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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).