中重型血友病A患儿关节出血情况与关节结构、功能相关性研究

Relationship among joint hemorrhage and joint structure and function in children with moderate to severe hemophilia A

目的对血友病关节出血情况和关节结构、功能的数据进行分析,以了解三者间的相关性,为临床治疗和预后判断提供理论依据。方法(1)选取4-8岁男性中型-重型血友病A且有关节出血记录的患儿,以每位患儿出血次数最多的1个关节为研究关节。收集患儿研究关节的出血情况,包括研究关节的首次关节出血年龄、年关节出血率、近3个月关节出血次数及总出血次数。同期采用Pettersson评分系统对关节结构评分,采用HJHS评分系统评估关节功能。(2)将研究关节的关节出血情况分别与该关节X线Pettersson评分和HJHS评分进行分析,并将X线评分与HJHS评分进行相关性分析。结果(1)共收集16例血友病A患儿,16例研究关节的总出血次数与HJHS评分呈正相关(P<0.05),年关节出血率、近3月总出血次数、首次出血年龄与HJHS评分无明显相关性。首次关节出血年龄、年关节出血率、近3月关节出血次数及总出血次数与Pettersson评分相关性均较小。(2)16例研究关节的Pettersson评分与HJHS评分呈正相关(P<0.05)。(3)4例无出血的研究关节,Pettersson或HJHS评分异常;3例Pettersson和HJHS评分均为0的研究关节,有关节出血;5例Pettersson或HJHS评分异常的研究关节,出血及另一项评分为0。结论关节总出血次数越多,对关节功能影响越明显。但仅根据出血评估关节病变情况来调整治疗是不全面的,需结合关节结构及功能综合评估。

Objective The correlations among Hemophilia joint bleeding, joint structure and joint function were investigated in order to provide a theoretical basis for clinical treatment and prognosis.Methods 1. Patient selection： The 4 to 8-year-old boys, who were diagnosed as medium or severe hemophilia A and with a history of joint hemorrage, were recruited in the present study. The joints of the patients that had the most frequent hemorrhage were used for the data analysis. The first joint bleeding age, annual joint bleeding rate, the total number of joint bleeding, and the number of joint bleeding in the last 3 months were collected and analyzed. Each patient underwent an X-ray biopsy of the joint during the study, and the Pettersson scoring system was used to score each study joint. At the same time, the HJHS scoring system was used to evaluate the joint function. 2. The correlation between X-ray score and HJHS score was analyzed. 3. SPSS22. 0 statistical software was used for statistical analysis, and binary correlation analysis was used to analyze the correlation. Results 1. Analysis of the correlation between the bleeding status of the joint and the HJHS score in 16 patients showed the number of total bleeding was positively correlated with HJHS score（rs =0. 604, P =0. 013）. The number of bleeding in the last 3 months, annual joint bleeding rate and the first bleeding age were not correlated with the HJHS score（rs=0. 154, =0.568; rs =0.412, P=0. 113）. The first bleeding age, annual joint bleeding rate,the number of bleeding in the last 3 months and the number of total bleeding were notcorrelated with the Pettersson score（ rs =0. 246, P = 0. 358; rs = 0. 387, P = 0. 139; rs = 0. 490, P = 0. 054）. 2. The Pettersson score of 16 children was significantly correlated with HJHS score（ rs=0. 646, P =0. 007）.3. 4 cases of bleeding without bleeding, Pettersson or HJHS score abnormalities; 3 cases of Pettersson and HJHS scores were 0 for the study of joints, with joint bleeding; 5 cases of Pettersson or HJHS score abnormal study of joint, bleeding and another score of 0. Conclusions The greater the number of joint bleeding, the more obvious the impact of joint function. But only according to the bleeding assessment of joint disease to adjust the treatment is not comprehensive, but also need to combine the joint structure and function of comprehensive assessment.