神经导管修复周围神经损伤的研究进展

随着神经修复技术特别是显微外科的发展，神经损伤修复的质量有了进一步的提高；利用神经导管桥接神经断端以实现修复周围神经损伤是目前的一个研究热点。本综述了神经导管修复周围神经损伤的发展历史，分析比较了非神经组织、非生物降解材料、可生物降解材料神经导管在神经损伤修复中的效果，讨论了导管的形态及导管内微环境对神经再生的影响。     

Infected infradiaphragmatic retroperitoneal extralobar pulmonary sequestration: a case report

Infradiaphragmatic extralobar pulmonary sequestration is an extremely rare congenital malformation. It is more frequently diagnosed in the antenatal period due to routine ultrasonic examination of the fetus or in the first 6 months of life, though on rare occasions it is discovered incidentally in adults. A 32-yr-old man presenting with epigastric discomfort and fever was referred. Computed tomographic scanning showed that a 16-cm, multiseptated, dumbbell-shaped, huge cystic tumor was located beneath the diaphragm. On the next day, 850 mL of thick yellowish pus was drained by sonography-guided fine needle aspiration for the purpose of infection control and diagnosis, but no microscopic organisms were found in repeated culture studies. Surgical removal of the cyst was performed through thoracoabdominal incision and most of these pathologic lesions were removed but we could not find the feeding arteries or any fistulous tract to surrounding structures. Histopathologic study revealed that it was extralobar pulmonary sequestration and culture study showed that many WBC and necrotic materials were found but there were no microorganisms in the cystic contents. We report the first case of an infected infradiaphragmatic retroperitoneal extralobar sequestration which was administered a staged management and achieved an excellent clinical course.     

异种脱细胞真皮基质修复膜在喉肿瘤切除术后黏膜缺损修复中的临床研究

目的探讨应用异种（牛）脱细胞真皮基质修复膜修复喉部手术后黏膜缺损的I临床使用价值。方法对20例喉癌术后的黏膜缺损应用异种脱细胞真皮基质修复膜1期修复，其中声门区癌18例，其中T2NoMo8例，T3N1MO5例，T3N2MO4例，T4N2MO1例。声门上区癌2例，T3N1MO1例，T3N2MO1例。结果20例患者黏膜缺损修复均1期愈合。患者术后3-6个月应用电子喉镜复查，修复膜均已替代缺损黏膜组织，局部功能恢复；20例喉癌中6月生存率100％（11／11）；一年生存率100％（3／3），其余9例术后不足6月，正在观察之中。讨论异种脱细胞基质修复膜为修复喉肿瘤术后的黏膜缺损提供了一种新的方法，手术操作简便，有很好的临床应用价值。     

皮肤扩张术修复头皮合并颅骨缺损

目的探讨头皮合并颅骨缺损的修复方法。方法对2000年至2007年治疗的6例头皮合并颅骨缺损患者，采用皮肤扩张术修复头皮，钛网修补颅骨缺损。结果6例患者中，1例患者出现皮瓣下积液，经抽吸、加压包扎后愈合；其余病例未出现感染、血肿及皮瓣坏死等并发症，外观满意。结论皮肤扩张术和钛网联合应用是修复头皮合并颅骨缺损的较好方法。     

不同骨移植材料修复骨缺损的初步实验研究

目的对比观察不同骨移植材料对兔桡骨节段性骨缺损的修复效果。方法48只新西兰大白兔采用桡骨15mm节段性骨缺损模型,随机分为4组,每组12只,A组植入深冻异体骨复合自体骨髓,B组植入深冻异体骨,C组植入自体骨,D组植入新鲜同种异体骨。术后不同阶段分别行组织学、透射电镜及X线检测。结果四组骨缺损均有成骨现象发生,骨生成、骨连接情况A、C组优于B组,B组优于D组;A、C组细胞增生活跃、核呈分裂相、胞质丰富、核膜光整、细胞器丰富,同比均优于B、D组;骨缺损愈合时间A、C组为8-10周,B组为12周,D组骨缺损在术后12周仍未愈合。结论提示兔深冻异体骨复合自体骨髓具有良好的组织相容性及成骨作用,其取代自体骨植骨修复骨缺损具有可能性。     

颅骨缺损状态下区域血流速度变化

目的 :探讨颅骨缺损病理状态下区域血流速度变化。方法 :应用TCD测定不同缺损面积的颅骨缺损病人术前患侧及健侧和术后患侧ICA、ACA、MCA、PCA、BA平均血流速度。结果 :≥ 35cm2 病人患侧颅内血管平均血流速度高于正常 ,而健侧颅内血管平均血流速度正常。 <35cm2 病人患侧及健侧颅内血管平均血流速度均在正常范围。结论 :颅骨缺损病理状态下常可引起区域血流速度改变 ,血流速度改变是指导颅骨缺损修补的重要依据     

