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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)
Please use this url to cite or link to this publication:
author
; and
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
  • pmid:22221195
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
2016-04-04 08:01:24
date last changed
2022-02-20 21:13:05
@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}},
  doi          = {{10.1111/j.1600-0609.2012.01750.x}},
  volume       = {{88}},
  year         = {{2012}},
}