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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 disease.Using anthropometric and laboratory parameters,our study aimed to develop a model to quantitatively evaluate liver fat content(LFC)in children with obesity.Methods 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 cohort.The external validation cohort comprised 77 children.The assessment of liver fat content was performed using proton magnetic resonance spectroscopy.Anthropometry and laboratory metrics were measured in all subjects.B-ultrasound examination was carried out in the external validation cohort.The Kruskal-Wallis test,spearman bivariate correlation analyses,univariable linear regressions and multivariable linear regression were used to build the optimal predictive model.Results The model was based on indicators including alanine aminotransferase,homeostasis model assessment of insulin resistance,triglycerides,waist circumference and Tanner stage.The 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 children.Thus,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

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A Clinical Observation Report on Prenatal Management and Postnatal Treatment of Congenital Granular Cell Epulis

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Maternal-Fetal Medicine 2024年第2期6卷 102-105页

作者： Feng Qin Xiaochuan Xu Yong Yang Qiong Li Ting Huang Xiaoyan Chen Xiaolan Chen Yamin Liu Gongli Chen Department of Gynecology and Obstetrics Chongqing Health Center for Women and ChildrenWomen and Children's Hospital of Chongqing Medical UniversityChongqing 401147China Department of Pathology Chongqing Health Center for Women and ChildrenWomen and Children's Hospital of Chongqing Medical UniversityChongqing 401147China.

Introduction Congenital granular cell epulis(CGCE)is a rare,benign tumor that manifests on the mucosal surface of the alveolar ridge in newborns.It was initially described by Neumann in 1871 and has been referred to by various names such as congenital epulis,congenital epulis of the newborn,and congenital granular cell tumor.1 Documentation on larger tumors is limited and reported diameters vary.2 Diagnosis necessitates histological examination and immunohistochemical staining,typically revealing negative immunoreactivity for s-100 and cluster of differentiation 68(CD68).3,4 This article presents a case of CGCE with weakly positive s-100 and CD68 staining,alongside a relatively large size.Additionally,it conducts a literature review on diverse aspects,encompassing multidisciplinary prenatal diagnosis,antenatal management,choice of delivery mode during childbirth,and postnatal treatment,with the objective of augmenting clinicians’comprehension of the ailment.The study adhered to the guidelines of the Declaration of Helsinki and obtained informed consent from the patient and approval from the medical ethics committee of Chongqing health Center for women and children.

关键词： Congenital epulis Fetal Immunohistochemical Prenatal management Postnatal treatment

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Proteomic profiling of cerebrospinal fluid in pediatric myelin oligodendrocyte glycoprotein antibody-associated disease

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World Journal of Pediatrics 2024年第3期20卷 259-271页

作者： Yi-Long Wang Meng-Ying Zhu Zhe-Feng Yuan Xiao-Yan Ren Xiao-Tong Guo Yi Hua Lu Xu Cong-Ying Zhao Li-Hua Jiang Xin Zhang Guo-Xia sheng Pei-Fang Jiang Zheng-Yan Zhao Feng Gao Department of Neurology Children's HospitalZhejiang University School of MedicineHangzhou 310052China children's Hospital Zhejiang University School of MedicineHangzhou 310052China children's Hospital National Clinical Research Center for Child HealthZhejiang University School of MedicineHangzhou 310052China

Background Myelin oligodendrocyte glycoprotein(MOG)antibody-associated disease(MOGAD)is an autoimmune demy-elinating disorder of the central nervous system.Methods Extracted proteins from 34 cerebrospinal fluid(CsF)samples[patients with MOGAD(MOG group,n=12);healthy controls(HC group,n=12);patients with MOG seronegative and metagenomics next-generation sequencing-negative inflammatory neurological diseases(IND group,n=10)]were processed and subjected to label-free quantitative proteomics.supervised partial least squares-discriminant analysis(PLs-DA)and orthogonal PLs-DA(O-PLs-DA)models were also performed based on proteomics data.Functional analysis of differentially expressed proteins(DEPs)was performed using Gene Ontology,InterPro,and Kyoto Encyclopedia Genes and Genomes.An enzyme-linked immunosorbent assay was used to determine the complement levels in serum from patients with MOGAD.Results Four hundred and twenty-nine DEPs(149 upregulated and 280 downregulated proteins)were identified in the MOG group compared to the HC group according to the P value and fold change(FC).Using the O-PLs-DA model,872 differentially abundant proteins were identified with variable importance projection(VIP)scores>1.Five proteins(gamma-glutamyl hydrolase,cathepsin F,interalpha-trypsin inhibitor heavy chain 5,latent transforming growth factor beta-binding protein 4 and leukocyte-associated immunoglobulin-like receptor 1)overlapping between the top 30 DEPs with top-ranked P value and FC and top 30 proteins in PLs-DA VIP lists were acquired.Functional analysis revealed that the dysregulated proteins in the MOG group were primarily involved in complement and coagulation cascades,cell adhesion,axon guidance,and glycosphingolipid biosynthesis compared to the HC group.Conclusion The proteomic alterations in CsF samples from children with MOGAD identified in the current study might provide opportunities for developing novel biomarker candidates.

