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Development and validation of a novel non-invasive test for diagnosing nonalcoholic fatty liver disease in Chinese children

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World Journal of Pediatrics 2024年第4期20卷 413-421页

作者： Zhe-Wen Qin Qian-Nan Ren Hong-Xi Zhang Ya-Ru Liu Ke Huang Wei Wu Guan-Ping Dong Yan Ni Jun-Fen Fu Division of Endocrinology National Clinical Research Center for Child HealthNational Children’s Regional Medical CenterThe Children’s HospitalDepartment of EndocrinologyNational Clinical Research Center for Child HealthNational Children’s Regional Medical CenterThe Children’s HospitalZhejiang University School of MedicineHangzhou310052China

With the exploding prevalence of obesity,many children are at risk of developing nonalcoholic fatty liver *** anthropometric and laboratory parameters,our study aimed to develop a model to quantitatively evaluate liver fat content(LFC)in children with *** A well-characterized cohort of 181 children between 5 and 16 years of age were recruited to the study in the Endocrinology Department as the derivation *** external validation cohort comprised 77 *** assessment of liver fat content was performed using proton magnetic resonance *** and laboratory metrics were measured in all subjects.B-ultrasound examination was carried out in the external validation *** Kruskal-Wallis test,spearman bivariate correlation analyses,univariable linear regressions and multivariable linear regression were used to build the optimal predictive *** The model was based on indicators including alanine aminotransferase,homeostasis model assessment of insulin resistance,triglycerides,waist circumference and Tanner *** adjusted R^(2)of the model was 0.589,which presented high sensitivity and specificity both in internal[sensitivity of 0.824,specificity of 0.900,area under curve(AUC)of 0.900 with a 95%confidence interval:0.783–1.000]and external validation(sensitivity of 0.918 and specificity of 0.821,AUC of 0.901 with a 95%confidence interval:0.818–0.984).Conclusions Our model based on five clinical indicators was simple,non-invasive,and inexpensive;it had high sensitivity and specificity in predicting LFC in ***,it may be useful for identifying children with obesity who are at risk for developing nonalcoholic fatty liver disease.

关键词： Biochemical markers children Magnetic resonance spectroscopy Nonalcoholic fatty liver disease Pediatric obesity

X-box binding protein 1 caused an imbalance in pyroptosis and mitophagy in immature rats with di-(2-ethylhexyl)phthalate-induced testis toxicity

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Genes & Diseases 2024年第2期11卷 935-951页

作者： Yifan Hong Xiazhu Zhou Qi Li Jing Chen Yuexin Wei Chunlan Long Lianju shen Xiangqin Zheng Dinggang Li Xia Wang Chenjun Yu shengde Wu Guanghui Wei Department of Urology Children's Hospital of Chongqing Medical UniversityChongqing 400014China Pediatric Research Institute Children's Hospital of Chongqing Medical UniversityChongqing 400014China Chongqing Key Laboratory of children Urogenital Development and Tissue Engineering Children's Hospital of Chongqing Medical UniversityChongqing 400014China Chongqing Key Laboratory of Pediatrics Children's Hospital of Chongqing Medical UniversityChongqing 400014China Ministry of Education Key Laboratory of Child Development and Disorders Children's Hospital of Chongqing Medical UniversityChongqing 400014China National Clinical Research Center for Child Health and Disorders Children's Hospital of Chongqing Medical UniversityChongqing 400014China China International science and Technology Cooperation Base of Child Development and Critical Disorders Children's Hospital of Chongqing Medical UniversityChongqing 400014China

