It has been well documented that grain size plays a critical role in the strain hardening behaviors of metals and alloys.However,the influence of grain size on the strain hardening of high entropy alloys(HEAs)was not ...It has been well documented that grain size plays a critical role in the strain hardening behaviors of metals and alloys.However,the influence of grain size on the strain hardening of high entropy alloys(HEAs)was not fully understood.Here,we report that the grain size not only affects the twinning-induced plasticity(TWIP)effect but also changes the dislocation-based deformation behaviors of face-centeredcubic(fcc)HEAs significantly.The strain hardening and deformation micro-mechanisms of NiCoCrFe and Ni_(2)CoCrFe were investigated using electron channeling contrast(ECCI)analysis.Our results showed that Ni_(2)CoCrFe exhibits a typical three-stage strain hardening behavior and NiCoCrFe shows the fourth stage at high strains due to the TWIP effect.For both NiCoCrFe and Ni_(2)CoCrFe,the increase of grain size leads to a transition of dislocation glide from wavy to planar mode,resulting in a low value and the recovery of strain hardening rate in stage II.The large-grain NiCoCrFe showed a higher strain hardening rate in stage IV due to the promoted deformation twinning.Combining the strain hardening behaviors of the TWIPNiCoCrFe and the mechanically stable Ni_(2)CoCrFe,we showed that the grain size influences the stage II hardening through tuning dislocation glide mode and the stage IV by tailoring deformation twinning activity of the Ni-Co-Cr-Fe HEAs.The grain size just affects stages I and III slightly in the current cases.These findings will also provide some insights into the understanding of strain hardening behaviors in other face-centered-cubic HEAs.展开更多
In the mammalian central ner vous system, neuronal loss induced by injuries or in neurodegenerative diseases is often irreversible(Quigley, 2016;Gan et al., 2018).Following the disease insult, the surviving neurons ma...In the mammalian central ner vous system, neuronal loss induced by injuries or in neurodegenerative diseases is often irreversible(Quigley, 2016;Gan et al., 2018).Following the disease insult, the surviving neurons may continue to lose their functionality because their axons degenerate and fail to maintain proper synaptic connections, and the underlying molecular and cellular mechanisms remain to be investigated(Raff et al., 2002;Bei et al., 2016;Quigley, 2016).展开更多
In neurodegenerative diseases,neuroinflammatory responses are often activated in resident immune cells in the central nervous system（CNS）（Schroder and Tschopp,2010）.Optic neuropathy refers to dysfunction and degen...In neurodegenerative diseases,neuroinflammatory responses are often activated in resident immune cells in the central nervous system（CNS）（Schroder and Tschopp,2010）.Optic neuropathy refers to dysfunction and degeneration of retinal ganglion cells（RGCs）and their axons,which is often induced by optic nerve injury or glaucomatous insult.Studies,展开更多
The three-dimensional hierarchical twin network has been proved to be the source of the excellent strength-ductility combination in the CoCrNi medium entropy alloy.Revealing the formation mechanism of hierarchical twi...The three-dimensional hierarchical twin network has been proved to be the source of the excellent strength-ductility combination in the CoCrNi medium entropy alloy.Revealing the formation mechanism of hierarchical twins,however,remains a challenge using either the post-mortem or the in-situ microstructural characterization.In this study,the atomistic formation mechanism of hierarchical twins was investigated using molecular dynamics simulations,with special focus on the effects of strain rate and deformation temperature.Compared to the primary twin boundaries kink-driven hierarchical twinning tendency in pure FCC metals,the chemical inhomogeneity in CoCrNi can reduce the necessary kink height to trigger conjugate twins(CTWs),fascinating the formation of twin networks.At room temperature,the plastic deformation is dominated by primary twins(PTWs)and conjugate slips at a relatively