重型血友病A患儿关节出血与关节评估的相关性研究

目的了解血友病患儿出血与关节病变之间的关系,为临床治疗和预后判断提供理论依据。方法选取2016年6月至2017年1月于首都医科大学附属北京儿童医院及成都市新世纪妇女儿童医院就诊的1~7岁重型血友病A且有关节出血记录的患儿,以患儿关节出血为研究关节,收集前3个月研究关节的出血次数,应用超声、X线、磁共振成像(MRI)和血友病关节健康评分(HJHS)评分系统对关节进行评估。将关节出血次数与超声、X线、MRI和HJHS评分进行相关性分析,并对超声、X线、MRI和HJHS评分间的相关性进行分析。结果1.共收集重型血友病A患儿18例,年龄(5.6±1.8)岁,共30个出血关节,中位年关节出血次数为4次(4~16次),中位年靶关节出血次数为8次(4~16次)。2.关节出血次数与超声、X线评估有相关性(r=0.390,P=0.033;r=0.517,P=0.008),与HJHS评估、MRI评估均无相关性(均P>0.05)。3.超声、X射线、HJHS和MRI两两之间均呈极显著正相关r=0.815(超声与X线),r=0.510(超声与HJHS),r=0.812(超声与MRI),r=0.666(X线与HJHS),r=0.911(X线与MRI),r=0.781(HJHS与MRI),均P<0.01]。4.关节超声和/或MRI评估异常的关节中,出血次数与关节评估间均无相关性(P>0.05)。结论关节出血与关节评估获得的关节病变程度结果不一致,因此准确判断关节病变情况需综合评估关节结构、功能等,以便更加全面地指导血友病患儿的治疗。

Relationship between joints bleeding and joint assessment in children with severe haemophilia A

Objective To understand the relationship between joint bleeding and joint disease in hemophilia children,and to provide a theoretical basis for clinical treatment and prognosis.Methods The patients with severe hemophilia A between 1 and 7 years old and with relevant nodal bleeding records were selected.All the patients admitted in Beijing Children′s Hospital,Capital Medical University,and Chengdu New Century Women′s and Children′s Hospital since June 2016 to January 2017.All the joint bleeding of each child was taken as the study joint,and the joint bleeding was collected during the last 3 months.The joints were assessed by using ultrasound,X-ray,magnetic resonance imaging(MRI)and Hemophilia Joint Health Score(HJHS)scoring systems.The correlation analysis was conducted between the joint bleeding,ultrasound,X-ray,MRI and HJHS scores.The correlation analysis was conducted for baseline ultrasound,X-ray,MRI and HJHS scores.Results(1)There were 18 patients enrolled.The mean age was(5.6±1.8)years old.There were 30 joints bleeding in the observation period in total,with the annul median joint bleeding times of 4(4-16 times),and the annul median target joint bleeding times of 8(4-16 times).(2)Joint bleeding times of was correlated with ultrasound and X-ray evaluation(r=0.390,P=0.033;r=0.517,P=0.008),and not correlated with HJHS or MRI(all P>0.05).(3)There was significantly positive correlation among ultrasound,X-ray,HJHS and MRIr=0.815(ultrasound vs.X-ray),r=0.510(ultrasound vs.HJHS),r=0.812(ultrasound vs.MRI),r=0.666(X-ray vs.HJHS),r=0.911(X-ray vs.MRI),r=0.781(HJHS vs.MRI),all P<0.01].(4)There was no correlation between times and assessment for joints whose ultrasound and/or MRI in joints with abnormal ultrasound and/or MRI evaluation(P>0.05).Conclusions The results of joint bleeding and joint evaluation are inconsistent.Joint bleeding can not truly reflect the situation of joint diseases.The assessment of hemophilia should include comprehensive evaluation of joint structure,function,activity ability and other aspects to guide the treatment of haemophi-lia children.