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Engelmann病1例报告 被引量:1
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作者 金旭 蔡松林 +3 位作者 崔林一 南军 崔星国 金成镇 《中国矫形外科杂志》 CAS CSCD 2004年第9期691-691,共1页
关键词 engelmann 泼尼松 药物治疗 对称性硬化性厚骨症 进行性骨干发育不全症
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Camurati-Engelmann病1例
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作者 袁普卫 杨威 +2 位作者 康武林 周国干 张晓亮 《临床骨科杂志》 2016年第3期381-382,共2页
患者,男,25岁,因四肢骨钝性疼痛伴全身乏力3月余,于2015年3月28日入院。现病史:患者3个月前无明显诱因出现四肢酸痛无力,疼痛范围和程度呈缓慢进行性加重,自觉症状稍轻后于西安某医院骨肿瘤科诊治,给予相关辅助检查后诊断为“... 患者,男,25岁,因四肢骨钝性疼痛伴全身乏力3月余,于2015年3月28日入院。现病史:患者3个月前无明显诱因出现四肢酸痛无力,疼痛范围和程度呈缓慢进行性加重,自觉症状稍轻后于西安某医院骨肿瘤科诊治,给予相关辅助检查后诊断为“畸形性骨炎”,建议口服钙剂,连续服用4周后症状未见明显缓解,反而有加重趋势. 展开更多
关键词 Camurati—engelmann 骨发育不良 骨痛 TGF—β1
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青少年进行性骨干发育不良^(99)Tc m-MDP全身骨显像1例
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作者 谢文华 刘彬 +1 位作者 唐仁明 赵丽 《标记免疫分析与临床》 CAS 2023年第5期898-900,共3页
目的通过1例经基因检测确诊为进行性骨干发育不良(progressive diaphyseal dysplasia,PDD)的病例,分析PDD^(99)Tc m-MDP全身骨显像特征。方法收集患者临床资料、检验及影像学检查结果,结合相关文献,探讨进行性骨干发育不良^(99)Tc m-MD... 目的通过1例经基因检测确诊为进行性骨干发育不良(progressive diaphyseal dysplasia,PDD)的病例,分析PDD^(99)Tc m-MDP全身骨显像特征。方法收集患者临床资料、检验及影像学检查结果,结合相关文献,探讨进行性骨干发育不良^(99)Tc m-MDP全身骨显像影像特征。结果进行性骨干发育不良患者全身骨显像阳性,颅底骨及四肢长骨对称性、弥漫性、不均匀性显像剂分布增高。结论PDD全身骨显像表现为病变骨骼显像剂分布明显增高,全身骨显像能为该病诊断提供全面有效的影像学依据,对于本病诊断及疗效监测具有重要意义。 展开更多
关键词 进行性骨干发育不良 全身骨显像 青少年 病例报道
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北美蓝云杉体细胞胚发生技术研究 被引量:7
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作者 孙敬爽 贾桂霞 《北京林业大学学报》 CAS CSCD 北大核心 2010年第1期44-51,共8页
研究北美蓝云杉体细胞胚发生技术,对该树种的胚胎发生机理和建立相关的快繁技术体系等具有一定的理论意义。以成熟胚为外植体,分别对基本培养基、生长调节剂种类和浓度对其体细胞胚发生各阶段的影响进行了研究,同时观察体细胞胚发生过... 研究北美蓝云杉体细胞胚发生技术,对该树种的胚胎发生机理和建立相关的快繁技术体系等具有一定的理论意义。以成熟胚为外植体,分别对基本培养基、生长调节剂种类和浓度对其体细胞胚发生各阶段的影响进行了研究,同时观察体细胞胚发生过程的形态特征。结果表明:1)胚性愈伤组织诱导培养基,首先在DCR培养基+2,4-D5.0mg/L+6-BA4.0mg/L+KT2.0mg/L诱导15d后,之后转入DCR培养基+2,4-D1.0mg/L+6-BA0.5mg/L+KT0.5mg/L,胚性愈伤诱导率最高为45.3%;2)胚性愈伤组织保持与增殖培养基为1/2LM培养基+2,4-D1.0mg/L+6-BA0.5mg/L+KT0.5mg/L+PEG40001.0g/L,胚性愈伤组织增殖率最高为102.27%;3)IBA可促进成熟体细胞胚的诱导,以1/2LM+IBA0.5mg/L+ABA2.0mg/L(以上4种培养基均附加蔗糖30g/L,谷氨酰胺450mg/L,水解酪蛋白500mg/L,琼脂5.0g/L,pH=5.8)的体细胞胚诱导率最高,为38.10%;4)在1/2MS+1.0g/L活性炭的培养基上,体细胞胚萌发,子叶生长但胚根发生较少。北美蓝云杉体细胞胚诱导过程中出现严重的畸形胚现象及生根难的问题,今后有待进一步研究。 展开更多
关键词 北美蓝云杉 基本培养基 生长调节剂 体细胞胚
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Long-term landscape changes in a subalpine spruce-fir forest in central Utah, USA
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作者 Jesse L.Morris R.Justin DeRose Andrea R.Brunelle 《Forest Ecosystems》 SCIE CSCD 2016年第2期83-94,共12页
Background: In Western North America, increasing wildfire and outbreaks of native bark beetles have been mediated by warming climate conditions. Bioclimatic models forecast the loss of key high elevation species thro... Background: In Western North America, increasing wildfire and outbreaks of native bark beetles have been mediated by warming climate conditions. Bioclimatic models forecast the loss of key high elevation species throughout the region. This study uses retrospective vegetation and fire history data to reconstruct the drivers of past disturbance and environmental change. Understanding the relationship among climate, antecedent disturbances, and the legacy effects of settlement-era logging can help identify the patterns and