Urinary medium-chain acylcarnitines in medium-chain acyl-CoA dehydrogenase deficiency, medium-chain triglyceride feeding and valproic acid therapy: sensitivity and specificity of the radioisotopic exchange/high performance liquid chromatography method.

To determine the sensitivity and specificity of detecting urinary medium-chain acylcarnitines for the diagnosis of MCAD deficiency, 114 urine specimens from 75 children with metabolic diseases and controls were analyzed in a blinded fashion using a radioisotopic exchange/HPLC method. All 47 patients with MCAD deficiency were correctly diagnosed using the criterion hexanoylcarnitine or octanoylcarnitine peak areas larger than those of other medium-chain acylcarnitines. The majority of them were tested during the asymptomatic state without L-carnitine loading. Four patients with other defects of fatty acid oxidation and three patients receiving valproic acid had a similar acylcarnitine excretion pattern. To further examine the specificity of the method, eight infants receiving a diet enriched with medium-chain triglycerides and 13 additional patients receiving valproic acid were studied. Most of these also tested positive for MCAD deficiency by the above criterion. Analysis by a new gas chromatographic-mass spectrometric procedure revealed that octanoylcarnitine, not valproylcarnitine, was the most abundant medium-chain carnitine ester excreted by a patient treated with valproic acid. Quantitation of urinary hexanoylcarnitine and octanoylcarnitine showed considerable overlap among patients with MCAD deficiency and those receiving valproic acid or a medium-chain triglyceride-enriched diet. MCAD deficiency can be reliably detected in urine specimens by this method without the need for prior carnitine loading. However, other defects in fatty acid oxidation must be differentiated from MCAD deficiency, and a history of medium-chain triglyceride or valproic acid administration must be considered if the diagnosis of MCAD deficiency is sought through analysis of urinary acylcarnitines.     

1株诱变枯草杆菌溶栓酶性质研究的初探

目的 对从1株诱变的枯草杆菌的发酵液中，通过分离纯化得到的一种活性较高的纤溶酶进行性质研究。方法 按胰酶活性测定法测其水解酪蛋白活性，用SDS-PAGE测定分子量，等电聚焦测定其等电点。采用硫酸酚法测定糖含量，并对其药理性质进行研究。结果 其水解酪蛋白活性为2.1U/mg，分子量为62kDa。等电点为8.5。含糖量为32%，并且发现该酶具有直接降解纤维蛋白的活性。结论 此纤溶酶具有较高的活性及药理价值。     

UVA对人角质形成细胞的氧化损伤作用

①目的　探讨紫外线A(UVA)对人角质形成细胞氧化损伤的机制。②方法　用 5J/cm2 UVA照射角质形成细胞 ,酶生化法检测活性氧 (ROS)的含量及超氧化物歧化酶 (SOD)、谷胱甘肽过氧化物酶 (GSH PX)活性的变化 ,流式细胞仪测定紫外线对角质形成细胞凋亡的影响 ,原位杂交技术观察 p2 1mRNA的变化 ,并与非照射组比较。③结果　与对照组比较 ,UVA照射组ROS含量升高 (t =113.6 8,P <0 .0 0 1) ,SOD、GSH PX活性下降 (t =5 7.2 3、19.0 4 ,P <0 .0 0 1) ,细胞凋亡率升高 (t=5 3.2 8,P <0 .0 0 1) ,p2 1mRNA表达增强。④结论　UVA对人角质形成细胞的损伤与氧自由基生成增多及细胞抗氧化能力抑制有关     

