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China Medical Abstracts(Internal Medicine) 2017年第1期34卷 12-13页

作者：吕华 Dept Hosp Infect Contr Children's Hosp Zhejiang Univ Med Sch

Objective To investigate the status and trends of nosocomial infection in children’s hospital to provide references for prevention and control of nosocomial *** The retrospective study was conducted to analyze the da... 详细信息

关键词： Current status and trends of nosocomial infection in Zhejiang University affiliated children’s hospital from 2005 to 2014

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Transient diabetes mellitus with ABCC8 variant successfully treated with sulfonylurea:Two case reports and review of literature

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World Journal of Diabetes 2024年第8期15卷 1811-1819页

作者： Ling-Hua shen Yan Cui Dong-Xia Fu Wei Yang sheng-Nan Wu Hui-Zhen Wang Hai-Hua Yang Yong-Xing Chen Hai-Yan Wei Department of Endocrinology and Metabolism Henan Key Laboratory of Children's Genetics and Metabolic DiseasesChildren's Hospital Affiliated to Zhengzhou UniversityHenan Children's Hospital Zhengzhou Children's HospitalZhengzhou 450018Henan ProvinceChina

BACKGROUND Transient neonatal diabetes mellitus(TNDM)is a rare form of diabetes mellitus that usually presents within the first 6 mo of *** often enter remission within several months,although relapse can occur later in *** in the ABCC8 gene,which encodes the sulfonylurea receptor 1 of the ATP-sensitive potassium channel in pancreatic beta cells,are associated with TNDM and permanent neonatal *** study describes a novel de novo c.3880C>T heterozygous ABCC8 variant that causes TNDM and can be treated with sulfonylurea *** sUMMARY We retrospectively analyzed 2 Chinese patients with TNDM who were diagnosed,treated,or referred for follow-up between september 2017 and september *** patients were tested for mutations using targeted next-generation *** with neonatal diabetes mellitus caused by a c.3880C>T heterozygous missense variant in the ABCC8 gene have not been reported *** children had an onset of post-infectious diabetic ketoacidosis,which is worth *** a follow-up visit after discontinuing insulin injection,oral glyburide was found to be effective with no adverse *** Early genetic testing of neonatal diabetes mellitus aids in accurate diagnosis and treatment and helps avoid daily insulin injections that may cause pain.

关键词： Neonatal diabetes mellitus ABCC8 sulfonylurea receptors 1 KATP channels sulfonylurea Glyburide Insulinulin Case report

Clinical characteristics and genetic mutations of 10 Chinese children with cystic fibrosis or cystic frbrosis transmembrane conductance regulator-related disorders

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中华医学杂志(英文版) 2024年第14期137卷 1753-1755页

作者： Tan shanshan Cao Ling Department of Pulmonology The Children’s Hospital Affiliated to the Capital Institute of Pediatrics Beijing China

To the Editor: Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. To date, over 2000 CFTR mutations have been identified. CF mostly occurs in the Caucasian population, and its incidence is approximately 1/25,000 to 1/1800. However, the number of CF cases in China is small. To discover the gene mutations and clinical characteristics of Chinese children with CF, as well as their correlation, the clinical data of 10 children with CF or CFTR-related disorders (CFTR-RD) were retrospectively analyzed in this study.

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somatic GNA11/GNAQ variants in a cohort of Chinese children with phakomatosis pigmentovascularis

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Pediatric Investigation 2024年第2期8卷 117-125页

作者： Bin Zhang Rui He Riga Wu Zhou Yang Man Hu Nan Zhang Wu Guo Zigang Xu Lin Ma Department of Dermatology Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Department of Dermatology Zhengzhou UniversityAffiliated Children’s HospitalHenan Children’s HospitalZhengzhou Children’s HospitalZhengzhouChina Department of Dermatology Affiliated Hospital of Inner Mongolia Medical UniversityHohhotChina Department of Ophthalmology Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Department of Pathology Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina

Importance:Postzygotic mutations in the GNAQ/GNA11 genes,which encode the G-protein nucleotide binding protein alpha subunits,have been identified in patients with phakomatosis pigmentovascularis(PPV).However,little is known about the Chinese ***:To identify pathogenic mutations in pediatric patients with PPV within the Chinese ***:We performed whole-exome sequencing(WEs)using skin lesion tissues from pediatric patients diagnosed with ***,ultradeep-targeted sequencing was conducted to validate the somatic mutations.A genotype-phenotype correlation was analyzed by integrating data from previous reports with the findings of the present ***:Thirteen patients were enrolled,all diagnosed with the cesioflammea type of PPV,except for one patient with an unclassifiable *** identified somatic GNA11 c.547C>T(p.R183C)variant in seven patients and GNAQ c.548G>A(p.R183Q)in four patients,with low allelic fractions ranging from 2.1%to 8.6%through ultradeep ***,a GNAQ c.548G>A(p.R183Q)variant was detected through targeted sequencing in one of two patients who did not exhibit detectable variants via *** genotype-phenotype correlation analysis,involving 15 patients with a GNA11 variant and 10 with a GNAQ variant,revealed that facial capillary malformation(87%vs.50%,P=0.075)and ocular melanocytosis(80%vs.40%,P=0.087)appeared to be more frequent in patients with GNA11 mutation compared to those with GNAQ *** four patients diagnosed with cesiomarmorata type or overlapping cesioflammea and cesiomarmorata type PPV carried the GNA11 ***:Our study demonstrated that the majority of PPV patients in the Chinese population carried a postzygotic variant of GNAQ/GNA11,thus further confirming the pathogenic role of GNAQ/GNA11 mosaicism in the development of PPV cesioflammea type.

关键词： Phakomatosis pigmentovascularis GNA11 GNAQ somatic variant

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Current status of cognition and skin care behavior in adolescent patients with acne:A survey in China

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Chinese Medical Journal 2024年第4期137卷 476-477页

作者： Jing Tian Hong shu Qiufang Qian Zhong shen Chunyu Zhao Li song Ping Li Xiuping Han Hua Qian Jinping Chen Hua Wang Lin Ma Yuan Liang Department of Dermatology Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijing 100045China Department of Dermatology Kunming Children’s HospitalKunmingYunnan 650034China Department of Dermatology Children’s Hospital of ShanghaiChildren’s Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200127China Loreal China Co. LtdShanghai 200040China Department of Dermatology Henan Children’s HospitalChildren’s Hospital Affiliated to Zhengzhou UniversityZhengzhou Hospital Beijing Children’s HospitalZhengzhou Children’s HospitalZhengzhouHenan 450052China Department of Dermatology Shenzhen Children’s HospitalShenzhenGuangdong 518026China Department of Dermatology Shengjing Hospital of China Medical UniversityShenyangLiaoning 110004China Department of Dermatology Children’s Hospital of Soochow UniversitySuzhouJiangsu 215025China Department of Dermatology Guangzhou Women and Children's Medical CenterGuangzhouGuangdong 510623China Department of Dermatology Children’s Hospital of Chongqing Medical UniversityChongqing 400014China

To the Editor:Acne vulgaris(AV)is a common inflammatory skin ***-based epidemiological studies have reported associations between dietary habits,lifestyle factors,family history,and the occurrence and development of acne.^([1–4])This study investigated the skincare behavior and cognition of patients with AV regarding predisposing factors using a designed electronic questionnaire.

关键词： patients dietary behavior

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Quantifying peripapillary vessel density and retinal nerve fibre layer in type 1 diabetic children without clinically detectable retinopathy using OCTA

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International Journal of Ophthalmology(English edition) 2024年第2期17卷 278-281页

作者： Ling Chen Yun Feng sha-sha Zhang Yan-Fang Liu Ping Lin Xi’an children’s hospital affiliated to Xi’an Jiaotong University Xi’an 710002Shaanxi ProvinceChina

AIM:To quantify changes in radial peripapillary capillary vessel density(ppVD)and the peripapillary retinal nerve fiber layer(pRNFL)in children with type 1 diabetes without clinical diabetic retinopathy by optical coherence tomography angiography(OCTA),providing a basis for early retinopathy in children with type 1 ***:This was a retrospective study.A total of 30 patients(3–14y)with type 1 diabetes without clinical diabetic retinopathy(NDR group)were included.A total of 30 age-matched healthy subjects were included as the normal control group(CON group).The HbA1c level in the last 3mo was measured once in the NDR *** pRNFL thickness and ppVD were automatically measured,and the mean pRNFL and ppVD were calculated in the nasal,inferior,temporal,and superior *** changes in ppVD and pRNFL in the two groups were ***:Compared with CON group,the nasal and superior ppVDs decreased in the NDR group(all Pss of the nasal pRNFL decreased significantly(Psuperior pRNFLs slightly decreased but not significant in the NDR group(all P>0.05).Person and spearman correlation analysis of ppVD and pRNFL thickness in each quadrant of the NDR group showed a positive correlation between nasal and superior(all Psignificant correlation(all P>0.05).There was no significant correlation between the HbA1c level and ppVD and pRNFL in any quadrant(all P>0.05).There was no significant correlation between the course of diabetes mellitus and ppVD and pRNFL in any quadrant(all P>0.05).CONCLUsION:ppVD and pRNFL decrease in eyes of children with type 1 diabetes before clinically detectable retinopathy and OCTA is helpful for early monitoring.

