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Role of practice standardization in outcome optimization for CDH

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World Journal of pediatric Surgery 2024年第2期7卷 110-118页

作者： Alexandra Dimmer Robert Baird Pramod Puligandla Harvey E.Beardmore division of pediatric Surgery Department of Pediatric SurgeryMontreal Children's HospitalMontrealQuebecCanada division of pediatric General and Thoracic Surgery British Columbia Children's HospitalVancouverBritish ColumbiaCanada Harvey E.Beardmore Department of pediatric Surgery McGill UniversityMontrealQuebecCanada

Standardization of care seeks to improve patient outcomes and healthcare delivery by reducing unwanted variations in care as well as promoting the efficient and effective use of healthcare *** are many types of standardization,with clinical practice guidelines(CPGs),based on a stringent assessment of evidence and expert consensus,being the hallmark of high-quality *** article outlines the history of CPGs,their benefits and shortcomings,with a specific focus on standardization efforts as it relates to congenital diaphragmatic hernia management.

关键词： congenital clinical shortcomings

Emerging role of regulated cell death in intestinal failure-associated liver disease

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Hepatobiliary & Pancreatic Diseases International 2024年第3期23卷 228-233页

作者： Si-Yang Cheng Lu Jiang Ying Wang Wei Cai Department of pediatric Surgery Xinhua HospitalSchool of MedicineShanghai Jiao Tong UniversityShanghai 200092China Shanghai Key Laboratory of pediatric Gastroenterology and Nutrition Shanghai 200092China Shanghai Institute for pediatric Research Shanghai 200092China division of pediatric Gastroenterology and Nutrition Xinhua HospitalSchool of MedicineShanghai Jiao Tong UniversityShanghai 200092China

Intestinal failure-associated liver disease(IFALD)is a common complication of long-term parenteral nutrition that is associated with significant morbidity and *** is mainly characterized by cholestasis in children and steatohepatitis in ***,there is no effective approach to prevent or reverse the *** cell death(RCD)represents a fundamental biological paradigm that determines the outcome of a variety of liver *** cell death is reclassified into several types,based on the mechanisms and morphological *** evidence has linked different modes of RCD,such as apoptosis,necroptosis,ferroptosis,and pyroptosis to the pathogenesis of liver *** studies have shown that different modes of RCD are present in animal models and patients with *** the pathogenic roles of cell death may help uncover the underlying mechanisms and develop novel therapeutic strategies in *** this review,we discuss the current knowledge on how RCD may link to the pathogenesis of *** highlight examples of cell death-targeted interventions aiming to attenuate the disease,and provide perspectives for future basic and translational research in the field.

关键词： Parenteral nutrition Apoptosis Necroptosis Cholestasis Steatosis

An evaluation of ureteral diameter ratio and vesicoureteral reflux index in the treatment of primary vesicoureteral reflux

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Asian Journal of Urology 2024年第3期11卷 437-442页

作者： Yusuf Atakan Baltrak Nebil Akdogan Mutlu Deger Volkan Izol Ibrahim Atilla Aridogan Nihat Satar division pediatric Urology Department of UrologyMedical FacultyCukurova UniversityAdanaTurkey

Objective:Vesicoureteral reflux(VUR)index is a simple,validated tool that reliably predicts significant improvement and spontaneous resolution of primary reflux in *** aim of this study was to evaluate and compare the ureter diameter ratio(UDR)and VUR index(VURx)of patients treated with endoscopic injection(EI)and ureteroneocystostomy(UNC)methods in the pediatric age group due to primary ***:Patients under the age of 18 years old who underwent EI and UNC with the diagnosis of primary VUR between January 2011 and September 2021 were determined as the *** UDR was assessed using voiding cystourethrography,and the VURx score was determined prior to treatment based on hospital records included in the ***:A total of 255 patients,60(23.5%)boys and 195(76.5%)girls,with a mean age of 76.5(range 13.0e204.0)months,were included in the *** was applied to 130(51.0%)patients and UNC was applied to 125(49.0%)patients due to primary *** optimum cut-off for the distal UDR was obtained as 0.17 with sensitivity and specificity of 73.0%and 63.0%,*** positive and negative predictive values were 66.0%and 70.0%,***:When the UDR and VURx score are evaluated together for the surgical treatment of primary VUR in the pediatric age group,it is thought that it may be useful in predicting the clinical course of the disease and evaluating surgical treatment options.

