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Similar bleeding phenotype in young children with haemophilia A or B : A cohort study

Clausen, Niels; Petrini, P.; Claeyssens-Donadel, S.; Gouw, S. C.; Liesner, R.; Altisent, C.; Auerswald, G.; Carcao, M.; Chalmers, E. and Chambost, H., et al. (2014) In Haemophilia 20(6). p.747-755
Abstract

The bleeding phenotype has been suggested to differ between haemophilia A and B. More knowledge on the bleeding phenotype at initiation of treatment is important to optimize patient care. The aim of this study was to investigate the severity of the bleeding phenotype and the variation in bleeding in children with severe or moderate haemophilia A and B. Consecutive, previously untreated patients with severe or moderate haemophilia A and B (factor VIII or IX activity <0.01 or 0.01-0.05 IU mL-1 respectively) born between January 1st 2000 and January 1st 2010 were included. Primary outcome was severity of bleeding tendency. Secondary outcome was variation in bleeding pattern. A total of 582 patients with severe haemophilia A... (More)

The bleeding phenotype has been suggested to differ between haemophilia A and B. More knowledge on the bleeding phenotype at initiation of treatment is important to optimize patient care. The aim of this study was to investigate the severity of the bleeding phenotype and the variation in bleeding in children with severe or moderate haemophilia A and B. Consecutive, previously untreated patients with severe or moderate haemophilia A and B (factor VIII or IX activity <0.01 or 0.01-0.05 IU mL-1 respectively) born between January 1st 2000 and January 1st 2010 were included. Primary outcome was severity of bleeding tendency. Secondary outcome was variation in bleeding pattern. A total of 582 patients with severe haemophilia A and 76 with severe haemophilia B did not differ in age at first exposure to clotting factor (0.81 vs. 0.88 years, P = 0.20), age at first bleed (0.82 vs. 0.88 years, P = 0.36), and age at first joint bleed (1.18 vs. 1.20 years, P = 0.59). Patients with moderate haemophilia were older compared to patients with severe haemophilia. In patients with moderate haemophilia there were no clear differences between haemophilia A and B. Severity and variation in bleeding phenotype are similar during the early stage of treatment in patients with severe and moderate haemophilia A and B respectively. The findings imply that children with haemophilia B should be observed and treated as vigilantly as those with haemophilia A.

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published
subject
keywords
Bleeding phenotype, Children, Haemophilia A, Haemophilia B, Haemorrhage
in
Haemophilia
volume
20
issue
6
pages
9 pages
publisher
Federation of European Neuroscience Societies and Blackwell Publishing Ltd
external identifiers
  • scopus:84926151321
ISSN
1351-8216
DOI
10.1111/hae.12470
language
English
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yes
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69af4c17-8b01-427d-8108-4f8ec634f673
date added to LUP
2016-11-25 15:33:11
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2017-09-03 05:15:32
@article{69af4c17-8b01-427d-8108-4f8ec634f673,
  abstract     = {<p>The bleeding phenotype has been suggested to differ between haemophilia A and B. More knowledge on the bleeding phenotype at initiation of treatment is important to optimize patient care. The aim of this study was to investigate the severity of the bleeding phenotype and the variation in bleeding in children with severe or moderate haemophilia A and B. Consecutive, previously untreated patients with severe or moderate haemophilia A and B (factor VIII or IX activity &lt;0.01 or 0.01-0.05 IU mL<sup>-1</sup> respectively) born between January 1st 2000 and January 1st 2010 were included. Primary outcome was severity of bleeding tendency. Secondary outcome was variation in bleeding pattern. A total of 582 patients with severe haemophilia A and 76 with severe haemophilia B did not differ in age at first exposure to clotting factor (0.81 vs. 0.88 years, P = 0.20), age at first bleed (0.82 vs. 0.88 years, P = 0.36), and age at first joint bleed (1.18 vs. 1.20 years, P = 0.59). Patients with moderate haemophilia were older compared to patients with severe haemophilia. In patients with moderate haemophilia there were no clear differences between haemophilia A and B. Severity and variation in bleeding phenotype are similar during the early stage of treatment in patients with severe and moderate haemophilia A and B respectively. The findings imply that children with haemophilia B should be observed and treated as vigilantly as those with haemophilia A.</p>},
  author       = {Clausen, Niels and Petrini, P. and Claeyssens-Donadel, S. and Gouw, S. C. and Liesner, R. and Altisent, C. and Auerswald, G. and Carcao, M. and Chalmers, E. and Chambost, H. and Cid, A. and Fischer, K. and van Geet, Ch and Peerlinck, K. and Kenet, G. and Kobelt, R. and Kreuz, W. and Escuriola, C. and Goethe, J. W. and Kurnik, K. and Ljung, R. and Mäkipernaa, A. and Molinari, A. and Muntean, W. and Nolan, B. and Oldenburg, J. and Pérez Garrido, R. and Platokouki, H. and Rafowicz, A. and Rivard, G. and Santagostino, E. and Mancuso, M. E. and Williams, M. and van der Bom, J. G. and van den Berg, H. M. and , },
  issn         = {1351-8216},
  keyword      = {Bleeding phenotype,Children,Haemophilia A,Haemophilia B,Haemorrhage},
  language     = {eng},
  month        = {11},
  number       = {6},
  pages        = {747--755},
  publisher    = {Federation of European Neuroscience Societies and Blackwell Publishing Ltd},
  series       = {Haemophilia},
  title        = {Similar bleeding phenotype in young children with haemophilia A or B : A cohort study},
  url          = {http://dx.doi.org/10.1111/hae.12470},
  volume       = {20},
  year         = {2014},
}