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spotlight on eltrombopag concentration in pediatric immune thrombocytopenia: A single-center observational study in China

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儿科学研究(英文) 2024年第1期8卷 44-52页

作者： Dong shuyue Wang Zhifa Wang Nan Ma Jingyao Meng Jinxi sun Yixin Cheng Xiaoling Wu Runhui Hematology Oncology Center Beijing Children’s Hospital Capital Medical University National Center for Children’s Health Beijing China Department of Pharmacy Beijing Children’s Hospital Capital Medical University National Center for Children’s Health Beijing China

Importance: Eltrombopag has been recommended for pediatric immune thrombocytopenia (ITP). Response and adverse drug reactions (ADRs) varied widely between individuals, even at the same dose of eltrombopag. The appropriate eltrombopag concentration in ITP has not been reported.Objective: This study aims to explore the appropriate eltrombopag concentration in pediatric ITP.Methods: This was a single-center, prospective cohort study. children diagnosed with refractory persistent/chronic ITP and platelet count st 2 months. Concentration was detected by high-performance liquid chromatography-mass spectrometry at least 2 weeks after eltrombopag. The clinical characteristics-concentration, concentration-response, and concentration-ADRs were analyzed.Results: A total of 30 patients were enrolled, comprising 13 males and 17 females, with a median age of 72 (45-94) months. The median dose and concentration were 1.39 (1.09-1.56) mg/kg and 2.70 (2.25-4.13) mg/L, respectively. Of the enrolled patients, 14 responded to treatment, whereas 16 did not. Additionally, five experienced adverse drug reactions. No linear correlation was observed between eltrombopag concentration and clinical characteristics. The concentration was lower in the response group than in the nonresponse group, but there was no significant difference (t = 0.755,P = 0.457). Patients who experienced ADRs had a higher concentration than those without ADRs (t = 2.538,P = 0.017). The area under the receiver operating characteristic curve of ADRs was 0.78 (95% confidence interval: 0.56-1.00). Youden’s index identified the cutoff point as 4.33 mg/L, with a sensitivity of 88% and a specificity of 60%. Logistic regression analysis demonstrated that a higher platelet count before eltrombopag predicted a favorable response.Interpretation: Eltrombopag proves efficacious and well-tolerated for treating pediatric ITP. However, prolonged and high-dose administration may increase the likelihood of ADRs. Thus

关键词： Concentration Efficacy Eltrombopag Immune thrombocytopenia Pediatrics safety

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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 function.Ultrasonography is an economical,radiationless,noninvasive,and high noise preliminary diagnostic step for UPJO.Artificial intelligence has been widely applied to medical fields and can greatly assist doctors'diagnostic abilities.The demand for a highly secure network environment in transferring electronic medical data online,therefore,has led to the development of blockchain technology.In this study,we built and tested a framework that integrates a deep learning diagnosis model with blockchain technology.Our diagnosis model is a combination of an attention-based pyramid semantic segmentation network and a discrete wavelet transformation-processed residual classification network.We also compared the performance between benchmark models and our models.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 environment.This 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

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Diagnose and treatment for Type D congenital esophageal atresia with tracheoesophageal fistula

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儿科学研究(英文) 2024年第1期8卷 37-43页

作者： Wang Dingding Zhao Yong Zhang Yanan Hua Kaiyun Gu Yichao Li shuangshuang Liao Junmin Yang shen Yang Ting Zhao Jiawei Huang Jinshi Department of Neonatal surgery Beijing Children’s Hospital Capital Medical University National Center for Children’s Health Beijing China

