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Epidemiology and natural history of Wilson's disease in the Chinese: A territory-based study in Hong Kong between 2000 and 2016

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World Journal of Gastroenterology 2017年第43期23卷 7716-7726页

作者： Ka-Shing Cheung Wai-Kay Seto james fung Lung-Yi Mak Ching-Lung Lai Man-fung Yuen Department of Medicine The University of Hong KongQueen Mary Hospital State Key Laboratory for Liver Research The University of Hong Kong

AIM To investigate the epidemiology and natural history of Wilson's disease in the *** Data were retrieved via electronic search of hospital medical registry of the Hong Kong Hospital Authority,which covers all the public healthcare services. We identified cases of Wilson's disease between 2000 and 2016 by the International Classification of Diseases(ICD)-9 code. We analyzed the incidence rate,prevalence and adverse outcomes of Wilson's *** We identified 211 patients(male cases 104; female cases 107; median age 27.2 years,IQR: 17.1-38.6 years; duration of follow-up 8.0 years,IQR: 5.0-14.0 years). The average annual incidence rate was 1.44 per million person-years while the prevalence was 17.93 per million. Between 2000 and 2016,there was a decrease in the annual incidence rate from 1.65 to 1.23 per million person-years(P = 0.010),whereas there was an increase in the annual prevalence from 7.80 to 25.20 per million(P < 0.001). Among the 176 cases with hepatic involvement,38(21.6%) had cirrhosis,three(1.7%) developed hepatocellular carcinoma,24(13.6%) underwent liver transplantations,and 26(14.8%) died. Seven patients had concomitant chronic viral hepatitis B or C. The 5-year and 10-years rates of overall survival were 92.6% and 89.5%,and for transplant-free survival rates 91.8% and 87.4%,respectively. Cirrhosis and possibly chronic viral hepatitis were associated with poorer overall survival. CONCLUSION There was a significant increase in the prevalence of Wilson's disease in Hong Kong. The prognosis was favorable except for those with cirrhosis or concomitant viral hepatitis.

关键词： Hepaticolenticular degeneration Cirrhosis Hepatocellular carcinoma Transplantation

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Prediction of hepatocellular carcinoma development by aminotransferase to platelet ratio index in primary biliary cholangitis

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World Journal of Gastroenterology 2017年第44期23卷 7863-7874页

作者： Ka-Shing Cheung Wai-Kay Seto james fung Lung-Yi Mak Ching-Lung Lai Man-fung Yuen Department of Medicine The University of Hong Kong Queen Mary Hospital State Key Laboratory for Liver Research The University of Hong Kong

AIM To investigate the usefulness of aspartate aminotransferase to platelet ratio index(APRI) in predicting hepatocellular carcinoma(HCC) risk in primary biliary cholangitis(PBC).METHODS We identified PBC patients between 2000 and 2015 by searching the electronic medical database of a tertiary center. The hazard ratio(HR) of HCC with different risk factors was determined by Cox proportional hazards model. RESULTS One hundred and forty-four PBC patients were recru-ited. Patients were diagnosed at a median age of 57.8 years [interquartile range(IQR): 48.7-71.5 years), and 41(28.5%) patients had cirrhosis at baseline. The median follow-up duration was 6.9 years(range: 1.0-26.3 years). Twelve patients developed HCC, with an incidence rate of 10.6 cases per 1000 patient-years. The overall 5-, 10-and 15-year cumulative incidences of HCC were 2.3% 95%CI: 0%-4.8%), 8.4%(95%CI: 1.8%-14.5%) and 21.6%(6.8%-34.1%), respectively. Older age(HR = 1.07), cirrhosis(HR = 4.38) and APRI at 1 year after treatment(APRI-r1) > 0.54(HR = 3.94) were independent factors for HCC development. APRI-r1, when combined with treatment response, further stratified HCC risk(log rank P < 0.05). The area under receiver operating curve of APRI-r1 in predicting HCC was 0.77(95%CI: 0.64-0.88).CONCLUSION APRI-r1 can be used to predict the development of HCC in PBC patients. Combination of APRI-r1 with treatment response can further stratify the HCC risk.

关键词： Aspartate aminotransferase Platelet ratio index Hepatocellular carcinoma Primary biliary cholangitis Ursodeoxycholic acid Cirrhosis

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Study of Dynamics and Kinetics of β-Lactamases towards Antibiotics by Hydrogen/Deuterium Exchange Mass Spectrometry

Study of Dynamics and Kinetics of β-Lactamases towards Anti...

