儿童帽状腱膜下血肿合并血友病临床特点并文献复习

Clinical characteristics of subgaleal hemorrhage complicated with hemophilia in children and literature review

目的探讨儿童帽状腱膜下血肿(SGH)合并血友病的临床特点,为临床早期识别该病提供帮助,减少临床对血友病的漏诊及其带来的不良结局。方法回顾性分析2015年1月—2021年1月重庆医科大学附属儿童医院收治的22例SGH患儿的临床资料。按照是否合并血友病分为合并组(n=7)和未合并组(n=15),对比两组的临床特点。结果合并组患儿中,血友病轻型3例,中间型2例,重型2例。合并组跌伤/摔伤后头部包块6例(6/7,85.7%),未合并组车祸伤/跌伤/高坠伤后发现头部包块8例(8/15,53.3%)。未合并组中位年龄7.0岁(最大15.0岁,最小1.3 d),合并组中位年龄0.9岁(最大5.0年,最小2.8 d),P=0.005;未合并组中位病程时间2.0 d(最长15.0 d,最短0.2 d),合并组中位病程时间7.0 d(最长5.0年,最短0.5 d),P=0.032;未合并组中位血红蛋白107 g/L(最高145 g/L,最低82 g/L),合并组中位血红蛋白69 g/L(最高92 g/L,最低58 g/L),P=0.001;未合并组入院中位活化部分凝血活酶时间(APTT)31.8 s(最长34.0 s,最短24.9 s),合并组中位活化部分凝血酶时间62.7 s(最长124 s,最短42 s),P=0.000;未合并组外伤后合并颅骨骨折/颅内出血8例(53.3%),合并组3例(42.9%)。结论相对于单纯性SGH,合并血友病的SGH患儿发病年龄小,以婴幼儿为主,病程较长,入院查血提示血红蛋白低,APTT延长,SGH合并血友病的类型以轻、中间型为主;跌伤/摔伤为血友病患儿形成SGH的主要诱因。早期识别该病有助于尽早干预,尽可能减少因血友病漏诊带来的不良结局。

Objective To explore the clinical characteristics of subgaleal hemorrhage(SGH)complicated with hemophilia in children,so as to provide help for early clinical identification of this disease and reduce the adverse outcomes of misdiagnosis of hemophilia.Methods The clinical data of 22 children with SGH diagnosed and treated in Children’s Hospital of Chongqing Medical University from January 2015 to January 2021 were retrospectively analyzed.They were divided into simple group(n=7)and combined group(n=15)according to whether they were complicated with hemophilia.The clinical characteristics of the two groups were compared.Results In the combined group,there were 3 of mild hemophilia,2 of intermediate hemophilia and 2 of severe hemophilia.Head mass was found in 6 cases(6/7,85.7%)in the combined group after falling or falling injury,and 8(8/15,53.3%)in the non combined group after traffic accident,falling or falling injury.The median age of the non combined group was 7.0 years(maximum 15.0 years,minimum 1.3 days),and 0.9 years(maximum 5.0 years,minimum 2.8 days)in the combined group,P=0.005.The median course of disease in the non combined group was 2.0 days(the longest was 15.0 days,the shortest was 0.2 days),and 7.0 days(the longest was 5.0 years,the shortest was 0.5 days)in the combined group,P=0.032.The median hemoglobin in the non combined group was 107 g/L(highest 145 g/L,lowest 82 g/L),and 69 g/L(highest 92 g/L,lowest 58 g/L)in the combined group,P=0.001.The median time of activated partial thromboplastin time(APTT)was 31.8 s(the longest was 34.0s and the shortest was 24.9s)in the non combined group,and 62.7s(the longest was 124s and the shortest was 42s)in the combined group,P=0.000.There were 8 cases(53.3%)with skull fracture/intracranial hemorrhage after trauma in the non combined group and 3 cases(42.9%)in the combined group.Conclusions Compared with simple SGH,the SGH complicated with hemophilia have younger age of onset,mainly infants,a longer course of disease,low hemoglobin and prolonged APTT.The types of SGH complicated with hemophilia are mainly mild and intermediate.Fall injury is the main inducement for SGH patients with hemophili.Early identification of this disease is helpful to early intervention and minimize the adverse outcomes caused by missed diagnosis of hemophilia.