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Improved survival at the cost of more chronic lung disease?Current management and outcomes in extremely preterm infants born in New south Wales and the Australian Capital Territory:2010-2020

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World Journal of Pediatrics 2024年第3期20卷 230-238页

作者： Nele Legge Himanshu Popat Dominic Fitzgerald Liverpool hospital Corner Elizabeth and Goulburn StreetsLiverpoolNSWAustralia University of sydney CamperdownAustralia children's hospital westmead WestmeadAustralia

Background since 2010,most tertiary care hospitals in Australia have changed how they care for extremely premature ***,in-hospital and longer-term outcome data have suggested unchanged or even worse health outcomes in later epochs,especially respiratory *** study examined the trend in outcomes since these changes were introduced,particularly the prevalence of chronic neonatal lung disease(CLD).Methods This is a retrospective cross-sectional analysis of data from the Neonatal Intensive Care Units'(NICUs)database of all perinatal intensive care units in New south Wales and the Australian Capital Territory,including infants born at≥24 and≤28 weeks of gestational age in tertiary perinatal units between January 1,2010,and December 31,*** trends and changes in primary outcome were examined by linear and adjusted multivariable logistic regression *** This study included 3258 *** saw significant changes in antenatal magnesium sulfate(75% increase),delayed cord clamping(66% increase),delivery room intubations(30% decrease),any time(20% decrease),duration on mechanical ventilation(100-hour decrease),and hours on noninvasive ventilation(200-hour increase).Mortality decreased from 17% to 6%.The incidence of CLD increased significantly even when adjusted for confounders(15% increase).Any time and mean hours spent on mechanical ventilation significantly increased the odds of *** study could not find a significant association of any of the protective antenatal treatments on *** The last decade saw a significant improvement in survival and survival to discharge without major *** was increased use of magnesium sulfate,delayed cord clamping,and less invasive respiratory management of extremely preterm *** avoidance of mechanical ventilation may impact the incidence of CLD.

关键词： Chronic lung disease Extreme prematurity Health outcomes survival

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Complications of button battery ingestion or insertion in children:a systematic review and pooled analysis of individual patient-level data

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World Journal of Pediatrics 2024年第10期20卷 1017-1028页

作者： Christopher Tran Carlos Nunez Guy D.Eslick Ruth Barker Elizabeth J.Elliott sydney Medical school Faculty of Medicine and HealthThe University of SydneySydneyNSWAustralia The Australian Paediatric surveillance Unit Level 2Kids ResearchThe Sydney Children’s Hospital Network(Westmead)Locked Bag 4001WestmeadNSW2145Australia Queensland children’s hospital BrisbaneQLDAustralia Queensland Injury surveillance Unit Jamieson Trauma InstituteRoyal Brisbane and Women’s HospitalHerstonQLDAustralia

Background Button battery(BB)exposures are common in children and can have devastating *** reviewed current evidence on the complications associated with BB exposure and identified predictors of outcomes using individual patient-level *** sources We carried out a systematic review and pooled analysis by searching MEDLINE,Embase,and scopus up to May 19,*** studies describe complications following BB exposures in children(ageds).Odds ratios(ORs)were calculated using binary logistic regression to measure associations between predictive factors and different *** Two-hundred seventeen studies(439 children)were *** median age at presentation was 1.75[interquartile range(IQR)1.00–3.00]years and 399(90.9%)exposures were *** the 380 cases reporting sex,162(42.6%)were ***(192,48.1%)and respiratory difficulties(138,34.6%)were common presenting features for ingestions,while symptomatology was site-specific for *** complications included oesophageal mucosal damage alone(105,26.3%)and tracheooesophageal fistula(93,23.3%)for ingestions,and nasal septal perforation(22,55.0%)and mucosal damage alone(13,32.5%)for *** perforation occurred in 2.5%of ingestion cases,including perforation of Meckel's diverticulum,peritonitis,and jejunocolic *** complications were common among children who ***(≤2 years),battery exposure duration(>6 hours),and battery diameter(≥20 mm)were associated with common and severe complications of *** BB injuries are time-critical,with severe sequelae predominantly affecting young *** is *** work through regulation and safer battery design are required to eliminate this problem.

