PMID- 15166930
OWN - NLM
STAT- MEDLINE
DCOM- 20050127
LR  - 20190917
IS  - 0957-5235 (Print)
IS  - 0957-5235 (Linking)
VI  - 15 Suppl 1
DP  - 2004 May
TI  - Systematic review of the management of patients with haemophilia A and 
      inhibitors.
PG  - S25-7
AB  - The London Directorate of Health and Social Care Research and Development Group 
      recently commissioned the School of Health and Related Research at the University 
      of Sheffield to perform a systematic review of the management of patients with 
      haemophilia A and inhibitors. The main areas of the review were the epidemiology 
      of inhibitors, the role and effectiveness of immune tolerance induction, and the 
      control of acute bleeding. In addition, an economic model of the different 
      treatment strategies was constructed. Among the findings was that the overall 
      prevalence of inhibitors in unselected haemophilic populations is 5-7%, with the 
      cumulative inhibitor risk varying from 0% to 39%. The prevalence of inhibitors 
      was inversely related to the size of the reported population. Immune tolerance 
      induction was considered desirable, with evidence suggesting that the Bonn 
      protocol may be more effective than either the Malmo or low-dose protocols. 
      Immunosuppressive drug regimens on their own were ineffective. Activated 
      prothrombin complex concentrates (aPCC) were more effective than non-aPCC. 
      Recombinant activated factor VII was also effective, but there were no randomized 
      trials comparing this agent with aPCC. Porcine factor VIII was highly effective 
      in both treating acute bleeding episodes and preventing bleeding after surgery.
FAU - Makris, Michael
AU  - Makris M
AD  - Sheffield Haemophilia and Thrombosis Centre, Sheffield, UK. 
      m.makris@sheffield.ac.uk
LA  - eng
PT  - Journal Article
PT  - Review
PT  - Systematic Review
PL  - England
TA  - Blood Coagul Fibrinolysis
JT  - Blood coagulation & fibrinolysis : an international journal in haemostasis and 
      thrombosis
JID - 9102551
SB  - IM
MH  - Disease Management
MH  - Hemophilia A/*drug therapy/economics/epidemiology/immunology
MH  - Humans
MH  - Immune Tolerance
MH  - Models, Economic
MH  - Prevalence
RF  - 6
EDAT- 2004/05/29 05:00
MHDA- 2005/01/28 09:00
CRDT- 2004/05/29 05:00
PHST- 2004/05/29 05:00 [pubmed]
PHST- 2005/01/28 09:00 [medline]
PHST- 2004/05/29 05:00 [entrez]
AID - 00001721-200405001-00005 [pii]
AID - 10.1097/00001721-200405001-00005 [doi]
PST - ppublish
SO  - Blood Coagul Fibrinolysis. 2004 May;15 Suppl 1:S25-7. doi: 
      10.1097/00001721-200405001-00005.