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Thromb Haemost 1961; 05(02): 179-186
DOI: 10.1055/s-0038-1654918
Originalarbeiten – Original Article – Travaux Originaux
Schattauer GmbH

A Hitherto Undescribed Congenital Haemorrhagic Diathesis Probably Due to Fibrin Stabilizing Factor Deficiency

F Duckert
1   Coagulation Laboratory (Prof. F. Koller) of the Department of Medicine (Prof. P. H. Rossier) University of Zürich and the Children’s Hospital (Prof. G. Fanconi) University of Zürich, Switzerland
,
E Jung
1   Coagulation Laboratory (Prof. F. Koller) of the Department of Medicine (Prof. P. H. Rossier) University of Zürich and the Children’s Hospital (Prof. G. Fanconi) University of Zürich, Switzerland
,
D. H Shmerling
1   Coagulation Laboratory (Prof. F. Koller) of the Department of Medicine (Prof. P. H. Rossier) University of Zürich and the Children’s Hospital (Prof. G. Fanconi) University of Zürich, Switzerland
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Publication Date:
21 June 2018 (online)

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Summary

A new congenital haemorrhagic diathesis is described. It is characterized by the poor and slaw wound healing, profuse and long bleeding and by probable absence of spontaneous and joint bleeding. It is due to the deficiency of a factor which makes the fibrin clots insoluble in urea. This factor is probably the fibrin stabilizing factor of Laki and Lorand, although no proof of their identity can yet be given. The deficiency appears in both sexes and is probably an autosomal recessive disease.

 

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