Comparative Analysis Of The Efficacy Of Standard Dose Versus Low Dose Coagulation Factor Ⅷ Prophylaxis On Joint Damage In Children With Hemophilia A

ObjectiveThe study investigates the progression of joint damage at each site(elbow,hip,knee and ankle)and the differences in joint damage at each site after different treatment and prophylaxis in children diagnosed with hemophilia A after standard dose or low dose prophylaxis of coagulation factor VIII,in order to provide reasonable guidance for the long-term prevention and treatment of hemophilia A in children.Methods17 male children diagnosed with hemophilia A were collected from the outpatient department of Shenyang children’s Hospital from April 2019 to August 2020,and a total of 102 joints were collected.After the kid and dependents obtaining informed consent,the children were treated with the standard dose group(n=6)or the low dose group(n=11).The standard dose group have 36 joints and low dose group have 66 joints.The standard dose group was treated with 25-40 IU / kg / time,three times a week or once every other day,while the low dose group was treated with 10 IU / kg /time,twice a week.After 1-year treatment,the joint bleeding condition of two groups were collected(time of joint bleeds and number of joint bleeds).The degree of joint injury(knee joint,elbow joint,ankle joint)of two groups was analyzed by joint ultrasound evaluation and joint health score(HJHS).The synovial thickness and joint effusion in different stage was tested by T-test analysis,while the HJHS score and the joint bleeding condition was tested by the wilcoxon rank sum test.Finally,according to the analysis and the comparison of different treatment,the result can show the difference between two groups in joint damage.Results1.The study collected 17 children diagnosed with hemophilia A in all,the joint-bleeding rate in standard dose group was 50%(3/6),which was 63.6%(7/11)in low-dose group.Compared with two groups,there was no significant difference in joint bleeding between the two groups(P>0.05).2.Comparison of synovial thickness in different joints(bilateral knees,bilateral elbows,bilateral ankles)was shown that there was no significant difference between standard-dose group and low-dose group(P>0.05).3.Compared with two groups in joint effusion,there was no significant difference between in left elbow,right elbow and right ankle(P>0.05).Whereas there was statistically significant(P<0.05)in left knee,right knee and left ankle and one-tailed T-test shown that the treatment efficacy of standard-dose group was better than the low-dose group.1.4.Comparing the HJHS scores of two groups,the result shown that the difference was statistically significant(P< 0.05)and the standard dose group was better than the low dose group in treatment efficacy.5.After one year treatment in standard dose group,there was no significant difference in synovial status,joint effusion and HJHS score in two groups(P>0.05).6.After one year treatment in low dose group,,there was no significant difference in synovial status,joint effusion and HJHS score between the two groups(P>0.05).Conclusion1.Standard dose prophylaxis in children diagnosed with hemophilia A is more effective than low dose prophylaxis in preventing joint damage.2.Both standard-dose prophylaxis and low-dose prophylaxis for coagulation factor VIII can delay the occurrence of joint damage to a certain extent.