检索结果-南通市图书馆

Development and validation of a novel non-invasive test.for diagnosing nonalcoholic fatty liver disease in Chinese children

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

World Journal of Pediatrics 2024年第4期20卷 413-421页

作者： Zhe-Wen Qin Qian-Nan Ren Hong-Xi Zhang Ya-Ru Liu Ke Huang Wei Wu Guan-Ping Dong Yan Ni Jun-Fen Fu Division of Endocrinology National Clinical Research Center for Child HealthNational Children’s Regional Medical CenterThe Children’s HospitalDepartment of EndocrinologyNational Clinical Research Center for Child HealthNational Children’s Regional Medical CenterThe Children’s HospitalZhejiang University School of MedicineHangzhou310052China

With the exploding prevalence of obesity,many children are at risk of developing nonalcoholic fatty liver *** anthropometric and laboratory parameters,our st.dy aimed to develop a model to quantitatively evaluate liver fat content(LFC)in children with *** A well-characterized cohort of 181 children between 5 and 16 years of age were recruited to the st.dy in the Endocrinology Department as the derivation *** external validation cohort comprised 77 *** assessment of liver fat content was performed using proton magnetic resonance *** and laboratory metrics were measured in all subjects.B-ultrasound examination was carried out in the external validation *** Kruskal-Wallis test.spearman bivariate correlation analyses,univariable linear regressions and multivariable linear regression were used to build the optimal predictive *** The model was based on indicators including alanine aminotransferase,homeost.sis model assessment of insulin resist.nce,triglycerides,waist.circumference and Tanner *** adjust.d R^(2)of the model was 0.589,which presented high sensitivity and specificity both in internal[sensitivity of 0.824,specificity of 0.900,area under curve(AUC)of 0.900 with a 95%confidence interval:0.783–1.000]and external validation(sensitivity of 0.918 and specificity of 0.821,AUC of 0.901 with a 95%confidence interval:0.818–0.984).Conclusions Our model based on five clinical indicators was simple,non-invasive,and inexpensive;it had high sensitivity and specificity in predicting LFC in ***,it may be useful for identifying children with obesity who are at risk for developing nonalcoholic fatty liver disease.

关键词： Biochemical markers Children Magnetic resonance spectroscopy Nonalcoholic fatty liver disease Pediatric obesity

Efficacy of physiological seawater nasal irrigation for the treatment of children with sARs-CoV-2 Omicron BA.2 variant infection:a randomized controlled trial

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

World Journal of Pediatrics 2024年第5期20卷 461-469页

作者： Ji-Lei Lin Fen Zhang Yan-Bo Li shu-Hua Yuan Jin-Hong Wu Jing Zhang Lei Zhang Yi He Jie Chen Yong Yin Department of Respiratory Medicine Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong University1678 Dongfang RoadPudong DistrictShanghaiChina University of British Columbia VancouverCanada Information Technology Department Shanghai Children’s Medical Center National Children’s Medical CenterSchool of MedicineShanghai Jiao Tong University1678 Dongfang RoadPudong DistrictShanghaiChina Department of Otorhinolaryngology Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong University1678 Dongfang RoadPudong DistrictShanghaiChina Department of Respiratory Medicine Sanya Women and Children’s Hospital Affiliated to Hainan Medical CollegeHainan Branch of Shanghai Children’s Medical CenterSanyaChina Department of Respiratory Medicine Linyi Maternal and Child Healthcare HospitalLinyiChina shanghai Children’s medical center Pediatric medical Complex(Pudong) ShanghaiChina Pediatric AI Clinical Application and Research center Shanghai Children’s Medical CenterShanghaiChina

Background saline nasal irrigation is an effective therapy for relieving common cold *** st.dy aimed to invest.gate and explore the efficacy of physiological seawater nasal irrigation(PsNI)on children with mild and asymptomatic infection with *** This randomized controlled trial was conducted in shanghai,China,and 403 children with mild and asymptomatic infection with Omicron were *** children were allocated into the PsNI group and the control *** primary outcome was the duration of viral shedding(DVs),and the secondary outcome was the change in clinical *** The median age of all participants was 5.59(6.26)years *** DVs was significantly shorter in the PsNI group[2.40(1.13)]than in the control group[3.09(2.14)](P=0.014).The multivariable Cox regression model also showed that patients in the PsNI group had an increased probability of shorter DVs compared with patients in the control group[hazard ratio(HR),1.27;95%confidence interval(CI),1.04–1.55;P=0.017].subgroup analysis suggest.d that the DVs of patients without full vaccination was significantly reduced in the PsNI *** proportions of runny nose and st.ffy nose were apparently reduced in the first.three days in the PsNI group or the control group,but there was no evidence showing that PsNI contributes to the benefit compared with the control *** PsNI can reduce the DVs of patients with mild and asymptomatic infection with sARs-CoV-2 Omicron BA.2 variant.

