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Proteomic profiling of cerebrospinal fluid in pediatric myelin oligodendrocyte glycoprotein antibody-associated disease

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World Journal of Pediatrics 2024年第3期20卷 259-271页

作者： Yi-Long Wang Meng-Ying Zhu Zhe-Feng Yuan Xiao-yan Ren Xiao-Tong Guo Yi Hua Lu Xu Cong-Ying zhao Li-Hua Jiang Xin Zhang Guo-Xia Sheng Pei-Fang Jiang zheng-yan zhao Feng Gao Department of Neurology Children's HospitalZhejiang University School of MedicineHangzhou 310052China Children's Hospital Zhejiang University School of MedicineHangzhou 310052China Children's Hospital National Clinical Research Center for Child HealthZhejiang University School of MedicineHangzhou 310052China

Background Myelin oligodendrocyte glycoprotein(MOG)antibody-associated disease(MOGAD)is an autoimmune demy-elinating disorder of the central nervous *** Extracted proteins from 34 cerebrospinal fluid(CSF)samples[patients with MOGAD(MOG group,n=12);healthy controls(HC group,n=12);patients with MOG seronegative and metagenomics next-generation sequencing-negative inflammatory neurological diseases(IND group,n=10)]were processed and subjected to label-free quantitative *** partial least squares-discriminant analysis(PLS-DA)and orthogonal PLS-DA(O-PLS-DA)models were also performed based on proteomics *** analysis of differentially expressed proteins(DEPs)was performed using Gene Ontology,InterPro,and Kyoto Encyclopedia Genes and *** enzyme-linked immunosorbent assay was used to determine the complement levels in serum from patients with *** Four hundred and twenty-nine DEPs(149 upregulated and 280 downregulated proteins)were identified in the MOG group compared to the HC group according to the P value and fold change(FC).Using the O-PLS-DA model,872 differentially abundant proteins were identified with variable importance projection(VIP)scores>*** proteins(gamma-glutamyl hydrolase,cathepsin F,interalpha-trypsin inhibitor heavy chain 5,latent transforming growth factor beta-binding protein 4 and leukocyte-associated immunoglobulin-like receptor 1)overlapping between the top 30 DEPs with top-ranked P value and FC and top 30 proteins in PLS-DA VIP lists were *** analysis revealed that the dysregulated proteins in the MOG group were primarily involved in complement and coagulation cascades,cell adhesion,axon guidance,and glycosphingolipid biosynthesis compared to the HC *** The proteomic alterations in CSF samples from children with MOGAD identified in the current study might provide opportunities for developing novel biomarker candidates.

关键词： Acute disseminated encephalomyelitis Cerebrospinal fluid Complement cascades Myelin oligodendrocyte glycoprotein-associated disease Proteomics

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Secondary genomic findings in the 2020 China Neonatal Genomes Project participants

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World Journal of Pediatrics 2022年第10期18卷 687-694页

作者： Hui Xiao Jian-Tao Zhang Xin-Ran Dong Yu-Lan Lu Bing-Bing Wu Hui-Jun Wang zheng-yan zhao Lin yang Wen-Hao Zhou Department of Neonatology Children’s Hospital of Fudan UniversityShanghaiChina Center for Molecular Medicine Children’s Hospital of Fudan UniversityShanghaiChina Department of Child Health Care Children’s HospitalZhejiang University School of MedicineHangzhouChina Department of Pediatric Endocrinology and Inherited Metabolic Diseases Children’s Hospital of Fudan UniversityShanghaiChina

Background During next generation sequencing(NGS)data interpretation in critically ill newborns,there is a potential for recognizing and reporting secondary findings(SFs).Early awareness of SFs may provide clues for disease *** this study,we assessed the frequency of SFs in the China Neonatal Genomes Project(CNGP)*** A total of 2020 clinical exome sequencing(CES)datasets were screened for variants from a list of 59 genes recommended by the American College of Medical Genetics and Genomics(ACMG)for secondary findings reporting v2.0(ACMG SF v2.0).Identified variants were classified according to the evidence-based guidelines reached by a joint consensus of the ACMG and the Association for Molecular Pathology(AMP).Results Among the 2020 CES datasets,we identified 23 ACMG-reportable genes in 61 individuals,resulting in an overall frequency of SFs at 3.02%.A total of 53 unique variants were identified,including 35 pathogenic and 18 likely pathogenic *** common disease categories of SFs associated were cardiovascular and cancer *** SF results affected the medical management and follow-up strategy in 49(80.3%)*** We presented the frequency of SFs and their impact on clinical management strategies in CNGP *** study demonstrated that SFs have important practical value in disease prevention and intervention at an early stage.

