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clinical and molecular characterization of 10 Chinese children with congenital adrenal hyperplasia due to 11beta‑hydroxylase deficiency

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World Journal of Pediatrics 2024年第4期20卷 422-433页

作者： Wen‑Li Lu Xiao‑Yu Ma Jiao Zhang Jun‑Qi Wang Ting‑Ting Zhang Lei Ye Yuan Xiao Zhi‑Ya dong Wei Wang Shou‑Yue Sun Chuan‑Yin Li Rong‑Gui Hu guang Ning Li‑Dan Zhang Department of Pediatrics Ruijin HospitalShanghai Jiao Tong University School of MedicineNo.197Ruijin 2nd RdHuangpu DistrictShanghai 200025China Department of Pediatric Genetic and metabolic Endocrinology West China Second University HospitalSichuan UniversityNo.20Section 3Renmin South RoadSichuan 610041China Department of Endocrine and metabolic diseases Shanghai Clinical Center for Endocrine and Metabolic DiseasesRuijin HospitalShanghai Jiao Tong University School of MedicineShanghai 200025China Cancer center School of MedicineShanghai Tenth People’s HospitalTongji UniversityYanchang Zhong Lu 301St RdJing’an DistrictShanghai 200031China

Background The clinical manifestations of nonclassical 11beta-hydroxylase deficiency are very similar to those of nonclassical 21-hydroxylase deficiency.For this study,we investigated the relationship between the clinical and molecular features of congenital adrenal hyperplasia caused by 11beta-hydroxylase deficiency and reviewed the related literature,which are expected to provide assistance for the clinical diagnosis and analysis of congenital adrenal hyperplasia.Methods clinical data for 10 Chinese patients diagnosed with congenital adrenal hyperplasia in our hospital from 2018 to 2022 were retrospectively analyzed.We examined the effects of gene mutations on protease activity and constructed threedimensional structure prediction models of proteins.Results We describe 10 patients with 11beta-hydroxylase gene mutations(n=5,46,XY;n=5,46,XX),with 10 novel mutations were reported.Female patients received treatment at an early stage,with an average age of 2.08±1.66 years,whereas male patients received treatment significantly later,at an average age of 9.77±3.62 years.The most common CYP11B1 pathogenic variant in the Chinese population was found to be c.1360C>T.All mutations lead to spatial conformational changes that affect protein stability.Conclusions Our study found that there was no significant correlation between each specific mutation and the severity of clinical manifestations.Different patients with the same gene pathogenic variant may have mild or severe clinical manifestations.The correlation between genotype and phenotype needs further study.Three-dimensional protein simulations may provide additional support for the physiopathological mechanism of genetic mutations.

关键词： 11β-hydroxylase deficiency CYP11B1 Enzymatic activity Phenotype-genotype correlation Threedimensional protein simulations

metabolic memory:mechanisms and diseases

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Signal Transduction and Targeted Therapy 2024年第3期9卷 1086-1114页

作者： Hao dong Yuezhang Sun Lulingxiao Nie Aimin Cui Pengfei Zhao Wai Keung Leung Qi Wang State Key Laboratory of Oral diseases&National center for Stomatology&National clinical research center for Oral diseases West China Hospital of StomatologySichuan UniversityChengduChina Department of Prosthodontics West China Hospital of StomatologySichuan UniversityChengduChina Periodontology and Implant Dentistry Division Faculty of DentistryThe University of Hong KongHong KongChina

metabolic diseases and their complications impose health and economic burdens worldwide.Evidence from past experimental studies and clinical trials suggests our body may have the ability to remember the past metabolic environment,such as hyperglycemia or hyperlipidemia,thus leading to chronic inflammatory disorders and other diseases even after the elimination of these metabolic environments.The long-term effects of that aberrant metabolism on the body have been summarized as metabolic memory and are found to assume a crucial role in states of health and disease.Multiple molecular mechanisms collectively participate in metabolic memory management,resulting in different cellular alterations as well as tissue and organ dysfunctions,culminating in disease progression and even affecting offspring.The elucidation and expansion of the concept of metabolic memory provides more comprehensive insight into pathogenic mechanisms underlying metabolic diseases and complications and promises to be a new target in disease detection and management.Here,we retrace the history of relevant research on metabolic memory and summarize its salient characteristics.We provide a detailed discussion of the mechanisms by which metabolic memory may be involved in disease development at molecular,cellular,and organ levels,with emphasis on the impact of epigenetic modulations.Finally,we present some of the pivotal findings arguing in favor of targeting metabolic memory to develop therapeutic strategies for metabolic diseases and provide the latest reflections on the consequences of metabolic memory as well as their implications for human health and diseases.

