A Cross-Sectional Analysis of Cardiovascular Disease in the Hemophilia Population

描述(由申请人提供)：本申请重点关注RFA的优先领域5，“慢性并存疾病，如肥胖、心血管疾病和糖尿病。”由于治疗的成功，患有血友病的男性活得更长，更有成效。然而，与结果和合并症及其管理有关的许多问题仍然存在。虽然血友病男性最初被认为可以预防心血管疾病，但现在很明显，动脉粥样硬化血栓形成事件发生在这一人群中。然而，此类事件的流行程度和造成风险的因素并未得到明确界定。心血管疾病(CVD)的主要治疗方法是抗血栓治疗，这会增加血友病患者出血的风险。我们假设：1)CVD在血友病人群中存在，但没有在美国普通人群中发现的那么普遍。我们进一步假设，2)心血管危险因素与普通美国男性人群相似，但3)血友病患者不太可能接受与年龄相适应的心血管危险因素筛查，4)与抗血栓治疗相关的出血并发症的风险增加。为了验证这些假设，我们计划利用美国血友病治疗中心(HTCS)的血栓和止血网络(ATHN)，并使用ATHN SECURE数据库输入以下特定目标的数据：1)确定200名54-73岁的中重度血友病A或B男性受试者心血管疾病的患病率；2)比较血友病受试者与ARIC数据库中相同年龄的男性受试者的患病率和心血管疾病危险因素；3)收集200名男性中与抗血栓治疗相关的使用和不良事件的数据；4)确定35~54岁血友病患者接受过建议年龄合适的心血管危险因素筛查的比例。这些研究的结果将为血友病的心血管疾病及其预防和治疗提供重要的新数据。这将改善这一患者群体的临床护理，并为该人群未来的干预性和其他研究提供数据基础。

DESCRIPTION (provided by applicant): This application focuses on Priority Area 5 of the RFA,"Chronic co-morbid conditions, such as obesity, cardiovascular disease, and diabetes." Due to successful treatment, men with hemophilia are living longer, more productive lives. However, many questions related to outcomes and co-morbidities and their management remain. While men with hemophilia were initially thought to be protected from cardiovascular disease, it is now clear that atherothrombotic events occur in this population. However, the prevalence of such events and the contributing risk factors are not clearly defined. The mainstay of treatment of cardiovascular disease (CVD) is antithrombotic therapy, which carries increased risks of bleeding in patients with hemophilia. We hypothesize that 1) CVD is present in the hemophilia population but less prevalent than found in the general U.S. population. We further hypothesize that 2) CVD risk factors are similar to those found in the general U.S. male population, but 3) patients with hemophilia are less likely to receive age- appropriate screening for CVD risk factors and 4) are at increased risk of bleeding complications related to antithrombotic treatment. To test these hypotheses we plan to utilize the American Thrombosis and Hemostasis Network (ATHN) of affiliate hemophilia treatment centers (HTCs) and data entry using the ATHN secure database in the following Specific Aims: 1) To determine the prevalence of cardiovascular disease in 200 male subjects with moderate or severe hemophilia A or B aged 54-73 years followed at HTCs; 2) To compare prevalence data and CVD risk factors in subjects with hemophilia with male subjects in the ARIC database of the same age; 3) To collect data on use and adverse events related to antithrombotic therapy in the 200 men; and 4) To determine the proportion of patients with hemophilia ages 35-54 years of age who have had recommended age appropriate screening for CVD risk factors. The results of these studies will provide important new data on CVD in hemophilia and its prevention and management. This should improve clinical care for this patient population as well as provide data on which to base future interventional and other studies in this population.