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Congenital Heart Disease 2024年第2期19卷 185-195页

作者： Camden L.Hebson Kyle Bliton Amr Y.Hammouda Kaitlyn Barr W.Hampton Gray Mohini Gunnett Waldemar F.Carlo department of pediatrics Division of Pediatric CardiologyUniversity of Alabama BirminghamBirmingham35233USA department of pediatrics Division of Pediatric PulmonologyUniversity of Alabama BirminghamBirmingham35233USA department of pediatrics University of Alabama BirminghamBirmingham35233USA department of surgery Division of Cardiothoracic SurgeryUniversity of Alabama BirminghamBirmingham35233USA

D-transposition of the great arteries(d-TGA)is surgically repaired with the arterial switch operation(ASO)with excellent results,however short and long-term morbidities still develop including neurocognitive *** significant central sleep apnea is uncommon in non-premature infants,but when present indicates immature autonomic control of respiration likely due to a neurologic *** report the unanticipatedfinding of central sleep apnea in four-term neonates with d-TGA after uncomplicated ASO,with the short-term complication of delayed hospital discharge and long-term concerns regarding this early marker of brain immaturity and its hindrance to normal *** this report,we will review each patient’s clinical course and then examine the literature on pediatric central sleep apnea,neurodevelopmental outcomes after ASO,and the important overlap of these entities in the care of patients going forward.

关键词： Transposition congenital heart disease sleep apnea polysomnography

Hepatic recompensation according to the Baveno VII criteria via a transjugular intrahepatic portosystemic shunt: Is this true?

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World Journal of Gastrointestinal surgery 2024年第8期16卷 2742-2744页

作者： Jin-Shan Zhang department of General surgery Capital Institute of PediatricsBeijing 100020China

Hepatic recompensation is firstly described in the Baveno VII criteria,which requires the fulfillment of strict ***,a primary cause of cirrhosis must be addressed,suppressed,or ***,complications of liver cirrhosis,inclu-ding ascites,encephalopathy,and variceal hemorrhage,must disappear without any ***,liver function indicators must be ***,without addressing/suppressing/curing cirrhosis and improvement in liver syn-thetic function,complications,including ascites and variceal hemorrhage can be improved by a transjugular intrahepatic portosystemic shunt(TIPS),which is not evidence of hepatic ***,on the basis of the definition of hepatic recompensation,TIPS does not achieve hepatic recompensation.

关键词： Hepatic recompensation Transjugular intrahepatic portosystemic shunt Liver function Cirrhosis

Fever assessment in children under five: Are we following the guidelines?

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World Journal of Clinical pediatrics 2024年第1期13卷 62-72页

作者： Hasan M Isa Ahmed J Isa Murtadha A Alnasheet Mahmood M Mansoor department of pediatrics Salmaniya Medical ComplexManama 26671Bahrain department of pediatrics Arabian Gulf UniversityManama 26671Bahrain department of General surgery Salmaniya Medical ComplexManama 26671Bahrain

BACKGROUND Fever is a common cause of medical consultation and hospital admission,particularly among ***,the United Kingdom’s National Institute for Health and Care Excellence(NICE)updated its guidelines for assessing fever in children under five years of *** efficient assessment and management of children with fever are crucial for improving patient *** To evaluate fever assessment in hospitalized children and to assess its adherence with the NICE Fever in under 5s *** We conducted a retrospective cohort review of the electronic medical records of children under five years of age at the department of pediatrics,Salmaniya Medical Complex,Bahrain,between June and July *** data,vital signs during the first 48 h of admission,route of temperature measurement,and indications for admission were *** was defined according to the NICE *** children were divided into five groups according to their age(0-3 months,>3-6 months,>6-12 months,>12-36 months,and>36-60 months).Patients with and without fever were compared in terms of demography,indication for admission,route of temperature measurement,and other vital *** with the NICE Fever in the under 5s guideline was *** compliance was defined as>95%,partial compliance as 70%-95%,and minimal compliance as≤69%.Pearson’sχ^(2),Student’s t test,the Mann-Whitney U test,and Spearman’s correlation coefficient(rs)were used for *** Of the 136 patients reviewed,80(58.8%)were *** median age at admission was 14.2[interquartile range(IQR):1.7-44.4]months,with the most common age group being 36-60 ***-six(26.4%)patients had fever,and 100(73.6%)were *** commonest age group for febrile patients(>12-36 months)was older than the commonest age group for afebrile patients(0-3 months)(P=0.027).The median weight was 8.3(IQR:4.0-13.3)*** with fever had higher weight than those without fever[10.2(IQR:7.3-13.0)vs 7

关键词： Fever pediatrics Admission patterns Temperature measurement Guidelines Bahrain

A novel use of the comprehensive complication index in perihilar cholangiocarcinoma surgery

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Hepatobiliary surgery and Nutrition 2024年第1期13卷 139-142页

作者： Edoardo Poletto Simone Conci Tommaso Campagnaro Mario De Bellis Laura Alaimo Andrea Ruzzenente department of surgery DentistryPediatrics and GynecologyHepatobiliary Surgery UnitUniversity Hospital Integrated Trust of VeronaUniversity of VeronaVeronaItaly HPB surgery and Transplantation Unit Erasmus MCRotterdamThe Netherlands

It is well known that radical intent surgery is,as of today,the only curative treatment available for perihilar cholangiocarcinoma(pCCA);the standard surgical procedure includes major hepatectomy with caudate lobe resection and extrahepatic bile duct resection(1).A recent paper set the benchmark values for pCCA surgery:in this paper,acceptable benchmark values for morbidity.

