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Kearns-Sayre综合征一例临床病理分析 被引量:1
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作者 许蕾 刘瑞春 +4 位作者 朱一飞 王丽琴 曹翠芳 魏素琴 薛平 《脑与神经疾病杂志》 2009年第5期379-382,共4页
目的探讨Kearns-Sayre综合征(KSS)的临床与病理形态学表现,旨在提高对该病的认识。方法对1例KSS患者的临床和病理特点进行分析。结果该患者表现为视物不清、双上睑下垂、慢性进行性眼外肌瘫痪、视网膜色素变性,眼底色素改变呈"椒盐... 目的探讨Kearns-Sayre综合征(KSS)的临床与病理形态学表现,旨在提高对该病的认识。方法对1例KSS患者的临床和病理特点进行分析。结果该患者表现为视物不清、双上睑下垂、慢性进行性眼外肌瘫痪、视网膜色素变性,眼底色素改变呈"椒盐状"、晕厥发作、心脏传导阻滞、小脑性共济失调、不育、脑脊液蛋白升高、脑脊液叶酸/血清叶酸比值降低。肌肉活检MGT染色发现有不整红边纤维(RRF),出现比例为11.6%。电镜观察表现为线粒体数目增多,形态异常,嵴排列紊乱等,可见线粒体内类结晶状包涵体。结论本例提示尽管KSS的临床表现多样,但对不明原因的单侧或双侧上睑下垂、眼球运动障碍伴视网膜色素变性和晕厥等症状的患者,应想到KSS的诊断,进行肌肉活检、遗传咨询和采取必要的措施,防止威胁生命的事件发生。 展开更多
关键词 Kearns—sayre综合征 视网膜色素变性 心脏传导阻滞 精子
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Kearns-Sayre syndrome的临床研究 被引量:1
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作者 卢国艳 刘瀚旻 +3 位作者 雷明雨 王涛 郑忠梅 罗蓉 《中华妇幼临床医学杂志(电子版)》 CAS 2009年第4期31-34,共4页
目的探讨Kearns-Sayre syndrome(KSS)的临床诊断及治疗方法。方法对2006年8月和2007年12月本院收治的2例Kearns-Sayre syndrome患儿的临床表现、诊断依据、实验室检查结果进行研究,并对随访1年的结果进行分析总结。结果2例患儿均表现为2... 目的探讨Kearns-Sayre syndrome(KSS)的临床诊断及治疗方法。方法对2006年8月和2007年12月本院收治的2例Kearns-Sayre syndrome患儿的临床表现、诊断依据、实验室检查结果进行研究,并对随访1年的结果进行分析总结。结果2例患儿均表现为20岁前发病、慢性进行性眼外肌麻痹、视网膜色素变性Kearns-Sayre syndrome三联征及心脏传导阻滞、脑脊液蛋白升高,同时伴小脑共济失调、听力损伤、智力低下等临床症状。病例1确诊后放弃治疗出院,1年后身高、智力较同龄人低下,肌无力进行性加重。病例2给予心脏起搏器安置后,起搏器工作良好,1年内身高增长约8cm,与同龄儿比较,生长速率无明显差异,肌无力症状无明显加重,生活质量改善。结论对于20岁前发病、慢性进行性眼外肌麻痹、视网膜色素变性的Kearns-Sayre syndrome三联征患者,并具备以下3点之一:心脏传导阻滞、脑脊液蛋白含量升高(>100mg/dL)[正常值为(20~40)mg/dL]、小脑共济失调,即可作出Kearns-Sayre syndrome的临床诊断,肌活检及基因检测,可进一步支持和确诊Kearns-Sayre syndrome。对该病患儿进行早期对症治疗可明显改善预后。 展开更多
关键词 Kearns—sayre syndrome(KSS) 诊断 治疗
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Kearns-Sayre综合征并完全性房室传导阻滞1例 被引量:1
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作者 赵亮 周同甫 +1 位作者 卢国艳 华益民 《实用儿科临床杂志》 CAS CSCD 北大核心 2009年第4期320-320,共1页
关键词 Kearns—sayre综合征 心脏传导阻滞 线粒体脑肌病
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Kearns——Sayre综合征
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作者 李康增 《中国医师进修杂志》 1990年第10期46-47,共2页
Kearns—Sayre综合征(KSS)是一种以眼肌麻痹、视网膜变性和心脏传导功能障碍三联征为特征的线粒体神经肌病。1958年,Kearns及Sayre等首次对本征作了描述。目前已逐渐被人们认识,KSS并非十分罕见。国外文献已报道105例。国内杨国芳等于1... Kearns—Sayre综合征(KSS)是一种以眼肌麻痹、视网膜变性和心脏传导功能障碍三联征为特征的线粒体神经肌病。1958年,Kearns及Sayre等首次对本征作了描述。目前已逐渐被人们认识,KSS并非十分罕见。国外文献已报道105例。国内杨国芳等于1988年首次报告1例。为便于发现和报道更多的KSS,笔者综述如下。 展开更多
关键词 Kearns sayre 传导功能障碍 肌病 眼肌麻痹 视网膜变性 线粒体功能 传导系统障碍 束支阻滞 MELAS
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基于口述史辨析互联网诞生的五大历史争议
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作者 金文恺 彭筱军 《汕头大学学报(人文社会科学版)》 2019年第12期80-88,共9页
诞生于50年前的阿帕网——互联网的前身,可以说是20世纪最深远改变人类文明的发明之一。互联网口述历史(OHI)项目组通过连续12年访问全球470余人次的互联网先驱,不断走入历史深处,结合第一手的口述历史资料,梳理互联网诞生的5个激烈争... 诞生于50年前的阿帕网——互联网的前身,可以说是20世纪最深远改变人类文明的发明之一。互联网口述历史(OHI)项目组通过连续12年访问全球470余人次的互联网先驱,不断走入历史深处,结合第一手的口述历史资料,梳理互联网诞生的5个激烈争议。互联网先驱们对某些问题的看法各执一词,甚至针锋相对。其实争议本身并不重要,丝毫不影响这些互联网先驱们各自的贡献和伟大,通过各自的角度引发的争议,不仅体现了Sayre法则,也符合开放、自由的互联网精神,更能让我们清晰了解很多历史细节和背后的故事,进一步理清当年互联网创始时的生动往事。总结这些争议,不是为了辩是非,而是通过系统解析这些争议,让大家更好地了解互联网历史。 展开更多
关键词 互联网 网络社会 sayre法则 历史争议
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Impacts of degrading permafrost on streamflow in the source area of Yellow River on the Qinghai-Tibet Plateau,China 被引量:16
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作者 MA Qiang JIN Hui-Jun +4 位作者 Victor F.BENSE LUO Dong-Liang Sergey S.MARCHENKO Stuart A.HARRIS LAN Yong-Chao 《Advances in Climate Change Research》 SCIE CSCD 2019年第4期225-239,共15页
