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Anticipation,anti-glaucoma drug treatment response and phenotype of a Chinese family with glaucoma caused by the Pro370Leu myocilin mutation

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International Journal of Ophthalmology(English edition) 2014年第1期7卷 44-50页

作者： Chun-Mei Li Yue-Hong Zhang Rong-Hua Ye chang-xian yi yi-Min Zhong Dan Cao Xing Liu State Key Laboratory of Ophthalmology Zhongshan Ophthalmic CenterSun Yat-sen University First Municipal People's Hospital of Guangzhou

AIM: To describe the anticipation and anti-glaucoma drugs response of a Chinese family with juvenile-onset open angle glaucoma(JOAG) caused by the Pro370Leu myocilin(MYOC) mutation. ·METHODS: Fifteen members of a three-generation Chinese family with JOAG were recruited to this study. They all underwent ophthalmic common examinations. Patients suspected to have JOAG got an assessment of visual field and optical coherence tomography. Intraocular pressures(IOPs) of four patients were measured at 8,10,12,14,17 o’clock respectively after using anti-glaucoma drugs. Mutation screening of all MYOC gene coding exons of the participants was performed by using direct sequencing of PCR products. ·RESULTS: Clinical examinations and pedigree analysis revealed eight family members were suffered from JOAG. Apparent genetics anticipation phenomenon was observed in this family. Their clinical features included elevated IOP of 35-55mmHg,loss of visual field,thinning of retinal nerve fiber layer,and glaucomatous optic disc damage. Noticeably,their intraocular pressure levels could be controlled within normal range at 8 and 10 o’clock by anti-glaucoma drugs,but their IOPs would elevate >21mmHg after 12 o’clock. Seven patients received trabeculectomy produced thin-walled,pale,and saccate filtering blebs maintaining lower intraocular pressure efficiently. Mutation screening indentified aheterozygous C→T missense mutation in the MYOC gene at position 1 109 in exon 3,corresponding to a substitution of a highly conserved proline to leucine at codon 370 in the olfactomedin domain of MYOC. ·CONCLUSION: The clinical characteristics of JOAG in this family were 1) genetics anticipation; 2) high IOP; 3) temporay response to anti-glaucoma drugs; 4) filtering surgery produced thin-walled and saccate filtering blebs,helping maintain lower IOP.

关键词： phenotype anticipation anti-glaucoma drugs myocilin

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Treatment of portal hypertension from portal vein cavernoma with the meso-Rex bypass

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Chinese Medical Journal 2013年第5期126卷 971-973页

作者： LI Jin-liang CHEN Wei-xiu XU chang-xian WANG Ruo-yi CHEN Yu-li Division of Pediatric Surgery Department of Surgery SecondHospital and Medical School of Shandong University JinanShandong 250033 China

Extrahepatic or prehepatic portal vein cavernoma and thrombosis is a more common condition in children than that in adult patients with extrahepatic or prehepatic portal vein obstruction （EHPVO）, which involves approximately 30% of children with portal hypertension and can involve all the portal hypertension complications. Cavernomatous transformation of the portal vein is common after portal thrombosis in non-cirrhotic patients. This challenging situation is relatively more frequent after liver transplantation, particularly in pediatric recipients. Acute bleeding from esophageal and gastric varices is temporarily treated by sclerotherapy or variceal banding. Children who experience continued bleeding despite medical management or who experience clinically significant hypersplenism are referred for surgery.

关键词： hypertension portal extrahepatic bypass surgery

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我国动漫产业风险投资机制研究

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特区经济 2010年第2期 270-272页

作者：蒋昌波贤成毅景德镇高等专科学校财经与法律系江西景德镇333000 广西师范大学经济管理学院广西桂林541004

本文试从国内外动漫产业风险投资现状及特点入手,结合已有的投融资案例和政策背景,分析我国动漫企业的投资价值与风险,提出要大力发展我国动漫产业,必须构建合理的我国动漫产业风险投资机制。

关键词：动漫产业风险投资投资机制

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精神压力在中心性浆液性脉络膜视网膜病变病因学中的作用(英文)

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国际眼科杂志 2009年第5期9卷 809-811页

作者：李春梅易长贤广州中山大学中山眼科中心中国广东省广州市510060

中心性浆液性脉络膜视网膜病变,简称中浆,是指由于视网膜色素上皮缺陷导致液体从脉络膜毛细血管局灶性渗出,引起视网膜神经感觉层浆液性脱离的一组疾病。确切的病因还不清楚,虽然眼科学者一直认为精神压力与中浆的发生有着密切的关系,... 详细信息

