| 1. |
- Astermark, J., et al.
(författare)
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Symposium in memory of Professor Inga Marie Nilsson
- 2001
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Ingår i: Haemophilia. - : Wiley. - 1351-8216. ; 7:4, s. 401-410
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Konferensbidrag (refereegranskat)abstract
- Professor Inga Marie Nilsson (1923-99) was a pioneer in the field of bleeding and thrombo-embolic disorders and made several major scientific contributions during her career. To honour her memory, colleagues from all over the world were invited to cover several aspects of haemostasis by giving state-of-the-art lectures at an international symposium in Malmö on September 22-23, 2000, chaired by Professors Lou Aledort and Erik Berntorp. Colleagues of Professor Nilsson in Malmö gave a short introduction to each topic. A short review of the meeting will be presented.
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| 2. |
- Wamers, F., et al.
(författare)
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Exclusive measurements of nuclear breakup reactions of 17Ne
- 2014
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Ingår i: EPJ Web of Conferences. - : EDP Sciences. - 2101-6275 .- 2100-014X. ; 66
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Konferensbidrag (refereegranskat)abstract
- We have studied one-proton-removal reactions of about 500MeV/u 17Ne beams on a carbon target at the R3B/LAND setup at GSI by detecting beam-like 15O-p and determining their relative-energy distribution. We exclusively selected the removal of a 17Ne halo proton, and the Glauber-model analysis of the 16F momentum distribution resulted in an s2 contribution in the 17Ne ground state of about 40%. © Owned by the authors, published by EDP Sciences, 2014.
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| 3. |
- Aksouh, F., et al.
(författare)
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STUDY OF THE O-15(2p,gamma)Ne-17 CROSS SECTION BY COULOMB DISSOCIATION OF Ne-17 FOR THE rp PROCESS OF NUCLEOSYNTHESIS
- 2014
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Ingår i: Acta Physica Polonica, Series B.. - 1509-5770 .- 0587-4254. ; 45:2, s. 229-234
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Tidskriftsartikel (refereegranskat)abstract
- The O-15(2p, gamma)Ne-17 cross section has been studied by the inverse reaction, the Coulomb dissociation of Ne-17. The experiment has been performed at the GSI. The Ne-17 excitation energy prior to decay has been reconstructed by using the invariant-mass method. The preliminary differential and integral Coulomb dissociation cross sections (sigma(Coul)) have been extracted, which provide a photoabsorption (sigma(photo)) and a radiative capture cross section (sigma(cap)). Additionally, important information about the Ne-17 nuclear structure will be obtained. The analysis is in progress.
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| 4. |
- Jeppesen, H. B., et al.
(författare)
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Investigation of the Li-9+H-2 -> Li-8+t reaction at REX-ISOLDE
- 2006
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Ingår i: Physics Letters, Section B: Nuclear, Elementary Particle and High-Energy Physics. - : Elsevier BV. - 0370-2693. ; 635, s. 17-17
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Tidskriftsartikel (refereegranskat)abstract
- The one-neutron transfer reaction Li-9 + H-2 -> Li-8 + t has been investigated in an inverse kinematics experiment by bombarding a deuterated polypropylene target with a 2.36 MeV/u Li-9 beam from the post-accelerator REX-ISOLDE at CERN. Excitation energies in Li-8 as well as angular distributions of the tritons were obtained and spectroscopic factors deduced. (c) 2006 Elsevier B.V. All rights reserved.
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| 5. |
- Jeppesen, H. B., et al.
(författare)
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Low energy reactions with radioactive ions at REX-ISOLDE - the Li-9+H-2 case
- 2005
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Ingår i: Nuclear Physics A. - : Elsevier BV. - 0375-9474. ; 748, s. 374-374
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Tidskriftsartikel (refereegranskat)abstract
- At the newly constructed postaccelerator REX-ISOLDE (Radioactive beam Experiment at ISOLDE) we have investigated reactions induced by a Li-9 beam incident on a deuterium target at an energy of 2.36 MeV/u. Most reaction channels were recorded. From one-neutron transfer the differential cross-section for the low lying Li-10 spectrum is found. The results from the other channels demonstrate the great applicability and many possibilities that REX-ISOLDE opens up.
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| 6. |
- Marganiec, C., et al.
(författare)
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Coulomb breakup of 17Ne from the viewpoint of nuclear astrophysics
- 2013
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Ingår i: Proceedings of Science.
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Konferensbidrag (refereegranskat)abstract
- By the Coulomb breakup of 17Ne, the time-reversed reaction 15O(2p,γ)17Ne has been studied. This reaction might play an important role in the rp process, as a break-out reaction of the hot CNO cycle. The secondary 17Ne ion beam with an energy of 500 MeV/nucleon has been dissociated in a Pb target. The reaction products have been detected with the LAND-R3B experimental setup at GSI. The preliminary differential and integral Coulomb dissociation cross section sCoul has been determined, which then will be converted into a photo-absorption cross section sphot o, and a two-proton radiative capture cross section σcap. Additionally, information about the structure of the 17Ne, a potential two-proton halo nucleus, will be received. The analysis is in progress.
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| 7. |
- Marganiec, J., et al.
