Haemophilia in Spain
Haemophilia in Spain
复制标题
DOI：
10.1111/j.1365-2516.2009.02001.x
复制
发表时间：
2009-05-01
期刊：
影响因子：
3.9
通讯作者：
Cortina, V. R.
中科院分区：
文献类型：
--
作者：
Aznar, J. A.;Lucia, F.;Cortina, V. R.
。确定血友病A和B及其并发症在西班牙的流行情况，并确定为血友病患者提供支持的卫生保健网络的特点。这项研究检查了临床和遗传特征，治疗选择，以及在疾病过程中观察到的并发症。横断面多中心研究。研究人群是2006年12月之前在西班牙任何一家医院积极随访的HA和HB患者。我们研究了2400例血友病患者，2081例(86.7%)HA和319例(13.3%)HB患者。32.3%的患者病情严重，16.4%的患者病情中等，51.3%的患者病情轻微。32.6%的患者进行了基因筛查。2006年实施的治疗包括60%的患者使用凝血因子浓缩物。截至2006年12月，45.8%的重症患者正在接受预防。2006年，未接受初级预防的患者平均出血次数为4次，接受初级预防的患者平均出血次数为1.3次。30%的患者至少有一个关节有血友病；16.8%的患者感染了艾滋病毒，34.8%的患者感染了丙型肝炎病毒。在10%的重度HA患者和6.5%的重度HB患者中检测到抑制剂。34例患者开始免疫耐受诱导治疗。这是西班牙首次对血友病的流行病学进行全面研究。这将使我们能够与邻近国家进行比较，评估为西班牙血友病患者提供的护理质量，并为公平提供和改善此类护理提供循证指导。
. To determine the prevalence of haemophilia A and B and their complications in Spain, and to characterize the health care network providing support to haemophiliac patients. The study examines clinical and genetic characteristics, treatment options, and complications observed during the course of the disease. Cross-sectional multi-centre study. The study population were patients with HA and HB in active follow-up at any Spanish hospital by December 2006. We studied 2400 haemophiliacs, 2081 (86.7%) HA and 319 (13.3%) HB patients. Illness was severe in 32.3% of patients, moderate in 16.4%, and mild in 51.3%. Genetic screening was carried out in 32.6% of the patients. Treatment administered in 2006 consisted of coagulation factor concentrates in 60% of patients. Until December 2006, 45.8% of severely ill patients were taking prophylaxis. The mean number of bleeding episodes in 2006 was four for patients not receiving primary prophylaxis and 1.3 for those taking primary prophylaxis. Thirty percent of patients had established haemophiliac arthropathy in at least one joint; 16.8% of patients were HIV-infected and 34.8% HCV-infected. Inhibitors were detected in 10% of severe HA patients and in 6.5% of severe HB patients. Immune tolerance induction therapy was started in 34 patients. This is the first comprehensive study on the epidemiology of haemophilia in Spain. It will enable us to draw comparisons with neighbouring countries, to assess the quality of care provided to haemophiliacs in Spain, and to provide evidence-based guidance for the even provision and improvement of such care.