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Summary of the Best Evidence for Tube Feeding Intolerance in Critically Ill Children
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作者 Juan Zhang Yuanqing Liang +2 位作者 Linghong He xiaoli ma Bingying ma 《Journal of Clinical and Nursing Research》 2024年第9期133-145,共13页
Objective:To summarize the evidence of tube feeding intolerance in critically ill children,aiming to provide evidence-based information for clinical nursing staff.Methods:Evidence search was done in Chinese and Englis... Objective:To summarize the evidence of tube feeding intolerance in critically ill children,aiming to provide evidence-based information for clinical nursing staff.Methods:Evidence search was done in Chinese and English databases to guide network and professional associations at home and abroad.The search time limit was from January 2014 to January 2024,nearly 10 years of relevant literature,mainly including guidelines,consensus,expert advice,best practice,evidence summary,system evaluation,and meta-analysis.Literature quality evaluation and evidence extraction were independently performed by two researchers.Results:This paper included 13 articles,including three guidelines,three systematic evaluations,three expert opinions,and four expert consensus.Twenty-six pieces of evidence were summarized from 10 aspects of feeding intolerance definition,team building,nutritional assessment,nutritional preparation,feeding protocol,feeding route,feeding management,pipeline management,gastric residual volume,and drug application.Conclusion:This paper summarized the evidence of tube feeding intolerance in critically ill children,which can provide evidence-based information for clinical practice.The abdominal signs should be closely observed when evaluating feeding intolerance,focusing on the prevention and reduction of feeding interruption. 展开更多
关键词 Feeding intolerance Tube feeding Enteral nutrition Summary of evidence
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Bioinformatics analysis of genes differentially expressed in autism and screening of hub genes in the occurrence and development of autism
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作者 manli Li xiaoli ma +2 位作者 Chendi mai Zhiru Fan Yankai Ren 《Journal of Translational Neuroscience》 2022年第1期15-22,共8页
Objective:to screen the genes differentially expressed in autism using bioinformatics methods,and to explore their functional enrichment,related signaling pathways and the tissue-specific expression of hub genes.Metho... Objective:to screen the genes differentially expressed in autism using bioinformatics methods,and to explore their functional enrichment,related signaling pathways and the tissue-specific expression of hub genes.Methods:the autism expression profile chip numbered GSE77103 in the Gene Expression Omnibus(GEO)database was selected for examination.R language and related R packages were used for the screening and visualization of the diflerentially expressed genes.Gene Ontology(GO)function enrichment and Kyoto Encyclopedia of Genes and Genomes(KEGG)signaling pathway analysis of the differential genes were carried out using the relevant R package of R language.The database STRING was used to construct the interaction network of the proteins encoded by the differentially expressed genes,and the software Cytoscape was used to screen the hub genes in the network.The selected hub genes were imported into the BioGPS database to analyze the tissue-specific expression of the hub genes.Results:six hundred and sixty diflerentially expressed genes were screened out.Three hundred and seventy-three up-regulated genes and 287 down-regulated genes in the peripheral blood mononuclear cells of autistic children were compared with the peripheral blood mononuclear cells of healthy children.GO functional enrichment results showed that biological processes(BP)were mainly involved in viral response,negative regulation of viral genome replication,negative regulation of multiple biological processes,negative reg ulation reg ulation of viral life cycle,and defense responses to viruses.Cell components(CC)were involved in vesicles,lysosomal membranes,lysosomal lumen,etc.;molecular functions(MF)were involved in regulating glutathione transferase activity peroxidase activity,oxidoreductase activity,Glutathione peroxidase and transferase activity,etc.The results of KEGG signaling pathway analysis showed that the differentially expressed genes were related to the lysosomal pathway,the glutathione metabolism pathway and the arachidonic acid metabolism pathway.The hub genes screened by cytoHubba were:interferon regulatory factor 7(IRF7),interferon stimulated exonuclease gene 15(ISG15),XIAP associated factor 1(XAF1),MX dynamin like GTPase 1(MX1),interferon induced protein with tetratricopeptide repeats 1(IFIT1),interferon induced protein with tetratricopeptide repeats 5(IFIT5),29-55-oligoadenylate synthetase 3(OAS3),interferon induced protein 44(IFI44),HECT and the RLD domain containing E3 ubiquitin protein ligase 5(HERC5),interferon stimulated exonuclease gene 20(ISG20).Conclusion:there are genes that are diflerentially expressed in the peripheral blood mononuclear cells of autistic toddlers and healthy toddlers.IRF7,ISG15,XAF1,MX],IFIT1,IFIT5,OAS3,IFI44,HERC5,ISG20 are the hub regulatory genes of autism. 展开更多
