Best vitelliform macular dystrophy ( BVMD ) is an autosomal dominant disease mostly caused by mutations in BEST1 gene. These mutations change the normal physiological functions of BEST1-encoded bestrophin-1 protein, and finally lead to a reduction of visual acuity. This review is composed of the following aspects: the structure and functions of BEST1 gene, the characteristics of the mutations, clinical features of BVMD, genotype-phenotype correlations as well as possible gene therapy. Our contribution serves for further research on BVMD and BEST1 gene.

Homocysteine ; pharmacology ; Human Umbilical Vein Endothelial Cells ; drug effects ; metabolism ; Humans ; Lipid Peroxidation ; drug effects ; Metallothionein ; pharmacology

Objective:To explore the characteristics of human melanoma-specific antigen peptides by HLA-A2 restricted tumor-infiltrating lymphocytes.Methods:The HLA-A2 protein and polypeptides molecules were purified from the three tumor cell lines(624-Mel, Chap-Mel and JY) by immunoaffinity chromatography, after the peptides bound to HLA-A2 protein solution were acidified with acetic acid and boiled by high temperature, and centrifuged through an Ultra-CL filter, then the peptides extracts were fractionated by revered phase high pressure liquid chromatography(RP-HPLC). Individual fractions were assessed for their ability to reconstitute melanoma-specific epitopes by adding to the HLA-A2 Ag-procceing mutant cell, T2. The biological feature of one of three active peptides from RT-HPLC samples was performed by mass spectrometric analysis. The synthetic peptides identical to active peptide sequences were determined in the reconstitute test.Results:Three prominent peaks(P19, P25 and P31) of the fraction from 624-Mel were observed in the reconstitute test, TIL killing rate was 67% for (P31) peptide fraction. The mass spectrometric analysis of one of active peptides (P31) showed that at mass-to-charge ratio(m/z) 948 has been usually nine residues. The sequence is H+ Ala Lue Trp Lue Phe Phe Gly Val Lue OH-. The peptide synthesized comprising epitopes were verified.Conclusion:These results showed the peptides derived from active fractions were related to human melanoma-specific tumor antigen peptides recognized by HLA-A2-restriced TIL. These peptides could develop novel peptide-based an anti-tumor vaccine for immunotherapy of CTL.

Adolescent ; Adult ; Drug Therapy, Combination ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Hepatitis B, Chronic ; drug therapy ; Humans ; Injections, Intravenous ; Male ; Middle Aged ; Phytotherapy ; RNA ; administration & dosage ; Salvia miltiorrhiza ; chemistry

BACKGROUND: It is necessary to keep a good cervical curvature for improving nerve symptom in patients with cervical spondylotic myelopathy (CSM). It is verified that cervical anterior plate internal fixation implant is effective to restore the physiological curvature of cervical vertebra.OBJECTIVE: To assess the physiological lordosis of cervical vertebra in CSM patients after cervical anterior plate internal fixation implantation.DESIGN: Observational study.SETTING: First Affiliated Hospital of Nanchang University.PARTICIpANTS: Totally 72 CSM patients were enrolled at Department of Orthopaedics, First Affiliated Hospital,Nanchang University from February 2003 to January 2006, including 46 males and 26 females, aged 45-70 years,averagely 54 years, with the history of 2-6 years. Inclusive criteria included ①cervical spinal cord compression shown in MRI, and ②using autogenous iliac bone. The experiment was approved by Hospital Ethics Committee, and all patients signed the informed consent. Cervical anterior plate was Orion locking plate offered by Shufamo Company. There were single segmental discectomy group (n =32) and two-level segmental discectomy group (n =40) according to experimental requirement.METHODS: ①Anterior decompression plus autogenous iliac bone implant plus titanium plate internal fixation were utilized.The patients were in supine position; shoulder and back were blocked up; neck backward, and endotracheal tube was done under general anesthesia. Transverse or oblique incision was made at right anterior neck. Discectomy decompression was performed with Caspar vertebra ecarteur. Three-dimensional autogenous iliac bone was implanted in decompressed intervertebral space. Immediate stability was obtained after Orion locking titanium plate was fixed, with placement drainage, and then the incision was sutured. ②Postoperative daily activities were conducted with cervical gear, which was removed 8 weeks later. ③Lateral cervical vertebrae X-rays were taken before operation, immediately after operation, 6 and 12 months after operation. Lordosis of the fusion segment was measured with Cobb's angle, and lordosis of cervical vertebra was assessed with D value. These data were analysed by statistic software. ④Bone fusion of the patients was followed up for 12 months after operation. Vertebral fusion standards included no abnormity between two spinous processes of the fusion segment, no lucency between implant and vertebra, as well as bone trabecula across the interface between implant bone and vertebra. ⑤Biocompatibility between material and host was observed during follow-up.MAIN OUTCOME MEASURES: ①Changes of Cobb's angle and D value at different time points before and after operation,and ②Bone fusion and rejection were followed up.RESULTS: Totally 72 patients were involved in the result analysis. ①Cobb's angle was larger and D value was higher immediately after operation, 6 and 12 months after operation than those before operation in the single segmental discectomy group and the two-level segmental discectomy group (P＜0.05). ②Findings of 12-month follow-up showed that bone fusion appeared in all patients of the two groups. ③No significant rejection was found after titanium plate internal fixation implantation. It was found that material and host had a good biocompatibility.CONCLUSION: It is satisfactory to restore physiological lordosis of cervical spine by using cervical anterior plate internal fixation plus autogenous iliac bone for CSM.

