Pancreas allograft thrombosis.

Thrombosis of the transplanted pancreas is a common and often catastrophic event. Predisposing factors include the hypercoagulable state of many patients with diabetic renal failure, preservation-related graft endothelial injury, and low-velocity venous flow. Clinical management includes optimization of modifiable risk factors, controlled anticoagulation, graft monitoring, and early therapeutic intervention.

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.

Since the 1970s, mortality in the hemophilia population has been dominated by human immunodeficiency virus (HIV) and few reports have described mortality in uninfected individuals. This study presents mortality in 6018 people with hemophilia A or B in the United Kingdom during 1977 to 1998 who were not infected with HIV, with follow-up until January 1, 2000. Given disease severity and factor inhibitor status, all-cause mortality did not differ significantly between hemophilia A and hemophilia B. In severe hemophilia, all-cause mortality did not change significantly during 1977 to 1999. During this period, it exceeded mortality in the general population by a factor of 2.69 (95% confidence interval [CI]: 2.37-3.05), and median life expectancy in severe hemophilia was 63 years. In moderate/mild hemophilia, all-cause mortality did not change significantly during 1985 to 1999, and median life expectancy was 75 years. Compared with mortality in the general population, mortality from bleeding and its consequences, and from liver diseases and Hodgkin disease, was increased, but for ischemic heart disease it was lower, at only 62% (95% CI: 51%-76%) of general population rates, and for 14 other specific causes it did not differ significantly from general population rates. There was no evidence of any death from variant Creutzfeldt-Jakob disease or from conditions that could be confused with it.

World Federation of Haemophilia programs in developing countries.

The World Federation of Haemophilia (WFH) is a global not-for-profit organization devoted to furthering the treatment of hemophilia and related disorders. Founded in Montreal in 1963, it has grown tremendously during the last 40 years and now has 107 National Member Organizations and has been recognized by the World Health Organization. The mission of the WFH is to introduce, improve, and maintain care for patients with hemophilia and related disorders. Several specific programs may be used as tools to achieve these goals. The WFH organizes regular educational workshops on key aspects of hemophilia care. Fellowships are offered to approximately 30 healthcare professionals each year that provide funding for a period of study of up to 8 weeks at an International Hemophilia Training Center of their choice. The WFH twinning program helps emerging hemophilia treatment centers develop partnerships with well-established centers. Although the ultimate goal of the WFH is to promote sustainable hemophilia treatment in developing countries, donations of concentrate are regularly made through the Humanitarian Aid program. The WHF launched the Global Alliance for Progress in its 40th anniversary year, establishing a partnership between the WFH and other stakeholders, including the pharmaceutical industry. The aim is to focus on 30 to 40 developing countries during the next 10 years, aiming to more than double the number of patients diagnosed with hemophilia in those countries. The WFH is also involved in fostering discussion on key issues such as safety and supply of blood products.

Blood products for hemophilia: past, present and future.

Hemophilia is an inherited bleeding disorder, which in its severe form is characterized by recurrent hemarthrosis and internal bleeding. In the absence of effective treatment the prognosis is poor, but the development of blood products in the last few decades has transformed the outlook, and patients can now live essentially normal lives. Treatment options vary around the world, with cryoprecipitate still the mainstay of therapy in many developing countries. Many patients were infected with hepatitis and/or HIV through the use of coagulation factor concentrates before the introduction of physical methods of viral inactivation in the mid-1980s. In more affluent countries, the debate in recent years has focused on the relative merits of plasma versus recombinant products. Coagulation factor concentrates are expensive, and cost-benefit and quality-of-life studies will assume an increasing importance in guiding the selection of products. Looking to the future, genetic engineering offers the potential to create coagulation factors with enhanced properties, such as reduced immunogenicity and prolonged half-life. Transgenic animals are a potential source of therapeutic materials. Several trials of gene therapy for hemophilia are already underway.

The impact of HIV on mortality rates in the complete UK haemophilia population.

OBJECTIVE: To estimate the effect of HIV-1 infection on subsequent mortality in a complete population. DESIGN: Prospective cohort study. SUBJECTS: A total of 7250 haemophilic males were registered in the UK Haemophilia Centre Doctors' Organisation database, 1977-1998. Most were infected with hepatitis C virus. In the early 1980s, 1246 were infected with HIV-1 from contaminated clotting factor concentrate. The main outcome measure was the date of death. RESULTS: During 1977-1984 annual mortality in severely haemophilic males was 0.9%. For those with HIV, annual mortality increased progressively from 1985 reaching over 10% during 1993-1996 before falling to 5% in 1997-1999, whereas without HIV it remained approximately 0.9% throughout 1985-1999. For moderately/mildly haemophilic males the annual mortality was 0.4% during 1977-1984. Without HIV it remained approximately 0.4% throughout 1985-1999, but with HIV it was similar to that in severe haemophilia with HIV. Survival was strongly related to age at HIV infection. The large temporal changes in mortality with HIV were largely accounted for by HIV-related conditions. Without HIV annual liver disease mortality remained below 0.2% throughout 1985-1999, but with HIV it was 0.2% during 1985-1990, 0.8% during 1991-1996, and 0.8% during 1997-1999. CONCLUSION: These data provide a direct estimate of the effect of HIV-1 infection on subsequent mortality in a population with a high prevalence of hepatitis C. From approximately 3 years after HIV infection, large, progressive increases in mortality were seen. From 1997, after the introduction of effective treatment, substantial reductions occurred, although mortality from liver disease remained high.

The airline passenger: current medical issues.

It is widely accepted that travel by air is unlikely to be a hazard to the vast majority of passengers. However, there are potentially adverse effects of cabin air of poor quality and of the reduced oxygen tension of the cabin environment. There is also the possibility of thrombosis related, at least in part, to the relative inactivity of a long journey. It may well be that the toxicity of the oil additives that are used in aircraft engines should be revisited, and that research should be carried out on the relative importance and potential interactions of the many risk factors for thrombosis that could be enhanced during a long flight. Formal guidelines need to be developed for passengers.