OBJECTIVE:To observe the safety and efficacy of second-generation recombinant factor Ⅷ (Kogenate FS) for treatment of patients with hemophilia A in China. METHODS: 16 patients with hemophilia A were treated with Kogenate FS. The factor Ⅷ antibodies and factorⅧ activities (FⅧ∶ C) were determined before and after the treatment. RESULTS: In the treatment of Kogenate FS for 16 patients with hemophilia A,15 (93.75%) recovered and 1 (6.25%) improved,and irreversible adverse drug reaction was seen in only 1 case. CONCLUSION: The second-generation recombinant factor Ⅷ product (Kogenate FS) was safe,efficacious and well-tolerated for patients with hemophilia A in China.

Objective To observe the effect of leflunomide and cyclophosphamide combined with prednisone in the treatment of idiopathic membranous nephropathy .Methods 30 patients with idiopathic membranous nephropa-thy were randomly divided into two groups ,15 cases in each group .A group was treated by leflunomide combined with prednisone , B group was treated by cyclophosphamide combined with prednisone , 6 months a course .The clinical effect,24h urine protein quantity,serum albumin levels,blood fat,renal functions and adverse reactions were com-pared.Results 3,6 months after treatment ,the remission rates in A group were 60.00%,73.33%,those in B group were 53.33%,66.67%(χ2 =0.965,0.896,all P>0.05).After treatment,24h urine protein quantity,serum albu-min levels in the two groups [A group:(1.33 ±1.25)g/24h,(38.24 ±4.84)g/L;B group:(1.42 ±1.37)g/24h, (37.12 ±5.43)g/L] were significantly lower than those before treatment [A group:(7.34 ±2.75)g/24h,(20.31 ± 7.33)g/L;B group:(7.22 ±2.84)g/24h,(20.46 ±7.73)g/L] (A group:t=6.232,5.734,all P<0.05;B group:t=5.934,5.267,all P<0.05).After treatment,total cholesterol,triglyceride in the two groups [A group:(6.74 ± 1.45)mmol/L,(2.43 ±0.75)mmol/L;B group:(6.63 ±1.45)mmol/L,(2.14 ±0.63)mmol/L] were significantly lower than those before treatment [A group:(11.94 ±2.98) mmol/L,(3.56 ±1.35) mmol/L;B group:(11.53 ± 2.84)mmol/L,(3.24 ±1.46)mmol/L] (A group:t=6.246,5.234,all P<0.05;B group:t=5.256,5.672,all P<0.05).There were no significant differences in urea nitrogen ,serum creatinine between before and after treatment in the two groups(all P>0.05).There were no significant differences in above indicators between A group and B group after treatment (all P>0.05).The incidence rate of adverse reactions in A group (13.33%) was significantly lower than that in B group(40.00%)(χ2 =4.246,P<0.05).Conclusion The clinical efficacy of leflunomide is similar to cyclophosphamide in treating idiopathic membranous nephropathy with fewer adverse reactions .

Objective To apply 3C3R model in problem design of PBL for pediatrics teaching.Methods The 3C3R model comprises two classes of components:core components and processing components.Core components of the model are 3C,which are content,context and connection while processing components are 3R,which are researching,reasoning and reflecting.3C3R model was used in the problem design for the PBL case of ‘ Why the mouth of Baobao became purple when he was crying?' Totally 76 eight-year program medical students and 7 tutors were enrolled as teaching object.The anonymous questionnaires from the students were collected for assessment of PBL teaching.Results The percentage of students with scores ≥4 for content in PLB problem design was 90.8％,for context was 80.3％,for connection was 64.5％,for researching was 81.6％,for reasoning was 69.7％ and for reflecting was 40.8％.The percentage of tutors with scores ≥4 for content in PLB problem design was 100％,for context was 71.4％,for connection was 28.6％,for researching was 71.4％,for reasoning was100％,for reflecting was57.1％.Both students and tutors held a positive attitude towards the component of content,context,researching and reasoning in problem design model.But the components of connection and reflection needed to be improved.Conclusion The 3C3R model is helpful for problem design of PBL in pediatric teaching.

