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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

Clinical-based phenotypes in children with pediatric post-COVID-19 condition

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World Journal of pediatrics 2024年第7期20卷 682-691页

作者： Lieke C.E.Noij Jelle M.Blankestijn Coen R.Lap Marlies A.van Houten Giske Biesbroek Anke-Hilse Maitland-van der Zee Mahmoud I.Abdel-Aziz Johannes B.van Goudoever Mattijs W.Alsem Caroline L.H.Brackel Kim J.Oostrom Simone Hashimoto Suzanne W.J.Terheggen-Lagro department of pediatric pulmonology and Allergy Amsterdam UMCLocation AMCEmma Children’s HospitalAmsterdam University Medical CenterRoom G2-222Meibergdreef 91105AZAmsterdamThe Netherlands department of Pulmonary Medicine Amsterdam UMCAmsterdamThe Netherlands department of pediatrics Spaarne GasthuisHoofddorpThe Netherlands department of pediatrics Emma Children’s HospitalAmsterdam University Medical CenterAmsterdamThe Netherlands department of Rehabilitation Medicine Amsterdam Movement SciencesMeibergdreef 9AmsterdamThe Netherlands department of Paediatric pulmonology Department of PaediatricsTergooi MCHilversumThe Netherlands department of Child and Adolescent Psychiatry and Psychosocial Care Amsterdam UMCAmsterdam Reproduction and DevelopmentAmsterdamThe Netherlands

Background pediatric post coronavirus disease 2019(COVID-19)condition(PPCC)is a heterogeneous syndrome,which can significantly affect the daily lives of *** study aimed to identify clinically meaningful phenotypes in children with PPCC,to better characterize and treat this *** Participants were children with physician-diagnosed PPCC,referred to the academic hospital Amsterdam UMC in the Netherlands between November 2021 and March *** factors and information on post-COVID symptoms,comorbidities,and impact on daily life were *** clusters were identified using an unsupervised and unbiased approach for mixed data *** Analysis of 111 patients(aged 3–18 years)revealed three distinct clusters within *** 1(n=62,median age=15 years)predominantly consisted of girls(74.2%).These patients suffered relatively more from exercise intolerance,dyspnea,and smell *** 2(n=33,median age=13 years)contained patients with an even gender distribution(51.5%girls).They suffered from relatively more sleep problems,memory loss,gastrointestinal symptoms,and *** 3(n=16,median age=11 years)had a higher proportion of boys(75.0%),suffered relatively more from fever,had significantly fewer symptoms(median of 5 symptoms compared to 8 and 10 for clusters 1 and 2 respectively),and experienced a lower impact on daily *** This study identified three distinct clinical PPCC phenotypes,with variations in sex,age,symptom patterns,and impact on daily *** findings highlight the need for further research to understand the potentially diverse underlying mechanisms contributing to post-COVID symptoms in children.

关键词： Adolescents Clusters Long COVID pediatric Phenotypes Post-COVID syndrome

Microphallus early management in infancy saves adulthood sensual life:A comprehensive review

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World Journal of Clinical pediatrics 2024年第2期13卷 16-37页

作者： Mohammed Al-Beltagi Nermin Kamal Saeed Adel Salah Bediwy Majed A Shaikh Reem Elbeltagi department of pediatric Faculty of MedicineTanta UniversityTanta 31511Egypt department of pediatric University Medical CenterKing Abdulla Medical CityArabian Gulf UniversityManama 26671Bahrain department of pediatric University Medical CenterSulaiman Al Habib Medical GroupManama 26671Bahrain department of Medical Microbiology Section and Pathology Salmaniya Medical ComplexMinistry of HealthKingdom of BahrainManama 12Bahrain department of Medical Microbiology Section and Pathology Irish Royal College of SurgeonBusaiteen 15503Bahrain department of pulmonology Faculty of MedicineTanta UniversityTanta 31527Egypt department of pulmonology University Medical CenterKing Abdulla Medical CityArabian Gulf UniversityManama 26671Bahrain department of pulmonology University Medical CenterSulaiman Al Habib Medical GroupManama 26671Bahrain department of pediatrics Ibn Al Nafees HospitalManama 54533Bahrain department of Medicine The Royal College of Surgeons in Ireland-BahrainBusiateen 15503Bahrain

