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51.
Neuronal ceroid lipofuscinoses (NCLs) represent a large group of inherited neurodegenerative disorders characterized by an abnormal accumulation of lipopigment in neuronal and extraneuronal cells. The authors present a case of juvenile neuronal ceroid lipofuscinosis in a 7-year-old boy. Ultrastructural examination of a skin biopsy disclosed deposits of curvilinear profiles and fingerprint-like structures in epithelial cells of sweat glands, endothelial cells, peripheral nerve endings, and fibroblasts. These findings allowed specific confirmation of the assumed diagnosis of juvenile neuronal ceroid lipofuscinosis. Due to the genotypic and phenotypic variability within the group of NCLs, the clinical investigation may be long and complicated. With the NCL disorders in mind, an accurate diagnosis based on ultrastructural examination of a skin biopsy may shorten this investigation, thus benefitting the patient.  相似文献   
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Vertical concentration profiles of airborne particulate matter were measured in four different indoor environments- library, coffee room, workshop and undergraduate student hostel- on the University Campus at Sutton Bonington. Measurements were carried out using an electronically-controlled lifting platform carrying a real-time optical particle monitor for sampling air sequentially at different heights within the breathing zone. Data was automatically logged at the different receptor levels, for the determination of the average vertical concentration profile of the various particle size ranges which include inhalable, thoracic, alveolic, PM10 and PM2.5. Vertical concentration profiles measured in these different indoor environments exhibited different characteristics but in almost all cases it was clear that different height groups of the population are exposed to different concentrations of the pollutant. This has implications on setting of air quality standards for the protection of public health. The results indicate that we may have to re-think the whole concept of air quality standards and develop protocols for indoor air quality monitoring and modelling which would take into account the above-mentioned factor.  相似文献   
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消毒供应中心(CSSD)的质量控制与一些相关因素有关,社会的发展,变迁,文明进步,工作人员对工作的理解,对技术的掌握,使目前的工作做的更好,使自己经手包装的每个手术器械包,各种穿刺包,急救包,治疗包,都按现行的标准准确无误.  相似文献   
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目的 观察血液透析过程对维持性血液透析 (maintenancehemodialysis,MHD)患者血浆中组织因子 (tissuefactor,TF)及其调节物 -组织因子途径抑制物 (tissuefactorpathwayinhibitor,TFPI)活性的影响。方法 应用发色底物法检测了 4 3名慢性肾衰竭患者的血浆组织因子、组织因子途径抑制物活性 ,其中 2 0例为未进行血液透析的终末期肾病 (end -stagerenaldisease ,ESRD)患者 (肾衰竭组 ) ,2 3例为慢性MHD患者在一次透析开始前 (透析前组 )和透析结束时 (透析后组 )的血浆TF、TFPI活性指标。同时观察透析过程中肝素对血浆TF、TFPI活性的影响。并对 2 3名健康自愿者 (正常对照组 )进行了血浆TF、TFPI活性的测定。结果 ①TF活性在肾衰竭组和透析前组均较正常对照组明显升高 ,分别为 [(1.1790± 0 .2 937)nmvs (0 .30 5 5±0 .190 1)nm ;(0 .884 8± 0 .5 4 6 1)nmvs (0 .30 5 5± 0 .190 1)nm ,(P均 <0 .0 1) ];肾衰竭组高于透析前组 ,但差异无显著性 (P >0 .0 5 ) ;②TFPI活性在透析前组和肾衰竭组均较对照组轻度升高 ,且肾衰竭组TFPI活性低于透析前组 ,但P均 >0 .0 5 ,无统计学意义 ;③一次透析过程中 ,透析后组TFPI活性明显高于透析前组[(2 .110 5± 1.36 37)u/mlvs (1.3347± 0 .84 19)u/ml,(P <0 .0 5 )  相似文献   
