| 1. |
|
|
| 2. |
|
|
| 3. |
- Ljung, Rolf, et al.
(författare)
-
Care of the Child with Hemophilia
- 2010
-
Ingår i: Textbook of Hemophilia, 2nd Edition. - Oxford, UK : Wiley-Blackwell. - 9781405169141 ; , s. 127-132
-
Bokkapitel (refereegranskat)
|
|
| 4. |
|
|
| 5. |
- Astermark, J., et al.
(författare)
-
Symposium in memory of Professor Inga Marie Nilsson
- 2001
-
Ingår i: Haemophilia. - : Wiley. - 1351-8216. ; 7:4, s. 401-410
-
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.
|
|
| 6. |
- Berntorp, E., et al.
(författare)
-
European retrospective study of real-life haemophilia treatment
- 2017
-
Ingår i: Haemophilia. - : Wiley. - 1351-8216. ; 23:1, s. 105-114
-
Tidskriftsartikel (refereegranskat)abstract
- Introduction: Haemophilia treatment varies significantly between individuals, countries and regions and details of bleed rates, factor consumption and injection frequency are often not available. Aim: To provide an overview of the FVIII/FIX treatment practice and outcome for patients with haemophilia A (HA) or haemophilia B (HB) across Europe. Methods: Non-interventional, 12-month retrospective study where anonymized data were retrieved from haemophilia centres/registers in Belgium, France, Germany, Italy, Spain, Sweden and the United Kingdom. Male patients (all ages) receiving coagulation factor treatment 24 months prior to the study, with basal FVIII/FIX levels ≤5 IU dL-1, without inhibitors, were included. Data were summarized descriptively. Results: In total, 1346 patients with HA and 312 with HB were included in the analysis; 75% and 57% had severe disease (FVIII/FIX < 1 IU dL-1) respectively. Prophylaxis was most common for severe haemophilia, especially for children, whereas on-demand treatment was more common for moderate haemophilia in most countries. The mean (SD) prescribed prophylactic treatment ranged from 67.9 (30.4) to 108.4 (78.1) (HA) and 32.3 (10.2) to 97.7 (32.1) (HB) IU kg-1 per week, across countries. Most patients on prophylaxis were treated ≥3 times/week (HA) or two times/week (HB). The median annual bleeding rate (ABR) for patients on prophylaxis ranged from 1.0 to 4.0 for severe HA, and from 1.0 to 6.0 for severe HB, while those with moderate haemophilia generally had slightly higher ABRs. Median ABRs for on-demand-treated severe HA ranged from 4.5 to 18.0, and for HB, 1.5 to 14.0. Conclusion: Treatment practice varied greatly between centres and countries and patients treated on-demand and prophylactically both experienced bleeds, emphasizing the need for further optimization of care.
|
|
| 7. |
- Berntorp, E., et al.
(författare)
-
Fifth Åland Island conference on von Willebrand disease
- 2018
-
Ingår i: Haemophilia. - : Wiley. - 1351-8216. ; 2424 Suppl 4, s. 5-19
-
Tidskriftsartikel (refereegranskat)abstract
- The fifth Åland Island meeting on von Willebrand disease (VWD) was held on the Åland Islands, Finland, from 22 to 24 September 2016—90 years after the first case of VWD was diagnosed in a patient from the Åland Islands in 1926. This meeting brought together experts in the field of VWD to share knowledge and expertise on current trends and challenges in VWD. Topics included the storage and release of von Willebrand factor (VWF), epidemiology and diagnostics in VWD, treatment of VWD, angiogenesis and VWF inhibitors.
|
|
| 8. |
- Berntorp, E.
(författare)
-
Haemophilia treatment in 2030
- 2016
-
Ingår i: Haemophilia. - : Wiley. - 1351-8216. ; 22:S5, s. 15-19
-
Tidskriftsartikel (refereegranskat)abstract
- Introduction: Looking into the future is difficult and sometimes hazardous. A reliable look into haemophilia treatment in 2030 should be based on history and contemporary progress as well as dilemmas. Today, the issue of inhibitors overshadows the entire haemophilia community together with lack of treatment for large parts of the world's persons with haemophilia. Aims: The aim of this paper was to provide a perspective on haemophilia treatment in 2030 and its provenance. Methods: Literature review on history, treatment of haemophilia today as well as of emerging therapies give a base for the author's opinion on haemophilia treatment in 2030. Results: Development of haemophilia treatment has virtually exploded during the last decade and a number of new clotting factor concentrates and alternative approaches are in the pipeline. Conclusion: The collection of treatment resources that we can see on the horizon gives hope that each person with haemophilia will get the care needed in 2030. The products used will be directed by individual needs and tailored to regional and local situations.
|
|
| 9. |
|
|
| 10. |
- Brackmann, H. H., et al.
(författare)
-
Immune tolerance induction : What have we learned over time?
- 2018
-
Ingår i: Haemophilia. - : Wiley. - 1351-8216. ; 24, s. 3-14
-
Forskningsöversikt (refereegranskat)abstract
- Development of inhibitory antibodies to infused factor VIII (FVIII) concentrates continues to be the most serious complication of haemophilia A management. Induction of immune tolerance by administering high doses of FVIII concentrate (antigen) and prothrombin complex concentrates to control bleeding was originated in the 1970s in Bonn, Germany, by Dr Hans-Hermann Brackmann, and became known as the Bonn protocol. ITI transformed the life of the index patient, who was 19 years of age when he began treatment, and dramatically improved the medical landscape for all patients with haemophilia and inhibitors. Over the past 40 years, variations to the Bonn protocol have been proposed. All protocols are effective although some are better suited than others for use in certain situations. The specific molecular defect in FVIII and the human leucocyte antigen (HLA) type of an individual with haemophilia are major codependent determinants to inhibitor development. Given the range of potential molecular defects and the staggering number of potential HLA types, it is likely that treatment arms of randomized studies in haemophilia represent highly diverse populations, which reduces the power of a study to demonstrate differences between treatments. Although available clinical guidelines and consensus recommendations for ITI therapy are not always in complete agreement, collectively the guidelines provide a reasonable level of guidance for administering ITI therapy under different clinical scenarios. Several studies of ITI therapy are ongoing with the aim of clarifying unresolved issues in haemophilia management including the role of von Willebrand factor in inhibitor eradication.
|
|