processes that create landscapes susceptible to bark beetle epidemics. Methods: Our analysis uses data from lake sediment cores, stand inventories, and historical records. Sediment cores were dated with radiometric techniques (14C and 210pb/137Cs) and subsampled for pollen and charcoal to maximize the temporal resolution during the historical period (1800 CE to present) and to provide environmental baseline data (last 10,500 years). Pollen data for spruce were calibrated to carbon biomass (C t/ha) using standard allometric equations and a transfer function. Charcoal samples were analyzed with statistical models to facilitate peak detection and determine fire recurrence intervals. Results: The Wasatch Plateau has been dominated by Engelmann spruce forests for the last ~10,500 years, with subalpine fir becoming more prominent since 6000 years ago. This landscape has experienced a dynamic fire regime, where burning events are more frequent and of higher magnitude during the last 3000 years. Two important disturbances have impacted Engelmann spruce in the historical period: 1) high-grade logging during the late 19th century; and (2) a high severity spruce beetle outbreak in the late 20th century that killed 〉90 % of mature spruce (〉10 cm dbh). Conclusions: Our study shows that spruce-dominated forests in this region are resilient to a range of climate and disturbance regimes. Several lines of evidence suggest that 19th century logging promoted a legacy of simplified stand structure and composition such that, when climate became favorable for accelerated beetle population growth, the result was a landscape-scale spruce beetle outbreak. The lasting impacts of settlement-era landscape history from the Wasatch Plateau, UT may be relevant for other areas of western North America and Europe where sufficient host carrying capacity is important in managing for resistance and resilience to outbreaks. 展开更多
关键词 Spruce beetle Climate change Dendroctonus engelmann spruce LOGGING Western North America WILDFIRE
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Heterotrophic and Autotrophic Soil Respiration under Simulated Dormancy Conditions
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作者 Daniel Beverly Scott Franklin 《Open Journal of Forestry》 2015年第3期274-286,共13页
Carbon cycling research has increased over the past 20 years, but less is known about the primary contributors to soil respiration (i.e. heterotrophic and autotrophic) under dormant conditions. It is understood that s... Carbon cycling research has increased over the past 20 years, but less is known about the primary contributors to soil respiration (i.e. heterotrophic and autotrophic) under dormant conditions. It is understood that soil CO2 effluxes are significantly lower during the winter of temperate ecosystems and assumed microorganisms dominate efflux origination. We hypothesized that heterotrophic contributions would be greater than autotrophic under simulated dormancy conditions. To test this hypothesis, we designed an experiment with the following treatments: combined autotrophic heterotrophic respiration, heterotrophic respiration, autotrophic respiration, no respiration, autotrophic respiration in vermiculite, and no respiration in vermiculite. Engelmann spruce seedlings and soil substrates were