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Diagnostic significance of serum levels of serum amyloid A,procalcitonin,and high-mobility group box 1 in identifying necrotising enterocolitis in newborns

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World Journal of Gastrointestinal Surgery 2024年第7期16卷 2003-2011页

作者： Li-Ming Guo Zhi-Hui Jiang Hong-Zhen Liu Lei Zhang department of General Surgery Qingdao Women and Children’s HospitalQingdao 266000Shandong ProvinceChina department of pediatric Surgery Children's Hospital Affiliated to Shandong UniversityJinan 250022Shandong ProvinceChina department of pediatric Surgery Jinan Children's HospitalJinan 250022Shandong ProvinceChina department of pediatric Surgery Qilu Hospital of Shandong University(Qingdao)Qingdao 260010Shandong ProvinceChina

BACKGROUND Necrotising enterocolitis(NEC)is a critical gastrointestinal emergency affecting premature and low-birth-weight *** amyloid A(SAA),procalcitonin(PCT),and high-mobility group box 1(HMGB1)have emerged as potential biomarkers for NEC due to their roles in inflammatory response,tissue damage,and immune *** To evaluate the diagnostic value of SAA,PCT,and HMGB1 in the context of NEC in *** The study retrospectively analysed the clinical data of 48 newborns diagnosed with NEC and 50 healthy newborns admitted to the ***,radiological,and laboratory findings,including serum SAA,PCT,and HMGB1 Levels,were collected,and specific detection methods were *** diagnostic value of the biomarkers was evaluated through statistical analysis,which was performed using chi-square test,t-test,correlation analysis,and receiver operating characteristic(ROC)*** The study demonstrated significantly elevated levels of serum SAA,PCT,and HMGB1 Levels in newborns diagnosed with NEC compared with healthy *** correlation analysis indicated strong positive correlations among serum SAA,PCT,and HMGB1 Levels and the presence of *** analysis revealed promising sensitivity and specificity for serum SAA,PCT,and HMGB1 Levels as potential diagnostic *** combined model of the three biomarkers demonstrating an extremely high area under the curve(0.908).CONCLUSION The diagnostic value of serum SAA,PCT,and HMGB1 Levels in NEC was *** biomarkers potentially improve the early detection,risk stratification,and clinical management of critical *** findings suggest that these biomarkers may aid in timely intervention and the enhancement of outcomes for neonates affected by NEC.

关键词： Serum amyloid A Procalcitonin High-mobility group box 1 Necrotising enterocolitis in newborns Serum biomarkers

Pulmonary artery diameter ratio as a prognostic indicator of congenital diaphragmatic hernia

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World Journal of pediatric Surgery 2024年第1期7卷 20-25页

作者： Yuichiro Miyake Hiroyuki Koga Shuko Nojiri Shunsuke Yamada Takanori Ochi Go Miyano Geoffrey J Lane Atsuyuki Yamataka Tadaharu Okazaki department of pediatric General and Urogenital Surgery Juntendo University School of MedicineBunkyo-kuTokyoJapan department of Medical Technology Innovation Center Juntendo UniversityBunkyo-ku TokyoJapan department of pediatric Surgery Juntendo University Urayasu HospitalUrayasuChibaJapan

