The epidemiology of Factor VIII and IX associated hepatitis in the UK

1982 ◽  
pp. 5-17
Author(s):  
Keyword(s):  
Factor Viii ◽  
Viii And Ix ◽  
The Uk

The diagnosis and management of factor VIII and IX inhibitors: a guideline from the UK Haemophilia Centre Doctors' Organization (UKHCDO)

2000 ◽  
Vol 111 (1) ◽  
pp. 78-90 ◽  
Author(s):  
C. R. M. Hay ◽  
T. P. Baglin ◽  
P. W. Collins ◽  
F. G. H. Hill ◽  
D. M. Keeling
Keyword(s):  
Factor Viii ◽  
Diagnosis And Management ◽  
Viii And Ix ◽  
The Uk

scholarly journals THE DIAGNOSIS and MANAGEMENT OF FACTOR VIII and IX INHIBITORS: A GUIDELINE FROM THE UK HAEMOPHILIA CENTRE DOCTORS' ORGANIZATION (UKHCDO)

2000 ◽  
Vol 111 (1) ◽  
pp. 78-90
Author(s):  
C. R. M. Hay ◽  
T. P. Baglin ◽  
P. W. Collins ◽  
F. G. H. Hill ◽  
D. M. Keeling
Keyword(s):  
Factor Viii ◽  
Diagnosis And Management ◽  
Viii And Ix ◽  
The Uk

Hepatitis C and Pasteurised Factor VIII and IX Concentrates

1995 ◽  
Vol 73 (04) ◽  
pp. 736-737 ◽  
Author(s):  
D Klarmann ◽  
W Kreuz ◽  
G Auerswald ◽  
K Auberger ◽  
H Rabenau ◽  
...  
Keyword(s):  
Hepatitis C ◽  
Factor Viii ◽  
Viii And Ix

scholarly journals The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation

2006 ◽  
Vol 133 (6) ◽  
pp. 591-605 ◽  
Author(s):  
Charles R. M. Hay ◽  
S. Brown ◽  
P. W. Collins ◽  
D. M. Keeling ◽  
R. Liesner
Keyword(s):  
United Kingdom ◽  
Factor Viii ◽  
Diagnosis And Management ◽  
The United Kingdom ◽  
Viii And Ix

Factor VIII and IX Inhibitors in Hemophilia

2014 ◽  
pp. 82-92
Author(s):  
Meera Chitlur ◽  
Jeanne Lusher
Keyword(s):  
Factor Viii ◽  
Viii And Ix

Leukemia and Hemophilia

PEDIATRICS ◽  
1986 ◽  
Vol 78 (3) ◽  
pp. 544-544
Author(s):  
SINASI OZSOYLU
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Fresh Frozen Plasma ◽  
Fresh Frozen ◽  
Viii And Ix ◽  
Immune Changes ◽  
Frozen Plasma

To the Editor.— I enjoyed reading the paper by Aronis et al,1 and would like to bring to your attention that we have also recently observed leukemia in two patients with hemophilia A and B, 10 and 1½ years of age, respectively.2 Because commercial factor VIII and IX were not used and only blood, fresh frozen plasma, and plasma were given on a few occasions, it was less likely that AIDS-like immune changes were responsible for the leukemia in our patients.

THE MODE OF ACTION OF PENTOSAN POLYSULPHATE IN PLASMA

1987 ◽  
Author(s):  
S Béguin ◽  
H C Hemker
Keyword(s):  
Factor Viii ◽  
Linear Increase ◽  
Factor Ix ◽  
Factor X ◽  
Heparin Cofactor Ii ◽  
Normal Plasma ◽  
Viii And Ix ◽  
Pentosan Polysulphate ◽  
Lag Times ◽  
Prothrombinase Activity

We developed a method which enables as to compute the course of prothrombinase activity in clotting plasma (H.C. Hemker, G.M. Willems, S. Béguin: Thromb. Haemostas. 56, 9-17, 1986) and used this for a study of the effect of pentosan polysulphate (PPS) on thrombin generation.When added to normal plasma in the concentration range of 0-8 μg/ml PPS induces a linear increase of the pseudo first order decay constant of endogenous thrombin like heparin does, 1 ug of PPS being equivalent to 0.045 Aig of heparin. Contrary to heparin this action is partly (∼ 65%) dependent upon AT III and partly (∼ 35%) upon heparin cofactor II.In normal plasma PPS causes an inhibition of both extrinsic and intrinsic prothrombinase formation. Only in the intrinsic system an increase of the lag time of prothrombinase appearance is observed. Unlike heparin, PPS does not inhibit factor IXa induced thrombin formation neither does it inhibit prothrombinase formation in the presence of preactivated factor VIII. The prolongation of the lag times must therefore be ascribed to inhibition by PPS of the activation of factor VIII.The inhibition of extrinsic prothrombinase formation by PPS increases with progressive dilution of thromboplastin and is not seen in haemophilia A or B plasma. This demonstrates the existance of a factor VIII and IX dependent process in extrinsic coagulation that gains in importance when the potency of factgr VII-tissue factor complex decreases, i.e. the Josso pathway.PPS, but also heparin causes an unexplained increase of prothrombinase action in haemophIIic plasma. The same phenomenon may be expected to exist in normal plasma, be it obscured by a concomitant inhibition. This, together with the incomplete inhibition of factor VIII activation by PPS makes that we cannot use this inhibitor as a means to quantitate the Josso pathway. The best estimate that we can obtain is that, in the presence of 2% thromboplastin, the factor IX dependent activation of factor X contributes more then 20% to prothrombinase generation.

Start of UK Confidential Haemophilia A Database: Analysis of 142 Patients by Solid Phase Fluorescent Chemical Cleavage of Mismatch

1999 ◽  
Vol 81 (06) ◽  
pp. 900-905 ◽  
Author(s):  
Naushin Waseem ◽  
Richard Bagnall ◽  
Peter Green ◽  
Francesco Giannelli ◽  
Keyword(s):  
Factor Viii ◽  
Solid Phase ◽  
Haemophilia A ◽  
National Strategy ◽  
Coding Region ◽  
Database Analysis ◽  
Chemical Cleavage ◽  
Signal Region ◽  
The Uk ◽  
Von Willebrand

SummaryA national strategy for optimising genetic services in haemophilia A has been initiated in the UK. Solid phase fluorescent chemical cleavage of mismatch is used to screen the entire coding region of factor VIII in six segments: four amplified from the trace of mRNA in blood lymphocytes and two from genomic DNA for the 3.4 kb exon 14 and flanking intron sequences. These segments are analysed in two threefold multiplexes so that the genes of 18 patients can be screened in a single ABI 377 gel. The promoter and polyadenylation signal region are amplified and sequenced directly. We have analysed 142 unrelated patients and identified 141 factor VIII mutations and one Normandy type von Willebrand homozygote. The former mutations include 89 missense, 10 nonsense, 5 frameshift, one 24 bp deletion and one splice signal defect. These comprise 71 different changes, of which 39 have not been previously observed.

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