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阿托伐他汀对糖尿病肾病患者血清肿瘤坏死因子-α的影响   总被引:1,自引:0,他引:1  
欧阳文 《医学临床研究》2011,28(6):1102-1104
【目的】通过观察阿托伐他汀对糖尿病肾病(DN)患者血清肿瘤坏死因子-α(TNF-α)的影响,探讨阿托伐他汀对肾脏的保护作用。【方法】将75例DN患者随机分为不同剂量治疗组(A组、B组)和对照组(C组),每组25例。C组给予常规西药治疗,A组在C组基础上加用阿托伐他汀20mg/d,B组则40mg/d。用酶联免疫吸附法(Elisa)测定三组患者治疗前后血清TNF-α的含量变化,同时观察空腹血糖(FBG)、血肌酐(SCr)、尿素氮(BUN)、总胆固醇(TC)、甘油三脂(TG)的变化。【结果】A、B组患者治疗后血清TNF-α、FBG、SCR、BUN及TC、TG水平均下降,与本组治疗前比较差异有统计学意义(P〈0.05或P〈0.01);A、B组TNFld、SCR、BUN及TC、TG均低于C组(P〈0.05或P〈0.01),且B组TNF-α、SCR及BUN优于A组(P〈0.05)。【结论】阿托伐他汀不但能降低DN患者的血脂水平。而且可能通过降低血清TNF—α的含量,达到对DN的肾脏保护作用。  相似文献   
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Because of the inherent complexity of human health, the provision of good quality patient care requires collaboration in multidisciplinary teams. Integrative healthcare provides a unique setting for the study of interprofessional collaboration in the context of power disparities. The research objective was to examine which means and mechanisms were used to facilitate interprofessional collaboration when integrating complementary medicine (CM) into a hospital’s surgical department. Throughout 2010 we conducted a qualitative study in an Israeli public hospital’s surgical department, using observations and 30 in‐depth interviews with managers, surgeons, physicians, nurses, patients and CM practitioners. The sociological concepts of boundary actor and boundary object and the context of power relations served as a framework for this research. This article contributes to the field of interprofessional collaborative care research by: analysing types of collaboration inhibitors – epistemological and social‐structural gaps; pointing to boundary actors who establish interprofessional collaboration in an integrative hospital setting and noting the boundary objects they use; and comparing collaboration levels. The collaboration between CM practitioners and the department’s staff is a loosely coupled system. When coordination was achieved, reaching profound agreements seemed of lesser importance to the parties. Closer collaboration and cross‐fertilisation were found among CM practitioners.  相似文献   
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目的:探讨显微外科手术治疗急性期颅内动脉瘤的临床疗效及其对炎症因子水平的影响。方法:选取于我院治疗的颅内动脉瘤患者176例,随机分为两组,对照组和观察组各88例,观察组行急性显微外科手术治疗,对照组给予血管内介入栓塞术治疗,比较两组术后的疗效、并发症发生情况差异;比较术前及术后两组间脑脊液中白介素-1(IL-1)、白介素-6(IL-6)、内皮素-1(ET-1)、肿瘤坏死因子-αa(TNF—α)水平差异;1年后随访,进行GoS预后评分,比较预后差异。结果:手术后观察组完全栓塞率高于对照组,并发症总发生率则低于对照组,差异具有统计学意义(P%0.05);术后1、3、7d,观察组脑脊液中IL-1、IL-6、ET-1、TNF—α水平均低于对照组,差异均具有统计学意义(P〈0.05);随访1年观察组中81例恢复良好,达到92.05%,而对照组仅79.55%恢复良好,差异具有统计学意义(P〈0.05)。结论:早期进行显微外科手术治疗动脉瘤能改善患者预后,提高生活质量,疗效优于血管内介入栓塞术。  相似文献   
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血管内皮生长因子及其受体与眼内新生血管性疾病   总被引:8,自引:1,他引:7  
赵世红  何守志 《眼科研究》2003,21(1):103-106
眼内新生血管性疾病是致盲的主要原因之一,vEGF是主要的血管生成因子,许多生长因子是通过诱导vEGF的表达而起作用的。对vEGF及其受体与眼内新生血管性疾病的关系和针对其作用机制采取的抗眼内新生血管形成的措施进行综述。  相似文献   
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