杨灿,班灵英,程山,颜永燕,金皎
YANG Can,BAN Lingying,CHENG Shan,YAN Yongyan,JIN Jiao

• 1:贵州医科大学附属医院儿科血液专科
• 2:贵州医科大学临床医学院
• 3:贵州医科大学
• 4:贵州医科大学附属医院超声科

摘要(Abstract)：

目的 探讨血友病A患儿关节出血与关节结构的相关性。方法 选取3～18岁血友病A患儿89例，收集患儿的一般资料(性别、年龄、诊断及治疗时间等);根据治疗方案不同分为预防治疗组(长期且定期静脉注射FⅧ,n=20)、按需治疗组(发生出血时注射FⅧ,n=15)及按需转预防组(早期使用按需治疗，后期转为预防治疗，n=54),预防治疗组又分为大剂量预防组(n=9,25～40 U/kg)、中剂量预防组(n=5,15～<25 U/kg)及小剂量预防组(n=6,10～<15 U/kg);采用近3年年关节出血率(AJBR)评价各组血友病A患儿关节出血情况，采用彩色多普勒超声诊断仪检查血友病A患儿双侧肘关节、踝关节及膝关节、并使用中国血友病早期关节超声检测(HEAD-US-C)量表进行关节结构评分，采用Pearson法分析血友病A患儿关节出血与关节结构评分的相关性。结果 3～6岁组血友病A患儿关节出血阳性率及关节病变检出率分别低于7～18岁组，差异均有统计学意义(P<0.05);预防治疗组血友病A患儿关节出血阳性率分别低于按需治疗组、按需转预防治疗组(P<0.05),按需转预防治疗组关节病变检出率低于按需治疗组(P<0.05);按需治疗组患儿关节软骨病变及骨病变检出率高于预防治疗组，差异均有统计学意义(P<0.05);血友病A患儿中关节出血阳性者的关节病变检出率高于关节出血阴性者，差异有统计学意义(P<0.05);血友病A患儿AJBR与HEAD-US-C总评分有相关性(r=0.548,P<0.05)。结论 血友病A患儿关节出血越多，关节结构损伤越重；FⅧ预防治疗可减少血友病A患儿关节出血。
Objective To explore the correlation between joint hemorrhage and joint structure in children with hemophilia A. Methods Eighty-nine children with hemophilia A aged from 3 to 18 were selected and their general data were collected(gender, age, diagnosis, and treatment time, etc.). According to different treatment methods, they were divided into prevention treatment group(long-term and regular intravenous injection of FⅧ, n=20), on-demand treatment group(FⅧ was injected when bleeding, n=15), and the on-demand transfer to the prevention group(the method was transferred from on-demand treatment to prevention treatment, n=54); the prevention treatment group was sub-divided into high-dose prevention group(n=9, 25-40 U/kg), medium-dose prevention group(n=5, 15-<25 U/kg) and low-dose prevention group(n=6, 10-<15 U/kg). The annual joint bleeding rate(AJBR) in recent 3 years was used to evaluate the joint bleeding of children with hemophilia A in each group; the bilateral elbow, ankle and knee joints of children with hemophilia A were detected by color Doppler ultrasound; Hemophilic Early Arthropathy Detection with UltraSound in China(HEAD-US-C) was used to score the joint structure; Pearson test was used to analyze the correlation between joint hemorrhage and joint structure score in children with hemophilia A. Results The positive rate of joint hemorrhage and the detection rate of joint lesions in the 3-6-year-old group were lower than those in the 7-18-year-old group(P<0.05) and the difference had statistical significance; the positive rate of joint hemorrhage in the preventive treatment group was lower than that in the on-demand treatment group and the on-demand preventive treatment group(P<0.05); the detection rate of joint lesions in the on-demand-to-preventive treatment group was lower than that in the on-demand treatment group(P<0.05); the detection rate of articular cartilage disease and bone disease in the on-demand treatment group was higher than that in the preventive treatment group(P < 0.05) and the difference had statistical significance; the detection rate of joint lesions in patients with positive joint hemorrhage was higher than that in patients with negative joint hemorrhage(P<0.05) and the difference had statistical significance. AJBR was correlated with the total score of HEAD-US-C(r = 0.548, P<0.05). Conclusions The more joint hemorrhage the children with hemophilia A have, the more serious joint structure damage they have; FⅧ preventive treatment can reduce joint hemorrhage in children with hemophilia A.

关键词(KeyWords)： 血友病A;关节出血;关节结构;年龄;治疗方式;个体化治疗
hemophilia A;joint bleeding;joint structure;age;treatment;individualized treatment

Abstract:

Keywords:

基金项目(Foundation): 贵州省科技计划项目(黔科合平台人才[2019]5406);; 贵州省卫生健康委科学技术基金(gzwjkj2019-1-002)

作者(Author): 杨灿,班灵英,程山,颜永燕,金皎
YANG Can,BAN Lingying,CHENG Shan,YAN Yongyan,JIN Jiao

DOI: 10.19367/j.cnki.2096-8388.2023.01.014

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