| [Objective]To understand the clinical history and treatment of hemophilia A patients in Yunnan Province,by ultrasonography and joint function score table of systematic evaluation of the degree of patients with joint disease,the joint bleeding,and joint damage,analysis of related factors of haemophilic arthropathy,according to the statistical analysis and results for the treatment of hemophilia A in Yunnan province the patient developed a more consistent with the characteristics of Yunnan Province,economic benefits,to improve hemophilia A patients in Yunnan Province,reduce joint abnormality rate,improve the overall quality of life.[Methods]The HJHS2.1 scores were evaluated in patients with hemophilia a joint health,through the joint ultrasound assessment of joint disease and according to the HEAD-US score table score,from September 2011 to December 2017 to the First Affiliated Hospital of Kunming Medical University were diagnosed with hemophilia A in our hospital and hemophilia disease information management center and the center of diagnosis and treatment of the registration and management of hemophilia A patients 44 cases,through the questionnaire survey and telephone follow-up and outpatient follow-up,parents diaries and other data collection methods,and evaluate the:knees,elbows and double ankle joint ultrasound examination and health survey table collected clinical information,ultrasound score results and joint health evaluation results using SPSS22.0 statistical software for statistical data analysis.Related factors of treatment of hemophilia and obtains Yunnan Province joint disease.[Results]1.the first bleeding age and the age of diagnosis:collected 44 cases of patients with hemophilia A the first bleeding age and the age of diagnosis,the first bleeding median age was 1 years,with a median age of 2 years,two sets of data for the rank sum test comparison,was statistically significant difference between the two results,P<0.0001 for the first time,bleeding age is the age of diagnosis compared to existing diagnosis delay.2.Severe bleeding incidence in light of severe hemophilia:44 cases of patients with hemophilia A first hemorrhage were divided into mild,moderate and severe bleeding bleeding bleeding in 3 groups,according to the activity of coagulation factors were divided into light,medium and severe haemophilia hemophilia,hemophilia,again using four tables from the Fisher exact test.The results showed that P = 1(>0.05),the difference was not statistically significant,that in this group of severe bleeding in severe hemophilia in light and medium had no significant difference.3.the total number of joint bleeding compared with 44 knees,elbows,patients with hemophilia A double ankle bleeding times,three subjects were analyzed,the results of single factor variance P<0.0001,the difference was statistically significant between the three groups,and then the double elbow and double ankle bleeding times for the total rank test,P=0.8853(>0.05),there was no significant difference between the two groups,so the data in the most easy bleeding for double knee joint,while the double elbow and double ankle bleeding times had little difference.4.Target joints(Target joint,TJ)The correlation analysis was made between the birth area,the education level of the parents,the education level,the number of education activities,the medical insurance,the number of annual bleeding,age and the number of the targets of the target joint.The results showed that the age,the number of annual bleeding and the activity of coagulation factor were related to the number of target joints(P=0.0392,P=0.0001,P=).0.0171,the P value is<0.05).5,the statistical treatment of hemophilia A patients 44 cases,most of the patients(55%)still use on-demand therapy in severe hemophilia patients,67%for on-demand treatment,only a small part(10%)of small dose of prevention and treatment,prevention and treatment of 1 cases of middle dose only,no standard dose prevention treatment 20%,and for lack of small dose of prevention and treatment.The lack of small dose prevention treatment and preventive treatment of age with small doses of prophylactic treatment compared to lack of small dose for prevention and treatment of low dose preventive treatment regimens,start treatment early age(P=0.0457,P=0.0427),and the on-demand treatment with the number of years of joint bleeding prevention and treatment and lack of prevention and treatment of small dose compared prevention and treatment of small dose,lack of treatment and prevention of double knee joint of 6 years the number of bleeding compared with on-demand therapy(P<0.05)less,according to the joint ultrasound treatment score with prevention and treatment and shortage of small dose of prevention and treatment,prevention and treatment group of the right knee and left elbow joint ultrasound score than according to the need of the low treatment group(P=0.0002,P=0.0104,P value<0.05),ultrasound score of small dose of preventive treatment group in double knee compared to on-demand treatment with low(P=0.0207,P=0.0075,The P value is<0.05).The data will be insufficient for prevention and treatment of low dose and small dose of prevention and treatment before and after treatment of joint bleeding times compared to years,lack of prevention and treatment of small dose and small dose of prevention and treatment in the prevention and treatment of bleeding compared to the number of years after the start of treatment before the reduction(P=0.0317,P= 0.326),so that our province still exists in patients with hemophilia prevention and treatment,treatment is not standardized,not universal factors such as insufficient dosage.In combination with the special economic situation in our province,the early and long course of low dose prevention and treatment can be recommended in some patients who can not be treated with small dose of prophylaxis.6.Comparison of joint bleeding times<3 years and more than 3 times/year/year difference between the two groups of ultrasound and HJHS score of 44 cases of patients with hemophilia 264 joints,according to the number of years is divided into joint bleeding less than 3 times per year and more than 3 times/year two groups,comparing the difference between two groups ultrasound score and HJHS score,joint annual bleeding more than 3 times/year than the number of bleeding<3 times/year of ultrasound and HJHS scores were high(P<0.0001,P<0.0001),recommended hemophilia patients control years joint bleeding<3 times in patients with delayed progression.7.The related factors of haemophilic arthropathy analysis 44 cases of 264 joints of patients with hemophilia A the course of time,the total number of joint bleeding,alternative treatment,coagulation factor levels,age,for the first time the first bleeding degree of bleeding,prevention treatment,prevention and treatment methods were analyzed with joint ultrasound and HJHS score correlation,results are shown the duration,joint bleeding times,alternative treatment,prevention treatment associated with haemophilic arthropathy(P<0.05),once again prompted our province hemophilia patients should be early,long course of prevention and cure for pre start.8.The ultrasound and HJHS score for the correlation of 264 joint analysis results showed:joint ultrasound scores were positively correlated with HJHS score(r=0.6697,p<0.0001),the joint ultrasound can effectively assess the haemophilia joint health,so I can be as regular ultrasound examination,joint of follow-up imaging tool.[Conclusions]In this experiment,through the analysis of the data,I draw the followingconclusions:Hemophilia A patients have low diagnosis rate,delay,inadequatetreatment,irregular,and joint disease development and disease duration,joint bleeding times,the alternative treatment plan and preventive treatment in our province,these problems lead to further joint damage our province hemophilia A patients were progressive,so according to the economic situation of Yunnan province is special,the author suggested that in our province can not carry out these small dose can promote early,long course of treatment of patients with hemophilia lack of small dose of prevention and treatment,to reduce the hemophilia patients to prevent the occurrence and development of joint bleeding,joint disease and improve the quality of life of patients. |
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