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Thromb Haemost 1973; 29(03): 536-546
DOI: 10.1055/s-0038-1647795
Original Article
Schattauer GmbH

Fibrinogen Montreal

A New Case of Congenital Dysfibrinogenemia with Defective Aggregation of Monomers
M Lacombe
1   Haematology Laboratory, Hôpital Maisonneuve Montreal, Canada ; Haematology Laboratory, Hôtel-Dieu, Paris (IV), France and the Biochemistry Laboratory, Hôpital Lariboisière Paris (X), France
,
J Soria   *
1   Haematology Laboratory, Hôpital Maisonneuve Montreal, Canada ; Haematology Laboratory, Hôtel-Dieu, Paris (IV), France and the Biochemistry Laboratory, Hôpital Lariboisière Paris (X), France
,
C Soria
1   Haematology Laboratory, Hôpital Maisonneuve Montreal, Canada ; Haematology Laboratory, Hôtel-Dieu, Paris (IV), France and the Biochemistry Laboratory, Hôpital Lariboisière Paris (X), France
,
G d’Angelo
1   Haematology Laboratory, Hôpital Maisonneuve Montreal, Canada ; Haematology Laboratory, Hôtel-Dieu, Paris (IV), France and the Biochemistry Laboratory, Hôpital Lariboisière Paris (X), France
,
R Lavallee
1   Haematology Laboratory, Hôpital Maisonneuve Montreal, Canada ; Haematology Laboratory, Hôtel-Dieu, Paris (IV), France and the Biochemistry Laboratory, Hôpital Lariboisière Paris (X), France
,
Y Bonny
1   Haematology Laboratory, Hôpital Maisonneuve Montreal, Canada ; Haematology Laboratory, Hôtel-Dieu, Paris (IV), France and the Biochemistry Laboratory, Hôpital Lariboisière Paris (X), France
› Author Affiliations
Further Information

Publication History

Publication Date:
30 June 2018 (online)

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Summary

A new case of congenital dysfibrinogenemia characterized by a prolonged thrombin clotting time and a low optical density of the polymerization curve has been discovered in Montreal. The functional defect is due to an abnormal aggregation of fibrin monomers.

The characteristics of this abnormal fibrinogen are serum gélification (Paracoagulation) at 37°, 22° and 4° C, a normal immuno-electrophoretic and electrofocusing pattern, a slight increase in the mobility in the α (A) chain by electrophoresis of the dissociated chains in polyacrylamide gel. However, no abnormality was found in the α (A) chain of the disulphide knot.

* Attaché de Recherches à TI. N. S. E. R. M., service du Professeur J. Bousser (Pr. Ag. M. Samama).


 

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