儿童血友病合并颅内出血临床分析  

Clinical analysis of intracraniai hemorrhage in children with hemophilia

作　　者：于增鹏[1] 梁平[1] 李映良[1] 翟瑄[1] 周渝冬[1] 李禄生[1] 夏佐中[1] 

机构地区：[1]重庆医科大学附属儿童医院神经外科,儿童发育疾病研究省部共建教育部重点实验室,儿科学重庆市重点实验室,重庆市儿童发育重大疾病诊治与预防国际科技合作基地,400014

出　　处：《中华实用儿科临床杂志》2013年第15期1131-1133,共3页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的探讨血友病患儿发生颅内出血的危险因素、临床特点、治疗及其预后。方法对2005年1月至2012年12月登记的24例血友病合并颅内出血的病例资料进行回顾性分析及随访。结果（1）本组血友病A15例，血友病B9例；均为男童；平均年龄1岁7个月；≤3岁病例占70．8％，且≤3岁年龄段88．9％为发生颅内出血后首次诊断为血友病；中、重型血友病占87．5％；仅37．5％的患儿有明确头部外伤史。（2）临床表现：高颅压症状、贫血、意识障碍、惊厥发作、肢体瘫痪等。（3）颅内出血部位：脑实质出血伴蛛网膜下腔出血7例，脑室出血2例，硬膜下出血伴蛛网膜下腔出血10例，硬膜外出血5例。（4）均予凝血因子替代治疗，其中5例行手术治疗。（5）13例无明确后遗症，9例有后遗症，2例死亡。结论3岁以下中、重型血友病患儿易发生颅内出血，部分患儿无明确诱因；临床表现与正常儿童颅内出血相似；早诊断、早治疗、足疗程的替代因子治疗是治疗的关键，规范治疗预后更好。在积极纠正凝血因子及凝血功能后，外科手术也是治疗手段之一。Objective To discuss the risk factors,clinical characteristics,treatment protocol and prognosis of intracranial hemorrhage （ICH） in children with hemophilia. Methods Twenty-four hemophilic children with ICH, which were registered in hospital between Jan. 2005 and Dec. 2012,were reviewed retrospectively. Results （ 1 ） Fifteen patients were hemophilia A and 9 patients were hemophilia B, all boys. The mean age of ICH was 1 year and 7 months old. The 70.8% of patients were less than 3 years old,among whom hemophilia was diagnosed after ICH in more than 88.9%. The 87.5% of patients had moderate or severe disease,and 37.5% had head trauma before ICH. （2）The cli- nical symptoms were high cranial pressure, anemia, disturbance of consciousness, seizure, hemiplegia. （ 3 ） ICH position ： cerebral hemorrhage with subarachnoid hemorrhage （SAH） in 7 patients, ventricular hemorrhage 2 patients, subdural hemorrhage with SAH in 10 patients, extradural hemorrhage 5 patients. （4） All patients were given blood coagulation factor replacement therapy,5 patients by operation. （5）Thirteen patients had not sequelae,9 patients had sequelae and 2 patients died. Conclusions The risk factors of ICH in hemophilic children include ages less than 3 years old, mode- rate or severe disease. Some patients have no predispositions. The clinical symptoms of patients are similar with normal children suffering from ICH. The keys of treatment are early diagnosis, early treatment and adequate course of treatment. Surgical operation could be in treatment after coagulation function gets corrected back to normal.

关 键 词：血友病 颅内出血 儿童 

分 类 号：R725.4[医药卫生—儿科]