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，“慢性合并症，例如肥胖、心血管疾病和糖尿病。“由于成功的治疗，血友病患者的寿命更长，生活更有成效。然而，与结局和合并症及其管理相关的许多问题仍然存在。虽然血友病男性患者最初被认为可以预防心血管疾病，但现在很清楚，动脉粥样硬化血栓形成事件发生在这一人群中。然而，此类事件的患病率和促成风险因素尚未明确定义。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的出血风险。我们假设：1）血友病人群中存在CVD，但患病率低于美国普通人群。我们进一步假设：2）CVD风险因素与一般美国男性人群中发现的风险因素相似，但3）血友病患者不太可能接受适合年龄的CVD风险因素筛查，4）与抗血栓治疗相关的出血并发症风险增加。为了检验这些假设，我们计划利用附属血友病治疗中心（HTC）的美国血栓形成和止血网络（ATHN），并使用ATHN安全数据库进行数据输入，具体目的如下：1）确定200例在HTC随访的54-73岁中度或重度血友病A或B男性受试者的心血管疾病患病率; 2）将血友病受试者与ARIC数据库中同龄男性受试者的患病率数据和CVD危险因素进行比较; 3）收集200名男性中与抗血栓治疗相关的使用和不良事件数据;和4）确定年龄在35-54岁之间的血友病患者中接受过推荐的适合年龄的CVD危险因素筛查的比例。这些研究的结果将为血友病CVD及其预防和管理提供重要的新数据。这将改善该患者人群的临床护理，并为该人群未来的干预性研究和其他研究提供数据。

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.