目的 运用国际血友病预防治疗研究小组(international prophylaxis study group,IPSG)MRI评分系统评估并量化儿童血友病性骨关节病的严重程度,预测评分结果与临床因素关系.方法 分析2008年9月至2012年6月间首都医科大学附属北京儿童医院18例中重度血友病患儿的22个出血关节的MRI改变.检查序列:冠状位及矢状位T1WI、T2WI、T2W FFE序列,对影像所见通过IPSG评分系统分别从软组织改变及骨软骨改变方面进行评分,评价受检关节改变的严重程度,同时进行关节受累与临床因素的相关性分析.结果 按照IPSG评估项目逐条评判,22个关节中全部可见软组织方面改变:关节积液18/22(81.8%),滑膜增厚20/22(90.9%),含铁血黄素沉积19/22(86.4%).关节软组织改变轻、中、重度比例分别为:9/22(40.4%),7/22(31.8%),6/22(27.3%);关节骨软骨方面正常、轻、中、重度比例分别为:8/22(36.4%),3/22(13.6%),7/22(31.8%),4/22(18.2%),其中关节表面骨破坏者14/22(63.6%),关节面下囊肿形成9/22(40.9%),关节软骨缺失14/22(63.6%).通过Kappa检验两评判者间评分结果的一致性良好.出血频度、年龄与MRI观察指标中骨软骨改变呈中度相关(r=0.501,P=0.018;r=0.557,P=0.007);年龄与IPSG总分呈中度相关(r=0.543,P=0.009);另外患儿首次出血年龄与关节面下囊肿形成呈负相关(r=-0.479,P=0.044).而关节软组织病变得分与临床因素间无明显相关性(|r|<0.3).结论 IPSG评分系统的应用可以将MRI图像异常征象量化,分别从关节软组织及骨软骨方面评估关节受累严重程度并分级,提示相关临床因素与关节病变之间的联系.%Objective The International Prophylaxis Study Group (IPSG) magnetie resonance imaging (MRI) scale was used to evaluate the severity of haemophilic arthropathy joints and analyze the correlation of MRI performance and clinical factors. Methods Sagittal and coronal T1W, T2W and T2W FFE MRI images of knees, ankles and elbows were obtained from 22 joints of 18 boys who were clinically diagnosed hemophilia were studied [age range from 2 to 15 years; mean (8.06 ± 3.37) years] with moderate and severe hemophilia diagnosed in our hospital. The severity of hemophilia from soft tissue and osteochondral domains with the IPSG MRI scale system was assessed. Results The soft tissue domain of 22 joints were appraised with regard to frequency of findings: effusion, 18/22 (81. 8% ) ; synovial hypertrophy, 20/22(90. 9% ) ; hemosiderin deposition, 19/22(86. 4% ). The severity of soft tissue joint changes on MRI was mild, 9/22 (40. 4% ) ; moderate, 7/22 (31. 8% ) ; and severe 6/22 (27. 3% ) , respectively. The severity of osteochondral joints changes on MRI was normal, 8/22(36. 4% ) ; mild, 3/22 ( 13. 6% ) ; moderate, 7/22(31. 8% ) ; and severe, 4/22 ( 18. 2% ) , respectively. The frequency of bone erosions was 14/22(63. 6% ) ,bone cysts, 9/22(40. 9% ) ,and cartilage loss, 14/22(63. 6% ). The intrareader reliability for each item of the IPSG MRI scale was excellent by Kappa analysis. The correlations between the osteochondral domain of the MRI scale and bleeding frequency as well as patients' age were moderate(r =0. 501 ,P = 0. 018 ;r = 0. 557 ,P = 0. 007). The correlation between the IPSG final score and patients' age was also moderate ( r = 0. 543 ,P = 0. 009). The correlation between age at first bleed and presence of subchondral cysts was moderate(r= -0. 479, P = 0. 044) as well. Otherwise, no significant correlations were noted between items of the soft tissue domain and clinical factors (|r| < 0. 3). Conclusion The IPSG scoring system is highly reliable within readers' interpretation, is able to discriminate severity of joint changes in HA and provide detailed evaluation of soft tissue and osteochondral joint components. However, its correlation with clinical constructs is suboptimal.
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