(författare)
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Coulomb breakup of 17Ne from the viewpoint of nuclear astrophysics
- 2012
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Ingår i: Proceedings of Science. - Proceedings of Science : Sissa. - 1824-8039.
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Konferensbidrag (refereegranskat)abstract
- By the Coulomb breakup of 17Ne, the time-reversed reaction 15O(2p,γ)17Ne has been studied. This reaction might play an important role in the rp process, as a break-out reaction of the hot CNO cycle. The secondary 17Ne ion beam with an energy of 500 MeV/nucleon has been dissociated in a Pb target. The reaction products have been detected with the LAND-R3B experimental setup at GSI. The preliminary differential and integral Coulomb dissociation cross section sCoul has been determined, which then will be converted into a photo-absorption cross section sphoto, and a two-proton radiative capture cross section σcap. Additionally, information about the structure of the 17Ne, a potential two-proton halo nucleus, will be received. The analysis is in progress. © Copyright owned by the author(s) under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike Licence.
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| 8. |
- Marganiec, J, et al.
(författare)
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Experimental study of the 15O(2p ,γ)17Ne cross section by Coulomb Dissociation for the rp process
- 2016
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Ingår i: Journal of Physics Conference Series. - : IOP Publishing. - 1742-6588 .- 1742-6596.
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Konferensbidrag (refereegranskat)abstract
- The time-reversed reaction 15O(2p,γ)17Ne has been studied by the Coulomb dissociation technique. Secondary 17Ne ion beams at 500 AMeV have been produced by fragmentation reactions of 20Ne in a beryllium production target and dissociated on a secondary Pb target. The incoming beam and the reaction products have been identified with the kinematically complete LAND-R3B experimental setup at GSI. The excitation energy prior to decay has been reconstructed by using the invariant-mass method. The preliminary differential and integral Coulomb Dissociation cross sections (σCoul) have been calculated, which provide a photoabsorption (σphoto) and a radiative capture cross section (σcap). Additionally, important information about the nuclear structure of the 17Ne nucleus will be obtained. The analysis is in progress.
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| 9. |
- Marganiec, J., et al.
(författare)
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Experimental study of the O-15(2p, gamma) Ne-17 cross section by Coulomb Dissociation for the rp process
- 2016
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Ingår i: Journal of Physics: Conference Series. - : IOP Publishing. - 1742-6588 .- 1742-6596. ; 665:1
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Konferensbidrag (refereegranskat)abstract
- The time-reversed reaction O-15(2p, gamma) Ne-17 has been studied by the Coulomb dissociation technique. Secondary 17Ne ion beams at 500 AMeV have been produced by fragmentation reactions of Ne-20 in a beryllium production target and dissociated on a secondary Pb target. The incoming beam and the reaction products have been identified with the kinematically complete LAND-(RB)-B-3 experimental setup at GSI. The excitation energy prior to decay has been reconstructed by using the invariant-mass method. The preliminary differential and integral Coulomb Dissociation cross sections (sigma(Coul)) have been calculated, which provide a photoabsorption (sigma(photo)) and a radiative capture cross section (sigma(cap)). Additionally, important information about the nuclear structure of the Ne-17 nucleus will be obtained. The analysis is in progress.
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| 10. |
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| 11. |
- Kröll, T., et al.
(författare)
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Transfer Reactions on Neutron-rich Nuclei at REX-ISOLDE
- 2009
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Ingår i: AIP Conference Proceedings. - 1551-7616 .- 0094-243X. ; 1165, s. 363-368 461
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Konferensbidrag (refereegranskat)abstract
- We report on one- and two-neutron transfer reactions to study the single-particle properties of nuclei at the border of the "island of inversion". The (d,p)- and (t,p)-reactions in inverse kinematics on the neutron-rich isotope Mg-30, delivered as radioactive beam by the REX-ISOLDE facility, have been investigated. The outgoing protons have been detected and identified by a newly built array of Si detectors. The gamma-decay of excited states has been detected in coincidence by the MINIBALL array. First results for Mg-31 and from the search for the second, spherical, 0(+) state in Mg-32 are presented.
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| 12. |
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| 13. |
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| 14. |
- Astermark, Jan, et al.
(författare)
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Anti- and procoagulant activities in factor VII-deficient subjects
- 2001
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Ingår i: Thrombosis Research. - 1879-2472. ; 101:6, s. 435-440
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Tidskriftsartikel (refereegranskat)abstract
- The clinical feature in patients with congenital factor VII deficiency is in part dependent on the underlying genetic defect, but the mechanisms influencing the genotype-phenotype correlation remain to be fully elucidated. In addition, thromboembolic events have been reported. Compensatory mechanisms involving vitamin K-dependent factors have been suggested. We have measured anticoagulant activities in 25 factor VII-deficient subjects (factor VII activity < or =36%) and 23 age-matched controls and correlated these to the vitamin K-dependent procoagulant activities. Two of the patients had a history of thromboembolism. The factor VII-deficient patients were found to have a significantly lower protein C activity than the controls [0.84 U/ml (95% CI 0.78; 0.89) vs. 0.98 U/ml (95% CI 0.91; 1.05), P=.004]. In addition, the protein C activity was correlated to that of factor VII (r=.36; P=.014), factor IX (r=.45; P=.002) and factor X (r=.50; P=.0006), respectively. The level of prothrombin fragment 1+2 was correlated to the protein C (r=.40; P=.012) and to the factor VII activity (r=.42; P=.011). No differences between patients and controls were seen regarding total and free protein S, antithrombin, plasminogen activator inhibitor-1 (PAI-1) and tissue factor pathway inhibitor (TFPI). Seven of the patients were found to have the Factor V Leiden mutation, but none of them had experienced any thromboembolic event. The present data support the notion that compensatory hemostatic mechanisms might exist in that the protein C activity was found to be decreased in the factor VII-deficient subjects. Whether this could influence the clinical feature, including the risk of thromboembolic events in association with replacement therapy, remains to be evaluated.
