Thrombosis research
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Thrombosis is a common pathology underlying ischemic heart disease, ischemic stroke, and venous thromboembolism (VTE). The Global Burden of Disease Study 2010 (GBD 2010) documented that ischemic heart disease and stroke collectively caused one in four deaths worldwide. GBD 2010 did not report data for VTE as a cause of death and disability. ⋯ VTE associated with hospitalization was the leading cause of disability-adjusted-life-years (DALYs) lost in low and middle income countries, and second in high income countries, responsible for more DALYs lost than nosocomial pneumonia, catheter-related blood stream infections, and adverse drug events. VTE causes a major burden of disease across low, middle, and high income countries. More detailed data on the global burden of VTE should be obtained to inform policy and resource allocation in health systems, and to evaluate if improved utilization of preventive measures will reduce the burden.
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Thrombosis research · Nov 2014
Long-term efficacy and safety of a pasteurized, plasma-derived factor VIII concentrate (Beriate® P) in patients with haemophilia A.
Beriate(®) P was first introduced in Germany in 1990 as factor VIII (FVIII):C(®) HS Behring and subsequent product improvements yielded an albumin-free formulation with a specific activity of approximately 170 IU/mg protein. In 1992, the concentration was raised to 100 IU FVIII/mL in the reconstituted product, with a mean specific activity of 270 IU/mg protein. Pathogen safety is achieved by careful donor selection and a combination of pasteurization and chromatographic purification steps. ⋯ Beriate(®) P has an excellent efficacy and safety profile. Many patients who were initiated on Beriate(®) P at our centre remain on the treatment today.
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Thrombosis research · Nov 2014
Multicenter Study Observational StudyImmune tolerance induction using a factor VIII/von Willebrand factor concentrate (BIOSTATE), with or without immunosuppression, in Australian paediatric severe haemophilia A patients with high titre inhibitors: a multicentre, retrospective study.
It has been postulated that factor VIII (FVIII) products containing von Willebrand factor (VWF) may improve immune tolerance induction (ITI) success rate in patients with haemophilia A and poor prognostic factors. ⋯ BIOSTATE was well-tolerated and effective when used for primary or salvage ITI in this cohort of paediatric patients with SHA and a high-level inhibitor.
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Thrombosis research · Nov 2014
Review Historical ArticleTreatment of haemophilia patients in East Germany prior to and after reunification in 1990.
The reunification of Germany in 1990 brought with it major challenges in terms of unifying the care offered to haemophilia patients. At that time, most of the treatment centres belonged to the largest regional hospitals. The centre for paediatric haemophilia patients in Leipzig was at the University Hospital. ⋯ Reunification coincided with the availability of virus-safe, high-purity plasma-derived factor VIII concentrates (e.g. Beriate(®) P), which changed the outlook for patients in terms of convenience, tolerability, and virus safety; and these new products quickly became the treatments of choice for haemophilia A patients at the Leipzig Children's Hospital. Today, 20 years later, nearly all of the patients initiated on Beriate(®) P at the time of reunification continue with that treatment, and are still benefitting from its excellent efficacy, tolerability, and virus-safety profile.
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Thrombosis research · Nov 2014
Review Historical ArticleThe history of haemophilia - a short review.
The history of haemophilia dates back to the 2nd century AD, with the first "modern" descriptions of the condition appearing during the 1800s. At that time, transfusion medicine and haemophilia became closely linked, with blood transfusion being the only possible treatment option. A turning point in the history of haemophilia came in the middle of the 20th century when researchers identified an "antihaemophilic globulin" that could reduce the clotting time in haemophilic blood, thereby paving the way for the introduction of cryoprecipitate and the first clotting factor concentrates for the treatment of haemophilia A, haemophilia B and von Willebrand disease. ⋯ Work on improving factor concentrates continues, with efforts directed towards extending their half-lives using recombinant albumin-fused proteins and other modern technologies. The past 20 years has witnessed major improvements in almost all aspects of haemophilia treatment. It is hoped the next 20 years will add promising new chapters to the haemophilia book of history.