关键词： diseases metabolism alterations

SGLT2 inhibitor monotherapy alleviates hyperglycemia in heredity severe insulin resistance syndrome

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中华医学杂志(英文版) 2024年第11期137卷 1372-1374页

作者： Wu Huixuan Iqbal Junaid Li Long Jiang Hongli Chao Chen Zhang Meibiao Li Ou Zhou Houde Department of Metabolism and Endocrinology National Clinical Research Center for Metabolic Diseases Hunan Provincial Key Laboratory for Metabolic Bone Diseases Key Laboratory of Diabetes Immunology Ministry of Education The Second Xiangya Hospital of Central South University Changsha Hunan China Department of Endocrinology and Metabolism The First People’s Hospital of Huaihua Huaihua Hunan China Department of Metabolism and Endocrinology San-Sui County Traditional Chinese Medicine Hospital Qiandongnan Miao and Dong Autonomous Prefecture Guizhou China

Hepatic Zbtb18 (Zinc Finger and BTB Domain Containing 18) alleviates hepatic steatohepatitis via FXR (Farnesoid X Receptor)

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Signal Transduction and Targeted Therapy 2024年第2期9卷 766-781页

作者： Lei Zhang Jiabing Chen Xiaoying Yang Chuangpeng Shen Jiawen Huang dong Zhang Naihua Liu Chaonan Liu Yadi Zhong Yingjian Chen Kaijia Tang Jingyi Guo Tianqi Cui Siwei Duan Jiayu Li Shangyi Huang Huafeng Pan Huabing Zhang Xiaoqiang Tang Yongsheng Chang Yong Gao State Key Laboratory of Traditional Chinese Medicine Syndrome Science and Technology Innovation CenterGuangzhou University of Chinese MedicineGuangzhouChina Key Laboratory of Immune Microenvironment and Disease(Ministry of Education) Tianjin Key Laboratory of Cellular Homeostasis and DiseaseDepartment of Physiology and PathophysiologyTianjin Medical UniversityTianjinChina Jiangsu Key Laboratory of Immunity and Metabolism Department of Pathogen Biology and ImmunologyJiangsu International Laboratory of Immunity and MetabolismXuzhou Medical UniversityXuzhouChina Department of Endocrinology The First Clinical CollegeGuangzhou University of Chinese MedicineGuangdongChina The Fourth clinical Medical College of guangzhou University of Chinese Medicine ShenzhenChina Jiangsu Collaborative Innovation center of Traditional Chinese Medicine in Prevention and Treatment of Tumor Nanjing University of Chinese MedicineNanjingChina Department of Biochemistry and Molecular Biology Metabolic Disease Research CenterSchool of Basic MedicineAnhui Medical UniversityHefeiChina Key Laboratory of Birth Defects and Related diseases of Women and Children of MOE State Key Laboratory of BiotherapyWest China Second University HospitalSichuan UniversityChengduChina