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| 15. |
- Astermark, Jan, et al.
(författare)
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Primary prophylaxis in severe haemophilia should be started at an early age but can be individualized
- 1999
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Ingår i: British Journal of Haematology. - : Wiley. - 0007-1048. ; 105:4, s. 1109-1113
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Tidskriftsartikel (refereegranskat)abstract
- The frequency of joint bleeds and orthopaedic joint scores were evaluated in 121 patients with severe haemophilia who had started prophylactic treatment with clotting factor concentrates at least once weekly before the age of 10. 75 of the patients started before the age of 3, 31 at the age of 3-5 and 15 at the age of 6-9. Each subgroup was evaluated separately. In addition, a regimen of one infusion weekly was compared with that of two (haemophilia B) or three (haemophilia A) infusions weekly in each patient. A significant decrease in the overall number of joint bleeds per year was found after shortening the infusion interval (P<0.005), but the individual bleeding pattern varied. In survival analysis of the first pathologic joint score event, those who started prophylaxis before the age of 3 had a better outcome overall than those starting at later ages (P=0.001). However, in subgroup analysis, no significant difference was seen in the annual number of joint bleeds and the development of arthropathy between those starting with, or shifting to, the more intensive regimen before the age of 3 and those that were put on this regimen at the age of 3-5. Age at start of prophylaxis was found to be an independent predictor for the development of arthropathy (P=0.0002), whereas dose and infusion interval at start were not. Our data emphasize the importance of starting replacement therapy during the first years of life. However, it seems that when beginning the regimen it can be individualized and adjusted according to the bleeding pattern. In this way, the need for a venous access system may be assessed on an individual basis.
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| 16. |
- Berntorp, Erik, et al.
(författare)
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An approach to study the viral safety of plasma-derived products in previously treated, non-infected patients
- 2001
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Ingår i: Haemophilia. - : Wiley. - 1351-8216 .- 1365-2516. ; 7:4, s. 360-363
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Tidskriftsartikel (refereegranskat)abstract
- Using the polymerase chain reaction (PCR), we designed a study concept to evaluate the safety of plasma derivatives in previously treated patients who are non-infected by the specific viruses studied. Several product lots can be studied in a single patient, with a study period for each lot of 3 months. In the present study 19 patients were included for treatment with Baxter Hyland Immuno's PCR-screened factor VIII concentrate Immunate (n=7), factor IX concentrate Immunine (n=10), the by-passing agent FEIBA plus Immunine (n=1), and the protein C concentrate Ceprotin (n=1). PCR testing for hepatitis B, C or HIV genomic material in patient samples was done as well as serological testing. All patients remained negative for the tested markers. All seven Immunate patients completed three treatment periods with three different lots of the study drug. The median study period was 282 days and the median dose 115 000 units, with a median of 115 exposure days. Five of the 10 Immunine patients completed three treatment periods and four patients, two treatment periods. One Immunine patient was discontinued from the study for reasons unrelated to the study drug administration. The median study period was 305 days and the median total dose 82 200 units, with a median of 88 exposure days. Our study presents a new design to approach the evaluation of viral safety of new plasma derivatives in previously treated, non-infected patients (NIPs) and offers several advantages over the currently recommended studies using testing for serological markers of infection in previously untreated patients (PUPs).
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| 17. |
- Berntorp, Erik, et al.
(författare)
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Centraliserad vård grundläggande i vårdprogram för blödarsjuka
- 1999
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Ingår i: Läkartidningen. - 0023-7205. ; 96:15, s. 1849-1852
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Tidskriftsartikel (refereegranskat)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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| 18. |
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| 19. |
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| 20. |
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| 21. |
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| 22. |
- Eriksson, Bengt I., 1946, et al.
(författare)
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Prevention of deep-vein thrombosis and pulmonary embolism after total hip replacement. Comparison of low-molecular-weight heparin and unfractionated heparin.
