Ⅰ型神经纤维瘤病成骨细胞生物学特性的研究

目的检测神经纤维瘤蛋白在Ⅰ型神经纤维瘤病(type1neurofibromatosis,NF1)脊柱侧凸患者成骨细胞中的表达,观察NF1脊柱侧凸患者成骨细胞的生物学特性。方法先天性脊柱侧凸患者10例,NF1脊柱侧凸患者8例,两组年龄和Cobb角相近。脊柱后路手术取髂骨松质骨,采用植块法进行成骨细胞培养。取第二代成骨细胞分别检测其增殖活性、碱性磷酸酶、Ⅰ型胶原和骨钙素等成骨细胞特异性分化指标,并采用免疫沉淀和Westernblot法检测神经纤维瘤蛋白在两组成骨细胞中的表达。结果NF1脊柱侧凸患者成骨细胞中神经纤维瘤蛋白表达水平明显低于先天性脊柱侧凸患者(灰度积分比值分别为1.05±0.06和2.59±1.40,P=0.002);碱性磷酸酶、Ⅰ型胶原和骨钙素水平均明显低于先天性脊柱侧凸患者(44.69IU/mg和51.38IU/mg,P=0.019;226.34ng/mg和249.93ng/mg,P=0.014;7.41ng/mg和8.87ng/mg,P=0.049)。NF1脊柱侧凸患者成骨细胞显示相对活跃的增殖活性(增殖倍数分别为3.34和2.70,P=0.049)。结论NF1脊柱侧凸患者成骨细胞神经纤维瘤蛋白表达降低的同时,其功能存在明显缺陷。成骨细胞功能缺陷可能是骨骼营养不良性改变和骨密度降低等各种骨骼异常共同的基础。     

青少年特发性脊柱侧凸患者体感诱发电位的特点及与Cobb角的关系

Objective To investigate the abnormality of somatosensory evoked potentials (SEPs) in adolescent idiopathic scoliosis (AIS) with different curve magnitudes, and to explore its effect on the etiopathogenesis of AIS. Methods Posterior tibial nerve SEPs were evaluated on 489 young operative treated AIS patients with a Cobb angle > 40°and 45 age-matched healthy control individuals. Absence of SEPs waveforms, prolongation of peek latency or asymmetrical peek latency were defined as pathological change. Base on the control reference, the incidence of pathological SEPs was determined in AIS group. The association of abnormal SEPs and curve severity for MS patients was also assessed. Results Peek latency corrected for body height was slightly longer in AIS patients than in controls, however, with no significant difference. Interside difference of latency was significantly larger in AIS patients. Abnormal SEPs were found in 166 of 489 AIS patients. Among these, 17 (10.2%) showed absent waveforms, 50 (30.1%) had unilateral latency prolongation, 38 (22.9%) had bilateral latency prolongation, and 120 (72.3%) showed significant interside difference. Statistical analysis failed to show a correlation between abnormal SEPs and the curve severity of spinal deformity. Conclusion Disorder of somatosensory pathways does exist in a subgroup of AIS patients, and it might be a primary factor other than secondary change, and could play an import role in the etiopathogenesis of AIS.     

