长期预防治疗的血友病儿童关节健康功能状况及其影响因素分析

Analysis of joint health function status and influencing factors in children with hemophilia after long-term preventive and treatment

目的探讨长期预防治疗血友病儿童的关节健康状况,并分析其影响因素。方法采用横断面研究,选取2018年1月至2021年8月于深圳市儿童医院康复科就诊的73例接受预防治疗的血友病儿童为研究对象,依据年龄分为幼儿组(22例)、少儿组(36例)和少年组(15例),使用关节健康功能评分(HJHS)评估其关节健康状态。将年龄、体质量、严重程度、预防治疗剂量、预防频率(次/周)、首次预防年龄、疗程、年出血次数、体育活动参与等因素作为变量进行相关分析和逐步线性回归分析关节健康功能影响因素。结果接受预防治疗的血友病儿童年出血次数为0(0,1.00)次,零出血率为61.64%。踝关节HJHS评分少年组5.00(1.00,12.00)分显著高于幼儿组0(0,0)分和少儿组1.00(0,4.75)分(P<0.05);少年组踝关节HJHS评分显著高于膝关节0(0,1.00)分和肘关节0(0,4.00)分(P<0.05)。相关性分析显示年龄、体质量、首次预防年龄、预防剂量与血友病儿童关节健康功能相关(P<0.05);多因素回归分析显示首次预防年龄、体质量、严重程度、体育活动参与和年出血次数是血友病儿童关节健康功能的独立影响因素。结论长期预防治疗的血友病儿童能够较好的维持关节健康功能,但踝关节仍有恶化可能,而影响关节健康功能的因素众多,预防治疗应基于疾病严重程度、体质量、年出血次数、生活方式和身体活动水平及其他因素尽早进行个体化治疗。

Objective To investigate the joint health status of children with hemophilia in long-term prevention and treatment,and analyze the influencing factors.Methods A cross-sectional study was conducted,73 children with hemophilia who underwent preventive treatment at the Rehabilitation Department of Shenzhen Children's Hospital from January 2018 to August 2021 were cselected as the study objects.They were assigned into three age groups:toddlers group(22 cases),children group(36 cases),and adolescents group(15 cases)according to age.Haemophilia Joint Health Score(HJHS)was used to assess joint health.The following factors including age,weight,severity,dosage of prophylaxis,frequency of prophylaxis(times/week),initial age of prophylaxis,treatment session,annual rate of bleeding,participation in sport activities,were variables for analysis of correlation and stepwise regression to explore effects on joint health.Results The annual rate of bleeding in children with haemophilia received prophylaxis was 0(0,1.00),zero bleeding rate was 61.64%.HJHS score for the ankle joint in the adolescent group 5.00(1.00,12.00)was significantly higher than that in the toddler group 0(0,0)and children group 1.00(0,4.75)(P<0.05).HJHS score for the ankle joint in the adolescent group was notably higher than that in the knee joint 0(0,1.00)and elbow joint 0(0,4.00)(P<0.05).Correlation analysis indicated that age,weight,initial age of prophylaxis,and dosage of prophylaxis were related with joint health in children with haemophilia(P<0.05).According to the multiple linear regression results,the age of initial regular prophylaxis,weight,severity,participation in sport activities and annual rate of bleeding were independent influencing factors on joint health function in children with haemophilia.Conclusions Children with hemophilia who undergo long-term prevention and treatment can maintain joint health and function well,but there is still a possibility of ankle joint deterioration.There are variable factors affecting joint health function,preventive treatment should be individualized as soon as possible based on the severity of the disease,body mass,annual bleeding rate,lifestyle,physical activity level and other factors.