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.

ObjectiveTo observe the effect of early bilateral limbs rehabilitation on nerve function of stroke patients.Methods60 cases of acute stroke were divided into unilateral group and bilateral group with 30 cases in each group. Cases in bilateral group were treated by training healthy side and sick side limbs alternatively and electric stimulating bilateral limbs at same time. Cases in unilateral group were treated only on sick limbs (both training and electric stimulating). After 4 weeks treatment, neurological deficit evaluation (NDE), motor function and activities of daily living of all cases in two groups were assessed.ResultsAfter treatment, scores of NDE, Fugl-Meyer Motor Assessment Scale and Barthel index of patients of bilateral group were 9.9±4.52, 71.21±22.43 and 78.6 ± 20.17 respectively. Those of patients of unilateral group were 13.46±9.2, 52.21±17.68 and 54.2±16.34 respectively. There was a significant difference between two groups (P<0.01) after 4 weeks treatment.ConclusionEarly rehabilitation can improve nerve function of stroke patients and the effect of bilateral limbs rehabilitation is better than that of unilateral limbs rehabilitation.

The oil content and fatty acid composition of Ganoderma lucidum collected from different producing areas, varieties, tissue types and growth periods were measured and analyzed. The results showed that the oil content was 23. 61%-34.17% in different domestic producing areas of China; the oil content of fruiting bodies from major varieties cultured in Zhejiang province were 0.81%-1.87%, wall-unbroken spores were 0.07%-0.24%, wall-broken spores were 27.54%-34.17%, so the oil content of wall-unbroken spores were much higher than fruiting bodies, and wall-breaking treatment would increase the oil extraction rate 150-340 times. G. lucidum spores oil was mainly composed of unsaturated fatty acid composition. oleic acid and linoleic content were 53.26%-58.16% and 10.69%-16.87% respectively. Fatty acid composition ratio of spores and fruiting bodies were significantly different by PLS-DA. Determining the composition of fatty acid, especially the content of oleic acid, stearic acid and palmitic acid, could identify the tissue types of G. lucidum products' sources. In addition, the study result showed that the spores and fruiting bodies collected in the first year contained richer oil and fatty acid than second year's samples from the same variety of G. lucidum.
Fatty Acids ; analysis ; Oils ; analysis ; Reishi ; chemistry

Chromatography, Gas ; methods ; Humans ; Toluidines ; blood

Objective To investigate the clinical effects of high-density micropulse photocoagulation (HD-SDM) combined with intravitreal injection of ranibizumab for diabetic macular edema (DME).Methods Thirty-one patients (31 eyes) with DME were randomly divided into two groups.Group A (15 eyes) received HD-SDM combined with intravitreal injection of ranibizumab.Group B (16 eyes) only received intravitreal injection of ranibizumab.The best corrected visual acuity (BCVA) and central macular thickness (CMT) of the two groups before and after treatment were analyzed,and the annual injection times of the two groups were compared.Results The average annual injection times was 3.67 ± 1.11 in group A,and 9.12 ±2.63 in group B.The difference was significant between the two groups (t =2.05,P ＜ 0.05).There were significant differences in CMT before and after treatment in both groups (all P ＜ 0.05).There was no significant difference in CMT between the two groups(t =1.19,P ＞ 0.05).There were significant differences in BCVA before and after treatment in both groups (all P ＜ 0.05),but there was no significant difference before and after treatment between the two groups(all P ＞ 0.05).Conclusion Both HD-SDM combined with intravitreal injection of ranibizumab and single intravitreal injection of ranlbizumab are effective for DME,but the combining treatment can remarkably decrease the annual injection times and had a good compliance of patients,is a good choice for DME patients.

OBJECTIVE:To compare in vivo and in vitro permeation behaviors of the ethosome gels of testosterone,testoster-one propionate and testosterone undecanoate. METHODS:The ethosome gels of testosterone,testosterone propionate and testoster-one undecanoate were prepared. With cumulative permeating amount and permeation rate as the indexes,Franz diffusion cell and HPLC were employed to compare in vitro permeation behaviors of 3 kinds of ethosome gels in mouse skin. With testosterone patch as the positive control drug, electrochemistry method was adopted to detect the concentration of testosterone in plasma 0,3,6, 9,12,24,36 and 48 h after applying such 3 kinds of ethosome gels on the back of rats,and then pharmacokinetic parameters were calculated with DAS 2.0 software. RESULTS:24 h cumulative permeating amounts of the ethosome gels of testosterone,tes-tosterone propionate and testosterone undecanoate were(234.31±13.8),(175.63±41.1)and(72.60±15.3)μg/cm2,and the per-meation rates were(10.25±1.9),(7.64±1.4)and(2.96±0.8)μg/(cm2·h),respectively. The pharmacokinetic parameters of the above-mentioned three kinds of ethosome gels and the positive control drug were respectively as follows as cmax of(20.19±2.57), (17.50±2.91),(0.23±0.04),(14.97±2.12)ng/ml,t1/2Ka of(2.80±0.45),(3.36±0.59),(4.02±0.62),(4.20±0.71)h,AUC0-48 h of(13.85±1.96),(13.93±2.13),(0.35±0.07),(11.76±2.31)ng·h/ml. CONCLUSIONS:in vivo and in vitro permeation behav-iors of the ethosome gels of testosterone and testosterone propionate are fairly good.