关键词： Distalureter diameter ratio Vesicoureteral reflux Vesicoureteral reflux index management Vesicoureteral reflux index

Nicotinamide adenine dinucleotide treatment confers resistance to neonatal ischemia and hypoxia:effects on neurobehavioral phenotypes

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Neural Regeneration Research 2024年第12期19卷 2760-2772页

作者： Xiaowen Xu Xinxin Wang Li Zhang Yiming Jin Lili Li Meifang Jin Lianyong Li Hong Ni division of Brain Science Institute of Pediatric ResearchChildren’s Hospital of Soochow UniversitySuzhouJiangsu ProvinceChina Department of pediatric Orthopedics Shengjing Hospital of China Medical UniversityShenyangLiaoning ProvinceChina

Neonatal hypoxic-ischemic brain injury is the main cause of hypoxic-ischemic encephalopathy and cerebral ***,there are few effective clinical treatments for neonatal hypoxic-ischemic brain ***,we investigated the neuroprotective and molecular mechanisms of exogenous nicotinamide adenine dinucleotide,which can protect against hypoxic injury in adulthood,in a mouse model of neonatal hypoxic-ischemic brain *** this study,nicotinamide adenine dinucleotide(5 mg/kg)was intraperitoneally administered 30 minutes befo re surgery and every 24 hours *** results showed that nicotinamide adenine dinucleotide treatment improved body weight,brain structure,adenosine triphosphate levels,oxidative damage,neurobehavioral test outcomes,and seizure threshold in experimental *** mass tag proteomics revealed that numerous proteins were altered after nicotinamide adenine dinucleotide treatment in hypoxic-ischemic brain injury *** reaction monitoring and western blotting confirmed changes in the expression levels of proteins including serine(or cysteine)peptidase inhibitor,clade A,member 3N,fibronectin 1,5'-nucleotidase,cytosolic IA,microtubule associated protein 2,and complexin *** analyses showed that nicotinamide adenine dinucleotide ameliorated hypoxic-ischemic injury through inflammation-related signaling pathways(e.g.,nuclear factor-kappa B,mitogen-activated protein kinase,and phosphatidylinositol 3 kinase/protein kinase B).These findings suggest that nicotinamide adenine dinucleotide treatment can improve neurobehavioral phenotypes in hypoxic-ischemic brain injury mice through inflammation-related pathways.

关键词： brain injury cerebral palsy hypoxia hypoxic-ischemic brain injury inflammation neuroprotection nicotinamide adenine dinucleotide neonate nicotinamide adenine dinucleotide proteomics

Stubborn Hypoxia in Neonates with D-Transposition of the Great Arteries after Arterial Switch Operation: Central Sleep Apnea as the Cause and Potential Indicator of Brain Immaturity

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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

Hepatocellular SETDB1 Regulates Hepatic Ischemia-Reperfusion Injury through Targeting Lysine Methylation of ASK1 Signal

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Research 2024年第3期6卷 305-317页

作者： Kang Xia Tianyu Wang Zhongbao Chen Jiayu Guo Bo Yu Qi Chen Tao Qiu Jiangqiao Zhou Shusen Zheng Department of Organ transplantation Renmin Hospital of Wuhan UniversityWuhanChina Department of Urology Renmin Hospital of Wuhan UniversityWuhanChina. Department of general surgery Renmin Hospital of Wuhan UniversityWuhanChina division of Hepatobiliary and Pancreatic Surgery Department of SurgeryFirst Affiliated HospitalSchool of MedicineZhejiang UniversityHangzhouChina Key Laboratory of Combined Multi-Organ transplantation Ministry of Public HealthHangzhouChina Key Laboratory of Organ transplantation HangzhouZhejiang ProvinceChina Key Laboratory of the Diagnosis and Treatment of Organ transplantation CAMSHangzhouChina.