关键词： Children Duration of viral shedding Omicron Physiological seawater nasal irrigation

Function and dysfunction of GEMIN5:underst.nding a novel neurodevelopmental disorder

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Neural Regeneration Research 2024年第11期19卷 2377-2386页

作者： Charles H.Nelson Udai B.Pandey Department of Pediatrics Division of Child NeurologyChildren’s Hospital of PittsburghUniversity of Pittsburgh Medical CenterPittsburghPAUSA Children’s Neuroscience Inst.tute Children’s Hospital of PittsburghUniversity of Pittsburgh Medical CenterPittsburghPAUSA

The recent identification of a neurodevelopmental disorder with cerebellar atrophy and motor dysfunction(NEDCAM)has resulted in an increased interest.in GEMIN5,a multifunction RNA-binding *** the largest.member of the survival motor neuron complex,GEMIN5 plays a key role in the biogenesis of small nuclear ribonucleoproteins while also exhibiting translational regulatory functions as an independent *** many quest.ons remain regarding both the pathogenesis and pathophysiology of this new disorder,considerable progress has been made in the brief time since its *** this review,we examine GEMIN5 within the context of NEDCAM,focusing on the st.ucture,function,and expression of the protein specifically in regard to the disorder ***,we explore the current animal models of NEDCAM,as well as potential molecular pathways for treatment and future directions of *** review provides a comprehensive overview of recent advances in our underst.nding of this unique member of the survival motor neuron complex.

关键词： cerebellar atrophy GEMIN5 neurodevelopmental disorder neurological disease sMN complex

Gp78 regulates PMP22 and causes ER st.ess and autophagy in EV71-VP1-overexpressing mouse schwann cells

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

BIOCELL 2024年第4期48卷 653-664页

作者： DANPING ZHU GUANGMING LIU KUAN FENG sUYUN LI DANDAN HU sIDA YANG PEIQING LI Pediatric Emergency Department Guangzhou Women and Children’s Medical CenterGuangzhou Medical UniversityGuangdong Provincial Clinical Research Center for Child HealthGuangzhouChina Department of Child Health Care Guangzhou Women and Children’s Medical CenterGuangzhou Medical UniversityGuangdong Provincial Clinical Research Center for Child HealthGuangzhouChina Pediatric Neurology Department Guangzhou Women and Children’s Medical CenterGuangzhou Medical UniversityGuangdong Provincial Clinical Research Center for Child HealthGuangzhouChina

Background:During Enterovirus type 71(EV71)infection,the st.uctural viral protein 1(VP1)activates endoplasmic reticulum(ER)st.ess associated with peripheral myelin protein 22(PMP22)accumulation and induces ***,the speciﬁc mechanism behind this process remains ***:In this research,we used the VP1-overexpressing mouse schwann cells(sCs)models co-transfected with a PMP22 silencing or Autocrine motility factor receptor(AMFR/gp78)overexpressing vector to explore the regulation of gp78 on PMP22 and its relationship with autophagy and ***:The activity of gp78 could be inﬂuenced by EV71-VP1,leading to a decrease in the ubiquitination and degradation of PMP22,resulting in PMP22 accumulation in *** VP1-overexpressing mouse sCs,all three ER st.ess sensors,including pancreatic endoplasmic reticulum kinase(PERK),activating transcription factor 6(ATF6)and inositol-requiring enzyme 1(IRE1)and the related downst.eam signals(C/EBP-homologous protein(CHOP)and Caspase 12)were activated,as well as the ER-resident chaperone Glucose-regulated protein 78(GRP78).In addition,VP1 upregulated the autophagy marker Microtubule-associated protein 1 light chain 3 beta(LC3B),while PMP22 silencing or gp78 overexpression reversed the ***,PMP22 silencing or gp78 overexpression increased proliferation of EV71-VP1-transfected mouse ***:Gp78 could regulate PMP22 accumulation through ubiquitination degradation and cause ER st.ess and autophagy in EV71-VP1-overexpressing mouse ***,the gp78/PMP22/ER st.ess axis might emerge as a promising therapeutic target for myelin and neuronal damage induced by EV71 infection.