- 1991
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Ingår i: The Journal of bone and joint surgery. American volume. - 0021-9355. ; 73:4, s. 484-93
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Tidskriftsartikel (refereegranskat)abstract
- In a prospective, randomized, double-blind study, the efficacy and safety of a low-molecular-weight heparin were compared with those of unfractionated sodium heparin (standard heparin) in 136 patients who had elective total hip replacement. The patients received subcutaneous injection of either 5000 international units of low-molecular-weight heparin once daily or 5000 international units of standard heparin three times a day. Treatment with low-molecular-weight heparin began twelve hours before the operation, and treatment with standard heparin began two hours preoperatively; both regimens were continued for ten days. Twelve days postoperatively, bilateral ascending phlebography was performed in 122 patients, sixty-three in the treatment group that received low-molecular-weight heparin and fifty-nine in the treatment group that received standard heparin. Pulmonary scintigraphy was performed in 127 patients. Deep-vein thrombosis was diagnosed in forty-four patients: nineteen (30 per cent) of the sixty-three who received low-molecular-weight heparin and twenty-five (42 per cent) of the fifty-nine who received standard heparin. All but four patients, two from each treatment group, were asymptomatic. The difference in the total rate of thrombosis in the two groups was not significant (p = 0.189). However, thrombosis occurred in the thigh in only six (10 per cent) of the patients who received low-molecular-weight heparin but in eighteen (31 per cent) of those who received standard heparin, a significant difference (p = 0.011). Pulmonary embolism was detected in twenty-seven patients: eight (12.3 per cent) of those who received low-molecular-weight heparin and nineteen (30.6 per cent) of those who received standard heparin. Only three patients had clinical signs of embolism. Pulmonary embolism was significantly more frequent in the group that received standard heparin (p = 0.016). Total loss of blood and the total amount of blood that was transfused were significantly reduced in the patients who received low-molecular-weight heparin compared with those who received standard heparin. Prophylaxis was not discontinued because of hemorrhage in any patient. The efficacy of low-molecular-weight heparin was superior to that of standard heparin in the prevention of femoral thrombosis and pulmonary embolism, although the over-all incidence of deep-vein thrombosis was not statistically different.(ABSTRACT TRUNCATED AT 400 WORDS)
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| 23. |
- Iversen, AKN, et al.
(författare)
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Limited protective effect of the CCR5 Delta 32/CCR5 Delta 32 genotype on human immunodeficiency virus infection incidence in a cohort of patients with hemophilia and selection for genotypic X4 virus
- 2003
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Ingår i: Journal of Infectious Diseases. - 1537-6613. ; 187:2, s. 215-225
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Tidskriftsartikel (refereegranskat)abstract
- The relationship among CCR5 genotype, cytomegalovirus infection, and disease progression and death was studied among 159 human immunodeficiency virus (HIV)-infected patients with hemophilia. One patient (0.6%) had the CCR5Delta32/CCR5Delta32 genotype (which occurs in similar to2% of the Scandinavian population) and a rapid disease course. His HIV V3 region contained genotypic features attributable to X4 virus and resembled functionally verified X4 virus and virus from patients treated with a CD4 cell-stimulating drug, tucaresol. Age-related differences in disease progression rate and survival time were seen for CCR5/CCR5 patients. Surprisingly, no protective effect of the CCR5/CCR5Delta32 genotype on disease progression or survival was seen for children but was evident for adults. Age group-related immunologic differences might explain this variation, and transmission route and/or viral phenotype variation within donor virus may be related to the limited protection of the CCR5Delta32/ CCR5Delta32 genotype. Sequence comparisons indicate that X4 virus can be selected in vivo due to either absence of CCR5 receptors or relative increase of CXCR4 receptors.
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| 24. |
- Jeppesen, H. B., et al.
(författare)
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Study of Li-10 via the Li-9(H-2, p) reaction at REX-ISOLDE
- 2006
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Ingår i: Physics Letters B. - : Elsevier BV. - 0370-2693 .- 1873-2445. ; 642:5-6, s. 449-454
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Tidskriftsartikel (refereegranskat)abstract
- The Click to view the MathML source reaction has been investigated at 2.36 MeV/u at the REX-ISOLDE facility. In this Letter we focus on the Click to view the MathML source channel which potentially holds spectroscopic information on the unbound nucleus 10Li. The experimental excitation energy spectrum and angular distribution are compared with CCBA calculations. These calculations clearly support the existence of a low-lying (s) virtual state, with a (negative) scattering length of the order asnot, vert, similar1324 fm and a p1/2 resonance with an energy of Ersimilar, equals0.38 MeV and a width of Γsimilar, equals0.2 MeV.
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| 25. |
- Jern, Christina, 1962, et al.
(författare)
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Changes of plasma coagulation and fibrinolysis in response to mental stress.
- 1989
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Ingår i: Thrombosis and haemostasis. - 0340-6245. ; 62:2, s. 767-71
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Tidskriftsartikel (refereegranskat)abstract
- To study the effects of standardized mental stress (arithmetic and the Stroop color word test) on plasma coagulation and fibrinolysis, blood samples were obtained before, during, and after 20 minutes of mental stress from 10 healthy, non-smoking young males aged 22 to 30 years. Reactions were compared with those observed during physical exercise and infusion of adrenaline. Both von Willebrand factor antigen and factor VIII coagulant activity increased significantly in response to mental stress (95 +/- 28 vs 123 +/- 56%; p less than 0.05 and 125 +/- 54 vs 217 +/- 170%; p less than 0.05, respectively). There was also a significant increase of factor VII coagulant activity (86 +/- 31 vs 108 +/- 51%; p less than 0.05). Furthermore, mental stress caused an activation of the fibrinolytic system with an elevation of tissue plasminogen activator activity and tissue plasminogen activator antigen (0.80 +/- 0.48 vs 1.23 +/- 0.96 IU/ml; p = 0.076 and 4.38 +/- 1.87 vs 5.78 +/- 2.58 IU/ml; p less than 0.01). Fibrinogen concentration increased during stress (1.95 +/- 0.29 vs 2.11 +/- 0.27 g/l; p less than 0.05). Similar but more pronounced responses were observed during exercise and adrenaline infusion. Parallel to the increases in coagulation and fibrinolytic factors there were significant increases in heart rate, and systolic and diastolic blood pressure. It is concluded that mental stress has significant effects on plasma coagulation and fibrinolysis, and that it could thus affect important risk factors for cardiovascular disease.