关键词： Genetics secondary findings Neonate Next generation sequencing

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Establishment of an efficient reverse genetic system of Mumps virus S79 from cloned DNA

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World Journal of Pediatrics 2019年第5期15卷 499-505页

作者： Duo Zhou Meng-Ying Zhu Yi-Long Wang Xiao-Qiang Hao Dong-Ming Zhou Rong-Xian Liu Chu-Di Zhang Chu-Fan Qu zheng-yan zhao Zhejiang University School of Medicine HangzhouChina Children's Hospital Zhejiang University School of MedicineHangzhou 310052China Department of Neurology Children's HospitalZhejiang University School of MedicineHangzhou 310052China

Background Mumps is a common type of respiratory infectious disease caused by mumps virus(MuV),and can be effectively prevented by *** this study,a reverse genetic system of MuV that can facilitate the rational design of safer,more efficient mumps vaccine candidates is *** MuV-S79 cDNA clone was assembled into a full-length plasmid by means of the GeneArtTM High-Order Genetic Assembly System,and was rescued via reverse genetic ***-PCR,sequencing,and immunofluorescence assays were used for rMuV-S79 *** replication kinetics and in vivo experimental models were used to evaluate the replication,safety,and immunogenicity of *** A full-length cDNA clone of MuV-S79 in the assembly process was generated by a novel plasmid assemble strategy,and a robust reverse genetic system of MuV-S79 was successfully *** established rMuV-S79 strain could reach a high virus titer in *** average viral titer of rMuV-S79 in the lung tissues was 2.68±0.14 log10PFU/g lung tissue,and rMuV-S79 group did not induce inflammation in the lung tissues in cotton *** antibody titers induced by rMuV-S79 were high,long-lasting and could provide complete protection against MuV wild strain *** We have established a robust reverse genetic system of MuV-S79 which can facilitate the optimization of mumps ***-S79 rescued could reach a high virus titer and the safety was proven in *** could also provide complete protection against MuV wild strain challenge.

关键词： Mumps virus Reverse genetics Plasmid Safety Immunogenicity

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Potential Association of Lead Exposure During Early Development of Mice With Alteration of Hippocampus Nitric Oxide Levels and Learning Memory

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Biomedical and Environmental Sciences 2005年第6期18卷 375-378页

作者： LI SUN zheng-yan zhao JIAN HU XIE-LAI ZHOU The Affiliated Children Hospital of Medical School Zhejiang University Hangzhou 310003 Zhejiang China The First Affiliated Hospital of Medical School Zhejiang University Hangzhou 310003 Zhejiang China Medical School Hangzhou Normal College No. 30 Wenhua Road Hangzhou 310012 Zhejiang China

Objective Chronic lead （Pb） exposure during development is known to produce learning deficits. Nitric oxide participates in the synaptic mechanisms involved in certain forms of learning and memory. This study was designed to clarify whether Pb-induced impairment in learning and memory was associated with the changes of nitric oxide levels in mice brains. Methods Sixty Balb/c mice aged l0 days were chosen. A model of lead exposure was established by drinking 0.025%, 0.05% 0.075% lead acetate, respectively for 8 weeks. The controls were orally given distilled water. The ability to learn and memorize was examined by open field test, T-water maze test. In parallel with the behavioral data, NO level of hippocampus tissue was detected by biochemical assay. Results Compared with control groups, （1） the weight of 0.075% group was significantly reduced （P〈0.05）; （2） The number of times in mice attaining the required standards in T-water maze test was lower in 0.075% group （P〈0.01）. No significant difference was found between experimental and control groups in open field test （P〉0.05）; （3） NO level of mouse hippocampus tissue was decreased in 0.075% group （P〈0.01）. Conclusions The findings suggest that decreased hippocampus NO level may contribute to the Pb-induced deficits in learning and memory processes.

