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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

Karin Fijnvandraat
1   Emma Kinder Ziekenhuis/Children’s Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
,
Bert Derkx
1   Emma Kinder Ziekenhuis/Children’s Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
,
Marjolein Peters
1   Emma Kinder Ziekenhuis/Children’s Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
,
Rob Bijlmer
1   Emma Kinder Ziekenhuis/Children’s Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
,
Augueste Sturk
2   The Center for Thrombosis, Haemostasis, Atherosclerosis and Inflammation Research, University of Amsterdam, Amsterdam, The Netherlands
,
Martin H Prins
3   Department of Clinical Epidemiology and Biostatistics, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
,
Sander J H van Deventer
2   The Center for Thrombosis, Haemostasis, Atherosclerosis and Inflammation Research, University of Amsterdam, Amsterdam, The Netherlands
,
Jan Wouter ten Cate
2   The Center for Thrombosis, Haemostasis, Atherosclerosis and Inflammation Research, University of Amsterdam, Amsterdam, The Netherlands
› Author Affiliations
Further Information

Publication History

Received 07 April 1994

Accepted after resubmission 29 September 1994

Publication Date:
09 July 2018 (online)

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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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