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Incidence, mortality rates and causes of deaths in haemophilia patients in Sweden.

Lövdahl, Susanna LU ; Henriksson, Marie LU ; Baghaei, F; Holmström, Eva M LU ; Nilsson, J-Å; Berntorp, Erik LU and Astermark, Jan LU (2013) In Haemophilia 19(3). p.362-369
Abstract
Sweden has been a pioneer in the treatment of haemophilia, with the first concentrate available in the 1950s. Treatment has improved over the years to its current state-of-the art. The aim of the current study was to evaluate the long-term outcome of haemophilia in terms of incidence, morbidity and mortality. Patients diagnosed with haemophilia A or B registered at the national haemophilia centres and/or the Patient Registry and born before 2009 and alive in 1968 were enrolled and linked to the Cause of Death-, Migration- and Medical Birth registries. Five age- and sex-matched controls were selected for each patient. A total of 1431 patients with haemophilia A or B were compared with 7150 controls. The 3-year moving average incidence rate... (More)
Sweden has been a pioneer in the treatment of haemophilia, with the first concentrate available in the 1950s. Treatment has improved over the years to its current state-of-the art. The aim of the current study was to evaluate the long-term outcome of haemophilia in terms of incidence, morbidity and mortality. Patients diagnosed with haemophilia A or B registered at the national haemophilia centres and/or the Patient Registry and born before 2009 and alive in 1968 were enrolled and linked to the Cause of Death-, Migration- and Medical Birth registries. Five age- and sex-matched controls were selected for each patient. A total of 1431 patients with haemophilia A or B were compared with 7150 controls. The 3-year moving average incidence rate per 100 000 population varied between 21 and 36. The hazard ratio for all-cause mortality compared with controls was 2.2, 95% CI: [1.8; 2.7], P < 0.001 for the entire group of patients and 1.7, 95% CI: [1.3; 2.2], P < 0.001 when patients with HIV and/or viral hepatitis were excluded. The corresponding figures for the severe haemophilia subgroup were 6.6, 95% CI: [4.5; 10.0], P < 0.001 and 8.2, 95% CI [3.2; 20.8], P < 0.001 respectively. The most common causes of death were related to malignancies and the haemostatic defect. People with haemophilia were 57% less likely to die from ischaemic heart disease than controls. People with haemophilia in Sweden demonstrate higher mortality over time, independent of HIV and viral hepatitis, despite relatively advantageous access to clotting factor concentrates. (Less)
Please use this url to cite or link to this publication:
author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Haemophilia
volume
19
issue
3
pages
362 - 369
publisher
Federation of European Neuroscience Societies and Blackwell Publishing Ltd
external identifiers
  • wos:000318078400025
  • pmid:23374117
  • scopus:84876811692
ISSN
1351-8216
DOI
10.1111/hae.12092
language
English
LU publication?
yes
id
78282995-292d-4389-8526-25fb7b8c59b1 (old id 3560275)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/23374117?dopt=Abstract
date added to LUP
2013-03-01 12:10:51
date last changed
2019-05-19 03:16:07
@article{78282995-292d-4389-8526-25fb7b8c59b1,
  abstract     = {Sweden has been a pioneer in the treatment of haemophilia, with the first concentrate available in the 1950s. Treatment has improved over the years to its current state-of-the art. The aim of the current study was to evaluate the long-term outcome of haemophilia in terms of incidence, morbidity and mortality. Patients diagnosed with haemophilia A or B registered at the national haemophilia centres and/or the Patient Registry and born before 2009 and alive in 1968 were enrolled and linked to the Cause of Death-, Migration- and Medical Birth registries. Five age- and sex-matched controls were selected for each patient. A total of 1431 patients with haemophilia A or B were compared with 7150 controls. The 3-year moving average incidence rate per 100 000 population varied between 21 and 36. The hazard ratio for all-cause mortality compared with controls was 2.2, 95% CI: [1.8; 2.7], P &lt; 0.001 for the entire group of patients and 1.7, 95% CI: [1.3; 2.2], P &lt; 0.001 when patients with HIV and/or viral hepatitis were excluded. The corresponding figures for the severe haemophilia subgroup were 6.6, 95% CI: [4.5; 10.0], P &lt; 0.001 and 8.2, 95% CI [3.2; 20.8], P &lt; 0.001 respectively. The most common causes of death were related to malignancies and the haemostatic defect. People with haemophilia were 57% less likely to die from ischaemic heart disease than controls. People with haemophilia in Sweden demonstrate higher mortality over time, independent of HIV and viral hepatitis, despite relatively advantageous access to clotting factor concentrates.},
  author       = {Lövdahl, Susanna and Henriksson, Marie and Baghaei, F and Holmström, Eva M and Nilsson, J-Å and Berntorp, Erik and Astermark, Jan},
  issn         = {1351-8216},
  language     = {eng},
  number       = {3},
  pages        = {362--369},
  publisher    = {Federation of European Neuroscience Societies and Blackwell Publishing Ltd},
  series       = {Haemophilia},
  title        = {Incidence, mortality rates and causes of deaths in haemophilia patients in Sweden.},
  url          = {http://dx.doi.org/10.1111/hae.12092},
  volume       = {19},
  year         = {2013},
}