关键词： Lead exposure Hippocampus Learning and memory Nitric oxide (NO)

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Attenuated MuV-S79 as vector stably expressing foreign gene

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World Journal of Pediatrics 2019年第5期15卷 511-515页

作者： Duo Zhou Meng-Ying Zhu Yi-Long Wang Xiao-Qiang Hao Dong-Ming Zhou Rong-Xian Liu Chu-Di Zhang Chu-Fan Qu zheng-yan zhao Zhejiang University School of Medicine HangzhouZhejiangChina Children's Hospital Zhejiang University School of MedicineHangzhou 310052China Department of Neurology Children's HospitalZhejiang University School of MedicineHangzhou 310052China

Background To describe mumps virus(MuV)used as a vector to express enhanced green fluorescent protein(EGFP)or red fluorescent protein(RFP)*** Molecular cloning technique was applied to establish the cDNA clones of recombinant mumps viruses(rMuVs).rMuVs were recovered based on our reverse genetic system of *** properties of rMuVs were determined by growth curve,plaque assay,fluorescent microscopy and determination of fluorescent *** Three recombinant viruses replicated well in Vero cells and similarly as parental rMuV-S79,expressed heterologous genes in high levels,and were genetically stable in at least 15 *** rMuV-S79 is a promising platform to accommodate foreign genes like marker genes,other antigens and immunomodulators for addressing various diseases.

关键词： Mumps virus Reverse genetic system Foreign genes

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Distribution of multiple chalazia in eyelids of pediatrics requiring surgery in southeast China:a hospital-based cross-sectional study

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World Journal of Pediatric Surgery 2022年第3期5卷 147-153页

作者： Xiao-Yu zheng Robert M Dorazio Bonnie Nga Kwan Choy Ting-yan Wang Su-Juan zhao zheng-yan zhao Department of Ophthalmology Children's HospitalZhejiang University School of MedicineHangzhouChina Children's Hospital Zhejiang University School of MedicineHangzhouChina Department of Ophthalmology The University of Hong KongHong KongChina Department of Child Health Care Children's HospitalZhejiang University School of MedicineHangzhouChina

Background Multiple chalazia are common in children,and many are treated by ***,the distribution of different types of multiple chalazia has not been *** research aimed to investigate the location and number of multiple chalazia in pediatrics who need surgical *** Patients with multiple chalazia treated by incision and curettage surgery(I&C)in a tertiary children’s hospital between June and December 2016 were *** data,locations,and numbers of chalazia were *** were analyzed using generalized linear models of the counts and the occurrences of *** were tested using likelihood ratio tests appropriate for each type of *** The study included 128 subjects,most of which were 1-3 years *** majority of patients had bilateral chalazia(95.3%),and the proportions of patients with internal,external,and marginal chalazion differed dramatically(99.2%,61.7%,and 2.3%,respectively).The number of internal and external chalazia did not vary significantly with gender,age,or residence of the *** chalazia were located more frequently in the upper lids(p<0.001).External chalazia showed no preference of *** average number of internal chalazia in each eyelid did not relate to the presence of external *** Multiple chalazia are common among younger children in southeast *** anatomical distribution varies depending on the type of *** chalazia often occur bilaterally and *** doctors are more aware of the anatomical distribution of chalazia,this might result in a higher success rate of I&C.

关键词： surgery southeast distribution

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Transoesophageal emergency ventricular pacing with esophageal balloon electrode catheter

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Journal of Geriatric Cardiology 2008年第3期5卷 142-145页

作者： Fangsheng zheng Rongqiang yan Hairong zhao Luxia Mao Department of Cardiology, Affiliated Hospital of Qingdao University Medical College, Qingdao Shandong 266003, China Department of Cardiology Affiliated Hospital of Qingdao University Medical College

Objective To evaluate the safety and possibility of transesophageal emergency ventricular pacing with esophageal balloonelectrode *** Sixty four patients with critical bradyarrhythmias were divided randomly to receive either transesophagealventricular pacing with esophageal balloon electrode catheter(*** group,n=32),or transesophageai ventricular pacing withnormal esophageal electrode catheter(normal catheter group,n=32).Pulse width(ms),current output(mA),and the success rate werecompared between the 2 *** Success rate in the balloon catheter group was 87.5%,which was significantly higher thanthat in the nodal catheter group(18.7%,X2=27.7,P＜0.01).The success rate oftransesophageal ventricular pacing with esophagealballoon electrode catheter was 87.5%.The average current output(mA)was significantly lower in the balloon catheter group than thatin the normal catheter group (24.5+2.5 mA VS 53±3.4mA,P＜0.05).Conclusions Esophageal balloon electrode catheter pacing couldbecome an available teclmiqBe for transesophageal emergency ventricular pacing.