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| 26. |
- Johansson, J O, et al.
(författare)
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Long-term treatment with growth hormone decreases plasminogen activator inhibitor-1 and tissue plasminogen activator in growth hormone-deficient adults.
- 1996
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Ingår i: Thrombosis and haemostasis. - 0340-6245. ; 76:3, s. 422-8
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Tidskriftsartikel (refereegranskat)abstract
- The syndrome of growth hormone deficiency (GHD) in adults is associated with premature atherosclerosis, increased cardiovascular mortality, abnormal lipoprotein patterns and abnormal body composition. We have previously shown that GH-deficient adults have increased concentrations of fibrinogen and plasminogen activator inhibitor (PAI-1) activity. The aim of the present investigation was to study coagulation and fibrinolysis in 17 patients with adult-onset GHD during two years of treatment with recombinant human GH (12 micrograms/kg body weight/day). The impact of the contemporary changes in metabolic variables and body composition on coagulation and fibrinolysis was studied. The patients received conventional thyroid, adrenal and gonadal hormone replacement therapy. PAI-1 activity, PAI-1 antigen and tissue plasminogen activator (t-PA) antigen levels decreased during the GH treatment period (p < 0.05). The decrease was more pronounced in patients with high pre-treatment levels of the different variables. alpha 2-antiplasmin decreased (p < 0.05), while plasminogen was unchanged during two years of GH treatment. Fibrinogen concentrations tended to decrease after two years of GH treatment (p = 0.06), while the coagulation factors VII and VIII were unchanged. von Willebrand factor demonstrated a transient decrease after 18 months of GH treatment. The coagulation inhibitor, protein C, decreased (p < 0.05), while antithrombin was unchanged. Fasting plasma insulin increased (p < 0.01), but blood glucose did not differ after two years of GH treatment. Serum high-density lipoprotein cholesterol, total cholesterol and triglycerides were unaltered. Body fat decreased during the initial GH treatment but was unaltered after two years, while lean body mass increased (p < 0.001) and the waist over hip circumference ratio tended to decrease (p = 0.06). In conclusion, PAI-1 activity, PAI-1 antigen and t-PA antigen decreased during long-term GH treatment. These changes may be a direct effect of GH itself or may be secondary to the favourable changes in body composition. It remains to be seen whether changes in these fibrinolytic variables during rhGH treatment reduces the cardiovascular risk in patients with GHD. The present results suggest that GH plays a role in the regulation of fibrinolysis.
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| 27. |
- Knobe, Karin, et al.
(författare)
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Factor VIII inhibitors in two families with mild haemophilia A: structural analysis of the mutations
- 2000
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Ingår i: Haemostasis. - : S. Karger AG. - 0301-0147. ; 30:5, s. 268-279
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Tidskriftsartikel (refereegranskat)abstract
- The development of inhibitory antibodies against coagulation factor VIII (FVIII) in patients with mild haemophilia A is uncommon. We describe here two families in which three or two members have developed inhibitors, suggesting a familial predisposition. The mutations found, in the A2 (Arg593Cys) and C1 domains (Tyr2105Cys), have been reported to give rise to inhibitor development in single individuals in addition to the family cluster we describe, strongly suggesting that these amino acid substitutions give rise to a more immunogenic protein. The analysis of structural models of activated factor VIII revealed that Arg593 is solvent-exposed and involved in a network of electrostatic interactions while Tyr2105 is partially buried and has hydrophobic interactions essentially with Ile2144. All these residues are strictly conserved in the FVIII amino acid sequence from man, pig and mouse, suggesting, at least, that they have structural roles. We propose that the two mutations in these families could cause mild haemophilia A because they induce local conformational changes (and possible secretion or intermolecular interaction problems, e.g., with von Willebrand factor) compatible with immunogenicity and production of inhibitors against the infused wild-type FVIII.
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| 28. |
- Knoebl, P, et al.
(författare)
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Demographic and clinical data in acquired hemophilia a: results from the european acquired haemophilia (each2) registry.
- 2012
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Ingår i: Journal of Thrombosis and Haemostasis. - : Elsevier BV. - 1538-7933 .- 1538-7836. ; 10:4, s. 622-631
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Tidskriftsartikel (refereegranskat)abstract
- Background: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous family or personal history of bleeding. Although data on several AHA cohorts have been collected, limited information is available on the optimal management of AHA. Objectives: The European Acquired Hemophilia registry (EACH2) was established to generate a prospective, large-scale, pan-European database on demographics, diagnosis, underlying disorders, bleeding characteristics, treatment and outcome of AHA patients. Results: 501 (266 male, 235 female) patients from 117 centers and 13 European countries were included in the registry between 2003-2008. In 467 cases, hemostasis investigations and AHA diagnosis were triggered by a bleeding event. At diagnosis, patients were a median of 73.9 years. AHA was idiopathic in 51.9%; malignancy or autoimmune diseases were associated with 11.8% and 11.6% of cases. 57% of the non-pregnancy-related cases were male. 474 bleeding episodes were reported at presentation, and hemostatic therapy initiated in 70.5% of patients. Delayed diagnosis significantly impacted treatment initiation in 33.5%. 477 patients underwent immunosuppression, and 72.6% achieved complete remission. Conclusions: Representing the largest collection of consecutive AHA cases to date, EACH2 facilitates the analysis of a variety of open questions in AHA.
