Objective:Autosomal recessive bestrophinopathy(ARB),a retinal degenerative disease,is characterized by central visual loss,yellowish multifocal diffuse subretinal deposits,and a dramatic decrease in the light peak on ...Objective:Autosomal recessive bestrophinopathy(ARB),a retinal degenerative disease,is characterized by central visual loss,yellowish multifocal diffuse subretinal deposits,and a dramatic decrease in the light peak on electrooculogram.The potential pathogenic mechanism involves mutations in the BEST1 gene,which encodes Ca2+-activated Cl−channels in the retinal pigment epithelium(RPE),resulting in degeneration of RPE and photoreceptor.In this study,the complete clinical characteristics of two Chinese ARB families were summarized.Methods:Pacific Biosciences(PacBio)single-molecule real-time(SMRT)sequencing was performed on the probands to screen for disease-causing gene mutations,and Sanger sequencing was applied to validate variants in the patients and their family members.Results:Two novel mutations,c.202T>C(chr11:61722628,p.Y68H)and c.867+97G>A,in the BEST1 gene were identified in the two Chinese ARB families.The novel missense mutation BEST1 c.202T>C(p.Y68H)resulted in the substitution of tyrosine with histidine in the N-terminal region of transmembrane domain 2 of bestrophin-1.Another novel variant,BEST1 c.867+97G>A(chr11:61725867),located in intron 7,might be considered a regulatory variant that changes allele-specific binding affinity based on motifs of important transcriptional regulators.Conclusion:Our findings represent the first use of third-generation sequencing(TGS)to identify novel BEST1 mutations in patients with ARB,indicating that TGS can be a more accurate and efficient tool for identifying mutations in specific genes.The novel variants identified further broaden the mutation spectrum of BEST1 in the Chinese population.展开更多
Background:High-fidelity simulation has been demonstrated to make great progress in learning.However,there is still ongoing exploration on how to fully harness the advantages of this teaching method and enhance its ef...Background:High-fidelity simulation has been demonstrated to make great progress in learning.However,there is still ongoing exploration on how to fully harness the advantages of this teaching method and enhance its effectiveness.This study conducted high-fidelity simulation in medical nursing based on the Healthcare Simulation Standards of Best Practice and evaluated its effect.Methods:The study was conducted from September 2019 to June 2020.A total of 82 undergraduate nursing students from a university in Shanghai participated in the high-fidelity simulation in medical nursing.The simulation design scale,educational practices in simulation scale,and students’satisfaction and self-confidence were used to evaluate the effect.Results:The mean score of simulation design scale was 4.06±0.63 with the mean scores of all dimensions being over 3.0.The mean score of educational practices in simulation scale was 4.14±0.56 with the mean scores of all dimensions being over 4.0.The mean scores of students’satisfaction and self-confidence were 4.07±0.72 and 3.89±0.58,respectively.Conclusion:Students reported high levels of simulation design and educational practices in simulation.They were also satisfied with learning and reported high levels of self-confidence.Some key points need to be considered so that the learning effects might be optimized.展开更多
