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Joint protection in haemophilia

Rodriguez-Merchan, E. C.; Jimenez-Yuste, V.; Aznar, J. A.; Hedner, Ulla LU ; Knobe, Karin LU ; Lee, C. A.; Ljung, Rolf LU ; Querol, F.; Santagostino, E. and Valentino, L. A., et al. (2011) In Haemophilia 17. p.1-23
Abstract
Haemarthroses (intra-articular haemorrhages) are a frequent finding typically observed in patients with haemophilia. Diagnosis and treatment of these bleeding episodes must be delivered as early as possible. Additionally, treatment should ideally be administered intensively (enhanced on-demand treatment) until the resolution of symptoms. Joint aspiration plays an important role in acute and profuse haemarthroses as the presence of blood in the joint leads to chondrocyte apoptosis and chronic synovitis, which will eventually result in joint degeneration (haemophilic arthropathy). Ultrasonography (US) is an appropriate diagnostic technique to assess the evolution of acute haemarthrosis in haemophilia, although magnetic resonance imaging... (More)
Haemarthroses (intra-articular haemorrhages) are a frequent finding typically observed in patients with haemophilia. Diagnosis and treatment of these bleeding episodes must be delivered as early as possible. Additionally, treatment should ideally be administered intensively (enhanced on-demand treatment) until the resolution of symptoms. Joint aspiration plays an important role in acute and profuse haemarthroses as the presence of blood in the joint leads to chondrocyte apoptosis and chronic synovitis, which will eventually result in joint degeneration (haemophilic arthropathy). Ultrasonography (US) is an appropriate diagnostic technique to assess the evolution of acute haemarthrosis in haemophilia, although magnetic resonance imaging remains the gold standard as far as imaging techniques are concerned. Some patients experience subclinical haemarthroses, which eventually tend to result in some degree of arthropathy, especially in the ankles. Nowadays, the most effective way of protecting these patients is primary prophylaxis, which in practice changes severe haemophilia into moderate haemophilia, preventing or at least minimizing the occurrence of haemarthrosis. If primary prophylaxis is, for whatever reason not an option, secondary prophylaxis and enhanced on demand treatment should be considered. Two alternatives are available for inhibitor patients: (i) control of haemostasis using by-passing agents (rFVIIa or aPCCs) either as enhanced on demand treatment or secondary prophylaxis, as appropriate, following the same basic principles used for non-inhibitor patients and (ii) immune tolerance induction (ITI) to eradicate the inhibitor. (Less)
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type
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publication status
published
subject
keywords
haemarthrosis, haemophilia, inhibitor, joint, prophylaxis, protection
in
Haemophilia
volume
17
pages
1 - 23
publisher
Federation of European Neuroscience Societies and Blackwell Publishing Ltd
external identifiers
  • wos:000296466200001
  • scopus:79961176998
ISSN
1351-8216
DOI
10.1111/j.1365-2516.2011.02615.x
language
English
LU publication?
yes
id
4501e6e5-2dbb-4fe1-9a10-2ff810503feb (old id 2254464)
date added to LUP
2012-01-02 08:06:04
date last changed
2017-11-05 03:19:13
@article{4501e6e5-2dbb-4fe1-9a10-2ff810503feb,
  abstract     = {Haemarthroses (intra-articular haemorrhages) are a frequent finding typically observed in patients with haemophilia. Diagnosis and treatment of these bleeding episodes must be delivered as early as possible. Additionally, treatment should ideally be administered intensively (enhanced on-demand treatment) until the resolution of symptoms. Joint aspiration plays an important role in acute and profuse haemarthroses as the presence of blood in the joint leads to chondrocyte apoptosis and chronic synovitis, which will eventually result in joint degeneration (haemophilic arthropathy). Ultrasonography (US) is an appropriate diagnostic technique to assess the evolution of acute haemarthrosis in haemophilia, although magnetic resonance imaging remains the gold standard as far as imaging techniques are concerned. Some patients experience subclinical haemarthroses, which eventually tend to result in some degree of arthropathy, especially in the ankles. Nowadays, the most effective way of protecting these patients is primary prophylaxis, which in practice changes severe haemophilia into moderate haemophilia, preventing or at least minimizing the occurrence of haemarthrosis. If primary prophylaxis is, for whatever reason not an option, secondary prophylaxis and enhanced on demand treatment should be considered. Two alternatives are available for inhibitor patients: (i) control of haemostasis using by-passing agents (rFVIIa or aPCCs) either as enhanced on demand treatment or secondary prophylaxis, as appropriate, following the same basic principles used for non-inhibitor patients and (ii) immune tolerance induction (ITI) to eradicate the inhibitor.},
  author       = {Rodriguez-Merchan, E. C. and Jimenez-Yuste, V. and Aznar, J. A. and Hedner, Ulla and Knobe, Karin and Lee, C. A. and Ljung, Rolf and Querol, F. and Santagostino, E. and Valentino, L. A. and Caffarini, A.},
  issn         = {1351-8216},
  keyword      = {haemarthrosis,haemophilia,inhibitor,joint,prophylaxis,protection},
  language     = {eng},
  pages        = {1--23},
  publisher    = {Federation of European Neuroscience Societies and Blackwell Publishing Ltd},
  series       = {Haemophilia},
  title        = {Joint protection in haemophilia},
  url          = {http://dx.doi.org/10.1111/j.1365-2516.2011.02615.x},
  volume       = {17},
  year         = {2011},
}