title: ADAMTS-13, von Willebrand factor and related parameters in severe sepsis and septic shock.
abstract: BACKGROUND
  Insufficient control of von Willebrand factor (VWF) multimer size as a result of severely deficient ADAMTS-13 activity results in thrombotic thrombocytopenic purpura associated with microvascluar thrombosis and platelet consumption, features not seldom seen in severe sepsis and septic shock.
  
  
  METHODS
  ADAMTS-13 activity and VWF parameters of 40 patients with severe sepsis or septic shock were compared with those of 40 healthy controls of the same age and gender and correlated with clinical findings and sepsis outcome.
  
  
  RESULTS
  ADAMTS-13 activity was significantly lower in patients than in healthy controls [median 60% (range 27-160%) vs. 110% (range 63-200%); P < 0.001]. VWF parameters behaved reciprocally and both VWF ristocetin cofactor activity (RCo) and VWF antigen (VWF:Ag) were significantly (P < 0.001) higher in patients compared with controls. Neither ADAMTS-13 activity nor VWF parameters correlated with disease severity, organ dysfunction or outcome. However, a contribution of acute endothelial dysfunction to renal impairment in sepsis is suggested by the significantly higher VWF propeptide and soluble thrombomodulin levels in patients with increased creatinine values as well as by their strong positive correlations (creatinine and VWF propeptide r(s) = 0.484, P < 0.001; creatinine and soluble thrombomodulin r(s) = 0.596, P < 0.001).
  
  
  CONCLUSIONS
  VWF parameters are reciprocally correlated with ADAMTS-13 activity in severe sepsis and septic shock but have no prognostic value regarding outcome.
authors.affiliation: Department of Hematology and Central Hematology Laboratory, Inselspital, University of Bern, Bern, Switzerland. johanna.kremer@insel.ch
authors.forename: J A
authors.initials: JA
authors.lastname: Kremer Hovinga
authors.forename: S
authors.initials: S
authors.lastname: Zeerleder
authors.forename: P
authors.initials: P
authors.lastname: Kessler
authors.forename: T
authors.initials: T
authors.lastname: Romani de Wit
authors.forename: J A
authors.initials: JA
authors.lastname: van Mourik
authors.forename: C E
authors.initials: CE
authors.lastname: Hack
authors.forename: H
authors.initials: H
authors.lastname: ten Cate
authors.forename: P H
authors.initials: PH
authors.lastname: Reitsma
authors.forename: W A
authors.initials: WA
authors.lastname: Wuillemin
authors.forename: B
authors.initials: B
authors.lastname: Lämmle
mesh_terms: D051722:ADAM Proteins / Q000378:metabolism*; D000071120:ADAMTS13 Protein; D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D003404:Creatinine / Q000097:blood; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D018805:Sepsis / Q000097:blood / Q000175:diagnosis*; D012772:Shock, Septic / Q000097:blood / Q000175:diagnosis*; D018180:Thrombomodulin / Q000097:blood; D014841:von Willebrand Factor / Q000032:analysis*
chemical_list: D018180:Thrombomodulin; D014841:von Willebrand Factor; D003404:Creatinine; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human