placed in specially designed respiration chambers and soil CO2 efflux measurements were taken four times over the course of a month. Soil microbial densities and root volumes were measured for each chamber after day thirty-three. Seedling presence resulted in significantly higher soil CO2 efflux rates for all soil substrates. Autotrophic respiration treatments were not representative of solely autotrophic soil CO2 efflux due to soil microbial contamination of autoclaved soil substrates;however, the mean autotrophic contributions averaged less than 25% of the total soil CO2 efflux. Soil microorganism communities were likely the primary contributor to soil CO2 efflux in simulated dormant conditions, as treatments with the greatest proportions of microbial densities had the highest soil CO2 efflux rates. Although this study is not directly comparable to field dormant season soil CO2 effluxes of Engelmann spruce forest, as snowpack is not maintained throughout this experiment, relationships, and metrics from such small-scale ecosystem component processes may yield more accurate carbon budget models. 展开更多
关键词 Dormant AUTOTROPHIC RESPIRATION HETEROTROPHIC RESPIRATION Dormant SOIL CO2 Efflux SOIL RESPIRATION engelmann SPRUCE
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萧氏松茎象不同寄主的为害部位调查初报
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作者 吴晓敏 余波 《贵州林业科技》 2008年第1期40-43,共4页
萧氏松茎象(Hylobitelus xiaoi Zhang)是危害松科植物的重要钻蛀性林业有害生物,对不同的寄主其为害部位不同,造成的损失也不相同。通过实地观察和调查统计分析发现,萧氏松茎象在绥阳县为害湿地松蛀道在松树树干基部4.6cm韵左右... 萧氏松茎象(Hylobitelus xiaoi Zhang)是危害松科植物的重要钻蛀性林业有害生物,对不同的寄主其为害部位不同,造成的损失也不相同。通过实地观察和调查统计分析发现,萧氏松茎象在绥阳县为害湿地松蛀道在松树树干基部4.6cm韵左右位置,连续为害蛀道高度可达41cm,为害华山松蛀道在树干基部以下2—5cm,危害马尾松蛀道在基部0.2cm左右的位置。3种不同的寄主受害后损失也不相同,湿地松受害损失最大,死亡率达到15%以上:对华山松的死亡率达到5%以上;危害马尾松后很少造成死亡,损失最小。 展开更多
关键词 萧氏松茎象 寄主 危害部位 损失分析
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进行性骨干发育不全患者TGFβ1基因R218H突变的检测(英文)
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作者 梁玥宏 李汶 +2 位作者 李麓芸 叶义言 卢光琇 《中华医学遗传学杂志》 CAS CSCD 北大核心 2006年第5期502-504,共3页
目的Camurati-Engelmann病(Camurati-Engelmann disease, CED),又称进行性骨干发育不全(progressive diaphyseal dysplasia, PDD),是一种罕见的常染色体显性遗传病。该病临床表现以四肢肌肉萎缩、步态摇摆、四肢酸痛、第二性征发... 目的Camurati-Engelmann病(Camurati-Engelmann disease, CED),又称进行性骨干发育不全(progressive diaphyseal dysplasia, PDD),是一种罕见的常染色体显性遗传病。该病临床表现以四肢肌肉萎缩、步态摇摆、四肢酸痛、第二性征发育不良为特征,影像学表现为四肢长管状骨对称性皮质增厚、骨干增粗呈梭形和髓腔狭窄,但不侵犯骨端或骨骺。该病由TGFβ1基因突变导致,迄今国际上已发现10种突变。作者对1例中国进行性骨干发育不全患者进行TGFβ1基因的突变位点检测。方法应用变性高效液相色谱技术对TGFβ1基因全部7个外显子及其外显子-内含子边界进行分析,对变性高效液相色谱技术显示可能存在突变的外显子进行DNA直接测序。结果在TGFβ1基因第4外显子发现错义突变R218H。此突变在2000年由日本学者首次报道。结论该突变的发现为下一步的治疗和遗传咨询提供依据。 展开更多
关键词 Camurati-engelmann 进行性骨干发育不全 转化生长因子Β1基因 变性高效液相色谱 基因突变
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进行性骨干发育不良伴右侧横窦狭窄一例
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作者 贾欢 武彦敏 +4 位作者 张令霞 田晔 韩旭 马改英 陈丽萍 《中华神经科杂志》 CAS CSCD 北大核心 2018年第7期540-543,共4页
进行性骨干发育不良,又称Camurati—Engelmann病,是一种累及长骨骨干、出现进行性和对称性皮质增厚为特点的罕见常染色体显性遗传病;其致病基因是位于19q13的TGFB1,引起骨骼代谢异常。我们报道1例青年Camurati—Engelmann病患者,... 进行性骨干发育不良,又称Camurati—Engelmann病,是一种累及长骨骨干、出现进行性和对称性皮质增厚为特点的罕见常染色体显性遗传病;其致病基因是位于19q13的TGFB1,引起骨骼代谢异常。我们报道1例青年Camurati—Engelmann病患者,以突出慢性高颅压、进行性视力下降为特征,经全脑血管造影发现右侧横窦狭窄。首次揭示在Camurati—Engelmann病患者中静脉窦狭窄也是造成慢性高颅压的病因,并进行了血管内治疗。 展开更多
关键词 Camurati—Engeimann综合征 TGFB1基因 慢性高颅压 静脉窦狭窄
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