scholarly journals Hereditary angioedema: Linking complement regulation to the coagulation system

2018 ◽  
Vol 3 (1) ◽  
pp. 38-43 ◽  
Author(s):  
Marcel Levi ◽  
Danny M. Cohn ◽  
Sacha Zeerleder
Keyword(s):  
Hereditary Angioedema ◽  
Coagulation System ◽  
Complement Regulation

Anaphylaxis in Response to C1 Esterase Inhibitor in a Patient with Hereditary Angioedema

2007 ◽  
Vol 19 (4) ◽  
pp. 159-163 ◽  
Author(s):  
Marco Cicardi ◽  
Lorenza Zingale ◽  
Andrea Zanichelli ◽  
Daniela Lambertenghi Deliliers
Keyword(s):  
Hereditary Angioedema ◽  
C1 Esterase Inhibitor ◽  
Esterase Inhibitor

Oxidative Stress and Atherosclerosis: Its Relationship to Growth Factors, Thrombus Formation and Therapeutic Approaches

1999 ◽  
Vol 82 (S 01) ◽  
pp. 32-37 ◽  
Author(s):  
Karlheinz Peter ◽  
Wolfgang Kübler ◽  
Johannes Ruef ◽  
Thomas K. Nordt ◽  
Marschall S. Runge ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Growth Factors ◽  
Vessel Wall ◽  
Inflammatory Responses ◽  
Plaque Rupture ◽  
Thrombus Formation ◽  
Vascular Lesion ◽  
Coagulation System ◽  
Therapeutic Approaches ◽  
Causative Relationship

SummaryThe initiating event of atherogenesis is thought to be an injury to the vessel wall resulting in endothelial dysfunction. This is followed by key features of atherosclerotic plaque formation such as inflammatory responses, cell proliferation and remodeling of the vasculature, finally leading to vascular lesion formation, plaque rupture, thrombosis and tissue infarction. A causative relationship exists between these events and oxidative stress in the vessel wall. Besides leukocytes, vascular cells are a potent source of oxygen-derived free radicals. Oxidants exert mitogenic effects that are partially mediated through generation of growth factors. Mitogens, on the other hand, are potent stimulators of oxidant generation, indicating a putative self-perpetuating mechanism of atherogenesis. Oxidants influence the balance of the coagulation system towards platelet aggregation and thrombus formation. Therapeutic approaches by means of antioxidants are promising in both experimental and clinical designs. However, additional clinical trials are necessary to assess the role of antioxidants in cardiovascular disease.

Markers of Hypercoagulability in Patients with Hemophilia B Given Repeated, Large Doses of Factor IX Concentrates during and after Surgery

1994 ◽  
Vol 71 (06) ◽  
pp. 737-740 ◽  
Author(s):  
E Santagostino ◽  
P M Mannucci ◽  
A Gringeri ◽  
G Tagariello ◽  
F Baudo ◽  
...  
Keyword(s):  
High Risk ◽  
Ex Vivo ◽  
Factor Ix ◽  
Hemophilia B ◽  
Coagulation System ◽  
Prolonged Treatment ◽  
Orthopedic Procedures ◽  
Factor X ◽  
Prothrombin Complex Concentrates ◽  
Increased Risk

SummaryPurer factor IX (FIX) concentrates have been produced for the treatment of hemophilia B in the attempt to reduce the risk of thrombotic complications associated with the use of prothrombin complex concentrates. To evaluate ex vivo whether or not FIX concentrates activate the coagulation system in conditions associated with a high risk for thrombosis, we measured markers of hypercoagulability in 10 patients with hemophilia B who underwent surgery, mainly orthopedic procedures, covered by multiple concentrate infusions (40-80 U/kg/day). Postinfusion plasma levels of prothrombin fragment 1+2 and factor X activation peptide did not differ significantly from the presurgical levels, neither before nor after each concentrate dose. Therefore, it appears that prolonged treatment of patients with hemophilia B undergoing high risk surgical procedures with high doses of FIX concentrate does not cause systemic activation of coagulation. This suggests that purified FIX concentrates are preferable to prothrombin complex concentrates for conditions associated with an increased risk of thrombosis.

The Hemostatic Mechanism after Open-Heart Surgery

1978 ◽  
Vol 39 (02) ◽  
pp. 474-487 ◽  
Author(s):  
E R Cole ◽  
F Bachmann ◽  
C A Curry ◽  
D Roby
Keyword(s):  
Extracorporeal Circulation ◽  
Heart Surgery ◽  
Polyacrylamide Gel Electrophoresis ◽  
Open Heart Surgery ◽  
Coagulation System ◽  
Open Heart ◽  
Time Activity ◽  
Post Surgery ◽  
Plasma Fractions ◽  
The Mean

SummaryA prospective study in 13 patients undergoing open-heart surgery with extracorporeal circulation revealed a marked decrease of the mean one-stage prothrombin time activity from 88% to 54% (p <0.005) but lesser decreases of factors I, II, V, VII and X. This apparent discrepancy was due to the appearance of an inhibitor of the extrinsic coagulation system, termed PEC (Protein after Extracorporeal Circulation). The mean plasma PEC level rose from 0.05 U/ml pre-surgery to 0.65 U/ml post-surgery (p <0.0005), and was accompanied by the appearance of additional proteins as evidenced by disc polyacrylamide gel electrophoresis of plasma fractions (p <0.0005). The observed increases of PEC, appearance of abnormal protein bands and concomitant increases of LDH and SGOT suggest that the release of an inhibitor of the coagulation system (similar or identical to PIVKA) may be due to hypoxic liver damage during extracorporeal circulation.

Premature Arterial Disease Associated with Familial Antithrombin III Deficiency

1990 ◽  
Vol 63 (01) ◽  
pp. 013-015 ◽  
Author(s):  
E J Johnson ◽  
C R M Prentice ◽  
L A Parapia
Keyword(s):  
Risk Factors ◽  
Venous Thromboembolism ◽  
Potential Risk ◽  
Antithrombin Iii ◽  
Arterial Disease ◽  
Coagulation System ◽  
Early Age ◽  
Arterial Thromboembolism ◽  
Potential Risk Factors ◽  
Antithrombin Iii Deficiency

SummaryAntithrombin III (ATIII) deficiency is one of the few known abnormalities of the coagulation system known to predispose to venous thromboembolism but its relation to arterial disease is not established. We describe two related patients with this disorder, both of whom suffered arterial thrombotic events, at an early age. Both patients had other potential risk factors, though these would normally be considered unlikely to lead to such catastrophic events at such an age. Thrombosis due to ATIII deficiency is potentially preventable, and this diagnosis should be sought more frequently in patients with arterial thromboembolism, particularly if occurring at a young age. In addition, in patients with known ATIII deficiency, other risk factors for arterial disease should be eliminated, if possible. In particular, these patients should be counselled against smoking.

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