关键词 AUTISM diflFerentially expressed genes BIOINFORMATICS hub genes
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Understanding the combustion behavior of electric bicycle batteries and unveiling its relationship with fire extinguishing
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作者 Zhanglong Yu Xueling Shen +6 位作者 Ran Xu Zheng Wang Zengming Wan Mingyang Chen Yi Cui Yanyan Fang xiaoli ma 《Journal of Energy Chemistry》 SCIE EI CAS CSCD 2024年第4期609-618,共10页
In this study,a detailed analysis of the combustion behaviors of the lithium iron phosphate(LFP)and lithium manganese oxide(LMO)batteries used in electric bicycles was conducted.This research included quantitative mea... In this study,a detailed analysis of the combustion behaviors of the lithium iron phosphate(LFP)and lithium manganese oxide(LMO)batteries used in electric bicycles was conducted.This research included quantitative measurements of the combustion duration,flame height,combustion temperature,heat release rate,and total heat release.The results indicated that LMO batteries exhibited higher combustion temperatures of 600–700°C,flame heights of 70–75 cm,a significantly higher heat release rate of40.1 k W(12 Ah),and a total heat release of 1.04 MJ(12 Ah)compared to LFP batteries with the same capacity.Based on these experimental results,a normalized total heat release(NORTHR)parameter was proposed,demonstrating good universality for batteries with different capacities.Utilizing this parameter,quantitative calculations and optimization of the extinguishing agent dosage were conducted for fires involving these two types of batteries,and the method was validated by extinguishing fires for these two types of battery packs with water-based extinguishing fluids. 展开更多
关键词 Combustion behavior Electric bicycle Lithium-ion battery Fire extinguishing Normalized total heat release
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Hydrogen sulfide promotes flowering in heading Chinese cabbage by S-sulfhydration of BraFLCs 被引量:9
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作者 xiaoli ma Liping Zhang +6 位作者 Zhuoya Pei Linlin Zhang Zhiqiang Liu Danmei Liu Xuefeng Hao Zhuping Jin Yanxi Pei 《Horticulture Research》 SCIE 2021年第1期452-462,共11页
Heading Chinese cabbage(Brassica rapa L.syn.B.campestris L.ssp.chinensis Makino var.pekinensis(Rupr.)J.Cao et Sh.Cao)is a cruciferous Brassica vegetable that has a triplicate genome,owing to an ancient genome duplicat... Heading Chinese cabbage(Brassica rapa L.syn.B.campestris L.ssp.chinensis Makino var.pekinensis(Rupr.)J.Cao et Sh.Cao)is a cruciferous Brassica vegetable that has a triplicate genome,owing to an ancient genome duplication event.It is unclear whether the duplicated homologs have conserved or diversi fied functions.Hydrogen sulfide(H_(2)S)is a plant gasotransmitter that plays important physiological roles in growth,development,and responses to environmental stresses.The modification of cysteines through S-sulfhydration is an important mechanism of H_(2)S,which regulates protein functions.H?S promotes flowering in Arabidopsis and heading Chinese cabbage.Here we investigated the molecular mechanisms of H_(2)S used to promote flowering in the latter.Four,five,and four BraFLC,BraSOC I,and BraFT homologs were identi fi ed in heading Chinese cabbage.Different BraFLC proteins were bound to different CArG boxes in the promoter regions of the BraSOC I and BraFT homologs,producing different binding patterns.Thus,there may be functionally diverse BraFLC homologs in heading Chinese cabbage.Exogenous H_(2)S at 100μmol L^(-1) significantly promoted flowering by compensating for insuf fi cient vernalization.BraFLC 1 and BraFLC_(3) underwent S-sulfhydration by H_(2)S,after which their abilities to bind most BraSOC I or BraFT promoter probes weakened or even disappeared.These changes in binding ability were consistent with the expression pattern of the BraFT and BraSOC I homologs in seedlings treated with H_(2)S.These results indicated that H_(2)S signaling regulates flowering time.In summary,H_(2)S signaling promoted plant flowering by weakening or eliminating the binding abilities of BraFLCs to downstream promoters through S-sulfhydration. 展开更多
关键词 HYDRATION HEADING FLOWERING
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Baoyuan decoction alleviates myocardial infarction through the regulation of metabolic dysfunction and the mitochondria-dependent caspase-9/3 pathway 被引量:2
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作者 Zhiyong Du Zeliu Shu +7 位作者 Chun Li Xiaomin Song xiaoli ma Lixi Liao Jun Li Pengfei Tu Kewu Zeng Yong Jiang 《Acupuncture and Herbal Medicine》 2021年第1期49-58,共10页