Background Following on from an earlier study published in 2008 about left pulmonary artery(LPA)flow measured on serial echocardiography being strongly prognostic in left-sided congenital diaphragmatic hernia(CDH)and the ratio of LPA to right pulmonary artery(RPA)diameters being a simple and reliable indicator for commencing nitric oxide(NO)therapy,the ratio of LPA:RPA diameters(PA ratio orPAR)was hypothesized to possibly reflect cardiopulmonary stresses accompanying CDH *** Subjects with isolated left-sided CDH treated between 2007 and 2020 at a single pediatric surgical center were recruited and classified according to *** obtained retrospectively for subject demographics,clinical course,LPA/RPA diameters,and PAR were compared between survivors and *** value of PAR for optimizing the prognostic value of PA diameter data in CDH were analyzed with receiver operating characteristic(ROC)curve *** Of 65 subjects,there were 54 survivors(82.3%)and 11 non-survivors(17.7%);7 of 11 non-survivors died before surgical repair could be *** PAR for survivors(0.851±0.152)was significantly higher than for non-survivors(0.672±0.108)(p=0.0003).Mean PAR for non-survivors was not affected by surgical *** of survivors were:LPA≥2 mm(n=52 of 54;mean PAR=0.866±0.146)and RPA≥3mm(n=46 of 54;mean PAR=0.857±0.152).Non-survivors with similar LPA and RPA diameters to survivors had significantly lower mean *** curve cut-off for PAR was *** with high PAR(≥0.762)required high-frequency oscillatory ventilation/NO less than subjects with low PAR(<0.762)(p=0.0244 and p=0.0485,respectively)and subjects with high PAR stabilized significantly earlier than subjects with low PAR(1.71±0.68 days vs 3.20±0.87 days)(p<0.0001).Conclusions PAR would appear to be strongly correlated with clinical outcome in CDH and be useful for planning management of cardiopulmonary instability in CDH.

关键词： hernia congenital diameters

Lin28 as a therapeutic target for central nervous system regeneration and repair

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Neural Regeneration Research 2024年第2期19卷 397-398页

作者： Shuo Wang Shuxin Li Shriners Hospitals pediatric Research Center Department of Neural SciencesLewis Katz School of Medicine at Temple UniversityPhiladelphiaPAUSA

Axon disconnection in the central nervous system(CNS) usually causes signal transduction failure and severe functional deficits in patients with neurological disorders. Currently, there is no cure for patients with CNS axon injury and they usually suffer from life-long neurological defects(e.g., paralysis, loss of sensory function, and autonomic dysfunction) and life-threatening complications(e.g., autonomic dysreflexia).

关键词： paralysis therapeutic system

Jaffe-Campanacci syndrome resulted in amputation: A case report

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World Journal of Clinical Cases 2024年第10期12卷 1785-1792页

作者： Jun Jiang Min Liu department of pediatric Surgery West China Hospital of Sichuan UniversityChengdu 610041Sichuan ProvinceChina

BACKGROUND Jaffe-Campanacci syndrome(JCS)is a very rare *** treatment of JCS is more conservative,and most authors recommend that no surgery should be done in asymptomatic *** conventional concept holds that the natural course of non-ossifying fibromas(NOFs)grows with the development of bones,and the osteolytic region gradually stops expanding and self-healing through bone ossifying around the lesion and ossification within the *** in this case,the bone lesions were potentially biologically aggressive,which led to severe limb deformities and *** SUMMARY We present the case of a 5-year-old girl with JCS presenting with not only NOF sand café-au-lait macules,but also showed features not mentioned before,severe limb pain,and at last resulted in *** was admitted to our hospital after presenting with claudication and mild pain over her right thigh,which worsened when stretching or being *** examination revealed multiple café-au-lait macules on the neck,arm,axilla,and torso,including the nipples and *** revealed multiple lytic lesions in the proximal part of the right humerus,distal part of the right clavicle,proximal and distal parts of the right femur,and proximal parts of the right tibia and *** and biopsy were performed on the distal part of the right *** the age of 7,the girl was re-admitted to our hospital for a pathological fracture in the middle in the right femur and underwent Intralesional excision,internal fixation,bone grafting,and spica *** the age of 10,the girl came to our hospital again for severe pain of the right *** from the middle level of the right femur was *** present the case of a 5-year-old girl with JCS presenting with not only NOFs and café-au-lait macules,but also showed features not mentioned before,severe limb pain,and at last resulted in *** was admitted to our hospital after presenting with claudication and mild pain o

关键词： Jaffe-Campanacci syndrome Multiple non-ossifying fibromas Café-au-lait macule Amputation Case report

Prognosis and outcome of intrauterine treatment of fetuses with critical congenital heart disease