关键词： ventriculax pacing balloon elcctrode catheter emergency.

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Application of next generation sequencing in the screening of monogenic diseases in China,2021:a consensus among Chinese newborn screening experts

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World Journal of Pediatrics 2022年第4期18卷 235-242页

作者： Fan Tong Jian Wang Rui Xiao Bing-Bing Wu Chao-Chun Zou Ding-Wen Wu Hua Wang Hui Zou Lian-Shu Han Lin yang Lin Zou Ming-yan Hei Ru-Lai yang Tian-Ming Yuan Wei Wen Xin-Wen Huang Xue-Fan Gu yan-Ling yang Yong-Lan Huang Yong-Jun Zhang Yong-Guo Yu zheng-Feng Xu Wen-Hao Zhou zheng-yan zhao Children’s Hospital Zhejiang University School of MedicineNational Clinical Research Center for Child Health3333 Binsheng RoadBinjiang DistrictHangzhou 310052China Shanghai Children’s Medical Center Shanghai Jiaotong University School of MedicineShanghaiChina National Engineering Laboratory for Key Technology of Birth Defect Control and Prevention Screening and Diagnostic R and D CenterHangzhouChina Center for Molecular Medicine Children’s Hospital of Fudan University399 Wan Yuan RoadMin Xing DistrictShanghai 200000China National Health Commission Key Laboratory for Birth Defect Research and Prevention Maternal and Child Health Hospital of Hunan ProvinceChangshaChina Jinan Maternity and Child Care Hospital Affiliated To Shandong First Medical University JinanChina Xinhua Hospital Shanghai Institute for Pediatric ResearchShanghai Jiao Tong University School of MedicineShanghaiChina Clinical Genetic Center Children’s Hospital of Fudan UniversityShanghaiChina Children’s Hospital Shanghai Jiaotong UniversityShanghaiChina Neonatal Center Beijing Children’s HospitalCapital Medical UniversityBeijingChina Affiliated Shenzhen Maternity and Child Healthcare Hospital Southern Medical UniversityShenzhenChina Xinhua Hospital School of MedicineShanghai Jiaotong UniversityShanghaiChina Peking University First Hospital BeijingChina Guangzhou Women and Children’s Medical Center GuangzhouChina Center of Genetic Medicine Women’s Hospital of Nanjing Medical UniversityNanjing Maternity and Child Health Care HospitalNanjingChina

Newborn screening(NBS)refers to a maternal and newborn healthcare technology,in which special examinations of congenital and genetic diseases that could seriously impact the health of newborns,are implemented during the neonatal period to provide early diagnosis and treatment[1].With a history of more than 60 years,NBS has advanced greatly due to technological progress resulting in significant improvement in the number of diseases covered by NBS and in screening efficiency[2-7].

关键词： diseases diagnosis congenital

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A multicenter prospective study of next-generation sequencing-based newborn screening for monogenic genetic diseases in China

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World Journal of Pediatrics 2023年第7期19卷 663-673页

作者： Ru-Lai yang Gu-Ling Qian Ding-Wen Wu Jing-Kun Miao Xue yang Ben-Qing Wu Ya-Qiong yan Hai-Bo Li Xin-Mei Mao Jun He Huan Shen Hui Zou Shu-Yuan Xue Xiao-Ze Li Ting-Ting Niu Rui Xiao zheng-yan zhao National Clinical Research Center for Child Health Children's HospitalZhejiang University School of MedicineHangzhouChina Chongqing Health Center for Women and Children Neonatal Screening CenterChongqingChina Guiyang Maternal and Child Health Hospital GuiyangChina University of the Chinese Academy of Science Shenzhen HospitalShenzhen 518000GuangdongChina Shanxi Children's Hospital Shanxi Maternal and Child Health Hospital TaiyuanShanxiChina The Central Laboratory of Birth Defects Prevention and Control Ningbo Women and Children's HospitalNingbo 315012ZhejiangChina Maternal and Child Health Hospital of Ningxia Hui Autonomous Region YinchuanChina Changsha Maternal and Child Health Hospital ChangshaHunanChina Yunnan Maternal and Child Health Hospital KunmingYunanChina Jinan Maternity and Child Care Hospital Affiliated to Shandong First Medical University JinanShandongChina Urumqi Maternal and Child Health Care Hospital Xinjiang Uygur Autonomous RegionUrumqi CityChina Medical Genetic Center Changzhi Maternal and Child Health Care HospitalChangzhiShanxiChina Maternal and Child Health Care Hospital of Shandong Province JinanShandongChina National Engineering Laboratory for Key Technology of Birth Defect Control and Prevention Screening and Diagnostic R and D CenterHangzhouChina

