Objective The article was to analyze the features of gene mutation and clinical phenotype in Alport syndrome. Methods Next-generation sequencing was applied to capture the exons of COL4A3, COL4A4, COL4A5 genes in 30 cases of children with suspected or confirmed diagnosis of Alport syndrome and Sanger method was used to identify gene mutations of related family mem-bers.Provean database was applied in protein function prediction.We collected and analyzed clinical data of AS patients on the basis of gene mutation. Results All 30 children were diagnosed with AS by gene sequencing, among whom 4 boys were autosomal reces-sive inheritance, 16 boys and 10 girls were X-linked Alport syndrome.Next-generation sequencing detected 35 different gene mutations of COL4A3, COL4A4, COL4A5, including 19 missense mutations, 2 synonymous mutations, 4 splice-site mutations, 3 truncating mu-tations, 2 insertion mutations, 4 deletion mutations and 1 compound mutations.It was observed by Sanger sequencing that 20 mutations were inherited from the mother, 8 from the father, homozygous mutation in 1 propositus from the parents respectively, 8 novel mutations and 1 with unidentified source.All the 30 children had an onset of hematuria or proteinuria, 17 cases had a positive family history, 1 case had hearing loss, and no pathogenesis or renal insufficiency was found in the children.Renal biopsy was performed on 23 children, 13 minimal change disease ( MCD) and 10 mesangial proliferative glo-merulonephritis ( MsPNG) by light microscope.Extensive lamination and split of glomerular basement membrane dense layers were found in 9 children by electron microscope. Conclusion XLAS ac-counts for most AS patients and missense mutation is the main type in pathogenic mutations.Altogether, 31 mutations without disease notification were found.Most of children showed MCD in renal biopsy, with atypical electron microscope manifestations and rare extra renal manifestations.

Hereditary glomerular disease is an important part of kidney diseases .In recent years , hereditary glomerular disea-ses had a high incidence and poor prognosis .Thus, the studies involving hereditary glomerular diseases such as Alport syndrome , he-reditary nephritis syndrome and thin basement membrane disease , etc.have significant implications .This review mainly focuses on the pathogenesis , clinical features , diagnosis and treatment of Alport syndrome and hereditary nephritis syndrome .

Objective To evaluate the results of teaching in both Chinese and English with 7-year-program clinical medicine majors and analyze the influencing factors.Methods Data from a questionnaire survey of 25 students exposed to bilingual teaching were analyzed.Results The students generally approved of the model of bilingual teaching,which was contributory to improving their proficiency in specialized English.However,uneven English levels on the part of teachers and students and imperfect materials and methods as well as discontinuity affected the overall results of bilingual teaching and to some extent the students' mastery of specialized knowledge.Conclusion Bilingual teaching with 7-year-program clinical medicine majors is a must,and yet persistent efforts need to be made in enhancing the teachers' English level,compiling proper textbooks and improving teaching methods so as to genuinely better the results of bilingual teaching.

Objective To observe and evaluate the expression and significance of Nogo-66 receptor (NgR) mRNA in adult rats′ optic nerve. Methods Optic and sciatic nerves of 8 adult rats were used to make the sections, which were divided into 3 groups: optic-nerve experimental group, sciatic-nerve control group, and optic-nerve negative control group. In situ hybridization was used to observe the expression of NgR mRNA in optic nerve and sciatic nerve. Results The expression of NgR mRNA in the 8 rats was positive in optic nerve and negative in sciatic nerve. The positive signals were arranged along the long axis of optic nerve. Conclusion The expression of NgR mRNA is positive in optic nerve while negative in sciatic nerve in adult rats, which suggests that the positive expression and distribution of NgR may be related to the poor regenerate ability of optic nerves.

ObjectiveTo investigate the effects of chitosan/PVA nerve conduits which used for repairing sciatics nerve defect in rats.MethodsTwenty-seven rats were divided into 3 groups randomly,with 9 rats in each group. Firstly, the 15mm defects in the left sciatic nerves were made in the rats and were respectively repaired with chitosan/PVA conduits graft (group A), the silicon conduits graft (group B),and autografts (group C). At 12 weeks after the operations, the left sciatic nerves were taken out, and the comparative evaluation was made on the repairing effects by wet weight of gastrocnemius and soleus muscles, histological examination,computerized imaging analysis and True Blue retrograde tracing. ResultsThe wet weight of gastrocnemius and soleus muscles showed no significant difference between the chitosan/PVA graft and autograft groups (P ＞ 0.05). The wet weight of gastrocnemius and soleus muscles in significant difference between the chitosan/PVA graft and the silicon group at 12 weeks after the operation(P ＜ 0.05). The nerve fiber density showed no statistically significant differences between the chitosan/PVA and autograft groups(P＞ 0.05).The regenerative nerve fiber in group B had normal morphological and structural characters under transmission electron microscope.True Blue-labeled neuron cell bodies were found within both anterior horn of gray matter in the spinal cord and dorsal root ganglions (DRGs) ipsilateral to the operated side of the tested rats on illumination with ultra-violet light 1 week after the injection of True Blue.Conclusion Chitosan/PVA nerve conduit can effectively promote the nerve regeneration and myelinization of rat sciatic nerve, which is expected to substitute for autograft to repair nerve defects succesfully.