lower strain rate(e.g.,5×10^(7)s^(−1)).The hierarchical twins can be activated in cases of deforming at a higher strain rate(e.g.,2×10^(8)s^(−1)).Further increasing the strain rate(e.g.,1×10^(10) s^(−1))leads to the phase-transformation induced plasticity.At cryogenic temperatures,the hierarchical twins are promoted within a large range of strain rates(e.g.,5×10^(7)–1×10^(10) s^(−1)).A higher temperature leads to the synergy of CTWs and primary slips at a lower strain rate,but hierarchical twins at a higher strain rate.On this basis,a qualitative comparison and scalable trends between experiments and simulations were revealed.The present study would not only provide the basic understanding for the twinning behavior found experimentally,but also contribute to the design of medium/high entropy alloys with excellent mechanical performances by tuning microstructures.展开更多
Retinal ganglion cells(RGCs)receive synaptic inputs through their dendritic trees in the inner plexiform layer(IPL)and convey the visual information via their axons which form the optic nerve to the brain(Sanes and Ma...Retinal ganglion cells(RGCs)receive synaptic inputs through their dendritic trees in the inner plexiform layer(IPL)and convey the visual information via their axons which form the optic nerve to the brain(Sanes and Masland,2015).In glaucoma,RGCs and their axons degenerate and die,leading to irreversible vision loss and eventually blindness if left untreated(Quigley,2016).展开更多
In the article“Long non-coding RNA LINC02163 accelerates malignant tumor behaviors in breast cancer by regulating the microRNA-511-3p/HMGA2 axis as a competing endogenous RNA”(Oncology Research,2020,Vol.28,No.5,pp....In the article“Long non-coding RNA LINC02163 accelerates malignant tumor behaviors in breast cancer by regulating the microRNA-511-3p/HMGA2 axis as a competing endogenous RNA”(Oncology Research,2020,Vol.28,No.5,pp.483–495.doi:10.3727/096504020X15928179818438),there was an error in the processing of data.To further confirm our observation,we repeated multiple experiments involving in this study,including Flow Cytometry,Transwell Cell Migration and Invasion Assays,Xenograft Tumor Model,and Western Blotting.We have revised the figures to correct these errors.Corrected versions of the Figs.2,4,5,6,and 7 are provided.The corrections do not change any results or conclusion of the article.We apologize for any inconvenience caused.展开更多
Objective:This study was conducted to compare the effect of structured education program versus traditional diabetes education on insulin injection technique as well as physiologically and psychosocially related outco...Objective:This study was conducted to compare the effect of structured education program versus traditional diabetes education on insulin injection technique as well as physiologically and psychosocially related outcomes in adults with Type 2 diabetes.Materials and Methods:This study was a randomized controlled trial.Totally,71 Type 2 diabetes patients of using the insulin pen were enrolled and randomly divided into the intervention group(n=36)and the control group(n=35)according to random number table method.Patients in the intervention group received structured education program,while patients in the control group received traditional diabetes education.The levels of glycosylated hemoglobin(HbA1c),blood glucose,and lipids were monitored before and 3 months after intervention,and the insulin injection techniques,diabetes distress,and diabetes self-management skills between groups were assessed after 3 months.Results:Finally,65 participants(intervention group:n=33,control group:n=32)finished the study.After the intervention,HbA1c was lower(7.42±0.80 vs.8.35±1.40,P=0.002),fasting blood glucose(FBG)was lower(7.27±1.06 vs.9.40±3.16,P<0.001),and postprandial blood glucose(PBG)was lower(10.33±2.20 vs.12.77±4.94,P=0.012)in the intervention group than in the control group.No statistically significance was identified between the two groups in scores of Diabetes Distress Scale and summary of diabetes self-care activities.Conclusion:Patients with Type 2 diabetes received structured education program,with greater improvements achieved in HbA1c,FBG,PBG,and measures about insulin injection techniques compared to the traditional education group,but there was no significant difference in diabetes distress or self-management behavior between groups.展开更多
