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Analysis of inborn errors of metabolism： disease spectrum for expanded newborn screening in Hong Kong

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Chinese Medical Journal 2011年第7期124卷 983-989页

作者： Han-Chih Hencher Lee Chloe Miu Mak Ching-Wan lam Yuet-Ping Yuen, Angel On-Kei Chan Chi-Chung Shek Tak-Shing Siu Chi-Kong Lai Chor-kwan Ching Wai-kwan Siu Sammy Pak-lam Chen Chun-Yiu Law Morris Hok-Leung Tai Sidney Tam Albert Yan-Wo Chan Chemical Pathology Laboratory Department of Pathology Princess Margaret Hospital Hong Kong SAR China Division of Clinical Biochemistry Queen Mary Hospital Hong Kong SAR China Department of Pathology Li Ka Shing Faculty of Medicine The University of Hong Kong Hong Kong SAR China Chemical Pathology Laboratory Department of Pathology Queen Elizabeth Hospital Hong Kong SAR China

Background Data of classical inborn errors of metabolism （IEM） of amino acids, organic acids and fatty acid oxidation are largely lacking in Hong Kong, where mass spectrometry-based expanded newborn screening for IEM has not been initiated. The current study aimed to evaluate the approximate incidence, spectrum and other characteristics of classical IEM in Hong Kong, which would be important in developing an expanded newborn screening program for the local area.

关键词： biochemical genetics chemical pathology expanded newborn screening Hong Kong," inborn errorsof metabolism tandem mass spectrometry

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Phenylketonuria in Hong Kong Chinese： a call for hyperphenylalaninemia newborn screening in the Special Administrative Region, China

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Chinese Medical Journal 2011年第16期124卷 2556-2558页

作者： Chloe Miu Mak Chun-hung Ko Ching-wan lam Wai-ling Lau Wai-kwan Siu Sammy Pak-lam Chen Chun-yiu Law Chi-kong Lai Chak-man Yu Albert Yan-wo Chan Kowloon West Cluster Laboratory Genetic Service ChemicalPathology Laboratory Department of Pathology Princess MargaretHospital Hong Kong China Department of Paediatrics and Adolescent Medicine CaritasMedical Centre Hong Kong China Department of Pathology Li Ka Shing Faculty of Medicine TheUniversity of Hong Kong Hong Kong China

Hyperphenylalaninemia is one of the commonest inborn errors of metabolism affecting approximately 1 in 15 000 live births. Among Chinese, BH4 deficiency leading to hyperphenylalaninemia is much commoner than in Caucasians. Exact diagnosis is important for the treatment and genetic counseling. In 2000, newborn screening for phenylketonuria is mandatory by law in China throughout the whole country. However, it is not yet included in the newborn screening program of the Hong Kong Special Administrative Region, China. Published data on hyperphenylalaninemia among Hong Kong Chinese are largely lacking. We report a 1-year-old Hong Kong Chinese girl with severe 6-pyruvoyl-tetrahydropterin synthase （PTPS） deficiency. The patient presented with infantile hypotonia and was misdiagnosed as cerebral palsy. She had very mild hyperphenylalaninemia （95 IJmol/L）, significantly high phenylalnine-to-tyrosine ratio （3.1）, and ele~,ated prolactin of 1109 m lU/L. Genetic analysis confirmed a homozygous known disease-causing mutation PTS NM_000317.1： c.259C〉T; NP_000308.1： p.P87S in the proband. In our local experience, while the estimated prevalence of hyperphenylalaninemia due to PTPS deficiency was reported to be 1 in 29 542 live births, not a single case of phenylalanine hydroxylase deficiency has been reported. Furthermore, there is a general lack of awareness of inherited metabolic diseases in the community as well as among the medical professionals. Very often, a low index of clinical suspicion will lead to delay in diagnosis, multiple unnecessary and costly investigations, prolonged morbidity and anxiety to the family affected. We strongly recommend that expanded newborn screening for hyperphenylalaninemia should be implemented for every baby born in the Hong Kong Special Administrative Region, China.

关键词： Hong Kong Chinese hyperphenylalaninemia newborn screening phenylketonuria 6-pyruvoyl-tetrahydropterin synthase deficiency

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Incidence and predictors of upper gastrointestinal bleeding in patients receiving low-dose aspirin for secondary prevention of cardiovascular events in patients with coronary artery disease

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World Journal of Gastroenterology 2006年第18期12卷 2923-2927页