后路经椎弓根儿童半椎体全切除术的近期疗效评估

Objective To evaluate the early outcomes of children with congenital scoliosis treated by one-stage transpedicular hemivertebra resection. Methods From July 2005 to June 2006, 27 consecutive cases of congenital scoliosis managed by one-stage transpedicular hemivertebra resection with instrumentation were investigated retrospectively. There were 11 girls and 16 boys, with a mean age of 5. 5 years at surgery (range 1.3-10.0 years). Location of the hemivertebra was in the thoracic spine in 12 cases and in the lumbar spine in 15 cases. Radiographic evaluations were performed on the preoperative, postoperative, and latest follow-up standing posteroanterior and lateral radiographs. Results The average operation time was 4 hours (range 3-6 hours), and the mean blood loss during operation was 750 ml (range 300-2200 ml). The mean fusion level was 2 to 7 segments, average 4. 4 segments. The average follow-up period was 16 months (range 12-34 months). Mean Cobb angle of the total main curve was 40.0° before surgery, 12. 6° after surgery, and 15. 2° at latest follow-up. Mean Cobb angle of the segmental main curve was 35. 6° before surgery, 11.6° after surgery, and 12. 1° at latest follow-up. The trunk shift was improved from 16. 5 mm before operation to that of 7. 5 mm after the operation and 7. 6 mm at the latest follow-up. Compensatory cranial curve improved from 19.4° before surgery to 8.9° after surgery, and compensatory caudal curve improved from 26. 3° to 12. 8°. The angle of segmental kyphosis was 26.4° before surgery and 14. 6° after surgery in cases with thorcic hemivertebrae, and averaged 11. 2° before surgery and 3. 9° after surgery in cases with lumbar hemivertebrae. Peri-operative complications included two pedicle screws malpositioning and one case with pelvic tilt. There was no neurological complication. Conclusion One-stage transpedicular hemivertebra resection with instrumentation has a good capability of correcting deformity on the frontal and sagittal planes, which is available in children with middle or lower thoracic or lumbar hemivertebrae.     

青少年特发性脊柱侧凸椎弓根螺钉的误置模式及危险因素

目的 评估青少年特发性脊柱侧凸(AIS)椎弓根螺钉不良置入的模式并分析其原因.方法 收集从2008年7月至9月行后路椎弓根螺钉固定的70例AIS患者的临床资料,其中男性12例,女性58例;年龄12～19岁,平均(14.5±2.7)岁;术前Cobb角40°～125°,平均62.0°±18.2°.术中根据解剖标志徒手置入椎弓根螺钉,所有患者术后均行CT检查.在PACS系统上通过PacsClient软件测量螺钉穿破椎弓根内、外壁以及椎体前缘的距离.定义穿破任一壁超过2 mm为不良置钉.不良置钉中穿破内壁超过4 mm或钉尖使主动脉变形定义为高危置钉.对数据进行统计学分析,探讨不良置钉的危险因素.结果 共置入椎弓根螺钉1030枚,胸椎773枚,腰椎257枚.不良置钉108枚(10.5%),其中穿破外壁35枚,穿破内壁56枚,穿破椎体前缘33枚(其中有16枚既穿破了椎体前缘又穿破了外壁).高危置钉16枚(1.6%).顶椎、顶椎近端第5节段和顶椎远端第4节段的不良置钉率高于其他节段,其中顶椎左侧和顶椎近端第5节段右侧不良置钉率均高于对侧.顶椎区高危置钉率最高,达4.8%.Cobb角>90°组不良置钉率高于Cobb角40°～90°组,椎体旋转Ⅲ～Ⅳ度组不良置钉率高于椎体旋转0～Ⅱ度组.结论 AIS不良置钉集中在顶椎、顶椎近端第5节段和顶椎远端第4节段3个区域,高危置钉多发生在顶椎区,危险因素包括Cobb角大小、旋转程度以及与顶椎的距离.     

腐胺提高高龄小鼠卵母细胞质量及其机制的研究

目的本研究探讨腐胺是否通过抗氧化应激的机制提高体外培养的高龄卵母细胞的质量。方法从9月龄C56/B6J雌鼠获取GV期卵丘-卵母复合体(COC),分为对照组、0.5mmol/L腐胺处理组、0.5mmol/Lα-二氟甲基鸟氨酸(DFMO,腐胺抑制剂)组。三组COC体外培养液16～18h,收集MⅡ期卵母细胞。计算各组高龄卵母细胞成熟率,检测卵母细胞mtDNA拷贝数,JC-1荧光分析染色线粒体膜电位,并分析卵母细胞内氧自由基和超氧化物歧化酶1(SOD_1)的表达。结果腐胺组卵母细胞成熟率显著高于对照组[(87.57±1.70)%vs.(66.02±4.05)%,P0.01]。DFMO处理组线粒体DNA拷贝数显著高于对照组(P0.05)。与对照组相比,腐胺组卵母细胞内氧化应激水平显著下降(P0.01);DFMO组氧化应激水平显著升高(P0.01)。与对照组比较,腐胺组卵母细胞线粒体膜电位显著升高(P0.01),而DFMO组显著下降(P0.05)。与对照组比较,腐胺组卵母细胞内SOD_1蛋白表达显著增高(P0.05),DFMO组显著下降(P0.05)。结论体外培养的高龄卵母细胞,腐胺通过抗氧化应激、改善线粒体功能提高卵母细胞质量和胚胎发育潜能,具有潜在的临床应用价值。     

