Objective This study aims to investigate the prevalence of familial cerebral cavernous malformations(FCCMs)in first-degree relatives(FDRs)using familial screening,to describe the distribution of initial symptoms,lesio...Objective This study aims to investigate the prevalence of familial cerebral cavernous malformations(FCCMs)in first-degree relatives(FDRs)using familial screening,to describe the distribution of initial symptoms,lesion count on cranial MRI and pathogenic gene in patients.Methods Patients with multiple CCMs who enrolled from the Treatments and Outcomes of Untreated Cerebral Cavernous Malformations in China database were considered as probands and FDRs were recruited.Cranial MRI was performed to screen the CCMs lesions,and whole-exome sequencing was performed to identify CCM mutations.MRI and genetic screening were combined to diagnose FCCM in FDRs,and the results were presented as prevalence and 95%CIs.The Kaplan-Meier(KM)method was used to calculate the cumulative incidence of FCCM.Results 33(76.74%)of the 43 families(110 FDRs)were identified as FCCM(85 FDRs).Receiver operating characteristic analysis revealed three lesions on T2-weighted imaging(T2WI)were the strong indicator for distinguishing probands with FCCM(sensitivity,87.10%;specificity,87.50%).Of the 85 FDRs,31 were diagnosed with FCCM,resulting in a prevalence of 36.5%(26.2%-46.7%).In families with FCCMs,the mutation rates for CCM1,CCM2 and CCM3 were 45.45%,21.21%and 9.09%,respectively.Furthermore,53.13%of patients were asymptomatic,17.19%were intracranial haemorrhage and 9.38%were epilepsy.The mean age of symptom onset analysed by KM was 46.67(40.56-52.78)years.Conclusion Based on MRI and genetic analysis,the prevalence of CCMs in the FDRs of families with FCCMs in China was 36.5%.Genetic counselling and MRI screening are recommended for FDRs in patients with more than three CCM lesions on T2WI.展开更多
Coupling effects of surface plasmon resonance(SPR)induce changes in the wavelength,intensity,and linewidth of plasmonic modes.Here,inspired by coupling effects,we reveal an abrupt linewidth-shrinking effect in 2 D gol...Coupling effects of surface plasmon resonance(SPR)induce changes in the wavelength,intensity,and linewidth of plasmonic modes.Here,inspired by coupling effects,we reveal an abrupt linewidth-shrinking effect in 2 D gold nanohole arrays at the azimuthal angle of 45°arising from the interference of two degenerate SPR modes.We further demonstrate the biosensing capability under various excitation conditions for detecting the critical molecular biomarker of prostatic carcinoma,and achieve the maximum sensitivity at this angle.Our study not only enhances the understanding toward plasmonic resonance-linewidth shrinking,but also provides a promising strategy to greatly improve biosensing performance by light manipulation on plasmonic nanostructures.展开更多
In view of the increasingly severe exploitation of minority shareholders and the existence of double agency costs in China, it is necessary to provide strong protection for minority shareholders in China in order to b...In view of the increasingly severe exploitation of minority shareholders and the existence of double agency costs in China, it is necessary to provide strong protection for minority shareholders in China in order to build an investor-friendly system. By enabling minority shareholders to prevent misconducts of majority shareholders and managers, legal system has made significant progress in the past twenty years. Nevertheless, many defects still exist. The first enactment of the PRC Company Law was passed in 1992 with primary goal to serve reform of state-owned enterprises and therefore protection for minority shareholders was excluded by the scope of these reforms. The revision of the Company Law in 2005 was regarded as historical progress of Chinese company law in respect of providing protection for minority shareholders as many rights are conferred on shareholders and more measures were adopted to restrain the power of directors and controlling shareholders. However, this paper identifies that these various rights and protective mechanisms have certain deficiencies, which means that the interests of minority shareholders suppose to be guaranteed would be inevitably affected. Without improvements and clarifications of the existing legal protection in the future, interests of shareholders and company as a whole will be obstructed ultimately and development of capital markets will be significantly impeded.展开更多
基金supported by grants from Technology Platform Construction Project of Fujian Province(2020Y2003,2021Y2001)supported the Fujian Province High level Neuromedical Center Construction Fund(principal investigator:DK),a grant from the Government of Fujian Province(grant number:HLNCC-FJFY 003)supported by grants from National Natural Science Foundation of China(8227051360).
