| ObjectiveTo know more about the clinical features,diagnosis,treatment and survival status of patients with hemophilia through investigation and analysis at the center in Chongqing,aiming to provide clinical data for hemophilia development in China.MethodsStatistically analyzed the data of 339 patients who diagnosed and registered in the hemophilia center from 2009 to 2017 through the electronic medical record reviews,questionnaires and telephone follow-up data collections,etc.(The significance level was set at P<0.05).Results1.339 patients were all male,5 special types included.286 of them had hemophilia A(severe 51.1%,moderate 39.5%,mild 9.4%),while 48 had hemophilia B(severe 62.5%,moderate 37.5%,mild 0%).Special types: 1 case lacked FV and FVIII;1 case lacked FVIII,FIX and FXI;1 case lacked FVII;1 case lacked FXI while 1 case was v WD.105 of them(30.97%)had hemophilia family history(90 cases of HA and 15 cases of HB).2.The median age of first bleeding in HA/HB was 1 year old(1month to 52 years old),average age was 2.92 years old.There was a significant difference among mild group,moderate group and severe group when it came to the first bleeding age of HA(P<0.05),there was also a significant difference between moderate group and severe group of HB(P<0.05).Patients were diagnosed at a median age of 2 years old(1month to 65 years old)with an average age of 7.5 years old.The median age of beginning replacement therapy was 10 years old(1month to 68 years old),and the average age was 15.32 years old.There were significant delays in diagnosis and treatment.3.The majority of the reason causing first bleeding in HA/HB both was traumatic bleeding,followed by spontaneous bleeding and iatrogenic bleeding.There was a significant difference in the reasons of first bleeding in HA with different severity(P<0.05),while there was no significant difference between severe and moderate group in HB(P>0.05).4.The majority of the first bleeding position in HA/HB was skin,followed by joints(37.43%),muscles(11.97%),internal bleeding(1.5%),and intracranial bleeding(1.2%).There was no difference in the incidence of skin bleeding in HA and HB with different severity(P>0.05).5.Among 334 patients with HA/HB,295 cases had joint bleeding(88.3%).Knee(74.24%)was the most common position,followed by ankle(35.25%)and elbow(32.2%).There were 51 cases of bleeding involving more than three joints(17.29%).6.Among 334 patients,291 cases(87.13%)had on-demand treatment,80.1% of them were able to infuse as early as possible,while 43 cases(12.87%)had prophylaxis treatment.The number of annual bleeding times and disability rate in patients undergoing prophylaxis treatment was significantly lower than those treated on-demand(P<0.05).In the prophylaxis treatment,there were 0 cases of primary treatment,19 cases of secondary treatment,and 24 cases of tertiary treatment.The disability rate of secondary prophylaxis treatment was significantly lower than that of tertiary prophylaxis treatment(P<0.05).(5 special types not included)7.A total of 200 knee joints in 103 patients were evaluated by ultrasound.The HEAD-US score was 0 to 8 with an average score 3.49.There was no difference in the ultrasound scores of knee joint with different severity in HA and HB(P>0.05),while there was a significant difference when it came to bleeding times in one year(P<0.05).8.The virus infection rate: HCV(2.64%),HBV(5.29%),HIV(0.44%).The rate of FVIII/FIX inhibitors was 3.9%.9.HJHS assessment was performed among 46 patients.The scores of patients who bled less than 10 times a year ranged from 0 to 12 with an average score of 6.67.The scores of patients who bled 10 to 30 times a year ranged from 7 to 30 with an average score of 18.44.The scores of patients who bled more than 30 times a year ranged from 14 to 81 with an average score of 31.22.ConclusionIn the survey,patients with hemophilia A?B was approximately 6?1,most of them were severe.The clinical manifestations were mainly skin bleeding and joint bleeding.The primary cause of first bleeding was trauma,and the main bleeding joint was knee.There were delays between diagnosis and therapy,and the treatment was mainly based on on-demand treatment while prophylaxis treatment was revealed superior to the former in reducing bleeding frequency and disability rate.The times of bleeding was a direct factor influencing the HEAD-US score of knee joints with hemophilia.The patients' awareness of self-management and prenatal diagnosis was poor.Dropouts and unemployment were common.Therefore,it is necessary to increase the support of the society.At the same time,hemophilia centers should play full roles in management,publicity,and education. |
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