Objective To investigate the clinical features of X-linked agammaglobulinemia (XLA)in children,and to improve the early diagnostic rate.Methods The medical records of 11 children with XLA between Dec 2003 and Nov 2011 were reviewed.Results The smallest age of 11 cases with XLA presenting the first symptoms was 0.4 years old and the oldest age was 4 years old with a mean of 2.4 years old.The first diagnosis time was at the age of 3.5 to 13 years old,with a mean of 7 years old.The age at first diagnosis in 63.6％ ( 7/11 ) of patients was more than 7 years old.Two patients ( 18.2％ ) had the family history of the similar disease.Two patients were died from the infection and 1 patient was missed.The other 8 patients were survived.Respiratory infections occurred in 100％ of the 11 patients.54.5％ (6/11 )of the patients were suffered with otitis media and digestive infections were seen in 36.4％ (4/11 ) of the patients.The levels of lgA,IgM and IgG in the serum of the pauents were all decreased.The levels of IgG in the serum of 9 patients were less than 2 g/L and in the serum of the other 2 patients were less than 2.4 g/L.The levels of CD19 in the serum were all less than 1％ and an inversed ratio of CD4/CD8 was observed in 9 patients.Eight patients were diagnosed as XLA by gene detection.Conclusion The age presenting first symptoms in children with XLA in this study is earlier than the reported data and the age at first diagnosis is later.Respiratory infection is the most common manifestation.High prevalence of otitis media and digestive infections are common.The patients with XLA rarely have the family history.The early diagosis and long-term treatment with the intravenous immunoglobulin may improve the prognosis of XLA.

Objective To evaluate different measurement methods in histogram for the diffusion and perfusion parameters from intravoxel incoherent motion (IVIM) MR imaging of different types of uterine fibroids.Methods 63 patients with confirmed uterine fibroids (80 in total) were examined with MR imaging.3D T2WI and IVIM imaging were performed for those patients.The fibroids were classified into three types (type 1, 2, 3) on the basis of different signal intensities on T2WI according to Funaki's theory.Real diffusion coefficient (D), pseudodiffusion coefficient (D*) and perfusion fraction (f) were calculated using IVIM analysis.25%,50% and 75% of those parameters (D25,D50,D75,D*25,D*50,D*75,f25,f50 and f75) as well as mean values (Dmean,D*mean and fmean) were calculated using histogram method.ANOVA was used to compare the IVIM parameters among the three types of fibroids.Results There were 44 type 1,24 type 2 and 12 type 3 fibroids in the total of 80 fibroids.There was significant difference among all the diffusion parameters from histogram and mean values of different types of fibroids, and only one perfusion parameter D*75 value showed significant difference (P<0.05).There was no significant difference among all the f values from histogram and mean values.Conclusion Different measurements of parameters from IVIM histogram showed no added value for diffusion features in different types of fibroids compared to mean value.While the perfusion parameter D*75 value from histogram can distinguish the features of perfusion within different types of fibroids compared to mean value.

Object To explore, on the whole, the anticancer activity and mechanism of the aqueous extract of Ruixiang Langdu (Stellera chamaejasme L.) (SCLA). Methods Mice were given different i.g. doses of SCLA and their serum collected at different intervals after drug administration. The effects of the serum on the proliferation of mouse L 1210 leukemic cells were observed with MTT assay, clone formation and incorporation of [ 3H] TdR into DNA of the cells. Results Serum SCLA collected 1, 2, 4, 8 h after administration of 3, 6, and 12 g/kg SCLA showed significant decrease of MTT formazans and clone formation. Serum SCLA collected 2 h after drug administration showed more strong inhibition of cancer cell proliferation. It also inhibited the incorporation of [ 3H] TdR into DNA of L 1210 cells. Conclusion SCLA showed a remarkable inhibitory effect on proliferation and DNA synthesis of cancer cells cultured in vitro, which may be the main anticancer mechanisms of SCLA.

ABSTRACT:Objective To evaluate the clinical therapeutic effect and safety of tumor necrosis factor alpha (TNF-α)blockers in treating moderately to severely active ulcerative colitis (UC)by meta-analysis.Methods Such databases as the Cochrane Central Register of Controlled Trials,PubMed,OVID,Embase,ISI,CBM,CNKI, VIP,and WanFang Data were searched from establishment to June 2013.All randomized clinical trials (RCTs)on tumor necrosis factor alpha blockers in treating UC were collected,and then selected on the basis of the inclusion and exclusion criteria.We assessed the methodological quality,extracted the data from the included articles and performed the meta-analysis with Revman 5.1.Results A total of 13 RCTs involving 3334 patients were analyzed.TNF-αblockers group was superior to the control group in the short-term clinical response (OR =2.5 1, 95% CI 1.73,3.64),short-term clinical remission (OR =2.74,95% CI 1.80,4.1 6),long-term clinical response (OR =2.98,95% CI 1.98,4.47),1ong-term clinical remission (OR =2.64,95% CI 1.89,3.67),and mucosal healing (OR =1.89,95% CI 1.39,2.59)compared with control group.TNF-αblockers could also reduce the rate of colectomy (OR =0.61,95% CI 0.41,0.89)and improve inflammatory bowel disease questionnaire scores (MD=14.74,95% CI 1 1.43,18.06 ).There was no significant difference between the two groups in all reported adverse effects (OR =1.14,95% CI 0.97,1.34)and serious adverse effects (OR=0.78,95% CI 0.56,1.09).Conclusion Compared with conventional therapy or placebo,TNF-αblocking agents can improve the therapeutics effect on UC in clinical response,clinical remission and mucosal healing,and also can reduce the rate of colectomy. In patients with moderately to severely active UC treated with TNF-α blocking agents,it is easier to achieve the improvement of life quality.TNF-αblocking agents treatment is safe for UC.This conclusion should be verified with more large-scale and high-quality RCTs.

