Wang T, Zhang L, Li H, Zhao H, Yang R
State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, 288 Nanjing Road, Tianjin 300020, China.
Haemophilia. 2004 Jul;10(4):370-5. doi: 10.1111/j.1365-2516.2004.00917.x.
Haemophilia is a group of inherited, congenital diseases present all through the life. Those disorders are associated with a chronic burden of morbidity punctuated by episodes of acute deterioration in health-related quality-of-life (HR-QL). The significances of assessing HR-QL in individuals with haemophilia as an important component of the clinical database have gained increasing recognition in recent years. To the best of our knowledge, reports on the HR-QL of Chinese patients with haemophilia are not available. The purpose of this paper was to assess the HR-QL of patients with haemophilia in China. In order to do this, 179 patients with haemophilia (age 16-70) were mailed China quality-of-life questionnaire, which was designed specially for this study taking into account the culture characters of Chinese. Compared with the normal male general population, individuals with haemophilia experienced significantly lower levels of HR-QL and within the subgroups of patients, there was a gradation of HR-QL from mild < moderate < severe haemophilia patients. Age, clinical severity, degree of arthropathy, hepatitis status, salary and mean annual expenditure for replacement therapy were predictors of HR-QL. Clinical severity of the disease manifestation rather than the severity of the disease based on molecular classification was a factor that negatively influenced the HR-QL of patients with haemophilia. Arthropathies of patients with haemophilia in China were more severe than that in western countries because of the poorer medical care and were associated with their lower income and therapy cost. A large number of patients never had hepatitis or HIV serological examination. The exact prevalence of hepatitis and HIV was difficult to obtain and the HR-QL of hepatitis and HIV positive patients required further study.
血友病是一组遗传性先天性疾病，伴随终生。这些疾病与慢性发病负担相关，健康相关生活质量（HR-QL）会不时出现急性恶化。近年来，将评估血友病患者的HR-QL作为临床数据库的重要组成部分的重要性已得到越来越多的认可。据我们所知，目前尚无关于中国血友病患者HR-QL的报道。本文旨在评估中国血友病患者的HR-QL。为此，向179名年龄在16至70岁之间的血友病患者邮寄了中国生活质量问卷，该问卷是考虑到中国人的文化特点专门为本研究设计的。与正常男性总体人群相比，血友病患者的HR-QL水平明显较低，在患者亚组中，HR-QL从轻度<中度<重度血友病患者呈梯度变化。年龄、临床严重程度、关节病程度、肝炎状况、工资和替代治疗的年均支出是HR-QL的预测因素。疾病表现的临床严重程度而非基于分子分类的疾病严重程度是对血友病患者HR-QL产生负面影响的一个因素。由于医疗条件较差，中国血友病患者的关节病比西方国家更为严重，且与他们较低的收入和治疗费用有关。大量患者从未进行过肝炎或HIV血清学检查。肝炎和HIV的确切患病率难以获得，肝炎和HIV阳性患者的HR-QL需要进一步研究。
