Triemstra M, Rosendaal F R, Smit C, Van der Ploeg H M, Briët E
Vrije Universiteit, Amsterdam, The Netherlands.
Ann Intern Med. 1995 Dec 1;123(11):823-7. doi: 10.7326/0003-4819-123-11-199512010-00002.
To determine causes of death and mortality rates in patients with hemophilia over a period of 20 years, to assess changes in mortality, and to distinguish between hemophilia-related death and recent death induced by viral infections.
Cohort study of 919 patients followed from January 1986 to June 1992. Results were compared with outcomes of previous follow-up from 1973 to 1986.
Consecutive national questionnaire surveys on hemophilia, using patient registries of the Netherlands Hemophilia Society and Dutch hemophilia centers.
919 males hemophilia A or B who participated in a national questionnaire survey on hemophilia in 1985. Median duration of follow-up was 6.4 years, which yielded 5753 person-years of follow-up. The mean age at study entry was 30 years (range, 1 to 85 years).
Standardized mortality ratios, causes of death, median life expectancy, age-adjusted relative risks associated with the type or severity of hemophilia, presence of inhibitors, prophylaxis, and human immunodeficiency virus infection.
45 patients (5%) died between January 1986 and June 1992; 22.6 patients had been expected to die. Thus, the overall standardized mortality ratio was 2.0. The overall median life expectancy was 66 years for the cohort studied from 1973 to 1986 and 68 years for the cohort studied from 1986 to 1992. When deaths related to viral infection were excluded, the life expectancy almost equaled that of the general male population. Between 1986 and 1992, 1 patient died of ischemic heart disease compared with the 5.2 who were expected to die of this disease. Infection with HIV was the strongest independent predictor of death (relative risk, 27.5 [95% CI, 5.7 to 132.8]). After adjustment for HIV infection, no other hemophilia-related risk factors were associated with the risk of death.
The acquired immunodeficiency syndrome and hepatitis strongly influence mortality in patients with hemophilia. In the absence of viral infections, the life expectancy of patients with hemophilia would almost equal that of the general male population.
确定血友病患者20年间的死亡原因和死亡率，评估死亡率的变化，并区分血友病相关死亡和病毒感染所致近期死亡。
对919例患者进行队列研究，随访时间从1986年1月至1992年6月。将结果与1973年至1986年的既往随访结果进行比较。
利用荷兰血友病协会和荷兰血友病中心的患者登记系统，对血友病患者进行连续的全国问卷调查。
919例甲型或乙型血友病男性患者，于1985年参与了一项血友病全国问卷调查。中位随访时间为6.4年，共产生5753人年的随访量。研究开始时的平均年龄为30岁（范围1至85岁）。
标准化死亡比、死亡原因、中位预期寿命、与血友病类型或严重程度、抑制剂的存在、预防措施以及人类免疫缺陷病毒感染相关的年龄调整相对风险。
1986年1月至1992年6月期间，45例患者（5%）死亡；预期死亡人数为22.6例。因此，总体标准化死亡比为2.0。1973年至1986年研究队列的总体中位预期寿命为66岁，1986年至1992年研究队列的为68岁。排除与病毒感染相关的死亡后，预期寿命几乎与普通男性人群相等。1986年至1992年期间，1例患者死于缺血性心脏病，而预期死于该病的人数为5.2例。感染HIV是最强的独立死亡预测因素（相对风险，27.5 [95% CI，5.7至132.8]）。在对HIV感染进行校正后，没有其他血友病相关危险因素与死亡风险相关。
获得性免疫缺陷综合征和肝炎对血友病患者的死亡率有强烈影响。在没有病毒感染的情况下，血友病患者的预期寿命几乎与普通男性人群相等。