关键词： Acute disseminated encephalomyelitis Cerebrospinal fluid Complement cascades Myelin oligodendrocyte glycoprotein-associated disease Proteomics

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Ultrasound blood flow characteristics changes in fetal umbilical artery thrombosis:A retrospective analysis

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World Journal of Clinical Cases 2024年第2期12卷 240-248页

作者： si-Jie Hong Li-Wei Hong Xiao-Qin He Xiao-Hong Zhong Department of Ultrasound Women and Children's HospitalSchool of MedicineXiamen UniversityXiamen 361000Fujian ProvinceChina Ministry of science and Education Women and Children's HospitalSchool of MedicineXiamen UniversityXiamen 361000Fujian ProvinceChina

BACKGROUND Umbilical artery thrombosis(UAT)is extremely uncommon and leads to adverse perinatal outcomes.Hypercoagulation of blood in pregnant women is suspected to be an important risk for UAT.Ultrasound is an effective way to detect thrombosis.The mother can monitor her own fetal health using ultrasound,which enables her to take preventative action in case of emergency.AIM To investigate ultrasonic blood signal after UAT in the umbilical artery,and evaluate the relationship between hypercoagulability and UAT.METHODs We described a case of a newly formed UAT with markedly altered ultrasonic indices of umbilical artery blood flow,and retrospectively studied it with 18 UAT patients confirmed by histopathology from October 2019 and March 2023 in Xiamen women and children's Hospital.Patients’information was collected from medical archives,including maternal clinical data,neonatal outcomes,pathological findings and ultrasonic indices of umbilical artery blood flow,such as systolic-diastolic duration ratio(s/D),resistance index(RI),pulsatility index(PI)and peak systolic velocity(PsV).Ultrasound and coagulation indices were analyzed with matched samples t-test and Wilcoxon rank sum test using the statistical packages in R(version 4.2.1)including car(version 3.1-0)and stats(version 4.2.1),and visualized by ggplot2 package(version 3.3.6).REsULTs A patient with normal findings in second and third-trimester routine ultrasound scan developed UAT with severe changes in ultrasonic indices of umbilical artery blood flow(within 2.5th of reference ranges)in a short period of time.statistical analysis of umbilical artery blood flow ultrasound indices for 19 patients with UAT showed that the decrease in s/D,RI,and PI and increase of PsV during the disease process was greater than that of non-UAT.All 18 patients delivered in our hospital showed characteristic manifestations of UAT on histological examination after delivery,most of which(16/18)showed umbilical cord abnormalities,with 15 umbilical cord torsion and 1 pseudoknot.Coagul

关键词： Umbilical artery thrombosis Obstetric ultrasonography Peak systolic velocity Hypercoagulation Umbilical cord abnormalities Fetus

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Trends in Respiratory Infectious Pathogens in children Under the Age of 14—Xiamen City,Fujian Province,China,2017-2023

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China CDC weekly 2024年第8期6卷 143-147页

作者： Jiali Cao Jie Pan Xiaoqing Yang Jumei Liu Min Zhu Zeyu Zhao Ling Chen Tianmu Chen Huiming Ye Department of Laboratory Medicine Fujian Key Clinical Specialty of Laboratory MedicineWomen and Children’s HospitalSchool of MedicineXiamen UniversityXiamen CityFujian ProvinceChina state Key Laboratory of Vaccines for Infectious Diseases Xiang An Biomedicine LaboratorySchool of Public HealthXiamen UniversityXiamen CityFujian ProvinceChina Department of Pediatrics Women and Children's HospitalSchool of MedicineXiamen UniversityXiamen CityFujian ProvinceChina

summary What is already known about this topic?Respiratory infections pose a significant burden on public health.Despite recent outbreaks occurring in various locations,there is limited information available on the pr... 详细信息

关键词： Xiamen spite Fujian

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severe pediatric COVID‑19:a review from the clinical and immunopathophysiological perspectives

