The structure-function relationship of a gellan family of polysaccharides, S-198 gum produced by Alcaligenes ATCC31853 was investigated in terms of rheological aspects. The flow curves of S-198 gum showed plastic beha...The structure-function relationship of a gellan family of polysaccharides, S-198 gum produced by Alcaligenes ATCC31853 was investigated in terms of rheological aspects. The flow curves of S-198 gum showed plastic behavior above 0.3%. Gelation did not occur in S-198 gum solution at low temperature (0℃), even at 0.8%. Both the viscosity and the elastic modulus remained constant with increasing temperature up to 80?C. The elastic modulus decreased a little with the addition of CaCl2 (6.8 mM), but then once again remained constant up to 80℃. The highest elastic modulus was observed for deacylated gellan gum with the addition of CaCl2 and increased slightly with increasing temperature up to 80℃, which was considered to be a transition temperature, after which it decreased rapidly. The elastic modulus of S-198 gum in the presence of urea (4.0 M) was lower than that in aqueous solution at low temperature (0℃), but remained constant with increasing temperature up to 80℃. The intramolecular associations, (hydrogen bonding and van der Waals forces of attraction), of S-198 gum molecules in aqueous solutions were proposed. The gellan family of polysaccharides, S-198, S-88, S-657, rhamsan, welan and gellan gum, provided a good opportunity to investigate the structure-function relationship for polysaccharides.展开更多
Introduction Family physicians(FPs)provide essential support during life’s most challenging moments,including experiences of grief and loss.After a patient’s death,FPs are expected to provide information and emotion...Introduction Family physicians(FPs)provide essential support during life’s most challenging moments,including experiences of grief and loss.After a patient’s death,FPs are expected to provide information and emotional support to bereaved family members.Prior research suggests that bereaved relatives expect follow-up contact from their FP,acknowledging it as part of the physician’s role.This study aims to explore the practices and attitudes of FPs towards bereaved family members.Methods A cross-sectional,descriptive study was conducted through an online questionnaire,exploring participants’perceived role in bereavement care,the type of support offered to families,available resources in primary care,as well as personal knowledge and approach on grief.This questionnaire was distributed via digital platforms to general and family medicine doctors.Results A total of 210 physicians participated(84.3%female;53.8%in residency),with a median age of 32 years old.On average,each FP experienced five patient deaths,most often reported by family members.More than half(53.3%)contacted bereaved families in fewer than 25%of cases,typically within 7 days of the death,most commonly by phone(76.5%).Conclusion This study highlights significant gaps in the bereavement follow-up practices of FPs in Portugal.Findings emphasise the need for integrated bereavement care guidelines in primary care,institutional policies and investment in targeted training programmes to enhance grief’s approach.The reported lack of grief training reinforces the need to integrate grief education into medical curriculum and continuous professional development.Structural improvements,such as the implementation of automatic death notifications within the healthcare system,are also critical for enabling timely and effective support.展开更多
Background:Continuous care for children with enterostomy and their families has been gaining popularity in China.Objective:To evaluate the feasibility of continuous care for children with enterostomy and their familie...Background:Continuous care for children with enterostomy and their families has been gaining popularity in China.Objective:To evaluate the feasibility of continuous care for children with enterostomy and their families in China.Methods:The PubMed,Web of Science,Embase,Cochrane Library,EBSCO,CNKI,CBM,VIP,and WanFang were searched for clinical trials until December 30,2025.Two reviewers independently searched articles,evaluated quality and extracted data.This review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses(PRISMA).Results:33 studies involving 2774 participants were included.The meta-analysis showed that continuous care strategy can significantly reduce the incidence of complications in children with enterostomy(OR=0.20,95%CI=0.16-0.26,p<0.001,I2=0%),effectively improve the family caregiver ability for enterostomy(MD=-10.34,95%CI=-13.82 to-6.85,p<0.001,I2=99%),shorten the time for family members to replace stoma bags(MD=-13.57,95%CI=-19.66 to-7.49,p<0.001,I2=100%),and alleviate negative emotions such as anxiety(SMD=-1.80,95%CI=-2.36 to-1.23,p<0.001,I2=92%)and depression(SMD=-1.54,95%CI=-2.04 to-1.04,p<0.001,I2=89%)in the families of the affected children.Conclusions:Continuous care can reduce complications of enterostomy in children,improve the family caregiver ability for enterostomy and alleviate negative emotions of family members such as anxiety and depression.展开更多
新学期伊始,翻开鲁科版五四制小学英语的第七单元,《Family》的标题赫然在目,和所有英语老师一样,我对这个单元的内容、流程再熟悉不过了。按照以往的经验,我会带着孩子们认读图片、跟读录音、分组进行角色扮演,最后再进行一次基础小测...新学期伊始,翻开鲁科版五四制小学英语的第七单元,《Family》的标题赫然在目,和所有英语老师一样,我对这个单元的内容、流程再熟悉不过了。按照以往的经验,我会带着孩子们认读图片、跟读录音、分组进行角色扮演,最后再进行一次基础小测验。孩子们大多能流利地指着图片说出“This is my father.”但我总感觉缺了点什么,这些词汇对于他们而言,似乎只是一张需要死记硬背的单词表,与那个充满烟火气、承载着他们所有喜怒哀乐的“家”,隔着一层看不见的屏障。直到我看到坐在窗边的小雅,在课本“全家福”插图的旁边,用铅笔认真画了一个歪歪扭扭的小人,旁边工工整整地写着“me”。展开更多
Objectives:In recent years,mental health has emerged as a pressing public health concern in China,driven by mounting societal pressures and fast-paced urban lifestyles.Physical activity,a well-established means of enh...Objectives:In recent years,mental health has emerged as a pressing public health concern in China,driven by mounting societal pressures and fast-paced urban lifestyles.Physical activity,a well-established means of enhancing psychological well-being,has received growing scholarly and policy attention.This study uses panel data from the 2020 and 2022 waves of the China Family Panel Studies(CFPS)to examine the impact of exercise frequency on mental health(with indicators such as CESD-8 depression scores)among college students and young employees,thereby providing empirical support for targeted mental health interventions.Methods:This study examines the relationship between individual exercise frequency and mental health among college students and young employees,using panel data from the 2020 and 2022 waves of the China Family Panel Studies(CFPS),with the Chinese version of the 8-item Center for Epidemiologic Studies Depression Scale(CESD-8)depression scores,self-rated health,and life satisfaction as outcome variables.Specifically,this study tests three hypotheses:(H1)increased exercise frequency significantly reduces depression symptoms and enhances well-being;(H2)the effects of exercise vary by social roles,with stronger mental health benefits among employed individuals and those with lower education;and(H3)lifestyle factors such as smoking amount,sleep duration,and Body Mass Index(BMI)partially mediate the relationship between exercise and mental health.Employing a two-way fixed effects model,baseline results indicate that a one-unit increase in exercise frequency significantly reduces the CESD-8 score by 0.183 points.To address potential endogeneity and spurious regression concerns,an instrumental variable(IV)approach is further applied.The heterogeneity analysis differentiates between students and employed individuals.Results:Among students,the effects of exercise on mental health are not statistically significant,regardless of education level.In contrast,for the employed,exercise demonstrates a significant positive impact on mental health,with particularly pronounced effects among those with lower educational attainment.These findings underscore the importance of promoting exercise as part of comprehensive mental health strategies.Mediation analysis indicates that the beneficial effect of exercise on mental health is partially transmitted through reductions in adverse health behaviors,especially smoking.Conclusions:Policymakers should integrate physical activity promotion into health interventions,prioritizing vulnerable groups to enhance psychological resilience and foster inclusive,health-oriented development.展开更多
