| ObjectiveWe reviewed analysis the clinical manifestation,treatment of 104 cases with moderate and severe haemophilia A to provide some clinical datas in diagnosis and treatment for children haemophilia A in Guangxi province.MethodWe reviewed analysis the clinical datas of 104 children who were diagnosed with haemophilia A in the First Affiliated Hospital of Guangxi Medical University from January 2011 to October 2016.The datas were analyzed with chi-square test,Kruskal-Wallis Test and t-test according to specific consideration.(The significance level was set at P<0.05).Results1.104 children with haemophilia A were enrolled in ourinvestgation,which were divided into moderate(60/104,57.7%)and severe group(44/104,42.3%).The positive rate of family inheritance histories for these cases was 15.4%,moderate and severe group were11.7%,20.5% respectively.There was no significant difference between the two groups(P = 0.207).2.The mean age at first bleeding were 2.86 ± 2.62 years old and1.56± 1.60 years old,the average age of first joint bleeding were 5.44 ± 2.34 years old and 4.48 ± 2.70 years old for moderate group and severe group.Among them,the primary bleeding group was mainly caused by post-traumatic hemorrhage(68.3%)in the moderate group and spontaneous hemorrhage(68.2%)in the severe group.There was significant difference between the two groups(P = 0.000).There were 14 cases of intracranial hemorrhage at the first bleeding site,the incidence was 13.5%,the mortality rate was 0%,moderate and severe group each have 7 cases,the incidence was 11.7%,15.9% respectively.3.Bleeding during the follow-up:(1)Hemorrhage site: 104 cases of hemophilia A children with bleeding parts include the skin and superficial mucosa,joint cavity,viscera and even intracranial and so on.There were 84 cases with skin and superficial mucosal hemorrhage,the incidence was 80.77%,33 cases with intra-articular bleeding,the incidence was 31.73%,the internal bleeding were 5 cases,the incidence was 4.8%;one case with intracranialhemorrhage,the incidence was 0.96%.(2)The level of bleeding: in this study,the most frequent sources of clinical bleeding level in the moderate group was mildly(39 cases,65.0%),followed by the moderate(13 cases,21.7%),extremely severe(8cases,13.3%),the most frequent sources of clinical bleeding level in the severe group was also mild(27 cases,61.4%),followed by the moderate and extremely severe(8 cases,18.2%),severe(1 case,2.2%).There was no significant difference in clinical bleeding level between the two groups(P = 0.143).(3)Bleeding frequency: among the 104 cases,There were 18 and13 cases in moderate and severe group with moderate or severe bleeding who without prophylaxis,and the incidence rate was 30%,29.5%;the average annual bleeding frequency were(9.5 ± 13.09)times,(12.13 ±13.66)times.There was no significant difference between the two groups(P = 0.514).(4)Target joint : In this study,104 cases children with haemophilia A,the target joint formation were 27 cases,the incidence was 26.0%,of which 12 cases in moderate group,15 cases in severe group;14 cases with joint deformity,moderate and severe group each had 7 cases,the incidence was 11.7%,15.9%.The difference between the two groups was statistically significant(P = 0.000).The average age of joint deformity were 9.5 years,mainly in school-age.The incidence of joint deformitywas 41% and 32% in the ankle joint and knee joint,the next was elbow joint,18%,shoulder joint and wrist deformity accounting for 5% and 4%.5.Replacement therapy: in the 104 cases children with hemophilia A,66 cases received replacement therapy,of which 55 cases with on-demand treatment,11 cases with prophylactic treatment.During the follow-up,the incidence of joint deformity was 21.8%,and there was no joint deformity in the prophylactic treatment group.There was significant difference between the two groups(P=0.02).7 cases with the prophylactic treatment after they suffer joint bleeding,the frequence of joint bleeding before and after the prevention treatment were(3.3 ± 1.6)times / month,(1.2 ± 1.3)times / month.6.Average annual F? usage: the mean annual use of F? factor was(0.77±10.6)thousands in moderate group and(1.84±1.89)thousands in severe group;the mean annual use of F? factor units(IU)were(92.6±78.1)IU / kg in moderate group,(326.1±123.7)IU / kg in severe group.There was significantly difference between the two groups(P =0.000).Conclusion:(1)The main cause of joint deformity is intra-articular bleeding,mainly located in the ankle,knee and other weight-bearing joints;(2)There was a certainly degree of clinical heterogeneity in haemophilia A,F ? : C could not accurately predict the frequency of bleeding and the level of bleeding,moderate hemophilia A maight alsooccur very severe bleeding;(3)Prophylactic therapy could reduce the frequency of joint deformity and morbidity;(4)In the study,the average annual F? dosage and the prevention treatment rate were lower,and the prophylactic treatment should be intensified of popularization prevention and treatment,and improve the life quality of children with haemophilia A. |
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