Paroxysmal dyskinesias are a group of neurological diseases characterized by intermittent episodes of involuntary movements with different causes.Paroxysmal kinesigenic dyskinesia(PKD)is the most common type of paroxy...Paroxysmal dyskinesias are a group of neurological diseases characterized by intermittent episodes of involuntary movements with different causes.Paroxysmal kinesigenic dyskinesia(PKD)is the most common type of paroxysmal dyskinesia and can be divided into primary and secondary types based on the etiology.Clinically,PKD is characterized by recurrent and transient attacks of involuntary movements precipitated by a sudden voluntary action.The major cause of primary PKD is genetic abnormalities,and the inheritance pattern of PKD is mainly autosomal-dominant with incomplete penetrance.The proline-rich transmembrane protein 2(PRRT2)was the first identified causative gene of PKD,accounting for the majority of PKD cases worldwide.An increasing number of studies has revealed the clinical and genetic characteristics,as well as the underlying mechanisms of PKD.By seeking the views of domestic experts,we propose an expert consensus regarding the diagnosis and treatment of PKD to help establish standardized clinical evaluation and therapies for PKD.In this consensus,we review the clinical manifestations,etiology,clinical diagnostic criteria and therapeutic recommendations for PKD,and results of genetic analyses in PKD patients performed in domestic hospitals.展开更多
Background: Glomerular disease is the leading cause of chronic kidney disease globally. No scoping review reports have focused on China’’s spectrum of glomerular diseases in children. This study aimed to systematica...Background: Glomerular disease is the leading cause of chronic kidney disease globally. No scoping review reports have focused on China’’s spectrum of glomerular diseases in children. This study aimed to systematically identify and describe retrospective studies on pediatric glomerular disease based on available data on sex, age, study period, and region.Methods: Six databases were systematically searched for relevant studies from initiation to December 2021 in PubMed, Embase, Web of Science, Global Health Library, Wangfang Database, and CNKI.Results: Thirty-four studies were identified in the scoping review, including 40,430 patients with biopsy-proven diagnoses. The proportion of boys was significantly higher than that of girls. In this study, 28,280 (70%) cases were primary glomerular disease, 10,547 (26.1%) cases were diagnosed as secondary glomerular disease, and 1146 (2.8%) cases were hereditary glomerular disease. Minimal change disease is the most common glomerular disease among children in China, followed by mesangial proliferative glomerulonephritis, IgA nephropathy, and purpura nephritis. We observed increments in glomerular diseases in periods 2 (2001–2010) and 3 (2011–2021). The proportion of major glomerular diseases varies significantly in the different regions of China.Conclusion: The spectrum of pediatric glomerular diseases varied across sex, age groups, study periods, and regions, and has changed considerably over the past 30 years.展开更多
Importance:Extensive population-based studies have explored the prevalence of primary hypertension(HTN)in children and adolescents.However,there is little published data on the characteristics of different types of pe...Importance:Extensive population-based studies have explored the prevalence of primary hypertension(HTN)in children and adolescents.However,there is little published data on the characteristics of different types of pediatric HTN and the causes of secondary HTN.Objective:To investigate the characteristics of different types of pediatric HTN and the causes of secondary HTN in a hospital setting.Methods:The study cohort comprised pediatric inpatients(<18 years of age)discharged with a diagnosis of HTN from Beijing