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Chinese Medical Journal 2024年第12期137卷 1431-1436页

作者： Liqing Zhao Lei Wang Hongping Xia Yurong Wu Xianting Jiao Hong Zhu Sun Chen Kun Sun department of pediatric Cardiology Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China department of Diagnosis and Treatment Center for in utero pediatric Disease Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China department of Neonatology Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China

Background:Intrauterine valvuloplasty is an innovative therapy,which promotes ventricular growth and function in some congenital heart diseases(CHDs).The technique remains challenging and can only be performed in a few *** study aimed to assess the feasibility and mid-term outcomes of fetal cardiac intervention(FCI)in fetuses with critical CHD in an experienced tertiary ***:Five fetal aortic valvuloplasty(FAV)or fetal pulmonary valvuloplasty(FPV)procedures were performed in our fetal heart center between August 2018 and May *** success was defined as crossing the aortic or pulmonary valve and balloon inflation,followed by evidence of increased blood flow across the valve and/or new ***-up clinical records and echocardiography were obtained during the prenatal and postnatal ***:Five fetuses received FAV or FPV,including critical aortic stenosis(n=2)and pulmonary atresia with intact ventricular septum(n=3).The mean maternal age was 33.0±2.6 *** median gestational age(GA)at diagnosis was 24 weeks(range,22-26 weeks).The median GA at intervention was 29 weeks(range,28-32 weeks).All five cases underwent successful or partially successful *** patient had pulmonary valve perforation without balloon *** procedure-related deaths or significant complications ***,one neonatal death occurred due to heart and renal *** median follow-up period was 29.5 months(range,8.0-48.0 months).The four surviving patients had achieved biventricular circulation,exhibited improved valve,and ventricular development at the last follow-up ***:Intrauterine FCI could be performed safely with good prognosis in critical CHD.

关键词： Fetal cardiac intervention Valvuloplasty Pulmonary atresia Critical aortic stenosis Congenital heart disease

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

A novel structural variant of RET causes familial Hirschsprung's disease via nonsense-mediated mRNA decay

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Genes & Diseases 2024年第1期11卷 7-10页

作者： Qi Wang Siyu Pu Bo Xiang Jing Chen department of pediatric Surgery and Laboratory of pediatric Surgery Rare Diseases CenterInstitute of Immunology and InflammationFrontiers Science Center for Disease-Related Molecular NetworkWest China HospitalSichuan UniversityChengduSichuan 610041China

Hirschsprung's disease(HsCR)is the most common type of developmental gastrointestinal malformation causing intestinal obstruction in children,with an incidence of 1/5000 in live births.1 it is characterized by the absence of intrinsic ganglion cells in the myenteric and submucosal plexuses of the distal gastrointestinal tract,leading to the dysregulation of smooth muscle contraction/relaxation and resulting in intestinal *** on the length of the aganglionic intestine,HSCR can be categorized into short-segmental HSCR(S-HSCR),which affects up to the sigmoid colon from the anus,and long-segmental HSCR(LHSCR),which affects beyond the sigmoid colon.1 In some rare conditions,aganglionic lesions can affect the full length of the colon or even the whole intestine.

关键词： intestine colon relaxation

Nucleotide excision repair gene polymorphisms and hepatoblastoma susceptibility in Eastern Chinese children:A five-center case-control study

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Chinese Journal of Cancer Research 2024年第3期36卷 298-305页

作者： Huimin Yin Xianqiang Wang Shouhua Zhang Shaohua He Wenli Zhang Hongting Lu Yizhen Wang Jing He Chunlei Zhou department of pediatric Surgery Guangzhou Institute of PediatricsGuangdong Provincial Key Laboratory of Research in Structural Birth Defect DiseaseGuangzhou Women and Children’s Medical CenterGuangzhou Medical UniversityGuangzhou 510623China department of pediatric Surgery Senior Department of Pediatricsthe Seventh Center of Chinese People’s Liberation Army(PLA)General HospitalBeijing 100000China department of General Surgery Jiangxi Provincial Children’s HospitalNanchang 330006China department of pediatric Surgery Shengli Clinical Medical College of Fujian Medical UniversityFuzhou 350001China department of pediatric Surgery Qingdao Women and Children’s HospitalQingdao 266000China department of Pathology Anhui Provincial Children’s HospitalHefei 230051China department of Pathology Children’s Hospital of Nanjing Medical UniversityNanjing 210008China

