| Objective: To evaluate the incidence and diagnosis and treatment status of hemophilia patients,and further optimize the management mode of hemophilia center for patients in Gansu Province;Methods: According to the patient information in the hemophilia registration system of our center,the data in the system were improved through telephone,QQ,We Chat and HIS case system of our hospital.Finally,the collected data were retrospectively analyzed.Results :1.There were 506 patients with reduced coagulation factor,including 500 males and 6females.There were 436 cases of hemophilia A,including 81 cases of mild type(18.5%),225 cases of intermediate type(51.7%),and 130 cases of severe type(29.8%).There were 41 cases of hemophilia B,including 12 cases of mild type(29.3%),19 cases of intermediate type(46.3%),10 cases of severe type(24.4%),4 cases of C type(24.4%),and 25 cases of deficiency of other coagulation factors.2.The 477 hemophiliacs were distributed in Dingxi,Lanzhou and Tianshui,followed by Linxia Hui Autonomous Prefecture.In 2016,12 cases were diagnosed and registered in Baiyin,and by 2020,47 cases were diagnosed and registered,significantly increasing compared with other regions.The number of enrolled patients was 43.2% with family history.3.Among the 477 hemophiliacs,102 patients(21.4%)had the highest education level from primary school,69 patients(14.5%)from junior high school,45 patients(9.4%)from senior high school or technical secondary school,16 patients(3.4%)from junior college,and 12 patients(2.5%)from university.43 patients(9.0%)were employed,97 patients(23%)were unemployed,4patients(0.8%)had intermittent employment,and 29 patients(6.1%)had dropped out of school due to disease.4.Among 477 hemophilia patients,316 cases(66.2%)were difficult to be referred to other places.Among the types of medical insurance,243 cases(50.9%)were covered by the new cooperative medical insurance,69 cases(14.5%)were covered by the resident medical insurance,14 cases(2.9%)were covered by the urban medical insurance,29 cases(6.1%)had no medical insurance,and 29 cases(6.1%)had no medical insurance.5.The mean age of the first hemorrhage of 477 hemophiliacs was 4.16±7.17 years old,and the median age was 2.00 years old;The age of diagnosis was 6.41±9.24 years old and the median age of diagnosis was 3.00 years old.The time of the first replacement therapy was 7.76±10.07 years old,and the median age of the first replacement therapy was 4.00 years old.The mean delayed diagnosis age was 2.24±6.39 years.6.In 477 patients with hemophilia,skin mucosa,joints,muscles and gums were the most common bleeding sites,no matter type A or type B.Among them,320 cases(67.1%)suffered from joint hemorrhage,and the knee joint hemorrhage was the most common.The maximum number of bleeding joints was 12,the minimum number was 1,and the average number was3.2.Seventy-five patients(33.9%)with joint bleeding developed joint deformity.7.Among 477 hemophilia patients,only 7.3% received different degrees of preventive treatment(intermittent prophylaxis and temporary prophylaxis),79.7% received on-demand treatment,and 5.0% had never received treatment.8.Among 477 hemophiliacs,7 cases(7.3%)had been tested for inhibitors,and 7 cases(18.9%)were positive.9.Among 477 hemophiliacs,the positive rates of hepatitis B virus,hepatitis C virus,syphilis and HIV were 2.6%,5.0%,1.9% and 2.5% respectively.Conclusion:1.Some hemophiliacs in our province are still missed diagnosis or have not been registered,and most of the confirmed cases are intermediate and severe patients.2.Dingxi,Lanzhou and Tianshui were the three regions with the most cases of diagnosis and registration,and the number of cases of diagnosis and registration in Baiyin increased significantly compared with 2016;Compared with the population of Linxia Hui Autonomous Prefecture,the incidence of hemophilia in this region is higher.3.The overall educational level of hemophiliacs is lower,and there are more dropouts and unemployment.4.The majority of hemophiliacs in our province are registered in rural areas,and the primary medical care cannot meet the medical needs of the patients.5.The age of delayed diagnosis is not different from that of the whole country,but the starting time of replacement therapy is still delayed.6.The incidence of joint bleeding and joint deformity was high and the quality of life was poor.7.The proportion of patients receiving preventive treatment is very small due to lack of timely treatment.8.The detection rate of coagulation factor inhibitors was low.9.The infection rate of hepatitis B,hepatitis C and HIV was high,but it was improved compared with the data of 2016 years. |
PDF Full Text Request