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Early-onset primary torsional dystonia in a 4-generation Chinese family with a mutation in the DyT1 gene

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Chinese Medical Journal 2005年第10期118卷 873-876页

作者： y.uNG Wai Lan LAM Ching Wan CHENG Wai Tsoi SIN Ngai Chuen wong Wing Kin wong Chun Nei TSE Ka Ming FOK Tai fai Departments of Paediatrics Prince of Wales HospitalThe Chinese University of Hong KongHong KongChina Chemical Patholog Prince of Wales HospitalThe Chinese University of Hong KongHong KongChina Department of Paediatrics and Adolescent Medicine Queen Mary HospitalUniversity of Hong Kong Nuclear Medicine&PET Division Scanning DepartmentSt.Teresa’s HospitalHong KongChina

Primary torsion dystonia(PTD)is a clinically and genetically heterogeneous movement *** least thirteen different types of dystonia can be distinguished on a genetic basis.~1 The DyT1 gene was first mapped by Ozelius et al in 1989.~2(Kramer et)al^3 linked the same locus to PTD in 12 Ashkenazi Jewish families in *** patients with early-onset generalized PTD were caused by the same three base pair(GAG)deletion in the DyT1 gene on chromosome 9q34.^(1,4,5)The product of the gene is a protein called torsinA.~5(Although the)function of this protein is as yet uncertain,it is widely distributed throughout the brain with high levels in the substantia nigra compacta dopamine neurones.

关键词： DyT1 gene dystonia positron emission tomography

Feasibility of gallbladder preservation during robotic left hepatectomy:A retrospective comparative study

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Laparoscopic, Endoscopic and Robotic Surgery 2022年第3期5卷 100-105页

作者： Kit-fai Lee Andrew Ky.Fung Hon-Ting Lok Janet WC.Kung Eugene yJ.Lo Charing CN.Chong John wong Kelvin KC.Ng Division of Hepatobiliary and Pancreatic Surgery Department of SurgeryPrince of Wales HospitalThe Chinese University of Hong KongHong KongChina

Objective:Traditionally,gallbladder is routinely removed during left hepatectomy even if there is no gallbladder ***,adverse consequence after cholecystectomy,though rare,still occa-sionally *** study aims to evaluate the feasibility of gallbladder preservation during robotic left ***:All consecutive robotic left hepatectomy cases between December 2010 and January 2022 in Prince of Wales Hospital,the Chinese university of Hong Kong were retrieved from a prospectively collected *** gallbladder was preserved by moving the liver transection line just away from the gallbladder *** were divided into two groups:gallbladder preservation(GBP)and non-gallbladder preservation(NGBP).Operative results and long-term outcomes were compared between these two ***:There were 11 cases in the GBP group and 25 cases in the NGBP *** two groups were comparable in terms of the patient demographics and disease *** was no operative *** was no difference between the two groups in operative time(GBP 270 min *** 332 min,p=0.132),blood loss(GBP 50 mL *** 150 mL,p=0.115)or complication rate(GBP 27.3%*** 24.0%,p>0.999).There was also no difference in 5-year overall *** the GBP group,no patient developed specific symptoms or complications related to the preserved ***-up ultrasound or computed tomography revealed a normal appearance of the preserved gallbladders except in one patient who developed a 3-mm gallbladder *** the other hand,one(4%)patient in the NGBP group developed troublesome diarrhoea after ***:Gallbladder preservation is safe and feasible during robotic left *** preserved gallbladder does not lead to any symptoms,while postcholecystectomy diarrhoea can be avoided.

关键词： Robotic left hepatectomy Gallbladder preservation Cholecystectomy

Garcinol suppresses the growth of human hepatocellular carcinoma by inducing abrogation of STAT3 phosphorylation,acetylation and dimerization

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中国药理学与毒理学杂志 2015年第S1期29卷 82-83页

作者： Muthu K SHANMuGAM Snehajyoti CHATTERJEE Peramaiyan RAJENDRAN Feng LI Parijat SENAPATI Kwong fai wong Alan Prem KuMAR John MLuK Kam Man HuI Gautam SETHI Tapas K KuNDu Department of Pharmacology yong Loo Lin School of Medicine National university of Singapore Singapore 117957 Jawaharlal Nehru Centre for Advanced Scientific Research Molecular Biology and Genetics unit Transcription and Disease Laboratory Jakkur P.O. Bangalore 560064 India Cancer Science Institute of Singapore National university of Singapore Singapore 117456 School of Biomedical Sciences Faculty of Health Sciences Curtin university Western Australia Division of Cellular and Molecular Research Humphrey Oei Institute of Cancer Research National Cancer Centre Singapore 169610 Institute of Molecular and Cell Biology Singapore 138673

