Sensory axonopathy in hereditary distal spinal muscular atrophy

A girl of 14 year is presented with a distal spinal muscular atrophy (SMA) with autosomal recessive inheritance. The technical findings are in agreement with the diagnosis. Light microscopical examination of sural nerve biopsy, including teased fiber studies and morphometry, showed no abnormalities. Electron microscopical investigation however demonstrated axonal pathology. The question arises if distal SMA is a distal axonopathy mainly of motor nerves, but to some extent also of sensory nerves.     

rhGM—CSF对小鼠口腔粘膜损伤的防治作用

目的：观察rhGM-CSF对TMX致实验性小鼠口腔粘膜损伤的疗效。方法：按随机分组原则将501只昆明种小白鼠分成8组，分别在相应时间给予不同药物（MTX，CF或rhGM-CSF）的处理因素，于第1－10天光镜下观察小鼠口腔粘膜的病理改变和积分情况。结果：IDMTX致口腔粘膜损伤病变率（45％－63％）和积分率（19．4％－56％）较其它组高，且死亡率很低（小于5％）；IDMTX＋GM0（或GM2）组的口腔粘膜损伤病变率（30．53％和30．99％）和积分率（19．47％和17．25％）比IDMTX组（55．56％和36．31％）明显减少，且溃疡严重程度较轻，二者相差显著（P＜0．01）。结论：IDMTX致小鼠口腔粘膜损伤模型可以用于粘膜损伤的研究；rhGM-CSF可以减少MTX致小鼠口腔粘膜损伤，并促进粘膜损伤恢复。     

谷氨酰胺肠外营养对创伤后多器官衰竭患者的营养作用

目的探讨补充谷氨酰胺(Gln)的肠外营养在严重创伤后多器官衰竭(MOF)患者治疗中的作用。方法采用随机对照方法,将60例MOF患者分为Gln组和对照组,每组30例。Gln组给予Gln1.5ml/kg联合常规治疗[热量104～125kJ/(kg·d),其中脂肪供热40%,氮供给量为0.2～0.25g/(kg·d)的完全胃肠外营养(TPN)支持];对照组仅给予常规治疗,连续7d。对照分析营养指标、免疫指标、并发症及临床结果。结果所有患者营养指标(血清白蛋白、前清蛋白、转铁蛋白)和免疫指标(IgG、IgA、IgM)明显减低,处于负氮平衡。第8天时,Gln组营养指标明显高于治疗前及对照组(P<0.05),负氮平衡得到纠正(P<0.05),而对照组仅血清白蛋白、转铁蛋白较治疗前增高(P<0.05),仍处于负氮平衡(P>0.05)。Gln组免疫指标明显高于治疗前及对照组(P<0.01),而对照组仅IgG高于治疗前(P<0.01)。对照组14例(46.7%)出现了血清胆红素、丙氨酸转氨酶和血糖的升高,而Gln组仅4例(13.3%)增高(P<0.05),至14d时对照组病死率为36.7%,而Gln组仅为10%,两者相比差异有显著性(P<0.05)。结论应用Gln的肠外营养能够明显纠正MOF患者的营养代谢障碍,纠正负氮平衡,增强免疫功能,降低并发症及病死率。     

青少年特发性胸椎侧凸患者的脊髓偏移及临床意义

[目的]揭示青少年胸椎特发性侧凸患者椎管内脊髓的偏移，明确其变化趋势，并探讨其可能的发生机制和临床意义。[方法]本研究包括39名以右胸弯为主弯的青少年特发性脊柱侧凸（adolescent idiopathic scoliosis，AIS）患者。测量主胸弯节段内（T5-12）椎管内脊髓与凸凹侧椎弓根之间的距离，计算脊髓的偏移，揭示其变化趋势，并分析顶椎区脊髓偏移与主胸弯Cobb＇s角和顶椎相对偏移之间的相关性。[结果]在T5-12节段椎管内，脊髓与凸侧椎弓根之间的距离显著大于与凹侧椎弓根之间的距离（P〈0．05），即脊髓向凹侧椎弓根偏移，且以顶椎区最为显著，而逐渐向两侧端椎区递减。顶椎区脊髓偏移与主胸弯Cobb’s角和顶椎相对偏移存在显著的正相关（相关系数分别为0．631和0．546）。[结论]胸椎特发性脊柱侧凸患者存在侧凸节段椎管内脊髓偏移的现象，且以顶椎区最显著。研究结果提示脊髓偏移可能与脊椎偏移后凹侧脊神经的牵拉有关，而且凹侧置钉的风险高于凸侧。     

神经干细胞静脉移植治疗脊髓损伤的实验研究

[目的]观察神经干细胞静脉移植对损伤大鼠脊髓功能的治疗作用。[方法]取孕14—16dSD胎鼠的脑室下区组织，体外培养后鉴定细胞。制作脊髓全切模型，伤后1周将Brdu标记好的神经干细胞通过尾静脉注射移植到大鼠体内，移植后及8周行皮层体感诱发电位（CSEP）检测和BBB功能评分，并留损伤脊髓处作病理切片及免疫组化染色。[结果]（1）移植后8周BBB评分损伤组、移植组都有所恢复，但都未达到正常水平，移植组恢复较好；（2）模型制作后，CSEP波均消失，细胞移植后8周移植组的波形有不同程度的恢复，但潜伏期延长；（3）移植组大鼠脊髓损伤处存在大量Brdu染色阳性细胞，表明移植的细胞在体内可到达损伤脊髓处并能存活；脊髓损伤部位NF-200及GFAP染色阳性的细胞表明移植的细胞可以分化为具有神经元和胶质细胞特性的细胞。[结论]静脉移植的神经干细胞能到达损伤区代替受损的神经元及神经胶质细胞，使损伤的脊髓功能得到一定程度的恢复。     