关键词： Child Electric power supplies Digestive systems Fatal outcome Foreign bodies

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Update on global epidemiology of viral hepatitis and preventive strategies

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World Journal of Clinical Cases 2018年第13期6卷 589-599页

作者： Meryem Jefferies Bisma Rauff Harunor Rashid Thao Lam shafquat Rafiq Drug Health Western Sydney Local Health District westmead Institute for Medical Research Westmead Hospital Sydney Medical School University of Sydney National Centre for Immunisation Research and surveillance of Vaccine Preventable Diseases the Children’s Hospital at Westmead and the Discipline of Child and Adolescent Health Sydney Medical School Croydon University hospital NHs Trust

Viral hepatitis is one of the major public health concerns around the world but until recently it has drawn little attention or funding from global health *** year 1.4 million people die from viral hepatitisrelated cirrhosis and liver ***,the majority of the infected population are unaware of their *** population have significant obstacles to overcome such as lack of awareness,vulnerability,increased migration,disease stigma,discrimination,as well as poor health resources,conflict in policy development and program *** implementing infection control measures over the last few decades eradication or significant disease reduction remains *** study aims to present the current global prevalence status and examines potential elimination *** information for this research were obtained through a systematic review,published scientific literatures,the official websites of various government organisations,international public health organisations and internationally recognised regulatory bodies over a period of 40 years between 1978 and2018.

关键词： Cirrhosis Global epidemiology Outreach clinic Liver cancer Vaccination Viral hepatitis

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Constitutive androstane receptor:A tumor suppressor and a biomarker for favorable prognosis of liver diseases

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Genes & Diseases 2024年第6期11卷 88-90页

作者： sarah Da Won Bae Romario Nguyen Lawrence Yuen Vincent Lam Jacob George Liang Qiao storr Liver Centre Westmead institute for Medical ResearchUniversity of Sydneyand Westmead HospitalWestmeadNSW2145Australia Department of surgery Westmead HospitalWestmeadNSW2145Australia

Constitutive androstane receptor(CAR)is a nuclear receptor that is encoded by the gene NR113(nuclear receptor subfamily 1 group I member 3)and is almost exclusively expressed in the liver.1 As reported in our recent publication,CAR is well established as a xenosensor for drugs and energy metabolism with newer implications in the regulation of normal liver physiology and liver regeneration.

关键词： metabolism drugs liver

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Ventricular Arrhythmia in the Fontan Circulation:Prevalence,Risk Factors and Clinical Implications

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Congenital Heart Disease 2023年第5期18卷 507-523页

作者： Charis Tan Diana Zannino Carley Clendenning sophie Offen Thomas LGentles Julian Ayer David Tanous Vishva Wijesekera Leeanne Grigg David Celermajer Mark McGuire Yves d’Udekem Rachael Cordina Department of Cardiothoracic surgery Royal Prince Alfred HospitalCamperdownAustralia sydney Medical school University of SydneyCamperdownAustralia Clinical Epidemiology and Biostatistics Unit Murdoch Children’s Research InstituteParkvilleAustralia Department of Cardiology Royal Prince Alfred HospitalCamperdownAustralia Department of Paediatric Cardiology Starship HospitalAucklandNew Zealand Department of Paediatric Cardiology The Children’s Hospital at WestmeadWestmeadAustralia Department of Cardiology Westmead HospitalWestmeadAustralia Adult Congenital Heart Disease Unit The Prince Charles HospitalBrisbaneAustralia Department of Cardiology The Royal Melbourne HospitalMelbourneAustralia Heart Research Institute CamperdownAustralia Department of Cardiac surgery The Royal Children’s Hospital MelbourneParkvilleAustralia Department of Paediatrics The University of MelbourneParkvilleAustralia

