Investigation And Analysis Of Diagnosis And Treatment Of The Children With Hemophilia A In Qingdao

Objective:To understand the current status of diagnosis and treatment of hemophilia A in children in Qingdao City,and to summarize the experience of diagnosis and treatment,so as to provide the basis for the implementation of comprehensive care for hemophilia.Methods:79 children with hemophilia A were enrolled and treated in our hospital from September 1,2009 to September 1,2016.All of them were male,consisted of 63 moderate haemophilia A and 16 severe haemophilia A.The children were divided into two groups according to their age,such as preschool children(0~7 years old)and school-age children(7~18 years old)and there were 28 preschool children and 51 school-age children.To understand the disease information of children through the questionnaire,and the questionnaire results were analyzed.Results:1.The median age of the first bleeding was 10 months,with the most common bleeding skin and muscle hematoma(48/79,60.76%),and spontaneous bleeding is the most common cause(42/79,53.16%);The median age of the first bleeding in moderate hemophilia A was 11 months,but severe hemophilia A was 7 months,and there was significant difference between the two severity groups(p<0.05).The first location of bleeding and Cause of the first bleeding between moderate haemophilia A and severe haemophilia A had no significant difference(p>0.05).2.69 cases had joint bleeding,and the rate of joint bleeding was 87.34%,with the most common knee joint bleeding(30/69,43.48%);The median age of the first joint bleeding was 1years and 6 months,and traumatic bleeding was the most common cause of joint bleeding(59/69,85.51%);The median age of the first joint bleeding in children with moderate hemophilia A was 24 months,and the severity of the disease was about 13 months.The difference between the two groups was statistically significant(p< 0.05).3.The median age of the first diagnosis was 1years and 4 months,with moderate hemophilia A 16 months and severe hemophilia A 15.5months,but there was no significant difference between the two severity groups(p > 0.05).4.There were 35 cases adopted on-demand treatment(44.30%),and 44 cases adopted prophylactic treatment(55.70%).Among the children who adopted prophylactic treatment,there were 23 cases adopted low dose prophylaxis,and 21 cases adopted moderate dose prophylaxis,but no standard dose prophylaxis was used regretfully.24 cases due to economic reasons or declined repeated puncture and did not take prophylactic treatment.5.In preschool children,the difference of the frequency of bleeding between the two treatment methods was statistically significant(p<0.05),but the difference of the frequency of joint bleeding and HEAD-US score between the two groups were not statistically significant(p>0.05).In school-age children,the difference of the frequency of bleeding and joint bleeding,HEAD-US score,the number of target joints between the two treatment methods were statistically significant(p<0.05).6.After treatment,the frequency of bleeding and the frequency of joint bleeding were significantly lower than those before treatment,and the difference was statistically significant(p<0.05);There were no significant difference in the frequency of bleeding and joint bleeding before treatment between low dose group and medium dose group(p>0.05).Compared with the low-dose prophylaxis group,the frequency of bleeding,the frequency of joint bleeding and HEAD-US score of the medium dose group were lower,and the number of target joints was less,the difference was statistically significant(p<0.05).7.There were no significant difference in the enrollment ratio of preschool children between on-demand treatment group and prophylaxis group(p>0.05);The difference of the enrollment ratio of school-age children between on-demand treatment group and prophylaxis group was statistically significant(p<0.05).8.The activities of daily living of children with hemophilia A were damaged to varying degrees,among them,the elder children(12-18 years old)on demand treatment group were more severe,in addition,2 cases lost the ability to walk independently.9.There were 4 patients Inhibitor test positive,with the positive rate 5.06%,among the positive,3 cases(8.57%)from on demand treatment group,and 1 cases(2.27%)from prophylaxis group,and the difference between them was statistically significant(p<0.05).10.All children with hemophilia A were enjoyed the serious illness insurance policy,with outpatient drug reimbursement up to 80%,and the cost of prophylactic treatment is 1.8 times as much as on demand.Conclusions:1.The skin and muscle hematoma is the most common site of first bleeding site in children with hemophilia A in Qingdao city.The age of first bleeding and the first joint bleeding in children with severe hemophilia A were earlier than that in moderate children.There was no significant difference in the location and cause of the first hemorrhage and the cause of the first joint bleeding and age of the diagnosis between moderate and severe hemophilia A.2.The effect of prophylactic treatment was better than on-demand treatment.and the effect of medium dose group is better than that of low dose prophylaxis.3.The replacement treatment of children with hemophilia A in Qingdao was better than that of the former,but the proportion of the overall prophylaxis and prophylaxis dose was still low.4.Economic reasons and the inability to receive repeated venipuncture are two major factors that prevent children from taking prophylactic treatment.5.The incidence of joint bleeding in children with hemophilia A in Qingdao City is high,and the school-age children are more severe than the preschool children.6.The positive rate of F? inhibitor was low,and the preventive treatment did not increase the incidence of inhibitor.7.The treatment costs of Hemophilia A is huge,and the perfect medical insurance system is the guarantee of children's reasonable treatment.