重型血友病患儿治疗方式与颅内出血的关系

Intracranial hemorrhage occured in children with severe hemophilia under different treatment

目的回顾性分析近10年我院门诊重型血友病患儿的治疗方案及其发生颅内出血(ICH)的临床资料,了解重型血友病患儿治疗方式与ICH的关系。方法 2014年4月1日收集并回顾性分析1993年1月1日-2012年12月31日出生的重型血友病患儿治疗方案和发生ICH的相关临床资料。结果共收血友病患儿病例197例,157例为血友病A,40例为血友病B。血友病A患儿中4.5%(7/157)以ICH为首发症状,8.2%(11/134)按需治疗患儿发生ICH,0%(0/16)预防治疗患儿出现ICH;血友病B患儿中10%(4/40)以ICH为首发症状,13%(4/32)的按需治疗患儿发生ICH,0%(0/4)的预防治疗患儿出现ICH。ICH近5年累积发生率为13.2%(26/197),42%(11/26)以ICH为首发症状,73%(19/26)的患儿发生ICH时年龄小于3岁,69%(18/26)的患儿发生ICH有明确外伤史。结论目前我院随诊的重型血友病患儿以ICH为首发症状约占40%,多发生于婴幼儿。预防治疗组患儿无ICH发生,提示及早诊断和积极开展预防治疗可以降低重型血友病患儿ICH的发生。

Objective To determine whether different treatment mode can influence the incidence of ICH in children with severe hemophilia by retrospectively collecting the clinical data about treatment and intracranial hemorrhage （ICH） of these patients recently ten years.Methods We started to retrospectively collect treatment and the incidence of ICH of patients who was born in January 1,1993 to December 31,2012 with severe hemophilia On April 1,2014.Results 197 cases were enrolled,157 were hemophilia A,40 were hemophilia B.In Hemophilia A,4.5% of which with ICH as the first symptom,8.2% of patients in on-demand treatment group occurred ICH,no patient in prophylaxis group occurred ICH;In Hemophilia B,10% of which with ICH as the first symptom,13% of patients in on-demand treatment group occurred ICH,no patient in prophylaxis group occurred ICH.In the past five years,the cumulative incidence of ICH was 13.2% in our hospital,42% of patients with ICH as the first symptom,73% children occurred ICH younger than 3 years old,69% of children occurs ICH with a clear history of trauma.Conclusions Among the patients with severe hemophilia in our hospital,nearly 40% patients manifested with ICH as the very beginning symptom,The ICH phenomenon were found in infants and young children,mostly.No ICH occurred in prophylaxis treatment group both in hemophilia A and B,which suggested that early diagnosis and aggressive prophylaxis treatment can reduce the occurrence of ICH.