| Background and Objective:1.Joint bleeding is the most common and characteristic bleeding manifestation of hemophilia patients.At present,there is no joint outcome study on young patients with severe hemophilia A a receiving long-term low and medium dose preventive treatment at home and abroad.Therefore,the first chapter of this study aims to compare and analyze the effects of different alternative treatment methods on long-term prevention of joint lesions in young patients with severe hemophilia A A by means of comprehensive evaluation,At the same time,the correlation between the evaluation indexes is analyzed to provide a basis for formulating a more suitable treatment plan for hemophilia patients in China.2.In patients with severe hemophilia,muscle bleeding accounts for about 30%of all bleeding episodes,and the incidence is second only to joint bleeding,while iliopsoas muscle bleeding accounts for 55%of all muscle bleeding events,and its clinical manifestations are diverse,which is easy to cause missed diagnosis and misdiagnosis.Those with insufficient treatment have a higher risk of early and late complications,which seriously affects the quality of life of patients and even threatens the life safety of patients.Therefore,the second chapter of this study aims to explore and analyze the clinical characteristics of iliopsoas muscle bleeding in patients with hemophilia in our center,in order to improve doctors’ and patients’ understanding of the disease.Methods:1.This study is a single center,prospective and observational study.The subjects were selected to enter the on-demand treatment group,low-dose prevention treatment group or medium dose prevention treatment group according to their family economic conditions and treatment willingness,and were followed up for 48 months according to their clinical bleeding phenotype,joint structure,joint function and quality of life.2.The case data of 28 patients with hemophilia complicated with iliopsoas muscle bleeding treated in our hospital from January 2008 to December 2021 were collected,and their clinical characteristics,including symptoms,signs,imaging data,complications and treatment,were analyzed retrospectively.Results:1.49 patients with relatively complete follow-up were included in this study.The median follow-up time was 43.13 months.A total of 118 target observation joints were followed up and analyzed.In terms of clinical bleeding phenotype,there was significant difference between the on-demand group and the prevention and treatment group,and there was no statistical significance between the low and medium dose groups.Long-term prevention and treatment could significantly improve the annualized joint bleeding rate.In terms of joint structure,the ultrasound and MRI scores of the three groups increased after 48 months of follow-up,but the growth rate of the three groups was significantly different.The ultrasound scores of the patients in the medium dose prevention group began to show a downward trend at 24 months of follow-up,and the MRI scores tended to be stable throughout the follow-up period.In terms of joint function,the hjhs score in the on-demand group showed a continuous growth trend,the low and medium dose group showed a downward trend as a whole,and there was no significant difference between the low and medium dose groups.In terms of quality of life,the CHO-KLAT scores of the three groups were improved,and the improvement degree of the low-dose group was the highest.In terms of correlation,there was a low positive correlation between the annual observation of joint bleeding rate and the score of joint structure and function;There was a significant positive correlation between joint structure and function,as well as between joint ultrasound and MRI scores.2.Iliopsoas bleeding:the 13-year cumulative incidence of single center hemophilia combined with iliopsoas bleeding in our hospital is 2.64%.28 hemophilia patients have 33 iliopsoas bleeding events,50%of which are non severe hemophilia patients.The median bleeding age is 22.0(7-46)years old.Nearly 2/3 of the bleeding events have no obvious inducement.The most common pain site is thigh root pain,the most common bleeding site is inguinal iliopsoas muscle,and the recurrent bleeding rate is 7.14%,The femoral nerve involvement rate was 69.70%,the median duration of symptoms before treatment was 5(0-15)days,and the median length of hospital stay was 10(4-44)days.64.00%of the patients received low-dose alternative treatment to stop bleeding.The hematoma absorption effect was poor,and the total course of treatment was prolonged.After treatment,1/3 of the patients still had no significant improvement in femoral nerve injury.22.22%of patients produced inhibitors during the treatment of iliopsoas bleeding,and 18.18%of patients formed hemophilic pseudotumor.Conclusions:1.The long-term follow-up study shows that compared with ondemand treatment,low and medium dose preventive treatment can significantly improve the clinical bleeding phenotype of adolescent patients with hemophilia A A,delay the progress of joint structure injury,reverse joint function and improve the quality of life of patients.Continuous adherence can better reflect the benefits of prevention and treatment;Moreover,long-term medium dose preventive treatment may reverse joint structural lesions.Therefore,it is advocated that Chinese patients with hemophilia should carry out medium dose preventive treatment for a long time when economic conditions permit and the acquisition of coagulation factors is not limited.Based on the correlation between different evaluation indexes,it is recommended to use comprehensive evaluation methods to monitor joint diseases and treatment effects.2.Iliopsoas bleeding mostly occurs in young patients with hemophilia.Most patients have delayed diagnosis and treatment,and due to limited economic conditions and access to coagulation factors,most patients have insufficient alternative treatment,poor treatment effect,prolonged course of treatment,and significantly increased risk of early and late complications.Hemophilia patients are prone to produce inhibitors after severe iliopsoas bleeding and high-intensity coagulation factor infusion.After hemostasis and inhibitor removal treatment,the curative effect of iliopsoas hematoma may still be poor,and serious complications are easy to occur.The length of hospital stay,the number of hospital stays and the consumption of coagulation factors are significantly increased. |