人脐血内皮祖细胞对脐动脉内膜损伤的修复作用

目的 体外分离培养人脐带静脉血内皮祖细胞(EPCs),观察EPCs对损伤脐动脉的修复作用.方法 采用磁珠分选法(MACS)从人脐血中分离培养EPCs,用流式细胞术、免疫细胞化学和免疫荧光检测进行鉴定;牵拉钳夹损伤法制备去内膜脐动脉段,与EPCs共孵育7 d后,通过病理切片、免疫组织化学和图像分析技术评价EPCs对动脉损伤的修复效果.结果 成功从人脐血中分离培养EPCs,流式细胞术分析结果 为培养7 d后CD133+细胞＞90%;CD34、vWF因子相关抗原免疫染色均为阳性.EPCs移植组新生内膜厚度(43.5±5.5)ìm显著低于对照组内膜厚度(90.7±12.7)ìm,(t=-28.88,P＜0.01);EPCs移植组的再内皮化程度(77.8±0.1)%明显高于对照组(52.2±0.1)%,(t=21.86,P＜0.01).结论 成功从人脐血中培养出EPCs,人脐血EPCs可修复内皮损伤血管.     

经前后路联合手术治疗L5S1椎体结核

目的 探讨一期经前路病灶清除后路植骨内固定治疗L5S1椎体结核的临床疗效.方法 对40例L5S1椎体结核患者,采用一期经前路病灶清除椎体间植骨加后路椎弓根螺钉内固定植骨融合治疗.结果 所有患者随访6～24个月,35例植骨融合良好,5例融合欠佳;有1例出现尿路感染.结论 一期经前路病灶清除椎体间植骨加后路椎弓根螺钉内固定椎板植骨治疗L5S1椎体结核能在彻底清除病灶的前提下保证脊柱的稳定性,提高植骨块的融合率和缩短患者卧床时间.     

腰椎间盘突出症合并椎管狭窄23 例治疗分析

目的探讨经后路椎间盘镜手术治疗合并腰椎管狭窄症的椎间盘突出症的临床应用。方法采用后路椎间盘镜进行单侧开窗减压术。通过术前标记腰椎正侧位片定位，于定位棘突间隙后正中偏患侧作长约1.5 cm小切口，逐级扩张后置入工作通道管，钻除部分椎板，置入内窥镜，于电视监视器下显露椎板、增生内聚的关节突、肥厚的黄韧带及突出的椎间盘髓核组织，彻底解除其对硬脊膜、神经根的压迫。结果本组共治疗合并腰椎管狭窄症的腰椎间盘突出症23例，平均随访7个月，按Prolo标准评定，治愈20例，有效2例，无效1例。结论本术式在严格掌握适应证前提下对合并腰椎管狭窄症的腰椎间盘突出症患者效果明显。     

Changes in T .lymphocyte subsets after severe traumatic brain inJury

BACKGROUND： Besides local changes of cranial parenchymal cells, hemorrhage, etc., severe traumatic brain injuries also cause the changes of total body fluid and various functions, and the changes of lymphocytes and T lymphocyte subsets should be paid more attention to. OBJECTIVE： To reveal the changing laws of T lymphocyte subsets after severe traumatic brain injury, and compare with mild to moderate brain injury. DESIGN： A comparative observation. SETTINGS： Department of Neurosurgery, Longgang District Buji People＇s Hospital of Shenzhen City; Central Laboratory of Shenzhen Hospital of Prevention and Cure for Chronic Disease. PARTICIPANTS： All the subjects were selected from the Department of Neurosurgery, Longgang District Buji People＇s Hospital of Shenzhen City from August 2002 to August 2005. Thirty patients with severe brain injury, whose Glasgow coma score （GCS） was ≤ 8 points, were taken as the experimental group, including 21 males and 9 females, aging 16 - 62 years. Meanwhile, 30 patients with mild traumatic brain injury were taken as the control group （GCS ranged 14- 15 points）, including 18 males and 12 females, aging 15 -58 years. All the subjects were in admission at 6 hours after injury, without disease of major organs before injury Informed consents were obtained from all the patients or their relatives. METHODS： （1） The T lymphocytes and the subsets in peripheral blood were detected with immunofluorescent tricolor flow cytometry at l, 3, 7 and 14 days after injury in both groups. （2） The conditions of pulmonary infections were observed at 4 days after injury. The differences of measurement data were compared with the t test. MAIN OUTCOME MEASURES： Changes of T lymphocytes subsets at 1 - 14 days after severe and mild or moderate traumatic injury. RESULTS： Finally, 28 and 25 patients with mild to moderate traumatic brain injury, whereas 25 and 21 patients with severe traumatic brain injury were analyzed at 7 and 14 days respectively, and the missed ones died due to the development of disease. （1） Changes of T lymphocyte subsets： At 1 and 3 days after injury, CD3, CD4, CD8, CD4/CD8 began to decrease, whereas CD8 increased in the experimental group, which were very significantly different from those in the control group （t =2.77 - 3.26, P 〈 0.01）, and began to recover at 7 days, which were significantly different from those in the control group （t = 2.06 - 2.24, P 〈 0.05）, and generally recovered to the normal levels at 14 days （P 〉 0.05）. （2） Conditions of pulmonary infections： At 4 days after injury, the rate of pulmonary infection was significantly different between the experimental group and control group [73% （22/30）, 0, x2=37.29, P 〈 0.01]. CONCLUSION： Patients with severe traumatic brain injury suffer from damages of cellular immune function at early period （within 7 days）, and they are easily to be accompanied by pulmonary infections.