Importance: Type D esophageal atresia (EA) with tracheoesophageal fistula (TEF) is characterized by EA with both proximal and distal TEFs. It is a rare congenital anomaly with a very low incidence.Objective: To investigate diagnostic and treatment strategies for this rare condition.Methods: We retrospectively reviewed the clinicopathological features of patients with EA/TEF treated at our institution between January 2007 and september 2021.Results: Among 386 patients with EA/TEF, 14 (3.6%) had type D EA/TEF. Only two patients were diagnosed with proximal TEF preoperatively. seven patients were diagnosed intraoperatively. Five patients were missed for diagnosis during the initial surgery but was later confirmed by bronchoscopy. During the neonatal period, seven patients underwent a one-stage repair of proximal and distal TEF via thoracoscopy or thoracotomy. Due to missed diagnosis and other reasons, the other 7 patients underwent two-stage surgery for repair of the proximal TEF, including cervical incision and thoracoscopy. Ten of the 14 patients experienced postoperative complications including anastomotic leakage, pneumothorax, esophageal stricture, and recurrence. Patients who underwent one-stage repair of distal and proximal TEF during the neonatal period showed a higher incidence of anastomotic leak (4/7). In contrast, only one of seven patients with two-stage repair of the proximal TEF developed an anastomotic leak.Interpretation: Type D EA/TEF is a rare condition, and proximal TEFs are easily missed. Bronchoscopy may aim to diagnose and determine the correct surgical approach. A cervical approach may be more suitable for repairing the proximal TEF.

关键词： Diagnosis Therapy Tracheoesophageal fistula Type D congenital esophageal atresia

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Current situation and prospect for the diagnosis and treatment of pediatric critical rare diseases in China

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儿科学研究(英文) 2024年第1期8卷 66-71页

作者： Liu Yingchao Qian suyun Pediatric Intensive Care Unit Beijing Children’s Hospital Capital Medical University National Center for Children’s Health Beijing China

The onset of critical rare diseases (RDs) in children is rapid and dangerous, accompanied by a high mortality rate, which brings a heavy burden to both families and society. Multiple malformations, neuromuscular diseases, metabolic diseases, and heart diseases are the most common types of RDs in children of China, often manifesting with multiple organ dysfunction. At present, the diagnosis and treatment of critical RDs in children face challenges such as prolonged diagnosis time, a high misdiagnosis rate, limited treatment modalities, and a significant disease burden. However, with the progress in genetic testing technology, the establishment of multidisciplinary diagnosis and treatment platforms, and the implementation of relevant RD policies in China, children with critical RDs will received enhanced medical services, experience improved prognoses, and reintegrate into social life.

关键词： children Critical Current situation Prospect Rare diseases

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The clinical characteristics and risk factors for necrotizing soft tissue infection in children

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儿科学研究(英文) 2024年第1期8卷 21-26页

作者： Liu Jing Chen Jigang Wang Yanni Qi Hongyan Yu Jing Department of Burn and Plastic surgery Beijing Children’s Hospital Capital Medical University National Center for Children’s Health Beijing China

Importance: Necrotizing soft tissue infection (NsTI) is a serious infectious disease. However, the early clinical manifestations and indicators of NsTI in children are still unclear.Objective: The purpose of this study was to analyze the clinical characteristics and risk factors of NsTI in pediatric patients.Methods: A total of 127 children with skin and soft tissue infection (ssTI) were treated at our hospital and divided into two groups: the NsTI group and the non-NsTI group, based on their discharge diagnosis from January 2011 to December 2022. Then, we collected and analyzed the clinical characteristics and risk factors of all patients, including sex and age, disease inducement, admission temperature, local skin manifestations, infection site, the presence of sepsis, bacterial culture, and laboratory indicators.Results: In our study, there was a statistical difference in the age distribution and disease inducement between NsTI and non-NsTI groups. The occurrence of local skin manifestations (blisters/bullae and ecchymosis) and the presence of sepsis significantly increased in the NsTI group compared to the non-NsTI group. Additionally, only the platelet count on laboratory tests was statistically different between the NsTI and non-NsTI groups. Finally, the logistic regression analysis suggested that local skin manifestations such as blisters/bullae, and ecchymosis, as well as the presence of sepsis, were identified as risk factors for NsTI.Interpretation: children with ssTI and skin manifestations such as blisters/bullae, ecchymosis, and the presence of sepsis are at a higher risk of developing NsTI. These symptoms serve as useful indicators for early detection of NsTI.

