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Lifelong prophylaxis in a large cohort of adult patients with severe haemophilia: a beneficial effect on orthopaedic outcome and quality of life.

Khawaji, Mohammed LU ; Astermark, Jan LU and Berntorp, Erik LU (2012) In European Journal of Haematology 88(4). p.329-335
Abstract
Background: In the 1950s, Sweden initiated prophylaxis as a lifelong treatment for haemophilia. It was the first country to do so. Objective: To describe and evaluate dosing and outcome of prophylactic treatment in a large cohort of adult people with severe haemophilia who have been using prophylaxis most of their lives. Methods: Eighty-one patients born between 1932 and1992 were divided into two groups (Group A started prophylaxis at the age of ≤ 3 years; Group B at three or more years of age) and evaluated retrospectively. Outcome was evaluated using the Hemophilia Joint Health Score (HJHS) and SF-36, a measure of quality of life. Results: The median number of joint bleeds per year was 0 in both study groups; however, the annual number... (More)
Background: In the 1950s, Sweden initiated prophylaxis as a lifelong treatment for haemophilia. It was the first country to do so. Objective: To describe and evaluate dosing and outcome of prophylactic treatment in a large cohort of adult people with severe haemophilia who have been using prophylaxis most of their lives. Methods: Eighty-one patients born between 1932 and1992 were divided into two groups (Group A started prophylaxis at the age of ≤ 3 years; Group B at three or more years of age) and evaluated retrospectively. Outcome was evaluated using the Hemophilia Joint Health Score (HJHS) and SF-36, a measure of quality of life. Results: The median number of joint bleeds per year was 0 in both study groups; however, the annual number of joint bleeds during the final three years of observation was higher in group B than group A (p< 0.006). Twenty-five of 30 patients in group A and 27/51 patients in group B had no joint bleeds in that period. Group A had significantly better joint outcomes than group B. Patients in group A experienced better physical and social health than those in group B. Conclusions: This follow-up has provided for the first time more extensive and detailed information regarding the practice of prophylactic treatment in a large cohort of adults with severe haemophilia. The present study confirms, that early start of prophylaxis and continuing throughout the lifespan has been successful in virtually eliminating joint bleeds, preserving a close to normal joint status, and keeping patients healthy and able to live normal lives. © 2012 John Wiley & Sons A/S. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
European Journal of Haematology
volume
88
issue
4
pages
329 - 335
publisher
Wiley-Blackwell
external identifiers
  • wos:000301175300006
  • pmid:22221195
  • scopus:84858290843
ISSN
1600-0609
DOI
10.1111/j.1600-0609.2012.01750.x
language
English
LU publication?
yes
id
838aee0e-19c5-4ea2-8d36-981036c893d6 (old id 2336674)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/22221195?dopt=Abstract
date added to LUP
2012-02-01 19:44:20
date last changed
2017-10-22 04:53:17
@article{838aee0e-19c5-4ea2-8d36-981036c893d6,
  abstract     = {Background: In the 1950s, Sweden initiated prophylaxis as a lifelong treatment for haemophilia. It was the first country to do so. Objective: To describe and evaluate dosing and outcome of prophylactic treatment in a large cohort of adult people with severe haemophilia who have been using prophylaxis most of their lives. Methods: Eighty-one patients born between 1932 and1992 were divided into two groups (Group A started prophylaxis at the age of ≤ 3 years; Group B at three or more years of age) and evaluated retrospectively. Outcome was evaluated using the Hemophilia Joint Health Score (HJHS) and SF-36, a measure of quality of life. Results: The median number of joint bleeds per year was 0 in both study groups; however, the annual number of joint bleeds during the final three years of observation was higher in group B than group A (p&lt; 0.006). Twenty-five of 30 patients in group A and 27/51 patients in group B had no joint bleeds in that period. Group A had significantly better joint outcomes than group B. Patients in group A experienced better physical and social health than those in group B. Conclusions: This follow-up has provided for the first time more extensive and detailed information regarding the practice of prophylactic treatment in a large cohort of adults with severe haemophilia. The present study confirms, that early start of prophylaxis and continuing throughout the lifespan has been successful in virtually eliminating joint bleeds, preserving a close to normal joint status, and keeping patients healthy and able to live normal lives. © 2012 John Wiley &amp; Sons A/S.},
  author       = {Khawaji, Mohammed and Astermark, Jan and Berntorp, Erik},
  issn         = {1600-0609},
  language     = {eng},
  number       = {4},
  pages        = {329--335},
  publisher    = {Wiley-Blackwell},
  series       = {European Journal of Haematology},
  title        = {Lifelong prophylaxis in a large cohort of adult patients with severe haemophilia: a beneficial effect on orthopaedic outcome and quality of life.},
  url          = {http://dx.doi.org/10.1111/j.1600-0609.2012.01750.x},
  volume       = {88},
  year         = {2012},
}