Epidemiology of Haemophilia in Greece: An Overview

Abstract

SummaryDemographic data of the Greek haemophilia A and B population for the period 1972-1993 were analyzed. Prevalence at birth including known not-registered patients was calculated at 23.1 per 100,000 male births. However, the observed prevalence in 1993 was only 61% of the expected. Since 1975 the proportion of mild cases had significantly increased. Adjusted by age, severity and HIV status reproductive fitness of haemophiliacs was 0.62. Overall mortality was 2.6 times higher than in the general population, but 7.9 times among patients with severe haemophilia and 16.4 among HIV(+) haemophiliacs. Fifty out of 78 deaths occurred among HIV(+) patients and 28 of these were caused by AIDS. Inhibitor patients did not show excess mortality due to bleeding. Cancer mortality was equal to normal, but the number of deaths from ischaemic heart disease was 0.25 of the expected. Risk of death due to cerebral haemorrhage was 3.8 times higher in HIV(+) haemophiliacs than in HIV(-).

Mortality rates, life expectancy, and causes of death in people with hemophilia A or B in the United Kingdom who were not infected with HIV

“…There was a clear reduction in the number of deaths from ischemic heart disease compared with the number expected from general population rates, as has previously been observed in studies of mortality in people with hemophilia in the Netherlands 7 (1 death observed, 5 expected) and Greece 23 (1 death observed, 4 expected). A study of hospital discharge rates in hemophilia in the United For personal use only.…”

Cardiovascular disease risk factors

“…In a larger US study using data from the six-state Hemophilia Surveillance System Project [5], Kulkarni et al [7] demonstrated that the known CVD risk factors (hypertension, diabetes mellitus and hyperlipidemia) were independently associated with IHD in the hemophilia population, similar to the general population. In a prospective study looking at 100 Dutch hemophilia patients, Biere-Rafi et al [16] found that the prevalence of CVD risk factors was similar to the general population, thus supporting the hypothesis that the reduction in cardiovascular mortality that has been reported in hemophilia patients [1][2][3][4]6] is due to their protective hypocoagulability state. Walsh et al [17] also looked at the prevalence of hypertension, diabetes and obesity as part of a study to measure health-related quality of life in the Canadian hemophilia population as compared to the general population.…”

“…Although data on the protective effect of hemophilia on cardiovascular diseases (CVDs) are conflicting [1][2][3][4][5][6][7][8], their longevity exposes this group of patients to the same CVD risk factors (hypertension, smoking, diabetes mellitus, hyperlipidemia, obesity) as the general population. There are limited data on the prevalence and management of such risk factors in patients with hemophilia when compared to the general population.…”

Prevalence and risk factors for heart disease among males with hemophilia

“…Several European studies [1][2][3] and one U.S. study [4] of cause-specific mortality have found lower rates of death from ischemic heart disease in persons with hemophilia compared to the general population. These findings have been interpreted as providing evidence that the hypocoagulable state offers protection from the thrombotic event that precipitates infarction.…”