Children's Hospital during 2015-2020.Pediatric patients with HTN were allocated to secondary and primary HTN groups on the basis of comprehensive analyses of their diagnoses,family history of HTN,and findings on physical examination,as documented in their medical records.The Mann-Whitney U test,χ^(2) and Fisher's exact test were used to assess differences in characteristics of patients with different HTN types and causes of secondary HTN.Results:Data of 1470 inpatients with HTN from 18 clinical departments were included in the analysis.Among them,458(31.2%)had primary HTN,and 1012(68.8%)had secondary HTN.Compared with patients had primary HTN,children with secondary HTN were younger and had lower body mass indexes and longer lengths of stay.Moreover,children with primary HTN had mostly been managed by the Endocrinology and Cardiology Departments,75.8%of them having obesity-related comorbidities.In contrast,most patients with secondary HTN had been managed by the Nephrology Department,renal diseases being the leading cause of their HTN(46.3%).Interpretation:Secondary HTN is more common than primary HTN in pediatric clinical settings,renal diseases being the leading cause of secondary HTN.展开更多
Today,earphones have almost been owned by everyone.However,wearing earphones for a long time can cause two serious problems:(1)irreversible damage to hearing;(2)rapid proliferation of bacteria in the ear canal.Herein,...Today,earphones have almost been owned by everyone.However,wearing earphones for a long time can cause two serious problems:(1)irreversible damage to hearing;(2)rapid proliferation of bacteria in the ear canal.Herein,an earphone modification strategy is developed for the first time,to reduce hearing loss and inhibit bacteria simultaneously.This earphone is equipped with a high purity yellow light(YL)light-emitting diode chip developed by our university.Then,the surface of the earphone is loaded with porous zinc oxide and silver nanoparticle composite material(ZnO-Ag)that can respond to the YL.Under the excitation of YL,the porous ZnO-Ag can release reactive oxygen species with strong antibacterial activity.More importantly,we discover that YL can reduce the expression of matrix metalloproteinase-3,the secretion of inflammatory factors,and apoptosis in the cochlea,thereby effectively reduce hearing loss.展开更多
The multiple PDZ domain crumbs cell polarity complex component gene(MPDZ;MIM:603785),is highly expressed in the brain across the whole lifespan.It encodes the multiple PDZ domain protein,which is a member of the NMDAR...The multiple PDZ domain crumbs cell polarity complex component gene(MPDZ;MIM:603785),is highly expressed in the brain across the whole lifespan.It encodes the multiple PDZ domain protein,which is a member of the NMDAR signaling complex that may play a role in the control of AMPAR potentiation and synaptic plasticity in excitatory synapses."Previously,MPDZ variants have been demonstrated to be associated with autosomal recessive congenital hydrocephalus-2(HYC2;MIM:615219)which is commonly complicated by brain abnormalities and developmental delay.Seizures were reported in only one case.The association between MPDz and epilepsy requires clarification.展开更多
Kidney disease is manifested in a wide variety of phenotypes,many of which have an important hereditary component.To delineate the genotypic and phenotypic spectrum of pediatric nephropathy,a multicenter registration ...Kidney disease is manifested in a wide variety of phenotypes,many of which have an important hereditary component.To delineate the genotypic and phenotypic spectrum of pediatric nephropathy,a multicenter registration system is being imple-mented based on the Chinese Children Genetic Kidney Disease Database(CCGKDD).In this study,all the patients with kidney and urological diseases were recruited from 2014 to 2020.Genetic analysis was conducted using exome sequencing for families with multiple affected individuals with nephropathy or clinical suspicion of a genetic kidney disease owing to early-onset or extrarenal features.The genetic diagnosis was confirmed in 883 of 2256(39.1%)patients from 23 provinces in