| 1. |
- Astermark, Jan, et al.
(författare)
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Non-genetic risk factors and the development of inhibitors in haemophilia: a comprehensive review and consensus report.
- 2010
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Ingår i: Haemophilia. - : Wiley. - 1351-8216. ; May 4, s. 747-766
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Tidskriftsartikel (refereegranskat)abstract
- Summary. The development of inhibitors to the infused factor in patients with haemophilia is a serious clinical problem. Recent evidence suggests that alongside the strong genetic contribution to inhibitor formation, there are a number of non-genetic factors - perceived by the immune system as danger signals - which promote formation of inhibitors. This study provides a comprehensive review of clinical studies relating to these factors and also presents a survey of opinion concerning their importance and clinical influence, conducted among the members of the European Haemophilia Treatment Standardisation Board (EHTSB). Taken together, this information highlights the lack of robust data concerning the influence of several non-genetic risk factors on inhibitor development, and an urgent need for prospective, well-conducted studies that adhere to recommendations made by the European Medicines Agency (EMEA) for studying inhibitors. Based on current literature, the EHTSB formulated consensus recommendations. It is desirable to minimize intensive treatment wherever possible, given the clinical situation. Prophylaxis should be offered to all children, although we still need to determine optimal dosing with respect to inhibitor development, and age for starting treatment. Vaccinations should be given subcutaneously and concomitant factor concentrate infusions avoided. According to the board, there is no evidence in the literature supporting suggestions that the type of concentrate influences inhibitor risk; but all patients should be monitored during their first exposures. Furthermore, there is no evidence to support an association between pregnancy-related issues, breast feeding and treatment-related factors (e.g. route of administration, or use of blood components) and inhibitor development.
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| 2. |
- DE Moerloose, P, et al.
(författare)
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Recommendations for assessment, monitoring and follow-up of patients with haemophilia.
- 2011
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Ingår i: Haemophilia. - : Wiley. - 1351-8216.
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Tidskriftsartikel (refereegranskat)abstract
- Summary. Over the last few decades, clinical follow-up of patients with haemophilia has become more complex as a result of the introduction of new treatment strategies, the presence of comorbidities related to haemophilia or ageing, as well as the emergence of new tools to evaluate the medical and social consequences of haemophilia. This publication describes the parameters and information that should be documented and the tests, examinations and interventions required for optimal follow-up of a patient with haemophilia. In the absence of formal studies, the present recommendations have been established as result of a series of consensus meetings in the frame of the European Haemophilia Therapy Standardization Board (EHTSB). The following 11 domains were identified: Baseline information, Current status, Treatment, Inhibitor status, Bleeding, Joint status and pain, Comorbidities, Dental care, Physical activities, Social participation and Quality of life. For each domain, details are proposed for the relevant parameters to be captured and monitored as well as the relevant tools that facilitate data collection. Adopting these recommendations should help the individual care of patients and, even though this is not the primary objective of this article, it should also help at national and international level to shape a new approach to haemophilia by working towards a more standardized outcome assessment. Greater standardization should have implications for data collection, improvements in treatment evaluation and optimizing resources.
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| 3. |
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| 4. |
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| 5. |
- Mariani, G, et al.
(författare)
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Thrombosis in inherited factor VII deficiency
- 2003
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Ingår i: Journal of thrombosis and haemostasis : JTH. - : Elsevier BV. - 1538-7933 .- 1538-7836. ; 1:10, s. 2153-2158
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Tidskriftsartikel (refereegranskat)
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