Lepirudin therapy for thrombotic complications in congenital afibrinogenaemia

Thrombosis and Haemostasis

Volumn 91, Issue 5, 2004, Pages 1044-1046

  Schuepbach, Reto A ^b   Meili, Esther O ^b   Schneider, Ernst ^b   Peter, Ueli ^b   Bachli, Esther B ^a  

^a UNIVERSITY HOSPITAL ZURICH   (Switzerland)

^b NONE

Author keywords

[No Author keywords available]

Indexed keywords

ACETYLSALICYLIC ACID; ANTITHROMBIN; BLOOD CLOTTING FACTOR 5 LEIDEN; CARDIOLIPIN ANTIBODY; DALTEPARIN; FIBRINOGEN; HAEMOCOMPLETTAN; HEPARIN; LEPIRUDIN; LOW MOLECULAR WEIGHT HEPARIN; LUPUS ANTICOAGULANT; PHOSPHOLIPID ANTIBODY; PROTEIN C; PROTEIN S; PROTHROMBIN; THROMBIN INHIBITOR; UROKINASE; UROKINASE HS MEDAC;

AFIBRINOGENEMIA; ARTERY THROMBOSIS; ARTICLE; BLEEDING; CASE REPORT; DRUG BLOOD LEVEL; DRUG EFFICACY; DRUG INFUSION; DRUG PROTEIN BINDING; ENZYME LINKED IMMUNOSORBENT ASSAY; FIBRINOGEN BLOOD LEVEL; GENE MUTATION; HUMAN; MALE; NEWBORN; PARTIAL THROMBOPLASTIN TIME; PERCUTANEOUS TRANSLUMINAL ANGIOPLASTY; PRIORITY JOURNAL; PROTHROMBIN TIME;

EID: 2442529557     PISSN: 03406245     EISSN: None     Source Type: Journal    
DOI: 10.1055/s-0037-1614201     Document Type: Article

References (16)

1. Neerman-Arbez M, de Moerloose P, Bridel C, et al. Mutations in the fibrinogen alpha gene account for the majority of cases of congenital afibrinogenemia. Blood 2000; 96: 149-52.
2. Lak M, Keihani M, Elahi F, et al. Bleeding and thrombosis in 55 patients with inherited afibrinogenaemia. Br J Haematol 1999; 107: 204-6.
3. Bamatter F, Fanconi G, Freudenberg E, et al. Kongenitale Afibrinogenaemie. Helvetica Pediatrica acta 1963; 18: 208-28.
4. Dupuy E, Soria C, Molho, P, et al. Embolized ischemic lesions of toes in an afibrinogenemic patient: possible relevance to in vivo circulating thrombin. Thromb Res 2001; 102: 211-9.
5. Cronin C, Fitzpatrick D, Temperley I. Multiple pulmonary emboli in a patient with afibrinogenaemia. Acta, Haematol 1988;79: 53-4.
6. De Vries A, Rosenberg T, Kochwa S, et al. Precipitating anti fibrinogen antibody appearing after fibrinogen infusion in a patient with congenital afibrinogenaemia. Am J Med 1961; 30: 486-94.
7. Watanabe K, Shibuya A, Ishii E, et al. Identification of simultaneous mutation of fibrinogen alpha chain and protein C genes in a Japanese kindred. Br J Haematol 2003; 120: 101-8.
8. De Mattia D, Regina G, Giordano P, et al. Association of congenital afibrinogenemia and K-dependent protein C deficiency -a case report. Angiology 1993; 44: 745-9.
9. de Bosch NB, Mosesson MW, Ruiz-Saez A, et al. Inhibition of thrombin generation in plasma by fibrin formation (Antithrombin I). Thromb Haemost 2002; 88: 253-8.
10. Korte W, Feldges A. Increased prothrombin activation in a patient with congenital afibrinogenemia is reversible by fibrinogen substitution. Clin Invest 1994; 72: 396-8.
11. Cattaneo M, Bettega D, Lombardi R, et al. Sustained correction of the bleeding time in an afibrinogenaemic patient after infusion of fresh frozen plasma. Br J Haematol 1992; 82: 388-90.
12. Weitz JI, Leslie B, Hudoba M. Thrombin binds to soluble fibrin degradation products where it is protected from inhibition by heparin-antithrombin but susceptible to inactivation by antithrombin-independent inhibitors. Circulation 1998; 97: 544-52.
13. Cattaneo M, Kinlough-Rathbone RL, Lecchi A, et al. Fibrinogen-independent aggregation and deaggregation of human platelets: studies in two afibrinogenemic patients. Blood 1987; 70: 221-6.
14. Ni H, Denis CV, Subbarao S, et al. Persistence of platelet thrombus formation in arterioles of mice lacking both von Willebrand factor and fibrinogen. J Clin Invest 2000; 106: 385-92.
15. Ni H, Yuen PS, Papalia JM, et al. Plasma fibronectin promotes thrombus growth and stability in injured arterioles. PNAS 2003;100: 2415-9.
16. Siegmund B., Richter H, Pollmann H. Prophylactic treatment of afibrinogenemia with the fibrinogen concentrate Haemocomplettan. J Thromb Haemost 2003; 1; Abstract P0270.