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Associations between PRF1 Ala91 Val polymorphism and risk of hemophagocytic lymphohistiocytosis: a meta-analysis based on 1366 subjects

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World Journal of Pediatrics 2020年第6期16卷 598-606页

作者： Guang-Hua Zhu Li-Ping Zhang Zhi-Gang Li Ang Wei Ying Yang Yu Tian Hong-Hao Ma Dong Wang Xiao-Xi zhao yun-ze zhao Na Li Wei Liu Tian-You Wang Rui Zhang Beijing Children's Hospital.Nanlishi Road No.56 Xicheng DistrictBeijingChina

Background Perforin(PRF1)gene mutation can cause the onset of hemophagocytic lymphohistiocytosis(HLH).It has reported that PRFl Ala91 Val polymorphism was related with HLH *** the meta-analysis,we aim to evaluate the association between PRF1 Ala91 Val polymorphism and HLH *** We accomplished a meta-analysis of six published case-control studies including 391 patients with HLH and 975 *** evaluated the quality of each study through Newcastle-Ottawa Scale(NOS).Data analysis was performed with Stata *** In general,all studies were of high quality(NOS score higher than 7).There were statistically significant between the PRFl Ala91 Val polymorphism and HLH risk though the pooled analysis[for Ala/Val ***/Ala:pooled odds ratio(OR)=3.22,95%confidence interval(Cl)1.08-9.56,P=0.035,random model;for Ala/Val+Val/Val ***/Ala:pooled OR=2.96,95%Cl 1.14-7.69,P=0.025,random model].Furthermore,sensitivity analysis also revealed a relationship between PRFl Ala91Val polymorphism and HLH risk(for Ala/Val ***/Ala:pooled OR=5.236,95%Cl 2.72-10.08,P<0.000,/2=12.1%,^heterogeneity=0-332;for Ala/Val+Val/Val ***/Ala,pooled OR=4.856,95%Cl 2.66-8.85,P<0.000,/2=5.9%,Pheterogeneity=0.373).Funnel plot and Egger’s test did not indicate obvious published bias(P=0.841 for Ala/Val ***/Ala;?=0.284 for Ala/Val+Val/Val ***/Ala).Conclusion This meta-analysis indicated that PRFl Ala91 Val polymorphism affects the factor for developing HLH and future studies of PRFl Ala91 Val on the onset of HLH will be guaranteed.

关键词： Ala91Val polymorphism Hemophagocytic lymphohistiocytosis PRFl

Clinical Research of Pulmonary Langerhans Cell Histiocytosis in Children

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Chinese Medical Journal 2018年第15期131卷 1793-1798页

作者： Dong Wang Lei Cui Zhi-Gang Li Li Zhang Hong-yun Lian Hong-Hao Ma yun-ze zhao Xiao-Xi zhao Tian-You Wang Rui Zhang Beijing Key Laboratory of Pediatric Hematology Oncology National Key Discipline of Pediatrics (Capital Medical University) Key Laboratory of Major Diseases in Children Ministry of Education Hematology Oncology Center Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing 100045 China Hematology and Oncology Laboratory Beijing Pediatric Research Institute Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing 100045 China Dong Wang and Lei Cui contributed equally to this work

Background： Pulmonary Langerhans cell histiocytosis （PLCH） is an interstitial primary pulmonary disease, characterized by Langerhans cell proliferation. It is easily misdiagnosed in children. This study aimed to characterize the clinical manifestations and features of PLCH by retrospective analysis. Meihods： A retrospective analysis was performed in 117 PLCH patients out of 338 LCH patients who were admitted in our center from November 2006 to October 2013. Variables between two groups were compared by Mann-Whitney U-test and Chi-square test. Kaplan-Meier curves were constructed to compare the survival rates and Cox regression to evaluate the effect of risk factors. Results： The median age of PLCH group was significantly lower than that ofnon-PkCH group （ 18.63 months vs. 43.4 months, P 〈 0.001 ）. All PLCH children had other organ involvement and only 11 cases （9.4%） had respiratory symptoms. The most common radiologic finding was cystic lesions （29 cases, 24.8%）. Pulmonary thnction abnormalities were dominated by obstructive ventilatory dysfunction （63 cases, 82.9%）. The 5-year overall survival （OS） of PECH children was 93.6% ± 2.3% and the event-free survival （EFS） was 55.7% ± 5.2%. Among the 38 cases with progressed or relapsed disease, five cases （13.2%） were due to progression or recurrence of lung damage. The 5-year OS of PECH children with ＂risk organ＂ involvement was significantly lower than those without ＂risk organ＂ involvement （86.0% ± 4.9% vs. 100%,X^2 = 8.793, P= 0.003）. The difference of EFS between two groups was also significant （43.7% ± 7.7% vs. 66.3% ± 6.5%,X^2 = 5.399, P = 0.020）. The ＂＇risk organ＂ involvement had a significant impact on survival （hazard ratio = 1.9, P = 0.039）. Conclusions： PLCH mainly occurs in young children, and only a small percentage of patients have respiratory symptoms. They generally have other organ involvement. Most of PLCH children have a good prognosis and most lung lesions could have improved or stabilized. Managemen