关键词： Comprehensive complication index(CCI) perihilar cholangiocarcinoma(pCCA) postoperative complication trajectory

An acoustofluidic device for the automated separation of platelet-reduced plasma from whole blood

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Microsystems & Nanoengineering 2024年第3期10卷 293-303页

作者： Zhehan Ma Jianping Xia Neil Upreti Emeraghi David Joseph Rufo Yuyang Gu Kaichun Yang Shujie Yang Xiangchen Xu Jean Kwun Eileen Chambers Tony Jun Huang Thomas Lord department of Mechanical Engineering and Materials Science Duke UniversityDurhamNCUSA department of Biomedical Engineering Duke UniversityDurhamNCUSA department of pediatrics Duke UniversityDurhamNCUSA Duke Transplant Center Department of SurgeryDuke University School of MedicineDurhamNCUSA

Separating plasma from whole blood is an important sample processing technique required for fundamental biomedical research,medical diagnostics,and therapeutic *** protocols for plasma isolation require multiple centrifugation steps or multiunit microfluidic processing to sequentially remove large red blood cells(RBCs)and white blood cells(WBCs),followed by the removal of small ***,we present an acoustofluidic platform capable of efficiently removing RBCs,WBCs,and platelets from whole blood in a single *** leveraging differences in the acoustic impedances of fluids,our device generates significantly greater forces on suspended particles than conventional microfluidic approaches,enabling the removal of both large blood cells and smaller platelets in a single *** a result,undiluted human whole blood can be processed by our device to remove both blood cells and platelets(>90%)at low voltages(25 Vpp).The ability to successfully remove blood cells and platelets from plasma without altering the properties of the proteins and antibodies present creates numerous potential applications for our platform in biomedical research,as well as plasma-based diagnostics and ***,the microfluidic nature of our device offers advantages such as portability,cost efficiency,and the ability to process small-volume samples.

关键词： remove separation removing

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

Height and body mass index trajectories from 1975 to 2015 and prevalence of stunting,underweight and obesity in 2016 among children in Chinese cities:findings from five rounds of a national survey

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World Journal of pediatrics 2024年第4期20卷 404-412页

作者： Xin‑Nan Zong Hui Li Ya‑Qin Zhang department of Growth and Development Capital Institute of PediatricsBeijing 100020China

Background A more comprehensive assessment of growth and nutrition in children is required in China due to rapid socioeconomic *** aimed to investigate height and body mass index(BMI)trajectories from 1975 to 2015 and the prevalence of stunting and obesity in 2016 among children in Chinese *** A total of 904,263 children from birth to 6.9 years were collected from a series of nationally representative surveys in *** and BMI trajectories and prevalence of stunting,underweight,overweight and obesity were *** The average height level of Chinese urban children under 7 years presented a positive secular trend from 1975 to 2015;however,a slowing tendency occurred in 2005‒*** apparent increase was observed at the 5th,50th and 95th percentiles of BMI in urban children aged 3 years and older,with a more prominent increase at the 95th *** total prevalence of stunting and underweight under 7 years was 1.4%and 2.0%,*** total prevalence of overweight and obesity under 7 years was 12.6%and 4.3%,respectively,with 12.7%and 4.9%for boys,12.6%and 3.6%for girls,12.1%and 4.0%in urban areas and 13.1%and 4.5%in suburban rural *** The average height level of Chinese urban children has reached World Health Organization child growth standards since 2005 and presented a slowing tendency in secular trend in 2005‒*** attention and efforts and public health interventions should be urgently made to combat overweight and obesity among preschool children.

关键词： Body mass index Children Height Obesity Stunting

Surgical Repair of Ventricular Septal Defect in Neonates: Indications and Outcomes

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Congenital Heart Disease 2024年第1期19卷 69-83页

作者： Jae Hong Lee Sungkyu Cho Jae Gun Kwak Hye Won Kwon Woong-Han Kim Mi Kyoung Song Sang-Yun Lee Gi Beom Kim Eun Jung Bae department of Thoracic and Cardiovascular surgery Research Institute for Convergence of Biomedical Science and TechnologyPusan National University Yangsan HospitalYangsanKorea department of Thoracic and Cardiovascular surgery Seoul National University Children’s HospitalSeoulKorea department of pediatrics Seoul National University Children’s HospitalSeoulKorea