Hodgkin lymphoma(HL)is a heterogenous lymphoproliferative disorder of B-cell origin and represents one of the most common malignancies in children and young adults.In addition to well-known underlying factors-such as ...Hodgkin lymphoma(HL)is a heterogenous lymphoproliferative disorder of B-cell origin and represents one of the most common malignancies in children and young adults.In addition to well-known underlying factors-such as Epstein-Barr virus infection-the familial aggregation demonstrated in large population studies suggested a genetic predisposition.First-degree relatives of patients with HL have an approximately threefold increased risk of developing the disease compared to the general population.These observations have recently prompted several whole-genome studies in affected families,identifying variants possibly implicated in lymphomagenesis,including alterations in DICER1(a member of the ribonuclease III family),POT1(protection of telomeres 1),KDR(kinase insert domain receptor),KLHDC8B(kelch domain-containing protein 8B),PAX5(paired box protein 5),GATA3(GATA binding protein 3),IRF7(interferon regulatory factor 7),EEF2KMT(eukaryotic elongation factor 2 lysine methyltransferase),and POLR1E(RNA polymerase I subunit E).In this article,we review current insights into the etiopathogenesis and risks of familial HL,and present case reports involving two sisters diagnosed with HL nearly 17 years apart.Recognizing the risk for first-degree relatives may potentially increase awareness of early symptoms among family members of HL patients,leading to earlier diagnosis and better outcomes.Conversely,understanding that the hereditary risk,though higher than in the general population,remains relatively low may provide reassurance for affected families.展开更多
BACKGROUND Familial adenomatous polyposis(FAP)is a disorder of autosomal dominant inheritance that is responsible for around 1%of colorectal cancer(CRC)cases.AIM To determine the mutation profile of FAP-specific to th...BACKGROUND Familial adenomatous polyposis(FAP)is a disorder of autosomal dominant inheritance that is responsible for around 1%of colorectal cancer(CRC)cases.AIM To determine the mutation profile of FAP-specific to the Hungarian population.METHODS This prospective single-center study enrolled patients with clinically suspected FAP or attenuated FAP(aFAP).Whole-exome next-generation sequencing was performed to detect variants of 50 FAP priority genes and 173 CRC predisposing genes or other CRC disease-associated genes.To identify larger deletions and insertions,a multiplex amplifiable probe hybridization technique was used.The identified genes were then classified according to the American College of Medical Genetics and Genomics guidelines.RESULTS A total of 26 index patients with clinically suspected FAP(n=21)and aFAP(n=5)were enrolled.APC gene alterations were confirmed in 92.31%of the cases(region 1B deletion,n=2;whole-gene deletion,n=4;frameshift mutation,n=2;nonsense mutation,n=5,and splice mutation,n=1),with the remaining two cases having CHEK2 and MSH3 gene alterations.According to pathogenicity,21 cases had pathogenic mutations,6 cases had likely pathogenic mutations,and 16 cases had variants of unknown significance(VUS).The most frequent of the latter were the POLE(n=5)and PIEZO1(n=4)gene variants.CONCLUSION Germline mutations in the APC gene were confirmed in more than 90%of Hungarian patients with clinically suspected FAP.Although the role of VUS genes is unclear,they are highly likely to play a role in the development of CRC.展开更多
Osmanthus fragrans is most famous for its strong aroma,and different varieties have different degrees of fragrance and color.Fragrance and color are important factors affecting the ornamental quality of O.fragrans.Ter...Osmanthus fragrans is most famous for its strong aroma,and different varieties have different degrees of fragrance and color.Fragrance and color are important factors affecting the ornamental quality of O.fragrans.Terpenoids are important secondary metabolites in plants,with β-carotene(C40)being the major pigment substance and linalool(C10)being the key aromatic component in O.fragrans.The geranylgeranyl pyrophosphate synthase genes(GGPPSs)play important roles in secondary metabolism in plants.However,the functions of the GGPPS family in floral color and fragrance formation has rarely been reported in O.fragrans.In this study,24 Of GGPPS genes were identified and classified into two subfamilies.The Of GGPPSs showed tissue-specific expression and Of GGPPS13 had highest expression in flowers.The Of GGPPS13 protein was localized to chloroplasts.The transcriptome data of Of GGPPS13 was verified by q RT-PCR and the expression level in‘Wanyingui'with strong aroma was higher than that in‘Zhuangyuanhong'with deep color at different flower development stages.Transient overexpression of Of GGPPS13 in O.fragrans petals showed that Of GGPPS13 increased the β-carotene content,the main color substance of O.fragrans,but decreased the linalool content,the main volatile organic compound(VOC)in the floral aroma of O.fragrans.Of GGPPS13 was indicated as the critical gene related to terpenoid synthesis in the floral aroma and color formation in O.fragrans.Our findings provide gene resources on the GGPPS gene family for further revealing the molecular regulation mechanism of the floral color and aroma formation in O.fragrans.展开更多
Background:Receptor-interacting protein kinases(RIPKs)regulate cell death,inflammation,and immune responses,yet their roles in cancer are not fully understood.This study investigates the expression,genomic alterations...Background:Receptor-interacting protein kinases(RIPKs)regulate cell death,inflammation,and immune responses,yet their roles in cancer are not fully understood.This study investigates the expression,genomic alterations,and functional implications of RIPK family members across various cancers.Methods:We collected multi-omics data from The Cancer Genome Atlas and other public databases,including gene expression,copy number variation(CNV),mutation,methylation,tumor mutation burden(TMB),and microsatellite instability(MSI).Differential expression and survival analyses were performed using DESeq2 and Cox proportional hazards models.CNV and mutation data were analyzed with GISTIC2 and Mutect2,and methylation data with the ChAMP package.Correlations with TMB and MSI were assessed using Pearson coefficients,and gene set enrichment analysis was conducted with the MSigDB Hallmark gene sets.Results:RIPK family members show significant differential expression in various cancers,with RIPK1 and RIPK4 frequently altered.Survival analysis reveals heterogeneous impacts on overall survival.CNV and mutation analyses identify high alteration frequencies for RIPK2 and RIPK7,affecting gene expression.RIPK1 and RIPK7 are hypermethylated in several cancers,inversely correlating with RIPK3 expression.RIPK1,RIPK2,RIPK5,RIPK6,and RIPK7 correlate positively with TMB,while RIPK3 shows negative correlations in some cancers.MSI analysis indicates associations with DNA mismatch repair.G ene set enrichment analysis highlights immune-related pathway enrichment for RIPK1,RIPK2,RIPK3,and RIPK6,and cell proliferation and DNA repair pathways for RIPK4 and RIPK5.RIPK family members showed heterogeneous alterations across cancers:for example,RIPK7 was mutated in up to~15%of u terine c orpus e ndometrial c arcinoma and l ung s quamous c ell c arcinoma cases,and RIPK1 and RIPK7 exhibited frequent promoter hypermethylation in multiple tumor types.Several genes displayed context-dependent associations with overall survival and with TMB/MSI.Conclusion:This pan-cancer analysis of the RIPK family reveals their diverse roles and potential as biomarkers and therapeutic targets.The findings emphasize the importance of RIPK genes in tumorigenesis and suggest context-dependent functions across cancer types.Further studies are needed to explore their mechanisms in cancer development and clinical applications.展开更多
