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  • 1
    Electronic Resource
    Electronic Resource
    Heterologous B-Cell Antisera May Detect Non-Ig, Non-HLA-DR Antigens (1979)
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 10 (1979), S. 0 
    Details
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: A heterologous antiserum (antiserum 7420) against B-lymphocyte antigen(s) was raised in a rabbit by immunization with peripheral blood lymphocytes from a patient with chronic lymphocytic leukaemia (CLL). After absorptions with pooled normal human serum, IgA, IgD, and IgM M-components, the fluorescein isothiocyanate-conjugated F(ab′)2 fragments were prepared and further absorbed with normal peripheral blood leucocytes. The F(ab′)2 fragments, studied in direct immunofluorescence, reacted with both normal and CLL B lymphocytes but not with T lymphocytes. Comparative studies with an HLA-DR antiserum showed that the antigen(s) detected by 7420 antiserum did not redistribute together with HLA-DR antigens in cocapping experiments, nor did the HLA-DR antiserum block the reaction of 7420 F(ab′)s fragments with B lymphocytes. The 7420 F(ab′)z fragments precipitated detergent-solubilized B-cell membrane material with a molecular weight of around 40,000 and 150,000 daltons. The conclusion drawn is that the 7420 and HLA-DR antigens are different. The 7420 antigen was also shown to be different from classical HLA antigens, β2-microglobulin, surface immunoglobulin, the Fc receptor, and HC protein.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-3083.1979.tb01329.x
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  • 2
    Electronic Resource
    Electronic Resource
    Cellular Expression of the Non-HLA-DR B-Cell Antigen 7420: Studies on Non-lymphoid Cells and on Lymphocytes from Different Species (1981)
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 13 (1981), S. 0 
    Details
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: We recently reported on a heterologous antiserum (antiserum 7420), raised against chronic lymphocytic leukaemic lymphocytes, that reacts with B-lymphocyte antigen(s) different from known B-cell markers such as HLA-DK (la-like) antigens, surface immunoglobulin, and the Fc receptor. This antiserum is now shown to react also with a C3-rcceptor-positive population of lymphocytes from the dog, monkey, ox and sheep but not from rodents and avians. The anti-serum does not react with the non-lymphoid human cells mature granulocytes, thrombocytes, erythrocytes, or spermatozoa, nor with the cultured cell lines tested, i.e. fibroblasts, melanoma cells. HeLa cells and green monkey kidney cells. This reactivity pattern differs somewhat from that reported for HLA-DR antiserum. The antigen(s) defined by the 7420 antiserum and the C3 receptor are different, since they redistribute independently on the lymphocyte surface.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-3083.1981.tb00157.x
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  • 3
    Electronic Resource
    Electronic Resource
    The administration of desmopressin by nasal spray: a dose-determination study in patients with mild haemophilia A or von Willebrand's disease (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: Desmopressin is an important haemostatic agent in many types of bleeding disorders. The intranasal route for administration of desmopressin has attracted much interest because it is convenient and makes self-treatment possible. In the present study the effects of three doses (300, 450 and 600 μg) of desmopressin delivered by nasal spray and those of 0.3 μg/kg injected intravenously were compared in five patients with haemophilia A and 11 with von Willebrand's disease. There was no statistically significant difference in peak VIII:C concentration in the haemophilia patients and no difference in peak vWF concentration in von Willebrand patients between the four desmopressin dosages. Bleeding time response was comparable after all four dosages. We recommend a spray dosage of 300 μg for home treatment. As cover for major surgery or in connection with severe bleedings, however, intravenous administration is to be recommended.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.1995.tb00047.x
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  • 4
    Electronic Resource
    Electronic Resource
    Clinical outcomes and resource utilization associated with haemophilia care in Europe (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: We conducted a multicentre, cross- sectional study of 1042 haemophilia subjects across Europe to compare various health outcomes associated with on-demand vs. prophylactic factor-substitution therapy. Demographic, medical history, and healthcare resource utilization data were analysed along with the number of bleeding events over the past 6 months. Treatment-cost data were also examined to provide preliminary information for future economic studies. A logistic regression analysis, controlling for other statistically significant covariates, showed that patients treated on demand were 3.4 times more likely to have had a joint bleed over the previous 6 months than those treated with prophylaxis. Multiple regression analyses further confirmed these findings, because on-demand subjects had, on average, 5.15 more joint bleeds over the reporting period than patients treated with prophylaxis. Notably, these findings were even more dramatic for younger haemophilia patients when our study sample was stratified by age. Due to the high cost of factor replacement, healthcare costs were significantly higher for subjects treated prophylactically. While hospital costs for prophylaxis subjects were, on average, lower, statistically significant cost savings for prophylactic subjects were not noted. These results suggest that clinicians and health policy decision-makers should consider the advantages of prophylactic therapy for haemophilia patients in formulating treatment protocols and allocating health resources.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.2002.00580.x
