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Study of effectiveness of prophylactic treatment of childhood hemophilia A in Qingdao hemophilia centerObjective : To discuss and analyze the clinical effects of prophylactic treatment in children with hemophilia A.Methods:32 children with hemophilia A who were diagnosed at the Affiliated Hospital of Qingdao University from September 1,2016 to February 1,2018 were recruited.They were grouped according to their ages: 16 preschool children and 16 school-age children.According to disease severing: medium-sized 27 cases,heavy 5 cases.All children were treated with prophylactic treatment,including 14 cases of moderate doses of prophylactic treatment,low-dose prophylactic treatment of 18 cases.To study the clinical manifestations,joint ultrasound scores,daily activities and disease family burdens before and after prophylactic treatment.Results : 1.(1)The frequency of bleeding,joint bleeding,and the incidence of major bleeding events in children after prophylaxis were significantly lower than those before treatment(p<0.05).(2)There was no significant difference in the frequency of bleeding and joint bleeding before treatments in the moderate-dose prophylactic treatment group compared with the low-dose group(p > 0.05).However,the frequency of bleeding and joint bleeding after treatments in the middle-dose group were significantly reduced compared with the low-dose group(p <0.05).(3)The frequency of bleeding and joint bleeding before prophylactic treatment of middle size children were less than that of the heavy group(p < 0.05).However,there was no significant difference in bleeding frequency and joint bleeding frequency between the middle group and the heavy group after prophylaxis(p>0.05).(4)The frequency of bleeding before prophylactic treatment in preschool children was significantly lower than that in children of school age,and the difference was statistically significant(p < 0.05).However,there is no significant difference in the frequency of bleeding between the school age children and the preschool age after prophylactic treatment(p>0.05).There was no significant difference in the frequency of joint bleeding before and after preventive treatment for school-age children with preschool children(p>0.05).2.There was no significant difference in the ultrasound scores of knee joints and ankle joints after and before prophylactic treatment.3.The ability of family life and the daily activities of school life after prophylactic treatment were significantly improved(p < 0.05).After prophylactic treatment,the children's schooling(6 years of or older and excluded has dropped out of school before prophylactic treatment)of 100%,100%~75%,75%~50%,50%~25% and 25%~0% were significantly improved(P < 0.05).4.The family burden,family activities,family entertainment activities,family relationships,family members' physical health and family members' mental health were significantly improved after preventive treatment(p<0.05).There was no significant difference in the economic burden on the family after preventive treatment in children Who were early treatment with on-demand treatment(p>0.05).Conclusions: 1.Severe hemophilia A children were more serious,and the incidence of bleeding and joint bleeding was higher than that of middle size.2.Preventive treatment can significantly reduce the frequency of bleeding and joint bleeding in children,and the clinical effect of middle dose prevention and treatment was better than low dose prophylactic treatment.3.Prophylactic treatment can obviously improve the progress of joint lesions.4.Prophylactic treatment can significantly improve the children's daily life ability,increase the proportion of schooling,reduce the family burden of the disease,and the family economic burden after the adoption of preventive treatment changes less than the previous on-demand treatment,which is worthy of clinical promotion.Part ?Hemophilia A osteoarthropathy in children: a report of 3 casesObjective : To study the efficacy of prophylaxis and treatment for children with hemophilia A osteoarthritis.Methods : Three children with hemophilia A with haemophilic osteoarthropathy who were dignosed at the Affiliated Hospital of Qingdao University from September 1,2009 to February 1,2018.To follow up their treatments and effects of preventive treatment.Results:Three patients had joint bleeding before joint prophylaxis,resulting in limited joint mobility.MRI examination showed hemophilic osteoarthropathy.After receiving prophylactic treatment,joint hemorrhage was significantly reduced compared with that before,and joint function gradually improved.But joint ultrasound showed that there are still different degrees of joint damage.Conclusions: Preventive treatment can significantly delay the progression of joint disease and reduce the disability rate,but it cannot change the existing bone and joint diseases.Part ?Hemophilia A Inhibitor Formation in Children:a report of 2 casesObjective:To study the treatment of acute hemorrhage and inhibitor removal in children with hemophilia A inhibitor.Methods : Two children with hemophilia A with inhibitors were diagnosed at the Affiliated Hospital of Qingdao University from September 1,2009 to February 1,2018 to follow up their treatments and effects of preventive treatment.Results : One case of hematuria recurred at the beginning of the disease and was improved after infusion of human prothrombin complex and dexamethasone immunosuppressive therapy.The later stage of the patient developed gastrointestinal hemorrhage and was gradually improved after being given recombinant human coagulation factor ?a(r F?a),human prothrombin complex,and blood product supportive therapy.One patient was treated with an immune tolerance treatment(ITI)clearance inhibitor,and the anti-F?antibody titer was gradually reduced to 1BU,thus resuming regular preventive treatment.Conclusions: 1.In the case of acute hemorrhage in children with positive inhibitors,the“reverse pathway” such as recombinant FVII(r F?a)and prothrombin complex(PCC)can effectively relieve bleeding.2.Immune tolerance treatment can effectively remove inhibitors. |
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