【摘要】 目的 采用成人血友病患者生活质量问卷（Haemo-QoL-A）对接受救助的山东省低收入血友病患者生活质量进行评价。通过比较救助前后患者生活质量的变化，评估血友病精准帮扶项目的实施效果。方法 本研究共纳入2017年至2019年期间接受救助的94名低收入成年血友病患者，收集患者年龄、地区分布、血友病分型、抑制物情况。采用中文版Haemo-QoL-A问卷，分别于救助前、救助后1年和救助后5年对患者进行生活质量评估，并进行统计学分析。结果 在94例低收入成年血友病患者中，血友病A 80例（85.11%），血友病B 14例（14.89%）。患者主要为重型和中间型，分别为30例（31.91%）和62例（65.96%），轻型患者仅有2例（2.13%）。12例患者抑制物阳性，均为血友病A。94例患者救助前与救助后1年生活质量评分差异具有统计学意义（P<0.05）。78例患者完成了救助后5年的随访，救助前、后5年生活质量评分差异具有统计学意义（P<0.05）。结论 山东省低收入成年血友病患者生活质量较差，以药品救助和患者教育为核心的精准帮扶措施使患者的生活质量得到了一定的改善，提示血友病精准帮扶工作可以利用有限的资源来改善患者生活质量。更多还原

【Abstract】 Objective The quality of life of low-income patients with hemophilia receiving precise assistance was evaluated using the haemophilia-specific quality of life questionnaire for adults(HaemoQoL-A).By analyzing changes in patients’ quality of life before and after intervention,the effectiveness of the precise assistance project for hemophilia was assessed.Methods This study included 94 lowincome adult patients with hemophilia who received precise assistance between 2017 and 2019.Data on patients’ age,geographical distribution,hemophilia type and inhibitor status were collected.The Chinese version of the Haemo-QoL-A was used to assess quality of life at three time points:before,1 year after,and 5 years after assistance. Statistical analysis was performed on the collected data. Results Among the 94 low-income patients withhemophilia,80 patients(85.11%) had hemophilia A and 14 cases(14.89%) had hemophilia B.The patients were mainly severe and intermediate types,including 30(31.91%) severe and 62(65.96%) intermediate type patients,respectively,and only 2 cases(2.13%) were mild.There were 12 patients with inhibitors,all of whom had hemophilia A. There was a statistically significant difference in the quality of life scores of 94 patients before and 1 year after assistance(P<0.05).A total of 78 patients completed the 5-year follow-up after rescue.The difference in quality of life scores before and 5 years after rescue was statistically significant(P<0.05). Conclusions Although the quality of life was poor among low-income adult patients with hemophilia in Shandong Province,it improved through precise assistance that included medication assistance and patient education.These findings suggest that the hemophilia precise assistance program can effectively utilize limited resources to enhance patients’ quality of life.更多还原