中、重度合并关节病变血友病患儿主观生活质量与疾病家庭负担评价简

目的了解合并关节病变的中、重度血友病患儿的主观生活质量与其家庭负担情况及其相关因素。方法研究对象包括符合世界血友病联盟（WFH）推荐诊断标准的42例血友病患儿（病例组）和62例对照者（对照组）。采用《血友病临床问卷（自编）》评估血友病患儿的临床情况；采用《少儿主观生活质量问卷》评估病例组与对照组的主观生活质量；采用《疾病家庭负担量表（FBS）》对91名病例组患儿家长及11名对照组儿童家长进行疾病家庭负担评估。通过t检验比较两组主观生活质量及疾病家庭负担的差异，通过Pearson相关分析及多元线性回归分析了解血友病儿童主观生活质量及其家庭负担的相关因素。结果病例组《主观生活质量问卷》的认知、情感、心理评分均明显低于对照组（P〈0．01）。病例组的主观生活质量与《血友病临床问卷（自编）》的年龄、出血频率、日常生活活动能力Bathel评分、平均失学天数、受累靶关节数、关节影像学Petterson评分、关节临床Gilbert评分相关不显著（P〉0．05）。病例组的疾病家庭负担高于对照组（23．87±10．90分VS5．36±3．10分，P〈0．01）。血友病患儿的疾病家庭负担与《血友病临床问卷（自编）》的靶关节数（r=1．8，P=0．011）及关节临床Gilbert评分（r=2．93，P=0．006）正相关，与平均失学天数（r=-2．75，P=0．007）及日常生活活动能力Bathel评分（r=-0．241，P=0．008）负相关，与患儿年龄、出血频率、关节影像学Petterson评分的相关不显著（P〉0．05）；而疾病严重程度、首诊年龄、受累关节数、日常生活活动能力Bathel评分、关节影像学Petterson评分归因效应显著（P〈0．05）。结论血友病患儿生活质量显著低于正常儿童，其家庭负担显著高于正常家庭，并与患儿的临床特征显著相关。

Assessment of the Quality of Life and the Family Burden of Diseases in Moderate or Severe Haemophilia Children with Joint Complications

Objective To assess the quality of life（QOL） and the family burden of diseases in moder- ate or severe haemophilia children with joint complications and correlative factors. Method The partition par- ticipants include 42 children with moderate or severe haemophilia children with joint complications （ case group） and 62 healthy control subjects （ control group）. The diagnoses were made according to the diagnostic criteria recommended by the world federation of hemophilia （WFH）. The patients were assessed with the Serf- administered Clinical Questionnaire for patients with haemophilia. Both case and control groups were evaluated with the Inventory of Subjective Life Quality（ISLQ）. 91 parents of case groups and 11 parents of control groups were evaluated with the Family Burden Scale（FBS）. The QOL and FBS scores were compared with the t tests or rand sum tests between case and control groups. The correlative factors were explored by using the analyses of correlation between ISLQ, FBS scores and the Self-administered Clinical Questionnaire. Results The scores of Cognitive, Affective and total scores of ISLQ in case group were lower than those in control group （P 〈 0. 01 ）. The QOL in case group was uncorrelated with the age,hemorrhage frequency, bathel score, miss-correlated with numbers of target joint （ r = 1.8,P = 0. 011 ） and Gilbert scores （ r = 0. 43, P = 0. 011 ）, nega- tively with missing days from school on average（ r = -2.75, P = 0. 007 ）and Bathel score （r = -0. 241, P = 0. 008）, but unrelated with the age, hemorrhage frequency, or petterson scores. Conclusion Children with haemophilia might suffer with obviously poor quality of life and higher family burden of disease compared with the normal children.