Significance Of Adjusting Coagulation Factor Ⅷ Prophylactic Dose In Children With Hemophilia A Under The Guidance Of Joint Ultrasound Assessment And Joint Health Score

Purpose: To investigate the joint injury status of children meeting the diagnostic criteria for hemophilia A based on joint Ultrasonic Assessment Scale(HEAD-US-C score)and joint health Assessment Form(HJHS score 2.1 version).Observe the improvement of joint injury(knee,ankle,elbow)and activity ability and quality of life of the children after adjusting coagulation factor Ⅷ preventive treatment dose according to HEAD-US-C and HJHS scores.Methods: A total of 20 male children with hemophilia A who were treated in the outpatient department of Shenyang Children’s Hospital from February 2019 to May 2021 were retrospectively analyzed,and a total of 120 joints were collected.All the children were diagnosed with severe hemophilia A when the level of coagulation factor VIII was below 1%.After informed consent of the children and their families,the children with hemophilia A were divided into two groups according to whether the dose was adjusted based on the HEAD-US-C and HJHS scores,that is,the children and their parents were strictly guided by the doctor to monitor the structure and function of the joint regularly.The children who adjusted the dose according to HEAD-US-C and HJHS scores were the case group(n=12).The compliance of children and their parents was poor,and HEAD-US-C and HJHS scores were not monitored regularly according to the doctor’s advice,and the treatment dose was adjusted according to the number and severity of clinically observable joint bleeding(n=8).Six joints(both knees,ankles and elbows)of each child were evaluated,with 72 joints in the case group and 48 joints in the control group.The differences of joint injuries between the two groups were observed,and the number and frequency of joint bleeding during treatment were recorded.Joint ultrasonic evaluation scale(HEAD-US-C score)and joint health evaluation form(HJHS score 2.1version)were used to comprehensively evaluate the extent of joint injuries(knees,ankles,elbows)between the two groups.FISH score and CHO-KLAT2.0 were used to evaluate the children’s mobility and quality of life.SPSS 26.0 statistical software was used for data processing,Fisher exact probability method was used to compare and analyze the changes in the incidence of joint injury in the two groups,and T-test was used to compare and analyze the changes in the scores of HEAD-US-C,HJHS,FISH and Cho-klat2.0 in the two groups.In order to clarify the improvement of joint injury and the improvement of mobility and quality of life in children with hemophilia A after adjusting the dosage of coagulation factor Ⅷ preventive therapy according to the HEAD-US-C and HJHS scores.At the test level P < 0.05,the difference was statistically significant.Results: 1.The study found that among 20 confirmed children with hemophilia A,the joint injury rate of children in the case group was 0%(0/12)and that of children in the control group was 37.5%(3/8).The joint injury rate of the two groups was significantly different(P<0.05).2.In terms of joint structure evaluation,the HEAD-US-C score of the two groups of children in all parts of the joint(knee,ankle,elbow)was compared,the case group was better than the control group,the difference was significant(P<0.05).3.In terms of joint health evaluation,the HJHS scores of all parts of the joints(knees,ankles,elbows)in the two groups were compared,and it was found that the case group was better than the control group,the difference was significant(P<0.05).4.In terms of functional independence score of hemophilia,the comparison of FISH score of daily activity ability between the two groups showed that the case group was better than the control group,and the difference was significant(P<0.05).5.In terms of quality of life evaluation of children with hemophilia,the comparison of CHO-KLAT quality of life score between the two groups showed that the case group was more conducive to improving the quality of life of children,and the difference was significant(P<0.05).6.In the case group,after adjusting the dose of preventive therapy according to HEAD-US-C score and HJHS score,the scores of HEAD-US-C,HJHS score and FISH,CHO-KLAT quality of life score of all joints(knees,ankles,elbows)were better than those before the dose adjustment,with significant differences(P<0.05).7.In the control group,there were no significant differences in the scores of HEAD-US-C,HJHS and FISH,CHO-KLAT quality of life in all joints(knees,ankles and elbows)after adjusting the dose of preventive treatment according to the number and severity of bleeding seen clinically(P>0.05).Conclusions: 1.In children with hemophilia A,the preventive effect of adjusting the dosage of coagulation factor Ⅷ on joint injury,the daily activity ability and the quality of life of the children according to the HEAD-US-C and HJHS scores are superior to the program of adjusting the dose according to the number and severity of bleeding.2.When the dose of preventive therapy was adjusted according to the number and severity of joint bleeding,the joint injury of some children was not significantly improved compared with that before adjustment,or even made some progress.The daily activity ability and quality of life of the children were not significantly improved compared with that before treatment.