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Risk factors for renal outcomes in children with antineutrophil cytoplasmic antibody-associated vasculitis:a nationwide retrospective study in China

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World Journal of Pediatrics 2024年第5期20卷 506-516页

作者： Li-Wen Tan Jun-Li Wan Chun-Hua Zhu Hong Xu Zheng-Kun Xia Li-Zhi Chen Xiao-Chuan Wu Fang Wang Xiao-Rong Liu Cheng-Guang Zhao Xiao-Zhong Li Jian-Hua Mao Xiao-Wen Wang Wen-Yan Huang Yu-Hong Li Jian-Jiang Zhang shi-Pin Feng Jun Yang Jiao-Jiao Liu Chun-Lin Gao Li-Ping Rong Lan-Jun shuai Ke Xu He-Jia Zhang Qiu Li Ai-Hua Zhang Mo Wang Department of Nephrology Children’s Hospital of Chongqing Medical UniversityZhongshan 2nd Rd.136Chongqing400014China Ministry of Education Key Laboratory of Child Development and Disorders National Clinical Research Center for Child Health and DisordersChina International Science and Technology Cooperation Base of Child Development and Critical DisordersChildren’s Hospital of Chongqing Medical UniversityChongqingChina chongqing Key Laboratory of Pediatrics Children’s Hospital of Chongqing Medical UniversityChongqingChina Department of Nephrology Children’s Hospital of Nanjing Medical University72 Guangzhou RoadNanjing210008China Department of Nephrology Children’s Hospital of Fudan UniversityNational Paediatric Medical Center of ChinaShanghaiChina Department of Pediatrics Jinling HospitalNanjing Medical UniversityNanjingChina Department of Pediatrics Jinling HospitalThe First School of Clinical MedicineSouthern Medical UniversityNanjingChina Department of Pediatrics Jinling HospitalMedical School of Nanjing UniversityNanjingChina Department of Pediatric Nephrology and Rheumatology The First Affiliated Hospital of Sun Yat-Sen UniversityGuangzhouChina Department of Pediatrics The Second Xiangya Hospital of Central South UniversityChangshaChina Department of Pediatrics Peking University First HospitalBeijingChina Department of Nephrology Beijing Children’s HospitalCapital Medical UniversityBeijing100045China National Center for children’s Health BeijingChina Department of Pediatrics Shengjing Hospital of China Medical UniversityShenyangChina Department of Nephrology and Immunology Children’s Hospital of Soochow UniversitySuzhouChina Department of Nephrology Children HospitalZhejiang University School of MedicineHangzhouChina Department of Nephrology Wuhan Children’s Hospital(Wuhan Maternal and Child Healthcare Hospital)Tongji Medical CollegeHuazhong University of Science&TechnologyWuhanChina Department of Nephrology and Rheumatology Shanghai Children’s HospitalSchool

Background Pediatric antineutrophil cytoplasmic antibody-associated vasculitis(AAV)is a life-threatening systemic vasculitis featured by liability to renal ***,there are few studies on the risk factors and predictive models for renal outcomes of AAV in *** Data from 179 AAV children in multiple centers between January 2012 and March 2020 were collected *** risk factors and predictive model of end-stage renal disease(EsRD)in AAV were *** Renal involvement was the most typical manifestation(95.5%),and the crescent was the predominant pathological lesion(84.9%).The estimated glomerular filtration rate(eGFR)was evaluated in 114 patients,of whom 59.6%developed EsRD,and the median time to EsRD was 3.20 *** eGFR[P=0.006,odds ratio(OR)=0.955,95%confidence interval(CI)=0.924–0.987]and the percentages of global glomerulosclerosis(pGGs;P=0.018,OR=1.060,95%CI=1.010–1.112)were independent risk factors for EsRD of renal *** on the pGGs and eGFR at renal biopsy,we developed three risk grades of EsRD and one predictive *** Kaplan‒Meier curve indicated that renal outcomes were significantly different in different risk grades(Pserum creatinine at baseline,the predictive model had higher accuracy(0.86 versus 0.58,Psus 0.92)in external *** Renal involvement is the most common manifestation of pediatric AAV in China,of which more than half deteriorates into *** predictive model based on eGFR at renal biopsy and the pGGs may be stable and accurate in speculating the risk of EsRD in AAV children.