China.Phenotypic profiles showed that the primary diagnosis included steroid-resistant nephrotic syndrome(SRNS,23.5%),glomerulonephritis(GN,32.2%),congenital anomalies of the kidney and urinary tract(CAKUT,21.2%),cystic renal disease(3.9%),renal calcinosis/stone(3.6%),tubulopathy(9.7%),and chronic kidney disease of unknown etiology(CKDu,5.8%).The pathogenic variants of 105 monogenetic disorders were identified.Ten distinct genomic disorders were identified as pathogenic copy number variants(CNVs)in 11 patients.The diagnostic yield differed by subgroups,and was highest in those with cystic renal disease(66.3%),followed by tubulopathy(58.4%),GN(57.7%),CKDu(43.5%),SRNS(29.2%),renal calcinosis/stone(29.3%)and CAKUT(8.6%).Reverse phenotyping permitted correct identification in 40 cases with clinical reassessment and unexpected genetic conditions.We present the results of the largest cohort of children with kidney disease in China where diagnostic exome sequencing was performed.Our data demonstrate the utility of family-based exome sequencing,and indicate that the combined analysis of genotype and phenotype based on the national patient registry is pivotal to the genetic diagnosis of kidney disease.展开更多
基金financial support from the National Natural Science Foundation of China(No.52001266)。
文摘It has been well documented that grain size plays a critical role in the strain hardening behaviors of metals and alloys.However,the influence of grain size on the strain hardening of high entropy alloys(HEAs)was not fully understood.Here,we report that the grain size not only affects the twinning-induced plasticity(TWIP)effect but also changes the dislocation-based deformation behaviors of face-centeredcubic(fcc)HEAs significantly.The strain hardening and deformation micro-mechanisms of NiCoCrFe and Ni_(2)CoCrFe were investigated using electron channeling contrast(ECCI)analysis.Our results showed that Ni_(2)CoCrFe exhibits a typical three-stage strain hardening behavior and NiCoCrFe shows the fourth stage at high strains due to the TWIP effect.For both NiCoCrFe and Ni_(2)CoCrFe,the increase of grain size leads to a transition of dislocation glide from wavy to planar mode,resulting in a low value and the recovery of strain hardening rate in stage II.The large-grain NiCoCrFe showed a higher strain hardening rate in stage IV due to the promoted deformation twinning.Combining the strain hardening behaviors of the TWIPNiCoCrFe and the mechanically stable Ni_(2)CoCrFe,we showed that the grain size influences the stage II hardening through tuning dislocation glide mode and the stage IV by tailoring deformation twinning activity of the Ni-Co-Cr-Fe HEAs.The grain size just affects stages I and III slightly in the current cases.These findings will also provide some insights into the understanding of strain hardening behaviors in other face-centered-cubic HEAs.
基金supported by NIH grants R01EY029121 (to XL and HFZ)R01EY026286 (to XL)。
文摘In the mammalian central ner vous system, neuronal loss induced by injuries or in neurodegenerative diseases is often irreversible(Quigley, 2016;Gan et al., 2018).Following the disease insult, the surviving neurons may continue to lose their functionality because their axons degenerate and fail to maintain proper synaptic connections, and the underlying molecular and cellular mechanisms remain to be investigated(Raff et al., 2002;Bei et al., 2016;Quigley, 2016).
文摘In neurodegenerative diseases,neuroinflammatory responses are often activated in resident immune cells in the central nervous system（CNS）（Schroder and Tschopp,2010）.Optic neuropathy refers to dysfunction and degeneration of retinal ganglion cells（RGCs）and their axons,which is often induced by optic nerve injury or glaucomatous insult.Studies,
基金the Natural Science Foundation of China(No.52175188)the State Key Laboratory for Mechanical Behavior of Materials(No.20222412)+2 种基金the Fundamental Research Funds for the Central Universities(No.3102019JC001)the Doctoral dissertation Innovation Fund for the Northwestern Polytechnical University(No.CX2022009)the Innovation and Entrepreneur-ship Training Program for College Students(No.202110699207).