As a widely used plasticizer,di-(2-ethylhexyl)phthalate(DEHP)is known to induce significant testicular ***,the potential mechanism and effects of pubertal exposure to DEHP on testis development remain *** vivo,postnatal day(PND)21 male rats were gavaged with 0,250,and 500 mg/kg DEHP for ten *** to the seminiferous epithelium and disturbed spermatogenesis were observed after DEHP ***,oxidative stress-induced injury and pyroptosis were *** endoplasmic reticulum(ER)stress and mitophagy were involved in this *** phthalate(MEHP)was used as the biometabolite of DEHP in *** GC-1 and GC-2 cell lines were exposed to 0,100μM,200μM,and 400μM MEHP for 24 *** oxygen species(ROs)generation,oxidative stress damage,ER stress,mitophagy,and pyroptosis were significantly increased after MEHP *** ultrastructure of the ER and mitochondria was destroyed.X-box binding protein 1(XBP1)was observed to be activated and translocated into the *** generation was inhibited by *** levels of antioxidative stress,ER stress,mitophagy,and pyroptosis were decreased as *** the administration of the ER stress inhibitor 4-phenyl-butyric acid,both mitophagy and pyroptosis were ***-induced XBP1 down-regulation decreased the levels of mitophagy and *** equilibrium between pyroptosis and mitophagy was disturbed by XBP1 *** summary,our findings confirmed that DEHP induced a ROs-mediated imbalance in pyroptosis and mitophagy in immature rat testes via ***,XBP1 might be the key target in DEHP-related testis dysfunction.

关键词： DEHP Mitophagy Pyroptosis Testicular development XBP1

Enhancing Diagnosis and Treatment Adjustments for Travelers Infected with Burkholderia pseudomallei:A Case Report-Beijing Municipality,China,June 2023

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China CDC weekly 2024年第25期6卷 614-616页

作者： shijie Li Yushan He Yiyang Mao Jun Liu Wenmiao Xu suyun Qian Quan Wang Pediatric Intensive Care Unit Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Capital medical University BeijingChina

summary What is already known about this topic?Burkholderia pseudomallei(BP)infection leads to melioidosis,a tropical disease endemic to coastal provinces of southern *** in nonendemic areas,do not commonly consider this disease as a primary differential diagnosis for febrile *** is added by this report?This article discusses a case of melioidosis in northern *** patient,who had recently visited a southeast Asian region with high melioidosis prevalence,fell ill after exposure to contaminated *** disease progressed quickly and with *** are the implications for public health practice?Healthcare workers need to remain vigilant regarding travel-related diseases for accurate differential diagnosis and to provide timely and effective treatment,especially for patients with recent travel history or symptoms during travel.

关键词： diagnosis China travel

Efficacy of physiological seawater nasal irrigation for the treatment of children with sARs-CoV-2 Omicron BA.2 variant infection:a randomized controlled trial

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World Journal of Pediatrics 2024年第5期20卷 461-469页

作者： Ji-Lei Lin Fen Zhang Yan-Bo Li shu-Hua Yuan Jin-Hong Wu Jing Zhang Lei Zhang Yi He Jie Chen Yong Yin Department of Respiratory Medicine Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong University1678 Dongfang RoadPudong DistrictShanghaiChina University of British Columbia VancouverCanada Information Technology Department Shanghai Children’s Medical Center National Children’s Medical CenterSchool of MedicineShanghai Jiao Tong University1678 Dongfang RoadPudong DistrictShanghaiChina Department of Otorhinolaryngology Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong University1678 Dongfang RoadPudong DistrictShanghaiChina Department of Respiratory Medicine Sanya Women and Children’s Hospital Affiliated to Hainan Medical CollegeHainan Branch of Shanghai Children’s Medical CenterSanyaChina Department of Respiratory Medicine Linyi Maternal and Child Healthcare HospitalLinyiChina shanghai children’s medical Center Pediatric medical Complex(Pudong) ShanghaiChina Pediatric AI Clinical Application and Research Center Shanghai Children’s Medical CenterShanghaiChina

