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Various regimens for prophylactic treatment of patients with haemophilia

Schwarz, Rudolf; Ljung, Rolf LU and Tedgård, Ulf LU (2015) In European Journal of Haematology 94. p.11-16
Abstract
Haemophilia prophylaxis is superior to on-demand treatment to prevent joint damage. High-dose prophylaxis' as used in Sweden is more effective in preventing arthropathy than an intermediate-dose regimen' (the Netherlands) and the Canadian tailored primary prophylaxis. Prophylaxis may reduce the risk of developing inhibitors. There is no difference in inhibitor risk between plasma derived and recombinant factor VIII (rFVIII) products but the Rodin study showed increased risk with second-generation rFVIII products. MRI is a new and very sensitive tool to detect the symptoms of early arthropathy but some results (soft tissue changes in bleed-free joints') still need to be investigated. Ultrasound is a very helpful method to aid diagnosis... (More)
Haemophilia prophylaxis is superior to on-demand treatment to prevent joint damage. High-dose prophylaxis' as used in Sweden is more effective in preventing arthropathy than an intermediate-dose regimen' (the Netherlands) and the Canadian tailored primary prophylaxis. Prophylaxis may reduce the risk of developing inhibitors. There is no difference in inhibitor risk between plasma derived and recombinant factor VIII (rFVIII) products but the Rodin study showed increased risk with second-generation rFVIII products. MRI is a new and very sensitive tool to detect the symptoms of early arthropathy but some results (soft tissue changes in bleed-free joints') still need to be investigated. Ultrasound is a very helpful method to aid diagnosis especially during the acute phase of a bleed. The risk of infection with central venous access remains a matter of debate. A fully implanted central venous access device (CVAD) has a significant lower risk of infection compared to external CVADs. Patient's age under 6yr and inhibitor presence are additional risk factors for infections. The role of arteriovenous fistulae needs to be investigated because significant complications have been reported. Disease-specific quality of life instruments are complementary to generic instruments evaluating QoL in patients with haemophilia and have become important health outcome measures. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
prophylaxis, children, haemophilia, treatment, inhibitors
in
European Journal of Haematology
volume
94
pages
11 - 16
publisher
Wiley-Blackwell
external identifiers
  • wos:000347371100004
  • scopus:84920265049
ISSN
1600-0609
DOI
10.1111/ejh.12496
language
English
LU publication?
yes
id
f8b97399-b131-4433-ad52-c33165614d92 (old id 5075990)
date added to LUP
2015-03-02 07:08:21
date last changed
2017-01-01 04:12:18
@article{f8b97399-b131-4433-ad52-c33165614d92,
  abstract     = {Haemophilia prophylaxis is superior to on-demand treatment to prevent joint damage. High-dose prophylaxis' as used in Sweden is more effective in preventing arthropathy than an intermediate-dose regimen' (the Netherlands) and the Canadian tailored primary prophylaxis. Prophylaxis may reduce the risk of developing inhibitors. There is no difference in inhibitor risk between plasma derived and recombinant factor VIII (rFVIII) products but the Rodin study showed increased risk with second-generation rFVIII products. MRI is a new and very sensitive tool to detect the symptoms of early arthropathy but some results (soft tissue changes in bleed-free joints') still need to be investigated. Ultrasound is a very helpful method to aid diagnosis especially during the acute phase of a bleed. The risk of infection with central venous access remains a matter of debate. A fully implanted central venous access device (CVAD) has a significant lower risk of infection compared to external CVADs. Patient's age under 6yr and inhibitor presence are additional risk factors for infections. The role of arteriovenous fistulae needs to be investigated because significant complications have been reported. Disease-specific quality of life instruments are complementary to generic instruments evaluating QoL in patients with haemophilia and have become important health outcome measures.},
  author       = {Schwarz, Rudolf and Ljung, Rolf and Tedgård, Ulf},
  issn         = {1600-0609},
  keyword      = {prophylaxis,children,haemophilia,treatment,inhibitors},
  language     = {eng},
  pages        = {11--16},
  publisher    = {Wiley-Blackwell},
  series       = {European Journal of Haematology},
  title        = {Various regimens for prophylactic treatment of patients with haemophilia},
  url          = {http://dx.doi.org/10.1111/ejh.12496},
  volume       = {94},
  year         = {2015},
}