OBJECTIVE Hepatocellular carcinoma(HCC)is the fifth most common malignancy worldwide and the third cause of global cancer *** of signal transducer and activator of transcription 3(STAT3)is commonly observed in tumor cells and is a critical mediator of on cogenic signaling in HCC and controls the expression of several genes involved in proliferation,survival,metastasis and *** drug-targeted therapies,besides being expensive,are associated with serious side effects and ***,novel agents that can suppress STAT3 activation have potential for both prevention and treatment of *** the present report,we investigated whether the potent HAT/KAT inhibitor,garcinol,(apolyisoprenylatedbenzophenone),could suppress STAT3 activation in HCC cells and in nude mice *** The effect of garcinol on HCC cell lines wasdetermined by MTT assay,immunoblotting,DNA binding assays,immuno-fluorescenceand immune-histochemical *** effect of garcinolon the inhibition of tumor growth in vivo was also investigated using HCCxenograft tumor modelin athymic nu/nu *** We found that garcinol could inhibit constitutive STAT3 activation in a dose-and time-dependent manner both by inhibiting STAT3 phosphorylation and acetylation in HCC *** investigated for molecular mechanism(s),we found that garcinol interferes with the dimer formation of STAT3 thereby inhibits its nuclear *** modeling showed that garcinol could bind to the SH2 domain of STAT3 and suppresses its dimerization in *** understand the cellular mechanism(s)of inhibition of STAT3 function by garcinol,we observed that upon inhibition of STAT3 dimerization bygarcinol,STAT3 DNA binding ability gets *** inhibition of STAT3 activation by garcinol led to the suppression of various gene products involved in proliferation,survival,and ***,when administered i.p.,garcinol inhibited the growth of human HCC xenograft tumors in

关键词： STAT3 HCC garcinol acetylation dimerization nude m

Lithographic patterning of ferromagnetic FePt nanoparticles from a single-source bimetallic precursor containing hemiphasmidic structure for magnetic data recording media

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Science China Materials 2019年第4期62卷 566-576页

作者： Zhengong Meng Cheuk-Lam Ho Hon-fai wong Zhen-Qiang yu Nianyong Zhu Guijun Li Chi-Wah Leung Wai-y.ung wong College of Chemistry and Environmental Engineering Shenzhen University Institute of Molecular Functional Materials and Department of Chemistry Hong Kong Baptist University HKBu Institute of Research and Continuing Education Department of Applied Biology and Chemical Technology The Hong Kong Polytechnic University Department of Applied Physics The Hong Kong Polytechnic University

Patterning of L10 FePt nanoparticles(NPs) with high coercivity offers a promising route to develop bit-patterned media(BPM) for the next generation magnetic data recording system, but the synthesis of monodisperse FePt NPs and mass production of their nanopatterns has been a longstanding challenge. Here, highly efficient nanoimprint lithography was applied for large-scale universal patterning, which was achieved by imprinting the solution of a single-source bimetallic precursor. The rigid coplanar metallic cores and the surrounding flexible tails in the bimetallic complex permit the spontaneous molecular arrangements to form the highly ordered negative morphology replicated from the soft ***-situ pyrolysis study was then investigated by one-pot pyrolysis of the precursor under an Ar/H2 atmosphere, and the resultant NPs were fully characterized to identify the phase,morphology and magnetic properties. Finally, highly-ordered patterns on certain substrates were preserved perfectly after pyrolysis and could be potentially.utilized in magnetic data recording media.

关键词： FePt nanoparticles single-source bimetallic precursors one-pot pyrolysis lithographic patterning magnetic data recording media

Phenomapping approach to interpreting coronary dimensions in febrile children

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Pediatric Investigation 2022年第4期6卷 233-240页

作者： Haoxun Tang Xin Guo Xiaolu Nie Lin Zheng Gang Liu Wilfred Hing-Sang wong y.u-fai Cheung Heart Center Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Department of Infectious Disease Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Center for Clinical Epidemiology and Evidence-based Medicine Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Department of Echocardiography Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Department of Paediatrics and Adolescent Medicine School of Clinical MedicineThe University of Hong KongHong KongChina