The growing recognition of the role of genetics in the development of amyotrophic lateral sclerosis is evident.However,there has yet to be a comprehensive analysis of the clinical characteristics and genetics of famil...The growing recognition of the role of genetics in the development of amyotrophic lateral sclerosis is evident.However,there has yet to be a comprehensive analysis of the clinical characteristics and genetics of familial amyotrophic lateral sclerosis in an Asian population.This study aimed to provide an in-depth analysis of the clinical features and genetic spectrum of familial amyotrophic lateral sclerosis over 15 years in a clinic-based cohort of patients from the Chinese mainland.Enrollment of 302 amyotrophic lateral sclerosis families from 28 provinces was undertaken from January 2008 to September 2023.A group-based trajectory model for disease progression based on amyotrophic lateral sclerosis Functional Rating Scale-Revised(ALSFRS-R)scores was validated using bootstrap internal validation in patients with familial amyotrophic lateral sclerosis,as well as patients with sporadic amyotrophic lateral sclerosis(matched at a 1:4 ratio,with replacement).DNA samples from 244 index patients were screened for variants in the pathogenic genes SOD1,FUS,TDP43,and C9ORF72,of which 146 were also subjected to genome-wide next-generation sequencing.Gene-level burden analysis was used to evaluate the distribution of rare variants in the cohort.We found that rapid dynamic disease progression was associated with an older age at onset,shorter diagnostic delay,lower body mass index,bulbar onset,and≥1 affected first-degree relative.Certain attributes,such as age at onset and time from onset to diagnosis,had comparable impacts on the clinical progression trajectories of both familial amyotrophic lateral sclerosis and sporadic amyotrophic lateral sclerosis.Harboring pathogenic/likely pathogenic variants in amyotrophic lateral sclerosis-causative genes reduced the age of onset of familial amyotrophic lateral sclerosis.Among the patients with familial amyotrophic lateral sclerosis,17.8%possessed≥2 pathogenic/likely pathogenic variants.Sequencing kernel association test analysis showed that the SOD1 rare variant burden(P=1.3e-15)was associated with a significant risk of familial amyotrophic lateral sclerosis.Our findings conclusively confirmed the clinical features and genetic spectrum of familial amyotrophic lateral sclerosis over 15 years in a clinical cohort from China,contributing to a deeper understanding of genotype-phenotype relationships in familial amyotrophic lateral sclerosis.This comprehensive evaluation of specific clinical characteristics,clinical prognosis,and genetic variants of amyotrophic lateral sclerosis based on detailed clinical and genetic information may lead to the development of genotype-specific treatment approaches.展开更多
The Changbai Mountains,in Northeast China's Jilin Province,are covered by vast,wild forests.The mountainous region has chilly,snowy weather for about nine months of the year.The lowest temperature is below-40 C in...The Changbai Mountains,in Northeast China's Jilin Province,are covered by vast,wild forests.The mountainous region has chilly,snowy weather for about nine months of the year.The lowest temperature is below-40 C in winter.In 2005,Huang Yi,a native of Southwest China's Sichuan Province,relocated to the Changbai Mountains,to work at the Mount Hengshan frontier inspection station.展开更多
AIM:To investigate whether vaccinia-related kinase 1(VRK1)mediates transforming growth factor-beta2(TGF-β2)-caused epithelial-mesenchymal transition(EMT)and inflammatory responses in retinal pigment epithelial(RPE)ce...AIM:To investigate whether vaccinia-related kinase 1(VRK1)mediates transforming growth factor-beta2(TGF-β2)-caused epithelial-mesenchymal transition(EMT)and inflammatory responses in retinal pigment epithelial(RPE)cells through regulating snail family transcriptional repressor 1(SNAI1),and to validate its role in a proliferative vitreoretinopathy(PVR)mouse model.METHODS:Human RPE cell line ARPE-19 cells were treated with TGF-β2 to construct an EMT model.Western blot detected VRK1 level.The effects of VRK1 on SNAI1 expression and biological behavior of ARPE-19 cells were detected by immunofluorescence,ELISA,Transwell,and scratch assay,and the interaction between VRK1 and SNAI1 was confirmed through immunoprecipitation.A PVR mouse model was constructed,and the effects of VRK1 or/and SNAI1 on retinal damage were assessed by pathologic staining.Inflammatory factors and EMT-related proteins were assessed with ELISA and Western blot.RESULTS:VRK1 was upregulated in ARPE-19 cells after TGF-β2 treatment.Overexpression of VRK1 increased cell viability,promoted cell migration and EMT,and the levels of inflammatory factors.Silencing of VRK1 reversed the above indexes.There was a direct interaction between VRK1 and SNAI1,and overexpresssion SNAI1 weakened the impacts of silencing of VRK1.In PVR mice,silencing of VRK1 ameliorated retinal structural damage,decreased proinflammatory factor levels,and suppressed SNAI1 and mesenchymal marker expression.SNAI1 overexpression antagonized the protective effects of silencing VRK1 and exacerbated EMT and inflammatory responses.CONCLUSION:VRK1 plays a key role in retinal structural and inflammatory damage in PVR mice by regulating SNAI1 and mediating TGF-β2-caused EMT and inflammatory responses in RPE cells.展开更多
Dear Editor,We reported a Chinese family carrying a novel Crumbs homologue 1(CRB1)variant(c.1737_1755del).Consanguineous marriage resulted in a homozygous mutation,leading to the onset of Leber congenital amaurosis(LC...Dear Editor,We reported a Chinese family carrying a novel Crumbs homologue 1(CRB1)variant(c.1737_1755del).Consanguineous marriage resulted in a homozygous mutation,leading to the onset of Leber congenital amaurosis(LCA)in their offspring.展开更多
Objective Fatal familial insomnia （FFI） is an autosomal dominant prion disease characterized clinically by inattention, sleep loss, dysautonomia, and motor signs. This study is aimed to investigate clinical and fami...Objective Fatal familial insomnia （FFI） is an autosomal dominant prion disease characterized clinically by inattention, sleep loss, dysautonomia, and motor signs. This study is aimed to investigate clinical and familial characteristics often Chinese Patients with FFI. Methods We identified ten FFI cases from the surveillance network for Creutafeldt- Jakob disease （CJD） in China.Final diagnosis of FFI cases was made in accordance with the WHO criteria for CJD.The main clinical features and family histories of these ten FFI cases were analyzed. Results The median age of ten cases at onset was 38 years （from 19 to 55）. The foremost symptoms seemed to be various, including sleep disturbances, vision disorder, dizziness and anorexia. Sleep disturbances appeared in all cases and lasted in the whole clinical courses. Progressive sympathetic symptoms, memory loss, movement disturbances, myoclonus and hypertension were also frequently observed. The median duration of the disease was 9.5 months. EEG and MRI did not figure out special abnormality. 14-3-3 protein in CSF was positive in five out of eight tested patients. Clear family histories were identified in 8 patients. Conclusion The data from our study confirm that the Chinese FFI cases have similar clinical characteristics as that of the Caucasian cases. Compared with other genetic CJD associated mutations, the genetic frequencies of D178N in PRNP are apparently high among the Chinese cases.展开更多
Magnesium-based materials not only exhibit desirable characteristics such as low density and high specific strength, but also possess exceptional functional properties, including high damping capacity, high thermal co...Magnesium-based materials not only exhibit desirable characteristics such as low density and high specific strength, but also possess exceptional functional properties, including high damping capacity, high thermal conductivity, high electromagnetic interference shielding capacity, flame retardancy, and dissolvability. However, achieving a balance between strength and functional properties remains a significant challenge in Mg alloys community. Typically, strength depends on the pinning effect of defects, such as solute atoms and second phases,which hinder dislocation motion. On the other hand, optimal functional properties usually necessitate relative perfect crystal structures, as the presence of solute atoms and second phases can have adverse effects on damping capacity and thermal conductivity. Balancing these conflicting requirements is difficult. The trade-off between strength and functional properties of the Mg alloys should be broken to meet the urgent need in aerospace, automotive, 3C(computers, communications, and consumer electronics) and energy industries for high performance structural-functional integrated Mg-based materials. This review summarizes recent progress in understanding the mechanisms and influencing factors for the functional properties of Mg alloys. The mechanisms underlying the trade-off between strength and functional properties of Mg alloys is discussed. The latest developed structural-functional integrated Mg alloys and their composites are summarized, including high strength Mg-based materials with high damping capacity/high thermal conductivity/strong electromagnetic shielding capability/excellent flame-resistance/high dissolution rate. The future works of developing structure-function integrated Mg-based materials are proposed.展开更多
