Reduced ADAMTS13 in children with severe meningococcal sepsis is associated with severity and outcome

Tamara N. Bongers, Marieke Emonts, Moniek P. M. de Maat, Ronald de Groot, Ton Lisman, Jan A. Hazelzet, Frank W. G. Leebeek*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

27 Citations (Scopus)

Abstract

Multiple organ failure is a common feature of pediatric meningococcal sepsis and is associated with an imbalance of coagulation and fibrinolysis. This is partly due to an increased secretion of prothrombotic ultra-large von Willebrand factor (VWF) as the result of vascular endothelial damage. Another factor that may Contribute is ADAMTS13, which converts VWF into smaller, less active, VWF multimers and thus influences VWF activity in plasma. We investigated the role of ADAMTS13 and VWF in the severity and outcome of sepsis. In 58 children with severe meningococcal sepsis we measured ADAMTS13 activity and antigen, VWF collagen binding activity (VWF:CB) and antigen levels (VWF:Ag), VWF propeptide and factor VIII at different time points during their stay in the paediatric intensive care unit. In the acute phase, both ADAMTS13 activity and antigen were decreased (median 23.4% and 33.7% of normal, respectively) and VWF:CB and VWF:Ag levels were strongly increased (325% and 348%, respectively.) ADAMTS13 antigen (23.9% vs. 34.6%; p=0.06) and VWF:CB (240% and 340% p

Original languageEnglish
Pages (from-to)1181-1187
Number of pages7
JournalThrombosis and Haemostasis
Volume103
Issue number6
DOIs
Publication statusPublished - Jun-2010

Keywords

  • ADAMS/ADAMTS 13
  • disseminated intravascular coagulation (DIC)
  • fibrinolytic disorders
  • paediatric haemostasis
  • sepsis
  • VON-WILLEBRAND-FACTOR
  • DISSEMINATED INTRAVASCULAR COAGULATION
  • CLEAVING PROTEASE ADAMTS13
  • SEPTIC SHOCK
  • FACTOR PROPEPTIDE
  • ISCHEMIC-STROKE
  • ORGAN FAILURE
  • RISK
  • INFLAMMATION
  • DEFICIENCY

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