Background saline nasal irrigation is an effective therapy for relieving common cold *** study aimed to investigate and explore the efficacy of physiological seawater nasal irrigation(PsNI)on children with mild and asymptomatic infection with *** This randomized controlled trial was conducted in shanghai,China,and 403 children with mild and asymptomatic infection with Omicron were *** children were allocated into the PsNI group and the control *** primary outcome was the duration of viral shedding(DVs),and the secondary outcome was the change in clinical *** The median age of all participants was 5.59(6.26)years *** DVs was significantly shorter in the PsNI group[2.40(1.13)]than in the control group[3.09(2.14)](P=0.014).The multivariable Cox regression model also showed that patients in the PsNI group had an increased probability of shorter DVs compared with patients in the control group[hazard ratio(HR),1.27;95%confidence interval(CI),1.04–1.55;P=0.017].subgroup analysis suggested that the DVs of patients without full vaccination was significantly reduced in the PsNI *** proportions of runny nose and stuffy nose were apparently reduced in the first three days in the PsNI group or the control group,but there was no evidence showing that PsNI contributes to the benefit compared with the control *** PsNI can reduce the DVs of patients with mild and asymptomatic infection with sARs-CoV-2 Omicron BA.2 variant.

关键词： children Duration of viral shedding Omicron Physiological seawater nasal irrigation

Mechanism of Endogenous Peptide PDYBX1 and Precursor Protein YBX1 in Hirschsprung’s Disease

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Neuroscience Bulletin 2024年第6期40卷 695-706页

作者： Qiaochu sun Zhengke Zhi Chenglong Wang Chunxia Du Jie Tang Hongxing Li Weibing Tang Department of Pediatric surgery Children’s Hospital of Nanjing Medical UniversityNanjing210008China

Endogenous peptides,bioactive agents with a small molecular weight and outstanding absorbability,regulate various cellular processes and ***,their role in the occurrence of Hirschsprung’s disease(HsCR)remains ***,we found that the expression of an endogenous peptide derived from YBX1(termed PDYBX1 in this study)was upregulated in the aganglionic colonic tissue of HsCR patients,whereas its precursor protein YBX1 was *** shown by Transwell and cytoskeleton staining assays,silencing YBX1 inhibited the migration of enteric neural cells,and this effect was partially reversed after treatment with ***,immunoprecipitation and immunofluorescence revealed that ERK2 bound to YBX1 and *** of YBX1 blocked the ERK1/2 pathway,but upregulation of PDYBX1 counteracted this effect by binding to ERK2,thereby promoting cell migration and *** together,the endogenous peptide PDYBX1 may partially alleviate the inhibition of the ERK1/2 pathway caused by the downregulation of its precursor protein YBX1 to antagonize the impairment of enteric neural ***1 may be exploited to design a novel potential therapeutic agent for HsCR.

关键词： Hirschsprung’s disease YBX1 Endogenous peptide PDYBX1 Enteric neural cell

Machine Learning-Based Intelligent Auscultation Techniques in Congenital Heart Disease:Application and Development

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Congenital Heart Disease 2024年第2期19卷 219-231页

作者： Yang Wang Xun Yang Mingtang Ye Yuhang Zhao Runsen Chen Min Da Zhiqi Wang Xuming Mo Jirong Qi Department of Cardiothoracic surgery Children’s Hospital of Nanjing Medical UniversityNanjingChina

Congenital heart disease(CHD),the most prevalent congenital ailment,has seen advancements in the“dual indi-cator”screening *** facilitates the early-stage diagnosis and treatment of children with CHD,subse-quently enhancing their survival *** cardiac auscultation offers an objective reflection of cardiac abnormalities and function,its evaluation is significantly influenced by personal experience and external factors,rendering it susceptible to misdiagnosis and *** recent years,continuous progress in artificial intelli-gence(AI)has enabled the digital acquisition,storage,and analysis of heart sound signals,paving the way for intelligent CHD auscultation-assisted diagnostic *** there has been a surge in studies based on machine learning(ML)within CHD auscultation and diagnostic technology,most remain in the algorithmic research phase,relying on the implementation of specific datasets that still await verification in the clinical *** paper provides an overview of the current stage of AI-assisted cardiac sounds(Cs)auscultation technology,outlining the applications and limitations of AI auscultation technology in the CHD *** aim is to foster further development and refinement of AI auscultation technology for enhanced applications in CHD.

