Un nouveau Cas d’Anomalie constitutionnelle et familiale du Fibrinogène sans Diathese hémorragique

Summary

A new family with congenital dysfibrinogenemia without bleeding tendency has been discovered in Nancy. Identical laboratory abnormalities were found in the mother and son plasma: prolongation of thrombin and reptilase clotting time.

The abnormal fibrinogen affects, only a little, the normal plasma fibrinoformation.

The functional characterization of the defect is localized in fibrin monomer aggregation. The release of fibrinopeptides is normal.

The abnormal fibrinogen shows

a) an alteration in glucidic moiety of the molecule with a high level of sialic acid,

b) an disturbance of chromatography on D. E. A. E. cellulose and

c) of the electrophoretic mobility.

^*) Attaché de Recherches à l’Inserm : service du Pr. Bousser (Laboratoire du Dr. M. Samama).

^**) Maître assistant de Biochimie appliquée (Pr. M. L. Girard) — Faculté de Pharmacie de Paris.

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