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Whole-exome sequencing of a multicenter cohort identifies genetic changes associated with clinical phenotypes in pediatric nephrotic syndrome

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Genes & Diseases 2022年第6期9卷 1662-1673页

作者： Jia Jiao Li Wang Fenfen Ni Mo Wang Shipin Feng Xiaojie Gao Han chan Xueying Yang Hao Lee Huan chi Xuelan Chen Daoqi Wu Gaofu Zhang Baohui Yang Anshuo Wang Qin Yang Junli Wan Sijie Yu Xiaoqin Li Mei Wang Xiaofeng Chen Xianying Mai Xiongzhong Ruan Haiping Yang Qiu Li Department of Nephrology Children’s Hospital of Chongqing Medical UniversityNational Clinical Research Center for Child Health and DisordersMinistry of Education Key Laboratory of Child Development and DisordersChongqing Key Laboratory of PediatricsChongqing 400015PR China Department of Nephrology Chengdu Women and Children Central HospitalChengduSichuan 610091PR China Department of Nephrology Sheen Children’s HospitalShenzhenGuangdong 518034PR China Centre for Lipid Research&Key Laboratory of Molecular Biology for Infectious Diseases(Ministry of Education) Institute for Viral HepatitisDepartment of Infectious DiseasesThe Second Affiliated Hospital Chongqing Medical UniversityChongqing 400016PR ChinaJohn Moorhead Research LaboratoryCentre for NephrologyUniversity College London Medical SchoolRoyal Free CampusUniversity College LondonLondon WC1E 6BTUnited Kingdom

Understanding the association between the genetic and clinical phenotypes in children with nephrotic syndrome(NS)of different etiologies is critical for early clinical guidance.We employed whole-exome sequencing(WES)to detect monogenic causes of NS in a multicenter cohort of 637 patients.In this study,a genetic cause was identified in 30.0%of the idiopathic steroid-resistant nephrotic syndrome(SRNS)patients.Other than congenital nephrotic syndrome(CNS),there were no significant differences in the incidence of monogenic diseases based on the age at manifestation.Causative mutations were detected in 39.5%of patients with focal segmental glomerulosclerosis(FSGS)and 9.2%of those with minimal change disease(MCD).In terms of the patterns in patients with different types of steroid resistance,a single gene mutation was identified in 34.8%of patients with primary resistance,2.9%with secondary resistance,and 71.4%of children with multidrug resistance.Among the various intensified immunosuppressive therapies,tacrolimus(TAC)showed the highest response rate,with 49.7%of idiopathic SRNS patients achieving complete remission.Idiopathic SRNS patients with monogenic disease showed a similar multidrug resistance pattern,and only 31.4%of patients with monogenic disease achieved a partial remission on TAC.During an average 4.1-year follow-up,21.4%of idiopathic SRNS patients with monogenic disease progressed to end-stage renal disease(ESRD).Collectively,this study provides evidence that genetic testing is necessary for presumed steroid-resistant and idiopathic SRNS patients,especially those with primary and/or multidrug resistance.

关键词： Clinical phenotypes Genetic phenotypes Multicenter cohort Nephrotic syndrome Pediatric Whole-exome sequencing

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Malignant fibrous histiocytoma presenting as hemoperitoneum mimicking hepatocellular carcinoma rupture

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World Journal of Gastroenterology 2007年第47期13卷 6441-6443页

作者： Hsin-chi Chen chi-Jen Chen chin-Ming Jeng chan-Ming Yang Department of Radiology Cathay General Hospital Hospital Sijhih Branch No. 2 Lane 59 Jiancheng Road Sijhih City Taipei Taiwan China Department of Radiology E-Da Hospital No. 1 E-Da Road Jiau-Shu Tsuen Yan-Chau Hsiang Kaohsiung Taiwan China Department of Radiology Cathay General Hospital No. 280 Jen-Ai Road Section 4 Taipei Taiwan China