中心性浆液性脉络膜视网膜病变,简称中浆,是指由于视网膜色素上皮缺陷导致液体从脉络膜毛细血管局灶性渗出,引起视网膜神经感觉层浆液性脱离的一组疾病。确切的病因还不清楚,虽然眼科学者一直认为精神压力与中浆的发生有着密切的关系,但中浆发生的病理生理学机制还不清楚。在本文中我们提出一种假设,认为精神压力通过下丘脑-垂体-肾上腺(HPA)系统诱发中浆的发生。精神压力能增加HPA系统活性,从而引起糖皮质激素水平相应增高。增高的糖皮质激素收缩脉络膜血管,导致脉络膜缺血和血管内皮损伤,血管渗透性增加。视网膜色素上皮血液供应来源于脉络膜,脉络膜血液循环异常也将引起色素上皮功能障碍。血液中大分子蛋白进入视网膜下间隙,导致视网膜下积液,视网膜脱离。

关键词：精神压力中心性浆液性脉络膜视网膜病变下丘脑-垂体-肾上腺系统糖皮质激素

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玻璃体腔注射曲安奈德治疗视网膜静脉阻塞黄斑水肿(英文)

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国际眼科杂志 2010年第6期10卷 1030-1032页

作者：郭梦翔易长贤项道满中国广东省广州市妇女儿童医疗中心眼科 510120 中山大学中山眼科中心中国广东省广州市510610

目的:评价玻璃体腔注射曲安奈德治疗视网膜静脉阻塞合并黄斑水肿的疗效及并发症。方法:患者30例30眼玻璃体腔注射曲安奈德4mg治疗视网膜静脉阻塞合并黄斑水肿,观察治疗前、后的最佳矫正视力、眼压、裂隙灯显微镜检查、眼底荧光血管造影... 详细信息

目的:评价玻璃体腔注射曲安奈德治疗视网膜静脉阻塞合并黄斑水肿的疗效及并发症。方法:患者30例30眼玻璃体腔注射曲安奈德4mg治疗视网膜静脉阻塞合并黄斑水肿,观察治疗前、后的最佳矫正视力、眼压、裂隙灯显微镜检查、眼底荧光血管造影和光学相干断层扫描的变化,采用SPSS 12.0软件进行统计学分析。结果:所有患者手术后视力均显著提高,平均黄斑中心凹厚度(CMT)显著减少。病程、年龄、注射前CMT及视网膜静脉阻塞的类型和视力预后无相关性,注射前视力与注射后末次视力呈正相关。结论:玻璃体腔注射曲安奈德治疗视网膜静脉阻塞合并黄斑水肿简单、安全、易操作,短期内可以迅速减轻黄斑水肿,最终的视力预后取决于治疗前的视力,部分患者在注射后3 ～6mo可能复发。

关键词：黄斑水肿视网膜静脉阻塞曲安奈德

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Spatiotemporal evolution of Ediacaran-Cambrian(ca. 551–523 Ma)sponges in South China and their simulated contributions to marine oxygenation

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Science China Earth Sciences 2025年

作者： Yan YE Lei ZHANG Can CHEN Shan chang Yuhao yi Qinglai FENG State Key Laboratory of Geological Processes and Mineral Resources China University of Geosciences(Wuhan) School of Earth Science China University of Geosciences(Wuhan) Department of Geology Lund University Institute of Sedimentary Geology Chengdu University of Technology Key Laboratory of Deep-time Geography and Environment Reconstruction and Applications of Ministry of Natural Resources Chengdu University of Technology Second Geological Brigade of Hubei Geological Bureau College of Urban and Environmental Sciences Hubei Normal University Exploration and Development Research Institute SINOPEC Jianghan Oilfield Company

The origin and evolution of sponges have attracted interest, not only because they are among the most basal metazoans, but also because they act as important ecological engineers in the ocean. Previous research suggested that sponge groups diversified quickly during the Cambrian Series 2, while Terreneuvian sponge fossils are conspicuously rare and poorly substantiated. Yet, an increasing number of sponge spicules have been reported from the Ediacaran-Cambrian(E-C, ca.551–523 Ma) transitional carbonates/cherts worldwide, particularly in South China, which calls for updated discussions. Ocean oxygenation has been widely proposed to play a critical role in organismal evolution, but its relationship with sponges is unclear and falls into a “chicken-egg” dilemma. In this study, we summarize reported sponge spicule distributions from five E-C sections in South China to investigate the early evolutionary pattern of siliceous sponges. According to current stratigraphic correlation,monaxons first appear in the late Ediacaran in the intrashelf basin-slope area, and then potentially expand to the continental shelf and deep basin in the lower Cambrian. In these sections, the abundance, size, and diversity of monaxons increase upwards,followed by the appearance of diaxons, triaxons and then polyactines. Despite the uncertain affinity of the sponge fossils, it is possible that siliceous hexactinellids evolved later than siliceous demosponges, given that the earliest spicule assemblages are exclusively composed of monaxons, which commonly form bundles comparable to modern demosponges. New geochemical data from the Sifangjing section, Guizhou Province and the Yanziqiao section, Hunan Province, South China show that the appearance of sponges usually corresponds to anoxic environment. In addition, we applied the recently proposed Biological Pump, Oxygen and Phosphorus(BPOP) model to trace the evolution of oceanic oxygen concentrations with the radiation of sponges. The results indic

