Coagulation Activation and Tissue Necrosis in Meningococcal Septic Shock: Severely Reduced Protein C Levels Predict a High Mortality

Karin Fijnvandraat; Bert Derkx; Marjolein Peters; Rob Bijlmer; Augueste Sturk; Martin H Prins; Sander J H van Deventer; Jan Wouter ten Cate

doi:10.1055/s-0038-1651669

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 1995; 73(01): 015-020
DOI: 10.1055/s-0038-1651669

Original Article

Clinical Studies

Schattauer GmbH Stuttgart

Coagulation Activation and Tissue Necrosis in Meningococcal Septic Shock: Severely Reduced Protein C Levels Predict a High Mortality

Authors

Karin Fijnvandraat

¹Emma Kinder Ziekenhuis/Children’s Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Bert Derkx

¹Emma Kinder Ziekenhuis/Children’s Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Marjolein Peters

¹Emma Kinder Ziekenhuis/Children’s Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Rob Bijlmer

¹Emma Kinder Ziekenhuis/Children’s Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Augueste Sturk

²The Center for Thrombosis, Haemostasis, Atherosclerosis and Inflammation Research, University of Amsterdam, Amsterdam, The Netherlands
Martin H Prins

³Department of Clinical Epidemiology and Biostatistics, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
Sander J H van Deventer

²The Center for Thrombosis, Haemostasis, Atherosclerosis and Inflammation Research, University of Amsterdam, Amsterdam, The Netherlands
Jan Wouter ten Cate

²The Center for Thrombosis, Haemostasis, Atherosclerosis and Inflammation Research, University of Amsterdam, Amsterdam, The Netherlands

Abstract

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Summary

In 35 consecutively admitted children (mean age: 4.3 years) with a clinical diagnosis of meningococcal septic shock (MSS), activation of the coagulation and fibrinolytic pathways was evaluated directly at admittance to the paediatric intensive care unit (ICU). The association of clinical signs and haemostatic abnormalities was assessed.

All patients had signs of extensive activation of the coagulation system. The 28-day mortality was 26%. Protein C activity was strongly reduced, especially in non-survivors in whom it was significantly lower than in survivors (5% versus 23%; p <0.0001). There was a strong negative correlation between protein C activity and the mean size of the skin lesions (r = -0.71, p <0.001). These results suggest that an acquired protein C deficiency in MSS is related to the pathogenesis of purpura fulminans.

Furthermore, no increase in C4b-binding protein (C4BP) and no decrease in the ratio free protein S/total protein S was observed, suggesting that a deficiency of free protein S as a result of increased C4BP does not play a pathogenetic role in meningococcal septic shock.

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References

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