作者： William Ng Wai-Man Wong Wai-Hong Chen Hung-Fat Tse Pui-Yin Lee Kam-Chuen Lai sheung-Wai Li Matthew Ng Kwok-Fai lam Xi Cheng Chu-Pak Lau Division of Cardiology University Department of Medicine Queen Mary Hospital Hong Kong SAR China Division of Gastroenterology University Department of Medicine Queen Mary Hospital Hong Kong SAR China Division of Rehabilitation Medicine University Department of Medicine Queen Mary Hospital Hong Kong SAR China Department of Statistics and Actuarial Science University of Hong Kong Hong Kong SAR China

AIM： The use of low-dose aspirin to prevent cardiovascular disease events is well established. However, the incidence and predictors of upper gastrointestinal bleeding （UGIB） with its use are unknown. We studied prospectively the incidence and outcome of peptic ulceration in low-dose aspirin users. METHODS： A total of 991 patients with coronary artery disease （CAD） on low-dose aspirin were prospectively followed-up for two years for the occurrence and clinical features of first hospitalized episode of UGIB. RESULTS： UGIB had a bimodal presentation with 45% occurring within four months of aspirin initiation and had an overall prevalence of 1.5% per year. There was no UGIB-related death. Hypertension （OR = 4.6, 95%CI 1.5 - 14.7, P = 0.009）, history of peptic ulceration （OR = 3.1, 95%CI 1.1 - 9.0, P = 0.039）, tertiary education （OR = 3.08, 95%CI 1.1 - 9.0, P = 0.039） and higher lean body mass （P = 0.016） were independent factors associated with UGIB. Use of nitrate did not reduce UGIB. CONCLUSION： The incidence of UGIB in patients with CAD on long-term low-dose aspirin is low, but is accompanied with significant morbidity. With prolonged use of aspirin, UGIB continues to be a problem for those with risk factors and especially in patients with a history of peptic ulcers, in which UGIB tends to occur early after aspirin therapy.

关键词： Coronary artery disease, Aspidn Gastrointestinal bleeding

HM30181A,a potent P-glycoprotein inhibitor,potentiates the absorption and in vivo antitumor efficacy of paclitaxel in an orthotopic brain tumor model

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Cancer Biology & Medicine 2020年第4期17卷 986-1001页

作者： Wu Zeng Betty Yuen kwan Law Vincent Kam Wai Wong Denise So Bik Chan Simon Wing Fai Mok Joyce Jia Ying Gao Rebecca Ka Yan Ho Xu Liang Jia Hao Li Ming Tsung Lee Weng Li Yoon Michael P Smolinski Johnson Yiu Nam Lau Christopher Wai Kei lam Manson Fok State Key Laboratory of Quality Research in Chinese Medicines Macao University of Science and TechnologyMacaoChina At he nex Hong Kong Irmo vative Limited Hong Kong China Faculty of Medici ne Macao Un iversity of Science and TechnologyMacaoChina Athenex Inc. New York 14203USA

Objective:Delivery of chemotherapeutic drugs to the brain has remained a major obstacle in the treatment of glioma,owing to the presence of the blood-brain barrier and the activity of P-gp,which pumps its substrate back into the systemic *** aim of the present study was to develop an intravenous formulation of HM30181 A(HM)to inhibit P-gp in the brain to effectively deliver paclitaxel(PTX)for the treatment of malignant ***:Two formulations of solubilized HM were designed on the basis of different solid dispersion strategies:i)spray-drying[polyvinlypyrrolidone(PVP)-HM]and ii)solvent evaporation[HP-β-cyclodextrin(cyclodextrin)-HM].The P-gp inhibition of these 2 formulations was assessed on the basis of rhodamine 123 uptake in cancer *** and brain pharmacokinetic parameters were also determined,and the antitumor effect of cyclodextrin-HM with PTX was evaluated in an orthotopic glioma xenograft mouse ***:Although both PVP-HM and cyclodextrin-HM formulations showed promising P-gp inhibition activity in vitro,cyclodextrin-HM had a higher maximum tolerated dose in mice than did *** study of cyclodextrin-HM revealed a plasma concentration plateau at 20 mg/kg,and the mice began to lose weight at doses above this ***-HM(10 mg/kg)administered with PTX at 10 mg/kg showed optimal antitumor activity in a mouse model,according to both tumor volume measurement and survival time(P<0.05).Conclusions:In a mouse orthotopic brain tumor model,the intravenous co-administration of cyclodextrin-HM with PTX showed potent antitumor effects and therefore may have potential for glioma therapy in humans.

