| [Objective]In this study,the clinical history and treatment of 70 hemophilia patients in Yunnan Province were collected and analyzed,combined with joint ultrasound examination,joint health status and quality of life questionnaire score.The purpose of this study was to evaluate the degree of joint disease and the living condition of the patients,combined with the HJHS score,in order to understand the condition of joint hemorrhage,the degree of joint injury,and analyze the joint status of hemophilia patients under different doses of on-demand treatment.Based on the analysis and statistical results,the characteristics of lower joint injury in Yunnan hemophilia patients treated on demand were evaluated to provide basic reference data for clinical prevention and treatment of hemophilia arthropathy,so as to standardize the treatment so as to facilitate the treatment of hemophilia in Yunnan Province.Improve the joint status of hemophilia patients in Yunnan Province,reduce the rate of joint deformity,improve the overall quality of life.[Methods]70 cases of hemophilia diagnosed in the first affiliated Hospital of Kunming Medical University from September 2017 to December 2018 and registered in the Department of Hematology of the first affiliated Hospital of Kunming Medical University,which are mainly treated on demand,are investigated by questionnaire,Telephone tracking,out-patient and in-patient follow-up were used to collect data.At the same time,ultrasonic examination of knee joint,elbow joint and ankle joint was carried out.HEAD-US was used to evaluate the joint.The joint health status of hemophilia patients was evaluated by HJHS2.1 scale,and the quality of life of included hemophilia patients was analyzed,and the health status was used to evaluate the quality of life of hemophilia patients.Questionnaire(the MOS item short form-36 health survey,SF-36),the clinical information data collected by the questionnaire,the results of ultrasonic examination and the score of joint health status were statistically analyzed by SPSS20.0 software.The joint injuries and characteristics of hemophilia patients treated with different doses on demand in Yunnan Province were discussed and obtained.[Results]1.Comparison of HJHS scores of joints treated with different doses on demand:of the 70 patients collected,29 cases were treated with foot dose on demand(42 joints involved in total),19 cases were treated on demand with medium dose(77 joints total involved).There were 22 cases treated with low dose on demand(total number of involved joints was 107).First,Pearsonka test was used to compare the correlation between the HJHS scores of hemophilia joints with different treatment doses.The results showed that P<0.05,P<0.05.The difference was statistically significant,that is,there was a correlation between dose and arthropathy.Re-use 2 independent samples The t-test was used to compare the HJHS scores of different dosage on-demand treatment.Compared with the middle-dose treatment group,the low-dose treatment group had P<0.05,the difference was statistically significant,even if the middle-dose on-demand treatment had less joint injury than the low-dose treatment group.P<0.05,the difference was statistically significant,that is,the effect of foot on-demand treatment was less than that of moderate-dose on-demand treatment on arthropathy.3.The results showed that there was no significant difference between the middle-dose group and the middle-dose group(P<0.05).2.Comparison of HJHS scores between insufficient low-dose prophylaxis and foot-dose on-demand treatment:in addition,7 cases of hemophilia A treated with insufficient low-dose prophylaxis were collected,and two independent samples were used for t-test.There was a statistical difference between insufficient low-dose prophylaxis and foot-dose on-demand treatment.The results showed that there was a significant difference between the two groups(P<0.05),and there was no significant difference between the two groups(P<0.05).That is,insufficient low-dose prevention and treatment of joint injury is less than foot-dose on-demand treatment of joint injury.3.The relationship between ethnic group,region and HJHS score:there are 56 ethnic Han,6 Bai nationality,4 Yi nationality,2 Zhuang nationality and Dai nationality among the 70 patients in this study,in which there are 56 Han nationality,6 Bai nationality,4 Yi nationality,2 Zhuang nationality and Bai nationality.One case from each Hui nationality(see figure 2 for specific ethnic composition),14 of them lived in Kunming,while the remaining 56 lived in other cities in Yunnan Province(see figure 1 for specific distribution).The ethnic groups were compared by using 2 independent sample t-tests,and the other 56 lived in other cities of Yunnan Province(see fig.1 for specific distribution).The relationship between region and HJHS