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  • 1
    Electronic Resource
    Electronic Resource
    Long-term survival of HIV-infected patients with haemophilia (1995)
    Oxford, UK : Blackwell Publishing Ltd
    Haemophilia 1 (1995), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: In France, patients with haemophilia were infected by HIV up until October 1985, with a maximum of seroconversion between 1983 and 1985. There was a progressive development of AIDS in the 1158 infected patients as reported by Health Authorities. By the end of 1992, 32% of the haemophilia population had developed AIDS and 38 had developed clinical or biological symptoms of immunodeficiency. However, 27% had no clinical symptoms and no severe disorder of the immune system. The present study was established to determine factors common to patients with prolonged survival.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.1995.tb00037.x
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Continuous infusion of B-domain deleted recombinant factor VIII (ReFacto) in patients with haemophilia A undergoing surgery: clinical experience (2004)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 10 (2004), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  This retrospective, open-label, non-comparative study evaluated continuous infusion of recombinant factor VIII (ReFacto®), B-domain deleted recombinant FVIII (BDDrFVIII), in patients with haemophilia A undergoing surgery and requiring 〉5 consecutive days of treatment. Sixteen patients from eight centres underwent a total of 20 procedures. Haemostatic outcome was assessed as ‘excellent’ or ‘good’ in 75% of procedures, and target FVIII:C levels were maintained throughout the continuous infusion period. The reported volume of blood loss during surgery was also within the normal range for non-haemophilic patients for the type of surgery performed. Red blood cell transfusions were required to balance excessive blood loss during BDDrFVIII continuous infusion in eight (40%) procedures (seven patients), five with bleeding or requiring volume replacement and three to treat anaemia secondary to blood loss. Non-serious adverse events considered by investigators as possibly or probably related to BDDrFVIII continuous infusion were infrequent (n = 5) considering the duration of treatment (n =239 cumulative days of continuous infusion), and all of these were mild-to-moderate in severity. No thromboembolic complications were reported except for one case of thrombophlebitis occurring at the infusion site. Only two patients (four events) experienced serious adverse bleeding; BDDrFVIII was otherwise well-tolerated. These data show that continuous infusion of BDDrFVIII provides reliable haemostasis and is an effective and well-tolerated regimen for patients with haemophilia A undergoing surgery.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.2004.01013.x
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    A retrospective study on chemical and radioactive synovectomy in severe haemophilia patients with recurrent haemarthrosis (1999)
    Oxford UK : Blackwell Science Ltd
    Haemophilia 5 (1999), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Between 1970 and 1994, 116 chemical and 90 radioactive synovectomies were performed in 107 patients with severe haemophilia and two with type 3 von Willebrand's disease. The products used were osmic acid (OA) in 100 cases, 90-Yttrium in 35 cases, 186-Rhenium in 48, 169-Erbium in two, hexacetonide triamcinolone in 16 and radioactive gold in five cases. The use of radioactive colloids is not allowed in France in patients under 15 years of age. Twenty-nine patients had more than one synovectomy per joint. All patients were evaluated for 6 months post-synovectomy, using both a clinical and a radiological score. Six months after synovectomy, a good or excellent result was obtained for 81% of the joints treated with isotopes, compared with 44% of those treated with OA, P 〈 0.001. This superiority of isotopes over osmic acid was still observed after 6 months for the 89 joints that were re-evaluated, with follow-up ranging from 1 to 9 years. It was possible to calculate a radiological score in 84 cases. With OA the best results were from the joints with the lowest scores pre-synovectomy (〈7). No correlation could be established between the clinical and the radiological scores, due to the small size of the sample. In summary: (1) chemical and radioactive synovectomy are simple and safe procedures for haemophilic arthropathy, (2) in our series, after 6 months the efficacy of isotopic synovectomy was greater than that of chemical synovectomy, and this benefit seems to persist after 6 months, and up to 9 years in the group of patients with longer-term follow-up.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.1999.00287.x
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    European data of a clinical trial with a sucrose formulated recombinant factor VIII in previously treated haemophilia A patients (2002)
    Oxford, UK : Blackwell Science, Ltd
    Haemophilia 8 (2002), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  To increase the safety of antihaemophilic treatment, the production process of full-length recombinant factor VIII (FVIII) KOGENATE® Bayer (Kogenate®FS)has been modified. Human albumin is no longer added as stabilizer during purification and in final formulation. Instead, the new KOGENATE® Bayer production process uses sucrose as a stabilizer in the formulation and adds solvent/detergent virus inactivation step. An European clinical trial was carried out in Germany and France in previously treated patients with severe haemophilia A who had more than 100 exposure days to exogenous FVIII. Pharmacokinetic data was analysed according to one-stage and chromogenic assays. Efficacy and safety during home therapy and in surgical procedures were evaluated; inhibitor formation was carefully monitored. Safety and efficacy were evaluated in 33 European patients for 24 months. Patients received more than 13 million IU KOGENATE® Bayer. Over 75% of patients accrued more than 100 exposure days with the new product. Of 875 bleeding episodes, 90.7% were treated with 1 or 2 infusions and 75.8% of responses to treatment were rated as ‘excellent’ or ‘good’. Prophylactic treatment was the most common mode of therapy (60.7% of infusions). The product was well-tolerated and FVIII recovery studies were consistent throughout the study period. Only 0.26% of adverse events were reported to be drug related. No evidence of de novo inhibitor formation was observed. Overall, KOGENATE® Bayer was efficacious, safe and well-tolerated for the treatment of haemophilia A in multitransfused patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1351-8216.2001.00131.x
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    Intracranial haemorrhages in French haemophilia patients (1991–2001): clinical presentation, management and prognosis factors for death (2005)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 11 (2005), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  Intracranial haemorrhage (ICH) is known to be a severe although uncommon complication of haemophilia. A national survey has been conducted in France in order to collect information about ICHs which occurred in haemophiliacs between 1991 and 2001 and to propose recommendations for the diagnostic and treatment of ICH. Within this period, 123 episodes of ICH were recorded from 106 patients. Two-thirds of ICH concerned patients with severe haemophilia. Half of the cases occurred in patients under 15 years of age, 67.2% of which were post-traumatic. Ten cases occurred in neonates with three fatal outcomes. Overall mortality was high (21.9%) suggesting that availability of clotting factor concentrates has not improved the prognosis of this event. Morbidity was also high with 60% of long-term sequelae. The following parameters have been identified as prognostic factors for death: thrombocytopenia, HCV infection, intraventricular or intraparenchymatous haemorrhage. A delay in diagnosis was mentioned in 43.3% of cases, often related to the lack of recognition of the initial symptoms, which may be very common (apathy, tearfulness in young children and headache in elder patients). Delayed replacement therapy was recorded in 37.2% of cases. Emergency units initially dealt with half of these patients. Information concerning recognition and management of these episodes, not only in severe haemophilia, but also in moderate and mild forms, should be regularly supplied to paediatricians in maternity and physicians from emergency units, as well as to patients and their relatives.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.2005.01090.x
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    Paper (German National Licenses)
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