Background: Hepatic ischemia-reperfusion injury (HIRI) stands as an unavoidable complication arising from liver surgery, profoundly intertwined with its prognosis. The role of lysine methyltransferase SET domain bifurcated 1 (SETDB1) in HIRI remains elusive, despite its confirmation as a potential therapeutic target for diverse diseases. Here, we investigated the mechanism by which SETDB1 regulated HIRI. Methods: RNA sequencing data were used to identify the expression and potential targets of SETDB1 through bioinformatics analysis. To elucidate the impact of SETDB1 on HIRI, both an in vivo model of HIRI in mice and an in vitro model of hepatocyte hypoxia/reoxygenation were established. Biochemical and histological analyses were used to investigate the influence of SETDB1 on liver damage mediated by HIRI. Chromatin immunoprecipitation and coimmunoprecipitation were implemented to explore the in-depth mechanism of SETDB1 regulating HIRI. Results: We confirmed that hepatocellular SETDB1 was up-regulated during HIRI and had a close correlation with HIRI-related inflammation and apoptosis. Moreover, inhibition of SETDB1 could mitigate HIRI-induced liver damage, inflammation, and apoptosis. Through our comprehensive mechanistic investigation, we revealed that SETDB1 interacts with apoptosis-signal-regulating kinase 1 (ASK1) and facilitates the methylation of its lysine residues. Inhibition of SETDB1 resulted in reduced phosphorylation of ASK1, leading to a marked suppression of downstream c-Jun N-terminal kinase (JNK)/p38 signaling pathway activation. The therapeutic effect on inflammation and apoptosis achieved through SETDB1 inhibition was nullified by the restoration of JNK/p38 signaling activation through ASK1 overexpression. Conclusions: The findings from our study indicate that SETDB1 mediates lysine methylation of ASK1 and modulates the activation of the ASK1–JNK/p38 pathway, thus involved in HIRI-induced inflammation and apoptosis. These results suggest that SETD

关键词： SETDB1 inflammation ASK1

Clinical application of COVID-19 vaccine in liver transplant recipients

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Hepatobiliary & Pancreatic Diseases International 2024年第4期23卷 339-343页

作者： Feng-Chao Liu Man Xie Wei Rao division of Hepatology Liver Disease CenterThe Affiliated Hospital of Qingdao UniversityQingdao 266000China Organ transplantation Center The Affiliated Hospital of Qingdao UniversityQingdao 266000China Institute of Organ Donation and transplantation of Qingdao University Qingdao 266000China Department of Gastroenterology The Affiliated Hospital of Qingdao UniversityQingdao 266000China

Background:Solid organ transplant(SOT)activities,such as liver transplant,have been greatly influenced by the pandemic of coronavirus disease 2019(COVID-19),a disease caused by severe acute respiratory syndrome coronavirus 2(SARS-CoV-2).Immunosuppressed individuals of liver transplant recipients(LTRs)tend to have a high risk of COVID-19 infection and related ***,COVID-19 vaccination has been recommended to be administered as early as possible in *** sources:The keywords“liver transplant”,“SARS-CoV-2”,and“vaccine”were used to retrieve articles published in ***:The antibody response following the 1st and 2nd doses of vaccination was disappointingly low,and the immune responses among LTRs remarkably improved after the 3rd or 4th dose of *** the 3rd or 4th dose of COVID-19 vaccine increased the antibody titer,a proportion of patients remained ***,recent studies showed that SARS-CoV-2 vaccine could trigger adverse events in LTRs,including allograft rejection and liver ***:This review provides the recently reported data on the antibody response of LTRs following various doses of vaccine,risk factors for poor serological response and adverse events after vaccination.