In family healthcare nursing, the family system unit (i.e., a group in which the members, seen as a whole, mutually interact) is the target of care. As nurses tend to obtain family-related information from particular ...In family healthcare nursing, the family system unit (i.e., a group in which the members, seen as a whole, mutually interact) is the target of care. As nurses tend to obtain family-related information from particular family members in the clinical setting, when assessing families, they often confront the issue of the differences between the theoretical level and methodological level. Although this issue needs resolving for evidence-based family nursing practice, sufficient research is lacking on the methodology related to family assessment. The present study aimed to clarify the factors that affected evaluation of family functioning among couples. Semi-structured interviews were conducted with 10 child-rearing families (couples) using the Survey of Family Environment (SFE) as a family functioning scale. Content analysis identified 12 factors that affected discrepancies in the couples’ evaluations and eight factors that affected agreement in those evaluations. These factors were classified into three categories: factors concerning family or family members;factors concerning questions related to the SFE;and factors concerning the view of the family as a whole. The results of this study should contribute to the development of family assessment tools and effective methods for evaluation of family care.展开更多
This study demonstrates the feasibility of producing three polysaccharides(neutral LJP-1,acidic LJP-2 and acidic LJP-3)with significant in vitro and in vivo anti-inflammatory activities from the flowers of Lonicera ja...This study demonstrates the feasibility of producing three polysaccharides(neutral LJP-1,acidic LJP-2 and acidic LJP-3)with significant in vitro and in vivo anti-inflammatory activities from the flowers of Lonicera japonica.The three polysaccharides differed in chemical composition,molecular weight(Mw)distribution,glycosidic linkage pattern,functional groups and morphology.They exhibited excellent protective effects(in a dose-dependent manner)in lipopolysaccharide-injured RAW264.7 macrophages and Cu SO4-damaged zebrafish via reducing NO production and inhibiting the overexpressions of inflammation-related transcription factors,inflammatory proteins and cytokines in the NF-κB/MAPK signaling pathways.Their antiinflammatory effects varied owing to their different molecular characteristics and chemical compositions.Overall,LJP-2 at 400μg/m L was the most effective.LJP-2 consisted mainly of→5)-α-L-Araf(1→,→4)-α-LGalp A(1→and→2)-α-L-Rhap(1→residues with terminal T-β-D-Glcp.Thus,honeysuckle flowers are good sources of anti-inflammatory polysaccharides,and precise fractionation enables the production of potent antiinflammatory agents for the development of functional foods and healthcare products.展开更多
Objectives: The families of these children experience distress both at the time of diagnosis and afterward.A top priority is to understand family empowerment,family function,and family members' quality of life (Qo...Objectives: The families of these children experience distress both at the time of diagnosis and afterward.A top priority is to understand family empowerment,family function,and family members' quality of life (QoL) and to effectively support these families in Japan.The objective of this study was to assess the actual conditions of families living with children having DDs and to explore the factors associated with family empowerment and parents' QoL.Methods: We surveyed ninety-three parents (78 mothers,15 fathers) from 78 families which lived with children with DDs in the capital region of Japan.We assessed two main outcomes using the Japanese versions of the following instruments: Family Empowerment Scale (FES),World Health Organization Quality of Life 26 (WHOQOL26),and other six outcomes.Correlation and multiple regression analyses were conducted.Results: No medication,cooperation with child rearing,assistance from a developmental support center,solved problems related to child rearing,and higher scores in Problem Solving contributed to higher FES scores.Higher WHOQOL26 scores were related to being a full-time housewife,higher self-esteem,no developmental support,a broad emotional support network,higher scores in Problem Solving and Role Function,and lower scores in Affective Reaction and General Function.Conclusions: We revealed that family empowerment and QoL of parents rearing children with DDs in Japan were affected by various subscales of family function and other family attributes.Effective interventions for improving family empowerment and QoL should be researched in the future.展开更多
Background: About 70% of Japanese children with severe motor and intellectual disabilities (SMID) live at home, and the number is increasing. Family members have an enormous burden of daily physical care for these chi...Background: About 70% of Japanese children with severe motor and intellectual disabilities (SMID) live at home, and the number is increasing. Family members have an enormous burden of daily physical care for these children. A top priority is to understand quality of life (QoL), family function, and family empowerment to effectively support these families. We aimed to assess current living situations of families with a SMID child, and to reveal the relationships between QoL, family function, and family empowerment. Methods: Sixty-five family members from 34 families with a SMID child participated in this study. We assessed 5 parameters using the Japanese versions of the following instruments: World Health Organization Quality of Life 26 (WHOQOL26), Kinder Lebensqualitats Fragebogen (KINDL), Family Assessment Device (FAD), Family Adaptability and Cohesion Evaluation Scale KG-4 (FACESKG-4), and Family Empowerment Scale (FES). Correlation and multiple regression analyses were conducted;QoL score was the objective variable. Results: Participants included 54 parents (34 mothers, 20 fathers) and 11 siblings. The mean age of SMID children was 10.4 ± 5.03 years. Twenty-two children needed multiple types of medical care. The mean age of parents and siblings was 41.5 ± 6.16 years and 15.5 ± 2.35 years, respectively. The mean QoL score (3.28 ± 0.5) was similar to the Japanese average. The mean KINDL score (77.2 ± 12.1) was higher than those of previous studies. The mean FAD score was 1.97 ± 0.32. For FACEKG-4, the score of adaptability was correlated with WHOQOL score (r = 0.459, p < 0.05). The mean score of FES was 113.6 ± 14. As the result of multiple regression analysis, lower family FAD scores ([sb] = ?0.61, p < 0.01) indicated higher family function and greater age of participants (sb = 0.495, p < 0.01) was correlated with higher WHOQOL scores (F = 15.208, p < 0.01). Conclusions: Our results indicated that the individual QoL depended on the age of participants (equals the years of experience caring for a SMID child) and the recognition of family function as a whole. Thus, to improve family members’ QoL, we should focus on individuals and also approach the family as a whole.展开更多
文摘The structure-function relationship of a gellan family of polysaccharides, S-198 gum produced by Alcaligenes ATCC31853 was investigated in terms of rheological aspects. The flow curves of S-198 gum showed plastic behavior above 0.3%. Gelation did not occur in S-198 gum solution at low temperature (0℃), even at 0.8%. Both the viscosity and the elastic modulus remained constant with increasing temperature up to 80?C. The elastic modulus decreased a little with the addition of CaCl2 (6.8 mM), but then once again remained constant up to 80℃. The highest elastic modulus was observed for deacylated gellan gum with the addition of CaCl2 and increased slightly with increasing temperature up to 80℃, which was considered to be a transition temperature, after which it decreased rapidly. The elastic modulus of S-198 gum in the presence of urea (4.0 M) was lower than that in aqueous solution at low temperature (0℃), but remained constant with increasing temperature up to 80℃. The intramolecular associations, (hydrogen bonding and van der Waals forces of attraction), of S-198 gum molecules in aqueous solutions were proposed. The gellan family of polysaccharides, S-198, S-88, S-657, rhamsan, welan and gellan gum, provided a good opportunity to investigate the structure-function relationship for polysaccharides.