关键词： Enterovirus type 71 AMFR/gp78 PMP22 Autophagy schwann cells

Ketogenic diet therapy in children with epilepsy caused by sLC2A1 mutations:a single-center single-arm retrospective st.dy

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

World Journal of Pediatrics 2024年第5期20卷 517-524页

作者： Ying-Yan Wang Yun-Qing Zhou Li-Juan Luo Cui-Jin Wang Nan shen Hao Li Ji-Wen Wang Department of Neurology Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina Department of Infectious Diseases Shanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina Clinical Research Ward Clinical Research CenterShanghai Children’s Medical CenterSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina

Background This retrospective st.dy assessed the efficacy and safety of ketogenic diet therapies in children with epilepsy caused by sLC2A1 genetic mutations and glucose transporter type 1 deficiency *** Pediatric patients with epilepsy symptoms admitted to our medical center between January 2017 and October 2021 were included if they presented with an sLC2A1 genetic mutation on whole-exome *** analyzed the patients’convulsions and treatment with antiepileptic *** patients were followed up at different time periods after ketogenic diet *** six patients with sLC2A1 mutations were included in this *** patients had seizures of different types and frequencies,and they took antiepileptic drugs to relieve their *** were then treated with a ketogenic diet for at least.four *** analyzed epilepsy control rates at 1,2,3,6,and 12 months after ketogenic diet *** patients were seizure-free within a month of receiving the diet *** patients were followed up for six months,three were followed up for 12 months after the treatment,and there was no recurrence of epilepsy during this *** antiepileptic drug withdrawal,none of the patients experienced seizure relapse when receiving ketogenic diet treatment *** severe adverse events occurred during the *** Ketogenic diet therapy is very effective and safe for the treatment of epilepsy caused by sLC2A1 ***,patients with glucose transporter type 1 deficiency syndrome caused by sLC2A1 mutations should begin ketogenic diet treatment as soon as possible.

关键词： Epilepsy Glucose transporter type 1 Ketogenic diet sLC2A1

Impact of pancreatic and biliary st.nt on post.endoscopic papillectomy complications: A single-center retrospective st.dy

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

中华医学杂志(英文版) 2024年第17期137卷 2111-2118页

作者： Ru Nan Chai Ningli Zhang Bo Li Longsong Bi Yawei Linghu Enqiang Department of Gast.oenterology The First Medical Center of Chinese People’s Liberation Army General Hospital Beijing China Digest.ve Endoscopy center The First Medical Center of Chinese People’s Liberation Army General Hospital Beijing China

Background: Endoscopic papillectomy (EP) is recommended as the first.line therapy for ampullary tumors, despite a relatively high incidence of complications. Pancreatic and/or biliary st.nts are placed at the endoscopist.s discretion to prevent post.EP complications. The present st.dy aimed to evaluate the efficacy of different ***: A total of 117 patients who underwent EP and met the criteria between June 2006 and October 2022 were enrolled in the st.dy. These patients were divided into a pancreatic st.nt group (Ps group,n = 47), a biliary st.nt group (Bs group,n = 38), and a two-st.nt group (PBs [Ps and Bs] group,n = 32). Relevant clinical data were collected and compared among the three groups. Multivariate logist.c analyses were performed to explore risk factors for post.EP ***: The incidence of all complications was 37.6% (44/117). Pancreatitis and hemorrhage were the two most.common complications with incidence rates of 14.5% (17/117) and 17.9% (21/117). The incidence rates of post.EP pancreatitis were 10.6% (5/47), 23.7% (9/38), and 9.4% (3/32) in the Ps group, Bs group, and PBs group, respectively, with no significant differences. There were also no significant differences in other complications among the three groups. Age (odds ratio [OR]: 0.95; 95% confidence interval [CI]: 0.91-0.99;P = 0.022) was independently associated with post.EP pancreatitis while tumor size (OR: 1.66; 95% CI: 1.06-2.60;P = 0.028) was independently associated with post.EP ***: While pancreatic st.nting is the first.choice to prevent post.EP pancreatitis, biliary st.nting could also be considered as a subst.tute for patients with difficulties in pancreatic cannulation. Two-st.nt (biliary and pancreatic st.nt) placement is unnecessary unless it is required due to other concerns.

关键词： Endoscopic papillectomy Complications Preventive measures Pancreatitis Hemorrhage

Novel de novo TRPV4 mutation identified in a Chinese family with metatropic dysplasia inhibits chondrogenic differentiation

同方期刊数据库评论

在线全文

同方期刊数据库

学校读者我要写书评

暂无评论

Genes & Diseases 2024年第3期11卷 83-86页

作者： Ping Wei Weizhe shi Tianying Nong Caixia Xian Xia Li Zhaohui Li Xin Li Jianping Wu Liyuan shang Fulong Xu Yibo Xu Hongwen Xu Mingwei Zhu Guangzhou Inst.tute of Pediatrics Guangzhou Women and Children's Medical CenterGuangzhou Medical UniversityGuangdong Provincial Clinical Research Center for Child HealthGuangzhouGuangdong 510623China Department of Pediatric Orthopedics Guangzhou Women and Children's Medical CenterGuangzhou Medical UniversityGuangdong Provincial Clinical Research Center for Child HealthGuangzhouGuangdong 510623China

Metatropicdysplasia(MD,MIM156530)is a rare congenital bone dysplasia primarily characterized by severe platyspondyly with long and wide vertebral bodies and dumbbell deformity of the tubular *** can be caused by heterozygous mutations in the gene encoding transient receptor potential vanilloid family member 4(TRPV4).We identifieda novel de novo mutation,c.2353T>C,in the TRPV4 gene,in a Chinese family with mild MD.