Many observations in and model simulations for northern basins have confirmed an increased streamflow from degrading permafrost,while the streamflow has declined in the source area of the Yellow River(SAYR,above the T... Many observations in and model simulations for northern basins have confirmed an increased streamflow from degrading permafrost,while the streamflow has declined in the source area of the Yellow River(SAYR,above the Tanag hydrological station)on the northeastern Qinghai-Tibet Plateau,West China.How and to what extent does the degrading permafrost change the flow in the SAYR?According to seasonal regimes of hydrological processes,the SAYR is divided intofour sub-basins with varied permafrost extents to detect impacts of permafrost degradation on the Yellow River streamflow.Results show that permafrost degradation may have released appreciable meltwater for recharging groundwater.The potential release rate of ground-ice melt-water in the Sub-basin 1(the headwater area of the Yellow River(HAYR),above the Huangheyan hydrological station)is the highest(5.6 mm per year),contributing to 14.4%of the annual Yellow River streamflow at Huangheyan.Seasonal/intra-and annual shifts of streamflow,a possible signal for the marked alteration of hydrological processes by permafrost degradation,is observed in the HAYR,but the shifts are minor in other sub-basins in the SAYR.Improved hydraulic connectivity is expected to occur during and after certain degrees of permafrost degradation.Direct impacts of permafrost degradation on the annual Yellow River streamflow in the SAYR at Tanag,i.e.,from the meltwater of ground-ice,is estimated at 4.9%that of the annual Yellow River discharge at Tanag,yet with a high uncertainty,due to neglecting of the improved hydraulic connections from permafrost degradation and the flow generation conditions for the ground-ice meltwater.Enhanced evapotranspiration,substantial weakening of the Southwest China Autumn Rain,and anthropogenic disturbances may largely account for the declined streamflow in the SAYR. 展开更多
关键词 Streamflow Warming climate Permafrost degradation Streamflow patterns Source area of Yellow River(SAYR)
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Evidence for a recent warming and wetting in the source area of the Yellow River (SAYR) and its hydrological impacts 被引量:13
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作者 TIAN Hui LAN Yongchao +4 位作者 WEN Jun JIN Huijun WANG Chenghai WANG Xin KANG Yue 《Journal of Geographical Sciences》 SCIE CSCD 2015年第6期643-668,共26页
Climate change investigation at a watershed-scale plays a significant role in re- vealing the historical evolution and future trend of the runoff variation in watershed. This study examines the multisource hydrologica... Climate change investigation at a watershed-scale plays a significant role in re- vealing the historical evolution and future trend of the runoff variation in watershed. This study examines the multisource hydrological and meteorological variables over the source area of the Yellow River (SAYR) from 1961 to 2,012 and the future climate scenarios in the region during 2006-2100 based on the CMIP5 projection data. It recognizes the significant charac-teristics of the recent climate change in the SAYR and predicts the change trend of future flow in the region. It is found that (1) The climate in the SAYR has experienced a significant warm-wet change since the early 2000s, which is very different from the antecedent warm-dry trend since the late 1980s; (2) The warm-wet trend in the northwestern SAYR (the headwater area of the Yellow River (HAYR), is more obvious than that in the whole SAYR; (3) With pre- cipitation increase, the runoff in the region also experienced an increasing process since 2006. The runoff variations in the region are sensitive to the changes of precipitation, PET and maximum air temperature, but not very sensitive to changes in mean and minimum air temperatures; (4) Based