关键词： Antineutrophil cytoplasmic antibody End-stage renal disease Glomerulonephritis Pediatric nephrology Vasculitis

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Integrated neural tracing and in-situ barcoded sequencing reveals the logic of sCN efferent circuits in regulating circadian behaviors

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science China(Life sciences) 2024年第3期67卷 518-528页

作者： Meimei Liao Xinwei Gao Chen Chen Qi Li Qingchun Guo He Huang Erquan Zhang Dapeng Ju College of Biological sciences China Agriculture UniversityBeijing 100193China National Institute of Biological sciences(NIBs) Beijing 102206China Chinese Institute for Brain Research Beijing 102206China school of Biomedical Engineering Capital Medical UniversityBeijing 100069China Department of Anesthesiology The Second Affiliated HospitalChongqing Medical UniversityChongqing 401336China Tsinghua Institute of Multidisciplinary Biomedical Research Tsinghua UniversityBeijing 102206China

The circadian clock coordinates rhythms in numerous physiological processes to maintain organismal homeostasis. since the suprachiasmatic nucleus(sCN) is widely accepted as the circadian pacemaker, it is critical to understand the neural mechanisms by which rhythmic information is transferred from the sCN to peripheral clocks. Here, we present the first comprehensive map of sCN efferent connections and suggest a molecular logic underlying these projections. The sCN projects broadly to most major regions of the brain, rather than solely to the hypothalamus and thalamus. The efferent projections from different subtypes of sCN neurons vary in distance and intensity, and blocking synaptic transmission of these circuits affects circadian rhythms in locomotion and feeding to different extents. We also developed a barcoding system to integrate retrograde tracing with in-situ sequencing, allowing us to link circuit anatomy and spatial patterns of gene expression. Analyses using this system revealed that brain regions functioning downstream of the sCN receive input from multiple neuropeptidergic cell types within the sCN, and that individual sCN neurons generally project to a single downstream brain *** map of sCN efferent connections provides a critical foundation for future investigations into the neural circuits underlying sCNmediated rhythms in physiology. Further, our new barcoded tracing method provides a tool for revealing the molecular logic of neuronal circuits within heterogeneous brain regions.

关键词： circadian rhythms sCN output circuit neural tracing barcoded GFP in situ sequencing

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The structure of TRAF7 coiled-coil trimer provides insight into its function in zebrafish embryonic development

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Journal of Molecular Cell Biology 2024年第1期16卷 43-53页

作者： Xiaozhen song Ruixing Hu Yi Chen Man Xiao Hong Zhang shengnan Wu Qing Lu Molecular Diagnostic Laboratory Shanghai Children’s HospitalSchool of MedicineShanghai Jiao Tong UniversityShanghai 200040China Bio-X Institutes Key Laboratory for the Genetics of Developmental and Neuropsychiatric Disorders(Ministry of Education)Shanghai Jiao Tong UniversityShanghai 200030China Laboratory of Development and Diseases and state Key Laboratory for medical Genomics Shanghai Institute of HematologyRuĳin HospitalShanghai JiaoTong University School of MedicineShanghai 200025China

TRAF7 serves as a crucial intracellular adaptor and E3 ubiquitin ligase involved in signal transduction pathways,contributing to immune responses,tumor progression,and embryonic *** mutations within the coiled-coil(CC)domain and WD40 repeat domain of TRAF7 could cause brain tumors,while germline pathogenic mutations contribute to severe developmental ***,the precise molecular mechanism underlying TRAF7 involvement in embryonic development remains *** this study,we employed zebrafish as an in vivo model ***7 knock down caused defects in zebrafish embryonic *** determined the crystal structure of TRAF7 CC domain at 3.3Åresolution and found that the CC region trimerization was essential for TRAF7 functionality during zebrafish embryonic ***,disease-causing mutations in TRAF7 CC region could impair the trimer formation,consequently impacting early embryonic development of ***,our study sheds light on the molecular mechanism of TRAF7 CC trimer formation and its pivotal role in embryonic development.