Malignant fibrous histiocytoma (MFH) is a pleomorphic mesenchynal sarcoma. It is uncommonly arises primarily from the intra-peritoneal cavity. Primary peritoneal MFH with tumor bleeding and rupture is rare. We describe the imaging features of a 70-year-old patient presenting with ruptured hemorrhagic peritoneal MFH at subhepatic area,accompanied by massive hemoperitoneum,mimicking a ruptured pedunculated hepatocellular carcinoma. Computed tomography (CT) revealed a large heterogeneous enhanced subhepatic mass with adjacent liver,gallbladder and colon invasion. Tumor hemorrhage and rupture complicated with peritoneal seeding and massive bloody ascites were also detected. Angiography showed a hypervascular tumor fed by enlarged right hepatic arteries,cystic artery and omental branches of gastroepiploic artery. The patient underwent laparotomy for tumor resection,but the tumor recurred one month after operation. To our knowledge,the CT appearance of ruptured intraperitoneal MFH complicated by hemoperitoneum has not been previously described.

关键词：恶性纤维组织细胞瘤腹膜腹腔积血自然破裂

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Some features of effective radius and variance of dust particles in numerical simulations of the dust climate on Mars

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Earth and Planetary Physics 2021年第1期5卷 11-18页

作者： chi-Fong Wong Kim-chiu Chow Kwing Lchan Jing Xiao Yemeng Wang Space Science Institute/State Key Laboratory of Lunar and Planetary Science Macao University of Science and TechnologyTaipaMacaoChina

Airborne dust is an important constituent in the Martian atmosphere because of its radiative interaction with the atmospheric circulation.Dust size is one crucial factor in determining this effect.In reality dust sizes are varied;however,in numerical modeling of dust processes,dust size has usually been described by choice of a particular size distribution function,or by use of fixed values of effective radius(ER)and effective variance(EV).In this work,we present analytical expressions that have been derived to specify ER and EV for Nbin dust schemes,based on a model-calculated dust mixing ratio.Numerical simulations based on this approach thus would consider the effects of variable ER on the atmospheric radiation and their interaction.Results have revealed some interesting features of the dust distribution parameters,such as seasonal and spatial variation of ER and EV,which are generally consistent with some previous observational and modeling studies.Compared with the usual approach of using a fixed ER,simulation results from the present approach suggest that the variability of ER can have significant effects on the simulated thermal field of the Martian atmosphere.

关键词： Mars dust effective radius effective variance general circulation model

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Electromagnetic Wave Tailoring:From One Dimension to Multiple Dimensions

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Electromagnetic Science 2023年第3期1卷 1-21页

作者： Jing Cheng Zhang Geng-Bo Wu Mu Ku Chen Xiaoyu Che Yao Liang Din Ping Tsai chi Hou chan Department of Electrical Engineering City University of Hong KongHong Kong SAR 999077China State Key Laboratory of Terahertz and Millimeter Waves City University of Hong KongHong Kong SAR 999077China Centre for Biosystems Neuroscienceand NanotechnologyCity University of Hong KongHong Kong SAR 999077China

Metasurfaces,known as arrays of subwavelength antennas,provide a wide range of options for controlling electromagnetic waves and effectively reducing the size and complexity of electromagnetic devices.Metasurfaces can manipulate five degrees of freedom of electromagnetic waves:amplitude,wavelength,polarization,phase,and orbital angular momentum;these are customized to provide a variety of remarkable functionalities,including metalenses and meta-holograms.With the advancement of simultaneously manipulating two or more degrees of freedom of the electromagnetic field,there has been a significant increase in the amount of information that electromagnetic waves can carry.The wavefront can be precisely tailored for specific applications,facilitating new possibilities for innovative applications with high performance and diverse functionalities,such as full-color vectorial meta-holograms achieved by a single-layer metasurface.This review briefly overviews the latest developments in metasurfaces,categorizing them based on their various degrees of freedom used to manipulate the electromagnetic waves.The use of metasurfaces to control electromagnetic waves from one dimension to multiple dimensions is systematically explored.The challenges and opportunities for future research are discussed.

