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

Background Corneal blindness caused by ocular surface disease is one of the main reasons for the global blinding corneal diseases.With the development and progress of tissue engineering technology,tissueengineered cornea offers a new approach to the treatment of ocular surface disease.Objective This study was to obscrve the growth and differentiation of human umbilical cord mesenchymal stem cclls (UC-MSCs) on thc corneal stroma of receipts and investigate the feasibility of human UC-MSCs differentiated into corneal epithelium-like cells and the reparation of injury cornea.Methods Human UC-MSCs were isolated from human umbilical cord using collagenase Ⅳ digestion and passaged in DMEM/F12 containing fetal bovine serum in vitro.The immunophenotype of cultured human UC-MSCs was evaluated by flow cytometry.The differentiated osteoblasts from the human UC-MSCs by directional induce was identified.Twenty-four New Zealand albino rabbits were randomly divided into 2 groups.The human UC-MSCs were cultured on porcine corneal matrix without corneal epithelium for 4 days and then transplanted onto the 12 left eyes of 12 New Zealand albino rabbits,and porcine corneal matrix without corneal epithelium was transplanted onto the left eyes of other 12 New Zealand albino rabbits as control group.The rabbits received keratoplasty were examined using in vivo confocal microscope through focusing(CMTF).The eyeballs were taken off after 2,4 and 8 weeks,the growth and differentiation,expression of cytokeratin 3 (CK3),CK12 and ATP-binding cassette superfamily G memben 2 (ABCG2)of human UC-MSCs were observed by histopathology and immunofluorescence staining.This use of the experimental animals complied with ARVO Statement.Results Digestive human UCMSCs formed round in shape and was large in size.The attached cells displayed long-fusiform shape like fibroblasts.The cultured human UC-MSCs phenotype was CD105+/CD29+/CD44+/CD34-/CD45-and could be induced toward osteoblast differentiation under the appropriate experimental conditions.Human UC-MSCs grew well on the porcine corneal matrix.The corneal grafts survived wcll without rejection till the experiment end in experimental eyes,but the rejection of corneal graft occurred in control eyes.Confocal microscope could observe corneal epithelium-like cells.The corneal epithelium cells showed the positive response for CK3 and CK12 and absent response for ABCG2.Conclusions Human UC-MSCs with porcine corneal matrix can survive,proliferate and differentiate into corneal epithelium-like cells after transplanting onto the corneal stroma of rabbits.This result suggests that human UC-MSCs is able to repair and reconstruct the injured corneal surfaces.

Objective To determine the correlation of plasma brain natriuretic peptide(BNP) with severe degree of dilated cardiomyopathy in children.Methods Thirty children with dilated cardiomyopathy and 30 healthy subjects were selected in this degree of study, plasma BNP concentration was measured and compared among groups by using t test.Correlation of BNP levels with left ventricular ejection fraction and heart function was investigated using linear regression analysis.Results Children with dilated cardiomyopathy had significantly higher mean BNP levels compared with healthy children [(429.4?270.2) ng/L vs (67.0?10.2) ng/L].Significantly positive correlations were found between BNP and heart classification(r=0.950 P