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2018年中国质谱学术大会（CMSC2018）

作者： tsz-fung wong Pui-Kin So Liwen Huang Zhong-Ping Yao Department of Applied Biology & Chemical Technology The Hong Kong Polytechnic University

Production of β-lactamases renders bacteria invulnerable towards β-lactam antibiotics. The caused antibiotic resistance of bacteria has become a severe health threat worldwide. In recent years, extended spectrum β-lactamases(ESBLs), which are mutated from wild type β-lactamases and are insusceptible to even later generation antibiotics, have been discovered. Understanding the dynamics and kinetics of β-lactamases towards antibiotics are essential for new antibiotics development. Hydrogen/deuterium exchange mass spectrometry(HDX-MS) is a powerful technique to study dynamics and conformational changes of proteins. In this study, structural and dynamic properties of a series of mutants of TEM-1, a class A β-lactamase, were investigated by HDX-MS. Results of global HDX-MS revealed that the flexibility of TEM-ESBLs was higher than that of TEM-1. Local HDX-MS results further revealed that the binding pocket of TEM-ESBLs was expanded, enabling better accommodation of bulky side chains of third generation cephalosporins and thus the enhanced hydrolytic power of TEM-ESBLs.

关键词： Hydrogen/deuterium exchange mass spectrometry extended spectrum β-lactamases antibiotics dynamics kinetics

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HepG2 cells recovered from apoptosis show altered drug responses and invasiveness

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Hepatobiliary & Pancreatic Diseases International 2014年第3期13卷 293-300页

作者： Shan-Shan Wang Xin Xie Chung Sing Timothy wong Pui Ying Choi Ming Chiu fung School of Life Sciences the Chinese University of Hong Kong Department of Anatomical and Cellular Pathology Prince of Wales Hospital the Chinese University of Hong Kong

BACKGROUND: Cancer relapse, associated with increased drug resistance and rate of metastasis, often follows completion of chemotherapy but the cancer escape mechanisms are still incompletely understood. Percutaneous ethanol injection(PEI)has been used for treating hepatocellular carcinoma(HCC)for decades, while the recurrence after PEI treatment remains a major limitation. Recent evidence mounted that cancer cells could survive from chemical induced apoptosis, suggesting a potential route through which cancer relapse may occur. This study focuses on the consequence of HepG2 recovery from ethanol-induced apoptotic ***: The model of HepG2 recovery from ethanolinduced apoptotic event was established by live cell imaging,BrdU assay and Western blotting. MTT assay, wound healing assay and invasion assay were used to investigate the behavior of HepG2 after ***: HepG2 cells could recover from ethanol-induced apoptosis. These cells changed their behaviors such as drug resistance, mobility and invasiveness. On average, the recovered HepG2 cell clones were found to be 46% more resistant to ethanol and 84% higher in mobility. The recovered clones became 58.2% more sensitive to ***: HepG2 cells can recover from ethanolinduced apoptotic event. These cells became more resistant to ethanol and more invasive. Although the recovered cell clones were more resistant to ethanol, they became more sensitive to5-fluorouracil treatment.

关键词： cancer recurrence apoptosis recovery liver cancer percutaneous ethanol injection 5-fluorouracil

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Parents making surgical decisions for their children:a pilot study

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World Journal of Pediatric Surgery 2023年第2期6卷 115-117页

作者： Adrian Chi Heng fung Kenneth Kak Yuen wong Department of Surgery The University of Hong KongHong KongChina

Parents play a key role in medical decision-making for their children,who are limited by their intellectual development in under-standing their own health status and treat-ment plans.1 These decisions are challenging as they may have a lifelong impact on patients'physical and mental well-being,and a signif-icant portion of parents are not satisfied with the informed consent *** factors affecting parental satisfaction with the informed consent process have been identi-fied,including parents'knowledge base,the specific types of decisions and the presence of decisional conflicts.

关键词： children their parents

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Attenuation of mitochondrial,but not cytosolic,Ca2+ overload reduces myocardial injury induced by ischemia and reperfusion

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Acta Pharmacologica Sinica 2006年第7期27卷 911-918页

作者： Kenneth WL KAM james SK SHAM Tlak-ming wong Department of Physiology the University of Hong KongHong Kong SARChina Division of Pulmonary Critical Care Medicine Johns Hopkins School of MedicineBaltimoreMaryland 21224USA

