Soucie J M, Evatt B, Jackson D
National Center for Infectious Diseases, Hematologic Diseases Branch, Centers for Disease Control and Prevention, Atlanta, Georgia 30333, USA.
Am J Hematol. 1998 Dec;59(4):288-94. doi: 10.1002/(sici)1096-8652(199812)59:4<288::aid-ajh4>3.0.co;2-i.
An active surveillance system was used to identify all residents with hemophilia in six U.S. states (Colorado, Georgia, Louisiana, Massachusetts, New York, and Oklahoma). A hemophilia case was defined as a person with physician-diagnosed hemophilia A or B and/or a measured baseline factor VIII or IX activity (FA) of 30% or less. Case-finding methods included patient reports from physicians, clinical laboratories, hospitals, and hemophilia treatment centers. Once identified, trained data abstractors collected clinical and outcome data retrospectively from medical records. Among cases identified in 1993-1995, 2,743 were residents of the six states in 1994, of whom 2,156 (79%) had hemophilia A. Of those with available FA measurements, 1,140 (43%) had severe (FA < 1%), 684 (26%) had moderate (FA 1%-5%), and 848 (31%) had mild (FA 6%-30%) disease. The mean and median age was 25.4 and 23 years, respectively. The age-adjusted prevalence of hemophilia in all six states in 1994 was 13.4 cases/100,000 males (10.5 for hemophilia A and 2.9 for B). The prevalence by race/ethnicity was 13.2 cases/100,000 among white, 11.0 among African American, and 11.5 among Hispanic males. Application of age-specific prevalence rates from the six surveillance states to the U.S. population resulted in an estimated national population of 13,320 cases of hemophilia A and 3,640 cases of hemophilia B. For the 10-year period 1982-1991, the average incidence of hemophilia A and B in the hemophilia surveillance system (HSS) states was estimated to be 1 in 5,032 live male births.
采用主动监测系统识别美国六个州（科罗拉多州、佐治亚州、路易斯安那州、马萨诸塞州、纽约州和俄克拉何马州）所有血友病患者。血友病病例定义为经医生诊断为甲型或乙型血友病的人，和/或测得的基线凝血因子 VIII 或 IX 活性（FA）为 30% 或更低。病例发现方法包括医生、临床实验室、医院和血友病治疗中心提供的患者报告。一旦确定，经过培训的数据提取人员会从医疗记录中回顾性收集临床和结局数据。在 1993 - 1995 年确定的病例中，1994 年有 2743 例是这六个州的居民，其中 2156 例（79%）为甲型血友病。在有 FA 测量值的患者中，1140 例（43%）为重度（FA < 1%），684 例（26%）为中度（FA 1% - 5%），848 例（31%）为轻度（FA 6% - 30%）疾病。平均年龄和中位年龄分别为 25.4 岁和 23 岁。1994 年所有六个州经年龄调整后的血友病患病率为每 10 万男性中有 13.4 例（甲型血友病为 10.5 例，乙型血友病为 2.9 例）。按种族/族裔划分的患病率为：白人每 10 万中有 13.2 例，非裔美国人每 10 万中有 11.0 例，西班牙裔男性每 10 万中有 11.5 例。将六个监测州的年龄特异性患病率应用于美国人口，得出全国估计有 13320 例甲型血友病病例和 3640 例乙型血友病病例。在 1982 - 1991 年的 10 年期间，血友病监测系统（HSS）各州甲型和乙型血友病的平均发病率估计为每 5032 例活产男婴中有 1 例。