文摘Introduction Family physicians(FPs)provide essential support during life’s most challenging moments,including experiences of grief and loss.After a patient’s death,FPs are expected to provide information and emotional support to bereaved family members.Prior research suggests that bereaved relatives expect follow-up contact from their FP,acknowledging it as part of the physician’s role.This study aims to explore the practices and attitudes of FPs towards bereaved family members.Methods A cross-sectional,descriptive study was conducted through an online questionnaire,exploring participants’perceived role in bereavement care,the type of support offered to families,available resources in primary care,as well as personal knowledge and approach on grief.This questionnaire was distributed via digital platforms to general and family medicine doctors.Results A total of 210 physicians participated(84.3%female;53.8%in residency),with a median age of 32 years old.On average,each FP experienced five patient deaths,most often reported by family members.More than half(53.3%)contacted bereaved families in fewer than 25%of cases,typically within 7 days of the death,most commonly by phone(76.5%).Conclusion This study highlights significant gaps in the bereavement follow-up practices of FPs in Portugal.Findings emphasise the need for integrated bereavement care guidelines in primary care,institutional policies and investment in targeted training programmes to enhance grief’s approach.The reported lack of grief training reinforces the need to integrate grief education into medical curriculum and continuous professional development.Structural improvements,such as the implementation of automatic death notifications within the healthcare system,are also critical for enabling timely and effective support.
文摘Background:Continuous care for children with enterostomy and their families has been gaining popularity in China.Objective:To evaluate the feasibility of continuous care for children with enterostomy and their families in China.Methods:The PubMed,Web of Science,Embase,Cochrane Library,EBSCO,CNKI,CBM,VIP,and WanFang were searched for clinical trials until December 30,2025.Two reviewers independently searched articles,evaluated quality and extracted data.This review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses(PRISMA).Results:33 studies involving 2774 participants were included.The meta-analysis showed that continuous care strategy can significantly reduce the incidence of complications in children with enterostomy(OR=0.20,95%CI=0.16-0.26,p<0.001,I2=0%),effectively improve the family caregiver ability for enterostomy(MD=-10.34,95%CI=-13.82 to-6.85,p<0.001,I2=99%),shorten the time for family members to replace stoma bags(MD=-13.57,95%CI=-19.66 to-7.49,p<0.001,I2=100%),and alleviate negative emotions such as anxiety(SMD=-1.80,95%CI=-2.36 to-1.23,p<0.001,I2=92%)and depression(SMD=-1.54,95%CI=-2.04 to-1.04,p<0.001,I2=89%)in the families of the affected children.Conclusions:Continuous care can reduce complications of enterostomy in children,improve the family caregiver ability for enterostomy and alleviate negative emotions of family members such as anxiety and depression.
文摘新学期伊始,翻开鲁科版五四制小学英语的第七单元,《Family》的标题赫然在目,和所有英语老师一样,我对这个单元的内容、流程再熟悉不过了。按照以往的经验,我会带着孩子们认读图片、跟读录音、分组进行角色扮演,最后再进行一次基础小测验。孩子们大多能流利地指着图片说出“This is my father.”但我总感觉缺了点什么,这些词汇对于他们而言,似乎只是一张需要死记硬背的单词表,与那个充满烟火气、承载着他们所有喜怒哀乐的“家”,隔着一层看不见的屏障。直到我看到坐在窗边的小雅,在课本“全家福”插图的旁边,用铅笔认真画了一个歪歪扭扭的小人,旁边工工整整地写着“me”。
文摘Objectives:In recent years,mental health has emerged as a pressing public health concern in China,driven by mounting societal pressures and fast-paced urban lifestyles.Physical activity,a well-established means of enhancing psychological well-being,has received growing scholarly and policy attention.This study uses panel data from the 2020 and 2022 waves of the China Family Panel Studies(CFPS)to examine the impact of exercise frequency on mental health(with indicators such as CESD-8 depression scores)among college students and young employees,thereby providing empirical support for targeted mental health interventions.Methods:This study examines the relationship between individual exercise frequency and mental health among college students and young employees,using panel data from the 2020 and 2022 waves of the China Family Panel Studies(CFPS),with the Chinese version of the 8-item Center for Epidemiologic Studies Depression Scale(CESD-8)depression scores,self-rated health,and life satisfaction as outcome variables.Specifically,this study tests three hypotheses:(H1)increased exercise frequency significantly reduces depression symptoms and enhances well-being;(H2)the effects of exercise vary by social roles,with stronger mental health benefits among employed individuals and those with lower education;and(H3)lifestyle factors such as smoking amount,sleep duration,and Body Mass Index(BMI)partially mediate the relationship between exercise and mental health.Employing a two-way fixed effects model,baseline results indicate that a one-unit increase in exercise frequency significantly reduces the CESD-8 score by 0.183 points.To address potential endogeneity and spurious regression concerns,an instrumental variable(IV)approach is further applied.The heterogeneity analysis differentiates between students and employed individuals.Results:Among students,the effects of exercise on mental health are not statistically significant,regardless of education level.In contrast,for the employed,exercise demonstrates a significant positive impact on mental health,with particularly pronounced effects among those with lower educational attainment.These findings underscore the importance of promoting exercise as part of comprehensive mental health strategies.Mediation analysis indicates that the beneficial effect of exercise on mental health is partially transmitted through reductions in adverse health behaviors,especially smoking.Conclusions:Policymakers should integrate physical activity promotion into health interventions,prioritizing vulnerable groups to enhance psychological resilience and foster inclusive,health-oriented development.