In the traditional data envelopment analysis （DEA） structure, the efficiency score for one decision making unit （DMU） is calculated by measuring the distance of the evaluated DMU to the best practice frontier. Rec...In the traditional data envelopment analysis （DEA） structure, the efficiency score for one decision making unit （DMU） is calculated by measuring the distance of the evaluated DMU to the best practice frontier. Recent researches have provided the reasonability of considering the worst practice frontier as a supple- ment to the traditional DEA techniques. The existing researches take only one type of frontier into account, and they cannot com- pare the evaluated DMU with both the best and the worst perform- ing DMUs. A DEA-based procedure is developed to consider the best and the worst frontiers in the same scenario where the ratio of two distances （RDS） measure is proposed. The principal appli- cation of this approach is for ranking, and, as a complement tool, for performance evaluation. The proposed approach can be used in a wide range of applications such as the performance evaluation of employees and others. Finally, a bookstore data set is used to illustrate the proposed approach.展开更多
It makes more sense to look at which is better for their own people.During a debate comparing Chinese and Western systems of governance,held in Cambridge,Massachusetts,by the American nonprofit educational organizatio...It makes more sense to look at which is better for their own people.During a debate comparing Chinese and Western systems of governance,held in Cambridge,Massachusetts,by the American nonprofit educational organization Intercollegiate Studies Institute on April 5,French entrepreneur and Internet influencer Arnaud Bertrand made a case for the suitability of China’s system for promoting the flourishing of its people.Edited excerpts of his presentation follow.展开更多
为了准确判断施工现场在突降暴雨情况下的安全状态,采用贝叶斯最优最劣法(Bayesian Best Worst Method,BBWM)和云模型方法,提出暴雨灾害下的建筑施工现场风险评价模型,以确定施工现场在遭受暴雨灾害时的风险等级。该模型利用了压力状态...为了准确判断施工现场在突降暴雨情况下的安全状态,采用贝叶斯最优最劣法(Bayesian Best Worst Method,BBWM)和云模型方法,提出暴雨灾害下的建筑施工现场风险评价模型,以确定施工现场在遭受暴雨灾害时的风险等级。该模型利用了压力状态响应模型(Pressure State Response,PSR)和灾害系统理论,在考虑致灾因子危险性、孕灾环境稳定性、承灾体脆弱性和减灾能力抵御性4方面的基础上,构建18个风险因素的施工现场风险评价指标体系,并以武汉市某施工现场为例进行验证。结果显示,施工现场的减灾能力抵御性处于最重要的地位,做好现场减灾应对措施对灾害有非常重要的帮助;案例项目的评价结果处于一般风险状态,与现场实际情况相符。展开更多
文摘Objective:Autosomal recessive bestrophinopathy(ARB),a retinal degenerative disease,is characterized by central visual loss,yellowish multifocal diffuse subretinal deposits,and a dramatic decrease in the light peak on electrooculogram.The potential pathogenic mechanism involves mutations in the BEST1 gene,which encodes Ca2+-activated Cl−channels in the retinal pigment epithelium(RPE),resulting in degeneration of RPE and photoreceptor.In this study,the complete clinical characteristics of two Chinese ARB families were summarized.Methods:Pacific Biosciences(PacBio)single-molecule real-time(SMRT)sequencing was performed on the probands to screen for disease-causing gene mutations,and Sanger sequencing was applied to validate variants in the patients and their family members.Results:Two novel mutations,c.202T>C(chr11:61722628,p.Y68H)and c.867+97G>A,in the BEST1 gene were identified in the two Chinese ARB families.The novel missense mutation BEST1 c.202T>C(p.Y68H)resulted in the substitution of tyrosine with histidine in the N-terminal region of transmembrane domain 2 of bestrophin-1.Another novel variant,BEST1 c.867+97G>A(chr11:61725867),located in intron 7,might be considered a regulatory variant that changes allele-specific binding affinity based on motifs of important transcriptional regulators.Conclusion:Our findings represent the first use of third-generation sequencing(TGS)to identify novel BEST1 mutations in patients with ARB,indicating that TGS can be a more accurate and efficient tool for identifying mutations in specific genes.The novel variants identified further broaden the mutation spectrum of BEST1 in the Chinese population.
基金supported by Fudan Good Practice Program of Teaching and Learning(2019C003).