关键词： TRPV4 dysplasia congenital

Exploration of pathogenic microorganism within the small intest.ne of necrotizing enterocolitis

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

World Journal of Pediatrics 2024年第2期20卷 165-172页

作者： Yan Wang Kun Jiang Qiao Xia Xia Kang shan Wang Ji-Hong Yu Wen-Feng Ni Xiao-Qin Qi Ying-Na Zhang Jin-Bao Han Gang Liu Lei Hou Zhi-Chun Feng Liu-Ming Huang Clinical Biobank center Medical Innovation Research Division of ChinesePLA General HospitalBeijingChina Inst.tute of Basic medical sciences Chinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina senior Department of Pediatrics The Seventh Medical Center of PLA General HospitalBeijing 100700China Department of Neurology The First Medical CenterChinese PLA General HospitalBeijing 100853China Department of Emergency center National Center for Children's HealthBeijing Children's HospitalCapital Medical UniversityBeijing 100045China

Background Necrotizing enterocolitis(NEC)is the most.common severe gast.ointest.nal emergency in *** designed this st.dy to identify the pathogenic microorganisms of NEC in the microbiota of the small intest.ne of *** Using the 16s ribosomal DNA(rDNA)sequencing method,we compared and analyzed the st.ucture and diversity of microbiotas in the intest.nal feces of different groups of neonates:patients undergoing jejunost.my to treat NEC(NP group),neonates undergoing jejunost.my to treat other conditions(NN group),and neonates with NEC undergoing conservative treatment(NC group).We took intest.nal feces and saliva samples from patients at different time *** The beta diversities of the NP,NN,and NC groups were all *** comparing the beta diversities between different time points in the NP group,we found similar beta diversities at time points El to E3 but significant differences between the E2-E3 and E4 time points:the abundances of Klebsiella and Enterococcus(Proteobacteria)were higher at the E1-E3 time points;the abundance of Escherichia-shigella(Proteobacteria)increased at the E2 time point,and the abundance of Klebsiella decreased significantly,whereas that of st.eptococcus increased significantly at the E4 time *** Our results suggest.that the pathological changes of intest.nal necrosis in the small intest.ne of infants with NEC are not directly caused by excessive proliferation of pathogenic bacteria in the small *** sources of microbiota in the small intest.ne of neonates,especially in premature infants,may be affected by multiple factors.

关键词： Microbiota Necrotizing enterocolitis-small intest.ne 16s rDNA

Therapeutic nucleic acids in regenerative medicine and tissue repair

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Nano Research 2024年第10期17卷 8942-8976页

作者： Qimanguli saiding Duotian Qin soohwan An Dylan Neal Patel Muhammad Muzamil Khan Na Kong Wei Tao center for Nanomedicine and Department of Anest.esiology Brigham and Women’s HospitalHarvard Medical SchoolBostonMassachusetts 02115USA

In the vanguard of biomedical innovation,regenerative medicine emerges as a transformative paradigm,concentrating its efforts on rectifying tissue deficits and functional aberrations in patients through the st.ategic augmentation of endogenous cellular *** to this approach is nucleic acid therapy,which offers a novel pathway for tissue regeneration by modulating key signaling *** one of the most.effective ways to regulate cell function,nucleic acids are crucial for various tissue regeneration;however,their in vivo therapeutic applications face subst.ntial challenges,including nuclease degradation,cell membrane impermeability,and targeted intracellular ***-based gene delivery syst.ms offer a solution for st.ble and localized drug delivery by enabling the controlled overexpression of therapeutic nucleic acids,producing functional regulatory *** review presents examples of nucleic acid applications in regenerative medicine,highlighting the synergy between nucleic acids and biomaterial *** underlines the importance of nucleic acid delivery techniques,the choice of therapeutic nucleic acids,and biomaterials for advancing tissue *** latest.developments in designing nucleic acid biomaterial-based delivery vehicles are explored,the mechanism of nucleic acids in tissue regeneration is elucidated,and their limitations are discussed while considering future directions for the clinical translation of nucleic acid-based therapeutics.

关键词： tissue repair nucleic acids biomaterials delivery syst.ms regenerative medicine