Importance:Coronary artery dilation may occur in febrile children with and without Kawasaki disease(KD).Objective:We explored the application of unsupervised learning algorithms in the detection of novel patterns of coronary artery phenotypes in febrile children with and without ***:A total of 239 febrile children(59 non-KD and 180 KD patients),were *** hierarchical clustering analysis of phenotypic data including age,hemoglobin,white cell count,platelet count,C-reactive protein,erythrocyte sedimentation rate,albumin,alanine aminotransferase,aspartate aminotransferase,and coronary artery z scores were ***:using a cutoff z score of 2.5,the specificity was 98.3%and the sensitivity was 22.1%for differentiating non-KD from KD *** analysis identified three phenogroups that differed in a clinical,laboratory,and echocardiographic *** with phenogroup I,phenogroup III had the highest prevalence of KD(91%),worse inflammatory markers,more deranged liver function,higher coronary artery z scores,and lower hematocrit and albumin *** blood parameters in febrile children with z scores of coronary artery segmentsut KD identified useful laboratory parameters that aid the diagnosis of KD in febrile children with relatively normal-sized coronary arteries.

关键词： Coronary artery Febrile children Kawasaki disease Phenomapping

Estimation of daily PM2.5 concentration and its relationship with meteorological conditions in Beijing

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Journal of Environmental Sciences 2016年第10期28卷 161-168页

作者： Qian yin Jinfeng Wang Maogui Hu Hoting wong LREIS Institute of Geographic Sciences and Nature Resources Research Chinese Academy of Sciences Beijing 100101 China Jiangsu Center for Collaborative Innovation in Geographical Information Resource Development and Application Nanjing 210023 China

When investigating the impact of air pollution on health, particulate matter less than 2.5μm in aerodynamic diameter （PM2.5） is considered more harrnful than particulates of other sizes. Therefore, studies of PM2.5 have attracted more attention. Beijing, the capital of China, is notorious for its serious air pollution problem, an issue which has been of great concern to the residents, government, and related institutes for decades. However, in China, significantly less time has been devoted to observing PM2.5 than for PM10. Especially before 2013, the density of the PM2.5 ground observation network was relatively low, and the distribution of observation stations was uneven. One solution is to estimate PM2.5 concentrations from the existing data on PM10. In the present study, by analyzing the relationship between the concentrations of PM2.5 and PM10, and the meteorological conditions for each season in Beijing from 2008 to 2014, a u-shaped relationship was found between the daily maximum wind speed and the daily PM concentration, including both PM2.5 and PM10. That is, the relationship between wind speed and PM concentration is not a simple positive or negative correlation in these wind directions; their relationship has a complex effect, with higher PM at low and high wind than for moderate winds. Additionally, in contrast to previous studies, we found that the PM2.5/PM10 ratio is proportional to the mean relative humidity （MRH）. According to this relationship, for each season we established a multiple nonlinear regression （MNLR） model to estimate the PM2.5 concentrations of the missing periods.

关键词： PM10 concentrationPM2.5 concentration estimationWind speedWind direction

Impact of disease severity on gastric residual volume in critical patients

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World Journal of Gastroenterology 2011年第15期17卷 2007-2012页

作者： Chien-Wei Hsu Shu-Fen Sun David Lin Lee Shoa-Lin Lin Kam-fai wong Hsiu-Hua Huang Hung-Ju Li Department of Medicine School of Medicine National YangMing University Taipei Taiwan China Intensive Care unit Department of Medicine Kaohsiung Veterans General Hospital Kaohsiung City 813 Taiwan China Department of Physical Medicine and Rehabilitation Kaohsiung Veterans General Hospital Kaohsiung City 813 Taiwan China Institute of Statistics National University of Kaohsiung Kaohsiung City 813 Taiwan China Department of Nutrition Kaohsiung Veterans General Hospital Kaohsiung City 813 Taiwan China

AIM: To investigate whether illness severity has an impact on gastric residual volume (GRV) in medical critically ill patients. METHODS: Medical intensive care unit (ICu) patients requiring nasogastric feeding were enrolled. Sequential Organ failure Assessment (SOFA) score was assessed immediately preceding the start of the study. Acute Physiology and Chronic Health Evaluation (APACHE) Ⅱ scores were recorded on the first, fourth, seventh, and fourteenth day of the study period. GRV was measured every 4 h during enteral feeding. The relationship be-tween mean daily GRV and SOFA scores and the correlation between mean daily GRV and mean APACHE Ⅱ score of all patients were evaluated and compared. RESuLTS: Of the 61 patients, 43 patients were survivors and 18 patients were non-survivors. The mean daily GRV increased as SOFA scores increased (P ysis of variance). Mean APACHE Ⅱ scores of all patients correlated with mean daily GRV (P = 0.011, Pearson correlation) during the study period. Patients with decreasing GRV in the first 2 d had better survival than patients without decreasing GRV (P = 0.017, log rank test). CONCLuSION: GRV is higher in more severely ill medical ICu patients. Patients with decreasing GRV had lower ICu mortality than patients without decreasing GRV.

