Congresses as Topic ; Humans ; Lysosomal Storage Diseases

Adenosine Triphosphatases ; genetics ; Alanine Transaminase ; blood ; Cation Transport Proteins ; genetics ; Ceruloplasmin ; metabolism ; Child ; Copper ; blood ; urine ; Copper-transporting ATPases ; Cornea ; pathology ; Diagnosis, Differential ; Genetic Testing ; Hepatolenticular Degeneration ; blood ; diagnosis ; genetics ; metabolism ; Humans ; Liver ; metabolism ; pathology ; Liver Function Tests ; Mutation

Objective:To study the clinical effect of etanercept combined with methotrexate in the treatment of rheumatoid arthritis ( RA) . Methods:Totally 62 patients with RA from May 2014 to May 2015 in our hospital were divided into the observation group and the control group with 31 cases in each according to the random number table. The control group was treated with methotrexate (10mg, po, once a week), the observation group was treated with etanercept (25 mg, twice a week) additionally, and the treatment course was 12 weeks. Before and after the treatment, serum TL1A, TNF-a, IL-6 and IL-17, time of morning stiffness, joint swelling, tenderness and pain change scores in the two groups were studied and compared. The clinical efficacy and adverse reactions of the two groups were analyzed as well. Results:After the theatment, compared with before theatment, two groups’ TL1A, TNF-a, IL-6 and IL-17 had de-creased, and observation group was lower than control group(P<0. 05). Two groups’ time of morning stiffness have shortened, and the joint swelling, tenderness and painscores were significantly decreased, and each index in the observation groups were better than control group(P<0. 05). The total effective rate of the observation group was higher than the control group(P<0. 05). There was no significant difference about adverse reaction in the two groups(P>0. 05). Conclusion:Etanercept combined with methotrexate in the treatment of RA can reduce serum levels of TL1A, TNF-a, IL-6 and IL-17, and significantly alleviate joint swelling, tenderness and pain symptoms with high safety.

The (GCT)o polymorphism of the MICA gene was investigated in 100 patients with latent autoimmune diabetes in adult (LADA) and 145 healthy controls by PCR and denaturing polyacrylamide gel electrophoresis.The A5.1 allele was present at a significantly higher frequency in LADA group (0.340) than that in control group (0.183) (P

Aim This study examined the uptake and transport of ecdysterone(EDS) using Caco-2 cell monolayers as a model of human intestinal mucosa.Methods Two kinds of Caco-2 cell monolayer model(Caco-2 cell monolayers;CYP3A4 expressing Caco-2 cell monolayers) were set up to study the uptake and transport of EDS.Results Compared bi-directional charaterizaton of Caco-2 cell,the apparent permeability coefficients(Papp) values of EDS were between 0.1?10-6 cm?s-1 and 1?10-6 cm?s-1.EDS absorption was 1%~10% for two kinds of Caco-2 models.The RPapp values were all less than 1.5 for 4 h.Conclusions The uptake and transport of EDS was a passive transcellular diffusion mechanism.Ecdysterone was not influenced by CYP3A4 mediate mechanism.

Objective To explore the cause and effective management and prevention of septic shock after minimally invasive percutaneous nephrolithotomy (MPCNL) in the elderly.Methods Clinical data of 13 patients with septic shock after MPCNL in our hospital were retrospectively analyzed,and the cause,prevention and treatment of septic shock were investigated.Results The incidence of septic shock in patients undergoing MPCNL was 3.54％ in this study.Single-factor analysis and logistic regression analysis showed that the risk factors for post-MPCNL septic shock were female and the history of diabetes (both P＜0.05).Conclusions Septic shock is a serious complication after MPCNL.Intensive perioperative prevention measures can reduce the incidence of septic shock.Early diagnosis and proper treatment are the keys to successful treatment of septic shock.

CXCR4 has long been considered as the unique receptor of CXCL12,and CXCL12/CXCR4 axis plays a prominent role in tumorigenesis.However,a novel receptor for CXCL12,named CXCR7,has been recently identified and also plays an important role in tumorigenesis.This review summarized current studies regarding the functions of CXCR4 and CXCR7 in cancer and the recent therapeutic approaches that target these receptors or their ligands.

Objective To investigate the current demand of expectations of family members of children in transfusion room. Methods By means of a self-designed questionnaire, the survey researched the demands of family members of children from 5 dimensions,the waiting time, transfusion environment, the quality of nursing,humanistic care and health education, and drug and safekeeping of security.The total 401 valid data were analyzed with descriptive statistics, chi square test, single factor variance analysis. Results No significant difference was found on their perception for the waiting time (χ2=1.051, P>0.05) and appropriate waiting time ( χ2=0.883, P>0.05). But there were significant differences between medical guarantee types and environmental needs (F=2.972, P<0.05 ), as well as between monthly income and the humanistic care and health education demand(F=2.566, P<0.05). Conclusion The expectation of respondents are diverse, changeable, showing high consistency for basic expectation,differences between different groups, discontinuity in different phase.

Ring finger protein 43 (RNF43) is a ring-type E3 ubiquitin ligase.As a negative regulater of Wnt signaling pathway, RNF43 has an important anti-tumor effect.The mutation of RNF43 may cause abnormal activation of Wnt signaling pathway, and then promote invasion, metastasis and proliferation of tumor cell.In addition, the act of RNF43 protein in the Wnt signal pathway is expected to be a molecular target in the therapy of cancer.In recent years, with the gradual deepening of related research, the molecular structure of RNF43 protein and its mechanism of action with the Wnt pathway-related proteins have been gradually clear.In clinical, RNF43 protein analogs and related vaccine also show the important position in the therapy of cancer.