Objective To investigate the effect of different doses of dexmedetomidine on the median effective concentration (EC50) of propofol required to prevent the response to Supreme laryngeal mask airway (LMA) insertion in aged patients.Methods ASA Ⅰ or Ⅱ patients of both sexes,aged ≥ 65 yr,with a body mass index of 20-28 kg/m2,undergoing knee operation under general anesthesia,were randomly divided into 3 groups:control group (group C),small dose dexmedetomidine group (group D1 ) and large dose dexmedetomidine group (group D2 ).Dexmedetomidine 0.4 and 0.8 μg/kg were infused intravenously over 10 min in groups D1 and D2 respectively,while group C received the equal volume of normal saline instead.Anesthesia was induced with target-controlled infusion of propofol.The initial target plasma concentration of propofol was set at 3.5,3.0 and 2.6 μg/ml in groups C,D1 and D2 respectively.Following equilibration between the plasma and effect-site concentration of propofol,LMA was inserted when BIS value was 50-60.EC50 was determined by up-and-down sequential trial.The target plasma concentration of propofol increased/decreased by 10％ in the next patient depending on whether or not the LMA insertion response occurred.Positive LMA insertion response was defined as body movement,comer of the mouth movement,biting LMA,bucking and/or wallowing during insertion.The EC50 and 95％ confidence interval (CI) of propofol required to prevent LMA insertion response were calculated with sequential method.Results EC50(95％ CI) of propofol was 3.57 μg/ml (2.91-3.87 μg/ml),3.09 μg/ml (2.66-3.53 μg/ml) and 2.62 μg/ml (2.30-3.15 μg/ml) in groups C,D1 and D2 respectively.EC50 was significantly lower in groups D1 and D2 than in group C,and in group D2 than in group D1 ( P ＜ 0.05 ).Conclusion Dexmedetomidine 0.4 and 0.8 μg/kg infused intravenously can reduce the EC50 of propofol required to prevent the response to Supreme LMA insertion in aged patients,and the effect of 0.8 μg/kg is more obvious.

ObjectiveTo investigate the correlation between the methylation status of HLA class Ⅰ genes(HLA-A, -B and -C) in psoriatic epidermis and disease severity in patients with psoriasis vulgaris. MethodsDNA specimens were obtained from the lesional and nonlesional epidermis of 46 patients with psoriasis vulgaris and from the normal skin of 28 human controls. Methylation specific PCR (MSP) was conducted to detect the methylation status of CpG islands in the promoter region of HLA-A, -B and -C genes. The severity of psoriasis was evaluated by psoriasis area and severity index(PASI) scores. ResultsThe percentage of promoter methylation of HLA-B and HLA-C genes was 4.35％(2/46) and 21.74％(10/46), respectively in nonlesional epidermis, 4.35％ (2/46) and 4.35％ (2/46), respectively in lesional epidermis from these patients. No methylation was observed for the promoter of HLA-A, -B or -C gene in the normal control epidermis or for that of HLA-A gene in the nonlesional or lesional epidermis from the patients. The frequency of HLA-C gene promoter methylation in the nonlesional epidermis was significantly higher than that in the lesional epidermis and control epidermis, but was uncorrelated to the disease severity. No significant difference was observed for the methylation frequency of HLA-A or -B gene promoter among the three groups of specimens. Conclusion Abnormal methylation of HLA-C gene promoter is observed in patients with psoriasis vulgaris.

Objective To evaluate the effects of dexmedetomidine on hemodynamics during induction of anesthesia in the patients with atrial fibrillation with rapid ventricular rate undergoing noncardiac surgery.Methods Fifty patients with rheumatic valvular heart disease complicated with atrial fibrillation,aged 45-64 yr,weighing 50-75 kg,with ventricular rate ≥ 90 bpm,of ASA physical status Ⅱ or Ⅲll (NYHA Ⅱ or Ⅲ),scheduled for elective surgery,were randomly divided into 2 groups (n =25 each) using a random number table:control group (group C) and dexmedetomidine group (group D).Dexmedetomidine 0.6 μg/kg was infused intravenously at 10 min prior to induction of anesthesia in group D.Anesthesia was induced with iv midazolam 0.06 mg/kg,sufentanil 0.6 μg/kg,and vecuronium 0.12 mg/kg.Tracheal intubation was performed when the BIS value≤≤ 55After admission to operating room (T0,baseline),immediately after the end of dexmedetomidine infusion (T1),immediately before intubation (T2),and at 1,3 and 5 min after intubation (T3-5),SP,DP,MAP and HR were recorded.The adverse cardiovascular events were recorded starting from induction of anesthesia to 5 min after intubation.Results Compared with the baseline value at T0,HR was significantly decreased at T2,5,while increased at T3,4 in group C,and HR was decreased at T1-5 in group D; SP,DP and MAP were decreased at T2,5,while increased at T3 in group C,and no significant changes were found in the indices mentioned above in group D.Compared with group C,the incidence of hypotension,hypertension and tachycardia was significantly decreased,and no significant changes were found in theincidence of bradycardia in group D.Conclusion Dexmedetomidine 0.6 μg/kg infused intravously is helpful in maintaining the hemadynamics stable during induction of anesthesia in the patients with atrial fibrillation with rapid ventricular rate underging noncardiac surgery.