关键词： Critical care Outcome Residual volume Severity of illness index Tube feeding

Efficient predictions of formation energies and convex hulls from density functional tight binding calculations

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Journal of Materials Science & Technology 2023年第10期141卷 236-244页

作者： Anshuman Kumar Zulfikhar A.Ali Bryan M.wong Materials Science&Engineering Program University of California-RiversideRiverside92521CAUSA Department of Physics&Astronomy University of California-RiversideRiverside92521CAUSA Materials Science&Engineering Program Department of Chemical&Environmental EngineeringDepartment of Physics&Astronomyand Department of ChemistryUniversity of California-RiversideRiverside92521CAUSA

Defects in materials significantly alter their electronic and structural properties,which affect the per-formance of electronic devices,structural alloys,and functional ***,calculating all the possible defects in complex materials with conventional Density Functional Theory(DFT)can be compu-tationally *** enhance the efficiency of these calculations,we interfaced Density Functional Tight Binding(DFTB)with the Clusters Approach to Statistical Mechanics(CASM)software package for the first *** SiC and ZnO as representative examples,we show that DFTB gives accurate results and can be used as an efficient computational approach for calculating and pre-screening formation ener-gies/convex *** DFTB+CASM implementation allows for an efficient exploration(up to an order of magnitude faster than DFT)of formation energies and convex hulls,which researchers can use to probe other complex systems.

关键词： CASM DFTB DFT Formation energy Convex hull

Chiral multiple-resonance thermally activated delayed fluorescence materials based on chiral spiro-axis skeleton for efficient circularly polarized electroluminescence

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Science China Chemistry 2024年第7期67卷 2257-2264页

作者： Shi-Quan Song Xiao Han Zhong-Zhong Huo Ching-fai yip Xian-Fang Hong Meng-Ning Ding y.u-Xuan Zheng State Key Laboratory of Coordination Chemistry Jiangsu Key Laboratory of Advanced Organic MaterialsSchool of Chemistry and Chemical EngineeringNanjing UniversityNanjing 210023China Key Laboratory of Mesoscopic Chemistry of Ministry of Education School of Chemistry and Chemical EngineeringNanjing UniversityNanjing 210023China

Chiral luminescence materials have potential applications in the field of three-dimensional displays due to their circularly polarized luminescence(CPL)***,the further development of circularly polarized organic light-emitting diodes(CP-OLEDs)needs to meet the requirements of high efficiency,high color purity,low cost,and high dissymmetry factor(gPLor gEL),chiral multiple resonance thermally activated delayed fluorescence(MR-TADF)materials are considered as candidates in these ***,based on a pair of chiral spirofluorene precursors,two pairs of high-performance chiral MR-TADF emitters((R/S)-p-Spiro-DtBuCzB and(R/S)-m-Spiro-DtBuCzB)are developed,which exhibit strong emissions peaking at 491 and 502 nm in toluene with full-width at half-maximum values of 25 and 33 nm,*** addition,small singlet–triplet energy gaps of 0.15 and 0.10 eV with high absolute photoluminescence efficiencies of 95.0%and 96.7%are observed for p-Spiro-DtBuCzB and m-Spiro-DtBuCzB molecules,*** based on p-Spiro-DtBuCzB and m-Spiro-DtBuCzB display high maximum external quantum efficiencies of 29.6%and 33.8%,*** importantly,CP-OLEDs present symmetric circularly polarized electroluminescence spectra with|gEL|factors of 3.36×10^(-4)and 7.66×10^(-4)for devices based on(R/S)-p-Spiro-DtBuCzB and(R/S)-m-Spiro-DtBuCzB enantiomers,respectively.

关键词： chiral spiro-axis skeleton-based material multiple resonance thermally activated delayed fluorescence circularly polarized electroluminescence dissymmetry factor external quantum efficiency

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