脊髓空洞源性脊柱侧弯椎旁肌乙酰胆碱受体向突触后膜外细胞膜面的扩散及意义

目的通过测定椎旁肌神经肌肉接头乙酰胆碱受体向突触后膜以外细胞膜面的扩散，判断脊髓空洞源性脊柱侧弯椎旁肌失神经支配的存在与否，并探讨与脊柱侧弯发生发展的关系。方法患儿分为3组：空洞-侧弯组25例，青少年特发性脊柱侧弯组16例，非脊柱侧弯组10例。切取脊柱左右两侧的骶棘肌组织，在-30℃下冰冻切片。乙酰胆碱受体用结合Texas Red-X的α-银环蛇毒素（红色荧光）染色，乙酰胆碱酯酶被结合Alexa Fluor 488的抗体（绿色荧光）染色。在共聚焦荧光显微镜下观察和拍照，红色荧光的强度和显影部位均明显大于绿色荧光为结果阳性。结果所有标本均成功双标荧光染色。非脊柱侧弯组和青少年特发性脊柱侧弯组标本中，脊柱左右两侧骶棘肌的检测结果均为阴性。空洞-侧弯组中14例出现阳性结果，阳性率为56．0％，其中凸侧阳性率为44．0％，凹侧阳性率为28．0％。在小脑扁桃体下移Ⅰ°与Ⅱ°以上组间，张力性空洞与非张力性空洞组间，空洞长度≤10个椎体与〉10个椎体组间，Cobb角≤45°与Cobb角〉45°组间，阳性率均无显著性差异（P〉0．05）。结论脊髓空洞源性脊柱侧弯患儿椎旁肌乙酰胆碱受体发生了向突触后膜以外细胞膜面的扩散，提示存在椎旁肌失神经支配现象，椎旁肌肌力不平衡可能为导致此类脊柱侧弯的发生机制之一。     

滑膜微循环相关疾病研究进展

<正>滑膜是关节囊内层薄层组织膜,可分泌滑液营养软骨并减少软骨间相互摩擦,发挥软骨保护作用。滑膜表面分布着丰富的有孔毛细血管,隶属微循环范畴。微循环是体循环末端血管网络,由直径小于150μm的微血管组成^[1],具有器官组织特异性,通过微血流动力学调控血流、氧流灌注分布、营养物质输送及二氧化碳和代谢产物清除。     

青少年特发性脊柱侧凸患者体感诱发电位的特点及与Cobb角的关系

Objective To investigate the abnormality of somatosensory evoked potentials (SEPs) in adolescent idiopathic scoliosis (AIS) with different curve magnitudes, and to explore its effect on the etiopathogenesis of AIS. Methods Posterior tibial nerve SEPs were evaluated on 489 young operative treated AIS patients with a Cobb angle > 40°and 45 age-matched healthy control individuals. Absence of SEPs waveforms, prolongation of peek latency or asymmetrical peek latency were defined as pathological change. Base on the control reference, the incidence of pathological SEPs was determined in AIS group. The association of abnormal SEPs and curve severity for MS patients was also assessed. Results Peek latency corrected for body height was slightly longer in AIS patients than in controls, however, with no significant difference. Interside difference of latency was significantly larger in AIS patients. Abnormal SEPs were found in 166 of 489 AIS patients. Among these, 17 (10.2%) showed absent waveforms, 50 (30.1%) had unilateral latency prolongation, 38 (22.9%) had bilateral latency prolongation, and 120 (72.3%) showed significant interside difference. Statistical analysis failed to show a correlation between abnormal SEPs and the curve severity of spinal deformity. Conclusion Disorder of somatosensory pathways does exist in a subgroup of AIS patients, and it might be a primary factor other than secondary change, and could play an import role in the etiopathogenesis of AIS.     

