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Chinese Medical Journal 2018年第15期131卷 1780-1785页

作者： Zhen-Ping Chen Pei-jing Li Gang Li Ling Tang Ying-Zi Zhen Xin-Yi Wu Xiao-Ling Cheng koon hung luke Victor S Blanchette Man-Chiu Poon Qiu-Lan Ding Run-Hui Wu Hematology Oncology Center Beijing Children's Hospital Capital Medical University Beijing Key Laboratory of Pediatric Hematology Oncology Discipline of Pediatrics Ministry of Education Beijing 100045 China Department of Pediatrics and Laboratory Medicine and Hemophilia Clinic Children's Hospital of Eastern Ontario and University of Ottawa National Key Ottawa. Ontario K1H8L1 Canada Department of Pediatrics and Child Health Evaluative Sciences Division of Hematology/Oncology Research Institute Hospital for Sick Children University of Toronto Toronto Ontario M5G lX8 Canada Department of Medicine Pediatrics and Oncology and Southern Alberta Rare Blood and Bleeding Disorders Comprehensive Care Program University of Calgary Foothills Hospital and Calgary Health Region Calgary Alberta T2N2T9 Canada Department of Laboratory Medicine Ruijin Hospital Shanghai Jiaotong University School of Medicine Shanghai 200025 China

Background： Although much attention has been paid to the pharmacokinetics （PKs） of different factor VIII （FVIlI） concentrates in persons with hemophilia A （HA）, limited information is available in young boys with severe HA. In this study, we aimed to assess the PK parameters of FVIII products in boys with severe HA in China. Methods： A total of 36 boys （plasma-derived [pd]-FVIII, n= 15; recombinant [r] FVIII, n = 21） were enrolled between January 2015 and May 2016 in Beijing Children＇s Hospital. PK characteristics of FVIII products were studied according to a reduced 4-sampling time point design （1 h, 9 h, 24 h, and 48 h postinfusion）. Results： The mean FVIII half-life （t1/2） was 10.99 ± 3.45 h （range 5.52-20.02 h）, the mean in vivo recovery （IVR） was 2.01 ± 0.42 IU/dl per IU/kg （range 1.24-3.02 IU/dl per IU/kg） and mean clearance （CL） of FVIII is 4.34 ± 1.58 ml·kg^-1·h ^-1 （range 2.29-7.90 ml·kg^-1·h·^-1）. We also analyzed the influence of several parameters that potentially modulate FVIII PK. The age was closely associated with FVIII half-life （R^2 = 0.32, P 〈 0.01 ）. The t1/2 of FVIII increased by 0.59 h per year. Besides age, yon Willebrand factor antigen （VWF：Ag） also was associated with FVIII half-life （R^2 = 0.52, P 〈 0.01）. Patients with blood Group O had a shorter FVIII halt-life than patients with non-O blood group （9.40 ± 0.68 h vs. 12.3 ± 0.79 h, t = 2.70, P = 0.01）. The FVIII IVR correlated with age （R^2 =0.21, P 〈 0.01） and VWF：Ag level （R^2 = 0.28, P 〈 0.01 ）. CL rates were taster in young patients and in those with low-VWF：Ag levels. CL rates of FVIII are higher in blood Group O versus non-blood Group O persons （5.02 ± 0.38 vs. 4.00 ± 0.32 ml·kg^-1·h^-1 , t = 2.53, P = 0.02）. Conclusions： Chinese boys with severe HA have similar PK values to other ethnic groups and large differences in FVIII PK between individual patients. Age, blood group, and VWF：Ag levels are important determining factors for FVlll CL.

关键词： Boys Factor VIII Hemophilia A Phannacokinetics

Value of Contrast-enhanced Ultrasound in Evaluating Synovitis and Predicting Recurrent Joint Bleeding of Hemophilia

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Current Medical Science 2022年第2期42卷 439-446页

作者： Fei MA Ya-ru ZHANG koon hung luke Hao LIU Wan-xian LUO Li ZHANG Li-ling XIAO Mei YI Yang GAO Wei-zhen WANG Shi-qiu QIU Xiao-qin FENG Jing SUN Ying-jia LI Department of Medicine Ultrasonics Nanfang HospitalSouthern Medical UniversityGuangzhou510515China Hematology and Lab Medicine Divisions Children Hospital of Eastern OntarioOttawaK1H 8L1Canada Department of Ultrasound The Second People’s Hospital of ShenzhenShenzhen518025China Department of Hematology Nanfang HospitalSouthern Medical UniversityGuangzhou510515China Department of Pediatrics Nanfang HospitalSouthern Medical UniversityGuangzhou510515China

