学龄前重型血友病A患儿无临床出血记录关节的病变分析

Analysis of the arthropathies on no-bleeding history joints in pre-school age severe hemophilia A children

目的了解学龄前期血友病A患儿无出血记录目标关节的病变情况，为进一步开展血友病A患儿关节病变防控提供依据。方法横断面研究，借助于中国血友病个体化预防研究（CHIPS），收集2016年8月到2017年6月就诊于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，并进行3个月观察。对入组的34例1-7岁重型血友病A患儿目标关节（双肘、双膝、双踝6个关节）行结构与功能检查[超声、X线及血友病关节健康评分（HJHS）]。寻找观察期结束时无出血记录关节病例是否存在病变关节并分析相关因素，应用χ2检验、秩和检验等统计方法进行数据分析。结果共有32例可供分析病例，其中有112个无出血纪录的目标关节，以肘关节居多为42.9%（48/112）。经关节结构与功能检查发现34.8%（39/112）的关节存在病变，其中46.2%（18/39）为踝关节（χ2=8.379，P=0.015）。超声、X线、HJHS发现关节存在异常的比例分别为18.3%（20/109）、3.8%（3/79）、25.3%（20/79）。超声与HJHS检查结果不相关（r=0.015，P=0.895），X线与HJHS检查结果不相关（r=-0.101，P=0.390），提示关节结构与功能检查相互不能替代。出现关节病变的危险因素有年龄〉4.91岁（OR=3.917，95%CI：1.610- 9.528），有靶关节（OR=3.530，95%CI：1.316-9.465）。结论对学龄前期血友病A患儿无出血记录关节进行关节结构和功能检查可发现关节病变，以踝关节比例最高；关节结构与功能检查不能相互替代；年龄〉4.91岁和存在靶关节是无出血记录关节发生关节病变的危险因素。

ObjectiveTo detect the arthropathies on no bleeding history joints in pre-school hemophilia A children in order to provide evidence for further prevention and control of joint disease in children with hemophilia A.MethodsThis study was a cross-sectional study based on China Hemophilia Individualized prophylaxis study （CHIPS）. The basic data of outpatients with hemophilia in Beijing Children＇s Hospital and Chengdu Women＇s and Children＇s Central Hospital between August 2016 and June 2017 were collected and a three-month follow-up was conducted. The target joints （six joints of bilateral elbows, knees and ankles） of thirty-four children aged 1-7 years old with severe hemophilia A were examined by ultrasound, X-ray and joint function examination （4-7 years old, hemophilia joint health score （HJHS））. To find out whether there are arthropathies in patient＇s joints with no bleeding history and analyze the relevant factors by chi-square test, rank sum test and other statistical methods.ResultsThere were 32 analyzable cases with 112 no-bleeding history target joints, 42.9% （48/112） were elbow joints. Arthropathies were revealed in 34.8% （39/112） of them by joint structural and functional examination and 46.2% （18/39） were ankles （χ2=8.379, P=0.015） . Ultrasound showed abnormalities in 18.3% （20/109） joints, X-ray showed abnormalities in 3.8% （3/79） joints and HJHS showed abnormalities in 25.3% （20/79） joints. There was no correlation between ultrasound and HJHS （r=0.015, P=0.895）, no correlation was found between X-ray and HJHS （r=-0.101, P=0.390） either, which suggested that joint structural and functional examination could not replace each other. The related risk factors of arthropathies in this group were 〉4.91 years old （OR=3.917, 95%CI：1.610-9.528） and combining with target joint （OR=3.530, 95%CI：1.316-9.465）.ConclusionsDetecting the joint structure and function on no bleeding history joints in pre-school hemophilia A children could reveal the arthropathies and majority of them were ankle arthropathies. Joint structural and functional examinations could not replace each other. For patients more than 5 years old and those with target joints, the joints with no complaint of bleeding should be examined regularly to reveal the arthropathies in time.