| 1. |
- Berntorp, Erik, et al.
(author)
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Centraliserad vård grundläggande i vårdprogram för blödarsjuka
- 1999
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In: Läkartidningen. - 0023-7205. ; 96:15, s. 1849-1852
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Journal article (peer-reviewed)abstract
- Haemophilia is a rare and potentially life-threatening disease. In Sweden, with a population of approximately 8.5 million, about 350 people suffer from the more severe forms of haemophilia or von Willebrand disease. Meticulous management is important if the patients are to be spared chronic disability and serious treatment complications. The disease is lifelong and affects psychosocial aspects of life among patients and their families. With the help of a grant from the Swedish Board of Halth and Welfare, a care programme has been designed to guarantee Swedish haemophiliacs comparable and optimal care. The programme has been drawn up by representatives of the three haemophilia centres in Sweden (at University Hospital, Malmo, Sahlgrenska University Hospital, Gothenburg, and Karolinska Hospital, Stockholm) in co-operation with the World Federation of National Haemophilia Organisations. To ensure optimal individual application of the programme, individualised management strategies and patient information leaflets have been prepared.
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| 2. |
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| 3. |
- Astermark, Jan, et al.
(author)
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Low recurrence rate after deep calf-vein thrombosis with 6 weeks of oral anticoagulation
- 1998
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In: Journal of Internal Medicine. - : Wiley. - 1365-2796 .- 0954-6820. ; 244:1, s. 79-82
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Journal article (peer-reviewed)abstract
- OBJECTIVES: To evaluate the recurrence rate after deep calf-vein thrombosis treated with 6 weeks of oral anticoagulation. DESIGN AND SUBJECTS: A 2 year follow-up of 126 consecutive patients admitted to the Department of Internal Medicine with venographically verified deep calf-vein thrombosis. RESULTS: One hundred and twenty-six patients were treated with warfarin for 6 weeks, 18 of them having had a previous episode of venous thrombosis (DVT). Eleven patients (8.7%) suffered a recurrent thromboembolic episode within 2 years, four of which were within the first 3 months. Eight of those without a history of DVT had a recurrence (7.4%). Three of these were activated protein C (APC)-resistant, one was protein C-deficient and one had malignant melanoma. Eight patients (6.3%) reported minor haemorrhagic complications, but no major bleeding was seen. CONCLUSION: Our data support the use of a 6 week regimen of secondary oral prophylaxis after a first episode of deep calf-vein thrombosis in patients without a permanent risk factor. Whether individuals with inherited thrombophilia require prolonged treatment remains to be evaluated.
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| 4. |
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| 5. |
- Astermark, Jan, et al.
(author)
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Blödarsjuka med HIV. Långsammare infektionsförlopp hos yngre och vid större förbrukning av faktorkoncentrat
- 1998
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In: Läkartidningen. - 0023-7205. ; 95:1, s. 48-50
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Journal article (peer-reviewed)abstract
- HIV disease progression and the effect of replacement therapy with clotting factor concentrates (CFCs) were studied in 100 Swedish haemophiliacs, mean age at seroconversion 29 years (range, 4-72). On average 16 years after seroconversion, 67 per cent of the patients had CD4+ cell counts of < 200 x 10(6)/l, 50 per cent had developed AIDS, and 58 per cent had died. HIV disease progression was significantly slower in those aged less than 28 (median age) at seroconversion (P = 0.004). Moreover, mortality was inversely correlated to total annual CFC consumption after adjustment for age and HIV-related therapy, i.e., Pneumocystis carinii prophylaxis and antiretroviral drugs (P = 0.014), but unrelated to the purity of the CFCs used. After adjustment for age, annual CFC consumption and HIV-therapy, prophylactic replacement therapy was not associated with significantly better survival than on-demand treatment. It is concluded that in HIV-positive haemophiliacs replacement therapy may have a beneficial effect on the immune system, and that CFC purity and the regimen (prophylaxis vs on-demand) would seem to be factors of minor importance.
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| 6. |
- Mätzsch, Thomas, et al.
(author)
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Laparoscopic cholecystectomy in a patient with hemophilia B
- 1992
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In: Surgical Laparoscopy & Endoscopy. - 1051-7200. ; 2:4, s. 339-340
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Journal article (peer-reviewed)abstract
- Surgical procedures in hemophiliacs is a demanding challenge for the surgeon and hematologist. This report deals with a successful laparoscopic cholecystectomy in a young patient with hemophilia B. Despite a prolonged operation time, because of a partially intrahepatic gallbladder, no bleeding complications were registered and the patient recovered well. Laparoscopic cholecystectomy seems to be the method of choice in hemophiliacs, as it minimizes tissue trauma. Nevertheless, the procedure should be performed only in specialized centers with a fully equipped coagulation laboratory and a team of surgeons and hematologists familiar with the special problems of surgery in hemophiliacs.