Objective:sudden cardiac death(sCD)and malignant ventricular arrhythmia(VA)are increasingly recognized as important issues for people living with a Fontan circulation,but data are *** sought to characterize the cohort who had sudden cardiac death,most likely related to VA and/or documented VA in the Australia and New Zealand Fontan Registry including risk factors and clinical ***:A retrospective cohort study was *** criteria were documented non-sustained ventricular tachycardia,sustained ventricular tachycardia,ventricular fibrillation,resuscitated cardiac arrest or sCD>30 days post-Fontan ***:Of 1611 patients,20(1.2%)had VA;14(1.0%)had VA without sCD and 6(sCD(6%of all deaths recorded in Registry;5 of those had documented VA at the time of arrest and 1 was presumed to be VA-associated).The median age at first VA was 20.5(14–32)years,10(50%)were females,and the median age at Fontan operation was 8(4–17)*** univariable analysis,hypoplastic left heart syndrome(p=0.03)and older age Fontan operation(pssociated with *** Fontan era(pst-Fontan atrial arrhythmia(p=0.010)were associated with *** with VA had a 3 times higher risk of death or heart transplant(HR 3.27(1.19,8.98),p=0.02).Conclusions:A proportion of people living with a Fontan circulation have malignant *** VA screening in this cohort is *** data are needed to aid risk stratification.

关键词： Fontan congenital cardiac arrhythmia sudden cardiac death ventricular tachyarrhythmia

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Current concepts on the role of nitric oxide in portal hypertension

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World Journal of Gastroenterology 2013年第11期19卷 1707-1717页

作者： Liang shuo Hu Jacob George Jian Hua Wang storr Liver Unit Westmead Millennium Institute and Westmead HospitalUniversity of SydneyWestmeadNSW 2145Australia

Portal hypertension(PHT) is defined as a pathological increase in portal venous pressure and frequently accompanies *** pressure can be increased by a rise in portal blood flow,an increase in vascular resistance,or the *** cirrhosis,the primary factor leading to PHT is an increase in intra-hepatic resistance to blood *** much of this increase is a mechanical consequence of architectural disturbances,there is a dynamic and reversible component that represents up to a third of the increased vascular resistance in *** vasoactive substances contribute to the development of *** these,nitric oxide(NO) is the key mediator that paradoxically regulates the sinusoidal(intra-hepatic) and systemic/splanchnic *** deficiency in the liver leads to increased intra-hepatic resistance while increased NO in the circulation contributes to the hyperdynamic systemic/splanchnic *** mediated-angiogenesis also plays a role in splanchnic vasodilation and collateral circulation *** donors reduce PHT in animals models but the key clinical challenge is the development of an NO donor or drug delivery system that selectively targets the liver.

关键词： Nitric oxide Portal hypertension Hepatic stellate cell Liver cirrhosis

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systemic postnatal corticosteroid use for the prevention of bronchopulmonary dysplasia and its relationship to early neurodevelopment in extremely preterm

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World Journal of Pediatrics 2023年第6期19卷 586-594页

作者： Emilia Esterman Traci-Anne Goyen Pranav Jani Gemma Lowe Jane Baird Rajesh Maheshwari Daphne D’Cruz Melissa Luig Dharmesh shah Department of Neonatology Westmead HospitalWestmeadAustralia Faculty of Medicine and Health The University of SydneySydneyAustralia

