Prevalent inhibitors in haemophilia B subjects enrolled in the Universal Data Collection database.
Prevalent inhibitors in haemophilia B subjects enrolled in the Universal Data Collection database.
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DOI：
10.1111/hae.12229
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发表时间：
2014-01
期刊：
影响因子：
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通讯作者：
Hemophilia Treatment Center Network (HTCN) Investigators
中科院分区：
文献类型：
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作者：
Puetz J;Soucie JM;Kempton CL;Monahan PE;Hemophilia Treatment Center Network (HTCN) Investigators
最近已经描述了几种抑制剂对血友病A的危险因素，有人认为类似的危险因素也与血友病B有关，但有有限的数据来证实这一概念。为了确定血友病B中抑制剂的患病率和与其相关的危险因素，查询了美国疾病控制中心1998年至2011年的通用数据收集(UDC)项目的数据库，以确定血友病B受试者中抑制剂的患病率。此外，还评估了疾病严重程度、种族/民族、年龄、因子暴露和预防性使用，以确定它们对抑制剂流行率的影响。在UDC数据库登记的3800名患有血友病B的男性受试者中，75人(2%)被确定在研究期间的某个时间点有抑制物。发现严重疾病(OR 13.1，95%CI 6.2~27.7)、黑人(OR 2.2，95%CI 1.2~4.1)和年龄小于11岁(OR 2.5，95%CI 1.5~4.0)与使用抑制剂显著相关。没有足够的数据来确定所使用的因子类型和预防措施是否与抑制剂有关。血友病B中的抑制剂比血友病A中的普遍程度要低得多，特别是在轻度疾病的患者中。与血友病A中的抑制剂有关的类似因素似乎也存在于血友病B中。这一大型监测项目收集的信息不允许评估与治疗方法和暴露有关的潜在风险因素，还需要进行额外的研究。
Several risk factors for inhibitors have recently been described for hemophilia A. It has been assumed that similar risk factors are also relevant for hemophilia B, but there is limited data to confirm this notion. To determine the prevalence of and risk factors associated with inhibitors in hemophilia B The database of the Universal Data Collection (UDC) project of the Centers for Disease Control for the years 1998 – 2011 was queried to determine the prevalence of inhibitors in hemophilia B subjects. In addition, disease severity, race/ethnicity, age, factor exposure, and prophylaxis usage were evaluated to determine their impact on inhibitor prevalence. Of the 3800 male subjects with hemophilia B enrolled in the UDC database, 75 (2%) were determined to have an inhibitor at some point during the study period. Severe disease (OR 13.1, 95% CI 6.2-27.7), black race (OR 2.2, 95% CI 1.2-4.1), and age less than 11 (OR 2.5, 95% CI 1.5-4.0) were found to be significantly associated with having an inhibitor. There was insufficient data to determine if type of factor used and prophylaxis were associated with inhibitors. Inhibitors in hemophilia B are much less prevalent than hemophilia A, especially in patients with mild disease. Similar factors associated with inhibitors in hemophilia A also seem to be present for hemophilia B. The information collected by this large surveillance project did not permit evaluation of potential risk factors related to treatment approaches and exposures, and additional studies will be required.