| Objective:To explore the correlation between joint bleeding and joint injury in children with hemophilia A could understand the bleeding,joint structure and joint function of children with hemophilia A in Guizhou province,timely adjust the treatment plan,reduce the joint damage and disability rate,and improve the quality of life of patients.Methods:Patients aged 3–18 years with hemophilia A and registered in the Guizhou Province emophilia diagnosis and treatment center were recruited from January 2021 to March 2021.The Hemophilic Early Arthropathy Detection with Ultra Sound in China（HEAD-US-C）,Hemophilia Joint Health Score（HJHS）were done in all patients and were performed on the elbows,knees and ankles.The data were collected on age,diagnosis,factor VIII activity,classification of extent of disease,duration of treatment,mode of treatment,and treatment regimen.The data were statistically analyzed by SPSS version 26.0,and Kolmogorov-Smirnov test was used to test data for normality of distribution.If the measurement data conform to the normal distribution,it shall be described by means±standard deviation,and if not,it shall be described by M(P25,P75).The counting data is described by the number of cases and percentage,and the comparison of the positive rates was performed by chi-square test.Row t-test or F-test were used to normal distribution of contrast between data,and rank sum test was used to non-normality of distribution.Correlations used Pearson correlation analysis,P<0.05 was statistically significant.Results:1)Basics characteristics:Eighty-nine patients with 534 examined joints were included,all of whom were male,with a median age of 9 years（range,3-18 years）.In this study,two patients are mild hemophilia A,six patients are intermediate hemophilia A,and eighty-one patients are severe hemophilia A.Duration of treatment ranged from 1-15years（median 7.0 years）.Fifteen received on-demand treatment,and twenty received prophylactic treatment,and fifty-four transferred on-demand to preventive treatment.2)Joint bleeding:The annual joint bleeding rate（AJBR）in recent 3 years is 0-20times,with a median of 4 times;The positive rate of joint hemorrhage in children with hemophilia A in the 3-6-year-old group was lower than that in the 7-8-year-old group（P<0.05）;The positive rate of joint hemorrhage in the preventive treatment group was lower than that in the on-demand treatment group and the on-demand-transferred-preventive treatment group（P<0.05）;In the preventive treatment and on-demand treatment groups,age and treatment time were correlated with AJBR;3)Joint injury:534 joints were assessed in 89 patients with HEAD-US-C scale.The median total score was 11 points（0-59 points）,and the right ankle was the most affected in joint diseases.The total joint detection rate was 26.22%.The detection rate of joint lesions in children with hemophilia A in the 3-6-year-old group was significantly lower than that in the 7-18-year-old group（P<0.05）;The detection rate of joint lesions in hemophilia A children in the on-demand-transferred-preventive treatment group was lower than that in the on-demand treatment group（P<0.05）;However,there was no significant difference in the detection rate of joint lesions between the preventive treatment group and the on-demand treatment group（P>0.05）;The mean value of the HEAD-US-C total score in the preventive treatment group was significantly lower than that in the on-demand treatment group and the on-demand-transferred-prevention group,and the difference between the preventive treatment group and the on-demand-transferred-prevention group was statistically significant（P<0.05）;The detection rate of articular cartilage disease and bone disease in the on-demand treatment group was significantly higher than that in the preventive treatment group（P<0.05）;72patients were assessed for joint function,namely HJHS scale,with a total score of 0-50 and a median of 6;The HJHS score of 15 children was 0,and that of 57 children was greater than 0;The positive rate of total HJHS score>0 in the 3-6-year-old group was significantly lower than that in the 7-18-year-old group（P<0.05）;The positive rate of HJHS total score of hemophilia A children in the preventive treatment group was significantly lower than that in the on-demand treatment group and the on-demand-transferred-prevention group,and the difference was statistically significant（P<0.05）;The mean value of the total HJHS score of the preventive treatment group was significantly lower than that of the on-demand treatment group and the on-demand-transferred-prevention group（P<0.05）;HEAD-US-C scale is correlated with HJHS scale（r=0.705,P<0.001）;4)Relationship between joint hemorrhage and joint injury:AJBR was correlated with the score of HEAD-US-C scale（r=0.548,P<0.001）;AJBR was correlated with HJHS scale scores（r=0.553,P<0.001）。Conclusions:1)Age and treatment duration at the time of the study were associated with AJBR,and the number of joint hemorrhages increased with advancing age and delayed treatment duration.2)Preventive treatment can effectively reduce joint bleeding in children with hemophilia A,so as to reduce joint structure and function damage;On-demand treatment leads to more severe joint lesions in children with hemophilia A.3)The HEAD-US-C and HJHS scores,which evaluated joint structure and function in hemophilia patients respectively,had good consistency and were served as regular monitoring items in the follow-up examination of hemophilia patients,which could help physicians keep abreast of the patients’joints,adjust the treatment plan,and improve the quality of patients’lives.4)AJBR was associated with joint function and structure,and the higher AJBR,the more severe the structural damage to the joint,and the worse the joint function. |