Microphallus/Micropenis is a rare condition with significant physical and psychological implications for affected *** article comprehensively reviews micropenis,its etiology,epidemiology,and various treatment *** conducted a thorough literature review to collect relevant information on micropenis and microphallus,as well as related *** primary databases were PubMed,Medline,and Google *** searched for articles published in English between 2000 and *** analysis included 67 review articles,56 research studies,11 case reports,one guideline,and one *** search terms included"microphallus","micropenis","congenital hypogonadotropic hypogonadism","androgen insensitivity syndrome","pediatric management of micropenis","testosterone therapy",and"psychosocial implications of micropenis".We focused on diagnosing micropenis and related conditions,including hormonal assessments,medical and surgical treatment options,psychosocial and psychological well-being,sexual development of adolescents,and sociocultural influences on men's perceptions of penile ***,we explored parenting and family dynamics in cases of micropenis and disorders of sex development,implications of hormonal treatment in neonates,and studies related to penile augmentation procedures and their *** article highlights the importance of early diagnosis and intervention in addressing the physical and psychological well-being of individuals with *** procedures,such as penile lengthening and girth enhancement,and non-surgical approaches like hormonal therapy are *** significance of psychological support,education,and lifestyle modifications is *** management and comprehensive care are crucial for individuals with micropenis,from infancy to adolescence and beyond.A multidisciplinary approach involving urologists,endocrinologists,and mental health professionals is *** assessment of treatment e

关键词： Microphallus Micropenis Hypogonadism Children Adults Sensual life Testosterone therapy

Mechanism of Endogenous Peptide PDYBX1 and Precursor Protein YBX1 in Hirschsprung’s Disease

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Neuroscience Bulletin 2024年第6期40卷 695-706页

作者： Qiaochu Sun Zhengke Zhi Chenglong Wang Chunxia Du Jie Tang Hongxing Li Weibing Tang department of pediatric Surgery Children’s Hospital of Nanjing Medical UniversityNanjing210008China

Endogenous peptides,bioactive agents with a small molecular weight and outstanding absorbability,regulate various cellular processes and ***,their role in the occurrence of Hirschsprung’s disease(HSCR)remains ***,we found that the expression of an endogenous peptide derived from YBX1(termed PDYBX1 in this study)was upregulated in the aganglionic colonic tissue of HSCR patients,whereas its precursor protein YBX1 was *** shown by Transwell and cytoskeleton staining assays,silencing YBX1 inhibited the migration of enteric neural cells,and this effect was partially reversed after treatment with ***,immunoprecipitation and immunofluorescence revealed that ERK2 bound to YBX1 and *** of YBX1 blocked the ERK1/2 pathway,but upregulation of PDYBX1 counteracted this effect by binding to ERK2,thereby promoting cell migration and *** together,the endogenous peptide PDYBX1 may partially alleviate the inhibition of the ERK1/2 pathway caused by the downregulation of its precursor protein YBX1 to antagonize the impairment of enteric neural ***1 may be exploited to design a novel potential therapeutic agent for HSCR.

关键词： Hirschsprung’s disease YBX1 Endogenous peptide PDYBX1 Enteric neural cell

Epidemic management in a measles outbreak in 2023,the return of vaccine-preventable diseases:A single center,retrospective observational study

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Asian Pacific Journal of Tropical Medicine 2024年第3期17卷 110-118页

作者： Sevgi Aslan Tuncay Gulsen Akkoc Seyhan Yilmaz Burcu Parlak Pinar Canizci Erdemli Aylin Dizi Isik Didem Buyuktas Aytac Meryem Cagla Abaci Capar Eda Kepenekli Kadayifci Marmara University Faculty of Medicine Department of Pediatric Infectious DiseaseIstanbulTurkey