Objective:Baoyuan decoction(BYD)is a traditional Chinese formula with myocardial protection efficacy validated by modern pharmacological tests.The present study aimed to investigate the effect and mechanism of BYD on ... Objective:Baoyuan decoction(BYD)is a traditional Chinese formula with myocardial protection efficacy validated by modern pharmacological tests.The present study aimed to investigate the effect and mechanism of BYD on alleviating myocardial infarction(MI).Methods:Nuclear magnetic resonance-based serum and urinary metabolomics were employed to explore the metabolic regulation effects of BYD in rats with MI induced by left anterior descending ligation.Oxygen-glucose deprivation/recovery(OGD/R)model in H9c2 cells and multiple molecular biology approaches were used to clarify the underlying action mechanisms of BYD.Results:BYD treatment recovered the serum and urinary metabolite profiles of the MI rats toward normal metabolic status and significantly improved mitochondrial energy metabolism and apoptosis pathways perturbed by MI.Analysis of the molecular mechanism of BYD indicated that it suppressed OGD/R-induced H9c2 cell apoptosis in a concentration-dependent manner by inhibiting the mitochondria-dependent caspase-9/3-poly ADP-ribose polymerase pathway.Conclusions:Our results demonstrate that BYD protects against myocardial apoptosis via the mitochondrial metabolic and apoptosis pathways.They also provide novel insights into the clinical application of BYD for the treatment of ischemic heart diseases. 展开更多
关键词 Baoyuan decoction Caspase-9/3 pathway Mechanisms Metabolomics Mitochondrial energy metabolism Myocardial infarction
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Analysis of Channeling-Path Phenomena in a Complex Fault-Block Reservoir with Low Recovery Factor and High Water-Cut Ratio
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作者 Qunyi Wang xiaoli ma +4 位作者 Yongbin Bi Haiyan He Xiao Gu Tongjing Liu Lekun Zhao 《Fluid Dynamics & Materials Processing》 EI 2023年第2期361-377,共17页
Current methods for the analysis of channeling-path phenomena in reservoirs cannot account for the influence of time and space on the actual seepage behavior.In the present study,this problem is addressed considering ... Current methods for the analysis of channeling-path phenomena in reservoirs cannot account for the influence of time and space on the actual seepage behavior.In the present study,this problem is addressed considering actual production data and dynamic characteristic parameters quantitatively determined in the near wellbore area by fitting the water-cut curve of the well.Starting from the dynamic relationship between injection and production data,the average permeability is determined and used to obtain a real-time quantitative characterization of the seepage behavior of the channeling-path in the far wellbore area.For the considered case study(Jidong oilfield),it is found that the seepage capacity of the channeling-path in the far wellbore area is far less(10 times smaller)than that of the channeling-path in the near wellbore area.The present study and the proposed model(combining near wellbore area and far wellbore area real-time data)have been implemented to support the definition of relevant adjustment measures to ultimately improve oil recovery. 展开更多
关键词 Complex fault-block reservoirs channeling path quantitative identification characteristic parameters dynamic inversion
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Effect of Narrative Therapy on the Psychological Status and Life Quality of Family Caregivers of Adolescents with First-episode Depression
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作者 Tingting CHEN Runchao WANG +3 位作者 Yan ZHAN xiaoli ma Chao SUN Longti LI 《Medicinal Plant》 CAS 2022年第6期72-74,84,共4页
[Objectives]The research aimed to explore the effect of narrative therapy on the negative emotion,quality of life,care burden and nursing satisfaction of family caregivers of adolescents with first-episode depression.... [Objectives]The research aimed to explore the effect of narrative therapy on the negative emotion,quality of life,care burden and nursing satisfaction of family caregivers of adolescents with first-episode depression.[Methods]Using convenient sampling method,the family caregivers of the adolescents with first-episode depression hospitalized in the Children and Adolescents Psychology Department of a GradeⅢLevel A hospital in Shiyan City,Hubei Province from July 2021 to April 2022 were selected as the research objects.The coin toss method was used to determine the test group and the control group,with 60 cases in each group.The control group received routine nursing care,and the experimental group implemented narrative therapy on this basis.Self-rating anxiety scale,self-rating depression scale,health status questionnaire(SF-36),and caregiver burden inventory were used to evaluate the anxiety and depression level,quality of life,and care burden level of the caregivers before and after the intervention.The nursing satisfaction questionnaire was used to evaluate the nursing satisfaction to the caregivers after the intervention.[Results]After 8 weeks of intervention,4 cases fell off in the control group and 3 cases fell off in the intervention group.The scores of anxiety and depression in the two groups were lower than those before intervention,and the scores in the test group were significantly lower than those in the control group(P<0.05).The life quality scores of life vitality,emotional function and mental health dimensions were higher than before the intervention,and the scores in the test group were significantly higher than those in the control group(P<0.05).The total score of care burden and the scores in time dependence,development limitation and physiological dimension of the two groups were lower than those before intervention,but the difference was not statistically significant(P>0.05).[Conclusions]Narrative therapy can effectively alleviate the negative emotions of family caregivers of adolescents with first-episode depression,and improve their quality of life and nursing satisfaction,but not their care burden. 展开更多