关键词： HM30181A glioma pharmacokinetics paclitaxel P-glycoprotein

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Environment Pollution and Human Health

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中国药理学与毒理学杂志 2006年第3期 174-177页

作者： Jia Gao Huan-Jun Tian Wei-Qun Shu Eun-Jung Park kwangsik Park Paul kwan-Sing lam T Prapamontol U Mevatee C Puaninta A Mangklabrucks N Udomvong T Kaewthummanukul S Sriboonruang S Nimsakul C Kingkeow Werawan Ruangyuttikarn

S5-1 Effects of persistent organic pollutants(POPs) in water on Chongqing population health CAO Jia, TIAN Huan-Jun, SHU Wei-Qun(Preventive Medicine College, the Third Military Medical University, Chongqing 400038, China)Abstract:Many organic pollutants occur in drinking water in considerable amounts, and mimic or interfere with reproductive development and function. Furthermore, it is not known whether multiple organic pollutants (MOP) from drinking water induce uterotrophic effects. In this study, we compared the effects of MOP on uterine wet weight by different exposure approaches (ip, sc, po) on prepubertal 21-d-old Wistar rats and Kunming mice. The multiple organic pollutants in drinking water were concentrated by solid-phase extraction (SPE), and dissolved in DMSO.

关键词： PM Environment Pollution and Human Health

Inflammatory pseudotumor of the liver in association with a gastrointestinal stromal tumor: A case report

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World Journal of Gastroenterology 2004年第12期10卷 1841-1843页

作者： Oswens S.Lo Ronnie T.Poon Chi Ming lam sheung Tat Fan Centre for the Study of Liver Disease and Department of Surgery The University of Hong KongPokfulamHong KongChina

Inflammatory pseudotumor of the liver is a rare benign lesion that can mimic a malignant liver nioplasm. A case of inflammatory pseudotumor of the liver found in association with a malignant gastrointestinal stromal tumor (GIST) of the small bowel was reported. The inflammatory pseudotumor was misdiagnosed as a metastasis from the GIST by frozen section. A correct diagnosis was made only after histopathological examination of the paraffin section of the resescted specimen. This case is psrticularly interesting because of the association of the two rare pathological entities and the diagnostic dilemma that arose from the similarity of their histological appearances. To our knowledge，this association has not been reported in the literature.

关键词：炎性肿瘤肝脏胃肠基质瘤 GIST 病理组织学

Cerebral vasodilator activities of major active constituents of a Danshen and Gegen formulation on rat basilar artery

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Cerebral vasodilator activities of major active constituents...

Leptin Signaling Protects NK Cells from Apoptosis During Development in Mouse Bone Marrow

中国药理学会第十一次全国学术会议

作者： ESK NG JHK YEUNG YW kwan CBS LAU JCM KOON PC LEUNG KP FUNG FFY lam School of Biomedical Sciences Faculty of MedicineThe Chinese University of Hong Kong Institute of Chinese Medicine The Chinese University of Hong Kong State Key Laboratory of Phytochemistry and Plant Resources in West China The Chinese University of Hong Kong

OBJECTIVE Danshen and Gegen are traditional Chinese medicines commonly used for the treatment of cardiovascular *** this study,we have identified the major constituents of a Danshen and Gegen formulation(DG;ratio 7:3) and investigated their actions on rat-isolated cerebral basilar *** Rat basilar artery rings were precontracted with 100 nmol·L *** of endothelium-dependent mechanisms was investigated by mechanical removal of the *** cyclase, guanylyl cyclase,and potassium channel involvement were examined by pretreatment of the artery rings with their respective inhibitors. Calcium channel involvement was tested in artery rings incubated with Ca-free buffer and primed with U46619 prior to adding CaCl to elicit *** constituents of a DG water extract were identified by *** Salvianolic acid B, danshensu,puerarin,daidzein and daidzin were identified in the DG water *** these agents produced concentration-dependent relaxation of the artery rings that were unaffected by adenylyl cyclase inhibitor,guanylyl cyclase inhibitor,or by endothelium removal, except the latter reduced the maximum response to puerarin by 28%.Moreover,puerarin had no influence on CaCl-induced vasocon-striction but all the other agents produced concentration-dependent *** with a combination of K channel inhibitors produced significant inhibition on the vasodilator actions to all these agents,but not on *** The vasorelaxant actions of salvianolic acid B,daidzein and daidzin involved opening of K channels and inhibition of Ca influx in the vascular smooth muscle *** produced vasorelaxation solely by inhibition of Ca influx in the vascular smooth muscle *** contrast, puerarin produced vasodilatation via an endothelium-dependent mechanism and an endothelium-independent pathway mediated by the opening of K *** spite of differences in their mechanisms of actions,the co

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Cellular & Molecular Immunology 2009年第5期6卷 353-360页

作者： Cherry Kam Chun Lo Queenie Lai kwan lam Min Yang King-Hung Ko Lingyun Sun Rui Ma Shengjun Wang Huaxi Xu Sidney Tam Chang-You Wu Bo-Jiang Zheng Liwei Lu Departments of Pathology and Microbiology Center of Infection andImmunology The University of Hong Kong Hong Kong China Department of Rheumatology Drum Tower Hospital Nanjing UniversityMedical School Nanjing China Department of Immunology School of Medical Science and LaboratoryMedicine Jiangsu University. Zhenjiang China epartment of Pathology Queen Mary Hospital Hong Kong China Department of Immunology Sun Yat-Sen University Guangzhou China These authors contributed equally to this work Department of Pathology and Center of Infection and Immunology The University of Hong Kong Pokfulam Road Hong Kong China