on the CMIP5 projection data, the warm-wet climate trend in SAYR are likely to continue until 2049 if considering three different (i.e. RCP2.6, RCP4.5 and RCP8.5) greenhouse gas emission scenarios, and the precipitation in SAYR will not be less than the current level before 2100; however, it is estimated that the recent flow increase in the SAYR is likely to be the decadal change and it will at most continue until the 2020s; (5) The inter-annual variations of the East Asian winter monsoon are found to be closely related to the variations of annual precipitation in the region. Meanwhile, the increased precipitation as well as the increase of potential evapotranspiration (PET) being far less than that of precipitation in the recent period are the main climate causes for the flow increase in the region. 展开更多
关键词 source area of the Yellow River (SAYR) climate warming and wetting decadal scale hydrological impacts
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Changes in lake area and water level in response to hydroclimate variations in the source area of the Yellow River:a case study from Lake Ngoring
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作者 Yang PU Min ZHAN +4 位作者 Xiaohua SHAO Josef PWERNE Philip AMEYERS Jiaojiao YAO Da ZHI 《Frontiers of Earth Science》 SCIE CSCD 2023年第4期920-932,共13页
In the north-eastern Qinghai-Tibet Plateau(QTP),the source area of the Yellow River(SAYR)has been experiencing significant changes in climatic and environmental conditions in recent decades.To date,few studies have co... In the north-eastern Qinghai-Tibet Plateau(QTP),the source area of the Yellow River(SAYR)has been experiencing significant changes in climatic and environmental conditions in recent decades.To date,few studies have combined modern hydrological conditions with paleoclimate records to explore the mechanism(s)of these changes.This study seeks to improve understanding of hydrological variability on decadal and centennial timescales in the SAYR and to identify its general cause.We first determined annual fluctuations in the surface area of Lake Ngoring from 1985 to 2020 using multi-temporal Landsat images.The results show that lake surface area changes were generally consistent with variations in precipitation,streamflow and the regional dry-wet index in the SAYR,suggesting that the water balance of the Lake Ngoring area is closely associated with regional hydroclimate changes.These records are also comparable to the stalagmite δ^(18)O monsoon record,as well fluctuations in the Southern Oscillation Index(SOI).Moreover,an association of high TSI(total solar insolation)anomalies and sunspot numbers with the expansion of Lake Ngoring surface area is observed,implying that solar activity is the key driving factor for hydrologic variability in the SAYR on a decadal timescale.Following this line of reasoning,we compared the δ^(13)C org-based lake level fluctuations of Lake Ngoring for the last millennium,as previously reported,with the hydroclimatic history and the reconstructed TSI record.We conclude that the hydrological regime of Lake Ngoring has been mainly controlled by centennial fluctuations in precipitation for the last millennium,which is also dominated by solar activity.In general,it appears that solar activity has exerted a dominant influence on the hydrological regime of the SAYR on both decadal and centennial timescales,which is clearly manifested in the variations of lake area and water level of Lake Ngoring. 展开更多
关键词 Qinghai-Tibet Plateau(QTP) source area of the Yellow River(SAYR) lake area/level Asian summer monsoon(ASM) El Nino-Southern Oscillation(ENSO) total solar insolation(TSI)
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