关键词： Children Langerhans Cell Histiocytosis Lung

Central Nervous System Involvement in 179 Chinese Children with Hemophagocytic Lymphohistiocytosis

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Chinese Medical Journal 2018年第15期131卷 1786-1792页

作者： yun-ze zhao Qing Zhang Zhi-Gang Li Li Zhang Hong-yun Lian Hong-Hao Ma Dong Wang Xiao-Xi zhao Tian-You Wang Rui Zhang Beijing Key Laboratory of Pediatric Hematology Oncology National Key Discipline of Pediatrics（Capital Medical University）Key Laboratory of Major Diseases in ChildrenMinistry of EducationHematology Oncology CenterBeijing Children＇s HospitalCapital Medical UniversityNational Center for Children＇s HealthBeijing 100045China Hematology and Oncology Laboratory Beijing Pediatric Research InstituteBeijing Children＇s HospitalCapital Medical UniversityNational Center for Children＇s HealthBeijing Key Laboratory of Pediatric Hematology OncologyKey Laboratory of Major Diseases in ChildrenMinistry of EducationNational Key Discipline of PediatricsMinistry of EducationBeijing 100045China yun-ze zhao and Qing Zhang contributed equally to this work

Background： Central nervous system （CNS） involvement is found in many patients with hemophagocytic lymphohistiocytosis （HLH）. In this study, we mainly analyzed neurological symptoms, imaging findings, cerebrospinal fluid （CSF）, and their relationship with outcomes of HLH children. Methods： Related data of 179 Chinese pediatric patients with HLH admitted to our center from January 2010 to December 2015 were analyzed retrospectively. Diagnosis and treatment were based on the H LH-2004 protocol. Two-tailed Chi-squared test was used to compare between different groups, and Kaplan-Meier survival curves were used to analyze the overall survival （OS） of patients with HLH. Results： In the present study, 21.2% （38/179） of total patients had neurological symptoms including seizure, irritability, somnolence, and unconsciousness. There were 80 （50.0%, excluding 19 patients without imaging data） patients with cranial imaging abnormalities. There were 14.7% （17/116, excluding 63 patients who did not accept lumbar puncture） of patients with abnormal CSF results. CNS involvement is defined as abnormalities in one or more of CNS symptoms, radiological findings, and CSF. Thus, 60.3% of them had CNS involvement, As for the prognosis, the median follow-up time was 3.2 years （17 lost to follow-up）. The probable 3-year OS of children was higher without CNS involvement （86.0% ± 4.6%） than those with CNS involvement （68.9% ± 4.9%, hazard ratio [HR] = 2.286, P = 0.019）, Among them, the probable 3-year OS of children without CNS symptoms was 76.0% ± 3.8%, higher than with CNS symptoms （59.5% ± 8.1%, HR = 2.147, P ： 0.047）. The 3-year OS of children with abnormal CSF was 64.7% ± 11.6%, compared with normal CSF （85.1% ± 3.7%, HR = 0.255, P = 0.038）. Conclusions： HLH patients with CNS involvement might have worse outcomes compared with those without CNS involvement, and CNS symptoms and CSF changes are more important to access the prognosis than imaging abnormality.

关键词： Central Nervous System Cerebrospinal Fluid Hemophagocytic Lymphohistiocytosis

A single-pixel elemental imaging method using neutron-induced gamma-ray activation

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Nuclear Science and Techniques 2025年第1期36卷 1-12页

作者： Can Cheng Yong-Ji Xie Xun-Rong Xia Jia-Yu Gu Dong zhao Yi-ze Chen Ai-yun Sun Xu-Wen Liang Wen-Bao Jia Da-Qian Hei Department of Ionizing Radiation and Medical Engineering Jiangsu Institute of MetrologyNanjing 210023China Institute of Nuclear Analytical Technology Nanjing University of Aeronautics and AstronauticsNanjing 211106China China Nuclear Power Engineering Co. LtdBeijing 100840China School of Nuclear Science and Technology Lanzhou UniversityLanzhou 730000China

Neutron-induced gamma-ray imaging is a spectroscopic technique that uses characteristic gamma rays to infer the elemental distribution of an ***,this technique requires the use of large facilities to supply a high neutron flux and a time-consuming detection procedure involving direct collimating *** this study,a new method based on low neutron flux was proposed.A single-pixel gamma-ray detector combined with random pattern gamma-ray masks was used to measure the characteristic gamma rays emitted from the *** of the elemental distribution in the sample,comprising 30×30 pixels,were reconstructed using the maximum-likelihood expectation-maximization *** results demonstrate that the elemental imaging of the sample can be accurately determined using this *** proposed approach,which eliminates the need for high neutron flux and scanning measurements,can be used for in-field imaging applications.

