Abdominal Pain ; etiology ; Child, Preschool ; Humans ; Male ; Porphyria, Acute Intermittent ; complications ; diagnosis ; therapy ; Prognosis ; Treatment Outcome

Objective To observe the delayed brain myelination of children with phenylketonuria(PKU)combined with epilepsia,and explore effectiveness of the treatment and provide an objective criteria for patient recovering evaluation.Methods There were 42 PKU patients,aged 3 to 72 months were selected.The concentration of phenylalanine tested by high pressure liquid chromatography was greater than 1.2 mmol/L in blood,diagnosed as PKU.According to electroencephalogram and clinical symptom,21 cases were diagnosed as epilepsy,the other 21 cases were used as control group.All patients were taken MRI before treatment.Myelination in 10 sections(cerebellum,pons,mesencephalon,internal capsule posterior limb,corpus callosum,internal capsule anterior limb,occipital lobe,parietal lobe,temporal lobe,frontal lobe)were evaluated.Results Delayed myelinations were located mainly in the cerebral lobes and corpus callosum,average delayed incidence of the 10 region was 44.8% in epilepsy group and 30.9% in control group.The incidence of the corpus callsum was 80.9% in epilepsy group,52.4% in control group,the number of sections of delayed myelination showed statistically significant between 2 groups(P

Humans ; Mandible ; surgery ; Osteogenesis, Distraction ; methods ; Reconstructive Surgical Procedures

Breast cancer is a multistep process with abnormalities of some biomarkers.Knowledge of biomarkers can provide prognostic information in addition to the clinicopathologic factors,and is useful to carry out the indiviual treat- ment.In this review,we summarized the most recent work in the field of biological markers used for the prognosis,such as Her-2/neu,P53,BRCA1,Angiogenesis,LOH,et al

Accidents, Traffic ; Forensic Medicine ; Hoarseness ; Humans

In the process of evolution, pathogenic Streptococcus pyogenes secretes an immunoglobulin G-degrading enzyme IdeS which can specifically cleave the hinge region of immunoglobulin G in order to escape the immune response against the host. On the one hand, IdeS can be used for IgG fingerprinting as a tool enzyme combined with mass spectrometry technology. On the other hand, IdeS can be used to treat the antibody-responsive diseases produced by autoimmunity as a therapeutic protein. In this study, the backbone of plasmid pCold was used to construct two expression vectors of recombinant protein IdeS, which were heterologously expressed in Escherichia coli Shuffle T7. After purification by affinity chromatography, the recombinant IdeS activity was detected and their activity differences between the two were compared. Among them, the yield of the recombinant IdeS containing the His6-tag at the N-terminus was 4 mg·L^-1, and the cleavage reaction with antibody IgG1 at 1∶200 (m/m) at 37 ℃ for 30 min could complete. However, the yield of the recombinant IdeS containing both the N-terminal His6 tag and the C-terminal silica affinity tag (silica bing peptide, SiBP) is 1.5 mg·L^-1, and the degradation reaction with antibody IgG1 at 1∶20 (m/m) at 37 ℃ for 30 min could reach the end. The C-terminal fusion peptide has a great influence on the yield and activity of IdeS, which is not conducive to subsequent application in drug development. Above all, the recombinant IdeS containing the His6-tag at the N-terminus expressed by this system has high activity and can fully meet the needs of antibody drug development and mapping analysis of IgG.

