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  • 1
    Electronic Resource
    Electronic Resource
    Immunoadsorption for coagulation factor inhibitors: a retrospective critical appraisal of 10 consecutive cases from a single institution (2003)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 9 (2003), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  Immunoadsorption is occasionally used as an adjuvant measure in the treatment of subjects with coagulation factor inhibitors. We reviewed our recent 3-year period experience with this procedure in 10 subjects. Immunoadsorption was used in the context of an immune tolerance protocol for 3 subjects with severe congenital deficiency in factor VIII, IX, and XIII; it was effective in lowering the level of inhibitor but immune tolerance was not achieved. It allowed successful use of porcine factor VIII in 4 cases of acquired hemophilia and in one case of inhibitor in mild hemophilia A. This therapeutic approach seems to be more useful in acquired hemophilia than in severe congenital factor deficiencies with inhibitors.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1351-8216.2003.00814.x
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Elective orthopaedic surgery for inhibitor patients (2003)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 9 (2003), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  We report on a series of 108 elective orthopaedic surgical procedures. It includes 88 radiosynoviortheses and 20 major orthopaedic procedures, performed on 51 patients at nine centres worldwide. The average age of patients was 28.5 years (range 5–40 years), and the average follow-up time was 2 years (range 1–5 years). There were 82 good results, 15 fair and 11 poor. In the synoviorthesis group (41 patients, 88 synoviortheses) the average age was 14.3 years (range 5–40 years) and the average follow-up was 6.5 years (range 1–10 years). There were 66 good results, 14 fair and eight poor. There were no complications. In the group of major orthopaedic procedures, the average age of the 10 patients was 32.5 years (range 27–40 years), and the average follow-up was 2.3 years (range 1–5 years). There were 16 good results, one fair and three poor. Postoperative bleeding complications occurred in three of the 20 major orthopaedic procedures performed (15% complications rate). They occurred in three patients treated with insufficient doses of recombinant activated factor VII. Despite such complications, the study has shown that haemophilic patients with inhibitors requiring elective orthopaedic surgery (EOS) can undergo such procedures with a high expectation of success. In other words, EOS is now possible in haemophilic patients with inhibitors, leading to an improved quality of life for these patients. Thorough analysis of each case as part of a multidisciplinary team will allow us to perform elective orthopaedic procedures in patients with inhibitors.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.2003.00803.x
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Late clinical, plain X-ray and magnetic resonance imaging findings in haemophilic joints treated with radiosynoviorthesis (2000)
    Oxford UK : Blackwell Science Ltd
    Haemophilia 6 (2000), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The clinical, plain X-ray and magnetic resonance imaging (MRI) findings were studied in 13 haemophilic joints previously treated with radiosynoviorthesis. 32P had been injected into the joints at a median of 16 years earlier in an attempt to halt recurrent haemorrhage. Prior to 32P injection, the majority of joints demonstrated bone damage evident on plain X-ray, secondary to recurrent haemorrhage. At the follow-up evaluation we found plain X-rays were adequate to identify cysts, erosions and cartilage loss in these very damaged joints. MRI was superior to clinical examination and plain X-ray in identifying synovial hyperplasia and effusions.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.2000.00433.x
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  • 4
    Electronic Resource
    Electronic Resource
    Can activated recombinant factor VII be used to postpone the exposure of infants to factor VIII until after 2 years of age? (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.  Two retrospective studies have suggested that exposure to factor VIII (FVIII) in early infancy is associated with an increased risk of FVIII inhibitor development. We prospectively studied 11 infants who needed replacement therapy for bleeding episodes before the age of 2 years. They received activated recombinant factor VII (rFVIIa) concentrate on demand, with the intention of postponing their first exposure to FVIII after 2 years of age. Thirty-three bleeding episodes were treated with 154 doses of rFVIIa with no evidence of adverse effect. Bleeding was controlled in 27 of 33 episodes. Mouth bleeds were most difficult to treat. The use of rFVIIa allowed postponement of the use of FVIII for a mean of 5.5 months (median 4, range 0–12) but in only three of 11 children could be the first exposure to factor postponed after the age of 2 years. With this modest effect of rFVIIa in postponing the first exposure to FVIII, more convincing evidence for the benefit of such a postponement will have to be demonstrated before rFVIIa could be recommended for this indication.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.2005.01088.x
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  • 5
    Electronic Resource
    Electronic Resource
    Chemical synovectomy in haemophilia: status and challenges (2001)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 7 (2001), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Chemical synovectomy has been used for the last three decades as an alternative for surgical synovectomy in the treatment of chronic haemophilic synovitis. Comparable results have been obtained with the many different radionuclides used: decreased bleeding, decreased pain and stable range of motion, in more than 50% of treated patients. Yttrium 90Y and Phosphorus 32P seem to be the isotopes of choice. Osmic acid and rifampicine have been used in a relatively small number of subjects. Global result of treatment with these two pro-ducts seems to be less favourable than with radionuclides. Late effects of chemical synovectomy are not known.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.2001.00113.x
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  • 6
    Electronic Resource
    Electronic Resource