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| 29. |
- Lindgren, A-C, et al.
(författare)
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Normal vaginal delivery is to be recommended for haemophilia carrier gravidae
- 1994
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Ingår i: Acta Paediatrica, International Journal of Paediatrics. - : Wiley. - 0803-5253. ; 83:6, s. 609-611
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Tidskriftsartikel (refereegranskat)abstract
- Every child with severe or moderate haemophilia A or B, born in Sweden during the period 1970-1990, was traced in the national haemophilia register, all 117 case records being surveyed for mode of delivery and perinatal complications. Of the 117 deliveries. 13 were by caesarean section and the remaining 104 vaginal. Of the 13 caesarean sections, 2 were performed because the woman was a haemophilia carrier the remaining 11 (5 emergency, 6 elective) for other reasons. Neonatal complications were: subgaleal or cephalic haematoma (n = 12), intracranial haemorrhage (n = 4), umbilical bleeding (n = 4), haematuria (n = 1), retro-orbital bleeding (n = 1) and abnormal bleeding after surgery. injection or venepuncture (n = 28). Of the 12 infants with subgaleal/cephalic haematoma, 10 were delivered by vacuum extraction. Seven more infants were delivered by vacuum extraction and another 11 were born without abnormal bleedings after laborious (> 24 h) delivery. Of the 4 children with intracranial haemorrhage, all were sporadic cases of haemophilia, 1 was a premature birth by caesarean section in the 27th week, 1 was delivered by vacuum extraction and the remaining 2 vaginally. In these 4 cases there were no sequelae or only minor ones. We conclude that the risk of serious bleeding in conjunction with normal vaginal delivery is small, but that vacuum extraction should be avoided when delivering offspring of haemophilia carriers.
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| 30. |
- Lindgren, A, et al.
(författare)
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Characterization of inhibitors to FVIII with an ELISA in congenital and acquired haemophilia A.
- 2002
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Ingår i: Haemophilia : the official journal of the World Federation of Hemophilia. - 1351-8216. ; 8:5, s. 644-8
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Tidskriftsartikel (refereegranskat)abstract
- Different methods can be used for the detection and quantification of inhibitors or antibodies to coagulation factor VIII (FVIII). Traditionally, clotting assays have been used, in particular the Bethesda assay. These assays have, however, several shortcomings, due to the complex reaction kinetics of some inhibitors and a low sensitivity to low-titre antibodies. In addition, a universal FVIII inhibitor standard is lacking. Furthermore, clotting assays do not detect noninhibitory antibodies. Use of ELISAs has been described and FVIII from various commercially available FVIII concentrates has been used as target antigen in the assays. In the present study, we systematically explored the influence of different FVIII concentrates on the performance of an ELISA for detection of FVIII antibodies. The described ELISA was also used for further characterization of FVIII inhibitors in patients with acquired and congenital haemophilia A. We found that the source of FVIII had a substantial impact on the frequency of antibody detection. Albumin-free recombinant FVIII as target antigen gave the highest sensitivity for the assay, whereas plasma-derived concentrates containing a high level of von Willebrand factor (vWF) gave the lowest sensitivity. Presumably vWF interferes with the binding of antibodies to FVIII. We suggest that albumin-free recombinant FVIII should be used as target antigen when ELISAs are used for detection of FVIII antibodies.
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| 31. |
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| 32. |
- Ljung, R., et al.
(författare)
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Port-A-Cath usage in children with haemophilia : Experience of 53 cases
- 1998
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Ingår i: Acta Paediatrica, International Journal of Paediatrics. - : Wiley. - 1651-2227. ; 87:10, s. 1051-1054
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Tidskriftsartikel (refereegranskat)abstract
- Experience of the Port-A-Cath implantable venous access system in 53 children with severe or moderate haemophilia A or B from seven centres in five countries is reviewed. The cumulative duration of follow-up was 1578 months (median 30 months, range 1-114). Of the devices implanted, 70% (37/53) were used without complications (median follow-up 32 months; range 1-114) and the remaining 30% (16/53) were associated with various types of complication: infection, bacteraemia or septicaemia in 56% (9/16) of cases, i.e. a rate of 0.07 per follow-up year or 0.19 per 1000 patient days, or various technical complications occurring after a median of 32 months (range 4-75) of uncomplicated use in the remaining 44% (7/16). Of the patients with inhibitors, 64% (7/11) manifested complications. Both doctors and parents considered that the Port-A-Cath device can be used with an acceptable frequency and severity of complications, and that it enables regular prophylactic or on-demand home treatment of children with haemophilia to be begun at an early age.
