0.05).When the concentration of ferumoxides exceeded 22.4?g/ml,there was significant difference(P

Objective:To discuss the function of PI3K (phosphatidylinositol-3 kinase)/Akt signal pathway in proliferation and differentiation of neural stem cells (NSCs).Methods:Control group,wortmannin group and LY294002 group were randomly assigned.The expression of Nestin,NSE and S100 were detected by immunohistochemistry.The ratio of cell apoptosis was estimated by TUNEL staining and the expressions of phosphorylated Akt and Caspase-9 were detected by Western blotting.Results:With the increase in concentration of wortmannin and LY294002,the ratio of NSCs apoptosis increased.The results of Western blotting indicated that the expression of phosphorylated Akt decreased when the concentration of wortmannin and LY294002 increased.High concentration of wortmannin and LY294002 inhitited Akt phosphorylated.At the same time,the expression of phosphorylated Caspase-9 decreased.Conclusion:The survival of NSCs depends on the activation of PI3K/Akt pathway,but PI3K/Akt pathway may have little function in the differentiation of NSCs.

Objective To evaluate the clinical significance of classifying vertebral artery-atlas complex using angiographic CT (computed tomography angiography,CTA).Methods The CTA data of 225 patients were reconstructed three-dimensionally in Mimics.The region of V3 segment of vertebral artery and isthmus of atlas's posterior arch were reconstructed as vertebral artery-atlas complex,classify according to the relationship of the two.The complex types were classed into 3 types and 7 subtypes.Type A was conventional type,which means V3 segment locates above the isthmus of atlas.The distance between vertebral artery and atlas was larger than 1.8 mm in A1 subtype.The distance was between 1.8 and 0.6 mm in A2,and less than 0.6 mm in A3.Type B was variation of vertebral artery.Subtype B1 was far-away variation,which means artery doesn't pass around posterior arch of atlas.Subtype B2 means the artery passed underneath the arch of atlas.Type C was variation of bone.Bone structure was closed in subtype C 1,and unclosed in subtype C2.The distance between artery and atlas was measured in subtype A1 and A2.Results There were 450 cases of vertebral artery-atlas complex in the 225 patients,which could be divided into A1,including 57 (12.7％),A2,including 115 (25.6％),A3,including 194 (43.1％); B1 including 20 (4.4％),B2,including 9 (2.0％); C1,including 34 (7.6％),C2,including 21 (4.7％).More than half patients (50.2％) had different subtype in both sides.The measured value of VAD was 2.42±0.52 and 1.20±0.36 mm in subtype A 1 and A2 respectively.Conclusion Reconstructing CTA images before fixation of atlas can clearly demonstrate the complex of V3 segment of vertebral artery and isthmus of the posterior arch of atlas.Classifying the complex and choose appropriate method of inserting screws can improve the security of the operation.

BACKGROUND:As a kind of fiber composite resin adopt the technology of fibre reinforcement composites, the everStick high-strength fiber composite resin has been applied in the field of dentistry prosthodontic treatment restoration.
OBJECTIVE:To summarize the research condition about the material performance and clinical application of the everStick high-strength fiber composite resin
METHODS:We searched the Medline, CNKI, and WanFang database in computer (from January 2000 to August 2013) using the keywords of“everStick high-strength fiber composite resin, material performance, clinical application”in English and Chinese.
RESULTS AND CONCLUSION:The everStick high-strength fiber composite resin has the unique structure of IPN, and has the characteristics of simple operation, minimal y invasive, higher strength, strong cohesive force and reversibility. Because of the minimal y invasive, the everStick high-strength fiber composite resin can retain the healthy tissue of tooth with whole hog and provide the possibility for other therapeutic method because of the reversibility. At the present time, the everStick high-strength fiber composite resin has already been extended in the fields of post core, periodontal splint, orthodontic retainer, ful crown, adhesive fixed bridge, fixed bridge, inlay fixed partial dentures, dental implant restoration and the base in removable partial denture. But whether the material can be made into resin veneer and be used in color front teeth, for instance, the dental fluorosis, tooth discoloration after root canal therapy, tetracycline pigmentation teeth and hypoplasia of enamel, there is lack of comprehensive studies.

Objective To explore the clinical and laboratory features,and gene diagnosis method of Menkes disease (MD).Methods The clinical and laboratory features and gene diagnosis method of 2 infants with MD were reviewed.Results (1) Clinical features:both infants mentioned in this article were male.Their clinical manifestations were both began at 3-4 months age,including peculiar kinky hair,pale skin,pudgy cheeks,inguinal hernia,vessel abnormality,epilepsy and mental retardation.(2) Laboratory features:the ceruloplasmin concentrations significantly reduced to be ＜ 20 mg/L and 47 mg/L,respectively.The magnetic resonance angiogram images of case 1 showed the abnormal tortuosity of his intracranial vessels.The magnetic resonance images of case 2 showed a rapid progress from normal to severe brain atrophy within half a year.(3) Gene diagnosis:the sequencing of ATP7A gene in case 1 showed a nonsense mutation of c.2110 C ＞ T.The pathogenicity of this mutation had not been reported previously at home and abroad.The sequencing of the gene panel without pathogenic mutation was detected in case 2.But the multiplex ligation-dependent probe amplification test showed a gross deletion of ATP7A gene containing 8-12 exons.This mutation had been documented as a pathogenic mutation of MD.Both mothers of 2 patients were heterozygous mutation carriers of normal phenotype.Conclusions MD is a multisystemic disease caused by ATP7A gene mutation resulting in copper metabolism disorder.MD is inherited as an X-linked recessive trait.MD is characterized by kinky hair,connective tissue abnormalities and progressive neurodegeneration.Clinical diagnosis can be made on the basis of clinical features,findings of blood biochemical examination,and radiological findings.Gene sequencing and multiplex ligation dependent probe amplification test are the main technique widely used for genetic diagnosis.

