|
|
 Previous Article | Table of Contents | Next Article 
Normalization of markers of coagulation activation with a purified protein
C concentrate in adults with homozygous protein C deficiency
J Conard, KA Bauer, A Gruber, JH Griffin, HP Schwarz, MH Horellou, MM Samama and RD Rosenberg
Service d'Hematologie-Immunologie, Hotel Dieu, Paris, France.
Homozygous or double heterozygous protein-C deficiency can present at birth
with purpura fulminans or later in life with venous thrombosis. Two
homozygous patients who had previously sustained thrombotic episodes were
investigated at a time when they were asymptomatic and not receiving
antithrombotic therapy. The plasma levels of protein-C antigen and activity
in both individuals were approximately 20% of normal. We administered a
highly purified plasma-derived protein C concentrate to these individuals
and monitored levels of several markers of in vivo coagulation activation.
Assays for protein-C activation (activated protein C and protein C
activation peptide) showed a sustained increase from reduced baseline
levels, whereas thrombin generation (as measured by prothrombin fragment F1
+ 2) gradually decreased over about 24 hours into the normal range. These
investigations provide direct evidence that protein C is converted to
activated protein C in vivo, and that the protein-C anticoagulant pathway
is a tonically active mechanism in the regulation of hemostatic system
activation in humans.
Volume 82,
Issue 4,
pp. 1159-1164,
08/15/1993
Copyright © 1993 by The American Society of Hematology
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
R. F. Macko, L. A. Killewich, J. A. Fernandez, D. K. Cox, A. Gruber, and J. H. Griffin
Brain-Specific Protein C Activation During Carotid Artery Occlusion in Humans
Stroke,
March 1, 1999;
30(3):
542 - 545.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. M. Healy, W. W. Hancock, P. D. Christie, H. B. Rayburn, and R. D. Rosenberg
Intravascular Coagulation Activation in a Murine Model of Thrombomodulin Deficiency: Effects of Lesion Size, Age, and Hypoxia on Fibrin Deposition
Blood,
December 1, 1998;
92(11):
4188 - 4197.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. A. Kyrle, C. Mannhalter, S. Beguin, A. Stumpflen, M. Hirschl, A. Weltermann, M. Stain, B. Brenner, W. Speiser, I. Pabinger, et al.
Clinical Studies and Thrombin Generation in Patients Homozygous or Heterozygous for the G20210A Mutation in the Prothrombin Gene
Arterioscler. Thromb. Vasc. Biol.,
August 1, 1998;
18(8):
1287 - 1291.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. S. Greengard, S. Eichinger, J. H. Griffin, and K. A. Bauer
Variability of Thrombosis among Homozygous Siblings with Resistance to Activated Protein C Due to an Arg-to-Gln Mutation in the Gene for Factor V
N. Engl. J. Med.,
December 8, 1994;
331(23):
1559 - 1562.
[Full Text]
|
|
|
|
|
