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 wound healing of semi-toe nail flap donor site after transplantation. Methods To repair small wounds,on 13 toes the first metatarsal dorsal flaps were applied,and on 7 toes the second toe digital flaps were applied.While,to repair large wounds,free flaps were applied,including foot inside flaps on 3 toes,peroneal artery perforator flaps on 5 toes,tarsal lateral artery flaps on 2 toes,and phil bowel artery inside branch flaps on 3 toes.Wounds remaining on semi-toe nail flaps of 7 toes were all covered with fibular island flaps of the first toes. Results Twenty cases were followed up,and the rang between 6 months to 5 years.The nail margin was exposed out of the lateral flaps in 2 cases.The first toes and the second toes appeared nearly normal in the other 18 cases.The flaps appeared smooth,flexible appearance with nice color and no ulceration.Seven cases applied with free flaps recovered protective sensibility with no complaint. Conclusion To cover wounds on semi-toe nail flaps of the first toes and the second toes with flaps is an ideal treatment method.The method of free flap transplantation is superior to vascular pedicle flap graft.

Objective:To describe the doctors' satisfaction of the doctor-patient relationship and find out the influencing factors of the patients,gathering evidence to improve the doctor-patient relationship.Methods:This study was a cross-sectional study,in which doctors and nurses in 10 hospitals of Beijing,Shandong and Chongqing were surveyed with structured questionnaires and in-depth interviews.Results:The mean score of the doctors' satisfaction of the doctor-patient relationship was 59.97,which was much lower than the patients'.The patients' socio-demographic characteristics,social economic status(SES) and behavior characteristics influence the interaction of the doctors and the patients.The doctors' satisfaction of the doctor-patient relationship was influenced by the patients' trust.Conclusion:The doctors' perspective is helpful to define the tension and the cause of the doctor-patient relationship.The patients' characteristics have important influence on the doctor-patient relationship.It's necessary to take action on the patients to improve the doctor-patient relationship.

106 ethmoidal specimens ranging from 12 to 40 weeks of embryonic age were cut into serial sections and observed by histological method. The results showed that the ethmoid sinus began its development at about 21-23 weeks of fetal life. The frontal folds or furrows were formed by extensions or evaginations of the nasal mucous membrane from the superior and lateral wall of the middle and superior nasal meatuses. The ethmoid cells appeared at 24-26 weeks and they kept their own small ostia that opened into the middle and superior nasal meatuses. The number and size of the ethmoid cells were increased with the age. The cell cavities showed balanced enlargement. At birth each ethmoid labyrinth had 6-11 cells, measuring on the average 1.7?1.3?2.0 mm~3 in size. The distribution of the ethmoid cells approached those of mature age. The epithelium of the ethmoid sinus was cuboidal or low columnar in shape. Cilia appeared sparse. Tunica propria, mainly composed of the connective tissue and its stroma, was thick, loose, and less vascular and glandular. It suggested that the ethmoid sinus was one of the accessory nasal sinuse that developed first. The ethmoid sinus presented at birth and its appreance were not synchronous, but their location were relatively invariable. The histological structure of the mucous membrane in the ethmoid sinus was somewhat different from those of the nasal mucous membrane. It showed that the development of the ethmoid mucous membrane was still imperfect in the newborn.

To investigate the changes in the activated T-lymphocyte CD3/HLA-DR and CD3/CD(16+56) populations in peripheral blood of the patients with allogeneic hand transplantation, lymphocytes from peripheral blood of the patients at different time points were immunologically labeled with dual color fluoresecent monoclonal antibodies CD3/CD(16+56) and CD3/HLA-DR, mono-color fluoresecent monoclonal antibody CD25. CD25, CD3/CD(16+56), and CD3/HLA-DR were determined with flow cytometry (FCM). The levels of activated T-lymphocyte (CD25 +,CD3 +/HLA-DR + ), silent T-lymphocyte [CD3 +/CD(16+56) -,CD3 +/HLA-DA - ] decreased significantly during the first week after transplantation and then increased gradually to the pre-operafive level. Nature killer cells [CD3 -/CD(16+56) +] increased significantly at the first day after transplantation, then decreased sharply and maintained a lower level. The results suggest that immunosuppressive agents have significantly effects on lymphocyte subsets in allogenaic hand transplanted patients, and dynamic determination of HLA-DR, CD3 /CD(16+56) could be valuable in immunomonitoring after allogeneichand transplantation.