关键词： Congenital heart disease heart sound auscultation artificial intelligence machine learning

Ketogenic diet therapy in children with epilepsy caused by sLC2A1 mutations:a single-center single-arm retrospective study

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World Journal of Pediatrics 2024年第5期20卷 517-524页

作者： Ying-Yan Wang Yun-Qing Zhou Li-Juan Luo Cui-Jin Wang Nan shen Hao Li Ji-Wen Wang Department of Neurology Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina Department of Infectious Diseases Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina Clinical Research Ward Clinical Research CenterShanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina

Background This retrospective study assessed the efficacy and safety of ketogenic diet therapies in children with epilepsy caused by sLC2A1 genetic mutations and glucose transporter type 1 deficiency *** Pediatric patients with epilepsy symptoms admitted to our medical center between January 2017 and October 2021 were included if they presented with an sLC2A1 genetic mutation on whole-exome *** analyzed the patients’convulsions and treatment with antiepileptic *** patients were followed up at different time periods after ketogenic diet *** six patients with sLC2A1 mutations were included in this *** patients had seizures of different types and frequencies,and they took antiepileptic drugs to relieve their *** were then treated with a ketogenic diet for at least four *** analyzed epilepsy control rates at 1,2,3,6,and 12 months after ketogenic diet *** patients were seizure-free within a month of receiving the diet *** patients were followed up for six months,three were followed up for 12 months after the treatment,and there was no recurrence of epilepsy during this *** antiepileptic drug withdrawal,none of the patients experienced seizure relapse when receiving ketogenic diet treatment *** severe adverse events occurred during the *** Ketogenic diet therapy is very effective and safe for the treatment of epilepsy caused by sLC2A1 ***,patients with glucose transporter type 1 deficiency syndrome caused by sLC2A1 mutations should begin ketogenic diet treatment as soon as possible.

关键词： Epilepsy Glucose transporter type 1 Ketogenic diet sLC2A1

Activation of LONP1 by 84-B10 alleviates aristolochic acid nephropathy via re-establishing mitochondrial and peroxisomal homeostasis

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Chinese Journal of Natural Medicines 2024年第9期22卷 808-821页

作者： XU Xinyue ZHU Wenping MIAO Mengqiu BAI Mi FAN Jiaojiao NIU Yujia LI Yuting ZHANG Aihua JIA Zhanjun WU Mengqiu school of Medicine Southeast UniversityNanjing 210009China Department of Nephrology Children’s Hospital of Nanjing Medical UniversityNanjing 210008China Nanjing Key Laboratory of Pediatrics Children’s Hospital of Nanjing Medical UniversityNanjing 210008China Jiangsu Key Laboratory of Early Development and Chronic Diseases Prevention in children Nanjing Medical UniversityNanjing 210029China

Pharmaceutical formulations derived from Aristolochiaceae herbs, which contain aristolochic acids(AAs), are widely used for medicinal purposes. However, exposure to these plants and isolated AAs is linked to renal toxicity, known as AA nephropathy(AAN). Currently, the mechanisms underlying AAN are not fully understood, leading to unsatisfactory treatment strategies. In this study, we explored the protective role of 84-B10(5-[[2-(4-methoxyphenoxy)-5-(trifluoromethyl) phenyl] amino]-5-oxo-3-phenylpentanoic acid) against AAN. RNA-seq analysis revealed that the mitochondrion and peroxisome were the most affected cellular components following 84-B10 treatment in AAN mice. Consistently, 84-B10 treatment preserved mitochondrial ultrastructure, restored mitochondrial respiration, enhanced the expression of key transporters(carnitine palmitoyltransferase 2) and enzymes(acylCoenzyme A dehydrogenase, medium chain) involved in mitochondrial fatty acid β-oxidation, and reduced mitochondrial ROs generation in both aristolochic acid I(AAI)-challenged mice kidneys and cultured proximal tubular epithelial cells. Additionally, 84-B10 treatment increased the expression of key transporters(ATP binding cassette subfamily D) and rate-limiting enzymes(acyl-CoA oxidase 1) involved in peroxisomal fatty acid β-oxidation and restored peroxisomal redox balance. Knocking down LONP1 expression diminished the protective effects of 84-B10 against AAN, suggesting LONP1-dependent protection. In conclusion, our study provides evidence that AAN is associated with significant disturbances in both mitochondrial and peroxisomal functions. The LONP1 activator 84-B10 demonstrates therapeutic potential against AAN, likely by maintaining homeostasis in both mitochondria and peroxisomes.

