目的描述广东地区法布雷病患者基因型情况,对比IVS4+919G>A突变患者与非IVS4+919G>A突变患者的地域分布和临床表型差异。方法回顾性收集2022年3月1日至2025年12月31日本中心(中山大学附属第一医院)47例(IVS4+919G>A突变患者12...目的描述广东地区法布雷病患者基因型情况,对比IVS4+919G>A突变患者与非IVS4+919G>A突变患者的地域分布和临床表型差异。方法回顾性收集2022年3月1日至2025年12月31日本中心(中山大学附属第一医院)47例(IVS4+919G>A突变患者12例)已出现器官受累的法布雷病患者的相关资料,分析总体、IVS4+919G>A突变和非IVS4+919G>A突变患者的地域分布和临床表现特点。结果IVS4+919G>A突变在本中心为最常见突变(25.5%)。本中心的该突变患者有半数(50%)分布在粤东地区,起病中位年龄显著高于非IVS4+919G>A突变组,差异有统计学意义(P<0.05)。IVS4+919G>A突变患者中出现心脏受累11例(91.7%),神经系统受累2例(16.7%),美因茨严重程度评分指数(Mainz severity score index,MSSI)评分有高于非IVS4+919G>A突变组的趋势(P=0.051);MSSI总体评分和神经评分低于非IVS4+919G>A突变组,差异有统计学意义(P<0.05)。相较非IVS4+919G>A突变患者,IVS4+919G>A突变患者的心率更低、心肌肌钙蛋白浓度更高,超声提示其左心房更大、室间隔与左心室后壁更厚、二尖瓣舒张早期充盈峰速度E峰和二尖瓣舒张晚期充盈峰速度A峰比值(ratio of early diastolic transmitral inflow E-peak velocity to late diastolic mitral annulus A-peakvelocity,E/A)更低,差异有统计学意义(P<0.05)。结论IVS4+919G>A突变患者在本中心为最常见突变。本中心的该突变患者有半数分布在粤东地区,其表型以迟发型和心脏受累为主,神经系统受累较少,心肌肥厚、舒张功能下降和心肌损伤情况较其他突变群体更严重。展开更多
Objective:Peritoneal fibrosis(PF)is an adverse event that occurs during long-term peritoneal dialysis,significantly impairing treatment efficiency and adversely affecting patient outcomes.Astragaloside IV(AS-Ⅳ),a pri...Objective:Peritoneal fibrosis(PF)is an adverse event that occurs during long-term peritoneal dialysis,significantly impairing treatment efficiency and adversely affecting patient outcomes.Astragaloside IV(AS-Ⅳ),a principal active component derived from Astragalus membranaceus(Fisch.)Bunge,has exhibited anti-inflammatory and antifibrotic effects in various settings.This study aims to investigate the potential therapeutic efficacy and mechanism of AS-Ⅳin the treatment of PF.Methods:The PF mouse model was established by intraperitoneal injection of 4.25%peritoneal dialysis fluid(100 mL/kg).The epithelial-mesenchymal transition(EMT)of HMrSV5 cells was induced by the addition of 10 ng/mL transforming growth factorβ(TGF-β).The differentially expressed genes in HMrSV5 cells treated with AS-Ⅳwere screened using transcriptome sequencing analysis.The potential targets of AS-Ⅳwere screened using network pharmacology and analyzed using molecular docking and molecular dynamics simulations.Results:Administration of AS-Ⅳat doses of 20,40,or 80 mg/kg effectively mitigated the increase in peritoneal thickness and the development of fibrosis in mice with PF.The expression of the fibrosis markerα-smooth muscle actin in the peritoneum was significantly decreased in AS-Ⅳ-treated mice.The treatment of AS-Ⅳ(10,20,and 40μmol/L)significantly delayed the EMT of HMrSV5 cells induced by TGF-β,as demonstrated by the decreased number of 5-ethynyl-2'-deox yuridine-positive cells,reduced migrated area,and decreased expression of fibrosis markers.A total of 460 differentially expressed genes were detected in AS-Ⅳ-treated HMrSV5 cells through transcriptome sequencing,with notable enrichment in the phosphatidylinositol-4,5-bisphosphate3-kinase(PI3K)-AKT serine/threonine kinase 1(AKT)signaling pathway.The reduced levels of phosphorylated PI3K(p-PI3K)and p-AKT were detected in HMrSV5 cells with AS-Ⅳtreatment.Epidermal growth factor receptor(EGFR)was predicted as a direct target of AS-Ⅳ,exhibiting strong hydrogen bond interactions.The activation of the PI3K-AKT pathway by the compound740Y-P,and the activation of the EGFR pathway by NSC 228155 each partially counteracted the inhibitory effect of AS-Ⅳon the EMT of HMrSV5 cells.Conclusion:AS-Ⅳdelayed the EMT process in peritoneal mesothelial cells and slowed the progression of PF,potentially serving as a therapeutic agent for the early prevention and treatment of PF.展开更多
