| Objective:Using conventional ultrasound and hemophilia joint health score as observed indicators,to observe the progress of joint structure and function in children with severe hemophilia A receiving low and intermediate-dose for prophylaxis,and explore the value of superb microvascular imaging and contrast-enhanced ultrasound in the evaluation of hemophilic arthropathy vascularity,in order to provide reference for guiding the selection of individualized treatment.Methods:1.Ultrasound(US)and hemophilia joint health score(HJHS 2.1)examination were performed on the elbows,knees and ankles of 27 children with severe hemophilia A who received low and medium dose prophylactic treatment in Nanfang Hospital from March 2017 to April 2018.Used hemophilia APP to accurately record clinical information.According to the dose and frequency of prophylactic treatment,the joints were divided into low-dose group joints and middle-dose group joints.On the basis of the ultrasound score at the time of admission,the joints were divided into normal joints and diseased joints.All patients were assessed at baseline and followed up every 3-4 months.After 1-year of follow-up,comparing the joint progress of two different prophylactic treatments with low and medium doses and the progress of joint between normal group and pathological group under the same preventive treatment regimen,to document the US or HJHS score changes in joints without history of hemarthrosis and the US score changes in joints without HJHS score changes and the HJHS score changes in joints without US score changes.Analyze the association between the changes of US score and HJHS score of index joints and clinical information.2.Gray-scale ultrasound and Color Doppler flow imaging(CDFI)were performed on 29 patients with hemophilia A in Nanfang Hospital from September to December 2018,including elbows,knees and ankles,to examine synovium with the richest color flow signals(or if there is no obvious color flow signal in the thickened synovium,the thickest section of the synovium is selected.)adopting microvascular imaging and contrast-enhanced ultrasound,to detect the synovium vascularity.Adler semi-quantitative scoring method was used to semi-quantitatively evaluate CDFI and SMI blood flow signals in synovium,and contrast-enhanced ultrasound semi-quantitative scoring method was used to evaluate the extent of synovium enhancement.The degree of synovial neovascularization at different time points(1W,2W,3W,3W-3 months,>3 months)after joint hemorrhage was quantitatively analyzed by SMI and CEUS quantitative parameters.Used hemophilia APP to accurately record clinical information.Comparing the CDFI,SMI and CEUS in the evaluation of synovial neovascularization in patients with hemophilia.Quantitative analysis of synovial neovascularization at different time points after joint hemorrhage by SMI and quantitative contrast-enhanced ultrasound.The correlation between the doppler semi-quantitative score and quantitative parameters of SMI and CEUS and the number of bleeding in the past six months was analyzed.Results:1.A total of 157 joints of 27 patients were followed up for one year.There were 80 joints in the low dose group and 77 joints in the middle dose group.The changes of US soft tissue score,osteochondral score,total score and HJHS score in the low dose group were higher than those in the middle dose group,but there was no significant difference(P=0.127-0.753).There were 116 joints in the normal group and 41 joints in the diseased group.The changes of US osteochondral score,total score and HJHS score of joints in the diseased group were significantly greater than those in the normal group,with the same dose of treatment(P=0.000-0.001).The change of US soft tissue score in the diseased group was higher than that in the normal group,but there was no statistical difference(P=0.119、0.979).Of the 157 index joints enrolled in our study,74 index joints had a negative clinical history of bleeding into the joints during follow-up period,the changes of US total score greater than 0 in 14/74(18.9%)of index joints,whereas the changes of HJHS score greater than 0 in 11/74(14.9%)of index joints,among them,ankle joint is the most common.There were 108 index joints with no changes of HJHS score,Ultrasound detected the changes of joint structure in 25/108(23.1%)of index joints.However,93 index joints have no changes of US score,the changes of HJHS score greater than 0 in 9/25(9.7%)of index joints.The changes of US total score and HJHS score were positively correlated with the age of patients,the number of joint bleeding during follow-up and the state of joint structure at admission(P=0.000-0.006).However,there was no significant correlation with body mass index,coagulation factor activity level,age of first bleeding,age of first joint bleeding and preventive treatment(P=0.053-0.698)2.Forty-one hemophiliac joints of 29 patients were examined by gray-scale ultrasound,CDFI,SMI and CEUS on the same day.The detection rates of blood flow in synovial lesion joints of HA patients displayed by CDFI,SMI and CEUS were 21/41(51%)、34/41(83%)、41/41(100%),and the difference was significant(P=0.000-0.009).Semi-quantitative grading of synovial blood flow signals:CDFI grading:20(48.8%)in grade 0;12(29.3%)in grade 1;6(14.6%)in grade 2;3(7.3%)in grade 3;SMI grading,7(17.0%)in grade 0;19(46.3%)in grade 1;10(24.4%)in grade 2;5(12.2%)in grade 3;CEUS grading:0(0%)in grade 0;3(7.3%)in grade 1;3(7.3%)in grade 2;35(85.4%)in grade 3.Comparing the semi-quantitative scores of CEUS with CDFI and SMI,38 and 33 joint scores were upgraded respectively,and Compared the semi-quantitative score of SMI with CDFI,20 joint scores were upgraded.The highest synovial neovascularization occurred in the third week after articular hemorrhage.SMI vascular index,CEUS Peak intensity(PT)and Area under curve(AUC)reached the highest level in the third week after articular hemorrhage,they were6.81±1.51、46.29±10.69、2.94 ± 0.51,respectively.The number of bleeding in the past six months was positively correlated with the semi-quantitative score of CDFI,SMI and CEUS,SMI vascular index and PT(r=0.408-0.597),P<0.05,and negatively correlated with The peak time(TTP)(r=-0.421),P<0.05.There was no correlation between the number of bleeding in the past six months with rising slope(AS)and AUC.Conclusions:1.(1)Low dose has a tendency of rapid progress in prevention and treatment of hemophilia joints with medium dose.However,short-term low-dose prophylaxis can also control the progress of joints,and long-term efficacy observation needs longer follow-up.(2)The difference of joint basic state has an effect on the progress of joint disease,and the worse the joint state is,the faster the progress is.(3)US and HJHS examination are better than clinical hemorrhage in evaluating the changes of joint structure and function.It is necessary to evaluate hemophilia joints comprehensively.2.(1)Compared with CDFI,SMI and CEUS showed a higher rate of synovial blood flow in hemophiliac joints,especially in CEUS.(2)The highest synovial neovascularization occurred in the third week after articular hemorrhage,and the number of bleeding in the past six months was positively correlated with the synovial neovascularization.Accurate assessment of synovial neovascularization is helpful for the early diagnosis and adjustment of treatment of hemophilia joints,in order to achieve individualized treatment. |
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