关键词： Elemental imaging Neutron-induced gamma-ray activation Single-pixel imaging

MXene Hybridized Polymer with Enhanced Electromagnetic Energy Harvest for Sensitized Microwave Actuation and Self‑Powered Motion Sensing

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Nano-Micro Letters 2025年第3期17卷 265-279页

作者： Yu‑ze Wang Yu‑Chang Wang Ting‑Ting Liu Quan‑Liang zhao Chen‑Sha Li Mao‑Sheng Cao School of Materials Science and Engineering Beijing Institute of TechnologyBeijing 100081People’s Republic of China School of Materials Science and Engineering Peking UniversityBeijing 100871People’s Republic of China School of Mechanical and Material Engineering North China University of TechnologyBeijing 100144People’s Republic of China Key Laboratory of Functional Inorganic Material Chemistry Ministry of Education of the People’s Republic of ChinaHeilongjiang UniversityHarbin 150080People’s Republic of China

Polymeric microwave actuators combining tissue-like softness with programmablemicrowave-responsive deformation hold great promise for mobile intelligentdevices and bionic soft robots. However, their application is challenged by restricted electromagneticsensitivity and intricate sensing coupling. In this study, a sensitized polymericmicrowave actuator is fabricated by hybridizing a liquid crystal polymer with Ti3C2Tx(MXene). Compared to the initial counterpart, the hybrid polymer exhibits unique spacechargepolarization and interfacial polarization, resulting in significant improvements of230% in the dielectric loss factor and 830% in the apparent efficiency of electromagneticenergy harvest. The sensitized microwave actuation demonstrates as the shortenedresponse time of nearly 10 s, which is merely 13% of that for the initial shape memory polymer. Moreover, the ultra-low content of MXene (upto 0.15 wt%) benefits for maintaining the actuation potential of the hybrid polymer. An innovative self-powered sensing prototype that combinesdriving and piezoelectric polymers is developed, which generates real-time electric potential feedback (open-circuit potential of ~ 3 mV) duringactuation. The polarization-dominant energy conversion mechanism observed in the MXene-polymer hybrid structure furnishes a new approachfor developing efficient electromagnetic dissipative structures and shows potential for advancing polymeric electromagnetic intelligent devices.

关键词： Microwave absorption Electromagnetic response Energy harvest Self-sensing Soft actuator

Understanding and probing progression of localized corrosion on inner walls of steel pipelines:an overview

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Journal of Iron and Steel Research International 2025年第1期32卷 1-18页

作者： Ming-yu Wang Hai-yuan Yao Yong-fei Liu Ye-sen Zhu Wan-bin Chen yun-ze Xu Yi Huang School of Naval Architecture and Ocean Engineering Dalian University of TechnologyDalian 116024LiaoningChina China National Offshore Oil Corporation Research Institute Co. Ltd.Beijing 100028China State Key Laboratory of Structural Analysis Optimization and CAE Software for Industrial EquipmentDalian University of TechnologyDalian 116024LiaoningChina

Corrosion poses a major threat to the safety of transportation ***,it is crucial to have an in-depth understanding of corrosion mechanisms in pipeline steels for the effective management of pipeline *** research on corrosion mechanisms relies on the use of efficient and reliable corrosion monitoring and analysis *** advancements in corrosion monitoring techniques specifically designed for the localized corrosion monitoring were aimed to be introduced,and a comprehensive overview of recent progress in understanding the localized corrosion mechanisms in pipeline steels was *** on the different corrosive environments encountered,the localized corrosion issues inside pipelines are classified into two categories:localized corrosion primarily influenced by electrochemical processes and localized corrosion controlled by both electrochemical and mechanical ***,a thorough analysis of the synergistic effects between micro-cell and macro-cell currents,as well as the interplay of mechanics and electrochemistry is ***,recommendations for future research on the mechanisms of internal localized corrosion in pipelines are provided.