AIM To study the therapeutic effects of Deketoprofen trometamol (D KPT) on adjuvant arthritis (AA) and its side effects on gastroduodena. METHEDS The change of primary and secondary inflammatory and the injury effect of gastroduodena treated with D KPT were observed. The effect of D KPT on prostaglandin E 2 (PGE 2) produced by peritoneal macrophage (PM) on AA rats in vitro was also observed. RESULTS D KPT (2 5, 5, 10 mg?kg -1 ) significantly inhibited primary inflammatory, secondary inflammatory and multiple arthritis of AA rats. D KPT(10 -8 ,10 -7 ,10 -6 ,10 -5 ,10 -4 mol?L -1 )inhibited the elevated PGE 2 released from PM of AA rats in vitro . D KPT (10 mg?kg -1 ) significantly injured gastroduodena on AA rats, but the degree of injuries was less than that of ketoprofen (KP) (10 mg?kg -1 ). CONCLUSION D KPT has therapeutic effects on AA rats, and its injury effect on gastroduodena is less than that of KP.

Objective To understand the current status of hypertension in Jingzhuang Town,Yanqing District,Beijing. Method With randomized cluster sampling,3 653 residents aged over 30 yrs old were investigated by a standard questionnaire on hypertension epidemiology.Result The prevalence rate of hypertension in Jingzhuang Town was 33.3%,and increased with the age(P0.05).The main patients were those with degree 1 hypertension(62.8%).There were 37.8%of people with normal blood pressure who had high-normal values,the highest(44.3%)in 60～69 yr group.In population with hypertension,the rate of people taking medication was 24.5%,disease control rate was 8.6%,while in patients aged 30～49 yrs,these rates were 7.1%and 2.9% respectively.Conclusion The prevalence of hypertension in Beijing's suburb was serious with a younger trend.There was a large part of people with high-normal blood oressure.

Objective To explore the feasibility of using composite scaffolds of rabbit oral epithelial cells and polycaprolactone (PCL) electrospun fibers for urethral repair. Methods The 25%PCL was prepared using a 5:1 by volume mixture of trichloromethane and anhydrous methanol, and PCL fiber tubular scaffolds were obtained by electrospinning. Rabbit oral mucosa epithelial cells (1.5 × 105) were implanted on the PCL scaffold. Subsequently, they were embedded in nude mice subcutaneous, explanted in 2 weeks. PCL fiber tubular scaffolds without rabbit oral mucosa epithelial cells were used as control. The complex urethral scaffolds were evaluated by immunofluorescence staining with cytokeratin antibody and HE staining. Results Compared with blank PCL group, the rabbit oral mucosa epithelial cell group showed a good cellularization. Rabbit oral mucosa epithelial cells formed a dense cell layer on the surface of PCL lumen, which suggested that rabbit oral mucosa epithelial cells can proliferate on the surface of PCL lumen. Conclusion Rabbit oral epithelial cells can be used as one of the seed cells for tissue engineered urethral scaffolds, and it is possible to construct tissue engineering substitute materials for urethral repair by rabbit oral epithelial cells combined with PCL.

AlM: To observe the treatment efficacy of eyelid herpeszoster with external use ganciclovir ophthalmic gel.METHODS:A total of 77 patients were randomly divided into treatment group and control group. ln the treatment group, ganciclovir ophthalmic gel was topical coated on the basis of antiviral drugs, while the control group was coated with conventional acyclovir ointment and antiviral treatment.
RESULTS: The cure rate was 95% in the treatment group, and the control group was 83% after 10d treatment. The difference was significant.
CONCLUSlON: The efficacy of ganciclovir ophthalmic gel treated eyelid herpes zoster is obvious.

Traditional Chinese ancient prescriptions have been used for treatment of liver cancer for a long history and the scientific and rational compatibility is a great wealth for modern research and development (R&D) of new drugs. The research and development of new drugs are often accompanied with a large investment, a long cycle and a high risk, especially for the anti-tumor drugs R&D which are facing more risks and lower successful rate. In this research, the regularity of compatibility of drugs was analyzed from 124 anti-hepatoma ancient prescriptions by computer program. The results can offer help to the R&D of anti-hepatoma new drugs and reduce the risk of drug screening. In addition, we surveyed 22 companies in this field from six provinces such as Beijing, Shanghai, Tianjin and so on and obtained 240 risk assessment questionaires. Then we used qualitative analysis method to interpret the greatest impacts for the risks in the process of R&D, production and sales of anti-hepatoma new drugs. The study provides a basis for anti-liver cancer drugs R&D researchers, who can take effective measures to reduce the R&D risks and improve successful rate.
Carcinoma, Hepatocellular ; drug therapy ; history ; China ; Drug Discovery ; history ; Drug Incompatibility ; Drug Prescriptions ; history ; Drugs, Chinese Herbal ; history ; therapeutic use ; History, Ancient ; Humans ; Liver Neoplasms ; drug therapy ; history ; Research ; history