关键词 Narrative therapy Adolescent with first-episode depression Family caregiver PSYCHOLOGY
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Treatment and outcomes of 1041 pediatric patients with neuroblastoma who received multidisciplinary care in China 被引量:20
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作者 Yan Su Hong Qin +15 位作者 Chenghao Chen Shengcai Wang Shihan Zhang Dawei Zhang Mei Jin Yun Peng Lejian He Xiaoman Wang Suyun Qian maoquan Qin Ming Ge Fuquan Zhang Qi Zeng Huanmin Wang xiaoli ma Xin Ni 《Pediatric Investigation》 CSCD 2020年第3期157-167,共11页
Importance:Neuroblastoma is the most common extracranial malignant solid tumor in children.Multidisciplinary care is critical to improving the survival of pediatric patients with neuroblastoma.Objective:To systematica... Importance:Neuroblastoma is the most common extracranial malignant solid tumor in children.Multidisciplinary care is critical to improving the survival of pediatric patients with neuroblastoma.Objective:To systematically summarize the clinical characteristics of children with neuroblastoma and evaluate their prognosis with multidisciplinary care provided in a single center.Methods:This retrospective study analyzed the clinical data of 1041 patients with neuroblastoma who were diagnosed,treated,and followed-up in the Hematology-Oncology Center of Beijing Children’s Hospital from 2007 to 2019.Results:The median age at diagnosis was 34 months;80.8%of the patients were younger than 5 years of age.Notably,243 patients(23.3%)were classified as low-risk,249 patients(23.9%)were classified as intermediate-risk,and 549(52.7%)were classified as high-risk.Furthermore,956 patients underwent surgical resections;986(94.7%)patients received chemotherapy;and 176 patients with high-risk neuroblastoma received hematopoietic stem cell transplantation.The 5-year event-free survival(EFS)rate was 91.3%and 5-year overall survival(OS)rate was 97.5%in low-risk group;in the intermediate-risk group,these rates were 85.1%and 96.7%,respectively,while they were 37.7%and 48.9%in the high-risk group(P<0.001 for both).The 5-year EFS and OS rates were significantly higher in patients diagnosed between 2015 and 2019 than in patients diagnosed between 2007 and 2014(P<0.001).In total,278 patients(26.7%)exhibited tumor relapse or progression;the median interval until relapse or progression was 14 months.Of the 233 patients who died,83%died of relapse or progression of neuroblastoma and 4.3%died of therapy-related complications.Interpretation:The 5-year OS rate was low in high-risk patients,compared with low-and intermediate-risk patients.Multidisciplinary care is critical for improvement of survival in pediatric patients with neuroblastoma.Additional treatment strategies should be sought to improve the prognosis of patients with high-risk neuroblastoma. 展开更多
关键词 NEUROBLASTOMA PEDIATRIC MULTIDISCIPLINARY CARE Prognosis
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Reciprocal activation ofα5-nAChR and STAT3 in nicotine-induced human lung cancer cell proliferation 被引量:3
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作者 Yao Zhang Yanfei Jia +4 位作者 Ping Li Huanjie li Dongjie Xiao YunshanWang xiaoli ma 《Journal of Genetics and Genomics》 SCIE CAS CSCD 2017年第7期355-362,共8页
Cigarette smoking is the top environmental risk factor for lung cancer. Nicotine, the addictive component of cigarettes, induces lung cancer cell proliferation, invasion and migration via the activation of nicotinic a... Cigarette smoking is the top environmental risk factor for lung cancer. Nicotine, the addictive component of cigarettes, induces lung cancer cell proliferation, invasion and migration via the activation of nicotinic acetylcholine receptors (nAChRs). Genome-wide association studies (GWAS) show that CI4RNA5 gene encoding α5-nAChR is especially relevant to lung cancer. However, the mechanism of this subunit in lung cancer is not clear. In the present study, we demonstrate that the expression of α5-nAChR is correlated with phosphorylated STAT3 (pSTAT3) expression, smoking history and lower survival of non-small cell lung cancer (NSCLC) samples. Nicotine increased the levels of α5-nAChR mRNA and protein in NSCLC cell lines and activated the ]AK2/STAT3 signaling cascade. Nicotine-induced activation of JAK2/STAT3 signaling was inhibited by the silencing of α5-nAChR. Characterization of the CHRNA5 promoter revealed four STAT3-response elements. ChiP assays confirmed that the CHRNA5 promoter contains STAT3 binding sites. By silencing STAT3 expression, nicotine-induced upregulation of α5-nAChR was suppressed. Downregulation of α5-nAChR and/or STAT3 expression inhibited nicotine-induced lung cancer cell proliferation. These results suggest that there is a feedback loop between α5-nAChR and STAT3 that contributes to the nicotine-induced tumor cell proliferation, which indicates that α5-nAChR is an important therapeutic target involved in tobacco-associated lung carcinogenesis. 展开更多
关键词 α5-nAChR STAT3 Cell proliferation NICOTINE Non-small cell lung cancer
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Effect of building interface form on thermal Check foi comfort in gymnasiums in hot and humid climates 被引量:2
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作者 Xiaodan Huang xiaoli ma Qingyuan Zhang 《Frontiers of Architectural Research》 CSCD 2019年第1期32-43,共12页