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World Journal of Pediatrics 2024年第4期20卷 307-324页

作者： Yi‑Kan sun Can Wang Pei‑Quan Lin Lei Hu Jing Ye Zhi‑Gang Gao Ru Lin Hao‑Min Li Qiang shu Li‑su Huang Lin‑Hua Tan children's Hospital Zhejiang University School f MedicineHangzhou 310052China The second Affiliated Hospital Zhejiang University School of MedicineHangzhou 310030China surgical Intensive Care Unit Children’s HospitalZhejiang University School of MedicineHangzhou 310052China Department of General surgery Children’s HospitalZhejiang University School of MedicineHangzhou 310052China Department of Cardiopulmonary and Extracorporeal Life support Children’s HospitalZhejiang University School of MedicineHangzhou 310052China Clinical Data Center Children’s HospitalZhejiang University School of MedicineHangzhou 310052China Department of Cardiac surgery Children’s HospitalZhejiang University School of MedicineHangzhou 310052China National Clinical Research Center for Child health Children’s HospitalZhejiang University School of MedicineHangzhou 310052China Department of Infectious Diseases Children’s HospitalZhejiang University School of MedicineHangzhou 310052China

Background Coronavirus disease 2019(COVID-19)tends to have mild presentations in children.However,severe and critical cases do arise in the pediatric population with debilitating systemic impacts and can be fatal at times,meriting further attention from clinicians.Meanwhile,the intricate interactions between the pathogen virulence factors and host defense mechanisms are believed to play indispensable roles in severe COVID-19 pathophysiology but remain incompletely understood.Data sources A comprehensive literature review was conducted for pertinent publications by reviewers independently using the PubMed,Embase,and Wanfang databases.searched keywords included“COVID-19 in children”,“severe pediatric COVID-19”,and“critical illness in children with COVID-19”.Results Risks of developing severe COVID-19 in children escalate with increasing numbers of co-morbidities and an unvaccinated status.Acute respiratory distress stress and necrotizing pneumonia are prominent pulmonary manifestations,while various forms of cardiovascular and neurological involvement may also be seen.Multiple immunological processes are implicated in the host response to COVID-19 including the type I interferon and inflammasome pathways,whose dysregulation in severe and critical diseases translates into adverse clinical manifestations.Multisystem inflammatory syndrome in children(MIs-C),a potentially life-threatening immune-mediated condition chronologically associated with COVID-19 exposure,denotes another scientific and clinical conundrum that exemplifies the complexity of pediatric immunity.Despite the considerable dissimilarities between the pediatric and adult immune systems,clinical trials dedicated to children are lacking and current management recommendations are largely adapted from adult guidelines.Conclusions severe pediatric COVID-19 can affect multiple organ systems.The dysregulated immune pathways in severe COVID-19 shape the disease course,epitomize the vast functional diversity of the pediatric immune system and highlight the immun

关键词： Immunopathophysiology MIs-C Pediatric critical care severe pediatric COVID-19

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NLRP3 activation in macrophages promotes acute intestinal injury in neonatal necrotizing enterocolitis

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World Journal of Pediatrics 2024年第2期20卷 153-164页

作者： Bo shi Cheng-Jie Lyu Zhen-Kai Le Hao-sen Ji Yi Xiao Yuan-Yuan Zhang shou-Jiang Huang Lin-Jun Yu Qiang shu Jin-Fa Tou Deng-Ming Lai Department of Neonatal surgery Children’s HospitalZhejiang University School of MedicineNational Clinical Research Center for Child HealthHangzhou 310052China Department of Pulmonology Children’s HospitalZhejiang University School of MedicineNational Clinical Research Center for Child HealthHangzhou 310052China Department of Thoracic and Cardiovascular surgery Children’s HospitalZhejiang University School of MedicineNational Clinical Research Center for Child HealthHangzhou 310052China