文摘Hodgkin lymphoma(HL)is a heterogenous lymphoproliferative disorder of B-cell origin and represents one of the most common malignancies in children and young adults.In addition to well-known underlying factors-such as Epstein-Barr virus infection-the familial aggregation demonstrated in large population studies suggested a genetic predisposition.First-degree relatives of patients with HL have an approximately threefold increased risk of developing the disease compared to the general population.These observations have recently prompted several whole-genome studies in affected families,identifying variants possibly implicated in lymphomagenesis,including alterations in DICER1(a member of the ribonuclease III family),POT1(protection of telomeres 1),KDR(kinase insert domain receptor),KLHDC8B(kelch domain-containing protein 8B),PAX5(paired box protein 5),GATA3(GATA binding protein 3),IRF7(interferon regulatory factor 7),EEF2KMT(eukaryotic elongation factor 2 lysine methyltransferase),and POLR1E(RNA polymerase I subunit E).In this article,we review current insights into the etiopathogenesis and risks of familial HL,and present case reports involving two sisters diagnosed with HL nearly 17 years apart.Recognizing the risk for first-degree relatives may potentially increase awareness of early symptoms among family members of HL patients,leading to earlier diagnosis and better outcomes.Conversely,understanding that the hereditary risk,though higher than in the general population,remains relatively low may provide reassurance for affected families.
基金Supported by the Research Grants of the National Research,Development and Innovation Office,No.K125377,No.K134863 and No.K143549New National Excellence Program of the Ministry of Human Capacities,No.UNKP-20-5-SZTE-161,No.UNKP-22-3-SZTE-233,No.UNKP-23-5-SZTE-719,No.UNKP-22-4-SZTE-296 and No.UNKP-22-3-SZTE-278+1 种基金Janos Bolyai Research Grant,No.BO/00723/22the Géza Hetényi Research Grant by Albert Szent-Györgyi Medical School,University of Szeged.
文摘BACKGROUND Familial adenomatous polyposis(FAP)is a disorder of autosomal dominant inheritance that is responsible for around 1%of colorectal cancer(CRC)cases.AIM To determine the mutation profile of FAP-specific to the Hungarian population.METHODS This prospective single-center study enrolled patients with clinically suspected FAP or attenuated FAP(aFAP).Whole-exome next-generation sequencing was performed to detect variants of 50 FAP priority genes and 173 CRC predisposing genes or other CRC disease-associated genes.To identify larger deletions and insertions,a multiplex amplifiable probe hybridization technique was used.The identified genes were then classified according to the American College of Medical Genetics and Genomics guidelines.RESULTS A total of 26 index patients with clinically suspected FAP(n=21)and aFAP(n=5)were enrolled.APC gene alterations were confirmed in 92.31%of the cases(region 1B deletion,n=2;whole-gene deletion,n=4;frameshift mutation,n=2;nonsense mutation,n=5,and splice mutation,n=1),with the remaining two cases having CHEK2 and MSH3 gene alterations.According to pathogenicity,21 cases had pathogenic mutations,6 cases had likely pathogenic mutations,and 16 cases had variants of unknown significance(VUS).The most frequent of the latter were the POLE(n=5)and PIEZO1(n=4)gene variants.CONCLUSION Germline mutations in the APC gene were confirmed in more than 90%of Hungarian patients with clinically suspected FAP.Although the role of VUS genes is unclear,they are highly likely to play a role in the development of CRC.
基金funded by the National Natural Science Foundation of China(32071828 and 32471943)the Central Finance Forestry Science and Technology Promotion Demonstration Project,China(Su2024TG04)+1 种基金the Priority Academic Program Development of Jiangsu Higher Education Institutions(PAPD),Chinapartly supported by the open funds of the National Key Laboratory for Germplasm Innovation&Utilization of Horticultural Crops,China。
文摘Osmanthus fragrans is most famous for its strong aroma,and different varieties have different degrees of fragrance and color.Fragrance and color are important factors affecting the ornamental quality of O.fragrans.Terpenoids are important secondary metabolites in plants,with β-carotene(C40)being the major pigment substance and linalool(C10)being the key aromatic component in O.fragrans.The geranylgeranyl pyrophosphate synthase genes(GGPPSs)play important roles in secondary metabolism in plants.However,the functions of the GGPPS family in floral color and fragrance formation has rarely been reported in O.fragrans.In this study,24 Of GGPPS genes were identified and classified into two subfamilies.The Of GGPPSs showed tissue-specific expression and Of GGPPS13 had highest expression in flowers.The Of GGPPS13 protein was localized to chloroplasts.The transcriptome data of Of GGPPS13 was verified by q RT-PCR and the expression level in‘Wanyingui'with strong aroma was higher than that in‘Zhuangyuanhong'with deep color at different flower development stages.Transient overexpression of Of GGPPS13 in O.fragrans petals showed that Of GGPPS13 increased the β-carotene content,the main color substance of O.fragrans,but decreased the linalool content,the main volatile organic compound(VOC)in the floral aroma of O.fragrans.Of GGPPS13 was indicated as the critical gene related to terpenoid synthesis in the floral aroma and color formation in O.fragrans.Our findings provide gene resources on the GGPPS gene family for further revealing the molecular regulation mechanism of the floral color and aroma formation in O.fragrans.
基金supported by grants from the Tianjin Health Technology Project(Grant no.2022QN106).
文摘Background:Receptor-interacting protein kinases(RIPKs)regulate cell death,inflammation,and immune responses,yet their roles in cancer are not fully understood.This study investigates the expression,genomic alterations,and functional implications of RIPK family members across various cancers.Methods:We collected multi-omics data from The Cancer Genome Atlas and other public databases,including gene expression,copy number variation(CNV),mutation,methylation,tumor mutation burden(TMB),and microsatellite instability(MSI).Differential expression and survival analyses were performed using DESeq2 and Cox proportional hazards models.CNV and mutation data were analyzed with GISTIC2 and Mutect2,and methylation data with the ChAMP package.Correlations with TMB and MSI were assessed using Pearson coefficients,and gene set enrichment analysis was conducted with the MSigDB Hallmark gene sets.Results:RIPK family members show significant differential expression in various cancers,with RIPK1 and RIPK4 frequently altered.Survival analysis reveals heterogeneous impacts on overall survival.CNV and mutation analyses identify high alteration frequencies for RIPK2 and RIPK7,affecting gene expression.RIPK1 and RIPK7 are hypermethylated in several cancers,inversely correlating with RIPK3 expression.RIPK1,RIPK2,RIPK5,RIPK6,and RIPK7 correlate positively with TMB,while RIPK3 shows negative correlations in some cancers.MSI analysis indicates associations with DNA mismatch repair.G ene set enrichment analysis highlights immune-related pathway enrichment for RIPK1,RIPK2,RIPK3,and RIPK6,and cell proliferation and DNA repair pathways for RIPK4 and RIPK5.RIPK family members showed heterogeneous alterations across cancers:for example,RIPK7 was mutated in up to~15%of u terine c orpus e ndometrial c arcinoma and l ung s quamous c ell c arcinoma cases,and RIPK1 and RIPK7 exhibited frequent promoter hypermethylation in multiple tumor types.Several genes displayed context-dependent associations with overall survival and with TMB/MSI.Conclusion:This pan-cancer analysis of the RIPK family reveals their diverse roles and potential as biomarkers and therapeutic targets.The findings emphasize the importance of RIPK genes in tumorigenesis and suggest context-dependent functions across cancer types.Further studies are needed to explore their mechanisms in cancer development and clinical applications.
