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Thromb Haemost 1983; 50(04): 810-813
DOI: 10.1055/s-0038-1665318
Original Article
Schattauer GmbH Stuttgart

Protein C Deficiency in Two Austrian Families

I Pabinger-Fasching
The Division of Hematology and Blood Coagulation, First Department of Medicine, University of Vienna, Austria
,
R M Bertina
1   The Thrombosis and Hemostasis Research Unit, Leiden University Hospital, Leiden, The Netherlands
,
K Lechner
The Division of Hematology and Blood Coagulation, First Department of Medicine, University of Vienna, Austria
,
H Niessner
The Division of Hematology and Blood Coagulation, First Department of Medicine, University of Vienna, Austria
,
Ch Korninger
The Division of Hematology and Blood Coagulation, First Department of Medicine, University of Vienna, Austria
› Author Affiliations
Further Information

Publication History

Received 06 May 1983

Accepted 29 August 1983

Publication Date:
18 July 2018 (online)

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Summary

Protein C antigen was determined by Laurell rocket immunelectrophoresis in 225 patients with a history of venous thrombosis. Among these patients two females with protein C deficiency were detected. Additional studies in the families of the protein C deficient patients revealed further 7 family members with protein C deficiency. In 8 not anticoagulated patients with protein C deficiency the protein C ranged from 36 to 62% (median: 45%). In one patient on oral anticoagulant treatment protein C antigen concentration was < 10%, FII and F X were 65 and 50%, respectively. The pattern of inheritance was consistent with autosomal dominant inheritance. 5 of the 9 protein C deficient patients had severe thrombotic tendency characterized by recurrent deep venous thrombosis (n = 4), pulmonary embolism (n = 1), probable mesenteric vein thrombosis (n = 1) and superficial thrombophlebitis (n = 2). All protein C deficient patients without thrombosis were less than 17 years old.

Keywords

Protein C deficiency - Thrombotic disease
 

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