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Relation between dysbiosis and inborn errors of immunity

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World Journal of Methodology 2024年第4期14卷 104-109页

作者： Öner Özdemir Department of pediatric allergy and immunology Sakarya UniversityMedical FacultyAdapazarı54100SakaryaTürkiye

Inborn errors of immunity(IEI)disorders,formerly primary immune deficiency diseases,are a heterogeneous group of disorders with variable hereditary transitions,clinical manifestations,complications and varying disease *** of the clinical symptoms,signs and complications in IEI patients can be attributed to inflammatory and immune dysregulatory processes due to loss of microbial diversity(dysbiosis).For example,in common variable immunodeficiency patients,the diversity of bacteria,but not fungi,in the gut microbiota has been found to be reduced and significantly ***,this was associated with a more severe disease *** of the STAT3/Th17 pathway in hyper-IgE syndrome may lead to dysbiosis of the oral microbiota in these patients,causing Candida albicans to switch from commensal to *** of the microbiota can be used as a therapeutic approach in patients with ***,probiotics,postbiotics and fecal microbiota transplantation can be used to restore the balance of the gut microbiota and reduce pathogenicity in IEI *** trials are currently underway to understand the impact of this dysbiosis on the phenotype of IEI diseases and its role in their treatment.

关键词： Immunodeficiency Microbiome Microbiota Fecal microbiota transplantation Immunoglobulin

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

Aryl hydrocarbon receptor:Linking environment to aging process in elderly patients with asthma

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Chinese Medical Journal 2024年第4期137卷 382-393页

作者： Tianrui Yang Rongjun Wan Wei Tu Sai Nithin Avvaru Peisong Gao division of allergy and Clinical immunology Department of MedicineJohns Hopkins University School of MedicineBaltimoreMD 21224USA Department of Geriatric Medicine The First People’s Hospital of Yunnan ProvinceKunmingYunnan 650032China Department of Respiratory Medicine Xiangya HospitalCentral South UniversityChangshaHunan 410008China The State Key Laboratory of Respiratory Disease for allergy Shenzhen Key Laboratory of Allergy and ImmunologyShenzhen University School of MedicineShenzhenGuangdong 518055China

Aging is a significant risk factor for various diseases,including asthma,and it often leads to poorer clinical outcomes,particularly in elderly *** is recognized that age-related diseases are due to a time-dependent accumulation of cellular damage,resulting in a progressive decline in cellular and physiological functions and an increased susceptibility to chronic *** effects of aging affect not only the elderly but also those of younger ages,posing significant challenges to global ***,understanding the molecular mechanisms associated with aging in different diseases is *** intriguing factor is the aryl hydrocarbon receptor(AhR),which serves as a cytoplasmic receptor and ligand-activated transcription factor and has been linked to the aging ***,we review the literature on several major hallmarks of aging,including mitochondrial dysfunction,cellular senescence,autophagy,mitophagy,epigenetic alterations,and microbiome ***,we provide an overview of the impact of AhR on these hallmarks by mediating responses to environmental exposures,particularly in relation to the immune ***,we explore how aging hallmarks affect clinical characteristics,inflammatory features,exacerbations,and the treatment of *** is suggested that AhR signaling may potentially play a role in regulating asthma phenotypes in elderly populations as part of the aging process.

关键词： Aging Environment Reactive oxygen species Senescence Aryl hydrocarbon receptor Asthma

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

A 63-Year-Old Male with D-Transposition of the Great Arteries Who Had an Early Form of the Arterial Switch Operation

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Congenital Heart Disease 2024年第1期19卷 65-68页

作者： Michael A.Rebolledo Jane S.Yao Jason N.Johnson Umar S.Boston Benjamin R.Waller III division of pediatric Cardiology Department of PediatricsThe University of Tennessee Health Science CenterThe Heart InstituteLe Bonheur Children’s HospitalMemphisUSA College of Medicine The University of Tennessee Health ScienceMemphisUSA division of pediatric Cardiothoracic Surgery Department of SurgeryThe University of Tennessee Health Science CenterHeart InstituteLe Bonheur Children’s HospitalMemphisUSA

We describe a 63-year-old male who appears to have undergone an early form of the arterial switch operation for D-transposition of the great arteries performed in the *** review the clinical and imaging data that support our *** had a diagnostic cardiac catheterization which demonstrated severe pulmonary hypertension responsive to epoprostenol and *** case may represent one example of the experimental surgical work done prior to *** Jatene’s description of theﬁrst successful arterial switch performed in 1975.

关键词： Adult congenital heart disease arterial switch operation great vessel anomaly imaging(all modalities)