BACKGROUND Liver transplantation aims to increase the survival of patients with end-stage liver diseases and improve their quality of life.The number of organs available for transplantation is lower than the demand.To...BACKGROUND Liver transplantation aims to increase the survival of patients with end-stage liver diseases and improve their quality of life.The number of organs available for transplantation is lower than the demand.To provide fair organ distribution,predictive mortality scores have been developed.AIM To compare the Acute Physiology and Chronic Health Evaluation IV(APACHE IV),balance of risk(BAR),and model for end-stage liver disease(MELD)scores as predictors of mortality.METHODS Retrospective cohort study,which included 283 adult patients in the postoperative period of deceased donor liver transplantation from 2014 to 2018.RESULTS The transplant recipients were mainly male,with a mean age of 58.1 years.Donors were mostly male,with a mean age of 41.6 years.The median cold ischemia time was 3.1 hours,and the median intensive care unit stay was 5 days.For APACHE IV,a mean of 59.6 was found,BAR 10.7,and MELD 24.2.The 28-day mortality rate was 9.5%,and at 90 days,it was 3.5%.The 28-day mortality prediction for APACHE IV was very good[area under the curve(AUC):0.85,P<0.001,95%CI:0.76-0.94],P<0.001,BAR(AUC:0.70,P<0.001,95%CI:0.58–0.81),and MELD(AUC:0.66,P<0.006,95%CI:0.55-0.78),P<0.008.At 90 days,the data for APACHE IV were very good(AUC:0.80,P<0.001,95%CI:0.71–0.90)and moderate for BAR and MELD,respectively,(AUC:0.66,P<0.004,95%CI:0.55–0.77),(AUC:0.62,P<0.026,95%CI:0.51–0.72).All showed good discrimination between deaths and survivors.As for the best value for liver transplantation,it was significant only for APACHE IV(P<0.001).CONCLUSION The APACHE IV assessment score was more accurate than BAR and MELD in predicting mortality in deceased donor liver transplant recipients.展开更多
In the current era marked by energy shortages,the advancement of nuclear energy stands as an inevitable progression.The reprocessing of spent nuclear fuel plays a crucial role in determining the sustainability of nucl...In the current era marked by energy shortages,the advancement of nuclear energy stands as an inevitable progression.The reprocessing of spent nuclear fuel plays a crucial role in determining the sustainability of nuclear energy as a viable energy source.Among these processes,the separation and recovery of Pu(Ⅳ)from high-level liquid waste(HLLW)hold paramount significance in terms of safety and strategic implications.Herein,this work focused on the synthesis of two acid-and radiation-resistant pyridine-based sp^(2)c-COFs(COF-IHEP3 and COF-IHEP4),followed by the creation of two pyridine-based ionized sp^(2)c-COFs named COF-IHEP3-CH_(3)NO_(3)and COF-IHEP4-CH3NO3through post-modification.These materials have potential anion exchange capacity for the selective separation of Pu(Ⅳ)in highly acidic conditions.Notably,in 8 mol/L nitric acid solution,COF-IHEP3-CH3NO3demonstrated the capability to eliminate plutonium within 20 min in 98%removal efficiency with a Kdvalue of 2450 m L/g.Experimental and theoretical analysis suggest that the ionized sp^(2)c-COFs exhibit exceptional stability,selectivity,and prevention of secondary contamination towards Pu(Ⅳ)in the presence of multiple ions environments.In short,this work provides an appropriate anion exchange strategy to design ionic sp^(2)c-COFs as a promising platform for Pu(Ⅳ)recovery from HLLW.展开更多