Background systemic postnatal corticosteroid use in extremely preterm infants poses a risk of adverse neurodevelopmental *** study explores their use beyond seven days of age with early neurodevelopmental assessments during the fidgety period(9-20 weeks postterm age).Methods This retrospective single-center cohort study included inborn extremely preterm infants from 1 January 2014 to 31 December *** infants,those with congenital or genetic abnormalities,and those who received postnatal cor-ticosteroids for nonrespiratory reasons were *** cohort was dichotomized based on the status of corticosteroid *** neurodevelopmental outcomes were reported using Prechtl's General Movements *** Of the 282 infants,67(23.75%)received *** these,34(50.75%)received them for dependency on invasive ventilation(intermittent positive-pressure ventilation),and the remainder received them for dependency on non-invasive ventilation continuous positive airway pressure(CPAP)or bi-level positive airway pressure(BiPAP).Abnormal or absent fidgety movements were observed in 13% of infants(7/54)who received corticosteroids compared to 2% of infants(3/146)who did *** increased odds for an abnormal general movements assessment from corticosteroid use after adjusting for gestational age[adjusted odds ratio(aOR)=5.5,95% confidence interval(CI)=1.14-26.56]was *** motor optimality scores differed between the two groups[corticosteroid group:25.5(23-26)versus no-corticosteroid group:26(24-28);z=-2.02].A motor optimality scores observed in 14.8% of infants(8/54)in the corticosteroid group compared to 2% of infants(3/146)in the noncorticosteroid *** difference was significant after adjustment for gestational age(aOR 5.96,95%CI 1.28-27.74).Conclusions Abnormal early neurodevelopment was observed in infants who received systemic postnatal *** relationship between these findings and other factors influen

关键词： Corticosteroids Early neurodevelopment General movement assessment Preterm infants

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A humanized mouse model to study human immune response in xenotransplantation

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Hepatobiliary & Pancreatic Diseases International 2012年第5期11卷 494-498页

作者： Peta Phillips Center for Transplant and Renal Research Westmead Millennium InstituteWestmead HospitalWestmeadNSW Sydney 2145Australia

BACKGROUND: A major barrier to the clinical application of xenotransplantation as a treatment option for patients is T cell-mediated rejection. studies based on experimental rodent models of xenograft tolerance or rejection in vivo have provided useful information about the role of T cell immune response in xenotransplantation. However not all observations seen in rodents faithfully recapitulate the human situation This study aimed to establish a humanized mouse model of xenotransplantation, which mimics xenograft rejection in the context of the human immune system. METHODs: NOD-sCID IL2rγ -/- mice were transplanted with neonatal porcine islet cell clusters (NICC) followed by reconstitution of human peripheral blood mononuclear cells (PBMC). Human leukocyte engraftment and islet xenograft rejection were confirmed by flow cytometric and histological analyses. REsULTs: In the absence of human PBMC, porcine NICC transplanted into NOD-sCID IL2rγ -/- mice revealed excellent graft integrity and endocrine function. Human PBMC demonstrated a high level of engraftment in NOD-sCID IL2rγ -/- mice. Reconstitution of NICC recipient NOD-sCID IL2rγ -/- mice with human PBMC led to the rapid destruction of NICC xenografts in a PBMC number-dependent manner. CONCLUsIONs: Human PBMC-reconstituted NOD-sCID IL2rγ -/- mice provide an ideal model to study human immune responses in xenotransplantation. studies based on this humanized mouse model will provide insight for improving the outcomes of clinical xenotransplantation.

关键词： humanized mice xenotransplantation neonatal porcine islet cell cluster xenorejection tolerance

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Resistin mediates the hepatic stellate cell phenotype

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World Journal of Gastroenterology 2013年第28期19卷 4475-4485页

作者： Zhi-Xia Dong Lin su Joanne Brymora Claire Bird Qing Xie Jacob George Jian-Hua Wang Department of Infectious Disease Rui jin Hospital Shanghai Jiao Tong University School of Medicine storr Liver Unit Westmead Millennium Institute and Westmead Hospital University of Sydney Westmead NSW 2145 Australia