自由基损伤和β2糖蛋白1在自身免疫疾病中的作用

目的 研究被自由基氧化修饰的β2糖蛋白1(β2GPl)在自身免疫疾病中自身抗体产生中的作用。方法 采用高氯酸沉淀，两步肝素连接的交联琼脂糖凝胶层析结合葡聚糖凝胶G—100分离纯化大鼠β2GP1；应用自由基氧化修饰大鼠β2GP1；氧化修饰的β2GP1(oxβ2GPl)、β2GPl分别加与不加心磷脂(CL)及CL免疫大鼠，ELISA方法检测免疫大鼠血清中抗β2GP1抗体和抗心磷脂抗体(ACA)。结果 两步亲和层析结合凝胶过滤层析有效地分离纯化了大鼠β2GPl，蛋白纯度达90．78％；通过碳基含量测定证实了自由基对β2GP1的氧化修饰；oxβ2GGP1加CL、β2GP1加CL、oxβ2GPl免疫组与空白对照组、实验对照组比较ACA水平有显著升高(P＜0.001)，oxβ2GP1加CL免疫组与β2GPl加CL免疫组比较ACA水平差异有显著性(P＜0.05)；oxβ2GP1免疫组与oxβ2GPl加CL免疫组比较ACA水平差异不显著(P＞0.05)；oxβ2GPl加CL、oxβ2GPl免疫组与对照组比较，抗oxβ2GP1抗体水平有显著升高(P＜0.05)。结论 两步肝素连接的交联琼脂糖凝胶层析结合葡聚糖凝胶G—100能有效地分离纯化β2GPl；oxβ2GPl诱导了ACA和抗oxβ2GPl抗体的产生；β2GPl可能是自身免疫疾病发生过程重要的辅助因子或靶抗原，而自由基对β2GP1损伤可能是自身免疫疾病发病机制中的一个重要病理过程。     

首例中国可视化人体心脏三维重建及临床意义

目的 对首例中国数字化可视人体的心脏进行三维重建，为心脏疾病的影像学诊断和外科手术提供三维解剖学依据。方法 将首例中国数字化可视人体心脏部分的薄层断面(层厚1．0mm)输入SGI工作站，经数据分割、对位重建、平滑处理和三维显示等步骤，完成对心脏结构的三维重建。结果 本研究完整地重建出了心脏结构，重建的结构能够以多结构、多彩色实体模型方式显示，可显示心脏内部各结构的空间位置和毗邻关系，可在三维空间伴置上绕任意轴旋转任意角度。结论 重建出的三维图像清晰地显示了心脏内部和整体的结构，实现了心脏的二维可视化。     

匹罗卡品致(癎)大鼠海马中表达生长抑素的中间神经元数目变化及其轴突出芽

Objective To investigate the roles of somatostatin(SS)positive intemeurons in the development and compensation of temporal lobe epilepsy.Methods Piloearpine-induced epilepsy rat model was established.Immunohistochemistry method was used to detect number changes and axonal sprouting of SS positive intemeurons in different domains of the hippocampus at difierent time points.Degeneration of SS positive interneurons and their neurophils were detected by the double immunofluorescence staining with SS and Fluoro-Jade B(FJB)at 7 and 60 days after status epilepticus (SE).Results In the exoerimental rat group,the number of SS positive neurons decreased in each hippocampal domain,and it reached the lowest at 7 days post-SE(There were 11.1±3.3 in hilus,2.8±0.9 in CA1region and 1.8±0.7 in CA1region,t=13.519,9.644 and 8.808,all P<0.01).In chronic phase,the number of SS neurons gradually recovered,and exceeded the control group in CA1 area at 60 days post-SE(12.8±1.5 vs 8.8±1.3,t=-4.506,P<0.01),however,the number of SS neurons in the hilus(25.5±4.6)and CA1 area(4.8±0.8)remained significantly less than normal levels(t value were 4.691 and 3.953.both P<0.01).Increased SS positive fibers were found in the lacunosum-molecular (1m)layer and outer molecular layer of dentate gyrus after 30 days post-SE,and numerous SS positive fibers were seen threnghout the layers of area CA1 at 60 days post-SE.Double immunofluuorescence revealed that a few SS positive interneurons and fibers were also labeled by FJB in area CA1 at 7 days post-SE and in CA domain/hilus at 60 days post-SE.Conclusions SS intemeurons loss plays an important role in the development of temporal lobe epilepsy.The loss is partially caIlsed by the degeneration and death of neurons;SS positive neurophils increase within area CA1 in chronic phase may play a significant role in the generation and compensation of temporal lobe epilepsy.     