The thermal environment and thermal comfort of a building are greatly affected by the desig n of the build ing interface form. Most con temporary architectural designs con sider only the relations between architectura... The thermal environment and thermal comfort of a building are greatly affected by the desig n of the build ing interface form. Most con temporary architectural designs con sider only the relations between architectural form and architectural beauty. Few studies on the correlati on of architectural form and thermal comfort address the in flue nee of architectural form on thermal comfort and thermal environment. These studies are particularly important for gymnasium architectures located in hot and humid areas, which have high requirements for thermal comfort. This paper presents an experimental investigati on and an an alysis of the effect of the building in terface form of gymnasiums on thermal comfort in hot and humid subtropical regions during summer. Results showed that the influence of the top interface forms on thermal comfort is mainly dominated by the mea n radiant temperature, which could be con trolled to improve thermal comfort. The in fluence of side interface forms on thermal comfort is mainly domi nated by air velocity, and thermal comfort could be improved by promoting natural ventilation on the side interface form design to reduce indoor heat. This research enhanced our understanding of the relation between the in terface form and the thermal comfort of gymnasiums. In addition, this paper provides a theoretical reference for the sustainable design of gymnasiums in hot and humid climates. 展开更多
关键词 THERMAL COMFORT THERMAL environment Interface FORM Standard effective temperature GYMNASIUM HOT and humid climate
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Challenges and opportunities for managing pediatric central nervous system tumors in China 被引量:4
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作者 Anthony Pak-Yin Liu Daniel CMoreira +10 位作者 Chenchen Sun Lisa Krull Yijin Gao Bo Yang Chenran Zhang Kejun He Xiaojun Yuan Godfrey Chi-Fung Chan Xiaofei Sun xiaoli ma Ibrahim AQaddoumi 《Pediatric Investigation》 CSCD 2020年第3期211-217,共7页
Central nervous system(CNS)tumors represent the most deadly cancer in pediatric age group.In China,thousands of children are diagnosed with CNS tumors every year.Despite the improving socioeconomic status and availabi... Central nervous system(CNS)tumors represent the most deadly cancer in pediatric age group.In China,thousands of children are diagnosed with CNS tumors every year.Despite the improving socioeconomic status and availability of medical expertise within the country,unique challenges remain for the delivery of pediatric neuro-oncology service.In this review,we discuss the existing hurdles for improving the outcome of children with CNS tumors in China.Need for precise disease burden estimation,lack of intra-and inter-hospital collaborative networks,high probability of treatment abandonment,along with financial toxicities from treatment represent the key challenges that Chinese healthcare providers encounter.The tremendous opportunities for advancing the status of pediatric neuro-oncology care in and beyond the country are explored. 展开更多
关键词 PEDIATRIC cancer Central nervous system TUMOR China MULTI-DISCIPLINARY TEAM COLLABORATION
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Diagnosis and treatment of infantile malignant solid tumors in Beijing,China:A multicenter 10-year retrospective study 被引量:4
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作者 Mei Jin Zhi Tian +17 位作者 Yao Xie Zhaoxia Zhang Miao Li YaxiongYu Weiling Zhang Junyang Zhao Huanmin Wang Qi Zeng Long Li Ming Ge Ning Sun Xiaolun Zhang Jian Gong Wanshui Wu Rong Liu Weihong Zhao Dongsheng Huang xiaoli ma 《Pediatric Investigation》 CSCD 2020年第3期178-185,共8页
Importance:Cancer is the main cause of death by disease in children.Children experience the highest incidence of cancer in the first year of life.However,there is no comprehensive registration system for children with... Importance:Cancer is the main cause of death by disease in children.Children experience the highest incidence of cancer in the first year of life.However,there is no comprehensive registration system for children with tumors in China.Objective:To summarize the diagnosis and treatment of infant cancer and analyze the status of standardized diagnosis and management among several treatment centers in Beijing,China,thereby providing evidence to guide further clinical research.Methods:From January 1,2010 to December 31,2019,patients with newly diagnosed infantile malignant solid tumors were admitted to six large tertiary pediatric solid tumor diagnosis and treatment centers in Beijing.The epidemiology,clinical features,and therapeutic effects of tumors in these patients were analyzed retrospectively.All patients were followed up until March 31,2020.Results:In total,938 patients were enrolled in this study.There were 530 boys(56.5%)and 408 girls(43.5%);the median age was 6.0 months(range,0-12.0 months).The three most common tumors were retinoblastoma in 366 patients(39.0%),neuroblastoma in 266 patients(28.4%),hepatoblastoma in 133 patients(14.2%),and central nervous system tumors in 52 patients(5.5%).The estimated 5-year overall survival rate was 81.3%±1.8%,and the 5-year event-free survival rate was 71.8%±2.9%.The 5-year overall survival rates of non-rhabdomyosarcoma soft tissue sarcoma,neuroblastoma,and retinoblastoma were 100%,88%±2.2%,and 86.9%±2.1%,respectively.The 5-year event-free survival rates were 81.1%±2.7%for neuroblastoma,81.6%±9.8%for non-rhabdomyosarcoma soft tissue sarcoma,and 72.7%±14.1%for extracranial malignant germ cell tumors.Interpretation:The three most common infantile malignant solid tumors were retinoblastoma,neuroblastoma,and hepatoblastoma.Multidisciplinary combined diagnosis and treatment is needed for infantile tumors. 展开更多