Objective To study the clinical characteristics and the effect of enzyme replacement therapy for late-onset glycogen storage disease typeⅡ(GSDⅡ). Methods The clinical, laboratory data and the result of genetic testing were retrospectively analyzed in a GSDⅡchild, the effect of enzyme replacement therapy was followed up and the relevant literature was reviewed. Results The patient had motor regression after 1 year old, the serum creatine kinase level is from 675 to 1286 U/L. The EMG test showed myopathic change, acid alpha-glucosidase activity is 12.0 nmol/(g·min), next generation sequencing of genetic muscle diseases panel found the GAA compound heterozygous mutations, both were tiny variations, and muscle biopsies showed the typical pathological features of GSD. The patient was given human recombinant of alpha glucoside enzyme 20 mg per kilogram of body weight, once every other week for 1 year. The weakness of the patient’s muscle strength had no obvious aggravation. Conclusions Early and adequate enzyme replacement therapy is the only possible treatment for GSDⅡ.

BACKGROUND:The fiber-reinforced composite resin adhesion fixed bridge produced by everStick fibre reinforced composites is a kind of minimaly invasive fixed prosthesis technology in recent years, which is wel thought of patients and clinicians because of its smal tooth preparation, high success rate of bonding, good esthetic effects, strong mechanical properties and easy to intraoral repair. OBJECTIVE:To investigate the advantage of everStick fibre reinforced composites on too smal anterior edentulous space restoration. METHODS:A total of 23 patients with maxilary lateral incisor missing and with smal edentulous space were colected and renovated with everStick fibre reinforced composites produced by StickTech in Finland. RESULTS AND CONCLUSION:everStick fibre reinforced composites for repair of too smal anterior edentulous space with adhesion fixed bridge could not only avoid the overclosed occlusion on adjacent teeth like fixed denture, but also avoid the usage of clasp and uncomfortableness such as removable denture. During the folow-up of 6-30 months, 23 patients had no signs of loosening, debonding, fracture, excessive wear, secondary caries, margin dying of restoration and exhibited good margins and composite luting-cement interface, unchanged color, good esthetic effects. The patients were very satisfied with the restoration. The results indicate that everStick fibre reinforced composites for restoration of too smal anterior edentulous space can achieve good abrasion resistance and high mechanical strength, unchanged color, have little damage to abutment and have good esthetic effects.

Parkinson's disease is characterized by degeneration and dysfunction of dopaminergic neurons in the mid-brain substantia nigra, neurons that project to the caudate putamen. The clinical symptoms are caused by the loss of dopamine and the consequent dysfunction of the nigrostriatal pathway. Neurotrophic factors that may prevent nigrostriatal degeneration by improving the function of remaining dopaminergic neurons has the potential to be a useful therapy for Parkinson's disease. Neurotrophic factors and their specific receptors could play an important role in the progress of Parkinson's disease. The neuroprotective effects of neurotrophic factors GDNF, BDNF, IL-6 on dopaminergic neurons in vivo and in vitro , and the potential therapeutic use in treating Parkinson's disease were discussed in this paper.

Objective To correlate the neutrophil extracellular trap (NET) mitochondrial DNA (mtDNA) and anti-mtDNA antibodies (Abs) with disease activity and clinical features in systemic lupus erythematosus (SLE) patients.Methods We enrolled 102 SLE patients,rheumatoid arthritis (RA) patients (n=30) and healthy donors as controls (n=40).NET were generated from phorbol 12-myristate 13-acetate (PMA)-stimulated peripheral neutrophils.mtDNA levels and the transcriptional levels of five interferon inducible genes(IFIGs)(OAS-1,Mx-1,Ly6e,IFIT1 and IFIT4) were measured by quantitative PCR.Interferon scores (IFN scores) were calculated.Anti-mtDNA Abs were detected by enzyme-linked immunosorbent assay.Spearman's correlation analysis,t test andx2 test were used for statistical analysis.Results mtDNA release by netting neutrophils was greatly enhanced in SLE patients (1 088 000 ±1 133 000) compared with healthy controls (465 900±447 200)(t=2.617,P＜0.01) and significantly correlated with IFN scores (r=0.460 6,P＜0.01).NETs mtDNA in moderate active group (728 300±1 003 000) and severe active group (1 093 000±946 500) were significantly higher than the mild active group (159 500±155 100) (t=2.240,P＜0.05,t=3.894,P＜0.01).Forty-one percent of SLE patients were positive for anti-mtDNA Abs(1.28±0.68),while none of the healthy donors (0.70±0.31) (P＜0.01) and RA controls (0.59±0.18)(P＜0.01) displayed a positive serology response to mtDNA.Addition-ally,the titers of anti-mtDNA Abs were also associated with IFN scores (r=0.292 8,P＜0.05).Anti-mtDNA Abs in moderate active group (1.3±-0.6) and severe active group(1.4±0.7)were significantly higher than the mild active group (0.7±0.4) (t=3.154,3.538,all P＜0.01).The levels of anti-mtDNA Abs significantly correlated with classic an-ti-dsDNA Abs titers measured by Farr assay (r=0.542 9,P＜0.001) and were associated with LN (x2=8.644,P＜0.01).Conclusion mtDNA in NET and anti-mtDNA Abs serve as new biomarkers for disease activity and renal involvement in SLE patients.