关键词： Electromagnetic wave manipulation Metasurfaces Single-dimensional manipulation Multidimensional manipulation.

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Crystal structures of D-psicose 3-epimerase from Clostridium cellulolyticum H10 and its complex with ketohexose sugars

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Protein & Cell 2012年第2期3卷 123-131页

作者： Hsiu-chien chan Yueming Zhu Yumei Hu Tzu-Ping Ko Chun-Hsiang Huang Feifei Ren Chun-chi Chen Yanhe Ma Rey-Ting Guo Yuanxia Sun Industrial Enzymes National Engineering Laboratory Institute of Industrial BiotechnologyChinese Academy of SciencesTianjin 300308China Institute of Biological Chemistry Academia SinicaTaipei 115TaiwanChina CAS Key Laboratory of Pathogenic Microbiology and Immunology Institute of MicrobiologyChinese Academy of SciencesBeijing 100101China

D-Psicose 3-epimerase(DPEase)is demonstrated to be useful in the bioproduction of D-psicose,a rare hexose sugar,from D-fructose,found plenty in nature.Clostridium cellulolyticum H10 has recently been identified as a DPEase that can epimerize D-fructose to yield D-psicose with a much higher conversion rate when compared with the conventionally used DTEase.In this study,the crystal structure of the C.cellulolyticum DPEase was determined.The enzyme assembles into a tetramer and each subunit shows a(β/α)8 TIM barrel fold with a Mn2+metal ion in the active site.Additional crystal structures of the enzyme in complex with substrates/products(D-psicose,D-fructose,D-tagatose and D-sorbose)were also determined.From the complex structures of C.cellulolyticum DPEase with D-psicose and D-fructose,the enzyme has much more interactions with D-psicose than D-fructose by forming more hydrogen bonds between the substrate and the active site residues.Accordingly,based on these ketohexosebound complex structures,a C3-O3 proton-exchange mechanism for the conversion between D-psicose and D-fructose is proposed here.These results provide a clear idea for the deprotonation/protonation roles of E150 and E244 in catalysis.

关键词： D-psicose 3-epimerase ketohexose complex structure

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Examining the Progress of Internationalizing Japanese Higher Education From 2000-2010： An Interactive Approach

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Cultural and Religious Studies 2017年第8期5卷 465-474页

作者： chi Ming Victor chan Hang Seng Management College Hong Kong China

This paper reveals a more comprehensive scenario for readers to generate better understanding of "internationalizing" Japanese higher education system from 2000-2010. In fact, it is a very complicated and dynamic process which involves the interactions between national and local actors. By adopting a national-local complexity framework, the paper examines the stances of multi-level stakeholders in contributing or obstructing the implementation in macro-, meta-, and micro-level. In macro level, the author scrutinizes how the national government during the period allowed a dual-track approach through providing financial resources in which public and private universities share diverse "responsibilities" – preserving traditional Japanese system and enhancing the globalized process respectively. In meta-level, the author examines the interactions between university administration and foreign students. It is true that university staff attempted to resolve the problems encountered by foreign students. However, the university administrative system inherited in Japanese management style disappointed those foreign students particularly from Anglophone countries. In micro level, the author looks into the daily life of international students and how the local community interpreted the increasing number of foreigners (kaikokujin) in mixed manner. Such complex analysis undoubtedly exposes that it was quite far for Japan to evolve a truly "internationalized" higher education system in the first decade of the 21st century.

