Background The main cause of X linked juvenile retinoschisis is mutation of RS1 gene.The phenotype of X linked juvenile retinoschisis is associated with the mutation types of RS1 gene.However,the relationship of genotype and phenotype of X linked juvenile retinoschisis is unclear.Objective The present study was to survey the clinical phenotype of X-linked juvenile retinoschisis in twelve Chinese families with eleven different mutations in the XLRS1 gene. Methods Complete ophthalmic examinations with slit lamp biomicroscopy,fundus examination and Dhotography were carried out in 28 affected males.Ganzfeld electroretinography (ERG),fundus fluorescein angiography,A and B-scan standardized echography and optical coherence tomography(OCT)were also performed in some patients.The coding regions of the XLRS1 gene that encodes retinoschisin were amplified by polymerase chain reaction(PCR)and analyzed by the single strand conformation polymorphism(SSCP)assay.The RS1 gene mutations were determined by direct sequencing in an automated sequencer.Written informed consent wasobtained prior to the survey. Results The 28 affected males showed a typical foveal schisis with or without peripheral retinoschisis.The typical response to white single flash ERG was seen with a reduction of the b-wave amplitude and a relative preservation of the a-wave amplitude.causing a reduced b/a ratio in the male patients.A total of eleven different XLRS1 mutations in 12 families were identified,four of these mutations,including one frameshift mutaion(22 del T)of exon 1,Asp145His,Arg156Gly and Trp163X mutations of exon 5,were first described in this survey.One non-disease-related polymorphism(NSP),or the 576C to T(Pro192Pro)change of exon 6 was also newly reported herein.In the families with a frameshift(22 del T)mutation of exon 1,a splice donor site mutation(IVS1+2T

AlM: To compare the results of 4 methods for measuring angle of exodeviation in the three types of intermittent exotropia, including when looking at indoor distance target of 6m, looking at indoor distance target of 30m, looking at outdoor far distance target, after 1h diagnostic occlusion test.
METHODS: Prospective case series study. Sixty-five patients with intermittent exotropia between June 2013 and June 2014 were enrolled in the Department of Ophthalmology, Affiliated Hospital to Qingdao University, included 37 males and 28 females with average age ( 12. 5 ± 6. 2 ) years. All the patients were measured when looking at indoor distance target of 6m, looking at indoor distance target of 30m, looking at outdoor far distance target, after 1h diagnostic occlusion test. lntermittent exotropia was divided into basic type, convergence insufficiency type and divergence excess type, which was based on the different result of between the distance and near measurements. The One-way test was applied to analyze the four methods of measuring angle of exodeviation in the three types of intermittent exotropia. LSD - t test was applied to compare the differences between each two methods in each type.
RESULTS: The distance exodeviations tested with looking at indoor distance target of 6m, looking at indoor distance target of 30m , looking at outdoor far distance target, after 1h diagnostic occlusion test were basic type (45. 4 ± 21. 0, 55. 0 ± 15. 0, 64. 68 ± 17. 7, 68. 75 ± 16. 6PD), convergence insufficiency type (33. 3 ± 14. 0, 44. 9 ± 12. 9, 43. 6±11. 8, 54. 6±11. 2PD), divergence excess type (55. 6± 17.4, 66.3±18.8, 76.9±16.4, 78.1±15.6PD). There were obviously differences between each two methods in each type ( basic type F = 9. 649, P = 0. 00; convergence insufficiency type F=6. 886, P=0. 001; divergence excess type F = 7. 989, P = 0. 00 ). Compared with looking at indoor distance target of 30m, looking at outdoor far distance target ( basic type P=0. 044, divergence excess type P = 0. 048 ) and after 1h diagnostic occlusion test (basic type P=0. 04, divergence excess type P=0. 027) had the statistical difference in the basic type and divergence excess type, and there was no obviously difference between looking at outdoor far distance target and after 1h diagnostic occlusion test ( basic type P=0. 353, divergence excess type P=0. 815). Compared with the other three measurements, 1h diagnostic occlusion test can elicit larger angle of deviation in the convergence insufficiency type.
CONCLUSlON: Both measurement with looking at outdoor far distance target and after 1h diagnostic occlusion test can elicit the larger angle of deviation in the basic type and divergence excess type; The measurement with after 1 hour diagnostic occlusion test can elicit the larger angle of deviation in the convergence insufficiency type.

