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Thromb Haemost 1974; 32(02/03): 564-577
DOI: 10.1055/s-0038-1647725
Original Article
Schattauer GmbH

Fibrinogen Valencia A New Case of Congenital Dysfibrinogenemia

J Aznar
1   Department of Clinical Pathology and Children’s Hospital. Ciudad Sanitaria “La Fe” Valencia, Spain
,
A Fernandez-Pavón
1   Department of Clinical Pathology and Children’s Hospital. Ciudad Sanitaria “La Fe” Valencia, Spain
,
E Regañóon
1   Department of Clinical Pathology and Children’s Hospital. Ciudad Sanitaria “La Fe” Valencia, Spain
,
V Vila
1   Department of Clinical Pathology and Children’s Hospital. Ciudad Sanitaria “La Fe” Valencia, Spain
,
F Orellana
1   Department of Clinical Pathology and Children’s Hospital. Ciudad Sanitaria “La Fe” Valencia, Spain
› Author Affiliations
Further Information

Publication History

Received 30 July 1974

Accepted 12 September 1974

Publication Date:
30 June 2018 (online)

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Summary

A new case of dysfibrinogenemia is described, the proband had low fibrinogen level and a moderate hyperfibrinolysis. Attention is drawn to the florid hemorrhagic picture and its early presentation.

The family history showed a moderate hyperfibrinolysis.

It appeared that the cause of the dysfibrinogenemia was due to a deficient polymerization of the α chains of the fibrinogen.

It is not yet known if this is caused by a defective liberation of fibrinopeptides. Due to the peculiar characteristic of this case and in accordance with the international norms, we propose to call this dysfibrinogenemia “Fibrinogen Valencia.”

 

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