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Primary prophylaxis in severe haemophilia should be started at an early age but can be individualized

Astermark, Jan LU ; Petrini, P; Tengborn, L; Schulman, S; Ljung, Rolf LU and Berntorp, Erik LU (1999) In British Journal of Haematology 105(4). p.1109-1113
Abstract
The frequency of joint bleeds and orthopaedic joint scores were evaluated in 121 patients with severe haemophilia who had started prophylactic treatment with clotting factor concentrates at least once weekly before the age of 10. 75 of the patients started before the age of 3, 31 at the age of 3-5 and 15 at the age of 6-9. Each subgroup was evaluated separately. In addition, a regimen of one infusion weekly was compared with that of two (haemophilia B) or three (haemophilia A) infusions weekly in each patient. A significant decrease in the overall number of joint bleeds per year was found after shortening the infusion interval (P<0.005), but the individual bleeding pattern varied. In survival analysis of the first pathologic joint score... (More)
The frequency of joint bleeds and orthopaedic joint scores were evaluated in 121 patients with severe haemophilia who had started prophylactic treatment with clotting factor concentrates at least once weekly before the age of 10. 75 of the patients started before the age of 3, 31 at the age of 3-5 and 15 at the age of 6-9. Each subgroup was evaluated separately. In addition, a regimen of one infusion weekly was compared with that of two (haemophilia B) or three (haemophilia A) infusions weekly in each patient. A significant decrease in the overall number of joint bleeds per year was found after shortening the infusion interval (P<0.005), but the individual bleeding pattern varied. In survival analysis of the first pathologic joint score event, those who started prophylaxis before the age of 3 had a better outcome overall than those starting at later ages (P=0.001). However, in subgroup analysis, no significant difference was seen in the annual number of joint bleeds and the development of arthropathy between those starting with, or shifting to, the more intensive regimen before the age of 3 and those that were put on this regimen at the age of 3-5. Age at start of prophylaxis was found to be an independent predictor for the development of arthropathy (P=0.0002), whereas dose and infusion interval at start were not. Our data emphasize the importance of starting replacement therapy during the first years of life. However, it seems that when beginning the regimen it can be individualized and adjusted according to the bleeding pattern. In this way, the need for a venous access system may be assessed on an individual basis. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Port-A-Cath, arthropathy, age, prophylaxis, haemophilia
in
British Journal of Haematology
volume
105
issue
4
pages
1109 - 1113
publisher
Federation of European Neuroscience Societies and Blackwell Publishing Ltd
external identifiers
  • pmid:10554828
  • scopus:0033028402
ISSN
0007-1048
DOI
10.1046/j.1365-2141.1999.01463.x
language
English
LU publication?
yes
id
1f128ce0-db21-434d-a6cb-220687f470a0 (old id 1115031)
date added to LUP
2008-07-07 12:41:05
date last changed
2017-11-19 03:25:04
@article{1f128ce0-db21-434d-a6cb-220687f470a0,
  abstract     = {The frequency of joint bleeds and orthopaedic joint scores were evaluated in 121 patients with severe haemophilia who had started prophylactic treatment with clotting factor concentrates at least once weekly before the age of 10. 75 of the patients started before the age of 3, 31 at the age of 3-5 and 15 at the age of 6-9. Each subgroup was evaluated separately. In addition, a regimen of one infusion weekly was compared with that of two (haemophilia B) or three (haemophilia A) infusions weekly in each patient. A significant decrease in the overall number of joint bleeds per year was found after shortening the infusion interval (P&lt;0.005), but the individual bleeding pattern varied. In survival analysis of the first pathologic joint score event, those who started prophylaxis before the age of 3 had a better outcome overall than those starting at later ages (P=0.001). However, in subgroup analysis, no significant difference was seen in the annual number of joint bleeds and the development of arthropathy between those starting with, or shifting to, the more intensive regimen before the age of 3 and those that were put on this regimen at the age of 3-5. Age at start of prophylaxis was found to be an independent predictor for the development of arthropathy (P=0.0002), whereas dose and infusion interval at start were not. Our data emphasize the importance of starting replacement therapy during the first years of life. However, it seems that when beginning the regimen it can be individualized and adjusted according to the bleeding pattern. In this way, the need for a venous access system may be assessed on an individual basis.},
  author       = {Astermark, Jan and Petrini, P and Tengborn, L and Schulman, S and Ljung, Rolf and Berntorp, Erik},
  issn         = {0007-1048},
  keyword      = {Port-A-Cath,arthropathy,age,prophylaxis,haemophilia},
  language     = {eng},
  number       = {4},
  pages        = {1109--1113},
  publisher    = {Federation of European Neuroscience Societies and Blackwell Publishing Ltd},
  series       = {British Journal of Haematology},
  title        = {Primary prophylaxis in severe haemophilia should be started at an early age but can be individualized},
  url          = {http://dx.doi.org/10.1046/j.1365-2141.1999.01463.x},
  volume       = {105},
  year         = {1999},
}