BACKGROUND:Tissue engineered skin has been used to repair defect skin, but it is not as good as to completely replace permanent skin. OBJECTIVE: To explore the biological characteristics and clinical application of various types of tissue engineered skin. METHODS: Pubmed data (1990-01/2005-12) was retrieved by computer with the key words of "tissue-engineered skin". CNKI database (2002-01/2007-12) was retrieved with the same key words. RESULTS AND CONCLUSION: Totally 226 literatures were primarily selected. After reading titles and Abstracts, studies addressing irrelative objective or repetitive content were excluded, and 11 literatures were included. The aim of skin tissue engineering is to create skin substitute that close to human.skin in histology and functional characteristics. There were many mature tissue engineered skin in clinic, and various artificial skins were developed. Their structure was similar to human, but only had skin barrier function and lack of skin appendages, so they did not have complete skin function, and did not reach skin reestablishment. Ideal skin substitute should process cuticular layer and dermial layer. The dermial layer can reach rapid vascularization and nerve redistribution, can promote physiological wound repair. The cuticular layer can rapidly obtain full barrier and protection function, which can be fully integration with wound surface. A good dermial carrier has good histocompatibility, and a certain physical and chemical properties, such as strength, scratchability, elasticity, gas permeation, water vapor permeability. These can provide a good adherence between dermis and wound surface, offer an ideal fiber stent for cell growth, and finally promote autologous skin growth. Tissue engineered skin has good prospect, but there are many problems that should be solved in biocompatibility and skin function reestablishment.

BACKGROUND:The thermosensitive chitosan is a kind of chitosan, its hemostatic effect, tissue compatibility andin vivo absorption need further investigations.
OBJECTIVE:To investigate the hemostasis,in vivo degradation and tissue compatibility of thermosensitive chitosan hemostatic film.
METHODS: A total of 48 Sprague-Dawley rats were randomly divided into four groups, and carried out two
experiments at the same time. (1) The incisions of the liver in three groups were covered with the thermosensitive chitosan hemostatic film, celulose hemostatic cotton and gelatin sponge, respectively. Blank control group
received no treatment. The bleeding time and bleeding amount were recorded. (2) The incisions of the quadriceps femoris muscle of rats in the above three groups were embedded with the same hemostatic materials respectively. Blank control group was not embedded. At 1, 2, 3, 4, 6 weeks, the incision tissues of the liver and the quadriceps femoris muscle were harvested for observation. After 4 weeks, the incisions were observed with hematoxylin-
eosin staining and transmission electron microscopy.
RESULTS AND CONCLUSION: The bleeding time and bleeding amount of thermosensitive chitosan hemostatic film and celulose hemostatic cotton groups were significantly lower than those of gelatin sponge and blank
control groups (P < 0.05). After 6 weeks, the thermosensitive chitosan hemostatic film was absorbed completely. After 3 weeks, the celulose hemostatic cotton was absorbed completely. After 2 weeks, the gelatin sponge was absorbed completely. The liver lobules of thermosensitive chitosan hemostatic film were complete, the liver cellwere normal structure, showing light sweling and little inflammatory cellinfiltration. Under transmission electron
microscopy, the liver cels had integral structure, cellnucleus and organeles remained intact. The muscle fibers showed complete structure and little inflammatory cellinfiltration. Under transmission electron microscopy, the muscle fibers
ranked tidily, with integral cellnucleus and organeles. The thermosensitive chitosan hemostatic film has good hemostasis effect and tissue compatibility.

Objective To analyze the correlation between non-motor symptoms (NMS) and the quality of life in the mild Parkinson's disease (PD) patients of different genders.Methods 85 mild PD patients (H-Y stage 1～2) were investigated.The PD NMS Quest was used to assess NMS.The quality of life was assessed with SF-36 questionnaire.Results Stepwise linear regression analysis showed that nocturia,restless legs,depression,daytime sleepiness,decreased interest,smell taste problems were positively correlated with the score of life quality of male and pain,increased sweating,taste smell problems,sleep problems,changes in libido,decreased memory,diplopia influenced life quality score of female.The scores of SF-36 questionnaire of the male with PD were lower than that of female patients.Conclusions The nonmotor symptom (NMS) There was a gender difference about the influence that NMS has made on PD patients.The male's life quality were lower than that of female patients about mild PD patients.

AIM: To observe the curative effect of titanium wiring in treatment of the fractures of Neer Ⅱ type distal clavicle and dislocation of Tossy Ⅲ type acromioclavicular joint. METHODS: From March 2004 to December 2006, 37 cases with Neer Ⅱ type distal clavicle fractures or Tossy Ⅲ type acromioclavicular joint dislocation were treated with titanium wiring in Changzheng Hospital, Second Military Medical University of Chinese PLA. Titanium wiring (produced by Sofamor-Danek Company in USA) had good histocompatibility, intensity of tension and flexibility. The broken coracoclavicular ligament was repaired directly by titanium wiring in all cases. The outcomes were evaluated according to Herscovici's criteria. RESULTS: All the cases were followed up for 6 to 24 months. All the dislocated acromioclavicular joints returned to normal 2 weeks after operation. Excellent and good rate of shoulder function was 100% according to Herscovici standard. X-ray imaging showed all clavicle fractures united, acromioclavicular joint had no redislocation or titanium wiring breaking. The incisions of all cases had no obvious swelling and exudation. CONCLUSION: When Neer Ⅱ type distal clavicular fracture and Tossy Ⅲ type acromioclavicular joint dislocation are treated with titanium wiring, the acromioclavicular joint can exercise earlier and recover quickly.

Steroid-resistant nephrotic syndrome (SRNS) is a relatively difficult clinical type of treatment.The major therapy measures in present include steroid and immunosuppressant.Commonly used immunosuppressant include tacrolimus,cyclosporin,cyclophosphamide,mycophenolate mofetil,ect.Tacrolimus-induced clinical remission rate is superior to other immunosuppressive agents,has been the first-line agent of SRNS.Because of the individual difference in metabolism,the drug concentration of tacrolimus should be determined periodically.In order to obtain optimal efficacy of tacrolimus and reduce renal toxicity,the treatment protocols of small doses with long courses for children with SRNS were recommended.