A high performance liquid chromatographic (HPLC) method using triethylamine (TEA), copper *" acetate and L-proline as the chiral mobile phase (CMP) and the enantiomric analysis of thyroxine (T4) are described. The influence of the mobile phase parameters including the TEA concentration, manipulation pH, concentration of Cu*", concentration of L-proline, temperature of column and flow rate of eluent on the enantiomers separation of thyroxine was studied. The HPLC-CMP is a simple method for separation and determination of D，L-T4 in drug or in human plasma. It can be used to therapeutic drug or adverse drug reactions monitor.

Objective To observe the preventive efficacy and safety of dexmedetomidine with parecoxib sodium on the patients with postoperative hyperalgesia induced by remifentanil. Methods A total of 100 female patients undergoing elective surgery under general anesthesia were as-signed into four groups according to the table of random number:the control group (group C),the parecoxib sodium group (group P),the dexmedetomidine group (group D)and the parecoxib sodium combined with the dexmedetomidine group (group DP).The vital signs were monitored and the total intravenous anesthesia was performed.All the patients were give intravenous injection of 0.2μg·kg-1 ·min-1 remifentanil and 4-12 mg·kg-1 ·h-1 propofol to maintain the anesthesia.Patients in group P were given 40 mg parecoxib sodium 30 minutes before the end of the operation.Patients in group D were give intravenous injection of 0.6μg·kg-1 ·min-1 dexmedetomidine consistently till 30 min before the end of the operation.Patients in group DP were given 0.6 μg·kg-1 ·min-1 till 30 min before the end of the operation and were given 40 mg parecoxib sodium.The VAS scores were re-corded at 1,2,6,12,24 hours.The cases of agitation,rigors,nausea and vomiting and increasing of analgesics were recorded.Results The postoperative VAS scores in group P,group D and group DP were significantly lower than group C(P <0.05).The postoperative VAS scores in group DP were significantly lower in group P and group D (P<0.05).Cases of agitation and rigors in group D and group DP were less than group C(P <0.05).The increasing of analgesics in group DP was much higher than other groups(P<0.05).Conclusion After induced,patients were given intravenous in-jection of 0.6 μg·kg-1 ·min-1 dexmedetoniding consistently till 30 min before the end of the opera-tion were given 40 mg parecoxib sodium can effectively prevent hyperalgesia after remifentanil anes-thesia without significant increase in revival time and obtain a better sedation.

After a description of the characteristics of novelty assessment of literature on information technology involving different subjects, multiple new technologies and deep knowledge, the strategies were elaborated for it.

Objective:To investigate the clinical efficacy of mechanical solitaire AB stents thrombectomy combined with intra-arterial thrombolysis in the treatment of patient with acute ischemic stroke.Methods:Fifteen patients with acute ischemic stroke admitted into our hospital from August 2014 to August 2016 were treated with mechanical thrombectomy with solitaire AB stents plus intra-arterial thrombolysis.The National Institutes of Health Stroke Scale score (NIHSS) of all patients were evaluated before and after treatment to compare the clinical efficacy.The prognosis ofpatients between two groups were compared via evaluating modified Rankin score (mRS)and gelasijia coma score (GCS).Results:After mechanical thrombectomy with solitaire AB stents plus intra-arterial thrombolysis treatment,14 patients achieved complete or part recanalization,and 1 patient was terminated treatment due to vital signs instability,and the rate ofrecanalization was 93.3％.The NIHSS score of patients before treatment was 12.93± 4.25,which was much higher than that after treatment (4.33± 1.45,P＜0.05).After follow-up by 3 months,the good mRS scores were obtained in all 18 patients,including 2 patients with mRS score of 2,5 patients with 1,and 8 patients with 0.Additionally,there was no patient with re-obstruction during follow-up period.Conclusion:Mechanical thrombectomy with solitaire AB stents combined with intra-arterial thrombolysis had a good capability and safety in the treatment of patients with acute ischemic stroke.

OBJECTIVE:To study the antitumor mechanism of Ruixiang Langdu(Stellera chamaejasme L ) abstracts(SCA) METHODS:SCA-contained serum was derived from mice pre-administrated with different oral dosages of SCA and at different times after administration The effects of the serum on the proliferation of K562 leukemic cells were observed with MTT assay and clone formation RESULTS:After mixing with the SCA-contained serum derived at 1,2,4,8h after giving SCA(3,6 and 12g/kg),the rates of MTT transformation and clone formation of K562 cells were decreased significantly The SCA-contained serum 12 h after giving drug was more effective than others CONCLUSION:The SCA-contained serum inhibited the proliferation of tumor cells,which may be one of its important antitumor mechanism

OBJECTIVE:To develop a capillary electrophoresis method for determing the enantiomer of ceftriaxone Na.METHODS:A chiral resolving agent,?-cyclodextrin,was employed as chiral additive for ceftriaxone Na enantiomeric separation by capillary electrophoresis.In different electrophoresis polarity mode,the effect of pH of background electrolyte and the concentration of ?-cyclodextrin were investigated.RESULTS:The optimal conditions for enantiomeric separation were as follows:separation voltage:28kV,buffer solution:NaH2 PO4 50mmol/L,?-CD 0.04mmol/L,Tirs 3.0mmol/L,pH7.15.CONC_LUSION:The method is simple,sensitive,rapid and accurate,and can be used for the quality control of ceftriaxone Na enantiomers.There was significant difference in contents of enantiomers of ceftriaxone Na between products of two factories.We suggest that a quality control method for ceftriaxone Na enantiomer should be established,which will provide scientific basis for quality control of drug and clinical choice of effective antibioties.