Objective To evaluate the effect of acupuncture point stimulation on the urodynamics of spinal cord injury patients.Methods Sixty spinal cord injury cases were divided into a research group and a comparison group.Both groups were given bladder training, but the research group also received acupuncture point stimulation.The subjects' maximum cystometric capacity, maximum detrusor pressure and residual urine were measured, along with renal function and any urinary tract infections.The scale of activity of their daily lives was compared before and 3 months and 6 months after the treatment.Results There were no statistically significant differences between the groups before the treatment.There were statistically significant differences in maximum cystometric capacity, maximum detrusor pressure, residual urine and Barthel index (BI) after 3 and 6 months of treatment.In the research group, maximum detrusor pressure, residual urine and BI scores had all improved after 6 months compared with the 3 month values.In the comparison group only the BI score had improved significantly between 3 and 6 months, and this was not reflected in a significant change in the scale of activity in daily life.After 3 months, only the average BI differed significantly between the two groups.After 6 months all indexes except the BI and maximum cystometric capacity differed significantly.There were also significant differences between the groups in reflex voiding, percussion voiding, and abdominal pressure voiding.Conclusions Acupuncture point stimulation can improve the bladder function of spinal cord injury patients.It can also decrease the incidence of urinary tract infection and the possibility of renal dysfunction.

OBJECTIVETo understand the changing trend on the way of delivery since 1970s and its related factors that influencing the attitude of choice on Cesarean section (C-section) in women at child-bearing age.

METHODSA face-to-face interview was conducted anonymously in pregnant and lying-in women visited at the out-patient department of Gynecology and Obstetrics, Tiantan Hospital of Beijing. Totally, 415 women at child-bearing age, with a history of previous birth were interviewed on date, place and way of delivery of last birth, as well as on information that could have had impact on the choice of C-section.

RESULTSThe average rate of C-section in Tiantan Hospital had been 29% since the year of 2000, much higher than that during 1970s, 1980s and 1990s (chi(2) = 22.81, P = 0.001) which showed an increasing trend. Rate of C-section among lying-in women with native Beijing origin was 25.0%, significantly higher than 9.6% (chi(2) = 21.96, P = 0.000 002) that in the migrants. Lying-in women with education level of high school or above had higher chance to choose C-section than those with lower level of education (chi(2) = 43.64, P < 0.000 01). Workers, managerial staff or clerks had more chance to choose C-section than those with other occupations (chi(2) = 20.07, P = 0.01). As reported by the interviewees, 93% (70/75) of C-section in the hospital were performed and recommended by obstetricians.

CONCLUSIONRate of C-section in the hospital showed an increasing trend which suggested that intervention with health education be carried out for both pregnant women and obstetricians.

Adult ; Cesarean Section ; statistics & numerical data ; China ; epidemiology ; Delivery, Obstetric ; statistics & numerical data ; Female ; Humans ; Pregnancy ; Surveys and Questionnaires

The simulator testing in vitro and computational simulation of the artificial knee joint wear are important methods to evaluate the wear performance of the prosthesis in vitro and to predict the clinical performance of knee joint products. Based on the method of literature search, this paper compares the mechanical and kinematic loading input curves carried out by Chinese scholars in recent years, standard curves, and Chinese measurement curves of two typical movements of gait. Data of vitro simulator test and computational simulation model are compared, summarized, and analyzed. The results show that the measured data of motion and load cannot be directly used as the loading conditions for the simulator wear test and computational simulation. The mechanics and kinematics data of Chinese people are different from the international standards. The domestic artificial knee joint in vitro simulator wear test methods are similar but the results of different test institutions are somewhat different. The computation wear prediction research is basically synchronized with foreign countries, but the problem that the calculated wear results are lower than that in vitro test is still unsolved. The artificial knee joint wear performance evaluation system based on Chinese knee joint mechanics and kinematics data is the forward direction of the research.

Objective To choose zebrafish as the experimental animal model for studying the spatiotemporal expression rule of rap1 gen in zebrafish embryo early development process .Methods The Rap1 gene fragment was cloned from the zebrafish emby‐oscDNA ,then the Rap1 gene fragment and pCS2+ plasmid were performed the in vitro connetion and recombination was extracted , the combinant plasmid was correct after the double enzyme digestion ,colony PCR and sequencing identification .T3 RNA polymer‐ase in vitro transcription system was used to obtain the digoxin (DIG)‐labeled anti‐sense mRNA probe of Rap1 gene .The whole mount in situ hybridization method was adopted to detect the Rap1 expression in zebrafish embryo early development process . Results The positive hybridization signal of Rap1 gene was detected at the cell division junction region of 0 .75 hpf ,animal pole of 3 .70 hpf and 6 .00 hpf ,and notochord of 12 .00 -72 .00 hpf .Conclusion Rap1 gene might be involved in the early development process of notochord nervous system in zebrafish .