基金supported by the Natural Science Foundation of Beijing,Nos.7244428(to WZ)and 7222215(to JH)the Peking University Medicine Sailing Program forYoung Scholars’Scientific and Technological Innovation,No.BMU2023YFJHPY034(to WZ)+4 种基金the National Natural Science Foundation of China,Nos.81873784,82071426(to DF),and81974197(to JH)the Clinical Cohort Construction Program of Peking University Third Hospital,No.BYSYDL2019002(to DF)Beijing Physician-Scientist TrainingProgram,No.BJPSTP-2024-03(to JH)the China Postdoctoral Science Foundation,Nos.2022TQ0014(to LX),2022M720284(to LX)the E-Town Cooperation&Development Foundation,No.YCXJ-JZ-2023-017(to LX).
文摘The growing recognition of the role of genetics in the development of amyotrophic lateral sclerosis is evident.However,there has yet to be a comprehensive analysis of the clinical characteristics and genetics of familial amyotrophic lateral sclerosis in an Asian population.This study aimed to provide an in-depth analysis of the clinical features and genetic spectrum of familial amyotrophic lateral sclerosis over 15 years in a clinic-based cohort of patients from the Chinese mainland.Enrollment of 302 amyotrophic lateral sclerosis families from 28 provinces was undertaken from January 2008 to September 2023.A group-based trajectory model for disease progression based on amyotrophic lateral sclerosis Functional Rating Scale-Revised(ALSFRS-R)scores was validated using bootstrap internal validation in patients with familial amyotrophic lateral sclerosis,as well as patients with sporadic amyotrophic lateral sclerosis(matched at a 1:4 ratio,with replacement).DNA samples from 244 index patients were screened for variants in the pathogenic genes SOD1,FUS,TDP43,and C9ORF72,of which 146 were also subjected to genome-wide next-generation sequencing.Gene-level burden analysis was used to evaluate the distribution of rare variants in the cohort.We found that rapid dynamic disease progression was associated with an older age at onset,shorter diagnostic delay,lower body mass index,bulbar onset,and≥1 affected first-degree relative.Certain attributes,such as age at onset and time from onset to diagnosis,had comparable impacts on the clinical progression trajectories of both familial amyotrophic lateral sclerosis and sporadic amyotrophic lateral sclerosis.Harboring pathogenic/likely pathogenic variants in amyotrophic lateral sclerosis-causative genes reduced the age of onset of familial amyotrophic lateral sclerosis.Among the patients with familial amyotrophic lateral sclerosis,17.8%possessed≥2 pathogenic/likely pathogenic variants.Sequencing kernel association test analysis showed that the SOD1 rare variant burden(P=1.3e-15)was associated with a significant risk of familial amyotrophic lateral sclerosis.Our findings conclusively confirmed the clinical features and genetic spectrum of familial amyotrophic lateral sclerosis over 15 years in a clinical cohort from China,contributing to a deeper understanding of genotype-phenotype relationships in familial amyotrophic lateral sclerosis.This comprehensive evaluation of specific clinical characteristics,clinical prognosis,and genetic variants of amyotrophic lateral sclerosis based on detailed clinical and genetic information may lead to the development of genotype-specific treatment approaches.
文摘The Changbai Mountains,in Northeast China's Jilin Province,are covered by vast,wild forests.The mountainous region has chilly,snowy weather for about nine months of the year.The lowest temperature is below-40 C in winter.In 2005,Huang Yi,a native of Southwest China's Sichuan Province,relocated to the Changbai Mountains,to work at the Mount Hengshan frontier inspection station.
文摘AIM:To investigate whether vaccinia-related kinase 1(VRK1)mediates transforming growth factor-beta2(TGF-β2)-caused epithelial-mesenchymal transition(EMT)and inflammatory responses in retinal pigment epithelial(RPE)cells through regulating snail family transcriptional repressor 1(SNAI1),and to validate its role in a proliferative vitreoretinopathy(PVR)mouse model.METHODS:Human RPE cell line ARPE-19 cells were treated with TGF-β2 to construct an EMT model.Western blot detected VRK1 level.The effects of VRK1 on SNAI1 expression and biological behavior of ARPE-19 cells were detected by immunofluorescence,ELISA,Transwell,and scratch assay,and the interaction between VRK1 and SNAI1 was confirmed through immunoprecipitation.A PVR mouse model was constructed,and the effects of VRK1 or/and SNAI1 on retinal damage were assessed by pathologic staining.Inflammatory factors and EMT-related proteins were assessed with ELISA and Western blot.RESULTS:VRK1 was upregulated in ARPE-19 cells after TGF-β2 treatment.Overexpression of VRK1 increased cell viability,promoted cell migration and EMT,and the levels of inflammatory factors.Silencing of VRK1 reversed the above indexes.There was a direct interaction between VRK1 and SNAI1,and overexpresssion SNAI1 weakened the impacts of silencing of VRK1.In PVR mice,silencing of VRK1 ameliorated retinal structural damage,decreased proinflammatory factor levels,and suppressed SNAI1 and mesenchymal marker expression.SNAI1 overexpression antagonized the protective effects of silencing VRK1 and exacerbated EMT and inflammatory responses.CONCLUSION:VRK1 plays a key role in retinal structural and inflammatory damage in PVR mice by regulating SNAI1 and mediating TGF-β2-caused EMT and inflammatory responses in RPE cells.
基金Supported by the National Natural Science Foundation of China(No.81970804)Natural Science Foundation of Hunan Province(No.2021JJ30949).
文摘Dear Editor,We reported a Chinese family carrying a novel Crumbs homologue 1(CRB1)variant(c.1737_1755del).Consanguineous marriage resulted in a homozygous mutation,leading to the onset of Leber congenital amaurosis(LCA)in their offspring.
基金supported by China Mega-Project for Infectious Disease(2009ZX10004-101,2008ZX)SKLID Development Grant(2008SKLID102,2011SKLID211)+3 种基金National Basic Research Program of China(973 Program)(2007CB310505)sponsored by the Young Scholar Scientific Research Foundation of China CDC(2012A102)Chinese National Natural Science Foundation Grants 30771914 and 30800975 Institution Technique R&D Grant(2008EG150300)
文摘Objective Fatal familial insomnia （FFI） is an autosomal dominant prion disease characterized clinically by inattention, sleep loss, dysautonomia, and motor signs. This study is aimed to investigate clinical and familial characteristics often Chinese Patients with FFI. Methods We identified ten FFI cases from the surveillance network for Creutafeldt- Jakob disease （CJD） in China.Final diagnosis of FFI cases was made in accordance with the WHO criteria for CJD.The main clinical features and family histories of these ten FFI cases were analyzed. Results The median age of ten cases at onset was 38 years （from 19 to 55）. The foremost symptoms seemed to be various, including sleep disturbances, vision disorder, dizziness and anorexia. Sleep disturbances appeared in all cases and lasted in the whole clinical courses. Progressive sympathetic symptoms, memory loss, movement disturbances, myoclonus and hypertension were also frequently observed. The median duration of the disease was 9.5 months. EEG and MRI did not figure out special abnormality. 14-3-3 protein in CSF was positive in five out of eight tested patients. Clear family histories were identified in 8 patients. Conclusion The data from our study confirm that the Chinese FFI cases have similar clinical characteristics as that of the Caucasian cases. Compared with other genetic CJD associated mutations, the genetic frequencies of D178N in PRNP are apparently high among the Chinese cases.