关键词： Clinical characteristics Risk factors Necrotizing soft tissue infection skin and soft tissue infection

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Tuberous sclerosis complex combined with primary lymphedema:A case report

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World Journal of Clinical Cases 2024年第15期12卷 2642-2648页

作者： Xing-Peng Li Xiao-Li sun Xin Liu Zhe Wen Li-Hua Jiang Yan Fu Yun-Long Yue Ren-Gui Wang Department of Radiology Beijing Shijitan Hospital Affiliated to Capital Medical UniversityBeijing 100038China Department of Lymph surgery Beijing Shijitan Hospital Affiliated to Capital Medical UniversityBeijing 100038China Department of Nuclear Medicine Beijing Shijitan Hospital Affiliated to Capital Medical UniversityBeijing 100038China Department of MRI Beijing Shijitan Hospital Affiliated to Capital Medical UniversityBeijing 100038China

BACKGROUND Tuberous sclerosis complex(TsC)and primary lymphedema(PLE)are both rare diseases,and it is even rarer for both to occur in the same patient.In this work,we have provided a detailed description of a patient's clinical presentation,imaging findings,and treatment.And a retrospective analysis was conducted on 14 published relevant case reports.CAsE sUMMARY A 16-year-old male came to our hospital for treatment due to right lower limb swelling.This swelling is already present from birth.The patient’s memory had been progressively declining.seizures had occurred 1 year prior at an unknown frequency.The patient was diagnosed with TsC combined with PLE through multimodal imaging examination:Computed tomography,magnetic resonance imaging,and lymphoscintigraphy.The patient underwent liposuction.The swelling of the patient's right lower limb significantly improved after surgery.Epilepsy did not occur.after taking antiepileptic drugs and sirolimus.CONCLUsION TsC with PLE is a rare and systemic disease.Imaging can detect lesions of this disease,which are important for diagnosis and treatment.

关键词： Tuberous sclerosis complex Lymphedema sirolimus Multimodal imaging examination Case report

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Pathogens of first-episode pulmonary infection in 141 children with chronic granulomatous disease

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Chinese medical Journal 2024年第4期137卷 502-504页

作者： Hui Liu shunying Zhao Haiming Yang Jinrong Liu Hui Xu Xiaolei Tang Yuelin shen Xiaoyan Zhang Xiaohui Wen Yuhong Zhao Ping Chu Huimin Li Department of second Respiratory Medicine National Clinical Research Center for Respiratory DiseasesBeijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijing 100045China Office of scientific Research Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijing 100045China

To the Editor:Chronic granulomatous disease(CGD)is an inherited primary immunodeficiency disease characterized by recurrent life-threatening bacterial or fungal infection and tissue granuloma formation.^([1])Pulmonary infection is the most frequent manifestation,affecting nearly 80%of patients,[2]and remains the major cause of morbidity and mortality in patients with CGD.Details of the pathogenic microorganisms responsible for first-episode pulmonary infection provide information on the spectrum of infection in patients with CGD,help guide the empirical choice of antibiotics or antifungal therapy,and contribute to the early identification and management of CGD.Aspergillus is the commonest causative agent of pulmonary infection,but the distribution of other pathogens varies among countries.^([2–5])Few studies have investigated the etiology of pulmonary infection in CGD in a large cohort in China.

关键词： granuloma infection mortality

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Effect of cytochrome P4502C19(CYP2C19)gene polymorphism and clopidogrel reactivity on long term prognosis of patients with coronary heart disease after PCI

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Journal of Geriatric Cardiology 2024年第1期21卷 90-103页

作者： Cheng-Yan HU Yan-Ling WANG Zhen-Xing FAN Xi-Peng sUN shuai WANG Zhi LIU Department of Geriatrics Fu Xing HospitalCapital Medical UniversityBeijingChina Department of Cardiology Xuanwu Hospital Capital Medical UniversityBeijingChina Emergency Department of Xuanwu hospital Capital Medical UniversityBeijingChina