    An evaluation of the stability of factor VIII inhibitors in plasma and plasma dried on filter paper discs stored at room temperature (2003)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 9 (2003), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary. The transportation of plasma specimens to specialized haemophilia centre laboratories for anti-factor VIII inhibitor titre determination is often necessary. The routine method of transporting frozen specimens on dry ice is limited by its cost and need for special handling. The present study was undertaken to evaluate the effect of storing specimens at room temperature on the FVIII inhibitor titre determinations using the Bethesda assay. Specimens stored both in liquid phase as well as adsorbed onto filter paper discs were studied. The results of the present study demonstrate that plasma specimens stored for up to 2 weeks at room temperature, either in liquid phase or adsorbed onto filter paper, yield equivalent measures of FVIII inhibitor titres using the Bethesda assay to plasma specimens stored frozen at −70 °C. Plasma specimens dried on filter paper discs and stored at room temperature offers a reliable, more practical and less expensive alternative to frozen plasma as a means of transport to specialized referral laboratories for analysis of anti-FVIII titres.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.2003.00713.x
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  • 7
    Electronic Resource
    Electronic Resource
    Compatible scales for progressive and additive MRI assessments of haemophilic arthropathy (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.  The international MRI expert subgroup of the International Prophylaxis Study Group (IPSG) has developed a consensus for magnetic resonance imaging (MRI) scales for assessment of haemophilic arthropathy. A MRI scoring scheme including a 10 step progressive scale and a 20 step additive scale with identical definitions of mutual steps is presented. Using the progressive scale, effusion/haemarthrosis can correspond to progressive scores of 1, 2, or 3, and synovial hypertrophy and/or haemosiderin deposition to 4, 5, or 6. The progressive score can be 7 or 8 if there are subchondral cysts and/or surface erosions, and it is 9 or 10 if there is loss of cartilage. Using the additive scale, synovial hypertrophy contributes 1–3 points to the additive score and haemosiderin deposition contributes 1 point. For osteochondral changes, 16 statements are evaluated as to whether they are true or false, and each true statement contributes 1 point to the additive score. The use of these two compatible scales for progressive and additive MRI assessments can facilitate international comparison of data and enhance the accumulation of experience on MRI scoring of haemophilic arthropathy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.2005.01049.x
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  • 8
    Electronic Resource
    Electronic Resource
    Consensus recommendations for use of central venous access devices in haemophilia (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.  Venous access is essential for delivery of haemophilia factor concentrate. Wherever possible, peripheral veins remain the route of choice, and the use of central venous access devices (CVADs) should be limited to cases of clear need in patients with caregivers able to exercise diligence in CVAD care and should continue no longer than necessary. CVADs are of recognized value for repeated administration of coagulation factors in haemophilia, particularly for prophylaxis and immune tolerance therapy and in young children. Evidence to guide best practices has been fragmentary, and standardized methods for CVAD usage have yet to be established. We have developed management recommendations based upon available published evidence as well as extensive clinical experience. These recommendations address patient and CVAD selection; CVAD placement, care and removal; caregiver/patient guidance; and complications, including infection and thrombosis. In the absence of inhibitors, ports are recommended, primarily because of fewer associated infections than with external catheters. For patients with inhibitors, ports also appear to be associated with fewer infections. Infection is the most frequent complication, and recommendations to prevent and treat infections are supported by extensive clinical data and experience. Strict adherence to handwashing and aseptic technique are essential elements of catheter care. Evidence-based data regarding the detection and treatment of CVAD-related thrombotic complications are limited. Caregiver education is an integral part of CVAD use and the procedural practices of users should be regularly re-assessed. These recommendations provide a basis for sound current CVAD practice and are expected to undergo further refinements as new evidence is compiled and clinical experience is gained.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.2004.00943.x
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    Paper (German National Licenses)
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  • 9
    Electronic Resource
    Electronic Resource
    Factor IX inhibitors and anaphylaxis in haemophilia B (1996)
    Oxford, UK : Blackwell Publishing Ltd
    Haemophilia 2 (1996), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.1996.tb00149.x
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    Paper (German National Licenses)
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  • 10
    Electronic Resource
    Electronic Resource
    Optimization of storage conditions for diluted working solutions of porcine factor VIII and performance of the Bethesda assay for the determination of antiporcine FVIII inhibitor titres (2003)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 9 (2003), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary. The use of porcine factor VIII (FVIII) (Hyate:C, Ipsen) has proven to be very successful in treating patients with FVIII inhibitors. The best way to predict the usefulness of porcine FVIII therapy, and/or to estimate the appropriate treatment dose in a given patient, is to measure the patient inhibitor titre against porcine FVIII with the Bethesda assay, using porcine FVIII as the source of FVIII in the assay. The goals of the present study were to (1) find the optimal storage temperature, diluent and concentration for a working solution of porcine FVIII to be used as the source of FVIII for the porcine Bethesda assay, (2) assess the reliability of the labelled FVIII units in the preparation of such working solutions of porcine FVIII and (3) compare the inhibitor titres determined by the Bethesda assay using both porcine and human standard reference curves for measuring residual FVIII. The results of the present study demonstrate that a ready-to-use working solution of 1 U mL−1 of Hyate:C diluted in human FVIII deficient plasma, either containing or deficient in von Willebrand factor antigen, is stable for up to 12 months, at −20 °C. The preparation of the 1 U mL−1 working solution could be reliably calculated based on the units indicated on the vial label. Finally, using the human standard curve yields similar results to using the porcine standard curve for measuring any titre of allo- or auto-antibody against FVIII in the Bethesda assay, using Hyate:C as the source of FVIII. These findings are of practical value when performing a porcine FVIII-based Bethesda assay.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.2003.00707.x
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