关键词 INFANT Solid tumor MALIGNANT MULTICENTER NEUROBLASTOMA RETINOBLASTOMA
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DICER1 mutations in twelve Chinese patients with pleuropulmonary blastoma 被引量:5
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作者 Siyu Cai Xisi Wang +3 位作者 Wen Zhao Libing Fu xiaoli ma Xiaoxia Peng 《Science China(Life Sciences)》 SCIE CAS CSCD 2017年第7期714-720,共7页
Our aim is to examine the impact of DICER1 mutations on the pathogenesis of pleuropulmonary blastoma(PPB) by evaluating the mutation frequency and investigating the family history of Chinese patients with PPB. The fam... Our aim is to examine the impact of DICER1 mutations on the pathogenesis of pleuropulmonary blastoma(PPB) by evaluating the mutation frequency and investigating the family history of Chinese patients with PPB. The family histories of 12 children with PPB recruited consecutively were surveyed. Blood samples from patients and their first-degree relatives were tested for DICER1 mutations. Whole-genome sequencing of blood samples and formalin-fixed and paraffin-embedded(FFPE) tumor tissue was performed in one family with twins. Twelve patients with PPB included six type II and six type III cases. Seven of the12 patients harbored DICER1 mutations, six of which were frameshift or nonsense mutations. Another case carried a germline DICER1 mutation affecting the splice site. FFPE sample had a nonsense mutation in TDG and missense mutations in DICER1.In addition, two cases with DICER1 mutations were found to have lung cysts preceding the diagnosis of PPB. Furthermore, one patient had a family history remarkable for thyroid diseases. Our results indicate that the germline mutation frequency in Chinese patients with PPB is similar to the ones reported for patients from USA, UK, and Japan. Moreover, our study strongly suggests that investigating the family history and detecting germline DICER1 mutations might be of benefit to increasing awareness and improving the accuracy of the differential diagnosis of PPB from non-malignant lung cysts. 展开更多
关键词 China DICER1 mutation pleuropulmonary blastoma
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Fanconi anemia gene-associated germline predisposition in aplastic anemia and hematologic malignancies 被引量:1
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作者 Daijing Nie Jing Zhang +14 位作者 Fang Wang Xvxin Li Lili Liu Wei Zhang Panxiang Cao Xue Chen Yang Zhang Jiaqi Chen xiaoli ma Xiaosu Zhou Qisheng Wu Ming Liu Mingyue Liu Wenjun Tian Hongxing Liu 《Frontiers of Medicine》 SCIE CSCD 2022年第3期459-466,共8页
Whether Fanconi anemia(FA)heterozygotes are predisposed to bone marrow failure and hematologic neoplasm is a crucial but unsettled issue in cancer prevention and family consulting.We retrospectively analyzed rare poss... Whether Fanconi anemia(FA)heterozygotes are predisposed to bone marrow failure and hematologic neoplasm is a crucial but unsettled issue in cancer prevention and family consulting.We retrospectively analyzed rare possibly significant variations(PSVs)in the five most obligated FA genes,BRCA2,FANCA,FANCC,FANCD2,and FANCG,in 788 patients with aplastic anemia(AA)and hematologic malignancy.Sixty-eight variants were identified in 66 patients(8.38%).FANCA was the most frequently mutated gene(n=29),followed by BRCA2(n=20).Compared with that of the ExAC East Asian dataset,the overall frequency of rare PSVs was higher in our cohort(P=0.016).BRCA2 PSVs showed higher frequency in acute lymphocytic leukemia(P=0.038),and FANCA PSVs were significantly enriched in AA and AML subgroups(P=0.020;P=0.008).FA-PSV-positive MDS/AML patients had a higher tumor mutation burden,higher rate of cytogenetic abnormalities,less epigenetic regulation,and fewer spliceosome gene mutations than those of FA-PSV-negative MDS/AML patients(P=0.024,P=0.029,P=0.024,and P=0.013).The overall PSV enrichment in our cohort suggests that heterozygous mutations of FA genes contribute to hematopoietic failure and leukemogenesis. 展开更多
关键词 Fanconi anemia aplastic anemia hematologic malignancy germline predisposition
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Acute myeloid leukemia following a primary mediastinal germ cell tumor 被引量:1
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作者 Huimin Hong Yan Su +9 位作者 Chenghao Chen Lejian He Shuai Zhu Wei Lin Mei Jin Xisi Wang Ruidong Zhang Huyong Zheng Qi Zeng xiaoli ma 《Pediatric Investigation》 CSCD 2020年第3期218-221,共4页
