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Haemophilia Care in Europe: the ESCHQoL study

Schramm, W.; Gringeri, A.; Ljung, Rolf LU ; Berger, K.; Crispin, A.; Bullinger, M.; Giangrande, P. L. F.; Von Mackensen, S.; Mantovani, L. G. and Nemes, L., et al. (2012) In Haemophilia 18(5). p.729-737
Abstract
The aim of this study was to determine the clinical conditions of patients with haemophilia within Europe as recommended by the European Commission. In this multicentre, cross-sectional, ambispective study, conducted within 21 European countries patients' clinical data were collected, amongst others haemophilia type, severity, treatment pattern, use of factor products, bleeding, orthopaedic joint scores and infections. A total of 1400 patients, 84.3% with haemophilia A and 15.7% with haemophilia B were enrolled by 42 centres between 2004 and 2006. Thereof, 417 were children (30.0%) and 983 were adults (70.0%). About 70% of patients had severe factor deficiency (<1%). More than half of the adults were carriers of chronic infections... (More)
The aim of this study was to determine the clinical conditions of patients with haemophilia within Europe as recommended by the European Commission. In this multicentre, cross-sectional, ambispective study, conducted within 21 European countries patients' clinical data were collected, amongst others haemophilia type, severity, treatment pattern, use of factor products, bleeding, orthopaedic joint scores and infections. A total of 1400 patients, 84.3% with haemophilia A and 15.7% with haemophilia B were enrolled by 42 centres between 2004 and 2006. Thereof, 417 were children (30.0%) and 983 were adults (70.0%). About 70% of patients had severe factor deficiency (<1%). More than half of the adults were carriers of chronic infections (12.6% HIV, 55.8% HCV), compared to only 3.8% children (no HIV, 2.9% HCV). Patients were grouped according to per capita amount of clotting factor used in patients' region of residence in 2005: region 1: >5 IU; region 2: 25 IU; region 3: <2 IU. Paediatric and adult patients in region 3 had median numbers of three and eight joint bleeds, respectively, with worse joint scores compared to region 1 with zero and one bleed. Prophylactic therapy was used in only 31.3% children and 8.9% adults with severe haemophilia in region 3 compared to 93.7% and 54.1%, respectively, in region 1. Statistical analysis revealed that residence in areas with low factor consumption/availability is the most prominent risk factor for joint disease. Access of European patients with haemophilia to optimal care with safe factor VIII concentrates is limited and depends on the region of residence. (Less)
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organization
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Contribution to journal
publication status
published
subject
keywords
arthropathy, clinical outcomes, European countries, factor consumption, haemophilia, severity, treatment
in
Haemophilia
volume
18
issue
5
pages
729 - 737
publisher
Federation of European Neuroscience Societies and Blackwell Publishing Ltd
external identifiers
  • wos:000307900700030
  • scopus:84865560607
ISSN
1351-8216
DOI
10.1111/j.1365-2516.2012.02847.x
language
English
LU publication?
yes
id
3e61aefe-4c53-4ad4-9410-6bb559f8b822 (old id 3147692)
date added to LUP
2012-11-01 09:32:32
date last changed
2017-07-30 03:01:09
@article{3e61aefe-4c53-4ad4-9410-6bb559f8b822,
  abstract     = {The aim of this study was to determine the clinical conditions of patients with haemophilia within Europe as recommended by the European Commission. In this multicentre, cross-sectional, ambispective study, conducted within 21 European countries patients' clinical data were collected, amongst others haemophilia type, severity, treatment pattern, use of factor products, bleeding, orthopaedic joint scores and infections. A total of 1400 patients, 84.3% with haemophilia A and 15.7% with haemophilia B were enrolled by 42 centres between 2004 and 2006. Thereof, 417 were children (30.0%) and 983 were adults (70.0%). About 70% of patients had severe factor deficiency (&lt;1%). More than half of the adults were carriers of chronic infections (12.6% HIV, 55.8% HCV), compared to only 3.8% children (no HIV, 2.9% HCV). Patients were grouped according to per capita amount of clotting factor used in patients' region of residence in 2005: region 1: &gt;5 IU; region 2: 25 IU; region 3: &lt;2 IU. Paediatric and adult patients in region 3 had median numbers of three and eight joint bleeds, respectively, with worse joint scores compared to region 1 with zero and one bleed. Prophylactic therapy was used in only 31.3% children and 8.9% adults with severe haemophilia in region 3 compared to 93.7% and 54.1%, respectively, in region 1. Statistical analysis revealed that residence in areas with low factor consumption/availability is the most prominent risk factor for joint disease. Access of European patients with haemophilia to optimal care with safe factor VIII concentrates is limited and depends on the region of residence.},
  author       = {Schramm, W. and Gringeri, A. and Ljung, Rolf and Berger, K. and Crispin, A. and Bullinger, M. and Giangrande, P. L. F. and Von Mackensen, S. and Mantovani, L. G. and Nemes, L. and Serban, M.},
  issn         = {1351-8216},
  keyword      = {arthropathy,clinical outcomes,European countries,factor consumption,haemophilia,severity,treatment},
  language     = {eng},
  number       = {5},
  pages        = {729--737},
  publisher    = {Federation of European Neuroscience Societies and Blackwell Publishing Ltd},
  series       = {Haemophilia},
  title        = {Haemophilia Care in Europe: the ESCHQoL study},
  url          = {http://dx.doi.org/10.1111/j.1365-2516.2012.02847.x},
  volume       = {18},
  year         = {2012},
}