关键词： Aristolochic acid nephropathy 84-B10 LONP1 Mitochondrion Peroxisome

Expert consensus on the diagnosis, treatment, and prevention of respiratory syncytial virus infections in children

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World Journal of Pediatrics 2024年第1期20卷 11-25页

作者： Xian-Li Zhang Xi Zhang Wang Hua Zheng-De Xie Han-Min Liu Hai-Lin Zhang Bi-Quan Chen Yuan Chen Xin sun Yi Xu sai-Nan shu shun-Ying Zhao Yun-Xiao shang Ling Cao Yan-Hui Jia Luo-Na Lin Jiong Li Chuang-Li Hao Xiao-Yan Dong Dao-Jiong Lin Hong-Mei Xu De-Yu Zhao Mei Zeng Zhi-Min Chen Li-su Huang Department of Infectious Disease Children's HospitalZhejiang University School of Medicine3333 Binsheng RoadBinjiang DistrictHangzhou 310052China Clinical Research Unit Xin Hua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghaiChina Beijing Pediatric Research Institute Beijing Children's HospitalCapital Medical UniversityBeijingChina Department of Pediatric Pulmonology West China Second University HospitalSichuan UniversityChengduChina Department of Pediatric Pulmonology the Second Affiliated Hospital&Yuying Children's HospitalWenzhou Medical UniversityWenzhouChina Department of Infectious Disease Anhui Provincial Children's HospitalHefeiChina Department of Pediatrics the Second Hospital of Hebei Medical UniversityShijiazhuangChina Department of Pediatrics Xijing Hospitalthe Fourth Military Medical UniversityXi'anChina Department of Infectious Disease Guangzhou Women and Children's Medicine CenterGuangzhou Medicine UniversityGuangzhouChina Department of Pediatrics Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhanChina Department of Respiratory Disease Beijing Children's HospitalCapital Medical UniversityBeijingChina Department of Pediatric Respiratory Shengjing Hospital of China Medical UniversityShenyangChina Respiratory Department Children's Hospital Affiliated to Capital Institute of PediatricsBeijingChina Department of Clinical Epidemiology Aarhus UniversityAarhusDenmark Department of Respirology Children's Hospital of Soochow UniversitySuzhouChina Department of Respiratory Children's Hospital of ShanghaiChildren's Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghaiChina Department of Infectious Disease Hainan Women and Children's Medical CenterHaikouChina Department of Infectious Disease Children's Hospital of Chongqing Medical UniversityChongqingChina Department of Respiratory Children's Hospital of Nanjing Medical UniversityNanjingChina Department of Infectious Di

Background Respiratory syncytial virus(RsV)is the leading global cause of respiratory infections and is responsible for about 3 million hospitalizations and more than 100,000 deaths annually in children younger than 5 years,representing a major global healthcare *** is a great unmet need for new agents and universal strategies to prevent RsV infections in early life.A multidisciplinary consensus development group comprising experts in epidemiology,infectious diseases,respiratory medicine,and methodology aims to develop the current consensus to address clinical issues of RsV infections in *** sources The evidence searches and reviews were conducted using electronic databases,including PubMed,Embase,Web of science,and the Cochrane Library,using variations in terms for"respiratory syncytial virus","RsV","lower respiratory tract infection","bronchiolitis","acute","viral pneumonia","neonatal","infant""children",and"pediatric".Results Evidence-based recommendations regarding diagnosis,treatment,and prevention were proposed with a high degree of *** supportive care remains the cornerstone for the management of RsV infections,new monoclonal antibodies,vaccines,drug therapies,and viral surveillance techniques are being rolled *** This consensus,based on international and national scientific evidence,reinforces the current recommendations and integrates the recent advances for optimal care and prevention of RsV *** improvements in the management of RsV infections will require generating the highest quality of evidence through rigorously designed studies that possess little bias and sufficient capacity to identify clinically meaningful end points.

关键词： Consensus prevention Respiratory syncytial virus Treatment