67例住院血友病患者的临床回顾性分析  

Clinical retrospective analysis of 67 hospitalized patients with hemophilia

作　　者：覃金球 吕福通[2] 谢丹尼[2] 丘玉铃[3] 韦红英[4] 林发全[1] 

机构地区：[1]广西医科大学第一附属医院检验科,南宁530021 [2]广西壮族自治区人口和计划生育研究中心,南宁530021 [3]广西医科大学地中海贫血防治研究实验中心,南宁530021 [4]广西医科大学第一附属医院儿科,南宁530021

出　　处：《广西医科大学学报》2017年第4期542-545,共4页Journal of Guangxi Medical University

基　　金：广西壮族自治区人口和计划生育委员会科研基金资助项目(No.1108)

摘　　要：目的:分析广西地区67例住院血友病患者的临床特征、诊断年龄、替代治疗情况。方法:收集并分析2012年1月至2015年9月在广西医科大学第一附属医院住院治疗的67例广西籍血友病患者的临床资料。结果:67例患者均为男性,血友病A 53例,血友病B 14例。20例(29.85%)有血友病家族史。首次出血中位年龄为3岁,以轻度出血者为主(40例),占59.70%。初次确诊中位年龄为6岁,诊断时机与患者首次出血程度(P=0.000)以及临床分型(P=0.010)有关。入院主诉以中度出血者居多(39例),误诊、漏诊11例。存在关节损伤者14例(20.90%),关节损伤发生率与入院时出血程度(P=0.008)及临床分型(P=0.000)有关。入院前从未进行过替代治疗者20例(29.85%),按需治疗患者40例(59.70%),曾进行预防治疗者6例(8.96%),乙型肝炎病毒(HBV)阳性5例,丙型肝炎病毒(HCV)阳性2例。结论:广西地区67例住院血友病患者存在初次确诊年龄延迟及替代治疗不充分的现象;获得诊断时机与首次出血程度及临床分型相关;关节损伤发生率与入院时出血程度及临床分型相关。Objective： To analyze the clinical features, diagnostic age and replacement therapy pattern of hospitalized patients with hemophiliain Guangxi Region. Methods： The clinical data of 67 patients with hemophilia treated in our hospital from January 2012 to September 2015 were retrospective analyzed. Results： All patients were male. Of them, 53 cases had hemophilia A and 14 cases had hemophilia B. 20 patients had family history of hemophilia （29.85%）. The median age of initial bleeding was 3 years old, and most of the cases showed mild hemorrhage （59.70%）. The median age of the first diagnosed was 6 years old. The diagnostic timing was related to the severity of the first hemorrhage （ P =0. 000） and clinical classification （ P=0. 010）. The majority of patients had got moderate hemorrhage on admission （39 cases）. 11 patients were misdiagnosed or missed diagnosis. 14 cases got joint damage （20.90%）. The incidence of joint injury was related to bleeding degree （ P =0. 008） and clinical classification （ P =0. 000）. 20 patients had never got any treatment prior to admission （29.85%）. 40 patients received on-demand treatment （59.70%） and 6 patients received preventive treatment （8.96%）. Hepatitis B virus （HBV） infection was found in 5 patients and hepatitis C virus （HCV） in 2 patients. Conclusion： Delayed first diagnosis and inadequate replacement treatment were found in 67 hemophilia patients in Guangxi. Diagnostic timing was associated with first hemorrhage and clinical classification. The incidence of joint damage was related to bleeding degree and clinical classification.

关 键 词：血友病 关节损伤 替代治疗 

分 类 号：R554.1[医药卫生—血液循环系统疾病]