CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Mortality among males with hemophilia: relations with source of medical care

Although persons with hemophilia are known to be at increased risk of death, no studies have examined the source of medical care and other personal charac-teristics for associations with mortality. To determine death rates and to identify causes of death and predictors of mortal-ity, we studied a cohort comprised of all hemophilic males identified by a six-state surveillance system. Data were obtained by medical record review of contacts with physicians, hemophilia treatment centers (HTCs), and other sources of care during 1993-1995 and from death certificates. Factors examined included age, race, state of residence, health insurance type, medical care source, hemophilia type/ severity, presence of inhibitor, liver dis-ease, HIV infection, and AIDS. A total of 2950 subjects were followed for an aver-age of 2.6 years. Their median age was 22 years; 73 % were white, 79 % had hemo-philia A, 42 % had severe disease, and 67 % had visited an HTC. During 7575 person years (PYs) of observation, 236 persons died—an age-adjusted mortality rate of 40.4 deaths/1000 PYs; 65 % of deaths were HIV related. In addition to age, factors independently associated with increased risk of death (relative risk, P value) were the following: AIDS (33.5