Objective To investigate the efficacy and safety of the combined therapy of cyclophosphamide and thalidomide in the treatment of refractory Crohn's disease (CD).Methods This study was a prospective and open study.A total of 15 patients with refractory CD were enrolled.All patients received intravenous cyclophosphamide 200 mg every other day for two weeks,then followed by intravenous 400 mg once a week until the cumulative dose reached 6 to 8 g.when the cyclophosphamide treatment started,at the same time thalidomide was taken 25 to 75 mg every night according to the tolerance of patients.Before the treatment,two weeks' after the treatment and at the time when the cumulative dose of cyclophosphamide reached 6 to 8 g,Crohn's disease activity index (CDAI),hemoglobin (Hb),white blood cell (WBC) count,erythrocyte sedimentation rate (ESR) and high-sensitivity C-reactive protein (hs-CRP) were recorded.Endoscopy examination was conducted before the treatment and at the time when the cumulative dose of cyclophosphamide reached 6 to 8 g.The condition of mucosa healing was observed and scored by simple endoscopic score for crohn's disease (SES-CD).Adverse effects of all patients were monitored.Paired t test was performed for statistical analysis.Results Before the treatment,the CDAI of 15 patients with refractory CD was 235.87±59.87,two weeks after the treatment the CDAI declined to 135.33 ± 29.23,and the difference was statistically significant (t=7.50,P＜0.01).Before the treatment,ESR and hs-CRP was (42.13±22.80) mm/1 h and (13.73± 2.18) mg/L.Two weeks after treatment they declined to (23.80±16.63) mm/1 h and (5.77±4.77) mg/L,and the differences were statistically significant (t=2.43 and 6.17,both P＜0.05).After two-week treatment,10 patients achieved clinical remission.After the cumulative dose of cyclophosphamide reached 6 to 8 g combined therapy,CDAI of patients was 108.14 ± 47.10,which decreased significantly compared with that before treatment (t=6.30,P＜0.01).ESR,hs-CRP and WBC count was (19.35± 19.18) mm/1 h,(6.16± 5.02) mg/L and (6.28 ± 3.42) × 109/L,respectively,which decreased compared with those before treatment,and the differences were statistically significant (t=5.90,5.40 and 3.71,all P＜0.01).Twelve patients achieved clinical remission.And the lesions of 12 patients improved under endoscope,furthermore,the mucosa of four patients healed.Before the treatment,SES-CD was 9.14 ± 5.39,which declined to 5.07 ± 4.58 after the treatment,and the difference was statistically significant (t =3.14,P ＜ 0.01).During the treatment,five patients had adverse effects.Alanine aminotransferases (ALT) increased in three patients,WBC count decreased in one patient and one patient got a severe urinary infection.Conclusions Patients with refractory CD could achieve clinical remission,mucosa healing under endoscopy and better efficacy with the combined therapy of cyclophosphamide and thalidomide.However,adverse effects should be monitored during the treatment.

Humans ; Metals ; Microwaves ; adverse effects ; Occupational Exposure ; adverse effects ; analysis ; Workplace

Background and Objective: The histone deacetylase inhibitor,trichostatin A(TSA),was shown to induce apoptosis in transformed cells at submicromolar concentrations. However, the effect of TSA on brain tumor cells is still unknown. This study was designed to investigate whether TSA posses antitumor activity and if any, its mechanism. Materials and Methods: A p53 mutant human glioma cell line T98G and a p53 wild type human neuroblastoma cell line SKNSH were exposed to TSA. Cell proliferation was assessed by sulforhodamine B (SRB) cytotoxicity assay. Apoptosis was quantified by flow cytometry and confirmed by apoptotic ladder formation. Expression patterns of accumulation of highly acetylated histone H3,H4; p53 and cell cycle-associated p21waf,p27 which were induced by TSA were determined by using Western blot analysis. Results: TSA inhibited the proliferation of brain tumor cell lines at nanomolar concentrations and induced accumulation of highly acetylased histone moleculars. Treatment with TSA at 0.33μ M for 24h significantly induced cell apoptosis.In addition to the suppression of cell growth, the up regulation of p21waf and p27 expression was observed within 48h after the treatment.p21 protein levels were increased at early time points and reached maximal levels at 8h, while p27 protein levels were increased after 8h. However, there was no significant changes of acetylased p53 and endogenous p53 protein levels were observed. Conclusion:TSA may inhibit brain tumor cell growth in vitro, which is otherwise particularly resistant to chemotherapy. TSA acts as an anti-tumor agent could be through co-operation between p21 and p27 in growth inhibition, irrespective of endogenous p53 status.

Objective To study the function of core protein (CORE) of genotype 1b hepatitis C virus (HCV) of different strains (T: derived from tumor tissues; NT: derived from non-tumor tissues; C191: HCV-J6) and different domains (1-172, 1-126, 1-58, 59-126, 127-172 AA) of T CORE in the pathogenesis of HCV infection and to find the therapy target. Methods Different truncated genotype 1b HCV CORE eukaryotic expression plasmids (T, NT, C191) and different domains of T CORE were constructed and transfected to HepG2 cells. Cell apoptosis and necrosis were quantified by flow cytometry. Cell growth curves were observed with real time cell growth instrument. Results COREs from different strains of genotype 1b and different domains of CORE induced cell apoptosis and necrosis, and inhibited HepG2 cell growth at different levels. CORE derived from T induced apoptosis and necrosis and inhibited cell growth higher than that derived NT and C191. N terminal 1-58 AA of CORE derived from T induced cell apoptosis and necrosis and inhibited cell growth higher than any other domains. Conclusion COREs from different strains of genotype 1b HCV and different domains of CORE from the same HCV strain play different roles in their molecular pathogenesis of HCV. Among different domains of CORE, N terminal 1-58 AA might play an important role in its pathogenesis and be one target of gene therapy.