AIM: To describe the role of resistin in liver fibrosis. METHODs: For the in vivo animal study, sprague Dawley rats were subjected to bile duct ligation (BDL) for 4 wk. Rat liver, adipose tissue (epididymal fat) and serum were analyzed for resistin expression. For the in vitro experiment, rat primary hepatic stellate cells (HsCs) and Kupffer cells (KCs) were used. HsCs were exposed to recombinant resistin, and collagenⅠ, transforming growth factor β1, α smooth muscle actin, tissue inhibitor of metalloproteinase 1 and connective tissue growth factor expression were analyzed. Resistin gene and protein expression was quantified as was the expression of pro-inflammatory cytokines including tumor necrosis factor α (TNFα), interleukin (IL)-1, IL-6, IL-8 and monocyte chemotactic protein-1 (MCP-1). The effects of resistin on HsC proliferation, migration and apoptosis were determined. The effects of resistin on KCs were also investigated. REsULTs: Following BDL, rat epididymal fat and serum rather than liver showed higher resistin expression compared to control rats. In liver, resistin was expressed in quiescent HsCs and KCs. Resistin treatment resulted in enhancement of TNFα , IL-6 , IL-8 and MCP-1 gene expression and increased IL-6 and MCP-1 protein in HsCs. Resistin activated HsC phospho-MAPK/p38, and p38 inhibition diminished IL-6 and MCP-1 expression. Furthermore, resistin facilitated HsC proliferation and migration, but decreased apoptosis which was via an IL-6 and MCP-1 mechanism. Finally, resistin-induced transforming growth factor β1 from KCs enhanced HsC collagenⅠexpression. CONCLUsION: Resistin directly and indirectly modulates HsC behavior towards a more pro-fibrogenic phenotype.

关键词： Resistin Hepatic stellate cell Kupffer cell Liver fibrosis Monocyte chemotactic protein-1

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Clinical features of nonalcoholic fatty liver disease-associated hepatocellular carcinoma

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Hepatobiliary & Pancreatic Diseases International 2012年第1期11卷 18-27页

作者： Xiao-Yan Duan, Liang Qiao and Jian-Gao Fan Department of Gastroenterology, Xinhua hospital, shanghai Jiaotong University school of Medicine, shanghai 200092, China storr Liver Unit at the westmead Millennium Institute, the University of sydney at westmead hospital, westmead, NsW 2145, Australia [a] Department of Gastroenterology Xinhua Hospital Shanghai Jiaotong University School of Medicine Shanghai 200092 China [b] storr Liver Unit at the westmead Millennium Institute the University of Sydney at Westmead Hospital Westmead NSW 2145 Australia

BACKGROUND: Nonalcoholic fatty liver disease (NAFLD), especially nonalcoholic steatohepatitis, is a recognized risk factor for hepatocellular carcinoma (HCC). However, detailed analysis of the clinical features in patients with NAFLD and their association with HCC is lacking. This study aimed to update the clinical features of patients with NAFLD-associated HCC. DATA sOURCEs: The clinical data of patients with NAFLD- associated HCC from 25 studies published between 1990 and 2010 in the Pubmed database were comprehensively reviewed. REsULTs: In a total of 169 patients with NAFLD-associated HCC, 72.8% were male. The median age at abnormal liver function tests and diagnosis of NAFLD and HCC was 60, 64 and 67 years, respectively. Most patients were obese (75%) and diabetic (59.8%), 32.3% had dyslipidemia, and 53% had hypertension. Nearly all patients (98.6%, 71/72) were complicated with at least one metabolic disorder. The majority (76%) of the HCC patients had a solitary tumor nodule, with the tumor size ranging from 0.8 to 20 cm in diameter (mean 3.4 cm). Most (61.1%) of the patients had moderately-differentiated HCC. In 40.2% of the patients, HCC occurred in the absence of cirrhosis. Among 130 patients, 57.7% underwent hepatectomy and 14.6% received liver transplantation. The mean follow-up of the treated patients for 25 months showed that 32.4% (24/74) died and 18.8% (9/48) had recurrence. CONCLUsIONs: Patients with NAFLD-associated HCC are usually accompanied with metabolic disorders. Regular surveillance in patients with NAFLD for HCC is necessary, especially for elderly men with metabolic syndrome.

关键词： obesity diabetes mellitus dyslipidemia nonalcoholic fatty liver disease nonalcoholic steatohepatitis hepatocellular carcinoma

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