Introduction:There is a known association between primary mediastinal germ cell tumor(PMGCT)and hematologic malignancy that is not linked to treatment.They are exceptionally rare entities with a low morbidity and a po... Introduction:There is a known association between primary mediastinal germ cell tumor(PMGCT)and hematologic malignancy that is not linked to treatment.They are exceptionally rare entities with a low morbidity and a poor prognosis.Case presentation:An 11-year-old boy presented with an anterior mediastinal mass diagnosed as a malignant germ cell tumor on the basis of an excisional biopsy.He was found to have acute myeloid leukemia(AML)two years after the chemotherapy for his germ cell tumor.The clinical course was very aggressive with a survival time of only 1 week after diagnosis of AML associated with PMGCT.Conclusion:AML associated with PMGCT needs to be diagnosed correctly.Relevant examinations should be carried out in patients with PMGCTs during and after chemotherapy,and long-term follow-up is still necessary to reduce the risk of morbidity and mortality. 展开更多
关键词 PRIMARY MEDIASTINAL GERM cell tumor Acute MYELOID LEUKEMIA CHEMOTHERAPY
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4SCAR-GD2-modified T-cell therapy in neuroblastoma withamplification: A case report with over 4-year follow-up data 被引量:1
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作者 Xiao Xu Wen Zhao +4 位作者 Zhixia Yue maoquan Qin Mei Jin Lung-Ji Chang xiaoli ma 《Pediatric Investigation》 CSCD 2020年第1期55-58,共4页
Introduction: Neuroblastoma (NB) is the most common extracranial solid tumor among children. The 5-year event-free survival rate for high-risk (HR) NB is still poor, especially for patients with advanced NB withMYCN g... Introduction: Neuroblastoma (NB) is the most common extracranial solid tumor among children. The 5-year event-free survival rate for high-risk (HR) NB is still poor, especially for patients with advanced NB withMYCN gene amplification. Chimeric antigen receptor T (CAR-T) cell therapy is a new treatment for HR-NB.Case presentation: A 55-month-old boy with stage IV HR-NB received 4th-generation CAR-T cells that target disialoganglioside GD2, as consolidation maintenance treatment after intensive chemotherapy, surgery, and autologous stem-cell transplantation. As of February 2019, his CAR-T follow-up time was 37.5 months, indicating prolonged survival. Cranial MRI and ultrasound showed no mass;123I-metaiodobenzylguanidine (123I-MIBG) scan was negative.Conclusion: GD2-CAR-T cells may be an effective treatment option for NB patients withMYCN amplification. 展开更多
关键词 NEUROBLASTOMA GD2 CAR-T MYCN amplification Bone marrow METASTASIS Encephalic metastasis Long term survival
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Analysis of the short-term curative effect of 356 cases of intraocular retinoblastoma in children 被引量:3
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作者 Mei Jin Junyang Zhao +8 位作者 Dawei Zhang Chengyue Zhang Wen Zhao Xisi Wang Qian Zhao Chao Duan Yan Su Huimin Hong xiaoli ma 《Pediatric Investigation》 2018年第4期236-241,共6页
Importance: Retinoblastoma (Rb) is the most common primary malignant intraocular cancer in children. Systemic chemotherapy combined with local therapy is safe and effective for intraocular Rb. Objective: To summarize ... Importance: Retinoblastoma (Rb) is the most common primary malignant intraocular cancer in children. Systemic chemotherapy combined with local therapy is safe and effective for intraocular Rb. Objective: To summarize the short-term outcomes of patients with Rb to provide evidence for optimizing treatment protocols and improving therapeutic safety and efficacy. Methods: The clinical data of 356 patients (486 eyes) with intraocular Rb admitted to our center from December 2009 to April 2017 were retrospectively analyzed. The measures included drug toxicity, eye-preservation rate, and survival rate, with an emphasis on safety and short-term efficacy. The date of last follow-up was 30 November, 2017. results: The patients comprised 226 unilateral Rb and 130 bilateral Rb. Enucleation before chemotherapy was performed in 72 patients. Among the 174 patients with unilateral Rb, enucleation after chemotherapy was performed in 80 patients (46.0%), and the eye was not enucleated in 89 (51.1%);68 eyes were preserved (68/114, 59.6%) in Group D and 20 eyes (20/59, 33.8%) in Group E. Among the 220 eyes in patients with bilateral Rb, enucleation after chemotherapy was performed for 35 eyes;the eye-preservation rate was 91.7% in Group C, 79.1% in Group D, and 52.1%in Group E. All patients developed grade II to IV myelosuppression after chemotherapy, among whom 18 patients (5%) requiring transfusion. Fourteen patients (3.9%) died of intracranial metastasis following self-elected discontinuation of treatment (n = 7). Patients were followed up for a median of 47 (range, 1–96) months. The expected 5-year overall survival rate was 95.3% (96.7% for unilateral Rb and 92.9% for bilateral Rb, P = 0.074). Interpretation: The VEC (vincristine, etoposide, and carboplatin) regimen with local treatment was safe for intraocular Rb. Intracranial metastasis remains the most common cause of Rb-related death. 展开更多
关键词 CHEMOTHERAPY ENUCLEATION INTRAOCULAR RETINOBLASTOMA Local therapy
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Prenatal and perinatal risk factors for solid childhood malignancies: A questionnaire-based study 被引量:1
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作者 Sihui Li Siyu Cai +7 位作者 Cheng Huang Xi Chai Xindi Wang Xisi Wang Wen Zhao Xiaolu Nie Xiaoxia Peng xiaoli ma 《Pediatric Investigation》 2018年第2期107-113,共7页