Background:The optimal surgical timing and clinical outcomes of ventricular septal defect(VSD)closure in neo-nates remain *** aimed to evaluate the clinical outcomes of VSD closure in neonates(age≤30 days).Methods:We retrospectively reviewed 50 consecutive neonates who underwent VSD closure for isolated VSDs between August 2003 and June *** for the procedure included congestive heart failure/failure to thrive and pulmonary *** adverse events(MAEs)were deﬁned as the composite of all-cause mortality,reoperation,persistent atrioventricular block,and signiﬁcant(≥grade 2)valvular ***:The median age and body weight at operation were 26.0 days(interquartile range[IQR],18.8–28.3)and 3.7 kg(IQR,3.3–4.2),*** median follow-up duration was 110.4 months(IQR,56.8–165.0).Seven patients required preoperative respiratory support,andﬁve had signiﬁcant(≥grade 2)preoperative valvular *** early mortality occurred due to irreversible cardiogenic shock;no late mortality was *** reopera-tion was due to hemodynamically signiﬁcant residual VSD at 103.8 months *** overall survival,freedom from reoperation,and freedom from MAE at 15-years were 98.0%,96.3%,and 94.4%,***-operative mechanical ventilation was associated with a longer duration of postoperative mechanical ventilation(p<0.001)and a longer length of intensive care unit stay(p<0.001).Conclusions:VSD closure with favorable outcomes without morbidities is feasible even in ***,neonates requiring preoperative respiratory support may require careful postoperative management considering the long-term postoperative ***,surgical VSD closure might be indicated earlier in neonates with respiratory compromise.

关键词： Ventricular septal defect neonate early surgery neonatal surgery

Precise genome-editing in human diseases:mechanisms,strategies and applications

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Signal Transduction and Targeted Therapy 2024年第3期9卷 1065-1085页

作者： Yanjiang Zheng Yifei Li Kaiyu Zhou Tiange Li Nathan J.VanDusen Yimin Hua Key Laboratory of Birth Defects and Related Diseases of Women and Children of MOE Department of PediatricsWest China Second University HospitalSichuan UniversityChengduSichuan 610041China department of Cardiovascular surgery West China HospitalSichuan UniversityChengduSichuan 610041China department of pediatrics Herman B Wells Center for Pediatric ResearchIndiana University School of MedicineIndianapolisIN 46202USA

Precise genome-editing platforms are versatile tools for generating specific,site-directed DNA insertions,deletions,and *** continuous enhancement of these tools has led to a revolution in the life sciences,which promises to deliver novel therapies for genetic *** genome-editing can be traced back to the 1950s with the discovery of DNA’s doublehelix and,after 70 years of development,has evolved from crude in vitro applications to a wide range of sophisticated capabilities,including in vivo ***,precise genome-editing faces constraints such as modest efficiency,delivery challenges,and off-target *** this review,we explore precise genome-editing,with a focus on introduction of the landmark events in its history,various platforms,delivery systems,and ***,we discuss the landmark events in the history of precise ***,we describe the current state of precise genome-editing strategies and explain how these techniques offer unprecedented precision and versatility for modifying the human ***,we introduce the current delivery systems used to deploy precise genome-editing components through DNA,RNA,and ***,we summarize the current applications of precise genome-editing in labeling endogenous genes,screening genetic variants,molecular recording,generating disease models,and gene therapy,including ex vivo therapy and in vivo therapy,and discuss potential future advances.

关键词： precise generating editing

Diseases of bile duct in children

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World Journal of Gastroenterology 2024年第9期30卷 1043-1072页

作者： Sutha Eiamkulbutr Chomchanat Tubjareon Anapat Sanpavat Teerasak Phewplung Nimmita Srisan Palittiya Sintusek department of pediatrics King Chulalongkorn Memorial HospitalBangkok 10330Thailand department of pediatrics Faculty of MedicineChulalongkorn University and King Chulalongkorn Memorial HospitalBangkok 10330Thailand department of Pathology Chulalongkorn UniversityBangkok 10330Thailand department of Radiology Chulalongkorn UniversityBangkok 10330Thailand department of surgery Faculty of MedicineChulalongkorn University and King Chulalongkorn Memorial HospitalBangkok 10330Thailand Center of Excellence in Thai Pediatric Gastroenterology Hepatology and ImmunologyDivision of GastroenterologyDepartment of PediatricsKing Chulalongkorn Memorial HospitalChulalongkorn UniversityBangkok 10330Thailand

Several diseases originate from bile duct *** studies on these diseases,certain etiologies of some of them still cannot be *** most common disease of the bile duct in newborns is biliary atresia,whose prognosis varies according to the age of surgical *** diseases such as Alagille syndrome,inspissated bile duct syndrome,and choledochal cysts are also time-sensitive because they can cause severe liver damage due to *** majority of these diseases present with cholestatic jaundice in the newborn or infant period,which is quite difficult to differentiate regarding clinical acumen and initial *** cholangiography is potentially necessary to make an accurate diagnosis,and further treatment will be performed synchronously or planned as findings *** article provides a concise review of bile duct diseases,with interesting cases.

关键词： Bile duct Cholestasis Biliary atresia Biliary hypoplasia Biliary imaging Inspissated bile syndrome Choledochal cyst