Aim:Attenuation of mitochondrial Ca2+([Ca2+]m),but not cytosolic Ca2+([Ca2+]c),overload improves contractile *** hypothesized that attenuation of[Ca2+]m,but not[Ca2+]c,overload confers cardioprotection against ischemia/reperfusion-induced ***:Infarct size from isolated perfused rat heart,cell viability,and electrically-induced Ca2+transient in isolated rat ventricularmyocytes were *** determined the effects of BAPTA-AM,a Ca2+chelator,at concentrations that abolish the overload of both[Ca2+]cand[Ca2+]m,and ruthenium red,an inhibitor of mitochondrial uniporter of Ca2+transport,atconcentrations that abolish the overload of[Ca2+]m,but not[Ca2+]c,on cardiacinjury induced by ischemia/***:Attenuation of both[Ca2+]mand[Ca2+]cby BAPTA-AM,and attenuation of[Ca2+]m,but not[Ca2+]c,overloadby ruthenium red,reduced the cardiac injury observations,indicating the impor-tance of[Ca2+]min cardioprotection and contractile recovery in response to is-chemia/***:The study has provided unequivocal evidenceusing a cause-effect approach that attenuation of[Ca2+]m,but not[Ca2+]c,over-load is responsible for cardioprotection against ischemia/*** also confirmed the previous observation that attenuation of[Ca2+]m,but not[Ca2+]c,by ruthenium red improves contractile recovery following ischemia/reperfusion.

关键词： ruthenium red BAPTA-AM infarct size electrically-induced Ca2+transient isolated perfused rat heart cardiac myocytes

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Biliary endoscopy in the management of primary sclerosing cholangitis and its complications

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Liver Research 2019年第2期3卷 106-117页

作者： Brian M.fung james H.Tabibian University of California Los Angeles-Olive View Internal Medicine Residency Program SylmarCAUSA Division of Gastroenterology Department of MedicineOlive View-University of California Los Angeles Medical CenterSylmarCAUSA

Primary sclerosing cholangitis(PSC)is a chronic,idiopathic,cholestatic liver disease characterized by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile *** can affect individuals of all age groups and gender,has no established pharmacotherapy,and is associated with a variety of neoplastic(***)and non-neoplastic(*** strictures)hepatobiliary *** these considerations,endoscopy plays a major role in the care of patients with *** this review,we discuss and provide updates regarding endoscopic considerations in the management of hepatobiliary manifestations and complications of *** evidence is limited,we suggest pragmatic approaches based on currently available data and expert opinion.

关键词： Primary sclerosing cholangitis(PSC) Biliary tract disease Balloon dilation Cholangioscopy Dominant stricture Cholangiocarcinoma(CCA) Endoscopic retrograde cholangiopancreatography(ERCP)

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Validated model for prediction of recurrent hepatocellular carcinoma after liver transplantation in Asian population

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World Journal of Gastrointestinal Oncology 2019年第4期11卷 322-334页

作者： Ka Wing Ma wong Hoi She Albert Chi Yan Chan Tan To Cheung james Yan Yue fung Wing Chiu Dai Chung Mau Lo Kenneth Siu Ho Chok Department of Surgery the University of Hong Kong Department of Surgery and State Key Laboratory for Liver Research the University of Hong Kong Department of Medicine and State Key Laboratory for Liver Research The University of Hong Kong

BACKGROUND Liver transplantation(LT) is regarded as the best treatment for both primary and recurrent hepatocellular carcinoma(HCC). Post-transplant HCC recurrence rate is relatively low but significant, ranging from 10%-30% according to different series. When recurrence happens, it is usually extrahepatic and associated with poor prognosis. A predictive model that allows patient stratification according to recurrence risk can help to individualize post-transplant surveillance protocol and guidance of the use of anti-tumor immunosuppressive *** To develop a scoring system to predict HCC recurrence after LT in an Asian *** Consecutive patients having LT for HCC from 1995 to 2016 at our hospital were recruited. They were randomized into the training set and the validation set in a60:40 ratio. Multivariable Cox regression model was used to identity factors associated with HCC recurrence. A risk score was assigned to each factor according to the odds ratio. Accuracy of the score was assessed by the area under the receiver operating characteristic *** In total, 330 patients were eligible for analysis(183 in training and 147 invalidation). Recurrent HCC developed in 14.2% of them. The median follow-up duration was 65.6 mo. The 5-year disease-free and overall survival rates were78% and 80%, respectively. On multivariate analysis, alpha-fetoprotein > 400 ng/mL [P = 0.012, hazard ratio(HR) 2.92], sum of maximum tumor size and number(P = 0.013, HR 1.15), and salvage LT(P = 0.033, HR 2.08) were found to be independent factors for disease-free survival. A risk score was calculated for each patient with good discriminatory power(c-stat 0.748 and 0.85, respectively,in the training and validation sets). With the derived scores, patients were classified into low-(0–9), moderate-(> 9–14), and high-risk groups(> 14), and the risk of HCC recurrence in the training and validation sets was 10%, 20%, 54%(cstat 0.67) and 4%, 22%, 62%(c-stat 0.811), accordin