A lasting imbalance between fatty acid synthesis and consumption leads to non-alcoholic fatty liver disease(NAFLD),coupled with hepatitis and insulin resistance.Yet the details of the underlying mechanisms are not fully understood.Here,we unraveled that the expression of the transcription factor Zbtb18 is markedly decreased in the livers of both patients and murine models of NAFLD.Hepatic Zbtb18 knockout promoted NAFLD features like impaired energy expenditure and fatty acid oxidation(FAO),and induced insulin resistance.Conversely,hepatic Zbtb18 overexpression alleviated hepato-steatosis,insulin resistance,and hyperglycemia in mice fed on a high-fat diet(HFD)or in diabetic mice.Notably,in vitro and in vivo mechanistic studies revealed that Zbtb18 transcriptional activation of Farnesoid X receptor(FXR)mediated FAO and Clathrin Heavy Chain(CLTC)protein hinders NLRP3 inflammasome activity.This key mechanism by which hepatocyte’s Zbtb18 expression alleviates NAFLD and consequent liver fibrosis was further verified by FXR’s deletion and forced expression in mice and cultured mouse primary hepatocytes(MPHs).Moreover,CLTC deletion significantly abrogated the hepatic Zbtb18 overexpression-driven inhibition of NLRP3 inflammasome activity in macrophages.Altogether,Zbtb18 transcriptionally activates the FXR-mediated FAO and CLTC expression,which inhibits NLRP3 inflammasome’s activity alleviating inflammatory stress and insulin resistance,representing an attractive remedy for hepatic steatosis and fibrosis.

关键词： hepatic Hepatic impaired

Diagnosis,treatment and prevention of severe acute respiratory syndrome coronavirus 2 infection in children:experts'consensus statement updatedforthe Omicron variant

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World Journal of Pediatrics 2024年第3期20卷 272-286页

作者： Rong-Meng Jiang Zheng-De Xie Yi Jiang Xiao-Xia Lu Run-Ming Jin Yue-Jie Zheng Yun-Xiao Shang Bao-Ping Xu Zhi-Sheng Liu Gen Lu Ji-Kui Deng guang-Hua Liu Xiao-Chuan Wang Jian-She Wang Lu-Zhao Feng Wei Liu Yi Zheng Sai-Nan Shu Min Lu Wan-Jun Luo Miao Liu Yu-Xia Cui Le-Ping Ye A-dong Shen Gang Liu Li-Wei Gao Li-Juan Xiong Yan Bai Li-Kai Lin Zhuang Wei Feng-Xia Xue Tian-You Wang dong-Chi Zhao Jian-Bo Shao Daniel Kwok-keung Ng Gary Wing-kin Wong Zheng-Yan Zhao Xing-Wang Li Yong-Hong Yang Kun-Ling Shen Diagnosis and Treatment center of Infectious diseases Beijing Ditan HospitalCapital Medical UniversityBeijing 100015China Beijing Key Laboratory of Pediatric Respiratory Infection diseases Key Laboratory of Major Diseases in ChildrenMinistry of EducationNational Clinical Research Center for Respiratory DiseasesResearch Unit of Critical Infection in ChildrenChinese Academy of Medical Sciences2019RU016Laboratory of Infection and VirologyBeijing Pediatric Research InstituteBeijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijing 100045China Department of Pediatrics Renmin Hospital of Wuhan UniversityWuhan 430060China Department of Respiratory Wuhan Children's HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430016China Department of Pediatrics Union HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430022China Department of Respiratory Shenzhen Children's HospitalShenzhen 518038China Department of Pediatric Respiratory Shengjing Hospital Affiliated to China Medical UniversityShenyang 110004China Department of Respiratory Beijing Children's HospitalCapital Medical UniversityNational Clinical Research Center for Respiratory DiseasesNational Center for Children's HealthBeijing 100045China Department of Neurology Wuhan Children's HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430016China Department of Respiratory Guangzhou Women and Children's Medical CenterGuangzhou 510623China Department of Infectious diseases Shenzhen Children's HospitalShenzhen 518038China Department of Pediatrics Fujian Branch of Shanghai Children's Medical CenterFujian Children's HospitalFuzhou350005China Department of clinical Immunology and Allergy Children's Hospital of Fudan UniversityNational Center for Children's HealthShanghai 201102China Department of Infectious diseases Children's Hospital of Fudan UniversityNational Center for Children's Health.Shanghai

It has been more than 3 years since the novel coronavirus(SARS-CoV-2)pandemic raged globally.The coronavirus disease 2019(COVID-19)has greatly influenced human society.According to data from the World Health Organization(WHO),there were over 656 million confirmed cases of COVID-19 in the world as of January 1,2023,including over 6.6 million deaths[1].

