Objective To analyze the electrophysiological and genetic examination results of children with congenital stationary night blindness(CSNB)without fundus abnormalities.Methods A retrospective study was conducted on 11 children diagnosed with CSNB in our department from August 2019 to March 2025.Their full-field electroretinogram(ffERG)and the results of genetic testing were analyzed.Results Among the 11 patients,there were 10 males and 1 female,at a mean age of 7.5±2.1 years.In the optometry,1 patient had hyperopia and the other had myopia,and all of them obtained a corrected visual acuity of 0.48±0.11 after wearing glasses.In fundus photography examination,except for the leopard pattern changes in high myopia,no obvious abnormalities were found in the remaining fundus.In ffERG examination for all patients,the amplitude of the a-wave of scotopic ERG 3.0/10.0 was normal or slightly to moderately decreased,the amplitude of the b-wave was decreased significantly,and the amplitude ratio of the b-wave to the a-wave was<1,showing a negative-phase wave,which belonged to the CSNB Schubert-Bornschein waveform.The results of genetic test revealed that all the 11 patients carried pathogenic mutations,including 6 cases with CACNA1F mutations,2 cases with GPR179 mutations,2 cases with NYX mutations,and 1 case with GRM6 mutation.Conclusion For children with normal fundus manifestations but corrected visual acuity lower than normal after wearing glasses,ffERG examination should be carried out as soon as possible to screen for CSNB,and a diagnosis should be made in combination with genetic testing.This is helpful for evaluating the heredity and prognosis of the disease,increasing the chance of early detection,and reducing excessive amblyopia training and treatment.

Objective To investigate the potential therapeutic targets and underlying molecular mechanisms of Z-ligustilide(Z-LIG)in treating retinitis pigmentosa(RP).Methods By integrating multiple databases,such as GeneCards and TargetNet,common targets for RP and Z-LIG were identified.Protein-protein interaction(PPI)network analysis of the targets was conducted using the STRING database and analyzed in Cytoscape to identify core targets.The DAVID database was employed for the functional enrichment analysis of Gene Ontology(GO)and the pathway enrichment analysis of Kyoto Encyclopedia of Genes and Genomes(KEGG)for the core targets.The PubChem database was accessed for molecular docking verification to validate the binding affinity between Z-LIG and the core targets.Finally,male rd10 mice were randomly divided into a control group(n=5)and a Z-LIG treatment group(n=5).The protective effect of Z-LIG on the visual function in rd10 mice was verified through visual electrophysiology and behavioral tests.Western blotting and RT-qPCR were utilized to investigate the molecular mechanism of action.Results A total of 66 shared targets between RP and Z-LIG were identified through the screening process.PPI network analysis identified 55 key targets.GO enrichment analysis yielded 623 terms,which covering 3 dimensions,including cellular component(CC),molecular function(MF),and biological process(BP),such as inflammatory response.KEGG pathway enrichment analysis further concentrated 124 terms,which were enriched in the PI3K-Akt signaling pathway.Molecular docking suggested that Z-LIG could specifically bind with high affinity to RP-related core targets(such as EGFR and STAT3)via hydrogen bonds.In animal experiments,compared to the control group,the rd10 mice from the Z-LIG group showed a greater preference for the dark environment in the light/dark transition test(P<0.05),exhibited significantly higher amplitudes of A-wave and B-wave in electroretinogram(ERG)(P<0.05),greater number of outer nuclear layers,with fewer apoptotic cells and less microglial activation(P<0.05),demonstrated obviously reduced protein levels of p-PI3K/PI3K and p-AKT/AKT in the retina(P<0.05),and had notably down-regulated mRNA levels of pro-inflammatory cytokines IL-1β and IL-6(P<0.05).Conclusion Z-LIG may exert a protective effect on the retina of rd10 mice by inhibiting the activation of the PI3K/AKT-inflammatory axis,thereby delaying retinal degeneration.

Objective:To compare the therapeutic effect between regular spherical corneal refractive therapy (CRT) and dual axis CRT on myopia with corneal astigmatism.Methods:A non-randomized controlled study was conducted.Aged 8 to 14 years old, forty-eight patients (48 eyes) wearing CRT orthokeratology enrolled from May, 2018 to December, 2018 in First Affiliated Hospital of Army Medical University were divided into dual axis CRT group (24 eyes) and spherical CRT group (24 eyes) according to their guardians' willingness.After 1-year follow-up, the visual acuity, eccentricity distance in treatment area, annual axial growth and the corneal punctate staining incidence of the two groups were compared.The study followed the Declaration of Helsinki, and was approved by an Ethics Committee of First Affiliated Hospital of Army Medical University (No.KY201975). Written informed consent was obtained from guardians prior to any examination.Results:The uncorrected visual acuity of the dual axis CRT group was better than that of the spherical CRT group at one week and one month after correction, and the differences were statistically significant (both at P<0.05), and there was no significant difference in uncorrected visual acuity between the two groups at other time points (all at P>0.05). The deviations of optical center in the spherical CRT group at each observation time points were significantly larger than that in the dual axis group (all at P<0.05), and the deviation was not obviously changed with time in both groups and there was no significant difference in deviation change between the two groups ( Ftime=2.301, P=0.074). The eccentricity was mainly in horizontal direction in spherical CRT group.The median annual axial growth was 0.12 (0.10, 0.45)mm in the dual axis CRT group and 0.14 (0.10, 0.46)mm in the spherical CRT group, with no significant difference between the two groups ( Z=0.248, P=0.804). There was 4.2%(1/24) of the patients having grade Ⅲ-Ⅳ corneal punctate staining in the dual axis CRT group, and 25.0%(6/24) in the regular spherical CRT group, showing significant difference between the two groups ( χ2=4.180, P=0.049). Conclusions:For the treatment of myopia with astigmatism, dual axis CRT shows better centricity and safety than spherical CRT.

Biocompatibility has always been the focal point in the study of biomaterials applied to medical apparatus. But at present, the standard system hasn't formed completely to evaluate the biomaterials. In recent years scientists tend to use general standard of evaluation. In this article, based on the recalcification time, the adhesion of platelets as well as the total quantity of plasma protein and some other evidences, combining with the Analytic hierarchy process (AHP), the method of general evaluation on the biocompatibility of anticoagulant biomaterials was discussed.
Anticoagulants ; analysis ; Biocompatible Materials ; standards ; Blood Proteins ; analysis ; Humans ; Materials Testing ; methods ; standards ; Platelet Adhesiveness ; drug effects

As a means to substitute the function of kidney, hemodialysis therapy has been applied to clinical therapy. The bio-compatibility of dialyser, which is the key of part of artificial kidney, is a determining factor for the therapy. This paper describes dialyser and the methods of improving bio-compatibility of dialyser.