The efficient recovery of silver(Ag)from retired photovoltaic(PV)panels is crucial for resource sustainability and envi-ronmental protection.This study developed an environmentally friendly leaching method using ammon...The efficient recovery of silver(Ag)from retired photovoltaic(PV)panels is crucial for resource sustainability and envi-ronmental protection.This study developed an environmentally friendly leaching method using ammonia(NH_(3)·H_(2)O)and hydrogen peroxide(H_(2)O_(2)),achieving the selective dissolution of Ag from retired crystalline silicon solar panels.Meanwhile,nonprecious metals such as aluminum(Al)and lead(Pb),which are commonly found in PV cells,were barely dissolved,dem-onstrating the excellent selectivity of this method for Ag.Light irradiation significantly improved the dissolution efficiency of Ag and reduced the amount of the reagent used.Ag dissolution occurred owing to a dual-pathway synergistic effect,which stemmed from the direct oxidation of Ag by H_(2)O_(2).The strongly oxidizing hydroxyl radicals generated by photocatalysis accelerated the oxidation and dissolution of Ag.In addition,NH 3·H_(2)O effectively promoted the dissolution and stabilization of oxidation products by forming soluble Ag–NH3·H2O complexes([Ag(NH3)2]+).This article reports an efficient,selective,and environmentally friendly strategy of Ag recovery and elucidates the radical-mediated dissolution mechanism under light-driven conditions,offering a feasible way for sustainably recovering valuable metals from retired PV panels.展开更多
文摘目的描述广东地区法布雷病患者基因型情况,对比IVS4+919G>A突变患者与非IVS4+919G>A突变患者的地域分布和临床表型差异。方法回顾性收集2022年3月1日至2025年12月31日本中心(中山大学附属第一医院)47例(IVS4+919G>A突变患者12例)已出现器官受累的法布雷病患者的相关资料,分析总体、IVS4+919G>A突变和非IVS4+919G>A突变患者的地域分布和临床表现特点。结果IVS4+919G>A突变在本中心为最常见突变(25.5%)。本中心的该突变患者有半数(50%)分布在粤东地区,起病中位年龄显著高于非IVS4+919G>A突变组,差异有统计学意义(P<0.05)。IVS4+919G>A突变患者中出现心脏受累11例(91.7%),神经系统受累2例(16.7%),美因茨严重程度评分指数(Mainz severity score index,MSSI)评分有高于非IVS4+919G>A突变组的趋势(P=0.051);MSSI总体评分和神经评分低于非IVS4+919G>A突变组,差异有统计学意义(P<0.05)。相较非IVS4+919G>A突变患者,IVS4+919G>A突变患者的心率更低、心肌肌钙蛋白浓度更高,超声提示其左心房更大、室间隔与左心室后壁更厚、二尖瓣舒张早期充盈峰速度E峰和二尖瓣舒张晚期充盈峰速度A峰比值(ratio of early diastolic transmitral inflow E-peak velocity to late diastolic mitral annulus A-peakvelocity,E/A)更低,差异有统计学意义(P<0.05)。结论IVS4+919G>A突变患者在本中心为最常见突变。本中心的该突变患者有半数分布在粤东地区,其表型以迟发型和心脏受累为主,神经系统受累较少,心肌肥厚、舒张功能下降和心肌损伤情况较其他突变群体更严重。
基金Zhejiang Medical Technology Project(No.2022RC009)Zhejiang Provincial Natural Science Foundation(No.LY23H050005)National Natural Science Foundation of China(No.81900692)。
文摘Objective:Peritoneal fibrosis(PF)is an adverse event that occurs during long-term peritoneal dialysis,significantly impairing treatment efficiency and adversely affecting patient outcomes.Astragaloside IV(AS-Ⅳ),a principal active component derived from Astragalus membranaceus(Fisch.)Bunge,has exhibited anti-inflammatory and antifibrotic effects in various settings.This study aims to investigate the potential therapeutic efficacy and mechanism of AS-Ⅳin the treatment of PF.Methods:The PF mouse model was established by intraperitoneal injection of 4.25%peritoneal dialysis fluid(100 mL/kg).The epithelial-mesenchymal transition(EMT)of HMrSV5 cells was induced by the addition