PMID- 14962219
OWN - NLM
STAT- MEDLINE
DCOM- 20040416
LR  - 20191108
IS  - 1351-8216 (Print)
IS  - 1351-8216 (Linking)
VI  - 10
IP  - 1
DP  - 2004 Jan
TI  - Successful treatment for patients with von Willebrand disease undergoing urgent 
      surgery using factor VIII/VWF concentrate (Humate-P).
PG  - 42-51
AB  - von Willebrand disease (VWD) is characterized by insufficient von Willebrand 
      factor (VWF) activity. It has been proposed that VWF:ristocetin cofactor 
      (VWF:RCo) activity may be useful in evaluating the response to VWD treatment in 
      patients who require replacement therapy. This prospective, open-label, 
      non-randomized study evaluated the safety and efficacy of a factor VIII 
      (FVIII)/VWF concentrate (Humate-P) used in treatment regimens based on VWF:RCo 
      activity in subjects with VWD in situations requiring urgent and necessary 
      surgery. This article summarizes the results for 39 subjects with 42 evaluable 
      surgical treatment events, 100% of which were rated as excellent/good for overall 
      efficacy (achievement of haemostasis). The median loading dose based upon VWF:RCo 
      activity was 82.3 international units/kilogram (IU kg(-1); range 32.5-216.8 IU 
      kg(-1)), and the median maintenance dose per infusion was 52.8 IU kg(-1) (range 
      24.2-196.5 IU kg(-1)) for a median of 3 days (range 1-50 days). The median number 
      of infusions per event was 6 (range 1-67 infusions). Three unanticipated adverse 
      events (peripheral oedema, extremity pain and pseudo-thrombocytopenia) from two 
      surgical treatment events were reported that were potentially treatment-related. 
      No serious drug-related adverse events (AEs) were observed, and no thrombotic 
      events were reported in this study. This study supports the safety and efficacy 
      of the FVIII/VWF concentrate Humate-P for the prevention of surgical haemorrhage 
      in patients with VWD when administered in doses calculated in VWF:RCo units.
FAU - Thompson, A R
AU  - Thompson AR
AD  - Puget Sound Blood Center, Seattle, WA 98104, USA. arthomps@u.washington.edu
FAU - Gill, J C
AU  - Gill JC
FAU - Ewenstein, B M
AU  - Ewenstein BM
FAU - Mueller-Velten, G
AU  - Mueller-Velten G
FAU - Schwartz, B A
AU  - Schwartz BA
CN  - Humate-P Study Group
LA  - eng
PT  - Clinical Trial
PT  - Journal Article
PT  - Multicenter Study
PL  - England
TA  - Haemophilia
JT  - Haemophilia : the official journal of the World Federation of Hemophilia
JID - 9442916
RN  - 0 (Coagulants)
RN  - 9001-27-8 (Factor VIII)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Blood Loss, Surgical/*prevention & control
MH  - Child
MH  - Child, Preschool
MH  - Coagulants/*administration & dosage
MH  - Drug Evaluation
MH  - Factor VIII/*administration & dosage
MH  - Female
MH  - Hemostasis, Surgical
MH  - Humans
MH  - Infant
MH  - Infusions, Intravenous
MH  - Male
MH  - Middle Aged
MH  - Prospective Studies
MH  - Treatment Outcome
MH  - von Willebrand Diseases/*drug therapy
EDAT- 2004/02/14 05:00
MHDA- 2004/04/17 05:00
CRDT- 2004/02/14 05:00
PHST- 2004/02/14 05:00 [pubmed]
PHST- 2004/04/17 05:00 [medline]
PHST- 2004/02/14 05:00 [entrez]
AID - 809 [pii]
AID - 10.1046/j.1351-8216.2003.00809.x [doi]
PST - ppublish
SO  - Haemophilia. 2004 Jan;10(1):42-51. doi: 10.1046/j.1351-8216.2003.00809.x.