关键词： prevention infection statement

Identification of novel mammalian viruses in tree shrews(Tupaia belangeri chinensis)

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Zoological research 2024年第2期45卷 429-438页

作者： Hong Zhou Ren-Rong Tian Xiu-Rong Wang Jin-Xuan Yang Yun-Xiao Wang Ming-Liang Zhao Xu-dong Zhang Yu-Hua Ma Long-Bao Lv Edward CHolmes Yong-Tang Zheng Wei-Feng Shi School of clinical and Basic Medical Sciences Shandong First Medical University&Shandong Academy of Medical SciencesJi’nanShandong 250117China Key Laboratory of Emerging Infectious diseases in Universities of Shandong Shandong First Medical University and Shandong Academy of Medical SciencesTaianShandong 271000China Key Laboratory of Animal Models and Human Disease Mechanisms of the Chinese Academy of Sciences/Key Laboratory of Bioactive Peptides of Yunnan Province KIZ-CUHK Joint Laboratory of Bioresources and Molecular Research in Common DiseasesCenter for Biosafety Mega-ScienceKunming Institute of ZoologyChinese Academy of SciencesKunmingYunnan 650223China School of Public Health Shandong First Medical University&Shandong Academy of Medical SciencesJi’nanShandong 250117China National Resource center for Non-Human Primates Kunming Primate Research CenterKunming Institute of ZoologyChinese Academy of SciencesKunmingYunnan 650107China Sydney Institute for Infectious diseases School of Medical SciencesThe University of SydneySydneyNew South Wales 2006Australia Department of Infectious diseases Ruijin HospitalShanghai Jiao Tong University School of MedicineShanghai 200025China Shanghai Institute of Virology Shanghai Jiao Tong University School of MedicineShanghai 200025China

The Chinese tree shrew(Tupaia belangeri chinensis),a member of the mammalian order Scandentia,exhibits considerable similarities with primates,including humans,in aspects of its nervous,immune,and metabolic systems.These similarities have established the tree shrew as a promising experimental model for biomedical research on cancer,infectious diseases,metabolic disorders,and mental health conditions.Herein,we used metatranscriptomic sequencing to analyze plasma,as well as oral and anal swab samples,from 105 healthy asymptomatic tree shrews to identify the presence of potential zoonotic viruses.In total,eight mammalian viruses with complete genomes were identified,belonging to six viral families,including Flaviviridae,Hepeviridae,Parvovirinae,Picornaviridae,Sedoreoviridae,and Spinareoviridae.Notably,the presence of rotavirus was recorded in tree shrews for the first time.Three viruses-hepacivirus 1,parvovirus,and picornavirus-exhibited low genetic similarity(94%)to known viral strains.Phylogenetic analyses also revealed that the rotavirus and mammalian orthoreovirus identified in this study may be novel reassortants.These findings provide insights into the diverse viral spectrum present in captive Chinese tree shrews,highlighting the necessity for further research into their potential for crossspecies transmission.

关键词： Tree shrew(Tupaia belangeri chinensis) Meta-transcriptomic sequencing Mammalian viruses Genomic analysis

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评价散寒化湿颗粒与奈玛特韦-利托那韦对照治疗轻型/中型新型冠状病毒感染有效性和安全性的随机、开放、多中心临床研究（英文）

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Science Bulletin 2024年第12期 1954-1963页