of 10 ng/mL transforming growth factorβ(TGF-β).The differentially expressed genes in HMrSV5 cells treated with AS-Ⅳwere screened using transcriptome sequencing analysis.The potential targets of AS-Ⅳwere screened using network pharmacology and analyzed using molecular docking and molecular dynamics simulations.Results:Administration of AS-Ⅳat doses of 20,40,or 80 mg/kg effectively mitigated the increase in peritoneal thickness and the development of fibrosis in mice with PF.The expression of the fibrosis markerα-smooth muscle actin in the peritoneum was significantly decreased in AS-Ⅳ-treated mice.The treatment of AS-Ⅳ(10,20,and 40μmol/L)significantly delayed the EMT of HMrSV5 cells induced by TGF-β,as demonstrated by the decreased number of 5-ethynyl-2'-deox yuridine-positive cells,reduced migrated area,and decreased expression of fibrosis markers.A total of 460 differentially expressed genes were detected in AS-Ⅳ-treated HMrSV5 cells through transcriptome sequencing,with notable enrichment in the phosphatidylinositol-4,5-bisphosphate3-kinase(PI3K)-AKT serine/threonine kinase 1(AKT)signaling pathway.The reduced levels of phosphorylated PI3K(p-PI3K)and p-AKT were detected in HMrSV5 cells with AS-Ⅳtreatment.Epidermal growth factor receptor(EGFR)was predicted as a direct target of AS-Ⅳ,exhibiting strong hydrogen bond interactions.The activation of the PI3K-AKT pathway by the compound740Y-P,and the activation of the EGFR pathway by NSC 228155 each partially counteracted the inhibitory effect of AS-Ⅳon the EMT of HMrSV5 cells.Conclusion:AS-Ⅳdelayed the EMT process in peritoneal mesothelial cells and slowed the progression of PF,potentially serving as a therapeutic agent for the early prevention and treatment of PF.
文摘BACKGROUND Liver transplantation aims to increase the survival of patients with end-stage liver diseases and improve their quality of life.The number of organs available for transplantation is lower than the demand.To provide fair organ distribution,predictive mortality scores have been developed.AIM To compare the Acute Physiology and Chronic Health Evaluation IV(APACHE IV),balance of risk(BAR),and model for end-stage liver disease(MELD)scores as predictors of mortality.METHODS Retrospective cohort study,which included 283 adult patients in the postoperative period of deceased donor liver transplantation from 2014 to 2018.RESULTS The transplant recipients were mainly male,with a mean age of 58.1 years.Donors were mostly male,with a mean age of 41.6 years.The median cold ischemia time was 3.1 hours,and the median intensive care unit stay was 5 days.For APACHE IV,a mean of 59.6 was found,BAR 10.7,and MELD 24.2.The 28-day mortality rate was 9.5%,and at 90 days,it was 3.5%.The 28-day mortality prediction for APACHE IV was very good[area under the curve(AUC):0.85,P<0.001,95%CI:0.76-0.94],P<0.001,BAR(AUC:0.70,P<0.001,95%CI:0.58–0.81),and MELD(AUC:0.66,P<0.006,95%CI:0.55-0.78),P<0.008.At 90 days,the data for APACHE IV were very good(AUC:0.80,P<0.001,95%CI:0.71–0.90)and moderate for BAR and MELD,respectively,(AUC:0.66,P<0.004,95%CI:0.55–0.77),(AUC:0.62,P<0.026,95%CI:0.51–0.72).All showed good discrimination between deaths and survivors.As for the best value for liver transplantation,it was significant only for APACHE IV(P<0.001).CONCLUSION The APACHE IV assessment score was more accurate than BAR and MELD in predicting mortality in deceased donor liver transplant recipients.