关键词： TRAF7 coiled-coil domain crystal structure trimerization embryonic development

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Effect of nourishing Yin and purging fire Chinese herbal mixture on delaying light-induced premature puberty in rats

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Journal of Traditional Chinese Medicine 2024年第1期44卷 95-102页

作者： sUN Yanyan XUE Yuanyuan sUN Wen WANG Yonghong YU Jian Department of Traditional Chinese Medicine Children's Hospital of Fudan UniversityShanghai 201102China Department of Pediatrics Zibo Central HospitalZibo 255000China

OBJECTIVE:To elucidate the mechanism of the nourishing Yin and purging fire Chinese herbal mixture(NYPF)in delaying light-induced premature puberty in ***:Twenty-one days old female spragueDawley rats were randomly assigned to normal group(N),long light exposure group(L),NYPF and normal saline group(Ns).Rats in the L,NYPF and Ns groups were exposed to 16 h:350 lux light/8 h:dark,while rats in the N group were exposed to 12 h:50 lux light/12 h:*** and normal saline was administered to the rats in the NYPF group or Ns group,respectively,from day *** rats in every group were sacrificed at 9 *** day 28(P28),on the day when rat's vulva opened in the L group(L-VO),on the day when the first estrous interphase occurred in rats of L group(L-E1),and on the day when the second estrous interphase occurred in rats of L group(L-E2),***:On day 34,all rats in the L group,80%of rats in the Ns group,40%of rats in the N group,and 20%of rats in the NYPF group showed complete opening of the *** P28,m RNA level of hypothalamic kisspeptin(Kiss-1)in the L group was significantly higher than that in the N group(Ps in the L and Ns groups had significantly lower hypothalamic argininephenylalanine-amide(RFamide)-related peptide 3(RFRP-3)m RNA levels than those in the N group(Ps RFRP-3 m RNA level was significantly higher in the NYPF group than that in the L group(Ps groups was significantly higher than that of the N group(Pstradiol(E2)level of the NYPF group was significantly lower than that of the N and Ns groups(Pss-1 m RNA level in the L and Ns groups was significantly higher than that in the N and NYPF groups(Ps hypothalamic RFRP-3 m RNA level in the L,NYPF,and Ns groups was significantly lower than that in the N group(Ps groups was significantly higher than that of the N group(P<0.01),wher

关键词： light puberty precocious kisspeptins RFRP-3 melatonin Nourishing Yin and Purging Fire

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PACs gene family-related neurological diseases: limited genotypes and diverse phenotypes

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World Journal of Pediatrics 2024年第1期20卷 82-91页

作者： Han Zhang Kai Gao shuang Wang Yue-Hua Zhang Zhi-Xian Yang Ye Wu Yu-Wu Jiang Department of Pediatrics Peking University First Hospital.No.1 Xi'an Men StreetWest DistrictBeijing 100034China Bejing Key Laboratory of Molecular Diagnosis and study on Pediatric Genetic Diseases BeijingChina children Epilepsy Center Peking University First HospitalBeijingChina Key Laboratory for Neuroscience Ministry of Education/National Health and Family Planning CommissionPeking UniversityBeijingChina Center of Epilepsy Beijing Institute for Brain DisordersBeijingChina

Background The PACs gene family has been demonstrated to be related to intracellular vesicular *** phenotypic manifestations caused by the pathogenic variants of PACs include epilepsy,intellectual disability/developmental delay,and malformations,such as facial *** We identified seven new cases with pathogenic or likely pathogenic PACs variants using next-generation *** information obtained from these patients was analyzed along with that obtained from previously reported *** With the inclusion of the newly diagnosed cases in this study,103 cases with PACs gene family-related neurological diseases were reported,of which 43 were PACs2-related cases and the remaining were PACsI-related *** patients had seizures,which have been reported to be effectively controlled by several types of anti-seizure medications(AsMs).The most efficacious and frequently prescribed AsMs included sodium valproate(43.3%,13/30),oxcarbazepine/carbamazepine(26.7%,8/30),and levetiracetam(20%,6/30).Almost all patients had intellectual disability/developmental *** most common pathogenic missense variants were PACsI ***203Trp and PACs2 *** addition,we report a patient carrying a likely pathogenic copy number variation(CNV)(de novo heterozygous deletion of chr14:105821380-106107443,286 kilobase,destroyed part of the furin-binding region domain and the protein structure after it)with more severe and refractory late-onset *** The clinical phenotypes of the different PACs heterozygous missense variants were *** pathogenic variant sites of PACsI and PACs2 were quite limited but located in different regions.A CNV destroying part of the PACs2 gene might also be *** findings may provide an important clue for further functional studies on the pathogenic mechanism of neurological disorders related to the PACs gene family.