Mutations of ZFPM2/FOG2 gene in sporadic cases of tetralogy of Fallot

Two out of 47 patients with sporadic tetralogy of Fallot (TOF), the most common cyanotic conotruncal heart defect (CTD), showed heterozygous missense mutations of the ZFPM2/FOG2 gene. Knockout mice carrying mutations in the ZFPM2/FOG2 gene have similarly been found to exhibit TOF. While both mutant ZFPM2/FOG2 proteins, E30G (c.88A>G) and S657G (c.1968A>G), retain the ability to bind the partner protein GATA4 and repress GATA4 mediated gene activation, the S657G, but not the E30G, mutation is subtly impaired in this function. ZFPM2/FOG2 gene mutations may contribute to some sporadic cases of TOF.     

Duchenne muscular dystrophy and idiopathic hyperCKemia segregating in a family

A 7-month-old boy with gross motor delay and failure to thrive presented with rhabdomyolysis following an acute asthmatic episode. During hospitalization an electrocardiographic conversion to a Wolff-Parkinson-White type 1 (WPW) pattern took place. Duchenne muscular dystrophy (DMD) was suspected based on elevated creatine kinase (CK) serum levels, muscle biopsy, and family history. The diagnosis was confirmed by molecular analysis, which documented a deletion corresponding to cDNA probe 1-2a in the dystrophin gene, in the propositus and in an affected male cousin of his mother. “Idiopathic” hyperCKemia was found in the propositus, his father, and 5 of his relatives. We suggest that the unusually early and severe manifestations of DMD in this patient may be related to the coincidental inheritance of the maternal DMD gene and of a paternal gene, causing hyperCKemia. © 1995 Wiley-Liss, Inc.     

远端蒂腓肠神经营养血管皮瓣与肌皮瓣的临床应用与改进

目的:报道应用远端蒂腓肠神经营养血管皮瓣,肌皮瓣修复小腿下段及足踝部软组织缺损的可行性安全性和临床效果。方法:对42例以远端蒂腓肠神经营养血管(肌)皮瓣修复小腿下段及足踝部不同原因所致软组织缺损病例进行总结分析。本组男36例,女6例;年龄最大75岁、最小6岁;皮瓣最大面积17.0cm×15.0cm,最小6.0cm×5.0cm,其中12例皮瓣面积在10.0cm×10.0cm以上;6例设计为肌皮瓣(腓肠肌外侧头),肌瓣最大为10.0cm×7.0cm×2.0cm,最小为6.0cm×5.0cm×1.0cm。结果:所有病例术后皆出现不同程度的皮瓣肿胀,暗道较明道者明显。2例大皮瓣经行小隐静脉远端结扎仍出现肿胀、色暗,皮瓣近侧1/3坏死。皮瓣边缘坏死3例,换药治愈。部分坏死需行植皮者3例。36例术后伤口I期愈合,骨外露软件组织缺损覆盖修复满意,6例II期愈合,其中糖尿病,地中海贫血各一例。结论:(1)远端蒂腓肠神经营养血管皮瓣转位修复小腿下1/3及足踝部缺损创面,极有临床实用价值;(2)设计切取腓肠神经营养血管肌皮瓣修复小腿及足踝填充感染创腔是可行的;(3)但对其皮瓣及所携带的肌瓣究竟切取多大面积是安全的、肌瓣的血运机理以及远端蒂筋膜皮瓣中小隐静脉干是否结扎,何处结扎等问题仍有待进一步研究。     

Velo-cardio-facial syndrome and DiGeorge sequence with meningomyelocele and deletions of the 22q11 region

Approximately 5% of children with neural tube defects (NTDs) have a congenital heart defect and/or cleft lip and palate. The cause of isolated meningomyelocele, congenital heart defects, or cleft lip and palate has been largely thought to be multifactorial. However, chromosomal, teratogenic, and single gene causes of combinations of NTDs with congenital heart defects and/or cleft lip and palate have been reported. We report on 3 patients with meningomyelocele, congenital heart defects, and 22q11 deletions. Two of the children had the clinical diagnosis of velo-cardio-facial syndrome (VCFS); both also have bifid uvula. The third child had DiGeorge sequence (DGS). The association of NTDs with 22q11 deletions has not been reported previously. An accurate diagnosis of the 22q11 deletions is critical as this micro-deletion and its associated clinical problems is transmitted as an autosomal dominant trait due to the inheritance of the deletion-bearing chromosome. We recommend that all children with NTDs and congenital heart defects, with or without cleft palate, have cytogenetic and molecular studies performed to detect 22q11 deletions. © 1994 Wiley-Liss, Inc.