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Prevention of arthropathy: may it be extrapolated from patients without inhibitors to patients with inhibitors?

Hedner, Ulla LU (2008) In Haemophilia 14(Suppl. 6). p.23-27
Abstract
Although no consensus on the exact dosing schedule of prophylaxis in non-inhibitor haemophilia patients has been achieved, regular administration of factor VIII (FVIII) or factor IX (FIX) concentrates is currently accepted as the most effective treatment model in order to prevent sick days, hospital stays and the development of arthropathy in patients with severe haemophilia. The basis for prophylaxis has been the idea to increase the plasma levels of FVIII or FIX above 1% thereby improving the haemostasis in severe haemophilia. Current treatment in haemophilia patients with inhibitors includes the use of activated prothrombin complex concentrates and recombinant FVIIa (rFVIIa). Regular administration of such agents has recently repeatedly... (More)
Although no consensus on the exact dosing schedule of prophylaxis in non-inhibitor haemophilia patients has been achieved, regular administration of factor VIII (FVIII) or factor IX (FIX) concentrates is currently accepted as the most effective treatment model in order to prevent sick days, hospital stays and the development of arthropathy in patients with severe haemophilia. The basis for prophylaxis has been the idea to increase the plasma levels of FVIII or FIX above 1% thereby improving the haemostasis in severe haemophilia. Current treatment in haemophilia patients with inhibitors includes the use of activated prothrombin complex concentrates and recombinant FVIIa (rFVIIa). Regular administration of such agents has recently repeatedly been reported to decrease the number of joint and muscle bleeds in these patients. Regarding rFVIIa, such an effect was confirmed in a controlled study published in 2007. Although the exact mechanism of action of rFVIIa in this setting is still not clear, it seems reasonable to assume that prophylaxis with rFVIIa will be able to prevent or minimize the development of haemophilic arthropathy. As arthropathy is a long-term complication in haemophilia and the result of repeated joint bleeds, a longer follow-up of patients on regular prophylaxis including measurements of the joint scores, as has been performed in FVIII/FIX prophylaxis in non-inhibitor patients, will be required for a definite conclusion. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
prophylaxis, haemophilia, inhibitors
in
Haemophilia
volume
14
issue
Suppl. 6
pages
23 - 27
publisher
Federation of European Neuroscience Societies and Blackwell Publishing Ltd
external identifiers
  • wos:000259624900006
  • pmid:19134030
  • scopus:53249134603
ISSN
1351-8216
DOI
10.1111/j.1365-2516.2008.01886.x
language
English
LU publication?
yes
id
1ac3b944-500d-4992-b00f-81c3daf9009a (old id 1287283)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/19134030?dopt=Abstract
date added to LUP
2009-01-22 12:24:12
date last changed
2017-01-01 04:41:49
@article{1ac3b944-500d-4992-b00f-81c3daf9009a,
  abstract     = {Although no consensus on the exact dosing schedule of prophylaxis in non-inhibitor haemophilia patients has been achieved, regular administration of factor VIII (FVIII) or factor IX (FIX) concentrates is currently accepted as the most effective treatment model in order to prevent sick days, hospital stays and the development of arthropathy in patients with severe haemophilia. The basis for prophylaxis has been the idea to increase the plasma levels of FVIII or FIX above 1% thereby improving the haemostasis in severe haemophilia. Current treatment in haemophilia patients with inhibitors includes the use of activated prothrombin complex concentrates and recombinant FVIIa (rFVIIa). Regular administration of such agents has recently repeatedly been reported to decrease the number of joint and muscle bleeds in these patients. Regarding rFVIIa, such an effect was confirmed in a controlled study published in 2007. Although the exact mechanism of action of rFVIIa in this setting is still not clear, it seems reasonable to assume that prophylaxis with rFVIIa will be able to prevent or minimize the development of haemophilic arthropathy. As arthropathy is a long-term complication in haemophilia and the result of repeated joint bleeds, a longer follow-up of patients on regular prophylaxis including measurements of the joint scores, as has been performed in FVIII/FIX prophylaxis in non-inhibitor patients, will be required for a definite conclusion.},
  author       = {Hedner, Ulla},
  issn         = {1351-8216},
  keyword      = {prophylaxis,haemophilia,inhibitors},
  language     = {eng},
  number       = {Suppl. 6},
  pages        = {23--27},
  publisher    = {Federation of European Neuroscience Societies and Blackwell Publishing Ltd},
  series       = {Haemophilia},
  title        = {Prevention of arthropathy: may it be extrapolated from patients without inhibitors to patients with inhibitors?},
  url          = {http://dx.doi.org/10.1111/j.1365-2516.2008.01886.x},
  volume       = {14},
  year         = {2008},
}