关键词： Developmental delay Epilepsy Intellectual disability PACsI PACs2

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Metformin promotes anti-tumor immunity in sTK11 mutant NsCLC through AXIN1-dependent upregulation of multiple nucleotide metabolites

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Oncology Research 2024年第10期32卷 1637-1648页

作者： ZHIGUO WANG KUNLIN LI CONGHUA LU MINGXIA FENG CAIYU LIN GUOFANG YIN DAN LUO WENYI LIU KAIYU JIN YUANYAO DOU DI WU JIE ZHENG KEJUN ZHANG LI LI XIANMING FAN Department of Respiratory and Critical Care Medicine Affiliated Hospital of Southwest Medical UniversityLuzhou646000China Department of Respiratory Disease Daping HospitalArmy Medical University(Third Military Medical University)Chongqing400042China Department of Trauma medical Center Daping HospitalState Key Laboratory of TraumaBurns and Combined InjuryArmy Medical UniversityChongqing400042China Department of Respiratory Disease People’s Hospital of Xuyong CountyLuzhou646000China Department of Outpatients Daping HospitalArmy Medical University(Third Military Medical University)Chongqing400042China

Background:Metformin has pleiotropic effects beyond glucose reduction,including tumor inhibition and immune *** enhanced the anti-tumor effects of programmed cell death protein 1(PD-1)inhibitors in serine/threonine kinase 11(sTK11)mutant non-small cell lung cancer(NsCLC)through an axis inhibition protein 1(AXIN1)-dependent ***,the alterations of tumor metabolism and metabolites upon metformin administration remain ***:We performed untargeted metabolomics using liquid chromatography(LC)-mass spectrometry(Ms)/Ms system and conducted cell experiments to verify the results of bioinformatics ***:According to the Kyoto Encyclopedia of Genes and Genomes(KEGG)pathway database,most metabolites were annotated into metabolism,including nucleotide ***,the differentially expressed metabolites in H460(refers to H460 cells),H460_met(refers to metformin-treated H460 cells),and H460_KO_met(refers to metformin-treated Axin1-/-H460 cells)were distributed into six clusters based on expression *** clusters with a reversed expression pattern upon metformin treatment were selected for further *** screened out metabolic pathways through KEGG pathway enrichment analysis and found that multiple nucleotide metabolites enriched in this pathway were ***,these metabolites enhanced the cytotoxicity of activated T cells on H460 cells in vitro and can activate the stimulator of the interferon genes(sTING)pathway independently of ***:Relying on AXIN1,metformin upregulated multiple nucleotide metabolites which promoted sTING signaling and the killing of activated T cells in sTK11 mutant NsCLC,indicating a potential immunotherapeutic strategy for sTK11 mutant NsCLC.

关键词： Metformin serine/threonine kinase 11(sTK11) Lung cancer Axis inhibition protein 1(AXIN1) Nucleotide metabolites

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Comprehensive genome sequencing analyses identify novel gene mutations and copy number variations associated with infant developmental delay or intellectual disability(DD/ID)

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Genes & Diseases 2022年第5期9卷 1166-1169页

作者： Yuxia Chen Xiang Tang Ling Liu Qinrong Huang Li Lin Guoing Liu Nong Xiao Department of children Rehabilitation Children's Hospital of Chongqing Medical UniversityMinistry of Education Key Laboratory of Child Development and DisordersNational Clinical Research Center for Child Health and DisordersChina International Science and Technology Cooperation Base of Child Development and Critical DisordersChildren's Hospital of Chongqing Medical UniversityChongqing Key Laboratory of Translational Medical Research in Cognitive Development and Learning and Memory DisordersChongqing 400014PR China

To the Editor,Developmental delay or intellectual disability(DD/ID)is one of the most common neurodevelopmental disabilities worldwide with high clinical and genetic *** etiology remains unexplained in nearly 70%of these patients,and an accurate diagnosis still poses a challenge in clinical *** DD/ID cohort studies mostly used panel sequencing or chromosome microarray analysis(CMA),but targeted capture probes could not be updated in time,resulting in an increased risk of missed detection of genetic abnormalities.