关键词： diabetic retinopathy children peripapillary vessel density peripapillary retinal nerve fiber layer optical coherence tomography angiography

A multicenter prospective study on the management of hepatoblastoma in children:a report from the Chinese children's Cancer Group

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World Journal of Pediatrics 2024年第6期20卷 590-601页

作者： Meng-Jie Tang Xiao-Li Ma Xiang-Ling He Wei-Hua Pan Xiao-Hong Zhang sha-Yi Jiang Ju Gao Fu Li Wei Yao song Gu Wei-Ling Zhang Qiang Zhao shi-Hao Huang Yong-Jun Fang Wei Liu Hui-Zhong Niu Chun-Mei Wang Li-Rong sun Hui Gao Yun-Peng Dai shun-Gen Huang Zhi-Yong Zhong Xi-Ge Wang Zhong-Rong Li Liang-Chun Yang Ye-Ming Wu Huan-Min Wang Xin sun Xiao-Jun Yuan Department of Pediatric Hematology/Oncology Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineNo.1665Kongjiang RoadYangpu DistrictShanghai 200092China Medical Oncology Department Pediatric Oncology CenterBeijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijing 100045China Department of Hematology/Oncology Children's Medical CenterHunan Provincial People's HospitalThe First Affiliated Hospital of Hunan Normal UniversityChangsha 41005China Department of Pediatric surgery Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China Department of Hematology/Oncology Guangzhou Women and Children's Medical CenterNational Children's Medical Center for South Central RegionGuangzhou 510623China Department of Hematology Shanghai Children's HospitalShanghai Jiao Tong UniversityShanghai 200062China Department of Pediatrics West China Second University HospitalSichuan UniversityChengdu 610041China Department of Hematology/Oncology Qilu Children's Hospital of Shandong UniversityJinan 250022China Department of Pediatric surgery Children's Hospital of Fudan UniversityShanghai 201102China Department of General surgery Shanghai Children's Medical Center(National Children's Medical Center-Shanghai)Shanghai Jiao Tong University School of MedicineShanghai 200127China Department of Pediatrics Beijing Tongren HospitalCapital Medical UniversityBeijing 100730China Department of Pediatric Oncology Tianjin Medical University Cancer Institute and HospitalTianjin 300060China Department of Hematology/Oncology Children's Hospital of Nanjing Medical UniversityNanjing 210008China Department of Hematology/Oncology Henan Children's HospitalZhengzhou 450018China Department of Pediatric General surgery Hebei Children's Hospital of Hebei Medical UniversityShijiazhuang City 050031China children's hospital the First Affliated Hospital of Zhengzhou UniversityZhengzhou 450052China Department of Paediatric

Background This study aimed to identify survival risk factors in Chinese children with hepatoblastoma(HB)and assess the effectiveness of the new treatment protocol proposed by the Chinese children's Cancer Group(CCCG)in *** A multicenter,prospective study that included 399 patients with HB from January 2015 to June 2020 was *** demographics,treatment protocols,and other related information were *** regression models and Kaplan-Meier curve methods were *** The 4-year event-free survival(EFs)and overall survival(Os)were 76.9 and 93.5%,*** 4-year EFs rates for the very-low-risk,low-risk,intermediate-risk,and high-risk groups were 100%,91.6%,81.7%,and 51.0%,*** 4-year 0s was 100%,97.3%,94.4%,and 86.8%,*** regression analysis found that age,tumor rupture(R+),and extrahepatic tumor extension(E+)were independent prognostic factors.A total of 299 patients had complete remission,and 19 *** with declining alpha-fetoprotein(AFP)>75%after the first two cycles of neoadjuvant chemotherapy had a better EFs and Os than those≤75%.Conclusions The survival outcome of HB children has dramatically improved since the implementation of CCCG-HB-2016 ***≥8 years,R+,and E+were independent risk factors for *** with a declining AFP>75%after the first two cycles of neoadjuvant chemotherapy had better EFs and Os.

