儿童血友病65例临床资料分析

发布时间：2019-03-14 18:25

【摘要】：目的：研究儿童血友病临床发病及诊断年龄、临床表现及治疗等情况。方法：回顾性分析2005年1月至2011年11月广西医科大学第一附属医院住院收治的65例来自广西各地的儿童血友病患者的临床资料。结果：65例血友病患儿中血友病A共54例(83.08%)：重型13例(24.07%),中型22例(40.74%),轻型17例(31.48%),亚临床型2例(3.70%)；血友病B共11例(16.92%)：重型3例(27.27%),中型4例(36.36%),轻型3例(27.27%),亚临床型1例(9.09%)。血友病A：血友病B=4.9：1。首次出血中位数年龄为1岁,首次诊断中位数年龄为2岁2个月。65例患儿中有关节出血症状33例(50.77%),其中重型血友病13例(81.25%),中型15例(57.69%),轻型5例(25.00%)。65例中发生关节畸形17例(26.15%),其中血友病A重型7例(53.85%),中型6例(27.27%)；血友病B重型2例(66.67%),中型2例(50.00%)。重度出血共10例,均为重型及中型患儿。两个以上部位出血共27例,其中重、中型患儿占24例。亚临床型患儿均为医源性出血。65例患儿中有明确血友病家族史18例(27.69%)。62例接受过替代治疗73例次,急性出血时单纯应用凝血因子输注者29例次(39.73%)；使用凝血因子的同时使用冷沉淀和(或)新鲜冰冻血浆35例次(47.95%)；仅使用冷沉淀或新鲜冰冻血浆9例次(12.33%)。无1例进行预防性替代治疗。65例患儿均未进行血制品相关性感染性疾病的系统监测。1例14岁患儿抗-HCV抗体阳性。结论：(1)血友病儿童首次诊断年龄与首次出血年龄之间存在差距,存在诊断延迟现象；(2)出血部位、出血严重程度与血友病临床分型密切相关；(3)关节出血及关节畸形发生率较高；(4)存在替代治疗不及时、使用因子制剂替代治疗率低的状况；(5)本组患儿均未进行血制品相关性感染性疾病的系统监测。
[Abstract]:Objective: to study the clinical onset, diagnostic age, clinical manifestation and treatment of hemophilia in children. Methods: the clinical data of 65 children hemophilia patients admitted to the first affiliated Hospital of Guangxi Medical University from January 2005 to November 2011 were retrospectively analyzed. Results: of the 65 cases of hemophilia, 54 cases (83.08%) were hemophilia A, 13 cases (24.07%) were severe, 22 cases (40.74%) were medium, 17 cases (31.48%) were mild, and 2 cases (3.70%) were subclinical type. There were 11 cases (16.92%) of hemophilia B, 3 cases of severe type (27.27%), 4 cases of medium type (36.36%), 3 cases of mild type (27.27%) and 1 case of subclinical type (9.09%). Hemophilia A: hemophilia B / 4. 9 / 1. The median age of the first hemorrhage was 1 year old and the median age of the first diagnosis was 2 years and 2 months. Of the 65 children, 33 (50.77%) had symptoms of joint hemorrhage, 13 (81.25%) had severe hemophilia, 15 (57.69%) had moderate hemophilia. Among 65 cases, 17 cases (26.15%) developed joint malformations, including 7 cases (53.85%) of hemophilia A and 6 cases (27.27%) of medium-sized hemophilia. Hemophilia B was severe in 2 cases (66.67%) and medium in 2 cases (50.00%). There were 10 cases of severe hemorrhage, all of them were severe and medium-sized children. There were 27 cases of bleeding in more than two sites, of which 24 cases were severe and medium-sized. 18 cases (27.69%) had a clear family history of hemophilia, 62 cases had received replacement therapy 73 times, 29 cases (39.73%) were treated with coagulation factor infusion alone during acute hemorrhage, and 18 cases (27.69%) had a clear family history of hemophilia, 62 cases had received replacement therapy for 73 times, and 29 cases (39.73%) had only used coagulation factor infusion during acute hemorrhage. Coagulation factors were used in 35 cases (47.95%) with cold precipitation and / or fresh frozen plasma, and 9 cases (12.33%) with only cryoprecipitation or fresh frozen plasma. None of them were treated with prophylactic replacement therapy. 65 children were not systematically monitored for infectious diseases associated with blood products. One 14-year-old child was positive for anti-HCV antibody. Conclusion: (1) there is a difference between the age of first diagnosis and the age of first hemorrhage in hemophilia children, and there is delay in diagnosis, (2) the location of hemorrhage and the severity of hemorrhage are closely related to the clinical classification of hemophilia. (3) the incidence of joint hemorrhage and joint deformity was high; (4) there was a situation that replacement therapy was not timely and the rate of replacement therapy with factor preparation was low; (5) there was no systematic monitoring of infectious diseases associated with blood products in this group.
【学位授予单位】：广西医科大学
【学位级别】：硕士
【学位授予年份】：2012
【分类号】：R725.5

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