Severe Protein C Deficiency in Congenital Thrombotic Disease – Description of an Immunoenzymological Assay for Protein C Determination

J Soria; C Soria; M Samama; G Nicolas; W Kisiel

doi:10.1055/s-0038-1661299

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 1985; 53(03): 293-296
DOI: 10.1055/s-0038-1661299

Original Article

Schattauer GmbH Stuttgart

Severe Protein C Deficiency in Congenital Thrombotic Disease – Description of an Immunoenzymological Assay for Protein C Determination

J Soria

The Services des Professeurs Bernadou, Fabiani, Samama, Hôtel-Dieu, Paris, the Services des Professeurs Caen et Rousselet, Hôpital Lariboisière, Paris

,

C Soria

The Services des Professeurs Bernadou, Fabiani, Samama, Hôtel-Dieu, Paris, the Services des Professeurs Caen et Rousselet, Hôpital Lariboisière, Paris

,

M Samama

The Services des Professeurs Bernadou, Fabiani, Samama, Hôtel-Dieu, Paris, the Services des Professeurs Caen et Rousselet, Hôpital Lariboisière, Paris

,

G Nicolas

¹The C.H.U., Nantes, France

The Services des Professeurs Bernadou, Fabiani, Samama, Hôtel-Dieu, Paris, the Services des Professeurs Caen et Rousselet, Hôpital Lariboisière, Paris

,

W Kisiel

²The University of Washington, Seattle, Washington, USA

The Services des Professeurs Bernadou, Fabiani, Samama, Hôtel-Dieu, Paris, the Services des Professeurs Caen et Rousselet, Hôpital Lariboisière, Paris

› Author Affiliations

Abstract

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Summary

An immunoenzymatic assay (ELISA) is described for the quantitative assay of protein C in plasma. This technique allows a safe, reliable, and sensitive assay of protein C, and is easily used for routine investigation. Using this technique, a protein C deficiency (0.16 U/ml) was discovered in a 16 year old patient with a history of very severe thrombotic disease. Protein C deficiency was also discovered in his mother (0.62 U/ml) and father (0.50 U/ml). We therefore suggest that this case could represent a homozygous deficiency of protein C.

Keywords

Protein C - Protein C deficiency - Thrombotic disease

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References

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