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| 7. |
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| 8. |
- Astermark, Jan, et al.
(author)
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Major surgery seems not to influence HIV disease progression in haemophilia patients
- 1998
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In: British Journal of Haematology. - : Wiley. - 0007-1048. ; 103:1, s. 10-14
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Journal article (peer-reviewed)abstract
- The influence of major surgery on HIV disease progression and decline in CD4+ cell count was evaluated in 23 seropositive haemophilia patients. 24 HIV-infected patients served as non-operated controls. In addition, 32 age-matched seronegative subjects were included. The follow-up time was up to 5 years. During the course of the study, eight of the operated (35%) and 11 of the non-operated (48%) subjects developed HIV-related symptoms (P=0.267). The relative risk for developing HIV-related symptoms after surgery was 0.60 (95% CI 0.25; 1.48). A significant decline in CD4+ cell counts was observed in both the surgery (4.0 x 10(6)/l/month, 95% CI 2.0; 6.0 x 10(6), P=0.001) and the non-surgery (4.0 x 10(6)/l/month, 95% CI 2.0; 6.0 x 10(6), P=0.004) seropositive subgroup, but no difference between the two subgroups was seen (P=0.793). HIV (6.0 x 10(6)/l/month, 95% CI 2.1; 9.9 x 10(6), P=0.0005) but not surgery (-1.0 x 10(6)/l/ month, 95% CI -3.0; 0.96 x 10(6), P=0.647) was an independent predictor for the decline in CD34+ cell count. No interaction effect was observed between HIV infection and surgery (P=0.361). The annual amount of factor concentrate used for regular replacement therapy did not influence the decline in CD4+ cell count (P=0.492). We conclude that major surgery may be considered in symptom-free HIV-seropositive haemophilia patients, with CD4+ cell counts > or = 0.20 x 10(9)/l under similar premises as for seronegative subjects.
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| 9. |
- Astermark, Jan, et al.
(author)
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Malmo International Brother Study (MIBS): an international survey of brother pairs with haemophilia
- 1999
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In: Vox Sanguinis. - 1423-0410. ; 77:Suppl. 1, s. 80-82
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Journal article (peer-reviewed)abstract
- Malmo International Brother Study (MIBS) was initiated in 1996 in order to set up an international registry of twins and non-twin brothers with haemophilia and to search for genetic and compound factors predisposing for inhibitor development. As of July, 1997, 178 brother pairs are registered (143 haemophilia A and 35 haemophilia B patients). Sixteen of these pairs are twins. In 48 of the brother pairs (27%) there is a history of inhibitors, in 25 of them involving only one of the brothers. Immune tolerance induction has been attempted in 13 brother pairs (27%) and in four pairs the inhibitor has been eradicated. Additional demographic data need to be collected and, if possible plasma, IgG and DNA samples will be taken from inhibitor patients to serve as a tool for basic inhibitor experiments.
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| 10. |
- Astermark, Jan, et al.
(author)
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No effect of a new second-generation B-domain-deleted recombinant product on lymphocyte transformation in vitro: a study of plasma-derived and recombinant products
- 1997
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In: British Journal of Haematology. - : Wiley. - 0007-1048 .- 1365-2141. ; 99:2, s. 289-294
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Journal article (peer-reviewed)abstract
- Immunomodulatory effects of various factor VIII and factor IX clotting factor concentrates (CFCs) and of albumin were evaluated by a sensitive assay measuring the incorporation of 3H-thymidine in phytohaemagglutinin-stimulated lymphocytes in the presence of monodansylthiacadaverine. In contrast to previous findings by others, we found lymphocyte transformation to be inhibited by all plasma-derived factor VIII concentrates at concentrations of 0.02, 0.2 and 2.0 IU/ml, including those purified by monoclonal antibodies (P < 0.05). Kryobulin TIM3 had the most pronounced effect. In addition, three plasma-derived human albumin preparations exerted a similar inhibitory effect as the factor VIII concentrates, whereas the corresponding plasma-derived factor IX concentrates only manifested minor immunomodulatory effects. Of the recombinant preparations, only Recombinate exerted an inhibitory effect at 0.02 and 0.2 IU/ml, whereas both Kogenate and Recombinate decreased 3H-thymidine incorporation at 2.0 IU/ml (P = 0.01). No immunomodulatory effect at all was observed with r-VIII SQ, a new B-domain-deleted recombinant factor VIII preparation free from added albumin. The significance of this finding regarding immunological side-effects including inhibitor development remains to be evaluated, but this second-generation recombinant product opens up new and interesting perspectives yet to be explored.
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