关键词： internationalization higher education Japan

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Paradigm Shift of Japan＇s Monetary Policymaking： Implications of Abe Shinzo＇s Return to Power

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International Relations and Diplomacy 2014年第4期2卷 259-270页

作者： Victor chi Ming chan Hang Seng Management College Hong Kong SAR China

The paper begins with reviewing the monetary policymaking of Japan since mid-1990s. The ―Big Bang‖ reforms in 1996-1997 had developed the foundations of financial restructuring in both private and public sectors. More importantly, under the 1998 Bank of Japan (BOJ) Law, BOJ successfully earned an independence status to formulate ―politically neutral‖ monetary policies but encountered political pressures from politicians and Ministry of Finance (MOF) bureaucrats. Then, the paper explores how the second Abe administration has forced BOJ to accommodate its financial agenda—-boost economic growth and fight against deflation - in order to gain political interests but hurt BOJ independence. The paper proceeds to explain the conflict between politicians and appointed technocrats as an institutional game under government change. The paper concludes that Abe Shinzo handled the rivalry properly by acting aggressively on both economic and strategic issues and brought a significant electoral success of the Liberal Democratic Party"s (LDP"s) in the 2013 upper house election.

关键词：国际关系外交外交行政外交政策

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Mechanistic action of the acute toxicity of Bajitian(Morinda officinalis)in zebrafish embryos

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Journal of Traditional chinese Medical Sciences 2020年第3期7卷 308-315页

作者： Lirong Tan Min Cheng Gigi chi Ting Auyeung Oscar Yu Suen chan Xueping Chen Infinitus(china)Company Ltd. Guangzhou529100China Vitargent(International)Biotechnology Limited. Hong Kong SAR999077China College of Veterinary Medicine and Life Science City University of Hong KongHong Kong SAR999077China

Objectives:To investigate acute toxicity of Bajitian(Morinda officinalis)in zebrafish embryos.Methods:Zebrafish embryos at 48-h post fertilization(hpf)were exposed to Bajitian ethanol extract for72 h.The causative action of a delay in yolk sac absorption by Bajitian was investigated by RT-PCR analysis of lipid metabolism-related microsomal triglyceride transfer protein(MTP),apolipoprotein CII(ApoC2)and lipogenesis-related liver x receptor(LXR)genes.The effect of Bajitian eliciting an inflammatory response was studied by exposing 72 hpf myeloperoxidase(MPO):GFP transgenic zebrafish embryos to Bajitian extract for 4 h.Assessment was done by TUNEL,caspase-3/7,and RT-PCR analysis of the apoptosis related pathway B-cell lymphoma 2 associated X protein(Bax),Nuclear factor kappa-lightchain-enhancer of activated B cells(NF-k B)genes,neutrophil development-related stem cell leukaemia(SCL)and transcription factor PU.1 genes,to reveal the causative action of Bajitian reducing neutrophils.Results:RT-PCR analysis found that Bajitian extract had no effect on the expression of MTP or ApoC2 genes,but upregulated LXR gene,which might explain the delay in yolk sac absorption.Analysis of the inflammatory response showed that compared with negative controls,Bajitian extract significantly(P<.05)reduced the number of neutrophils in MPO:GFP embryos.TUNEL,caspase-3/7,and RT-PCR analysis of Bax and NF-k B genes found that Bajitian extract did not trigger the cell apoptosis.Further RT-PCR analysis found that Bajitian extract did not affect SCL expression,but did lead to down-regulation of PU.1.The inhibition of neutrophil development/differentiation may explain the decline in the total number of neutrophils following Bajitian treatment,which could be attributed to the anti-inflammatory effects found clinically for this drug.Conclusions:This study demonstrated that Bajitian caused a delay in yolk sac absorption and a decrease neutrophil in zebrafish embryos,which may be related to the inhibition of neutrophil development.

关键词： Bajitian Morinda officinalis Neutrophil Zebrafish PU.1 Liver x receptor(LXR) Anti-inflammation

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Predictors of the outcomes of acute-on-chronic hepatitis B liver failure

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World Journal of Gastroenterology 2012年第36期18卷 5078-5083页

作者： Hsiu-Lung Fan Po-Sheng Yang Hui-Wei Chen Teng-Wei Chen De-Chuan chan chi-Hong Chu Jyh-Cherng Yu Shih-Ming Kuo Chung-Bao Hsieh Division of General Surgery Department of SurgeryTri-Service General HospitalNational Defense Medical Center Division of General Surgery Department of SurgeryMackay Memorial Hospital School of Medicine University of PittsburghPittsburghPA 15261United States Division of Pediatric Surgery Department of SurgeryTri-Service General HospitalNational Defense Medical Center