score,the results showed that there was no significant difference between nationality and region and hemophilia,P value>0.05,P=0.463,P=0.960,P>0.05,P>0.05.The joint HJHS score was not significant.4.Relationship between hemophilia type(type A or B)and nationality,age,HJHS score:59 cases of hemophilia A and 11 cases of hemophilia B were compared between hemophilia type A and nationality,age and age by using 2 independent samples t test.HJHS scores were correlated with each other.The results showed that there was no significant correlation between hemophilia type and nationality and age,P<0.05,P>0.05,P<0.01,P>0.05,P>0.05.There was no significant correlation between hemophilia type and degree of joint lesion.5.Joint damage in different age groups:70 patients were divided into different age groups(<10 years,10 years old,20 years old,29 years old,30 years 39 years old,40 years old).The rate of joint involvement in different age groups were 16.67%,respectively.37.5%,50%,88.33%,80.76%,respectively.Using Chi-square test to compare the average rate of joint involvement in patients of all ages,the results showed that P<0.05,the difference was statistically significant,that is,under on-demand treatment,the joint involvement rate was significantly higher than that of the control group(P<0.05).There is a correlation between age growth and joint involvement,and the trend of change is shown in Fig.4.6.Correlation between 6 joints HJHS scores and HEAD-US score,SF-36 score:HJHS score was positively correlated with HEAD-US score and Pearson score of SF-36 evaluation branch.The results showed that HJHS score was positively correlated with HEAD-US score and SF-36 score.P<0.0001,suggesting that the three methods should be combined in evaluating hemophilia patients' joint system.7.Treatment of type B hemophilia:of the 11 cases of type B hemophilia,only 1 patient was treated with foot dose on demand,5 cases with moderate dose and 5 cases with low dose,accounting for 9.1%of full dose,while among 59 cases with hemophilia A,28 cases(47.5%)were treated with full dose therapy,which was significantly higher than that of type B hemophilia.The results showed that the treatment of type B hemophilia was more irregular than that of type A hemophilia,and the treatment rate and effective rate were lower than those of type B hemophilia.8.In patients with moderate dose of on-demand treatment,changing the treatment dose to full dose also improved the HJHS score of the target joint of the patients,suggesting that hemophilia patients,especially those with arthropathy in our province,Early start full-dose or low-dose prophylaxis can delay the progress of hemophilia arthropathy and improve the quality of life of patients.[conclusions]Based on the analysis of joint involvement in hemophilia patients treated with different doses on demand,the following conclusions are drawn:most of the hemophilia patients in our province choose the treatment mode on demand,and the joint condition is not optimistic,and the joint condition of hemophilia patients in our province is not optimistic.In particular,adult joint involvement rate is higher.Before the establishment of hemophilia diagnosis and treatment center in our province,most of the medical staff and patients had low knowledge of hemophilia and the joint treatment was not in place.At present,there are inadequate treatment,irregular,irregular conditions.The occurrence and development of hemophilia arthropathy in the 20-year-old age group is faster than that in the 0-20 years old age group.The reason is that the joint load increases with the increase of activity and body weight,resulting in the joint bearing weight.The damage was increased.There was no significant difference in joint injury between type B hemophilia and type A hemophilia,but there was no significant correlation between joint involvement rate and nationality and region in Yunnan province where ethnic minorities were widely distributed.In view of the relatively backward economic situation in Yunnan Province,early full dose therapy can be advocated in these groups of hemophilia patients who are unable to carry out preventive treatment in Yunnan Province,especially for children under the age of 10,Foot dose on demand can delay the occurrence and development of hemophilia joint disease and improve the overall quality of life of the patients.Full-dose on-demand treatment under current conditions of on-demand treatment It can reduce the rate of joint injury,and it is worth carrying out in Yunnan province,but the prevention and treatment of small dose is less than that of treating joint injury on demand,so it is still necessary to advocate prevention and treatment.Therefore,if the economy does not permit,preventive treatment can first choose insufficient low-dose preventive treatment. |
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