Objective Contrast-enhanced ultrasound(CEUS)is advantageous for evaluating microcirculation,and has been applied to assess arthritis in previous ***,CEUS examinations have not been studied for hemophilia *** arthritis is different from other arthritis,because it is induced by spontaneous joint ***,CEUS may have special value in evaluating hemophilia *** present study assessed the value of CEUS in evaluating synovial hypertrophy and predicting recurrent joint bleeding in severe hemophilia A *** From August 2016 to January 2017,81 severe hemophilia A patients,who were referred to our hospital for ultrasound joint assessment with conventional ultrasound,were *** these 81 patients,46 patients consented for CEUS examinations on the same *** Compared to color Doppler flow imaging(CDFI),four more joints presented with a blood flow signal under CEUS *** addition,the synovial hypertrophy measured by CEUS was thicker than that measured by conventional *** ultrasound scores(including the total grey-scale ultrasound score,joint effusion/hemarthrosis,synovial hypertrophy,CDFI semi-quantitative score,and CEUS semi-quantitative score)were significantly higher in the joint bleeding group than in the no joint bleeding group(P<0.05).Furthermore,these ultrasound scores were positively correlated with the joint bleeding frequency,and had the highest correlation with the CEUS score(r=0.620,P<0.05).Conclusion CEUS can more accurately assess the degree of synovial hypertrophy and vascularization,and diagnose synovitis,when compared to conventional *** addition,CEUS appears to be essential for evaluating the possibility of recurrent joint bleeding,and providing more reliable evidence for individualized treatment.

关键词： contrast-enhanced ultrasound hemophilia arthropathy synovial hypertrophy synovial vascularity

A survey on treatment and quality of life(QoL) of hemophilia A(HA) and hemophilia B(HB) children in Zhejiang Province in Southern China

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A survey on treatment and quality of life(QoL) of hemophilia...

中国南方浙江省血友病儿童的治疗和生活质量的调查(英文)

2014浙江省医学会血液病学学术年会

作者： Weiqun Xu Jianya Yang Yongmin Tang koon-hung luke Nancy L Young Man-Chiu Poon Runhui Wu 浙江大学医学院附属儿童医院血液肿瘤科

*** of hemophilia in China is a challenge because of economic constraint and availability of clotting factors,The HTC at the Children’s Hospital,Zhejiang University started care and registration of hemophilia boys in Zhejiang Province in *** recently surveyed these boys on their treatment,joint and QoL status to form a baseline to direct their future ***:(1)Retrospective chart review and telephone survey for treatment and joint status;(2)CHO-

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中国南方浙江省血友病儿童的治疗和生活质量的调查(英文)

Prenatal diagnosis of choledochal cyst using magnetic resonance imaging: A case report

2014年浙江省医学会儿科学分会学术年会暨儿内科疾病诊治新进展国家级继续教育学习班

作者：徐卫群 Linyan Zang Jinyin Zhang Yongmin Tang koon-hung luke Nancy L Young Man-Chiu Poon Runhui Wu Children’s Hospital Zhejiang UniversitySchool of Medicine

目的 Management of hemophilia in China is a challenge because of economic constraint and availability of clotting factors,The HTC at the Children’s Hospital,Zhejiang University started care and registration of hemophilia boys in Zhejiang Province in *** recently surveyed these boys on their treatment,joint and QoL status to form a baseline to direct their future care.

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World Journal of Gastroenterology 2005年第32期11卷 5082-5083页

作者： Alex Mun-Ching Wong Yun-Chung Cheung Yu-hung Liu koon-Kwan Ng Siu-Cheung Chan Shu-Hang Ng Department of Diagnostic RadiologyChang Gung Memorial Hospital Kwei-ShanTao-YuanTaiwanChina Obstetrics and GynecologyChang Gung Memorial Hospital 222Mei Chin RoadKeelungTaiwanChina

Choledochal cysts are congenital anomalies of the biliary ducts, characterized by cystic dilatation of the *** diagnosis of this anomaly using ultrasonography (US) has been well documented. Magnetic resonance imaging (MRI) has recently become an important complement to US in prenatal diagnosis of fetal anomalies. We herein report a patient in whom at 24 wk' gestation US suggested a right upper quadrant abdominal cyst and in whom at 26 wk' gestation MRI more clearly delineated the cyst and its surrounding structures and suggested a choledochal cyst, which was confirmed at postnatal surgery and histopathology.