关键词： Alpha-fetoprotein Hepatoblastoma Multicenter Prospective study survival

Deep Learning Blockchain Integration Framework for Ureteropelvic Junction Obstruction Diagnosis Using Ultrasound Images

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Tsinghua science and Technology 2024年第1期29卷 1-12页

作者： Yu Guan Pengceng Wen Jianqiang Li Jinli Zhang Xianghui Xie Faculty of Information Technology Beijing University of TechnologyBeijing 100124China Beijing children’s hospital affiliated to Capital Medical University Beijing 100020China

UreteroPelvic Junction Obstruction(UPJO)is a common hydronephrosis disease in children that can result in an even progressive loss of renal *** is an economical,radiationless,noninvasive,and high noise preliminary diagnostic step for *** intelligence has been widely applied to medical fields and can greatly assist doctors'diagnostic *** demand for a highly secure network environment in transferring electronic medical data online,therefore,has led to the development of blockchain *** this study,we built and tested a framework that integrates a deep learning diagnosis model with blockchain *** diagnosis model is a combination of an attention-based pyramid semantic segmentation network and a discrete wavelet transformation-processed residual classification *** also compared the performance between benchmark models and our *** diagnosis model outperformed benchmarks on the segmentation task and classification task with MloU=87.93,MPA=93.52,and accuracy=91.77%.For the blockchain system,we applied the InterPlanetary File system protocol to build a secure and private sharing *** framework can automatically grade the severity of UPJO using ultrasound images,guarantee secure medical data sharing,assist in doctors'diagnostic ability,relieve patients'burden,and provide technical support for future federated learning and linkage of the Internet of Medical Things(loMT).

关键词： data mining image processing and computer vision machine learning medical information systems

Expert consensus on the diagnosis, treatment, and prevention of respiratory syncytial virus infections in children

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World Journal of Pediatrics 2024年第1期20卷 11-25页

作者： Xian-Li Zhang Xi Zhang Wang Hua Zheng-De Xie Han-Min Liu Hai-Lin Zhang Bi-Quan Chen Yuan Chen Xin sun Yi Xu sai-Nan shu shun-Ying Zhao Yun-Xiao shang Ling Cao Yan-Hui Jia Luo-Na Lin Jiong Li Chuang-Li Hao Xiao-Yan Dong Dao-Jiong Lin Hong-Mei Xu De-Yu Zhao Mei Zeng Zhi-Min Chen Li-su Huang Department of Infectious Disease Children's HospitalZhejiang University School of Medicine3333 Binsheng RoadBinjiang DistrictHangzhou 310052China Clinical Research Unit Xin Hua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghaiChina Beijing Pediatric Research Institute Beijing Children's HospitalCapital Medical UniversityBeijingChina Department of Pediatric Pulmonology West China Second University HospitalSichuan UniversityChengduChina Department of Pediatric Pulmonology the Second Affiliated Hospital&Yuying Children's HospitalWenzhou Medical UniversityWenzhouChina Department of Infectious Disease Anhui Provincial Children's HospitalHefeiChina Department of Pediatrics the Second Hospital of Hebei Medical UniversityShijiazhuangChina Department of Pediatrics Xijing Hospitalthe Fourth Military Medical UniversityXi'anChina Department of Infectious Disease Guangzhou Women and Children's Medicine CenterGuangzhou Medicine UniversityGuangzhouChina Department of Pediatrics Tongji HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhanChina Department of Respiratory Disease Beijing Children's HospitalCapital Medical UniversityBeijingChina Department of Pediatric Respiratory Shengjing Hospital of China Medical UniversityShenyangChina Respiratory Department Children's Hospital Affiliated to Capital Institute of PediatricsBeijingChina Department of Clinical Epidemiology Aarhus UniversityAarhusDenmark Department of Respirology Children's Hospital of Soochow UniversitySuzhouChina Department of Respiratory Children's Hospital of ShanghaiChildren's Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghaiChina Department of Infectious Disease Hainan Women and Children's Medical CenterHaikouChina Department of Infectious Disease Children's Hospital of Chongqing Medical UniversityChongqingChina Department of Respiratory Children's Hospital of Nanjing Medical UniversityNanjingChina Department of Infectious Di

