Retrospective case-analysis of the magnetic resonance imaging characteristics in the brain of patients with Wilson disease

BACKGROUND:Wilson disease (WD)damages liver,brain,kidney,cornea and nervous system severely.It is manifested in four ways:brain,liver,kidney and bone muscle.Whether or not magnetic resonance imagling (MRI)can clearly display the diseased region and range in brain of patient with WD,which provides imageological evidence for clinical practice,is unclear.OBJECTIVE:To observe the charactedstics of MRI of brain in patient with SD,and analyze the correlation of diseased region with clinical symptoms.DESIGN:Retrospective case-analysis.SETTING:Department of Radiology,Second Hospital Affiliated to the General Hospital of Chinese PLA.PARTICIPANTS:Thirty-one patients,including 18 males and 13 females,with WD admitted to the Department of Neurology,Second Hospital Affiliated to the General Hospital of Chinese PLA between January 1999and December 2005 were retrieved.The involved patients presented serum copper oxidase (sCP) activity decreasling and/or caruloplasmin Ievel decreasling and/or urinary copper content increasling;typical extrapyramidal symptoms and/or physical sign;abnormality showed by slit-lamp examination,Kayser-Fleischer rling positive.METHODS:①All the involved patients underwent MRI examination.A GE 1.5T imagling equipment was used.Spin-echo sequence was adopted to perform T2 and T1-weighed image at transverse axis level.Partial cases subjected to head scannling at coronal and/or sagittal level.Gd-DTPA With dosage of 0.1 mmol/kg was the strongest in 4 cases.②MRI characteristics of patients with dliferent clinical symptoms were observed.MAIN OUTCOME MEASURES:MRI detection results of patients with WD and MRI characteristics of patients with different clinical symptoms.RESULTS:Thirty-one patients with WD participated in the result analysis.①Imageological examination results:WD lnvolved many regions in the brain:dorsal caudate putamen(n=28),thalamencephalon(n=25),mesencaphalon(n=25),globus pallidus(n=23),pons(n=21),posterlor limb of intemal capsule(n=16),dentate nucleus(n=16),caudata nucleus(n=15)and cerebral cortex(n=11).MRI presented hypo-intensity signal on T2-weighted image and T1-weighted image or isointensity signal on T1-weighted image in 24 patients,characteristic hypo-intensity signal of globus pallidus in 4 patients,mixed signal of hyper-and hypo-intensity in 2 patients,hypo-intensity signal of globus pallidus,pars anterior pedunculi carebd and pontine tegmentum on T1-weighted image in 1 patient.Pathological changes distdbuted in symmetry and focus of infection mostly presented mottling,lamellar or strip.Different degrses of cerebral cortex atrophy,especially subtentodal cerebellar atrophy,ware found in 20 patients.Four patients subjected to enhancement scannling,but no clear imaqling was found.③MRI characteristics of patients with different symptoms:Abnormal signal of dorsal caudate putamen was found in 28 patients with dystonia,21 patients with dysarthda and 16 patients with bradykinesia;Abnormal signal of mesencaphalon was found in 22 patients with trepidation,among which,18 presented abnormal signal of pons;Abnormal signal of caudate nucleus and lenticular nucleus was found in 15 patients with dysphagia;Abnormal signal of dentate nucleus was found in 16 patients with carebellar ataxia;Different degrees of changes in cerebral atrophy were found in 14 patients with detedoratling memory and dementia.CONCLUSION:MRI can clearly display the characteristics of diseased regions in brain of patient with WD.Diseased regions reflected by MRI have obvious differences in patients with different clinical neurosigns.     

体外循环浅低温心脏不停跳心内直视手术的临床研究

目的:探讨体外循环浅低温心脏不停跳心内直视手术的方法、疗效评价及其对心肌保护作用。方法:总结2004年3月～2008年12月间12例施行浅低温心脏不停跳心内直视手术病例,并行循环不阻断主动脉,鼻咽温维持在32～35℃,阻断上、下腔静脉,完成心内直视手术。结果:全组无围手术期死亡,心脏手术结束能顺利停机,术后血流动力学平稳,无严重心律失常,血尿发生率0%,无1例发生空气栓塞。1例心房切口缝合针眼渗血,行再次开胸止血,1例术后第32h发生急性肺梗塞,均经治疗痊愈。结论:浅低温心脏不停跳心内直视手术技术安全可行,是一种接近生理状态的心肌保护方法,可避免再灌注损伤,有较理想的心肌保护效果。     

病变椎体切除脊柱稳定性重建治疗胸腰椎转移瘤

目的:探讨病变椎体切除脊柱稳定性重建治疗胸腰椎转移瘤可行性与临床价值。方法:1999年7月-2006年4月,采用后正中加后外侧"T"形切口行病变椎体切除、钛网 切除的肋骨或取自体髂骨填充植骨、椎弓根钉棒系统内固定治疗16例胸腰椎转移瘤患者,随访观察患者术后局部疼痛缓解、脊髓神经功能恢复及脊柱稳定性情况。结果:术后患者腰背痛及放射性疼痛基本缓解,均于术后早期开始肢体功能锻炼,术后2-3周离床活动。随访8个月-3年,患者神经系统压迫症状明显改善,3例C级患者2例恢复到D级,1例恢复到E级;D级5例均恢复到E级。影像学复查内固定物无松动、断钉、断棒现象,钛网无移位,椎体无塌陷。结论:经后正中加后外侧"T"形切口手术切除病变椎体并重建脊柱的三柱稳定性结构治疗胸腰椎转移瘤是可行的,可提高患者生存期内的生活质量。     

