Haemostasis contact system and fibrinolysis in hereditary angioedema (C1-inhibitor deficiency).
Factors of the classical complement pathway, the contact system and fibrinolysis were evaluated both with functional and immunochemical methods, in patients with inherited deficiency of C1-inhibitor. Evaluations were performed under basal conditions, during acute attacks and during prophylaxis with low doses of anabolic steroids. Patients in the basal state showed no significant abnormalities of any of the parameters that we investigated. During acute attacks a slightly reduced prekallikrein concentration was registered. During treatment with low doses of danazol and stanozolol, protein C and plasminogen were found to be increased. Our data suggest that C1-inhibitor deficiency per se does not lead to a derangement of the fibrinolysis and coagulation contact system, and that the kinin system may be involved during acute attacks of angioedema.
Cugno
M
M
Clinica Medica V, Università di Milano, Italy.
Bergamaschini
L
L
Uziel
L
L
Cicardi
M
M
Agostoni
A
A
Jie
A F
AF
Kluft
C
C
D000293:Adolescent; D000328:Adult; D000799:Angioedema / Q000097:blood* / Q000235:genetics; D001779:Blood Coagulation Factors / Q000032:analysis; D001798:Blood Proteins; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D005260:Female; D005342:Fibrinolysis; D006023:Glycoproteins / Q000097:blood; D006487:Hemostasis; D006801:Humans; D008297:Male; D008875:Middle Aged; D010958:Plasminogen / Q000032:analysis; D011506:Proteins; D012016:Reference Values; D010959:Tissue Plasminogen Activator / Q000097:blood; D000099053:Histidine-Rich Glycoprotein
D001779:Blood Coagulation Factors; D001798:Blood Proteins; D003174:Complement C1 Inactivator Proteins; D006023:Glycoproteins; D011506:Proteins; D000099053:Histidine-Rich Glycoprotein; D010958:Plasminogen; D010959:Tissue Plasminogen Activator