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| 33. |
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| 34. |
- Madurga, M., et al.
(författare)
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Evidence of a new state in Be-11 observed in the Li-11 beta-decay
- 2009
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Ingår i: Physics Letters, Section B: Nuclear, Elementary Particle and High-Energy Physics. - : Elsevier BV. - 0370-2693. ; 677:5, s. 255-259
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Tidskriftsartikel (refereegranskat)abstract
- Coincidences between charged particles emitted in the beta-decay of Li-11 were observed using highly segmented detectors. The breakup channels involving three particles were Studied in full kinematics allowing for the reconstruction of the excitation energy of the Be-11 states participating in the decay. In particular, the contribution of a previously unobserved state at 16.3 MeV in Be-11 has been identified selecting the alpha + He-7 -> alpha + He-6 + n channel. The angular correlations between the alpha particle and the center of mass of the He-6 + n system favors spin and parity assignment of 3/2(-) for this state as well as for the previously known state at 18 MeV. (C) 2009 Elsevier B.V. All rights reserved.
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| 35. |
- Madurga, M., et al.
(författare)
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Kinematic identification of the t branch in the decay of 11Li
- 2009
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Ingår i: European Physical Journal A. - : Springer Science and Business Media LLC. - 1434-601X .- 1434-6001. ; 42:3, s. 415-419
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Tidskriftsartikel (refereegranskat)abstract
- The -delayed 8 Li + t branch of the 11Li decay was identified via kinematical analysis of the charged particles observed in coincidence. The back-to-back events observed in a narrow angular acceptance were compared to a Monte Carlo simulation. The 8 Li + t sum energy spectrum was fitted assuming that the decay occurs through an intermediate state in 11Be . The resulting energy centroid and full-width half maximum for the intermediate state are 18.35(30)MeV and 1.5(4)MeV, respectively. The -delayed 8 Li + t branching ratio obtained from the coincidence spectrum, BR t = 0.93(8)×10-4 , is in agreement with previous measurements but more precise.
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| 36. |
- Madurga, M., et al.
(författare)
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Study of $[$beta$]$-delayed 3-body and 5-body breakup channels observed in the decay of 11Li
- 2008
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Ingår i: Nuclear Physics A. - : Elsevier BV. ; 810, s. 1-12
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Tidskriftsartikel (refereegranskat)abstract
- The [beta]-delayed charged particle emission from 11Li has been studied with emphasis on the three-body n[alpha]6He and five-body 2[alpha]3n channels from the 10.59 and 18.15 MeV states in 11Be. Monte Carlo simulations using an R-matrix formalism lead to the conclusion that the AHe resonance states play a significant role in the break-up of these states. The results exclude an earlier assumption of a phase-space description of the break-up process of the 18.15 MeV state. Evidence for extra sequential decay paths is found for both states.
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| 37. |
- Madurga, M., et al.
(författare)
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Study of β-delayed charged particle emission of 11Li: Evidence of new decay channels
- 2008
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Ingår i: Journal of Physics: Conference Series. - : IOP Publishing. - 1742-6588 .- 1742-6596. ; 111:1
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Tidskriftsartikel (refereegranskat)abstract
- The break-up of the 18.2 MeV state in 11Be was studied in a 11Li β-decay experiment. We report here on the study of the dominating breakup channels involving na6He or 3n2α in the final state, with special emphasis dedicated in this contribution to the three-particle channel. The two emitted charged particles were detected in coincidence using a highly segmented experimental set-up. The observed experimental energy-vs-energy scatter plot indicates a sequential breakup where nuclei of mass 4, alpha particles, and mass 7, 7He, are involved. A Monte-Carlo simulation of the sequential channel, 11Be* → α + 7He → nα6He was performed and compared to the experimental data and to a simulation of the direct break-up of the 18.2 MeV state nα6He by phase space energy distribution. The energy-versus-energy plot are explained by the sequential simulation but not by the phase space simulation.
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| 38. |
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| 39. |
- Ridefelt, P, et al.
(författare)
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Ny in vitro-analys prövad: Blödningstid fortfarande bästa metod för test av den primära hemostasen
- 2001
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Ingår i: Lakartidningen. - 0023-7205. ; 98:37, s. 4-3922
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Tidskriftsartikel (refereegranskat)abstract
- The need is great for a simple, cheap and readily accessible method for the evaluation of primary hemostasis in work-ups at both out-patient clinics and units caring for surgical or intensive care patients. PFA-100 is a recently introduced instrument for in vitro testing of platelet function. We report experiences from Stockholm, Gothenburg and Malmo of PFA-100 measurements performed on samples from healthy controls and from patients with von Willebrand disease or platelet disorders. It is shown that the PFA-100 system has a high sensitivity for von Willebrands disease, while the sensitivity for hereditary platelet dysfunction is low. In its present design this new device could not replace the template bleeding time as a screening test for primary hemostasis.
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| 40. |
- Tengborn, Elisabeth, 1978, et al.