Background Newborn screening(NBS)is an important and successful public health program that helps improve the long-term clinical outcomes of newborns by providing early diagnosis and treatment of certain inborn *** develop-ment of next-generation sequencing(NGS)technology provides new opportunities to expand current newborn screening *** We designed a a newborn genetic screening(NBGS)panel targeting 135 genes associated with 75 inborn disorders by multiplex PCR combined with *** this panel,a large-scale,multicenter,prospective multidisease analysis was conducted on dried blood spot(DBS)profiles from 21,442 neonates *** We presented the positive detection rate and carrier frequency of diseases and related variants in different regions;and 168(0.78%)positive cases were ***-6-Phosphate Dehydrogenase deficiency(G6PDD)and phenylketonuria(PKU)had higher prevalence rates,which were significantly different in different *** positive detection of G6PD variants was quite common in south China,whereas PAH variants were most commonly identified in north *** addi-tion,NBGS identified 3 cases with DUOX2 variants and one with SLC25A13 variants,which were normal in conventional NBS,but were confirmed later as abnormal in repeated biochemical testing after *** percent of high-frequency gene carriers and 60%of high-frequency variant carriers had obvious regional *** the premise that there was no significant difference in birth weight and gestational age,the biochemical indicators of SLC22A5 c.1400C>G and ACADSB c.1165A>G carriers were significantly different from those of *** We demonstrated that NBGS is an effective strategy to identify neonates affected with treatable diseases as a supplement to current NBS *** data also showed that the prevalence of diseases has significant regional charac-teristics,which provides a theoretical basis for screening diseases in di

关键词： Monogenic genetic diseases Newborn screening Next-generation sequencing Monogenic genetic diseases Regional features

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Newborn screening for Duchenne muscular dystrophy in China: follow-up diagnosis and subsequent treatment

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World Journal of Pediatrics 2017年第3期13卷 197-201页

作者： Qing Ke zheng-yan zhao Robert Griggs Veronica Wiley Anne Connolly Jennifer Kwon Ming Qi Daniel Sheehan Emma Ciafaloni R Rodney Howell Petra Furu Peter Sazani Arvind Narayana Michele Gatheridge Department of Neurology The First Affiliated HospitalZhejiang University School of MedicineHangzhouChina Department of Child Health Care Children's HospitalZhejiang University School of MedicineHangzhouChina Department of Neurology University of Rochester School of Medicine and DentistryRochesterNew YorkUSA Department of Pediatrics University of Rochester School of Medicine and DentistryRochesterNew YorkUSA Disciplines of Genetic Medicine and Pediatric and Child Health University of SydneyAustralia Departments of Neurology and Pediatrics Washington University School of MedicineSt.LouisMissouriUSA Center for Genetic&Genomic Medicine Zhejiang University School of Medicine and James Watson Institute of Genome SciencesHangzhouChina Department of Pediatrics University of BuffaloBuffaloNew YorkUSA University of Miami MiamiFloridaUSA Neonatal Screening PerkinElmerTurkuFinland Field Medical Affairs Marathon PharmaceuticalsNorthbrookIllinoisUSA Medical Affairs Sarepta TherapeuticsCambridgeMassachusettsUSA

Background:Newborn screening for Duchenne muscular dystrophy (DMD) is currently being initiated in Zhejiang Province,China and is under consideration in other countries,including the United *** China begins to implement DMD newborn screening (DMD-NBS),there is ongoing discussion regarding the steps forward for follow up care of positively identified patients as well as false positive and false negative *** sources:Relevant papers related to DMD-NBS,and NBS in China were reviewed in ***:The current state of DMD-NBS is discussed,along with the steps needed to effectively screen infants for this disease in China,recommendations for establishment of follow up care in patients with positive and negative screens,and measurement of patient ***:Zhejiang Province,China is ready to implement *** challenges that exist for this program,and other countries,include the ability to track patients,assist with access to care,and ensure adequate follow-up care according to evidence-based *** addition,China's large rural population,lack of specialty providers,and difficulty in educating patients regarding the benefits of treatment create challenges that will need to be addressed.

关键词： Duchene muscular dystrophy neurology neuromuscular disorders newborn screening

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