Objective To investigate the cytolytic activity of extracellular cytolytic toxin rVVC of Vibrio vulnificus on the apoptosis of human ECV304 cells, and to analyze the activities of Caspase-3,-8 and -9. Methods The cytotoxic effect of refolded rVVC on the growth and apoptosis of ECV304 cells was identified by MTT, Hochest33342/PI fluorescent staining, flow cytometry and DNA agarose electrophoresis analysis, respectively. The activities of Caspase-3, -8 and -9 was measured using a colorimetric method. Results The viability of human ECV304 cells exposed to rVVC was inhibited by rVVC after 24 h. 2.0 HU/ml rVVC groups had a better cytotoxic effect to human ECV304 than that of 0.5 HU /ml rVVC groups. The apoptosis of human ECV304 cells in 2.0 HU/ml rVVC+40 μmol/L Z-VAD-FMK groups was relative reduced than that of 2.0 HU/ml of rVVC groups. After 0.5 h treatment with 2.0 HU/ml of rVVC, the Caspase-3 activity in human ECV304 cells increased gradually and reached the peak at 3 h (versus control groups, P<0.01). The activity of Caspase-8 and -9 remained unchanging. Conclusion The rVVC has cytotoxic effect on human ECV304 and the cytolysin is probably correlated with Caspase-3.

?AlM: To evaluate the clinical effects and security of posterior chamber implantable Collamer lens ( lCL ) implantation in patients with extreme highly myopia.
?METHODS:ln this study, 18 patients ( 32 eyes ) with extreme highly myopic patients who had undergone posterior chamber lCLs implantation from July 2010 to July 2013 were evaluated. Diopter -10. 5 ~ 19. 0D, and astigmia -0. 5 ~4. 5DC. Changes in intraocular pressure ( lOP ) , refraction, visual acuity and corneal endothelium, anterior chamber depth, iris, high arch, lens were noted at 1d, 1wk, 1, 3mo and 1a after surgery respectively, and follow-up was of 1a.
? RESULTS: Before surgery, the uncorrected visual acuity (UCVA) were 0. 01~0. 05, and the best spectacle-corrected visual acuity ( BSCVA) were 0. 4 ~ 1. 0. One month after surgery, the UCVA were 0. 5~1. 2. The mean vault were 547±222 μm (95%CI 442~672μm) and 528±268μm (95%CI 354 ~635μm) for 1mo and 1a, respectively (P = 0. 81), and there was no significant difference. Anterior subcapsular opacities in 1 eye, mild and transient increase in lOP in 3 eyes, and chronic pigment dispersion in 2 eyes were observed. There was no serious complication.
?CONCLUSlON: Posterior chamber phakic intraocular lens implantation is an effective and safe method for correcting patients with extreme highly myopia.

Animals ; China ; epidemiology ; Epidemiologic Studies ; Molecular Epidemiology ; Rickettsia ; genetics ; isolation & purification ; Rickettsia Infections ; epidemiology ; transmission ; Ticks ; microbiology

Adult ; Coal Mining ; Dermatitis, Occupational ; epidemiology ; Humans ; Male ; Middle Aged ; Prevalence

Objective By analyzing the surveillance result of Brucellosis in human being of Guizhou province from 2005 to 2008,to understand the current situation of relevant population with brucella infection,and then to provide the basis for the development of prevention and control measures.Methods According to the Brucella Disease Monitoring Standards (GB 16885-1997),in Guizhou province,Huaxi,Wudang,Xingyi,Dushan,Ceheng,Long Lane,Xifeng,Carey,Ziyun and so on other areas(city,county) were selected as monitoring points,and occupational groups of animal husbandry in agricultural areas,as well as farmers and students contacted with livestock were selected as monitoring subjects.Rose bengal plate agglutination test(RBPT) and tube agglutination test (SAT) were used to detect Brucellosis antibody.Results From 2005 to 2008,Brucellosis antibody detection rate was 0.63％(37/5904) in target groups of Guizhou province,specifically,the rates in Huaxi,Wudang,Xingyi and Ceheng counties(towns or districts) were 2.28％(19/832),0.16％(2/1274),1.84％(15/815) and 0.14％ (1/735),respectively; the rates in livestock workers,peasants and students contacted with livestock in rural areas were 1.29％ (36/2800),0.04％ ( 1/2814) and 0.00％ (0/290),respectively.In all antibody positive carriers,most were dairy cattle raisers which accounted for 83.78％ (31/37) in the total infected persons.Conclusions Dairy cattle and goat raisers in some counties(towns or districts) of Guizhou province have infected Brucellosis,and direct contacts with brucella-carrying cattle is the major route of Brucellosis transmission in human being.Strengthen livestock quarantine and dispose infected livestock timely are the key of Brucellosis control.