甲磺酸罗比卡因与盐酸罗比卡因用于硬膜外阻滞的效应比较

目的 评价甲磺酸罗比卡因用于硬膜外阻滞的效应和安全性。方法 45例择期行下腹或下肢手术病人,随机分别接受用甲磺酸罗比卡因(8.94 mg/ml,观察组)或盐酸罗比卡因(7.5mg/ml,对照组)施行的硬膜外阻滞。观察两组在感觉阻滞、运动阻滞、镇痛和肌肉松弛方面的效果,同时观察用药前后肝肾功能变化。结果 观察组和对照组感觉阻滞平面达到T6以上的病例分别为84.3％和76％(P>0.05),Bromage≥3级的病例分别90％和92％(P>0.05)。两组感觉阻滞平面固定时间、Bromage达到最大级别时间、最大级别维持时间和运动阻滞维持时间均无显著性差异(P>0.05)。两组镇痛及肌松满意率无显著性差异。观察组术中2例发生低血压,2例发生心动过缓,而对照组仅1例发生低血压。两组术后24 h天冬氨酸氨基转移酶(AST)、天冬氨酸转氨酶(ALT)、尿素氮(BUN)和肌酐(Cr)均在正常范围。结论 甲磺酸罗比卡因与盐酸罗比卡因行硬膜外阻滞的效应基本相同,且无明显毒性。     

多站点综合性毕业考试的改革与实践

我校改革临床医学专业毕业考试模式与方法，在临床实习过程中组织多站点综合性考试，采用闭卷笔试方法考核临床理论知识，占70％；采用实践操作与现场口试方法考核临床技能及相关知识，占20％；采用开卷笔试方法考核医疗文书书写，占10％。实践证明，此项改革能促进医学生端正实习态度，调动实习的积极性与主动性，引导医学生在实习中重视理论学习与临床实践的密切结合，促进临床教学质量的提高。     

神经导航下经岩骨幕上下联合入路中面神经管的解剖研究

OBJECTIVE: To study the value of neuronavigation in the transpetroal approach, and to provide anatomic data for the protection of the nerves in the facial nerve canal (FNC) during surgeries. METHODS: Simulated surgery through the transpetroal approach was performed on 16 sides of 8 adult cadaver heads with the assistance by neuronavigation. The anatomy of the facial nerve and the relationship of related structures were observed and the distances from the utmost external edge of the mastoid to different segments of the FNC were measured. RESULTS: Neuronavigation was successful with all the FNC, with the mean error of less than 0.9 mm. The FNC could be divided into 3 segments, the labyrinthine, the tympanic and the mastoid segments, stretching 3.6+/-1.2 mm, 11.2+/-2.5 mm and 16.1+/-3.6 mm respectively and with diameters of 1.2+/-0.3 mm, 1.4+/-0.1 mm and 1.7+/-0.2 mm, respectively. CONCLUSION: Neuronavigation may help protect the FNC during surgical procedures, and a thorough knowledge of the anatomic features of the FNC can be significant for preservation of the facial nerves.     

市级医院临床护士工作压力调查

目的:调查分析邯郸市第四医院141名临床护士的工作压力情况,为寻找减轻压力措施及提高护理工作质量提供实验数据。方法:2004-02-06/2004-02-10对邯郸市第四医院141名护士采用自填式问卷进行调查,问卷内容分为引起护士压力的常见因素及压力程度两部分。引起压力常见因素包括:担心差错事故、生活规律改变、晋升问题、人际关系、工作任务繁重、工资收入、学习考试。压力程度:压力很大指护理人员自我感觉疲惫心慌,对日常工作和生活造成显著的影响;压力较大指自我感觉压力较大,对日常工作和生活有一定程度的影响;压力一般指自我感觉存在一定程度的压力,但主观上能够适应,对自己不是一种负担;没有压力指工作轻松愉快,主观感觉没有任何不适应。结果:①不同科室压力分布:内科系统45名中有8名认为压力很大或较大,外科系统62名中有34名认为压力很大或较大,急诊科15名中有13名认为自己的工作压力较大。与内科系统比较,外科系统、急诊科普遍认为工作压力较大(P<0.01或0.05)。②年龄、性别、职称、学历对压力分布的影响:年龄、性别、职称、学历对护士压力分布无明显影响(P>0.05)。③各种压力因素及所占比例分析:担心差错事故的发生位居首位,高达78.01%;其次为生活规律改变及晋升问题,分别为48.94%和47.52%。结论:临床护士压力分布是不平衡的,外科系统和急诊科的工作压力相对较大,担心差错事故发生是引起护士压力的主要因素。