Background Macrophages are involved in various immune inflammatory disease conditions.This study aimed to investigate the role and mechanism of macrophages in regulating acute intestinal injury in neonatal necrotizing enterocolitis(NEC).Methods CD68,nucleotide-binding oligomerization domain,leucine-rich repeat,and pyrin domain-containing 3(NLRP3),cysteine aspartate-specific protease-1(caspase-1),and interleukin-1β(IL-1β)in paraffin sections of intestinal tissues from NEC and control patients were detected with immunohistochemistry,immunofluorescence,and western blot.Hypertonic pet milk,hypoxia and cold stimulation were used to establish a mouse(wild type and Nlrp3^(-/-))model of NEC.The mouse macrophage(RAW 264.7)and rat intestinal epithelial cell-6 lines were also cultured followed by various treatments.Macrophages,intestinal epithelial cell injuries,and IL-1β release were determined.Results Compared to the gut“healthy”patients,the intestinal lamina propria of NEC patients had high macrophage infiltration and high NLRP3,caspase-1,and IL-1β levels.Furthermore,in vivo,the survival rate of Nlrp3^(-/-)NEC mice was dramatically improved,the proportion of intestinal macrophages was reduced,and intestinal injury was decreased compared to those of wild-type NEC mice.NLRP3,caspase-1,and IL-1β derived from macrophages or supernatant from cocultures of macrophages and intestinal epithelial cells also caused intestinal epithelial cell injuries.Conclusions Macrophage activation may be essential for NEC development.NLRP3/caspase-1/IL-1β cellular signals derived from macrophages may be the underlying mechanism of NEC development,and all these may be therapeutic targets for developing treatments for NEC.

关键词： Caspase-1 Interleukin-1β Macrophage Necrotizing enterocolitis NLRP3

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Coinfection of sARs-CoV-2 Omicron variant and other respiratory pathogens in children

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World Journal of Pediatrics 2024年第1期20卷 92-96页

作者： Wei Li Bing-Han Wang Bao-Hai Chen Yi sun Lin Li Wen-Qing Xiang Ahmed Faisal Ali Lin-Xuan su Hai-Yan Mao Hong-Qiang shen Qiang shu Department of Clinical Laboratory Children's HospitalNational Clinical Research Center for Child HealthZhejiang University School of MedicineHangzhouChina school of Public health Zhejiang University School of MedicineHangzhouChina Department of Information Center Children's HospitalZhejiang University School of MedicineHangzhouChina Zhejiang Provincial Center for Disease Control and Prevention HangzhouChina Department of Thoracic&Cardiovascular surgery Children's HospitalNational Clinical Research Center for Child HealthZhejiang University School of MedicineHangzhouChina

since the outbreak of coronavirus disease 2019(COVID-19),the entire world has been affected by the epidemic[1,2].On November 9,2021,a variant of the novel coronavirus B.1.1.529 was detected for the first time from case samples in south Africa.The Omicron strain spread and mutated significantly fast.This variant has the characteristics of escape immunity,which can induce relatively extreme disease and reduce the neutralization of antibodies in vaccinators,and is more likely to be reinfected[3,4].The clinical presentation of severe acute respiratory syndrome coronavirus 2(sARsCoV-2)in children has mild symptoms and a lower risk of hospitalization and death.

关键词： respiratory immunity acute

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Inadvertent Laparoelytrotomy During the second-stage Cesarean section:Relooking the Lost Art and Proposing surgical Management and Prevention strategies

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Maternal-Fetal Medicine 2024年第1期6卷 9-12页

作者： Krystal Koh shahul Hameed Mohamed sira Department of Obstetrics and Gynecology KK Women’s and Children’s HospitalSingapore 229899Singapore

Introduction Laparoelytrotomy,or anterior vaginotomy,describes a transverse vaginal incision.It has been described since the 19th century1–3 as a method of delivering the fetus during a second stage cesarean section(Cs).4,5 More recently,reports have been published on inadvertent laparoelytrotomy during emergency Cs at full cervical dilatation,due to the anatomical changes that occur with prolonged duration of obstructed labor.

关键词： Cesarean section Laparoelytrotomy Labor

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Mitochondria replacement from transplanted amniotic fluid stem cells:a promising therapy for non-neuronal defects in spinal muscular atrophy

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Neural Regeneration Research 2024年第5期19卷 971-972页

作者： Michela Pozzobon Camilla Bean women’s and children’s health Department University of Padova Foundation Institute of Pediatric Research Cittàdella speranza PadovaItaly Department of Medicine University of UdineUdineItaly

spinal muscular atrophy(sMA)is a genetic disorder that primarily affects infants and leads to muscle weakness,atrophy,and paralysis.The main cause is the homozygous mutation or deletion of the sMN1 gene,resulting in inadequate levels of the survival motor neuron(sMN)protein.Approved treatments focus on restoring sMN levels through various approaches,but there is a need for“sMN-independent”therapies that target other pathological processes.skeletal muscle is closely involved in sMA pathology,with impaired muscle function observed before motor neuron degeneration.studies have revealed that sMN loss leads to skeletal muscle mitochondrial structural abnormalities,impaired respiration,and accumulation of reactive oxygen species.

关键词： impaired atrophy muscular

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