Objective:To investigate the importance of immunization in preventing measles infection and to determine the most useful laboratory tests for confirmation of ***:This study included pediatric cases evaluated with a presumed diagnosis of measles between December 2022 and June 2023,at Marmara University Pendik Training and Research *** effects of vaccination status and underlying disease on the clinical course,treatments,and complications were ***:In total,117 patients were enrolled in the study with a median age of 80 months(IQR:32.5-125.0).Twelve patients with contact history were asymptomatic and had an underlying disorder,and intravenous immunoglobulin was given to them for post-exposure ***-one patients had confirmed measles *** treatment was given to three patients(a newborn,a girl with rhabdomyosarcoma,and a healthy boy)with respiratory ***-eight percent of confirmed measles cases were unvaccinated,and all hospitalized cases were unvaccinated or *** full-vaccinated children had confirmed measles *** PCR from nasopharyngeal swabs was negative in all of them,and their diagnosis was established with anti-measles IgM ***:The measles vaccine is the most effective way to protect from measles and measles-related *** measles can also occur in fully vaccinated patients,the disease is milder than in unvaccinated *** ELISA and RT-PCR tests together may be beneficial in patients with high clinical suspicion for early diagnosis.

关键词： Measles Outbreak Vaccination Vitamin A Ribavirin

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

Longitudinal changes in body weight of breastfeeding mothers in the first year after delivery and its relationship with human milk composition:a combined longitudinal and cross-sectional cohort study

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Food Science and Human Wellness 2024年第1期13卷 254-264页

作者： Huijuan Ruan Yajie Zhang Qingya Tang Xuan Zhao Xuelin Zhao Yi Xiang Wei Geng Yi Feng Wei Cai department of Clinical Nutrition Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China Shanghai Key Laboratory of pediatric Gastroenterology and Nutrition Shanghai Institute of Pediatric ResearchShanghai 200092China department of pediatric Surgery Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China

Objective:Postpartum weight retention(PPWR)is a common problem among women after *** main objectives of this study are to understand the changes in body weight of breastfeeding mothers during long-term follow-up and preliminarily explore the relationship between maternal body weight and human milk composition,including macronutrients,leptin,and ***:The study included a longitudinal cohort(122 mothers),and a cross-sectional cohort(37 mothers).The human milk,maternal weight,and dietary surveys were collected in the longitudinal cohort at different follow-up time points(1-14 days postpartum,2-4 months postpartum,5-7 months postpartum,and 12-17 months postpartum).The maternal body weight was analyzed using the responses in the survey questionnaires.A milk analyzer based on the mid-infrared spectroscopy(MIRS)was used to determine milk composition,and nutrition analysis software evaluated dietary *** the cross-sectional cohort,participating mothers were asked to provide blood and human milk samples and pertinent information related to maternal body *** body composition was measured by bioelectrical impedance analysis(BIA),while ELISA analyzed leptin and adiponectin in milk and ***:At 5-7 months postpartum,the PPWR of breastfeeding mothers was(2.46±3.59)*** 12-17 months postpartum,the PPWR was(0.98±4.06)*** was found to be negatively correlated with milk fat content within 14 days postpartum and positively correlated at 2-4 months *** addition,the maternal weight and body muscle mass were positively correlated with leptin and adiponectin in *** leptin was positively correlated with the mother’s body weight,body mass index(BMI),FAT percentage,and body fat mass,while plasma adiponectin did not correlate with any *** results also indicate that the PPWR did not correlate with leptin and adiponectin in plasma or ***:Breastfeeding mothers may retain considerable weight gai

关键词： Human milk Milk composition Body weight Body mass index(BMI) Weight gain Postpartum weight retention

Primary repair of esophageal atresia Gross type C via thoracoscopic magnetic compression anastomosis:Is it the best option?

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World Journal of Gastrointestinal Surgery 2024年第5期16卷 1474-1481页

作者： Sonia Pérez-Bertólez Jorge Godoy-Lenz department of pediatric Surgery Sant Joan de Deu HospitalUniversity of BarcelonaBarcelona 08950Spain department of pediatric Surgery Clínica Alemana of SantiagoSantiago 7650568Chile

Magnetic compression anastomosis is a promising treatment option for patients with complex esophageal atresia;but,at the present time,should not be the first therapeutic option in those cases where the surgeon can per... 详细信息

关键词： Esophageal atresia Tracheoesophageal fistula Thoracoscopy Magnamosis Magnetic anastomosis

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

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