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  • 5
    Electronic Resource
    Electronic Resource
    Quality-of-life differences between prophylactic and on-demand factor replacement therapy in European haemophilia 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: The European Study on the Clinical Outcomes and Resource Utilization associated with Haemophilia Care was designed to compare various health outcomes associated with on-demand and prophylactic factor substitution methods in European haemophilia patients. While the primary objective of this research is to conduct an economic analysis, an important component of this study is to evaluate quality-of-life differences that may exist between patients who utilize these two styles of therapy. Quality-of-life research has emerged as a primary measure of health outcomes because it allows the augmentation of traditional clinical indicators of health with data gathered from the patient's perspective. A total of 1033 haemophilia patients from 16 European haemophilia treatment centres were enrolled in this study. The SF-36, a multidimensional quality-of-life instrument, was administered to all participants. This instrument measures eight health-related quality-of-life dimensions: physical functioning, physical role limitations, bodily pain, general health, vitality, social functioning, emotional role limitations, and mental health. All haemophilia subjects enrolled in the study scored significantly lower than the population normative means in the three physical dimensions and in the general health dimension. HIV-negative haemophiliac subjects differed significantly by factor substitution type in a multivariate analysis examining all eight health dimensions. Univariate analyses testing each dimension separately indicated that patients treated prophylactically reported significantly less bodily pain, better general health, and scored significantly higher in the physical functioning, mental health, and social functioning dimensions. While these results suggest that health-related quality-of-life may be better for haemophilia patients treated prophylactically, future prospective studies that gather periodic quality-of-life data over time should be conducted.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.2002.00581.x
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  • 6
    Electronic Resource
    Electronic Resource
    Pharmacokinetics of recombinant factor IX in relation to age of the patient: implications for dosing in prophylaxis (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: The aims of this study were to investigate possible age-related changes in the disposition of factor IX procoagulant activity (FIX:C) after administration of recombinant factor IX (rFIX) and to translate the pharmacokinetic findings into suggestions for dosing of rFIX during prophylactic treatment of haemophilia B. Pharmacokinetic data were available from a previous study on 56 patients, aged 4–56 years (one of whom was excluded from analysis). FIX:C curves during prophylactic dosing were computer-simulated from the single-dose data. Clearance and volume of distribution at steady state of FIX:C increased linearly with body weight of the patients, consequently increasing during childhood and adolescence but remaining fairly constant during adulthood. The terminal half-life of FIX:C showed no correlation with age, while in vivo recovery (in U dL–1 per U kg–1 given) tended to increase. Computer-predicted trough levels of exogenous FIX:C during repeated doses of rFIX (50 U kg–1) and, conversely, doses (in U kg–1) needed to maintain a 1-U dL–1 trough level showed little or no dependence on age. There was considerable interindividual variation in disposition and required doses of rFIX, emphasizing the need for individual dose titration. Dosing of rFIX according to lean body mass instead of body weight did not reduce this variability. During prophylaxis a 1-U dL–1 trough level can normally be maintained by dosing every 2–3 days, the former schedule resulting in, on average, a 45% lower consumption of rFIX.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.2001.00465.x
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  • 7
    Electronic Resource
    Electronic Resource
    von Willebrand disease: an update in the Åland islandsSummary of a Nordic Symposium on von Willebrand disease, 24–25 September 1998, Mariehamn, Åland (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: In the past decades von Willebrand disease (vWD) has, in several respects, fallen into the shadow of classical haemophilia due to all problems that have faced those dealing with congenital bleeding disorders, not least regarding blood-borne diseases. The time has come to revisit and refocus on vWD, probably the most common bleeding disorder. Accordingly, a number of Nordic physicians and scientists working in this field organized a meeting on the ferry boat from Stockholm to Mariehamn, in the Åland islands, the home of the index families with this disease, as described by Erik von Willebrand in 1926. The objective of the meeting was to make a comprehensive survey of vWD as seen from the situation in the Nordic countries. In order to achieve all goals some scientists of note from countries outside the Nordic area were invited as well.This paper gives a summary of the symposium, mainly based on the separate papers published in this issue.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.1999.0050s2001.x