Objective:Nucleotide excision repair(NER)plays a vital role in maintaining genome stability,and the effect of NER gene polymorphisms on hepatoblastoma susceptibility is still under *** study aimed to evaluate the relationship between NER gene polymorphisms and the risk of hepatoblastoma in Eastern Chinese Han ***:In this five-center case-control study,we enrolled 966 subjects from East China(193 hepatoblastoma patients and 773 healthy controls).The TaqMan method was used to genotype 19 single nucleotide polymorphisms(SNPs)in NER pathway genes,including ERCC1,XPA,XPC,XPD,XPF,and ***,multivariate logistic regression analysis was performed,and odds ratios(ORs)and 95%confidence intervals(95%CIs)were utilized to assess the strength of ***:Three SNPs were related to hepatoblastoma *** rs2229090 and XPD rs3810366 significantly contributed to hepatoblastoma risk according to the dominant model(adjusted OR=1.49,95%CI=1.07−2.08,P=0.019;adjusted OR=1.66,95%CI=1.12−2.45,P=0.012,respectively).However,XPD rs238406 conferred a significantly decreased risk of hepatoblastoma under the dominant model(adjusted OR=0.68,95%CI=0.49−0.95;P=0.024).Stratified analysis demonstrated that these significant associations were more prominent in certain ***,there was evidence of functional implications of these significant SNPs suggested by online expression quantitative trait loci(eQTLs)and splicing quantitative trait loci(sQTLs)***:In summary,NER pathway gene polymorphisms(XPC rs2229090,XPD rs3810366,and XPD rs238406)are significantly associated with hepatoblastoma risk,and further research is required to verify these findings.

关键词： Nucleotide excision repair polymorphisms hepatoblastoma susceptibility

Characteristics and Risk Factors for pediatric Sepsis

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Current Medical Science 2024年第3期44卷 648-656页

作者： Yong-bing ZHU Tong-lin LIU Qi DAI Shu-fan LIU Peng XIONG Hao HUANG Yi YUAN Tian-nan ZHANG Yu CHEN department of pediatric Intensive Care Unit Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan430030China

Objective Sepsis is considered a major cause of health loss in children and had high mortality and ***,there is no reliable model for predicting the prognosis of pediatric patients with *** study aimed to analyze the clinical characteristics of sepsis in children and assess the risk factors associated with poor prognosis in pediatric sepsis patients to identify timely interventions and improve their *** This study analyzed the clinical indicators and laboratory results of septic patients hospitalized in the pediatric Intensive Care Unit of Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology,China,from January 1,2019,to December 31,*** factors for sepsis were identified by logistic regression *** A total of 355 children with sepsis were enrolled,with 333 children(93.8%)in the good prognosis group,and 22 children(6.2%)in the poor prognosis *** them,there were 255 patients(71.8%)in the sepsis group,and 100 patients(28.2%)in the severe sepsis *** length of hospital stay in the poor prognosis group was longer than that in the good prognosis group(P0.05),and the platelet(PLT),albumin(ALB),and hemoglobin(Hb)levels were lower in the poor prognosis group(P<0.01).The IL-8 levels in the severe sepsis group were higher than those in the sepsis group(P<0.05).Multiple logistic regression analysis suggested that lower Hb levels,ALB levels,peak PLT counts,and higher IL-1βlevels were independent risk factors for poor prognosis in children with *** Lower Hb,ALB,and PLT counts and elevated IL-1βare independent risk factors for poor prognosis in children with sepsis.

关键词： pediatric sepsis inflammation severity prognosis risk factors