Incidence, mortality rates and causes of deaths in haemophilia patients in Sweden.
(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:
https://lup.lub.lu.se/record/3560275
- author
- Lövdahl, Susanna LU ; Henriksson, Marie LU ; Baghaei, F ; Holmström, Eva M LU ; Nilsson, J-Å ; Berntorp, Erik LU and Astermark, Jan LU
- organization
- publishing date
- 2013
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Haemophilia
- volume
- 19
- issue
- 3
- pages
- 362 - 369
- publisher
- Wiley-Blackwell
- 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
- 2016-04-01 10:44:18
- date last changed
- 2022-07-14 17:26:24
@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 < 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.}},
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 = {{Wiley-Blackwell}},
series = {{Haemophilia}},
title = {{Incidence, mortality rates and causes of deaths in haemophilia patients in Sweden.}},
url = {{http://dx.doi.org/10.1111/hae.12092}},
doi = {{10.1111/hae.12092}},
volume = {{19}},
year = {{2013}},
}