Background Respiratory syncytial virus(RsV)is the leading global cause of respiratory infections and is responsible for about 3 million hospitalizations and more than 100,000 deaths annually in children younger than 5 years,representing a major global healthcare *** is a great unmet need for new agents and universal strategies to prevent RsV infections in early life.A multidisciplinary consensus development group comprising experts in epidemiology,infectious diseases,respiratory medicine,and methodology aims to develop the current consensus to address clinical issues of RsV infections in *** sources The evidence searches and reviews were conducted using electronic databases,including PubMed,Embase,Web of science,and the Cochrane Library,using variations in terms for"respiratory syncytial virus","RsV","lower respiratory tract infection","bronchiolitis","acute","viral pneumonia","neonatal","infant""children",and"pediatric".Results Evidence-based recommendations regarding diagnosis,treatment,and prevention were proposed with a high degree of *** supportive care remains the cornerstone for the management of RsV infections,new monoclonal antibodies,vaccines,drug therapies,and viral surveillance techniques are being rolled *** This consensus,based on international and national scientific evidence,reinforces the current recommendations and integrates the recent advances for optimal care and prevention of RsV *** improvements in the management of RsV infections will require generating the highest quality of evidence through rigorously designed studies that possess little bias and sufficient capacity to identify clinically meaningful end points.

关键词： Consensus prevention Respiratory syncytial virus Treatment

severe pediatric COVID‑19:a review from the clinical and immunopathophysiological perspectives

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World Journal of Pediatrics 2024年第4期20卷 307-324页

作者： Yi‑Kan sun Can Wang Pei‑Quan Lin Lei Hu Jing Ye Zhi‑Gang Gao Ru Lin Hao‑Min Li Qiang shu Li‑su Huang Lin‑Hua Tan children's hospital Zhejiang University School of MedicineHangzhou 310052China The second affiliated hospital Zhejiang University School of MedicineHangzhou 310030China surgical Intensive Care Unit Children’s HospitalZhejiang University School of MedicineHangzhou 310052China Department of General surgery Children’s HospitalZhejiang University School of MedicineHangzhou 310052China Department of Cardiopulmonary and Extracorporeal Life support Children’s HospitalZhejiang University School of MedicineHangzhou 310052China Clinical Data Center Children’s HospitalZhejiang University School of MedicineHangzhou 310052China Department of Cardiac surgery Children’s HospitalZhejiang University School of MedicineHangzhou 310052China National Clinical Research Center for Child Health Children’s HospitalZhejiang University School of MedicineHangzhou 310052China Department of Infectious Diseases Children’s HospitalZhejiang University School of MedicineHangzhou 310052China

Background Coronavirus disease 2019(COVID-19)tends to have mild presentations in ***,severe and critical cases do arise in the pediatric population with debilitating systemic impacts and can be fatal at times,meriting further attention from ***,the intricate interactions between the pathogen virulence factors and host defense mechanisms are believed to play indispensable roles in severe COVID-19 pathophysiology but remain incompletely *** sources A comprehensive literature review was conducted for pertinent publications by reviewers independently using the PubMed,Embase,and Wanfang *** keywords included“COVID-19 in children”,“severe pediatric COVID-19”,and“critical illness in children with COVID-19”.Results Risks of developing severe COVID-19 in children escalate with increasing numbers of co-morbidities and an unvaccinated *** respiratory distress stress and necrotizing pneumonia are prominent pulmonary manifestations,while various forms of cardiovascular and neurological involvement may also be *** immunological processes are implicated in the host response to COVID-19 including the type I interferon and inflammasome pathways,whose dysregulation in severe and critical diseases translates into adverse clinical *** inflammatory syndrome in children(MIs-C),a potentially life-threatening immune-mediated condition chronologically associated with COVID-19 exposure,denotes another scientific and clinical conundrum that exemplifies the complexity of pediatric *** the considerable dissimilarities between the pediatric and adult immune systems,clinical trials dedicated to children are lacking and current management recommendations are largely adapted from adult *** severe pediatric COVID-19 can affect multiple organ *** dysregulated immune pathways in severe COVID-19 shape the disease course,epitomize the vast functional diversity of the

关键词： Immunopathophysiology MIs-C Pediatric critical care severe pediatric COVID-19