AIM:To identify the risk factors in predicting the outcome of acute-on-chronic hepatitis B liver failure patients.METHODS:We retrospectively divided 113 patients with acute-on-chronic liver failure-hepatitis B virus(ACLF-HBV) and without concurrent hepatitis C or D virus infection and hepatocellular carcinoma into two groups according to their outcomes after anti-HBV therapy.Their demographic,clinical,and biochemical data on the day of diagnosis and after the first week of treatment were analyzed using the Mann-Whitney U test,Fisher's exact test,and a multiple logistic regression analysis.RESULTS:The study included 113 patients(87 men and 26 women) with a mean age of 49.84 years.Fiftytwo patients survived,and 61 patients died.Liver failure(85.2%),sepsis(34.4%),and multiple organ failure(39.3%) were the main causes of death.Multivariate analyses showed that Acute Physiology and Chronic Health Evaluation(APACHE) Ⅱ scores ≥ 12 [odds ratio(OR) = 7.160,95% CI:2.834-18.092,P < 0.001] and positive blood culture(OR = 13.520,95% CI:2.740-66.721,P = 0.001) on the day of diagnosis and model for end-stage liver disease(MELD) scores ≥ 28(OR = 8.182,95% CI:1.884-35.527,P = 0.005) after the first week of treatment were independent predictors of mortality.CONCLUSION:APACHE Ⅱ scores on the day of diagnosis and MELD scores after the first week of anti-HBV therapy are feasible predictors of outcome in ACLFHBV patients.

关键词：慢性乙型肝炎肝功能衰竭预测因子急性 Logistic回归分析 Fisher精确检验 APACHE 乙肝病毒

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Echocardiographic mapping of left ventricular resynchronization during cardiac resynchronization therapy procedures

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chinese Medical Journal 2010年第13期123卷 1645-1651页

作者： chan Ngai-yin CHOY chi-chung CHEUNG Kar-chun LAU Chun-leung LO Ying-keung CHU Pui-shan YUEN Ho-chuen LAU Suet-ting CHOI Yuen-choi Department of Medicine & Geriatrics Princess Margaret Hospital 2-10 Princess Margaret Hospital Road Lai Chi Kok Kowloon Hong Kong China

Background Cardiac resynchronization therapy （CRT） is an effective electrical therapy for patients with moderate to severe heart failure and cardiac dyssynchrony. This study aimed to investigate the degree of acute left ventricular （LV） resynchronization with biventricular pacing （BVP） at different LV sites and to examine the feasibility of performing transthoracic echocardiography （TTE） to quantify acute LV resynchronization during CRT procedure. Methods Fourteen patients with NYHA Class Ⅲ-Ⅳ heart failure, LV ejection fraction 〈35%, QRS duration 〉120 ms and septal-lateral delay （SLD）〉60 ms on tissue Doppler imaging （TDI）, underwent CRT implant. TDI was obtained from three apical views during BVP at each accessible LV site and SLD during BVP was derived. Synchronicity gain index （Sg） by SLD was defined as （I＋（SLD at baseline - SLD at BVP）/SLD at baseline）. Results Seventy-two sites were studied. Positive resynchronization （R＋, Sg〉1） was found in 42 （58%） sites. R＋ was more likely in posterior or lateral than anterior LV sites （66% vs. 36%, P 〈0.001）. Concordance of empirical LV lead implantation sites and sites with R＋ was 50% （7/14）. Conclusions The degree of acute LV resynchronizaUon by BVP depends on LV lead location and empirical implantation of LV lead results in only 50% concordance with R＋. Performing l-rE during CRT implantation is feasible to identify LV sites with positive resynchronization.

关键词： echocardiography cardiac resynchronization therapy heart failure left ventricular lead device implantation optimization

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