Importance:Childhood solid tumors account for the highest proportion of childhood cancers and are one of the leading causes of death in childhood.However,their pathogenesis is unclear.Objective:To explore prenatal and... Importance:Childhood solid tumors account for the highest proportion of childhood cancers and are one of the leading causes of death in childhood.However,their pathogenesis is unclear.Objective:To explore prenatal and perinatal risk factors for solid malignancies in children.Methods:We enrolled 71 consecutive pediatric patients (44 boys and 27 girls;median age,30 months) with solid tumors who were diagnosed and treated at our center from January 2013 to December 2016 as the case group.We also enrolled 211 age-and residence-matched healthy children (ratio of approximately 3:1 with the case group) as the control group.We conducted a questionnaire-based survey with the parents of these 282 children.Univariate and multivariate conditional logistic regression analyses of the collected data were performed.Results:Confirmed solid malignancies included neuroblastoma (n =32),rhabdomyosarcoma (n =18),retinoblastoma (n =7),renal tumors (n =3),and other tumors (n =11).Risk factors for solid childhood tumors in the univariate analysis were the parents' age,gravidity,parity,abortion history,vaginal bleeding,family history of malignancy,and prenatal use of folic acid or hematinics/iron supplements (P < 0.05),and those in the multivariate analysis were higher parity (odds ratio [OR],2.482;95% confidence interval [CI],1.521-4.048),family history of malignancy (OR,3.667;95% CI,1.679-8.009),and prenatal use of hematinics/iron supplements (OR,2.882;95% CI,1.440-5.767).In contrast,use of prenatal folic acid was protective (OR,0.334;95% CI,0.160-0.694).Interpretation:A family history of malignancy,use of prenatal hematinics/iron supplements,and higher parity are risk factors for solid childhood tumors,whereas use of prenatal folic acid is a protective factor. 展开更多
关键词 CASE-CONTROL STUDY Children Maternal PERINATAL Risk factors SOLID MALIGNANCIES
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Alveolar rhabdomyosarcoma with bone marrow metastasis causes disseminated intravascular coagulation 被引量:1
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作者 Yan Su Xisi Wang +4 位作者 Jing Yang Wen Zhao Chao Duan Mei Jin xiaoli ma 《Pediatric Investigation》 2018年第4期263-266,共4页
INTRODUCTIONDisseminated intravascular coagulation (DIC) is an abnormal activation of the coagulation cascade that occurs in response to different diseases. Tumors such as malignant hematological diseases and solid tu... INTRODUCTIONDisseminated intravascular coagulation (DIC) is an abnormal activation of the coagulation cascade that occurs in response to different diseases. Tumors such as malignant hematological diseases and solid tumors are important causes of DIC. The incidence of DIC is reported to be 7% in patients with solid tumors. 展开更多
关键词 ALVEOLAR RHABDOMYOSARCOMA DISSEMINATED INTRAVASCULAR COAGULATION diseases
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Dimensional crossover tuned by pressure in layered magnetic NiPS_(3)
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作者 xiaoli ma Yimeng Wang +9 位作者 Yunyu Yin Binbin Yue Jianhong Dai Jinguang Cheng Jianting Ji Feng Jin Fang Hong Jian-Tao Wang Qingming Zhang Xiaohui Yu 《Science China(Physics,Mechanics & Astronomy)》 SCIE EI CAS CSCD 2021年第9期104-111,共8页
The physical properties of most 2D materials are highly dependent on the nature of their interlayer interaction.In-depth studies of the interlayer interaction are beneficial to the understanding of the physical proper... The physical properties of most 2D materials are highly dependent on the nature of their interlayer interaction.In-depth studies of the interlayer interaction are beneficial to the understanding of the physical properties of 2D materials and permit the development of related devices.Layered magnetic NiPS_(3)has unique magnetic and electronic properties.The electronic band structure and corresponding magnetic state of NiPS_(3)are expected to be sensitive to the interlayer interaction,which can be tuned by external pressure.Here,we report an insulator-metal transition accompanied by the collapse of magnetic order during the 2D-3D structural crossover induced by hydrostatic pressure.A two-stage phase transition from a monoclinic(C2/m)to a trigonal(P31m)lattice is identified via ab initio simulations and confirmed via high-pressure X-ray diffraction and Raman scattering;this transition corresponds to a layer-by-layer slip mechanism along the a-axis.Temperature-dependent resistance measurements and room temperature infrared spectroscopy under different pressures demonstrate that the insulator-metal transition and the collapse of the magnetic order occur at~20 GPa,which is confirmed by low-temperature Raman scattering measurements and theoretical calculations.These results establish a strong correlation between the structural change,electric transport,and magnetic phase transition and expand our understanding of layered magnetic materials.Moreover,the structural transition caused by the interlayer displacement has significance for designing similar devices at ambient pressure. 展开更多
关键词 metal-insulator transition magnetic phase transition structural phase transition
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