文摘Background:High-fidelity simulation has been demonstrated to make great progress in learning.However,there is still ongoing exploration on how to fully harness the advantages of this teaching method and enhance its effectiveness.This study conducted high-fidelity simulation in medical nursing based on the Healthcare Simulation Standards of Best Practice and evaluated its effect.Methods:The study was conducted from September 2019 to June 2020.A total of 82 undergraduate nursing students from a university in Shanghai participated in the high-fidelity simulation in medical nursing.The simulation design scale,educational practices in simulation scale,and students’satisfaction and self-confidence were used to evaluate the effect.Results:The mean score of simulation design scale was 4.06±0.63 with the mean scores of all dimensions being over 3.0.The mean score of educational practices in simulation scale was 4.14±0.56 with the mean scores of all dimensions being over 4.0.The mean scores of students’satisfaction and self-confidence were 4.07±0.72 and 3.89±0.58,respectively.Conclusion:Students reported high levels of simulation design and educational practices in simulation.They were also satisfied with learning and reported high levels of self-confidence.Some key points need to be considered so that the learning effects might be optimized.
基金supported by the National Natural Science Foundation of China(7112106171271195+2 种基金71322101)the National Social Science Fund of China(13CTQ042)the USTC Foundation for Innovative Research Team(WK2040160008)
文摘In the traditional data envelopment analysis （DEA） structure, the efficiency score for one decision making unit （DMU） is calculated by measuring the distance of the evaluated DMU to the best practice frontier. Recent researches have provided the reasonability of considering the worst practice frontier as a supple- ment to the traditional DEA techniques. The existing researches take only one type of frontier into account, and they cannot com- pare the evaluated DMU with both the best and the worst perform- ing DMUs. A DEA-based procedure is developed to consider the best and the worst frontiers in the same scenario where the ratio of two distances （RDS） measure is proposed. The principal appli- cation of this approach is for ranking, and, as a complement tool, for performance evaluation. The proposed approach can be used in a wide range of applications such as the performance evaluation of employees and others. Finally, a bookstore data set is used to illustrate the proposed approach.
文摘It makes more sense to look at which is better for their own people.During a debate comparing Chinese and Western systems of governance,held in Cambridge,Massachusetts,by the American nonprofit educational organization Intercollegiate Studies Institute on April 5,French entrepreneur and Internet influencer Arnaud Bertrand made a case for the suitability of China’s system for promoting the flourishing of its people.Edited excerpts of his presentation follow.
文摘为了准确判断施工现场在突降暴雨情况下的安全状态,采用贝叶斯最优最劣法(Bayesian Best Worst Method,BBWM)和云模型方法,提出暴雨灾害下的建筑施工现场风险评价模型,以确定施工现场在遭受暴雨灾害时的风险等级。该模型利用了压力状态响应模型(Pressure State Response,PSR)和灾害系统理论,在考虑致灾因子危险性、孕灾环境稳定性、承灾体脆弱性和减灾能力抵御性4方面的基础上,构建18个风险因素的施工现场风险评价指标体系,并以武汉市某施工现场为例进行验证。结果显示,施工现场的减灾能力抵御性处于最重要的地位,做好现场减灾应对措施对灾害有非常重要的帮助;案例项目的评价结果处于一般风险状态,与现场实际情况相符。
文摘为针对性地解决转炉炼钢作业时的消防安全问题,提出将熵权-最优最劣法(Best Worst Method,BWM)和物元可拓模型相结合的转炉车间火灾风险评估模型。通过将指标分为人、物料设备、环境、管理及消防设施5大类,构建转炉车间火灾风险评估指标体系;利用复合语言对35个指标进行评价,引入有序加权平均算子(Ordered Weighted Averaging,OWA)计算各语言的犹豫语言术语集(Hesitant Fuzzy Linguistic Term Set,HFLTS)模糊包络,再根据各专家权重进行加权计算并将语言量化构成评价矩阵;基于熵权、BWM分别算得各指标权重,再运用最小二乘法得到综合权重;最后利用物元可拓模型确定各指标及转炉车间的火灾风险等级。以河北省某炼钢转炉车间为例进行评估得出:该转炉车间的综合风险等级为I级(安全),其中动火监护的人员监管情况、氧枪法兰松紧度、炉前温度环境、作业现场物品摆放情况、作业巡查及设备检查情况和消防疏散通道堵塞情况仍需改善。
