血友病关节健康评分在学龄前儿童血友病关节评价中的应用--一项多中心儿童重型血友病A临床数据分析

Role of hemophilia joint health score(HJHS)in the evaluation of hemophilia joints in preschool children:A clinical data analysis of a multicenter children with severe hemophilia A

目的探讨血友病关节健康评分(hemophilia joint health score,HJHS)在重型血友病A(hemophilia A,HA)患儿关节病变评价及早期发现关节功能障碍的意义。方法本研究为观察性队列研究,以中国血友病个体化预防研究(China hemophilia individualized prophylaxis study,CHIPS)为背景,分析2016年8月至2017年6月就诊于首都医科大学附属北京儿童医院和成都市妇女儿童中心医院血友病门诊的患儿基本资料,入组的20例4~7岁曾有关节出血史的重型HA患儿进行双侧肘、膝、踝6个目标关节进行血友病关节健康评分(HJHS)及超声(US)检查;并回顾性收集入组前3个月关节出血情况数据。对上述数据进行统计分析。结果对20例中位年龄4.75(4.00~7.58)岁重型HA病例,共进行了120个目标关节HJHS及US检查:其中26.7%(32/120)的关节有出血记录;入组前3个月内20例中位关节出血次数1(0~6)次,有出血的关节HJHS评分与无出血的关节HJHS评分差异有统计学意义(Z=-2.608,P=0.009),即有出血的关节HJHS评分分数高。HJHS评分与US评分结果存在差异(χ2=24.221,P<0.001)。故两者不能互相替代。88个无出血记录的关节中有22.7%(20/88)US评分异常、36.3%(33/88)HJHS评分异常,其中12个关节(17.6%)US评分正常,但HJHS评分异常,此12个关节与有关节出血伴HJHS评分和US评分异常的关节HJHS评分差异有统计学意义(Z=-2.513,P=0.012)。关节异常部位以膝关节(50%,6/12)最多,肿胀(58.3%,7/12)为最常见的评分异常项目。结论对学龄前重型血友病A患儿开展HJHS检查,可发现无出血关节早期病变史,且HJHS评分与US评分结果存在差异,因此对患儿关节应进行综合评估,以保持患儿关节健康状态提供保障。

Objective To understand the significance of hemophilia joint health score(HJHS)in the evaluation of joint disease in children with severe hemophilia A(HA)and early detection of early joint dysfunction. Methods The study was an observational cohort study with the Chinese Hemophilia Individualized Prophylaxis Study(CHIPS) as the background. The basic data of outpatients with hemophilia in Beijing Children’s Hospital,Capital Medical University and Chengdu Women and Children’s Central Hospital between August 2016 and June 2017 were analyzed. Twenty patients with severe HA who had a history of joint bleeding at 4 to 7 years of age were enrolled. Six index joints of the bilateral elbow,knee and ankle were examined by HJHS and ultrasound(US). The data of joint bleeding of the group in 3 months before the study were collected retrospectively. The above data were analyzed retrospectively. Results A total of 20 patients with a median age of 4.75(4.00-7.58)were enrolled in the study and HJHS and US examinations were performed on 120 target joint. Among them,26.7%(32/120)of joints had bleeding records and the median number of joint bleeding in 20 patients was 1(0-6)times in 3 months before the study.There was statistical difference between the cases with joint bleeding and those without in the HJHS score(Z=-2.608,P=0.009),which showed that HJHS score of the joint with bleeding was higher. There was difference between the HJHS score and the US score(χ2=24.221,P<0.001),which showed that the two scores couldn’t substitute for each other.There were 88 joints without bleeding records,22.7%(20/88)of them had abnormal US scores,36.3%(33/88)had abnormal HJHS scores,and 12(17.6%)joints had abnormal HJHS scores but normal US scores. There was a statistically significant difference in joint HJHS scores between these 12 joints and those with bleeding and abnormal HJHS scores and US scores(Z=-2.513,P=0.012),with 50%(6/12)being the knee joints and 58.3%(7/12)being swelling.Conclusion Joint diseases can be found by HJHS examination in pre-school severe hemophilia A children as the complement with US;there is difference between HJHS scores and US scores. Therefore,these children should receive comprehensive evaluation in order to ensure the health of the joints.