A novel series of benzyl urea analogues based on the structural modification of sorafenib were synthesized. Their in vitro antitumor activities against MX-1, HepG2, Ketr3 and HT-29 were evaluated using the standard MTT assay. While several target compounds showed inhibitory activity against multiple cancer cell lines, compound 9 was of particular interest, demonstrating IC50 values (5.69-13.6 micromol x L(-1)) comparable to those of sorafenib. Furthermore, compounds 20 and 23 showed more potent inhibitory activity against HT-29 and MX-1 when compared to sorafenib. In particular, compound 20 bearing the N-3-pyridyl moiety not only exhibited greater inhibitory activity against HT-29 cell line (IC50 3.82 micromol x L(-1)), but also had improved solubility at pH 7.2, is worthy of further investigation as a lead to identify novel antitumor agents.

Objective To study the therapeutic effect and safety of recombinant human growth hormone in short children born small for gestational age (SGA).Methods Twenty-two short children born SGA were randomly divided into 2 groups and were exposed to different doses of recombinant human growth hormone,which were low dose group [0.1 IU/(kg · d)] and high dose group [0.2 IU/(kg · d)].Treatment was carried out for 2 years.Before and after treatment,height,weight,bone age,insulin-like growth factor 1 (IGF-1),insulin-like growth factor-binding protein 3 (IGFBP-3),growth rate (GV),height standard deviation scores (HtSDS),predicted adult lifetime height (PAH),fasting and postprandial blood glucose,insulin,thyroid stimulating hormone (TSH),T3,T4,and glycosylated hemoglobin were measured.Results Basic value of growth hormone in SGA infant was (2.94 ± 3.27) μg/L.Two years after treatment of growth hormone in high dose group,growth rate [(8.11 ± 1.31) cm/year vs (4.21 ± 0.99) cm/ year],HtSDS(-1.16 ±0.83 vs-3.00 ±0.71),and PAH[(163.68 ±6.76) cm vs (156.54 ±7.39) cm] were significantly higher than those before treatment (F =110.3,30.47,26.20,all P ＜ 0.01).Similar changes were observed in low dose group except for PAH.In high dose group after 2 years of treatment,IGF-1,IGFBP-3 were significantly higher than those before the treatment and the difference was statistically significant (all P ＜ 0.05).Although the plasma levels of IGF-1 and IGFBP-3 in low dose group in 2 years of treatment were significantly higher than those before the treatment,the difference was not statistically significant (P ＞ 0.05).Compared with that before treatment,the added value of IGF-1 had a positive correlation with the added values of growth rate,HtSDS and PAH(r =0.567 4,0.652 4,0.584 3,0.499 8,all P ＜ 0.05).Similar observations were found in low dose group (r =0.437 1,0.405 6 and 0.501 1,all P ＜ 0.05).However,the added value of IGF-1 in low dose group had no correlation with PAH (r =0.200 8,P ＞ 0.05).Compared with that before treatment,2 groups had no differences in fasting and postprandial blood glucose,insulin,TSH,T3,T4 and glycosylated hemoglobin (all P ＞ 0.05).Conclusions Recombinant human growth hormone [0.20 IU/(kg · d)] may significantly increase the growth rate and PAH of short children born SGA,which is a safe and effective strategy for the treatment of short SGA.

The paper summarizes application of new media in patient informatization services in hospitals, introduces exploration and practices made by Children′s Hospital of Shanghai on new media informatization platform construction from such aspects of OA system-basedwindowservice platform,order typeservice model provided to the public onMicroblogandWeChatplatforms andex-press typemobile network terminal service for hospital staffs.The service effects are summarized at last.

In recent studies some urea derivatives have been identified as potent anti-tuberculosis agents by targeting mycobacterial membrane protein large 3 (MmpL3). However, this compound series as exemplified by AU1235 exhibited poor in vitro pharmacokinetic profile. With AU1235 as the lead, we have identified a novel benzimidazole series as potential anti-tuberculosis agents by using scaffold hopping approach. Among these synthesized compounds, 2-aminobenzimidazole derivative 8b showed the potent anti-tuberculosis activity with the MIC value of 0.03 microg x mL(-1). This compound also showed improved metabolic stability compared to AU1235. Our investigation indicated that benzimidazole derivatives are the promising lead for further optimization as anti-tuberculosis agents.