关键词： Pipeline steel Localized corrosion Corrosion type Corrosion monitoring Corrosion mechanism

Nickel-copper alloying arrays realizing efficient co-electrosynthesis of adipic acid and hydrogen

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Journal of Energy Chemistry 2025年第02期101卷 7-15页

作者： Xuhui Ren Qianyu Zhang yun Tong Guorong Zhou Cong Lin Yanying zhao Pengzuo Chen School of Chemistry and Chemical Engineering Key Laboratory of Surface & Interface Science of Polymer Materials of Zhejiang Province Zhejiang Sci-Tech University

Constructing electrocatalytic overall reaction technology to couple the electrosynthesis of adipic acid with energy-saving hydrogen production is of significant for sustainable energy ***,the development of highly-active bifunctional electrocatalysts remains a ***,3D hierarchical nickel-copper alloying arrays with dendritic morphology are manufactured by a simple electrodeposition process,standing for the excellent bifunctional electrocatalyst towards the co-production of adipic acid and H2from cyclohexanone and *** membrane-free flow electrolyzer of Cu0.81Ni0.19/NF shows the superior electrooxidation performance of ketone-alcohol（KA） oil with high faradaic efficiencies of over 90% for adipic acid and H2,robust stability over 200 h as well as a high yield of 0.6 mmol h-1for adipic acid at 100 mA ***-situ spectroscopy indicates the Cu0.81Ni0.19alloy contributes to forming more active NiOOH species to involve in the conversion of cyclohexanone to adipic acid,while the proposed reaction pathway undergoes the 2-hydroxycyclohexanone and 2,7-oxepanedione ***,the theoretical calculations confirm that the optimal electronic interaction,boosted reaction kinetics as well as improved adsorption free energy of reaction intermediates,synergistically endows Cu0.81Ni0.19alloy with superior bifunctional performance.

关键词： Adipic acid Cu0.81Ni0.19 alloy Cyclohexanone oxidation reaction Hydrogen evolution reaction Hydrogen fuel

Adaptive Control of a Flexible Manipulator With Unknown Hysteresis and Intermittent Actuator Faults

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IEEE/CAA Journal of Automatica Sinica 2025年第1期12卷 148-158页

作者： Shouyan Chen Weitian He Zhijia zhao yun Feng Zhijie Liu Keum-Shik Hong School of Mechanical and Electrical Engineering Guangzhou University the School of Control Science and Engineering Shandong University IEEE the College of Electrical and Information Engineering Hunan University the National Engineering Research Center for Robot Visual Perception and Control Technology Hunan University the School of Intelligence Science and Technology University of Science and Technology Beijing (USTB) the Institute for Future the School of AutomationQingdao University the School of Mechanical Engineering Pusan National University

In this study, we consider a single-link flexible manipulator in the presence of an unknown Bouc-Wen type of hysteresis and intermittent actuator faults. First, an inverse hysteresis dynamics model is introduced, and then the control input is divided into an expected input and an error compensator. Second,a novel adaptive neural network-based control scheme is proposed to cancel the unknown input hysteresis. Subsequently,by modifying the adaptive laws and local control laws, a fault-tolerant control strategy is applied to address uncertain intermittent actuator faults in a flexible manipulator system. Through the direct Lyapunov theory, the proposed scheme allows the state errors to asymptotically converge to a specified interval. Finally,the effectiveness of the proposed scheme is verified through numerical simulations and experiments.

关键词： Adaptive control flexible manipulator intermittent actuator faults inverse hysteresis dynamics vibration control

Neurological Abnormality Could be the First and Only Symptom of Familial Hemophagocytic Lymphohistiocytosis: Report of Two Families

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Chinese Medical Journal 2018年第24期131卷 3004-3006页

作者： yun-ze zhao Hua Cheng Chang-Hong Ding Hong-Hao Ma Tong-Li Han Jiu-Wei Li Dong Wang Zhi-Gang Li Tian-You Wang Rui Zhang Beijing Key Laboratory of Pediatric Hematology Oncology National Key Discipline of Pediatrics (Capital Medical University)Key Laboratory of Major Diseases in ChildrenMinistry of EducationHematology Oncology CenterBeijing Children's HospitalCapital Medical UniversityNational Center for Children's Health Beijing 100045China Department of Radiology Beijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijing 100045China Department of Neurology Beijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijing 100045China

To the Editor:Hemophagocytic lymphohistiocytosis (HLH)is a life-threatening disease which impacts many parts of the body including the digestive,circulatory,and respiratory *** central nervous system (CNS)can also be affected,particularly in patients with familial HLH-2 (FHL-2),responsible for approximately 20% of all ***-2 is a result of mutations to the perforin 1 (PRF1)gene,which can occur at numerous sites on the gene.

关键词： Neurological Abnormality Symptom of Familial Hemophagocytic Lymphohistiocytosis