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  • 8
    Electronic Resource
    Electronic Resource
    Costs of on-demand and prophylactic treatment for severe haemophilia in Norway and Sweden (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.  The expected annual cost (in the year 2000 prices) for a 30-year-old patient with average individual and treatment characteristics for on-demand EUR 51 832 (95% CI: 44 324–59 341) and for prophylaxis EUR 146 118 (95% CI: 129 965–162 271), was obtained from panel-data analysis of an 11-year retrospective panel of 156 patients with severe haemophilia in Norway and Sweden. Costs included haemophilia-related treatment costs within the health-care sector (factor concentrate, doctors’ visits, diagnostic procedures, hospitalisation, invasive procedures, etc.) and cost for haemophilia-related resource use in other sectors (lost production, use of special equipment, adaptation of workplace and domicile, etc). Although costs of lost production, reconstructive surgery and hospitalisation were higher for on-demand, they did not balance out the higher costs of factor-concentrate consumption in prophylaxis. The cut-off risk of premature death, where on-demand and prophylaxis would have been equally costly, was 3.7 percentage units higher for on-demand than for prophylaxis. Such a great risk difference has not been reported elsewhere to our knowledge. Estimated cost-elasticities indicated that annual costs of prophylaxis would increase by approximately the same proportion as a potential increase in the price of factor concentrate and decrease less than proportionately with a reduction in prescribed dose kg−1. For on-demand, the annual costs would increase by approximately the same proportion as an increase in the prescribed dose kg−1.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.2004.00952.x
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  • 9
    Electronic Resource
    Electronic Resource
    Clinical evaluation of an advanced category antihaemophilic factor prepared using a plasma/albumin-free method: pharmacokinetics, efficacy, and safety in previously treated patients with haemophilia A (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.  The efficacy and safety of an advanced category recombinant antihaemophilic factor produced by a plasma- and albumin-free method (rAHF-PFM) was studied in 111 previously treated subjects with haemophilia A. The study comprised a randomized, double-blinded, crossover pharmacokinetic comparison of rAHF-PFM and RECOMBINATE rAHF (R-FVIII); prophylaxis (three to four times per week with 25–40 IU kg−1 rAHF-PFM) for at least 75 exposure days; and treatment of episodic haemorrhagic events. Median age was 18 years, 96% of subjects had baseline factor VIII 〈1%, and 108 received study drug. Bioequivalence, based on area under the plasma concentration vs. time curve and adjusted in vivo recovery, was demonstrated for rAHF-PFM and R-FVIII. Mean (±SD) half-life for rAHF-PFM was 12.0 ± 4.3 h. Among 510 bleeding events, 473 (93%) were managed with one or two infusions of rAHF-PFM and 439 (86%) had efficacy ratings of excellent or good. Subjects who were less adherent to the prophylactic regimen had a higher bleeding rate (9.9 episodes subject−1 year−1) than subjects who were more adherent (4.4 episodes subject−1 year−1; P 〈 0.03). One subject developed a low titre, non-persistent inhibitor (2.0 BU) after 26 exposure days. These data demonstrate that rAHF-PFM is bioequivalent to R-FVIII, and suggest that rAHF-PFM is efficacious and safe, without increased immunogenicity, for the treatment of haemophilia A.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.2004.00932.x
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  • 10
    Electronic Resource
    Electronic Resource
    Consensus perspectives on prophylactic therapy for haemophilia: summary statement (2003)
    Oxford, UK : Blackwell Publishing Ltd
    Haemophilia 9 (2003), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  Participants in an international conference on prophylactic therapy for severe haemophilia developed a consensus summary of the findings and conclusions of the conference. In the consensus, participants agreed upon revised definitions for primary and secondary prophylaxis and also made recommendations concerning the need for an international system of pharmacovigilance. Considerations on starting prophylaxis, monitoring outcomes, and individualizing treatment regimens were discussed. Several research questions were identified as needing further investigation, including when to start and when to stop prophylaxis, optimal dosing and dose interval, and methods for assessment of long-term treatment effects. Such studies should include carefully defined cohorts, validated orthopaedic and quality-of-life assessment instruments, and cost-benefit analyses.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.9.s1.17.x
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