropic wettability due to the roughness of the hydrogel textile surface and the incomplete evaporation of PLA solution when reaching the surface.For wound treatment with the hydrophobic PLA side contacting the wound surface,the wound exudate can be pumped from the hydrophobic to the hydrophilic side based on the wettability differential derived drainage force.During this process,the hydrophobic side of the Janus textile can prevent excess fluid from infiltrating the wound again,preventing excessive moisture and preserving the breathability of the wound.In addition,the silver nanoparticles contained in the hydrophobic nanofibers could impart the textiles with good antibacterial effect,which further promote the wound healing efficiency.These features indicate that the described Janus fiber textile has great application potential in the field of wound treatment.

关键词： silver spinning healing

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Style-conditioned music generation with Transformer-GANs

引用

Frontiers of Information Technology & Electronic Engineering 2024年第1期25卷 106-120页

作者： Weining WANG jiahui LI Yifan LI Xiaofen XING School of Electronic and Information Engineering South China University of TechnologyGuangzhou 510600China

Recently,various algorithms have been developed for generating appealing music.However,the style control in the generation process has been somewhat overlooked.Music style refers to the representative and unique appearance presented by a musical work,and it is one of the most salient qualities of music.In this paper,we propose an innovative music generation algorithm capable of creating a complete musical composition from scratch based on a specified target style.A style-conditioned linear Transformer and a style-conditioned patch discriminator are introduced in the model.The style-conditioned linear Transformer models musical instrument digital interface(MIDI)event sequences and emphasizes the role of style information.Simultaneously,the style-conditioned patch discriminator applies an adversarial learning mechanism with two innovative loss functions to enhance the modeling of music sequences.Moreover,we establish a discriminative metric for the first time,enabling the evaluation of the generated music’s consistency concerning music styles.Both objective and subjective evaluations of our experimental results indicate that our method’s performance with regard to music production is better than the performances encountered in the case of music production with the use of state-of-the-art methods in available public datasets.

关键词： Music generation Style-conditioned Transformer Music emotion

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Layer-by-layer fabrication of montmorillonite coating immobilizing Cu_(2)O nanoparticles for continuously catalyzing glycerol to dihydroxyacetone

引用

Chinese Journal of Chemical Engineering 2024年第4期68卷 263-275页

作者： Kejin Li jiahui Liu Dajian Li Xiaolan Chen Chunhui Zhou Research Group for Advanced Materials&Sustainable Catalysis(AMSC) State Key Laboratory Breeding Base of Green Chemistry-Synthesis TechnologyCollege of Chemical EngineeringZhejiang University of TechnologyHangzhou 310032China Qing Yang Institute for Industrial Minerals Qingyang 242804China Engineering Research Center of Non-metallic Minerals of Zhejiang Province Zhejiang Institute of Geology and Mineral ResourcesHangzhou 310007China

Microreactors are increasingly used for green and safe chemical processes owing to their benefits of superior mass and heat transfer,increased yield,safety,and simplicity of control.However,immobilizing catalysts in microreactors remains challenging.In this investigation,a technique for creating Cu_(2)O/montmorillonite catalyst coating,using electrostatic attraction for layer-by-layer self-assembly,was proposed.The montmorillonite film's morphology and thickness could be efficiently regulated by adjusting the degree of exfoliation and surface charge of montmorillonite,alongside layer-by-layer coating times.The Cu_(2)O nanoparticles were immobilized using the flow deposition approach.The resulting Cu_(2)O@montmorillonite-film-coated capillary microreactor successfully transformed glycerol into dihydroxyacetone.The conversion of glycerol and product selectivity could be controlled by adjusting the molar ratio of reactants,temperature,residence time,and Cu_(2)O loading.The maximum glycerol conversion observed was 47.6%,with a 27%selectivity toward dihydroxyacetone.The study presents a technique for immobilizing montmorillonite-based catalyst coatings in capillary tubing,which can serve as a foundation for the future application of microreactors in glycerol conversion.

关键词： Microreactor Coating Montmorillonite Glycerol Cu_(2)O

来源：

维普期刊数据库

同方期刊数据库博看期刊评论

在线全文

学校读者我要写书评

暂无评论

Upgrading waste NO_(x) into amino acids via electrocatalysis on Co nanoparticles encapsulated in hollow carbon nanofibers

引用

Science China Chemistry 2024年第6期67卷 1946-1952页

作者： jiahui Xian Kaixing Cai Peisen Liao Shihan Wang Guangqin Li Key Laboratory of Bioinorganic and Synthetic Chemistry of Ministry of Education LIFMIGCMESchool of ChemistrySun Yat-Sen UniversityGuangzhou510275China

Electrocatalytic conversion of waste nitrogen oxide(NO_(x))into value-added nitrogenous chemicals,especially amino acid,is an emerging strategy to alleviate NO_(x)pollution.However,this process suffers from the coexistence of multiple competitive reactions leading to complex product distribution and low efficiency,and thus the rational design and modulation of efficient catalysts remains a formidable challenge.Herein,well-designed Co nanoparticles encapsulated in hollow carbon nanofiber(CoHCNF)were constructed to promote the amino acid electrosynthesis via dealing waste NO_(x)coupling with biomass-derived keto acids.Ascribed to the rapid reactant diffusion,electron transport and efficient contact with abundant accessible Co catalytic sites stemmed from the unique hollow nanotubular structure,Co-HCNF manifested impressive glycine synthesis performance in one-pot adopting NO as the nitrogen source with Faradaic efficiency(FE)of 41.7%and a corresponding yield rate of 222.3μmol h^(-1)as well as 120 h robust stability.Interestingly,it also demonstrates an excellent performance with the FE of 42.8%for NO_(3)~-solution as the nitrogen source.Specially,this strategy displays a broad universality for the synthesis of other amino acids.This work not only highlights the importance of hollow-structuring electrocatalyst towards upgrading wasted NO_(x)species into amino acids,but also promotes the further development of electrosynthetic systems for high-valued N-containing organic chemicals.

关键词： electrocatalysis NO_(x)reduction amino acid porous materials hollow structure

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

欢迎您,

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案：

请选择收藏分类：

通借通还

欢迎您,

建议与咨询 留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案： 新增检索档案 确定 取消

请选择收藏分类： 新增自定义分类 确定 取消

通借通还

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

请选择保存的检索档案：

请选择收藏分类：