文摘The three-dimensional hierarchical twin network has been proved to be the source of the excellent strength-ductility combination in the CoCrNi medium entropy alloy.Revealing the formation mechanism of hierarchical twins,however,remains a challenge using either the post-mortem or the in-situ microstructural characterization.In this study,the atomistic formation mechanism of hierarchical twins was investigated using molecular dynamics simulations,with special focus on the effects of strain rate and deformation temperature.Compared to the primary twin boundaries kink-driven hierarchical twinning tendency in pure FCC metals,the chemical inhomogeneity in CoCrNi can reduce the necessary kink height to trigger conjugate twins(CTWs),fascinating the formation of twin networks.At room temperature,the plastic deformation is dominated by primary twins(PTWs)and conjugate slips at a relatively lower strain rate(e.g.,5×10^(7)s^(−1)).The hierarchical twins can be activated in cases of deforming at a higher strain rate(e.g.,2×10^(8)s^(−1)).Further increasing the strain rate(e.g.,1×10^(10) s^(−1))leads to the phase-transformation induced plasticity.At cryogenic temperatures,the hierarchical twins are promoted within a large range of strain rates(e.g.,5×10^(7)–1×10^(10) s^(−1)).A higher temperature leads to the synergy of CTWs and primary slips at a lower strain rate,but hierarchical twins at a higher strain rate.On this basis,a qualitative comparison and scalable trends between experiments and simulations were revealed.The present study would not only provide the basic understanding for the twinning behavior found experimentally,but also contribute to the design of medium/high entropy alloys with excellent mechanical performances by tuning microstructures.
基金supported by National Institute of Health (NIH) grants R01EY029121 (to XL and HFZ) and U01EY033001 (to HFZ)
文摘Retinal ganglion cells(RGCs)receive synaptic inputs through their dendritic trees in the inner plexiform layer(IPL)and convey the visual information via their axons which form the optic nerve to the brain(Sanes and Masland,2015).In glaucoma,RGCs and their axons degenerate and die,leading to irreversible vision loss and eventually blindness if left untreated(Quigley,2016).
文摘In the article“Long non-coding RNA LINC02163 accelerates malignant tumor behaviors in breast cancer by regulating the microRNA-511-3p/HMGA2 axis as a competing endogenous RNA”(Oncology Research,2020,Vol.28,No.5,pp.483–495.doi:10.3727/096504020X15928179818438),there was an error in the processing of data.To further confirm our observation,we repeated multiple experiments involving in this study,including Flow Cytometry,Transwell Cell Migration and Invasion Assays,Xenograft Tumor Model,and Western Blotting.We have revised the figures to correct these errors.Corrected versions of the Figs.2,4,5,6,and 7 are provided.The corrections do not change any results or conclusion of the article.We apologize for any inconvenience caused.
文摘Objective:This study was conducted to compare the effect of structured education program versus traditional diabetes education on insulin injection technique as well as physiologically and psychosocially related outcomes in adults with Type 2 diabetes.Materials and Methods:This study was a randomized controlled trial.Totally,71 Type 2 diabetes patients of using the insulin pen were enrolled and randomly divided into the intervention group(n=36)and the control group(n=35)according to random number table method.Patients in the intervention group received structured education program,while patients in the control group received traditional diabetes education.The levels of glycosylated hemoglobin(HbA1c),blood glucose,and lipids were monitored before and 3 months after intervention,and the insulin injection techniques,diabetes distress,and diabetes self-management skills between groups were assessed after 3 months.Results:Finally,65 participants(intervention group:n=33,control group:n=32)finished the study.After the intervention,HbA1c was lower(7.42±0.80 vs.8.35±1.40,P=0.002),fasting blood glucose(FBG)was lower(7.27±1.06 vs.9.40±3.16,P<0.001),and postprandial blood glucose(PBG)was lower(10.33±2.20 vs.12.77±4.94,P=0.012)in the intervention group than in the control group.No statistically significance was identified between the two groups in scores of Diabetes Distress Scale and summary of diabetes self-care activities.Conclusion:Patients with Type 2 diabetes received structured education program,with greater improvements achieved in HbA1c,FBG,PBG,and measures about insulin injection techniques compared to the traditional education group,but there was no significant difference in diabetes distress or self-management behavior between groups.