Objective To investigate the impact of CYP2C19 gene polymorphism on clopidogrel reactivity and its association with longterm clinical outcome in patients with coronary heart disease(CHD)undergoing percutaneous coronary intervention(PCI).Methods In total,675 patients were enrolled.Based on the platelet inhibition rate,patients were categorized into two groups:clopidogrel low responsiveness(CLR)and normal clopidogrel responsiveness(NCR).The CLR group was divided into ticagrelor and clopidogrel group based on the antiplatelet drugs used in the follow-up treatment.Patients were classified into three groups(normal metabolizer,intermediate metabolizer,and poor metabolizer)based on the CYP2C19 genotype.We aimed to evaluate the impact of CYP2C19 gene polymorphism on clopidogrel reactivity.The cumulative rates of 12-month all-cause deaths,major adverse cardiovascular events(MACCEs),and bleeding events were calculated.Results CLR was observed in 44.4%of the overall population.significant differences were observed in the platelet inhibition rate of clopidogrel among the three metabolic genotypes(Ps(1.9%)died and 96 patients(14.2%)experienced MACCEs.Patients with CLR(9.6%vs.11.7%vs.22.1%,Ps.16.4%vs.22.6%,P=0.026)experienced a higher rate of MACCEs.A MACCEs risk score between zero and two was calculated.The highest incidence of MACCEs significantly increased with the 2-positive results,and the area under the curve(AUC)was 0.712(95%CI:0.650-0.774,Ps no significant difference between the group with a score of one and the occurrence of MACCEs(P>0.05).Conclusions Low response to clopidogrel in CHD patients is correlated with CYP2C19 gene polymorphism.CYP2C19 genotyping combined with platelet reactivity is an independent predictor of 12-months MACCEs in patients with clopidogrel treatment after PCI,which is better than either test alone.

关键词： CYP2C19 drugs PCI

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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 burden.There 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 children.Data 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 consensus.Although supportive care remains the cornerstone for the management of RsV infections,new monoclonal antibodies,vaccines,drug therapies,and viral surveillance techniques are being rolled out.Conclusions 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 infections.Further 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

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LONP1 ameliorates liver injury and improves gluconeogenesis dysfunction in acute-on-chronic liver failure

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Chinese medical Journal 2024年第2期137卷 190-199页

作者： Muchen Wu Jing Wu Kai Liu Minjie Jiang Fang Xie Xuehong Yin Jushan Wu Qinghua Meng Department of Liver Disease Beijing You-An HospitalCapital Medical UniversityBeijing 100069China beijing Institute of Hepatology Beijing You-An HospitalCapital Medical UniversityBeijing 100069China Department of General surgery Beijing You-An HospitalCapital Medical UniversityBeijing 100069China

Background:Acute-on-chronic liver failure(ACLF)is a severe liver disease with complex pathogenesis.Clinical hypoglycemia is common in patients with ACLF and often predicts a worse prognosis.Accumulating evidence suggests that glucose metabolic disturbance,especially gluconeogenesis dysfunction,plays a critical role in the disease progression of ACLF.Lon protease-1(LONP1)is a novel mediator of energy and glucose metabolism.However,whether gluconeogenesis is a potential mechanism through which LONP1 modulates ACLF remains unknown.Methods:In this study,we collected liver tissues from ACLF patients,established an ACLF mouse model with carbon tetrachloride(CCl 4),lipopolysaccharide(LPs),and D-galactose(D-gal),and constructed an in vitro hypoxia and hyperammonemia-triggered hepatocyte injury model.LONP1 overexpression and knockdown adenovirus were used to assess the protective effect of LONP1 on liver injury and gluconeogenesis regulation.Liver histopathology,biochemical index,mitochondrial morphology,cell viability and apoptosis,and the expression and activity of key gluconeogenic enzymes were detected to explore the underlying protective mechanisms of LONP1 in ACLF.Results:We found that LONP1 and the expressions of gluconeogenic enzymes were downregulated in clinical ACLF liver tissues.Furthermore,LONP1 overexpression remarkably attenuated liver injury,which was characterized by improved liver histopathological lesions and decreased serum alanine aminotransferase(ALT)and aspartate aminotransferase(AsT)in ACLF mice.Moreover,mitochondrial morphology was improved upon overexpression of LONP1.Meanwhile,the expression and activity of the key gluconeogenic enzymes were restored by LONP1 overexpression.similarly,the hepatoprotective effect was also observed in the hepatocyte injury model,as evidenced by improved cell viability,reduced cell apoptosis,and improved gluconeogenesis level and activity,while LONP1 knockdown worsened liver injury and gluconeogenesis disorders.Conclusion:We demonstrated that gluconeogenesis dysfunc

关键词： Acute-on-chronic liver failure Lon protease-1 Gluconeogenesis disturbance Mitochondrial dysfunction Metabolism

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