Increasing evidence indicates a role of leptin in immune response, but it remains largely unclear whether leptin signaling is involved in regulating NK cell development in the bone marrow （BM）. In this study, we have characterized NK cell differentiation and maturation in the BM of leptin-receptor deficient db/db mice at a prediabetic stage. Although the BM cellularity was similar to the control value, the total number of NK cells was severely reduced in mutant mice. Flow cytometric analysis of db/db BM cells revealed significantly decreased frequencies of developing NK cells at various stages of differentiation. BM db/db NK cells displayed markedly increased apoptosis but maintained normal cell cycling status and proliferative capacity. Moreover, recombinant leptin could significantly enhance the survival of NK cells from wild-type mice in cultures. Further examination on NK cell functional activity showed that db/db NK cells exhibited normal intrinsic cytotoxicity with significantly increased IL-10 production. Taken together, our findings suggest that leptin signaling regulates NK cell development via enhancing the survival of immature NK cells in mouse BM.

关键词： NK cell bone marrow lymphocyte development

The Hong Kong consensus statements on unresectable hepatocellular carcinoma:narrative review and update for 2021

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Hepatobiliary Surgery and Nutrition 2023年第3期12卷 366-385页

作者： Tan-To Cheung Simon Chun-Ho Yu Stephen L.Chan Ronnie T.P.Poon Philip Kwok Ann-Shing Lee Anna Tai Derek Tam Chin-Cheung Cheung Tak-Wing Lai Nam-Hung Chia Ada Law Tracy Shum Yim-kwan lam Vince Lau Victor Lee Charing Chong Chung-Ngai Tang Thomas Yau Department of Surgery Queen Mary HospitalThe University of Hong KongHong KongChina Department of Imaging&Interventional Radiology Prince of Wales HospitalThe Chinese University of Hong KongHong KongChina State Key Laboratory of Translational Oncology and Department of Clinical Oncology Sir YK Pao Centre for CancerPrince of Wales HospitalThe Chinese University of Hong KongHong KongChina Department of Radiology and Imaging Queen Elizabeth HospitalHong KongChina Department of Clinical Oncology Tuen Mun HospitalHong KongChina Department of Clinical Oncology Queen Elizabeth HospitalHong KongChina Department of Surgery United Christian HospitalHong KongChina Department of Surgery Tuen Mun HospitalHong KongChina Department of Surgery Princess Margaret HospitalHong KongChina Department of Surgery Queen Elizabeth HospitalHong KongChina Department of Clinical Oncology Pamela Youde Nethersole Eastern HospitalHong KongChina Department of Clinical Oncology Princess Margaret HospitalHong KongChina Department of Medicine and Geriatrics United Christian HospitalHong KongChina Department of Radiology Queen Mary HospitalHong KongChina Department of Clinical Oncology Queen Mary HospitalThe University of Hong KongHong KongChina Department of Surgery Prince of Wales HospitalThe Chinese University of Hong KongHong KongChina Department of Surgery Pamela Youde Nethersole Eastern HospitalHong KongChina Department of Medicine Queen Mary HospitalThe University of Hong KongHong KongChina

Background and Objective:Hong Kong,like many parts of Asia,faces a high burden of hepatocellular carcinoma(HCC)caused by high endemic rates of hepatitis B virus *** Kong clinicians have developed a high level of expertise in HCC treatment across surgical,transarterial,ablative,radiotherapeutic and systemic *** publication summarizes the latest evidence-based recommendations on how these modalities should be ***:In two meetings held in 2020,a multidisciplinary panel of surgeons,oncologists and interventional radiologists performed a narrative review of evidence on the management of HCC,with an emphasis on treatment of HCC not amenable to surgical *** attention was paid to new evidence published since the previous version of these statements in *** Content and Findings:The expert panel has formulated 60 consensus statements to guide the staging and treatment of unresectable *** the previous version of these statements,considerable additions have been made to the recommendations on use of targeted therapies and immunotherapies because of the large volume of new ***:Our consensus statements offer guidance on how to select HCC patients for surgical or non-surgical treatment and for choosing among non-surgical modalities for patients who are not candidates for *** particular,there is a need for more evidence to aid physicians in the selection of second-line systemic therapies,as currently most data are limited to patients with disease progression on first-line sorafenib.

关键词： Hepatocellular carcinoma(HCC) Hong Kong guidelines consensus