作者：邹晓辉常康范国辉郑欢伟神和正唐亮杨映映王业明赵林华吕红周昕沈小明陈利强仝小林曹彬 The Second Affiliated Hospital of Jiaxing University Huizhou First Hospital National center for Integrated Traditional Chinese Medicine and Western Medicine China-Japan Friendship Hospital 中国肺炎研究网 National center for Respiratory Medicine State Key Laboratory of Respiratory Health and Multimorbidity National Clinical Research Center for Respiratory Diseases Institute of Respiratory Medicine Chinese Academy of Medical Sciences Laboratory of Clinical Microbiology and Infectious Diseases Department of Pulmonary and Critical Care Medicine Center of Respiratory Medicine China-Japan Friendship Hospital National center for Respiratory Medicine State Key Laboratory of Respiratory Health and Multimorbidity National Clinical Research Center for Respiratory Diseases Institute of Respiratory Medicine Chinese Academy of Medical Sciences Department of Clinical Research and Data Management Center of Respiratory Medicine Center of Respiratory Medicine China-Japan Friendship Hospital Shijiazhuang Hospital of Traditional Chinese Medicine Linyi Traditional Chinese Medicine Hospital Wuxi Traditional Chinese Medicine Hospital National center for Respiratory Medicine State Key Laboratory of Respiratory Health and Multimorbidity National Clinical Research Center for Respiratory Diseases Institute of Respiratory Medicine Chinese Academy of Medical Sciences Department of Traditional Chinese Medicine for Pulmonary Diseases Center of Respiratory Medicine China-Japan Friendship Hospital Institute of metabolic diseases Guang'anmen Hospital of China Academy of Chinese Medical Sciences Taicang Traditional Chinese Medicine Hospital The First Hospital of Qiqihar

Sanhan Huashi granules(SHG) demonstrated therapeutic effects against coronavirus disease 2019(COVID-19) in observational studies. In order to compare the effectiveness and safety of SHG and nirmatrelvir–ritonavir in treating adults with mild-to-moderate COVID-19, we conducted a randomized, activecontrolled, open-label, multi-center trial conducted between February and July in 2023. The patients were randomized in a 1:1 ratio to the SHG group and the nirmatrelvir–ritonavir group. A total of 400participants were randomized, among which 200 participants ultimately received SHG and 198 received nirmatrelvir–ritonavir. The primary outcome was time to sustained clinical recovery through day 28. SHG significantly shortened the median time to sustained clinical recovery compared to nirmatrelvir–ritonavir(6.0(95% CI, 5.0 to 6.0) vs. 8.0(95% CI, 6.0 to 9.0) d; P = 0.001), particularly for individual symptoms including fever, sore throat, cough and fatigue. No participants in either group died and incidence of severe COVID-19 showed no difference between two groups. Participants who received nirmatrelvir–ritonavir demonstrated a higher rate of virus clearance on day 5 compared to those received SHG(46.4%(95% CI, 39.1 to 53.7) vs. 65.6%(95% CI, 58.3 to 72.4); P clinical recovery in participants with mild-to-moderate COVID-19, despite a lower virus clearance rate observed after 5 d of treatment(Chinese clinical Trial Registry Identifier: Chi CTR2300067872).

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Associations of sleeping patterns and isotemporal substitution of other behavior with the prevalence of CKD in Chinese adults

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Frontiers of Medicine 2024年第2期18卷 303-314页

作者： Yi Ding Xiaoli Xu Zhuojun Xin Qiuyu Cao Jiaojiao Huang Xianglin Wu Yanan Huo Qin Wan Yingfen Qin Ruying Hu Lixin Shi Qing Su Xuefeng Yu Li Yan Guijun Qin Xulei Tang Gang Chen Min Xu Tiange Wang Zhiyun Zhao Zhengnan Gao Guixia Wang Feixia Shen Zuojie Luo Li Chen Qiang Li Zhen Ye Yinfei Zhang Chao Liu Youmin Wang Tao Yang Huacong Deng Lulu Chen Tianshu Zeng Jiajun Zhao Yiming Mu Shengli Wu Yuhong Chen Jieli Lu Weiqing Wang guang Ning Yu Xu Yufang Bi Mian Li Department of Endocrine and metabolic diseases Shanghai Institute of Endocrine and Metabolic DiseasesRuijin HospitalShanghai Jiao Tong University School of MedicineShanghai200025China Shanghai National clinical research center for metabolic diseases Key Laboratory for Endocrine and Metabolic Diseases of the National Health Commission of ChinaShanghai Key Laboratory for Endocrine TumorState Key Laboratory of Medical GenomicsRuijin HospitalShanghai Jiao Tong University School of MedicineShanghai200025China Jiangxi Provincial People’s Hospital Affiliated to Nanchang UniversityNanchang330000China Department of Endocrinology The Affiliated Hospital of Southwest Medical UniversityLuzhou646000China Department of Endocrinology The First Affiliated Hospital of Guangxi Medical UniversityNanning530000China Zhejiang Provincial center for Disease Control and Prevention Hangzhou310000China Department of Endocrinology Affiliated Hospital of Guiyang Medical UniversityGuiyang550000China Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai200000China Tongji Hospital Tongji Medical CollegeHuazhong University of Science and TechnologyWuhan430000China Sun Yat-sen Memorial Hospital Sun Yat-sen UniversityGuangzhou510000China Department of Endocrinology The First Affiliated Hospital of Zhengzhou UniversityZhengzhou450000China Department of Endocrinology The First Hospital of Lanzhou UniversityLanzhou730000China Fujian Provincial Hospital Fujian Medical UniversityFuzhou350000China Dalian Municipal Central Hospital Affiliated of Dalian Medical UniversityDalian116000China Department of Endocrinology The First Hospital of Jilin UniversityChangchun130000China Department of Endocrinology The First Affiliated Hospital of Wenzhou Medical UniversityWenzhou325000China Qilu Hospital of Shandong University Jinan250000China Department of Endocrinology The Second Affiliated Hospital of Harbin Medical UniversityHarbin150000China Department of Endocrinology Cen