关键词： Choledochal cyst Prenatal diagnosis MRI

The impact of ipsilateral testicular atrophy on semen quality and sperm DNA fragmentation response to varicocele repair

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Asian Journal of Andrology 2021年第2期23卷 146-149页

作者： Parviz K Kavoussi Natasha Abdullah Melissa S Gilkey Caitlin Hunn G luke Machen Shu-hung Chen Keikhosrow M Kavoussi Amy Esqueda J David Wininger Shahryar K Kavoussi Austin Fertility and Reproductive Medicine/Westlake IVF 300 Beardsley LaneBuilding BSuite 200AustinTX 78746USA

Varicoceles adversely impact semen quality and sperm DNA fragmentation,which typically improve with surgical *** men with varicoceles have ipsilateral testicular atrophy due to damage from the *** study assessed semen quality and the sperm DNA fragmentation index(DFI)response to varicocele repair in men with ipsilateral testicular atrophy(TA)versus men with no testicular atrophy(NTA).Semen parameter values and DFI in both groups were compared preoperatively and *** Mann-Whitney U test and the Wilcoxon signed-rank test were used where *** were 20 men in the TA group and 121 men in the NTA group with no difference in age,varicocele grade,or preoperative semen parameter values between the two *** NTA group had a higher preoperative DFI than the TA *** groups showed improvement in semen quality postoperatively,only the TA group showed a significant improvement in DFI,whereas the NTA group showed significant improvements in several parameter values and *** change from preoperative to postoperative parameter values when comparing the two groups revealed a difference in total sperm motile count and DFI,with a larger mean improvement in the NTA group than in the TA *** TA and NTA groups showed improved semen quality and DFI after varicocele repair,but the NTA group had more improvement than the TA ***,only total motile count(TMC)and DFI had a significantly greater mean change in preoperative to postoperative response in the NTA group than in the TA group.

关键词： spermatozoa testicular atrophy varicocele

重组人凝血因子Ⅸ预防性治疗中国儿童中、重型血友病B的单中心临床观察

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中华实用儿科临床杂志 2013年第15期28卷 1128-1130页

作者：李刚吴润晖吴心怡甄英姿张宁宁王岩何雯雯彭芸 luke koon-hung POON Man-Chiu ANDREA Doria 首都医科大学附属北京儿童医院血友病组 100045 University of Ottawa Children's Hospital of Eastern Ontario Ottawa Ontario Canada University of Calgary Foothills Hospital Calgary Al- bertaCanada University of Toronto Sickkids Children's Hospital of TorontoToronto. Canada

目的应用重组人凝血因子Ⅸ（FIX）预防性治疗中国儿童中、重型血友病B，了解其疗效及安全性。方法使用重组人FIX开展儿童血友病B小剂量预防治疗，重组人FIX20IT]／（kg·次），1次／周，I临床观察指标包括：（1）疗效：出血减少率、日... 详细信息

目的应用重组人凝血因子Ⅸ（FIX）预防性治疗中国儿童中、重型血友病B，了解其疗效及安全性。方法使用重组人FIX开展儿童血友病B小剂量预防治疗，重组人FIX20IT]／（kg·次），1次／周，I临床观察指标包括：（1）疗效：出血减少率、日常活动参与改善情况；（2）安全性：用药中不良事件及抑制物产生情况。结果共收集11例病例，观察时间18～27周（平均25．5周）；年龄9个月～15岁（平均7岁1个月）；重型9例，中间型2例；开始观察前接受按需治疗6例，预防治疗4例，无治疗1例；中位暴露日（ED）63ED（范围0～〉150ED）。（1）疗效：总出血减少率67．7％（与原按需治疗／无治疗比较减少78．6％；与原预防治疗比较减少47．8％）；9例中8例日常活动参与改善（与原按需／无治疗比较6例中5例改善，与原预防治疗比较3例全部改善）；（2）安全性：应用过程中无不良事件发生；治疗过程中仅有1例有1次抑制物检测滴度达0．6BU／mL，治疗结束后降至0BU／mL。结论重组人FIX应用于中国中、重型血友病B儿童的小剂量预防治疗安全、有效。

关键词：血友病B 重组人凝血因子IX 预防治疗儿童

Fulminant ulcerative colitis associated with steroid-resistant minimal change disease and psoriasis: A case report

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World Journal of Gastroenterology 2007年第29期13卷 4022-4024页

作者： Ka-Ho Lok Hiu-Gong hung Wai-Man Yip Kin-Kong Li Kam-Fu Li Ming-Leung Szeto Division of Gastroenterology and Hepatology Department of Medicine and Geriatrics Tuen Mun Hospital Tsing Chung Koon Road Tuen Mun Hong Kong China

A 43-year-old Chinese patient with a history of psoriasis developed fulminant ulcerative colitis after immunosuppressive therapy for steroid-resistant minimal change disease was stopped. Minimal change disease in association with inflammatory bowel disease is a rare condition. We here report a case showing an association between ulcerative colitis, minimal change disease, and psoriasis. The possible pathological link between 3 diseases is discussed.

关键词： Ulcerative colitis Inflammatory bowel disease Minimal change disease