基金supported by the National Natural Science Foundation of China(Nos.U2067212,22176191)the National Science Fund for Distinguished Young Scholars(No.21925603)。
文摘In the current era marked by energy shortages,the advancement of nuclear energy stands as an inevitable progression.The reprocessing of spent nuclear fuel plays a crucial role in determining the sustainability of nuclear energy as a viable energy source.Among these processes,the separation and recovery of Pu(Ⅳ)from high-level liquid waste(HLLW)hold paramount significance in terms of safety and strategic implications.Herein,this work focused on the synthesis of two acid-and radiation-resistant pyridine-based sp^(2)c-COFs(COF-IHEP3 and COF-IHEP4),followed by the creation of two pyridine-based ionized sp^(2)c-COFs named COF-IHEP3-CH_(3)NO_(3)and COF-IHEP4-CH3NO3through post-modification.These materials have potential anion exchange capacity for the selective separation of Pu(Ⅳ)in highly acidic conditions.Notably,in 8 mol/L nitric acid solution,COF-IHEP3-CH3NO3demonstrated the capability to eliminate plutonium within 20 min in 98%removal efficiency with a Kdvalue of 2450 m L/g.Experimental and theoretical analysis suggest that the ionized sp^(2)c-COFs exhibit exceptional stability,selectivity,and prevention of secondary contamination towards Pu(Ⅳ)in the presence of multiple ions environments.In short,this work provides an appropriate anion exchange strategy to design ionic sp^(2)c-COFs as a promising platform for Pu(Ⅳ)recovery from HLLW.
基金supported by the National Science Foundation of China(Nos.22525606,22176128,22236005,22406131,22506126)the Innovation Program of Shanghai Municipal Education Commission(No.2023ZKZD50)+13 种基金Shanghai Leading Talent Program of Eastern Talent Plan(No.LJ2023002)Shanghai Government(Nos.22dz1205400,23520711100)Chinese Education Ministry Key Laboratory and International Joint Laboratory on Resource ChemistryShanghai Eastern Scholar ProgramThe authors also thank Fellowship of China National Postdoctoral Program for Innovative Talents(No.BX20240229)the China Postdoctoral Science(No.2024M762100)the Foundation the Shanghai Science and Technology Commission Project(No.24ZR1455700)Shanghai Post-doctoral Excellence Pro-gram(No.2024787)the Chenguang Program of Shanghai Education Development Foundation and Shanghai Municipal Education Com-mission(No.24CGA49)the“111 Innovation and Talent Recruitment Base on Photochemical and Energy Materials”(No.D18020)Yunnan University Collaborative Innovation Center(Qujing Green Photovoltaic Industry Collaborative Innovation Center)Technology Talent and Platform Plan Project of Yunnan Provincial Department of Science and Technology(No.202305AF150088)Shanghai Engineering Research Center of Green Energy Chemical Engineering(No.18DZ2254200)Shanghai Frontiers Science Center of Biomimetic Catalysis.
文摘The efficient recovery of silver(Ag)from retired photovoltaic(PV)panels is crucial for resource sustainability and envi-ronmental protection.This study developed an environmentally friendly leaching method using ammonia(NH_(3)·H_(2)O)and hydrogen peroxide(H_(2)O_(2)),achieving the selective dissolution of Ag from retired crystalline silicon solar panels.Meanwhile,nonprecious metals such as aluminum(Al)and lead(Pb),which are commonly found in PV cells,were barely dissolved,dem-onstrating the excellent selectivity of this method for Ag.Light irradiation significantly improved the dissolution efficiency of Ag and reduced the amount of the reagent used.Ag dissolution occurred owing to a dual-pathway synergistic effect,which stemmed from the direct oxidation of Ag by H_(2)O_(2).The strongly oxidizing hydroxyl radicals generated by photocatalysis accelerated the oxidation and dissolution of Ag.In addition,NH 3·H_(2)O effectively promoted the dissolution and stabilization of oxidation products by forming soluble Ag–NH3·H2O complexes([Ag(NH3)2]+).This article reports an efficient,selective,and environmentally friendly strategy of Ag recovery and elucidates the radical-mediated dissolution mechanism under light-driven conditions,offering a feasible way for sustainably recovering valuable metals from retired PV panels.