AIMTo study the effects of rhBNP on the cardiac hemodynamics and renal function in dogs with heart failure.

METHODSCongestive heart failure in dogs was induced by either rapid ventricular pacing (RVP), 250 beats.min-1 for 7-14 days or by thoracic inferior vena cava constriction (TIVCC) to 1/2 its original diameter. When remarkable hemodynamic changes appeared rhBNP was infused intravenously at the dosage of 10, 30 and 100 ng.kg-1.min-1, each dose lasting 30 min.

RESULTSIn dogs (n = 7) with RVP heart failure, intravenous infusion of rhBNP at 10-100 ng.kg-1.min-1, caused decreases in mean arterial pressure (MAP), left ventricular systolic pressure (LVSP), LVdP/dtmax, pulmonary arterial pressure (PAP), left ventricular end diastolic pressure (LVEDP), total peripheral vascular resistance (TPR) and renal vascular resistance (RVR) dose-dependently, without significant changes in cardiac output (CO), LVdp/dt/P, left ventricular work (LVW), renal blood flow (RBF) and heart rate (HR). This suggested that rhBNP reduced the pre-load and after-load of the dogs with congestive heart failure but showed no distinct effect on the contractility of the heart. In dogs (n = 7) with TIVCC heart failure, there were remarkable decreases in MAP and LVEDP following the rhBNP infusion, without further reduction of CO, but no marked change in HR, LVSP, LVdP/dtmax, RAP and TPR. In both animal models of heart failure, there were significant increases in urine volume and sodium excretion which were more significant in TIVCC dogs than in RVP dogs.

CONCLUSIONrhBNP reduced the pre-load and after-load in dogs with heart failure and showed remarkable diuretic effect, but did not affect the contractility of the heart.

Animals ; Blood Pressure ; drug effects ; Diuretics ; pharmacology ; therapeutic use ; Dogs ; Female ; Heart Failure ; drug therapy ; physiopathology ; urine ; Hemodynamics ; drug effects ; Kidney ; blood supply ; drug effects ; Kidney Function Tests ; Male ; Natriuretic Peptide, Brain ; pharmacology ; therapeutic use ; Recombinant Proteins ; pharmacology ; therapeutic use ; Sodium ; urine ; Vascular Resistance ; drug effects ; Vasodilator Agents ; pharmacology

OBJECTIVETo introduce a novel digital surgery technique based on partial reconstruction of skull and its application in tumor resection and on-sport reconstruction surgery.

METHODSThe partial 3d reconstruction according to CT scanning data was obtained to get 3d model of both tumor and normal skull, then to define the resection area on tumor data through people-computer interaction process, do reconstruction the simulation on the remained model, pre-fabricate the cutting template and custom the implant. The resection template to confirm the message in surgery simulation can be transferred to real surgery accurately.

RESULTSAll the four cases by using this method showed great consistency of the real and simulated surgery and got a good appearance of reconstruction.

CONCLUSIONSThe surgery simulation technique based on partial 3d reconstruction has great assistance to tumor resection and one-stage reconstruction surgery.

Adult ; Female ; Head and Neck Neoplasms ; surgery ; Humans ; Imaging, Three-Dimensional ; Male ; Middle Aged ; Reconstructive Surgical Procedures ; methods ; Surgery, Computer-Assisted ; methods ; Young Adult

Purpose:To avoid using steroid in long term and decrease its side effects. Methods:We analyzedthe data of 9 cases of patients whose immunosuppressive therapy were based on FK506 and MMF. Steroid hasbeen withdrawn in 6 to 8 months after kidney transplantation. Results:All patients have been followed-up. Theresults indicated that no patient exhibited any acute rejection episode, and the side effects of steroid were dimin-ished. Conclusions :We believe that withdrawal of steroid on basis of FK506 and MMF may be safe. and furtherinvestigations are necessary.