(författare)
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The 8Li + 2H reaction studied in inverse kinematics at 3.15 MeV/nucleon using the REX-ISOLDE post-accelerator
- 2011
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Ingår i: Physical Review C - Nuclear Physics. - 2469-9985 .- 2469-9993. ; 84:6, s. 064616-
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Tidskriftsartikel (refereegranskat)abstract
- The reaction 8Li + 2H has been studied in inverse kinematics at the incident energy of 3.15 MeV/nucleon, using the REX-ISOLDE post-accelerator. The reaction channels corresponding to (d,p), (d,d), and (d,t) reactions populating ground states and low-lying excited states in 7–9Li have been identified and the related angular distributions extracted and compared with coupled-channels, distorted-wave Born approximation (DWBA), and coupled-reaction-channels calculations. For the inelastic and (d,t) channels we find that higher order effects are very important and hence one needs to go beyond the simple DWBA to extract reliable structure information from these processes.
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| 41. |
- Tengborn, L., et al.
(författare)
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Anaphylactoid reactions and nephrotic syndrome - A considerable risk during factor IX treatment in patients with haemophilia B and inhibitors : A report on the outcome in two brothers
- 1998
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Ingår i: Haemophilia. - : Wiley. - 1351-8216. ; 4:6, s. 854-859
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Tidskriftsartikel (refereegranskat)abstract
- Anaphylaxis/anaphylactoid reactions have recently been reported after few treatments with factor IX concentrates in patients with haemophilia B at the same time as inhibitors to factor IX were demonstrated. In some of these cases nephrotic syndrome has appeared during immune tolerance induction (ITI) with high doses of factor IX concentrates. Gene deletions seem to be associated with a high risk of developing antibodies to factor IX. This report presents two brothers with deletion of 1 bp in exon f of the factor IX gene. Both showed anaphylactoid reactions and they were desensitized using slow i.v. injections of factor IX. At the time of anaphylaxis, inhibitors of factor IX in a low titre could be demonstrated. The elder brother responded well after a short time on ITI and has no spontaneous bleedings on regular prophylaxis although in a somewhat higher dose than expected. On the other hand, in spite of comparable regimens, the younger brother has so far been resistant to ITI. Moreover, during treatment with extremely high doses of factor IX concentrate he developed nephrotic syndrome which only slowly subsided after treatment with corticosteroids and withdrawal of factor IX.
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| 42. |
- Tengborn, Lilian, et al.
(författare)
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Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry.
- 2012
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Ingår i: BJOG: An International Journal of Obstetrics & Gynaecology. - : Wiley. - 1471-0528 .- 1470-0328. ; 119:12, s. 1529-1537
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Tidskriftsartikel (refereegranskat)abstract
- Objective: The European Acquired Haemophilia registry (EACH2) collected data on the demographics, diagnosis, underlying disorders, bleeding characteristics, treatment, and outcome of women with acquired haemophilia A (AHA), a rare and often severe bleeding disorder caused by autoantibodies directed against coagulation factor VIII. Design: Prospective, multi-centre, large-scale, pan-European registry. Setting A total of 117 haemophilia centres in 13 European countries. Population Pregnancy-associated AHA. Methods Data were reported using a web-based electronic case report form. Diagnosis was based on the presence of a prolonged activated partial thromboplastin time, reduced coagulation Factor VIII level and positive inhibitor assay. Main outcome measures: Presenting characteristics, time to diagnosis, haemostatic treatment and outcome, immunosuppressive treatment and outcome. Results The EACH2 registry (n = 501) documented 42 (8.4%) cases of AHA associated with the peripartum period, a median Factor VIII level at diagnosis of 2.5 (range 0-25) IU/dl and inhibitor titre of 7.8 (range 0.7-348) BU/ml. Antepartum inhibitors were evident in eight women. Time to diagnosis of AHA after delivery was 89 (range 21-120) days. First-line haemostatic treatment was successful in 20/23 (87%) women treated. Bleeding episodes resolved in 17/18 (94%) women treated with a bypassing agent and 29/39 (74%) women achieved complete remission with first-line immunosuppressive treatment. Two babies experienced postnatal bleeding, suggesting transplacental transfer of the antibody. All women were alive at last follow-up. Conclusions: Although rare, pregnancy-associated AHA may cause severe bleeding-related morbidity. Once diagnosed, women respond well to haemostatic treatment with bypassing agents and immunosuppression. Awareness of peripartum AHA requires improvement to facilitate rapid and appropriate management.
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| 43. |
- Vagianos, C, et al.
(författare)
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Intraoperative collection of shed blood with citrated compresses for autotransfusion. An experimental study in pigs
- 1990
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Ingår i: Acta Chirurgica Scandinavica. - 0001-5482. ; 156:2, s. 121-126
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Tidskriftsartikel (refereegranskat)abstract
- Six pigs were used to study whether the collection of shed blood by means of surgical compresses instead of suction traumatizes such blood. In an in vivo part of the study, the whole blood volume of the pigs was retransfused after treatment with citrated compresses, and in an in vitro part the blood was treated extensively with compresses and analyzed. All animals survived, with a minor fall in hemoglobin after 48 hours and a rise in serum citrate concentration at the end of the experiment. A study of the coagulation system revealed no important change after retransfusion of compress-treated blood. In the in vitro part of the study, repeatedly squeezing blood from the compresses increased the concentration of free hemoglobin to a maximum of 5 g/l after ten squeezes, whereas platelets were numerically unchanged. The study indicates that collecting shed blood by means of surgical compresses may be a safe and efficient method.
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