关键词： Hepatocellular carcinoma Liver transplantation Post-transplant recurrence Predictive model

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Crocodile blood supplementation protect svascular function in diabetic mice

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Food Production, Processing and Nutrition 2021年第1期3卷 283-295页

作者： Chui Yiu Bamboo Chook Francis M.Chen Gary Tse fung Ping Leung Wing Tak wong School of Life Sciences Faculty of ScienceThe Chinese University of Hong KongShatinNTHong Kong SAR State Key Laboratory of Agrobiotechnology The Chinese University of Hong KongHong KongChina

Cardiovascular disease is a major cause of mortality in diabetic patients due to the heightened oxidative stress and pro-inflammatory state in vascular *** approaches targeting cardiovascular health for diabetic patients are urgently *** blood,an emerging dietary supplement,was suggested to have anti-oxidative and anti-inflammatory effects in vitro,which have yet to be proven in animal *** study thereby aimed to evaluate whether crocodile blood can protect vascular function in diabetic mice against oxidation and *** db/db mice and their counterparts db/m+mice were treated daily with crocodile blood soluble fraction(CBSF)or vehicle via oral gavage for 4 weeks before their aortae were harvested for endothelium-dependent relaxation(EDR)quantification using wire myograph,which is a well-established functional study for vascular function *** culture experiments culturing mouse aortae from C57BL/6 J mice with or without IL-1βand CBSF were done to evaluate the direct effect of CBSF on endothelial *** oxygen species(ROS)levels in mouse aortae were assessed by dihydroethidium(DHE)staining with inflammatory markers in endothelial cells quantified by quantitative polymerase chain reaction(qPCR).CBSF significantly improved deteriorated EDR in db/db diabetic mice through both diet supplementation and direct culture,with suppression of ROS level in mouse *** also maintained EDR and reduced ROS levels in mouse aortae against the presence of pro-inflammatory IL-1β.Under the pro-inflammatory state induced by IL-1β,gene expressions of inflammatory cytokines were downregulated,while the protective transcripts UCP2 and SIRT6 were upregulated in endothelial *** study suggests a novel beneficial effect of crocodile blood on vascular function in diabetic mice and that supplementation of diet with crocodile blood may act as a complementary approach to protect against vascular diseases through anti

关键词： Crocodile blood Diabetes mellitus Vascular endothelial function Anti-oxidative Anti-inflammatory

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Outcomes of radical prostatectomy in a 20-year localized prostate cancer single institution series in China

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Asian Journal of Andrology 2023年第3期25卷 345-349页

作者： Xiao-Hao Ruan Tsun Tsun Stacia Chun Da Huang Hoi-Lung wong Brian Sze-Ho Ho Chiu-fung Tsang Terence Chun-Ting Lai Ada Tsui-Lin Ng Rong Na james Hok-Leung Tsu Division of Urology Department of SurgeryThe University of Hong KongHong KongChina Department of Urology Ruijin HospitalShanghai Jiao Tong University School of MedicineShanghai 200025China Division of Urology Department of SurgeryQueen Mary HospitalHong KongChina

The long-term survival outcomes of radical prostatectomy(RP)in Chinese prostate cancer(PCa)patients are poorly *** conducted a single-center,retrospective analysis of patients undergoing RP to study the prognostic value of pathological and surgical *** April 1998 to February 2022,782 patients undergoing RP at Queen Mary Hospital of The University of Hong Kong(Hong Kong,China)were included in our *** Cox regression analysis and Kaplan–Meier analysis with stratification were *** 5-year,10-year,and 15-year overall survival(OS)rates were 96.6%,86.8%,and 70.6%,respectively,while the 5-year,10-year,and 15-year PCa-specific survival(PSS)rates were 99.7%,98.6%,and 97.8%,*** International Society of Urological Pathology PCa grades(ISUP Grade Group)≥4 was significantly associated with poorer PSS(hazard ratio[HR]=8.52,95%confidence interval[CI]:1.42–51.25,P=0.02).Pathological T3 stage was not significantly associated with PSS or OS in our *** node invasion and extracapsular extension might be associated with worse PSS(HR=20.30,95%CI:1.22–336.38,P=0.04;and HR=7.29,95%CI:1.22–43.64,P=0.03,respectively).Different surgical approaches(open,laparoscopic,or robotic-assisted)had similar outcomes in terms of PSS and *** conclusion,we report the longest timespan follow-up of Chinese PCa patients after RP with different approaches.

关键词： China oncologic outcome prostate cancer radical prostatectom

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