文摘Paroxysmal dyskinesias are a group of neurological diseases characterized by intermittent episodes of involuntary movements with different causes.Paroxysmal kinesigenic dyskinesia(PKD)is the most common type of paroxysmal dyskinesia and can be divided into primary and secondary types based on the etiology.Clinically,PKD is characterized by recurrent and transient attacks of involuntary movements precipitated by a sudden voluntary action.The major cause of primary PKD is genetic abnormalities,and the inheritance pattern of PKD is mainly autosomal-dominant with incomplete penetrance.The proline-rich transmembrane protein 2(PRRT2)was the first identified causative gene of PKD,accounting for the majority of PKD cases worldwide.An increasing number of studies has revealed the clinical and genetic characteristics,as well as the underlying mechanisms of PKD.By seeking the views of domestic experts,we propose an expert consensus regarding the diagnosis and treatment of PKD to help establish standardized clinical evaluation and therapies for PKD.In this consensus,we review the clinical manifestations,etiology,clinical diagnostic criteria and therapeutic recommendations for PKD,and results of genetic analyses in PKD patients performed in domestic hospitals.
基金Pillar Program of National Science & Technology(Grant/Award Number: 2015BAI12B06)National key clinical specialty capacity building project(Grant/Award Number: 2019-542)。
文摘Background: Glomerular disease is the leading cause of chronic kidney disease globally. No scoping review reports have focused on China’’s spectrum of glomerular diseases in children. This study aimed to systematically identify and describe retrospective studies on pediatric glomerular disease based on available data on sex, age, study period, and region.Methods: Six databases were systematically searched for relevant studies from initiation to December 2021 in PubMed, Embase, Web of Science, Global Health Library, Wangfang Database, and CNKI.Results: Thirty-four studies were identified in the scoping review, including 40,430 patients with biopsy-proven diagnoses. The proportion of boys was significantly higher than that of girls. In this study, 28,280 (70%) cases were primary glomerular disease, 10,547 (26.1%) cases were diagnosed as secondary glomerular disease, and 1146 (2.8%) cases were hereditary glomerular disease. Minimal change disease is the most common glomerular disease among children in China, followed by mesangial proliferative glomerulonephritis, IgA nephropathy, and purpura nephritis. We observed increments in glomerular diseases in periods 2 (2001–2010) and 3 (2011–2021). The proportion of major glomerular diseases varies significantly in the different regions of China.Conclusion: The spectrum of pediatric glomerular diseases varied across sex, age groups, study periods, and regions, and has changed considerably over the past 30 years.
基金National Natural Science Foundation of China(81973110)。
文摘Importance:Extensive population-based studies have explored the prevalence of primary hypertension(HTN)in children and adolescents.However,there is little published data on the characteristics of different types of pediatric HTN and the causes of secondary HTN.Objective:To investigate the characteristics of different types of pediatric HTN and the causes of secondary HTN in a hospital setting.Methods:The study cohort comprised pediatric inpatients(<18 years of age)discharged with a diagnosis of HTN from Beijing Children's Hospital during 2015-2020.Pediatric patients with HTN were allocated to secondary and primary HTN groups on the basis of comprehensive analyses of their diagnoses,family history of HTN,and findings on physical examination,as documented in their medical records.The Mann-Whitney U test,χ^(2) and Fisher's exact test were used to assess differences in characteristics of patients with different HTN types and causes of secondary HTN.Results:Data of 1470 inpatients with HTN from 18 clinical departments were included in the analysis.Among them,458(31.2%)had primary HTN,and 1012(68.8%)had secondary HTN.Compared with patients had primary HTN,children with secondary HTN were younger and had lower body mass indexes and longer lengths of stay.Moreover,children with primary HTN had mostly been managed by the Endocrinology and Cardiology Departments,75.8%of them having obesity-related comorbidities.In contrast,most patients with secondary HTN had been managed by the Nephrology Department,renal diseases being the leading cause of their HTN(46.3%).Interpretation:Secondary HTN is more common than primary HTN in pediatric clinical settings,renal diseases being the leading cause of secondary HTN.
基金the National Natural Science Foundation of China(No.31860263)Key Youth Project of Jiangxi Province(No.20202ACB216002).
