Although many studies of the impact of haemophilia on the quality of life were conducted, there is hardly any data on the social status of haemophiliacs. It was the aim of our study to obtain data on the social status of Austrian haemophiliacs and to compare these with an age- and sex-matched reference population. Furthermore, we collected data on the quality of life of haemophilia patients. We conducted a case-control study in two Austrian haemophilia centres with 53 patients (mean age 36.7 +/- 10.6 years) and 104 male controls (mean age 36.7 +/- 11.1 years). Socio-demographic data were collected using a standardized questionnaire and quality-of-life data using the SF-36. More patients (56.6%) than controls (37.5%) were married (P = 0.023), whereas more controls (17.3%) than patients (3.8%) had a partner with whom they were not married (P = 0.016). The percentage having children was equivalent in both groups (47% and 41% respectively), but controls had more children (mean number 1.5 in patients and 2.1 in controls, P < 0.007). A greater number of patients was unemployed (34% of patients, 9% of controls, P < 0.001) as well as retired (23% and 4% respectively; P < 0.001). Patients had worse scores regarding physical functioning, role-physical, bodily pain and general health (P < 0.001), whereas vitality, social functioning, role-emotional and mental health were similar in both groups. Despite their disability, most of the Austrian haemophiliacs share a sound family environment. This suggests that they are highly capable of coping with their chronic disease and is indicated by good scores for role-emotional and mental health.

An anti-inflammatory efficacy of PoCo on the elicitation phase of the ICD induced by repeated SLS test could be observed and quantified by three independent, non-invasive biophysical assessment parameters. This effect can be explained by its influence on pro-inflammatory enzymes, namely phospholipase A2.

SummaryLevels of anticardiolipin antibodies (ACA) were measured in 55 patients with haemophilia A in serum samples obtained in 1983 and in 1987. Twenty-one patients were negative for anti HIV-1 antibodies in 1983 and remained negative in 1987; 34 patients had anti HIV-1 antibodies in 1983; 17 of these latter patients remained asymptomatic, whereas 17 patients developed ARC or AIDS during the 4 years follow-up. Thirteen anti HIV-1 negative patients had elevated ACA levels in 1983; subsequently, a significant decrease was observed in all these subjects (p <0.001). All anti HIV-1 positive patients had elevated ACA levels in 1983; normal values were found in 9 patients in 1987. Yet, these changes were not significant (p >0.05). ACA levels were significantly higher in HIV-1 infected patients than in those without anti HIV-1 antibodies (p <0.05). There was no difference of ACA levels between the two anti HIV-1 positive patient groups, be it in 1983 or be it in 1987 (p >0.05). There was no correlation of ACA levels with serum IgG concentrations, CD4+ lymphocytes, or the consumption of factor VIII concentrates.

Patient registries are databases essential for identifying and tracking individuals with a particular disease and for collecting epidemiological information. The data obtained are necessary for quality control and quality assurance in treatment and for studying the impact of new developments on prevention, diagnosis and treatment. The Austrian Hemophilia Registry is a joint initiative between Austrian hemophilia treaters, represented by the Austrian Hemophilia Society's scientific advisory board, and the Austrian Hemophilia Society (OHG). The registry's main objective is to record information on patients with hemophilia, such as severity of disease, types of treatment and general health status. The registry also aims to improve the planning of supply of factor concentrates and to provide an instrument for early detection of side effects, such as an increase in inhibitor development or certain infections. The registry consists of three parts: the first contains basic information on quality control, the second contains extended data for quality control collected annually, and the third, "study part", covers scientific data and is also updated annually. For the latter, written informed consent of each patient is a prerequisite. Data are stored centrally on a server of an independent, public institution (University for Health Sciences, Medical Informatics and Technology: UMIT). The server is situated in a data-processing center with sophisticated security measures including physical control of access. Participating institutions comprise the main hemophilia care centers in Austria. Statistical analysis is carried out on anonymized data only. The project was financed by a public-private partnership with financial resources provided by the Austrian Ministry of Health (BMGFJ) and the pharmaceutical industry. The entire project, which is planned to be long term, will be monitored, evaluated and adjusted by the scientific advisory board accordingly.

SummaryTwenty-one immunodeficiency virus 1 (HIV 1)-positive hemophilic patients were treated with Azidothymidine (AZT) for symptomatic HIV infection. The median observation period was 20.5 months.At 25 months the probability of survival was 82%, the probability of progression of disease from CDC III or IV C2 to IV C1 (AIDS) was 20% in patients on continuous AZT treatment and 50% in patients with intermption of treatment. Three patients developed severe leukopenia and 3 patients severe anemii during AZT treatment. In 1 patient a dose-dependent striking increase of transaminases during AZT treatment was observed. In 7 patients treatment was intermpted, in 1 patient because of anemia, in 1 because of pruritus and in 5 patients because of noncompliance.No signiticant changes in the consumption of clotting factor concentrates and number of bleeding episodes before and during AZT treatment were noted.We conclude, that both hematological and non-hematological side effects of AZT in HIV 1-infected hemophilic patientr ur. comparable to those seen in other risk groups . AzT does not increase the bleeding tendency in this patient group.