       Document 0041
 DOCN  M9620041
 TI    Mortality in patients with hemophilia. Changes in a Dutch population
       from 1986 to 1992 and 1973 to 1986.
 DT    9602
 AU    Triemstra M; Rosendaal FR; Smit C; Van der Ploeg HM; Briet E; Vrije
       Universiteit, Amsterdam, The Netherlands.
 SO    Ann Intern Med. 1995 Dec 1;123(11):823-7. Unique Identifier : AIDSLINE
       MED/96061860
 AB    OBJECTIVE: 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. DESIGN: 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. SETTING:
       Consecutive national questionnaire surveys on hemophilia, using patient
       registries of the Netherlands Hemophilia Society and Dutch hemophilia
       centers. PATIENTS: 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).
       MEASUREMENTS: 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. RESULTS: 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. CONCLUSIONS: 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.
 DE    Adult  Aged  Cause of Death  Cohort Studies
       Hemophilia/COMPLICATIONS/*MORTALITY  Human  HIV
       Infections/COMPLICATIONS/MORTALITY  Life Expectancy  Male  Middle Age
       Netherlands/EPIDEMIOLOGY  Questionnaires  Retrospective Studies  Risk
       Factors  Support, Non-U.S. Gov't  JOURNAL ARTICLE

       SOURCE: National Library of Medicine.  NOTICE: This material may be
       protected by Copyright Law (Title 17, U.S.Code).

