Tusell J M, Aznar J A, Querol F, Quintana M, Moreno M, Gorina E
University Hospital Vall d'Hebron, Barcelona, Spain.
Haemophilia. 2002 Mar;8 Suppl 2:38-42. doi: 10.1046/j.1351-8216.2001.00119.x.
This paper outlines the results obtained in a cross-sectional study of a group of young patients with severe haemophilia A and B. The primary aim of the study was to ascertain the level of orthopaedic complications in the group, the effects that these complications have on quality of life, and the medical resources used on these patients. The secondary aim was to relate their current orthopaedic state to the type of treatment received before the study. The study was carried out in 11 hospitals in Spain, where 70 severe haemophilia patients (factor VIII [FVIII] < 2%), with an average age of 21.6 and a median age of 22, and no inhibitors, were monitored. The percentage of patients suffering from articular complaints was 84.3% and 85.7% according to the Gilbert and Petterson scales, respectively. Furthermore, pain was reported in 16.1% of joints, most frequently in ankles. Using the SF-36 Health Survey, patients were observed to have a poorer quality of life relative to healthy controls. Despite high levels of both the coagulant factor given to patients and the financial resources dedicated to their treatment, the type of treatment (on demand and/or as secondary prophylaxis) provided to the young adults was found to be incapable of preventing haemophilic arthropathy and subsequent negative quality of life consequences.
本文概述了对一组重度甲型和乙型血友病年轻患者进行横断面研究所得的结果。该研究的主要目的是确定该组患者的骨科并发症水平、这些并发症对生活质量的影响以及用于这些患者的医疗资源。次要目的是将他们当前的骨科状况与研究前接受的治疗类型联系起来。该研究在西班牙的11家医院开展,对70名重度血友病患者(凝血因子VIII[FVIII]<2%)进行了监测,这些患者的平均年龄为21.6岁,中位年龄为22岁,且无抑制物。根据吉尔伯特(Gilbert)和彼得森(Petterson)量表,分别有84.3%和85.7%的患者存在关节问题。此外,16.1%的关节出现疼痛,最常见于脚踝。使用SF-36健康调查发现,与健康对照组相比,这些患者的生活质量较差。尽管给予患者的凝血因子水平较高,且用于其治疗的财政资源充足,但发现给予这些年轻人的治疗类型(按需治疗和/或二级预防)无法预防血友病性关节病及随后的负面生活质量后果。