术前大质量Halo-股骨髁上牵引在治疗僵硬型特发性脊柱侧凸中的应用

目的 探讨术前快速大质量Halo-股骨髁上牵引在治疗僵硬型特发性脊柱侧凸中的应用价值.方法 对2003年7月至2006年5月行术前快速大质量Halo-股骨髁上牵引治疗且临床资料完整的16例僵硬型特发性脊柱侧凸患者进行回顾性分析.其中男性4例,女性12例;年龄12～20岁,平均16岁,术前冠状面Cobb角平均111°,胸椎后凸(T_(5～12))平均64°.术前行Halo-双侧股骨髁上牵引,最大质量(可达体质量的1/2)牵引2～3周后,行后路矫形内固定融合术.对术前Bending像、牵引后和术后侧凸纠正率进行比较.结果 随访12～60个月(平均36个月).最大牵引质量平均19 kg,为体质量的35%～56%.1例患者在牵引12 d后出现右侧臂丛神经麻痹,减轻牵引质量后逐渐恢复.矫形术后无瘫痪、死亡等并发症,无呼吸衰竭的发生.术前平卧位Bending像X线片的侧凸纠正率平均18.7%,Halo-股骨髁上牵引后侧凸纠正率平均达31.9%.与Bending像相比,牵引后侧凸纠正率提高了13.2%,差异具有统计学意义(P<0.05).后路矫形术后侧凸矫正率平均为48.6%,胸椎后凸矫正率平均为51.9%.末次随访时冠状面矫正丢失率平均为2.0%,矢状面矫正丢失率平均为5.8%.结论 术前短时间大质量Halo-股骨髁上牵引结合后路广泛松解可改善僵硬型特发性脊柱侧凸畸形矫形率,但应注意与牵引相关的并发症.     

脊髓空洞源性脊柱侧凸椎旁肌肌纤维分型及群化的对比研究

目的分析脊髓空洞源性脊柱侧凸患者椎旁肌肌纤维分型及群化的特征,探讨其与脊柱侧凸发病的关系.方法病例分为三组：脊髓空洞源性脊柱侧凸（SS）组20例,青少年特发性脊柱侧凸（AIS）组16例,非脊柱侧凸（NS）组10例.术中切取两侧椎旁肌组织,统计三组的椎旁肌肌纤维分型及群化,并进行对比.结果SS组凸侧Ⅰ型肌纤维比例和横截面积均小于凹侧,差异无统计学意义;AIS组凸侧Ⅰ型肌纤维比例和横截面积均显著高于凹侧;NS组左、右侧之间差异无统计学意义.各组间比较显示：（1）凸侧Ⅰ型肌纤维所占比例,SS组和AIS组均高于NS组;凹侧Ⅰ型肌纤维所占比例,SS组高于AIS组和NS组.（2）凸侧Ⅰ型肌纤维横截面积,SS组低于AIS组;凹侧Ⅰ型肌纤维横截面积,AIS组低于NS组.（3）凸侧Ⅱ型肌纤维横截面积,SS组＜AIS组＜NS组;凹侧Ⅱ型肌纤维横截面积,SS组和AIS组均低于NS组.SS组中11例观察到椎旁肌Ⅰ型肌纤维群化,凹侧群化程度大于凸侧,但差异无统计学意义.AIS组中8例观察到凸侧Ⅰ型肌纤维群化,无凹侧肌纤维群化和Ⅱ型肌纤维群化.NS组左、右两侧均未见肌纤维群化.结论SS组患者椎旁肌肌纤维分型及群化的特征与AIS组明显不同,提示椎旁肌存在原发性失神经支配,可能与脊柱侧凸的发生、发展有一定相关性.