新型重组人源抗狂犬病毒糖蛋白单链抗体的制备及活性分析

目的：利用大肠杆菌表达新型人源抗狂犬病毒糖蛋白单链抗体ScFv,并验证其活性。方法：采用基因融合获得ScFv基因,构建重组表达质粒pET-22b（＋）-ScFv,转化大肠杆菌BL21（DE3）,经IPTG诱导获得高效表达。结果：Western印迹显示目的蛋白表达正确,表达产物以包涵体形式存在,经Ni-NTA柱纯化和体外复性,获得纯度达90%的ScFv蛋白。ELISA结果显示在PBS及人血清中ScFv的结合稳定性有所提高,流式细胞术证明目的蛋白能靶向结合狂犬病毒,通过中和效价测定实验测得ScFv的中和效价为40 U/mg。结论：成功利用原核表达系统实现了对人源抗GPRV ScFv的表达,并且具有一定的中和活性。     

Ilizarov技术矫正儿童僵硬型马蹄内翻足畸形

[目的]探讨Ilizarov技术矫正僵硬性马蹄内翻足畸形的方法和效果。[方法]作者在2000年3月～2005年3月间，使用Ilizarov技术矫正9例11足重度僵硬性马蹄内翻足畸形，将连接于胫骨、跟骨、跖骨的外固定环互相连接、组合成复杂的三维外固定架，通过逐渐调整外固定架矫正畸形，从而使患足达到或接近正常足的外形和功能。[结果]按Garceau标准评定疗效，优6足，良4足，差1足。[结论]Ilizarov外固定架三维矫正马蹄内翻足畸形效果确实，尤其适用于大年龄儿童之僵硬、复发或难治性马蹄内翻足，有一定的临床应用价值。     

陈旧性髋臼骨折的治疗

目的探讨陈旧性髋臼骨折手术和影响临床结果的相关因素。方法2001年2月～2005年7月，收治陈旧性髋臼骨折37例，其中简单型骨折10例，复合型骨折27例。受伤至手术时间平均44．8d，选择Kocher—Langenbeek入路6例，髂腹沟入路1例，延长髂腹沟人路2例，前后联合入路28例。手术平均耗时226min，术中平均失血l798mL。结果全部患者平均随访18．9个月，采用Matta的复位标准以及改良的D’Aubigne和Postel临床结果评分。解剖复位31例，不满意4例，差2例；临床结果优21例，良9例，一般5例，差2例，优良率为81．1％。股骨头坏死1例，异位骨化11例，坐骨神经一过性麻痹4例。结论除单纯的后壁或／和后柱、前壁与前柱骨折选择单一的入路外，对于陈旧性髋臼骨折原则上采用前后联合入路或延长的髂腹沟人路；骨关节炎的发生与骨折的严重程度有关；股骨头坏死与术前股骨头是否向内或向后移位并无直接关系；临床结果、手术耗时和术中失血不仅与骨折程度和损伤至手术的时间有关，而且与手术医生的经验有关。     

妇产科理论授课方法改革的尝试

改革妇产科理论课讲授。改革课程设置，以病例为中心讲授，把讨论教学形式引入课堂，取得较好效果。     

鼻咽癌放疗后咽部菌群的变化

目的:了解鼻咽癌放疗后患者咽部菌群的变化,为临床防治继发感染提供实验依据。方法:32例鼻咽癌放疗患者,于放疗前后分别在照射区域粘膜采集唾液标本,进行细菌的分离培养鉴定,测量可培养细菌的检出率、检出量和构成比。结果:与放疗前相比,放疗后照射区域的链球菌属、葡萄球菌属、白色念珠菌和绿脓杆菌检出增加;而放线菌属和奈瑟菌属检出减少(P<0.05)。结论:鼻咽癌放疗可影响咽部的微生态平衡,是导致鼻咽癌放疗后患者发生感染的重要原因之一。     

电子教育资源整合综述

电子学习平台在依据用户特性提供合适的教育资源整合成高质量的课件上仍存在一些挑战,教育资源利用率低和不同电子学习平台上的教育资源交互不便等问题仍未完全解决.解决这些问题的一个办法就是高质量的教育资源整合.本文对教育资源整合中的几个关键性环节做了介绍,探讨了教育资源描述语言和教育资源整合中的知识表示问题,分析了几种教育资源开发方法,最后指出了今后教育资源整合的研究方向.     

肌电引导治疗仪的设计

介绍一种操作简单、成本较低、便于医生确定靶肌肉的肌电引导治疗仪.该仪器经过临床初步试验,效果良好.