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Thromb Haemost 1990; 64(02): 206-210
DOI: 10.1055/s-0038-1647286
Original Article
Schattauer GmbH Stuttgart

Hereditary Protein S Deficiency in Young Adults with Arterial Occlusive Disease

C F Allaart
1   The Haemostasis and Thrombosis Research Unit, University Hospital Leiden, The Netherlands
,
D C Aronson
2   The Dept. of Surgery, University Hospital Leiden, The Netherlands
,
Th Ruys
2   The Dept. of Surgery, University Hospital Leiden, The Netherlands
,
F R Rosendaal
3   The Dept. of Clinical Epidemiology, University Hospital Leiden, The Netherlands
,
J H van Bockel
2   The Dept. of Surgery, University Hospital Leiden, The Netherlands
,
R M Bertina
1   The Haemostasis and Thrombosis Research Unit, University Hospital Leiden, The Netherlands
,
E Briët
1   The Haemostasis and Thrombosis Research Unit, University Hospital Leiden, The Netherlands
› Author Affiliations
Further Information

Publication History

Received 29 August 1989

Accepted after revision 08 August 1990

Publication Date:
25 July 2018 (online)

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Summary

Protein S is the vitamin K dependent cofactor of activated protein C. It has an important role in the regulation of blood coagulation and fibrinolysis. Hereditary protein S deficiency is associated with familial venous thrombophilia. Since a few patients with arterial occlusions have been reported to be protein S deficient, it is speculated that hereditary protein S deficiency may be also a risk factor for the development of arterial thrombosis. In a group of 37 consecutive patients with arterial occlusive disease presenting before the age of 45, three patients were found heterozygous for hereditary protein S deficiency. None of the patients had a protein C deficiency or an antithrombin III deficiency. Family investigations showed a clear association between the hereditary deficiency and venous thrombosis, but a relation between the deficiency and arterial thrombosis was less obvious. A review of previous literature on patients with arterial thrombosis and protein S deficiency revealed that more extensive studies are needed to demonstrate whether or not hereditary protein S deficiency is a risk factor for the development of arterial thrombosis.

 

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