| ObjectiveThis study retrospectively analyzed the clinical data and related quality of life data of 32 children with hemophilia A in Shenyang,in order to provide scientific basis for further comprehensive diagnosis and treatment of hemophilia.MethodsThe clinical data of 32 patients diagnosed with hemophilia A in Shenyang from2019 to 2021 were collected by means of hemophilia registration form,telephone follow-up,inquiry of inpatient medical records and electronic records of outpatient medical records.The clinical data of 32 patients were analyzed by SPSS25.0,P < 0.05 was statistically significant.Results（一）General information32 cases of hemophilia A were male,of which 26 cases were from urban areas and6 cases were from rural areas.3 cases were ≤ 3 years old,6 cases were 4 6 years old,14 cases were 7 10 years old and 9 cases were ≥ 11 years old.Light in 3 cases,medium in 8 cases and severe in 21 cases.There were 7 cases of type a blood,10 cases of type B blood,4 cases of type AB blood and 11 cases of type O blood.There were 5cases with definite family history,2 cases without family history and 25 cases with unknown situation.The parents of all patients did not carry out reproductive genetic counseling.（二）Clinical characteristics and data5 children were not screened for virus infection,27 children had no HIV,HBV and HCV infection.There was no inhibitor in 22 children except 10 patients who had not been tested.The age of first bleeding was concentrated in infants and young children.The degree of illness was negatively correlated with the age of first bleeding（r =-0.456,P < 0.05）.22 cases were spontaneous bleeding after operation.The common bleeding site is the joint,and the frequently affected joint is the knee joint.（三）Treatment informationThree children were treated on demand and 29 were treated with preventive treatment（11 cases of standard dose preventive treatment and 18 cases of medium dose;3 cases of primary preventive treatment,11 cases of secondary preventive treatment and 15 cases of tertiary preventive treatment）.（四）Assessment informationThe annual bleeding times,HJHS and US scores in the standard dose prevention and treatment group were lower than those in the medium dose prevention and treatment group,and the CHO-KLAT score was higher than that in the medium dose prevention and treatment group（P < 0.05）.secondary prevention treatment groups,the annual bleeding times,HJHS and US scores of the primary and secondary prevention and treatment groups were lower than those of the tertiary prevention treatment group,and the CHO-KLAT score was higher than that of the tertiary prevention treatment group.There were significant differences between secondary and tertiary preventive treatment（P < 0.05）.There was a negative correlation between CHO-KLAT score（quality of life）and US score（joint structure）and HJHS score（joint function）in 32 children with hemophilia A in Shenyang（r =-0.773,P < 0.05;r =-0.804,P < 0.05）.Conclusion1.There were 32 children with hemophilia A in Shenyang,most of them were urban residents,mainly school-age children,and the disease type was mainly severe.2.32 children with hemophilia A in Shenyang are mainly treated with prevention treatment.In terms of reducing bleeding times,protecting joint structure and function and improving quality of life,standard dose prevention treatment is better than medium dose prevention treatment,and secondary prevention treatment are better than tertiary prevention treatment.3.The quality of life of 32 patients with hemophilia A in Shenyang is affected by the state of joint function.The worse the state of joint function,the lower the quality of life. |
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