关键词： Liver transplant COVID-19 SARS-CoV-2 Vaccine Antibody response

The Impact of a Prior Norwood Procedure on Cardiac transplantation in Failed Fontan Physiology

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Congenital Heart Disease 2024年第3期19卷 257-266页

作者： Ryan G.McQueen Nikki M.Singh Ronald K.Woods Medical College of Wisconsin Herma Heart Institute at Children’s WisconsinMilwaukeeUSA division of pediatric Cardiology Medical College of WisconsinHerma Heart Institute at Children’s WisconsinMilwaukeeUSA division of pediatric Cardiothoracic Surgery Medical College of WisconsinHerma Heart Institute at Children’s WisconsinMilwaukeeUSA

Objective:The objective of this study was to compare cardiac transplant operative and postoperative courses of patients with failed Fontan physiology who were initially palliated with a Norwood(FFN)to those without a prior Norwood(FF).Methods:A single-institution retrospective review of all patients with Fontan failure who under-went cardiac transplantation from 2003–2021 was completed-22 underwent prior Norwood(FFN)and 11 did not(FF).Descriptive and inferential statistics were calculated for operative course and patient ***:The operative course of the FFN cohort appeared to be more complex(not statistically significant,but clinically relevant)-this group exclusively experienced sternal re-entry events(3 of 22 patients)and concomitant neo-aor-tic reconstruction(6 patients),had a longer duration of surgery(median of 682 min vs.575.5 min),more time on circulatory arrest(median of 25.5 min vs.12.5 min),and more frequent use of open sternal management[50%of patients(11/22)vs.27.3%of patients(3/11)].Postoperatively,these patients underwent more mediastinal explora-tions[other than sternal closure;40.9%of patients(9/22)vs.18.2%of patients(2/11)],spent more time on mechanical ventilation(median of 5 days vs.2 days),had a longer length of stay(median of 30 days vs.19 days),and required more catheter-based re-interventions[22.7%of patients(5/22)vs.9.1%of patients(1/11)].Conclu-sion:Although underpowered,our results suggest that the operative course of FFN patients is more challenging,based mostly on neo-aortic arch *** turn,this likely leads to a more complex postoperative *** are currently collaborating with other institutions to increase the cohort size and power of the study.

关键词： Fontan Fontan failure Norwood cardiac transplantation

Mechanical upper bowel obstruction caused by a large trichobezoar in a young woman: A very unusual case report

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World Journal of Clinical Cases 2024年第4期12卷 777-781页

作者： Magdalena Scherrer Peter Kornprat Robert Sucher Johanna Muehlsteiner Doris Wagner Department of Surgery Division for GeneralVisceral and Transplantation SurgeryMedical University of GrazGraz 8036Austria Department of Surgery Clinic RohrbachRohrbach 4150Austria

BACKGROUND Bezoars usually compile human fibers and debris.A special form of bezoar in case of psychologically altered individuals is the *** consists of voluntarily swallowed hair bulks and is normally removed via *** leading to ileus have rarely been *** SUMMARY A 24-year-old female patient presented to the emergency room with abdominal pain,nausea,and vomiting for 3 *** previous medical and psychiatric history was *** analysis showed iron deficiency anemia,leukocytosis,and elevated liver *** abdominal CT scan revealed a dense structure in the patients’stomach which turned out to be a huge trichobezoar completely obstructing the *** trichobezoar had to be removed *** her postoperative course,a subcutaneous seroma *** a single puncture,the rest of the recovery process was unremarkable,and the patient recovered *** A mechanical bowel obstruction is a potentially life-threatening event for every *** our case a young female was suffering from severe symptoms of an obstruction which might have resulted in serious harm without successful surgical management.

关键词： Rapunzel syndrome Hair ingestion Gastroscopy Bezoar Case report