文摘Today,earphones have almost been owned by everyone.However,wearing earphones for a long time can cause two serious problems:(1)irreversible damage to hearing;(2)rapid proliferation of bacteria in the ear canal.Herein,an earphone modification strategy is developed for the first time,to reduce hearing loss and inhibit bacteria simultaneously.This earphone is equipped with a high purity yellow light(YL)light-emitting diode chip developed by our university.Then,the surface of the earphone is loaded with porous zinc oxide and silver nanoparticle composite material(ZnO-Ag)that can respond to the YL.Under the excitation of YL,the porous ZnO-Ag can release reactive oxygen species with strong antibacterial activity.More importantly,we discover that YL can reduce the expression of matrix metalloproteinase-3,the secretion of inflammatory factors,and apoptosis in the cochlea,thereby effectively reduce hearing loss.
基金funded by the National Natural Science Foundation of China(No.82201609)Shandong Medical and Health Science and Technology Development Plan(China)(No.202106010271)+2 种基金Scientific Research Project of Hunan Provincial Health Commission(China)(No.D202303077290)Guangdong Basic and Applied Basic Research Foundation(China)(No.2021A1515111064)Science and Technology Project of Guangzhou,Guangdong,China(No.202102021059,202201020106,202235395).
文摘The multiple PDZ domain crumbs cell polarity complex component gene(MPDZ;MIM:603785),is highly expressed in the brain across the whole lifespan.It encodes the multiple PDZ domain protein,which is a member of the NMDAR signaling complex that may play a role in the control of AMPAR potentiation and synaptic plasticity in excitatory synapses."Previously,MPDZ variants have been demonstrated to be associated with autosomal recessive congenital hydrocephalus-2(HYC2;MIM:615219)which is commonly complicated by brain abnormalities and developmental delay.Seizures were reported in only one case.The association between MPDz and epilepsy requires clarification.
基金J.R.is supported by National Natural Science Foundation of China(NSFC-8182207)Shanghai Academic/Technology Research Leader(19XD1420600)Chinese Academy of Medical Sciences(2019-RC-HL_020).
文摘Kidney disease is manifested in a wide variety of phenotypes,many of which have an important hereditary component.To delineate the genotypic and phenotypic spectrum of pediatric nephropathy,a multicenter registration system is being imple-mented based on the Chinese Children Genetic Kidney Disease Database(CCGKDD).In this study,all the patients with kidney and urological diseases were recruited from 2014 to 2020.Genetic analysis was conducted using exome sequencing for families with multiple affected individuals with nephropathy or clinical suspicion of a genetic kidney disease owing to early-onset or extrarenal features.The genetic diagnosis was confirmed in 883 of 2256(39.1%)patients from 23 provinces in China.Phenotypic profiles showed that the primary diagnosis included steroid-resistant nephrotic syndrome(SRNS,23.5%),glomerulonephritis(GN,32.2%),congenital anomalies of the kidney and urinary tract(CAKUT,21.2%),cystic renal disease(3.9%),renal calcinosis/stone(3.6%),tubulopathy(9.7%),and chronic kidney disease of unknown etiology(CKDu,5.8%).The pathogenic variants of 105 monogenetic disorders were identified.Ten distinct genomic disorders were identified as pathogenic copy number variants(CNVs)in 11 patients.The diagnostic yield differed by subgroups,and was highest in those with cystic renal disease(66.3%),followed by tubulopathy(58.4%),GN(57.7%),CKDu(43.5%),SRNS(29.2%),renal calcinosis/stone(29.3%)and CAKUT(8.6%).Reverse phenotyping permitted correct identification in 40 cases with clinical reassessment and unexpected genetic conditions.We present the results of the largest cohort of children with kidney disease in China where diagnostic exome sequencing was performed.Our data demonstrate the utility of family-based exome sequencing,and indicate that the combined analysis of genotype and phenotype based on the national patient registry is pivotal to the genetic diagnosis of kidney disease.