文摘Objective This study aims to investigate the prevalence of familial cerebral cavernous malformations(FCCMs)in first-degree relatives(FDRs)using familial screening,to describe the distribution of initial symptoms,lesion count on cranial MRI and pathogenic gene in patients.Methods Patients with multiple CCMs who enrolled from the Treatments and Outcomes of Untreated Cerebral Cavernous Malformations in China database were considered as probands and FDRs were recruited.Cranial MRI was performed to screen the CCMs lesions,and whole-exome sequencing was performed to identify CCM mutations.MRI and genetic screening were combined to diagnose FCCM in FDRs,and the results were presented as prevalence and 95%CIs.The Kaplan-Meier(KM)method was used to calculate the cumulative incidence of FCCM.Results 33(76.74%)of the 43 families(110 FDRs)were identified as FCCM(85 FDRs).Receiver operating characteristic analysis revealed three lesions on T2-weighted imaging(T2WI)were the strong indicator for distinguishing probands with FCCM(sensitivity,87.10%;specificity,87.50%).Of the 85 FDRs,31 were diagnosed with FCCM,resulting in a prevalence of 36.5%(26.2%-46.7%).In families with FCCMs,the mutation rates for CCM1,CCM2 and CCM3 were 45.45%,21.21%and 9.09%,respectively.Furthermore,53.13%of patients were asymptomatic,17.19%were intracranial haemorrhage and 9.38%were epilepsy.The mean age of symptom onset analysed by KM was 46.67(40.56-52.78)years.Conclusion Based on MRI and genetic analysis,the prevalence of CCMs in the FDRs of families with FCCMs in China was 36.5%.Genetic counselling and MRI screening are recommended for FDRs in patients with more than three CCM lesions on T2WI.
基金National Natural Science Foundation of China(21673192,91850119)NSAF Joint Fund(U1830116)+2 种基金Ministry of Science and Technology of the People’s Republic of China(2016YFA0200601,2017YFA0204902)Natural Science Foundation of Jiangxi Province(20192ACB20032)Fundamental Research Funds for the Central Universities(20720190010)。
文摘Coupling effects of surface plasmon resonance(SPR)induce changes in the wavelength,intensity,and linewidth of plasmonic modes.Here,inspired by coupling effects,we reveal an abrupt linewidth-shrinking effect in 2 D gold nanohole arrays at the azimuthal angle of 45°arising from the interference of two degenerate SPR modes.We further demonstrate the biosensing capability under various excitation conditions for detecting the critical molecular biomarker of prostatic carcinoma,and achieve the maximum sensitivity at this angle.Our study not only enhances the understanding toward plasmonic resonance-linewidth shrinking,but also provides a promising strategy to greatly improve biosensing performance by light manipulation on plasmonic nanostructures.
文摘In view of the increasingly severe exploitation of minority shareholders and the existence of double agency costs in China, it is necessary to provide strong protection for minority shareholders in China in order to build an investor-friendly system. By enabling minority shareholders to prevent misconducts of majority shareholders and managers, legal system has made significant progress in the past twenty years. Nevertheless, many defects still exist. The first enactment of the PRC Company Law was passed in 1992 with primary goal to serve reform of state-owned enterprises and therefore protection for minority shareholders was excluded by the scope of these reforms. The revision of the Company Law in 2005 was regarded as historical progress of Chinese company law in respect of providing protection for minority shareholders as many rights are conferred on shareholders and more measures were adopted to restrain the power of directors and controlling shareholders. However, this paper identifies that these various rights and protective mechanisms have certain deficiencies, which means that the interests of minority shareholders suppose to be guaranteed would be inevitably affected. Without improvements and clarifications of the existing legal protection in the future, interests of shareholders and company as a whole will be obstructed ultimately and development of capital markets will be significantly impeded.