基金partially supported by National Natural Science Foundation of China (No.U21A2047,No.51971076,and No.51771062)National Key Research and Development Program of China (No.2022YFE0109600)。
文摘Magnesium-based materials not only exhibit desirable characteristics such as low density and high specific strength, but also possess exceptional functional properties, including high damping capacity, high thermal conductivity, high electromagnetic interference shielding capacity, flame retardancy, and dissolvability. However, achieving a balance between strength and functional properties remains a significant challenge in Mg alloys community. Typically, strength depends on the pinning effect of defects, such as solute atoms and second phases,which hinder dislocation motion. On the other hand, optimal functional properties usually necessitate relative perfect crystal structures, as the presence of solute atoms and second phases can have adverse effects on damping capacity and thermal conductivity. Balancing these conflicting requirements is difficult. The trade-off between strength and functional properties of the Mg alloys should be broken to meet the urgent need in aerospace, automotive, 3C(computers, communications, and consumer electronics) and energy industries for high performance structural-functional integrated Mg-based materials. This review summarizes recent progress in understanding the mechanisms and influencing factors for the functional properties of Mg alloys. The mechanisms underlying the trade-off between strength and functional properties of Mg alloys is discussed. The latest developed structural-functional integrated Mg alloys and their composites are summarized, including high strength Mg-based materials with high damping capacity/high thermal conductivity/strong electromagnetic shielding capability/excellent flame-resistance/high dissolution rate. The future works of developing structure-function integrated Mg-based materials are proposed.
文摘In family healthcare nursing, the family system unit (i.e., a group in which the members, seen as a whole, mutually interact) is the target of care. As nurses tend to obtain family-related information from particular family members in the clinical setting, when assessing families, they often confront the issue of the differences between the theoretical level and methodological level. Although this issue needs resolving for evidence-based family nursing practice, sufficient research is lacking on the methodology related to family assessment. The present study aimed to clarify the factors that affected evaluation of family functioning among couples. Semi-structured interviews were conducted with 10 child-rearing families (couples) using the Survey of Family Environment (SFE) as a family functioning scale. Content analysis identified 12 factors that affected discrepancies in the couples’ evaluations and eight factors that affected agreement in those evaluations. These factors were classified into three categories: factors concerning family or family members;factors concerning questions related to the SFE;and factors concerning the view of the family as a whole. The results of this study should contribute to the development of family assessment tools and effective methods for evaluation of family care.
基金supported by Key R&D Program of Shandong Province,China(2021CXGC010508)。
文摘This study demonstrates the feasibility of producing three polysaccharides(neutral LJP-1,acidic LJP-2 and acidic LJP-3)with significant in vitro and in vivo anti-inflammatory activities from the flowers of Lonicera japonica.The three polysaccharides differed in chemical composition,molecular weight(Mw)distribution,glycosidic linkage pattern,functional groups and morphology.They exhibited excellent protective effects(in a dose-dependent manner)in lipopolysaccharide-injured RAW264.7 macrophages and Cu SO4-damaged zebrafish via reducing NO production and inhibiting the overexpressions of inflammation-related transcription factors,inflammatory proteins and cytokines in the NF-κB/MAPK signaling pathways.Their antiinflammatory effects varied owing to their different molecular characteristics and chemical compositions.Overall,LJP-2 at 400μg/m L was the most effective.LJP-2 consisted mainly of→5)-α-L-Araf(1→,→4)-α-LGalp A(1→and→2)-α-L-Rhap(1→residues with terminal T-β-D-Glcp.Thus,honeysuckle flowers are good sources of anti-inflammatory polysaccharides,and precise fractionation enables the production of potent antiinflammatory agents for the development of functional foods and healthcare products.
基金This study was funded by the Ministry of Education,Culture,Sports,Science,and Technology of Japan (Grant-in-Aid for Young Scientists (A),2014-2018,No.26713057)
文摘Objectives: The families of these children experience distress both at the time of diagnosis and afterward.A top priority is to understand family empowerment,family function,and family members' quality of life (QoL) and to effectively support these families in Japan.The objective of this study was to assess the actual conditions of families living with children having DDs and to explore the factors associated with family empowerment and parents' QoL.Methods: We surveyed ninety-three parents (78 mothers,15 fathers) from 78 families which lived with children with DDs in the capital region of Japan.We assessed two main outcomes using the Japanese versions of the following instruments: Family Empowerment Scale (FES),World Health Organization Quality of Life 26 (WHOQOL26),and other six outcomes.Correlation and multiple regression analyses were conducted.Results: No medication,cooperation with child rearing,assistance from a developmental support center,solved problems related to child rearing,and higher scores in Problem Solving contributed to higher FES scores.Higher WHOQOL26 scores were related to being a full-time housewife,higher self-esteem,no developmental support,a broad emotional support network,higher scores in Problem Solving and Role Function,and lower scores in Affective Reaction and General Function.Conclusions: We revealed that family empowerment and QoL of parents rearing children with DDs in Japan were affected by various subscales of family function and other family attributes.Effective interventions for improving family empowerment and QoL should be researched in the future.
文摘Background: About 70% of Japanese children with severe motor and intellectual disabilities (SMID) live at home, and the number is increasing. Family members have an enormous burden of daily physical care for these children. A top priority is to understand quality of life (QoL), family function, and family empowerment to effectively support these families. We aimed to assess current living situations of families with a SMID child, and to reveal the relationships between QoL, family function, and family empowerment. Methods: Sixty-five family members from 34 families with a SMID child participated in this study. We assessed 5 parameters using the Japanese versions of the following instruments: World Health Organization Quality of Life 26 (WHOQOL26), Kinder Lebensqualitats Fragebogen (KINDL), Family Assessment Device (FAD), Family Adaptability and Cohesion Evaluation Scale KG-4 (FACESKG-4), and Family Empowerment Scale (FES). Correlation and multiple regression analyses were conducted;QoL score was the objective variable. Results: Participants included 54 parents (34 mothers, 20 fathers) and 11 siblings. The mean age of SMID children was 10.4 ± 5.03 years. Twenty-two children needed multiple types of medical care. The mean age of parents and siblings was 41.5 ± 6.16 years and 15.5 ± 2.35 years, respectively. The mean QoL score (3.28 ± 0.5) was similar to the Japanese average. The mean KINDL score (77.2 ± 12.1) was higher than those of previous studies. The mean FAD score was 1.97 ± 0.32. For FACEKG-4, the score of adaptability was correlated with WHOQOL score (r = 0.459, p < 0.05). The mean score of FES was 113.6 ± 14. As the result of multiple regression analysis, lower family FAD scores ([sb] = ?0.61, p < 0.01) indicated higher family function and greater age of participants (sb = 0.495, p < 0.01) was correlated with higher WHOQOL scores (F = 15.208, p < 0.01). Conclusions: Our results indicated that the individual QoL depended on the age of participants (equals the years of experience caring for a SMID child) and the recognition of family function as a whole. Thus, to improve family members’ QoL, we should focus on individuals and also approach the family as a whole.
