Thromb Haemost 1970; 23(02): 340-346
DOI: 10.1055/s-0038-1654146
Originalarbeiten – Original Articles – Travaux Originaux
Schattauer GmbH

Congenital Hemorrhagic Diathesis with Deficiency of Factor XIII

A Case Report and a Family Study

Authors

  • J Abels M.D.,*

    1   Division of Hematology, Department of Medicine, University of Groningen, Groningen, The Netherlands
  • C. Th Smit Sibinga M.D.

    1   Division of Hematology, Department of Medicine, University of Groningen, Groningen, The Netherlands
  • L Meyleb M.D.

    1   Division of Hematology, Department of Medicine, University of Groningen, Groningen, The Netherlands
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Publikationsverlauf

Publikationsdatum:
27. Juni 2018 (online)

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Summary

A male patient with a severe congenital bleeding disorder due to factor XIII deficiency is described. An extensive family study revealed no additional demonstrable deficiencies of this factor. A lowered fibrinogen level in four of the father’s relatives was found, and remained unexplained. Prophylactic treatment with plasma transfusions appeared to be effective.

* Present address : Department of Medicine, University of Wisconsin, 1300 University Avenue, Madison, Wisconsin 53706 USA.