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Constructing 2D bismuth-based heterostructure for highly efficient photocatalytic CO2 reduction

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Tungsten 2025年第1期7卷 71-79页

作者： Ruo-Hang Xu Hao-Yu Jiang Dan-Dan Cui Kun-Rong Du Hong-Run Zhang Sheng-Jie Xu Ya-Qi Li Zhi-Zhen Ren Liang Wang Wei-chang Hao yi Du School of Physics and BUAA-UOW Joint Research Centre Beihang University Shenzhen Institute of Beihang University Centre for Catalysis and Clean Energy School of Environment and Science Griffith University

Reforming carbon dioxide（CO2） into fuels and valuable chemicals using solar energy holds significant potential for emission reduction and addressing energy supply *** recombination of photocarriers remains a major obstacle,however,to enhancing solar energy conversion efficiency in the photocatalytic reaction *** two-dimensional materials,constructing heterogeneous structures has emerged as an effective strategy to enhance the separation of photoexcited carriers by leveraging the internal electric field at the heterogeneous ***,we present a facile strategy for preparing Bi4NbO8Br/Bi2WO6plate-on-plate Z-scheme junctions with a strong internal electric field by in situ *** unique heterostructure facilitates efficient separation and transport of photoexcited charges by harnessing the internal electric field induced by chemical bonding at the lateral ***,the Bi4NbO8Br/Bi2WO6heterostructure exhibits enhanced CO2photoreduction properties,along with a CO yield rate of approximately 35 μmol·g-1·h-1under full light *** work offers novel insights into the synthesis of unique hetero structures intended to enhance the efficiency of photocatalytic CO2reduction.

关键词： Bi4NbO8Br/Bi2WO6 CO2 reduction Heterostructures Photocatalysts

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矮嵩草抗氧化物质含量的季节变化及与太阳辐射的关系

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四川草原 2001年第3期 32-36页

作者：张长青易现峰青海大学农牧学院农学系西宁810016

对移植西宁地区矮嵩草几种抗氧化物质含量的季节变化测定发现 ,矮嵩草叶片内紫外线吸收色素、抗坏血酸含量以及超氧化物歧化酶的活性在 5到 8月间呈“拱型”变化 ,6、 7月份最高。超氧阴离子自由基的相对含量呈现略微下降趋势。

关键词：魏嵩草抗氧化物质太阳辐射季节变化

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Informational uncertainties of risk assessment about accidents of chemicals

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Journal of Environmental Sciences 2001年第1期13卷 69-74页

作者： ZHANG yi-xian Suzhou Institute of Urban Construction and Environmental Protection Suzhou 215011China

An analysis system of informational uncertainties for accidental risk assessment of chemicals is introduced. Statistical test methods and fuzzy sets method can do the quantitative analysis of the input parameters. The uncerainties of the model can be used by quantitative compared method for the leakage accidents of chemicals. The estimation of the leaking time is important for discussing accidental source term. The uncertain analyses of the release accident for pipeline gas (CO) liquid chlorine and liquid propane gas (LPG) have been discussed.

关键词： uncertainty risk assessment accident chemicals pipeline gas liquid chlorine LPG

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Sensitive fluorescence probes for dihydrogen phosphonate anion based on calix[4]arene bearing naphthol-hydrazone groups

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Science China Chemistry 2010年第3期53卷 568-574页

作者： Chen, yi chang Liu, xian xian Huang, Hong Wu, Wei Wei Zheng, Yan Song Department of Chemistry Huazhong University of Science and Technology Wuhan 430074 China Department of Chemistry Guilin Normal College Guilin 541001 China

t-Butyl and t-pentylcalix[4]arenes bearing two 2-naphthol-1-hydrazone groups at the lower rim were synthesized,and showed excited-state intermolecular proton transfer fluorescent signal with basic *** are more sensitive to dihydrogen phosphate anion than to fluoride anion,although the latter has stronger *** with t-butylcalix[4]arene bearing two 2-naphthol-1-hydrazone groups,t-pentylcalix[4]arenes derivative has a larger fluorescent difference between dihydrogen phosphate and fluoride *** finding may be used to analyze dihydrogen phosphate anion in the presence of fluoride anion and provide a new approach for designing fluorescence probes that are highly selective for H2PO4-.

关键词： calixarene probe for anions fluorescence dihydrogen phosphate anion excited-state proton transfer

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