关键词： diagnosis clinical infant

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Clinical Characteristics of Human Adenovirus Plastic Bronchitis in 10 Pediatric Cases: A Retrospective study of seven Years

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Virologica sinica 2021年第3期36卷 550-554页

作者： Lingjian Zeng Jianhua Wei Yuyi Tang Enmei Liu Qubei Li Na Zang Department of Respiratory Medicine Ministry of Education Key Laboratory of Child Development and DisordersNational Clinical Research Center for Child Health and DisordersChina International Science and Technology Cooperation Base of Child Development and Critical DisorderChildren’s Hospital of Chongqing Medical UniversityChongqing Key Laboratory of PediatricsChongqing 400014China

Dear Editors,In recent years,human adenovirus (HAd V) infection has been prevalent in various regions in China (Duan et al.2021).Research in Guangzhou reported that 9.14%of fatal pneumonia cases showed HAd V-PCR posit... 详细信息

关键词： mortality Clinical

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Application of topical gentamicin-a new era in the treatment of genodermatosis

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World Journal of Pediatrics 2021年第6期17卷 568-575页

作者： shan Wang Zhou Yang Ying Liu Mu-Tong Zhao Juan Zhao Huan Zhang Zong-Yang Liu Xiao-Ling Wang Lin Ma Yong-Hong Yang Department of Dermatology Beijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthNo.56 South Li Shi RoadXi Cheng DistrictBeijing 100045China Department of Pharmacy.Beijing children's hospital Capital Medical UniversityNational Center for Children's HealthNo.56 South Li Shi RoadXi Cheng DistrictBeijing 100045China China National Clinical Research Center for Respiratory Diseases Department of Respiratory MedicineBeijing Children's HospitalCapital Medical UniversityNational Center for Children’s HealthNo.56 South Li Shi RoadXi Cheng DistrictBeijing 100045China

Background The clinical use of gentamicin always lies in its antimicrobial activity in the past as an aminoglycoside ***,in the past decade,there were considerable interests in therapeutic approaches in treating hereditary *** of the genodermatosis is caused by nonsense mutations that create premature termination codons and lead to the production of truncated or non-functional *** could induce readthrough of nonsense mutations and enable the synthesis of full-length *** focus on previous publications on topical application of gentamicin and review its utility in genetic skin *** sources We search the MEDLINE through PubMed,EMBAsE databases,and the Clinical Trials Registry Platform from January 1960 to July 2020 using the key search terms"gentamicin,topical gentamicin,genodermatosis,genetic skin diseases".Results The application of gentamicin in genodermatosis yielded promising results,both in vivo and in vitro,including Nagashima-type palmoplantar keratosis,epidermolysis bullosa,Hailey-Hailey disease,hereditary hypotrichosis simplex of the scalp,*** Topical gentamicin is a potential treatment option for genodermatosis caused by nonsense mutation.

关键词： Epidermolysis bullosa Gentamicin Nagashima-type palmoplantar keratosis Nonsense mutation Readthrough

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Male external genitalia growth curves and charts for children and adolescents aged 0 to 17 years in chongqing, China

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Asian Journal of Andrology 2018年第6期20卷 567-571页

作者： Yi-Nan Wang Qmg Zeng Feng Xiong Yah Zeng Department of medical statistics and Information Management School of Public Health and Management Chongqing Medical University Chongqing 400016 China Department of Endocrinology the Children's Hospital of Chongqing Medical University Chongqing 400014 China

Genital size is a crucial index for the assessment of male sexual development, as abnormal penile or testicular size may be the earliest visible clinical manifestation of some diseases. However, there is a lack of data regarding penile and testicular size measurements for Chinese boys at all stages of childhood and puberty. This cross-sectional study aimed to develop appropriate growth curves and charts for male external genitalia among children and adolescents aged 0-17 years in chongqing, China. A total of 2974 boys were enrolled in the present study. Penile length was measured using a rigid ruler, penile diameter was measured using a pachymeter, and testicular volume was determined using a Prader orchidometer. Age-specific percentile curves for penile length, penile diameter, and testicular volume were drawn using the generalized additive models for location, scale, and shape. Very similar growth curves were found for both penile length and penile diameter. Both of them gradually rose to 10 years of age and then sharply increased from 11 to 15 years of age. However, testicular volume changed little before the age of 10 years. This study contributes to the literature covering age-specific growth curve and charts about male external genitalia in Chinese children and adolescents. These age-related values are valuable in evaluating the growth and development status of male external genitalia and could be helpful in diagnosing genital disorders.

关键词： adolescents children generalized additive model for location scale and shape growth curves penile size testicular volume

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