Studies have found a U-shaped relationship between sleep duration and chronic kidney disease(CKD)risk,but limited research evaluated the association of reallocating excessive sleep to other behavior with CKD.We included 104538 participants from the nationwide cohort of the Risk Evaluation of Cancers in Chinese Diabetic Individuals:A Longitudinal Study,with self-reported time of daily-life behavior.Using isotemporal substitution models,we found that substituting 1 h of sleeping with sitting,walking,or moderate-to-vigorous physical activity was associated with a lower CKD prevalence.Leisure-time physical activity displacement was associated with a greater prevalence reduction than occupational physical activity in working population.In stratified analysis,a lower CKD prevalence related to substitution toward physical activity was found in long sleepers.More pronounced correlations were observed in long sleepers with diabetes than in those with prediabetes,and they benefited from other behavior substitutions toward a more active way.The U-shaped association between sleep duration and CKD prevalence implied the potential effects of insufficient and excessive sleep on the kidneys,in which the pernicious link with oversleep could be reversed by time reallocation to physical activity.The divergence in the predicted effect on CKD following time reallocation to behavior of different domains and intensities and in subpopulations with diverse metabolic statuses underlined the importance of optimizing sleeping patterns and adjusting integral behavioral composition.

关键词： sleep physical activity chronic kidney disease isotemporal substitution behavioral pattern

Association of Early-Life Famine Exposure with metabolic Dysfunction-Associated Fatty Liver Disease and Fibrosis in Adulthood

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Biomedical and Environmental Sciences 2022年第6期35卷 558-562页

作者： WEI Ran QI Hong Yan LIN Lin ZHU Yuan Yue ZHANG Yi ZHANG Jie WU Xue Yan HU Chun Yan WANG Shuang Yuan LIN Hong XU Yu XU Min BI Yu Fang WANG Wei Qing LU Jie Li NING guang CHEN Yu Hong Department of Endocrine and metabolic diseases Shanghai Institute of Endocrine and Metabolic DiseasesRuijin HospitalShanghai Jiaotong University School of MedicineShanghai 200025China Shanghai National clinical research center for metabolic diseases Key Laboratory for Endocrine and Metabolic Diseases of the National Health Commission of the PR ChinaShanghai National Center for Translational MedicineRuijin HospitalShanghai Jiaotong University School of MedicineShanghai 200025China

Nonalcoholic fatty liver disease(NAFLD)is known for its insidious onset and chronic nature,which have jeopardized the health and life of 29.2%of adults in China[1].In 2020,metabolic dysfunctionassociated fatty liver disease(MAFLD),a more accurate nomenclature to replace NAFLD,was put forward in an international consensus of experts involving 22 countries[2].In China,at least 300 million people will suffer from MAFLD by 2030,which will be a heavy burden on national health[3].

关键词： NAFLD Liver metabolic

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