A congenitally abnormal fibrinogen was isolated from blood of a young man with deep-vein thrombosis. Two other affected members of this family had three episodes of severe arterial thrombosis. The fibrinogen showed a delayed clotting by thrombin, but a normal clotting by Arvin®, Reptilase®, and prothrombin-staphylocoagulase complex. Analysis of the fibrinopeptides A and B by high performance liquid chromatography did not reveal an abnormal peptide structure. The rate of release of A and B peptides by thrombin was strongly delayed, whereas the rate of release of fibrinopeptide A by Arvin® appeared to be normal. The fibrin polymerization rate was normal. Interactions between the abnormal fibrinogen, platelets and the fibrinolytic interaction between fibrinogen Milano II and thrombin is associated with a defective binding of thrombin to the fibrin moiety of the abnormal fibrinogen.
Fibrinogen Milano II: a congenital dysfibrinogenaemia associated with juvenile arterial and venous thrombosis / F. Haverkate, J. Koopman, C. Kluft, A. Dangelo, M. Cattaneo, P. Mannucci. - In: THROMBOSIS AND HAEMOSTASIS. - ISSN 0340-6245. - 55:1(1986), pp. 131-135.
|Titolo:||Fibrinogen Milano II: a congenital dysfibrinogenaemia associated with juvenile arterial and venous thrombosis|
CATTANEO, MARCO NATALE (Penultimo)
MANNUCCI, PIER MANNUCCIO (Ultimo)
|Parole Chiave:||Pedigree; Thrombin; Blood Coagulation Disorders; Humans; Fibrinogen; Fibrinopeptide A; Thrombosis; Fibrinogens, Abnormal; Fibrin; Fibrinopeptide B; Platelet Aggregation; Adult; Male; Thrombophlebitis|
|Settore Scientifico Disciplinare:||Settore MED/09 - Medicina Interna|
|Data di pubblicazione:||1986|
|Appare nelle tipologie:||01 - Articolo su periodico|