title: Correlation between plasma activity of ADAMTS-13 and coagulopathy, and prognosis in disseminated intravascular coagulation.
abstract: BACKGROUND
  In disseminated intravascular coagulation (DIC), widespread activation of intravascular coagulation accompanied with florid endothelial activation results in release of unusually large von Willebrand factor (ULvWF) from endothelium. Circulating a disintegrin-like and metalloprotease with thrombospondin type 1 repeats (ADAMTS)-13 may be consumed through the ongoing cleavage of ULvWF, resulting in a secondary deficiency of ADAMTS-13 in DIC. We determined whether ADAMTS-13 activity showed a significant correlation with the activation status of the coagulation system and hospital mortality in DIC.
  
  
  METHODS
  ADAMTS-13 activity was assayed by fluorescence resonance energy transfer assay in 97 patients who were clinically suspected to have DIC.
  
  
  RESULTS
  ADAMTS-13 activity gradually decreased based on the DIC score and D-dimer levels and was correlated with the antithrombin level, representing the consumption of ADAMTS-13 during the ongoing coagulation process. There were no correlation between ADAMTS-13 activity and neutrophil CD64 expression as a neutrophil activation marker and circulating IL-6 level as an inflammatory marker. Patients with a low activity of ADAMTS-13 (< or = 56.4%) had a poor survival rate compared to patients with a high activity of ADAMTS-13.
  
  
  CONCLUSIONS
  We conclude that ADAMTS-13 activity is strongly correlated with the severity of coagulopathy and hospital mortality. ADAMTS-13 may serve as a diagnostic and prognostic marker of DIC.
authors.affiliation: Department of Laboratory Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea.
authors.forename: Jungwon
authors.initials: J
authors.lastname: Hyun
authors.forename: Hyun Kyung
authors.initials: HK
authors.lastname: Kim
authors.forename: Ji-Eun
authors.initials: JE
authors.lastname: Kim
authors.forename: Min-Gyu
authors.initials: MG
authors.lastname: Lim
authors.forename: Jae Seol
authors.initials: JS
authors.lastname: Jung
authors.forename: Seonyang
authors.initials: S
authors.lastname: Park
authors.forename: Han-Ik
authors.initials: HI
authors.lastname: Cho
mesh_terms: D051722:ADAM Proteins / Q000097:blood*; D000071120:ADAMTS13 Protein; D000368:Aged; D000991:Antithrombins / Q000032:analysis; D004211:Disseminated Intravascular Coagulation / Q000097:blood* / Q000175:diagnosis / Q000401:mortality*; D004730:Endothelium, Vascular / Q000378:metabolism; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis; D031541:Fluorescence Resonance Energy Transfer; D006801:Humans; D053208:Kaplan-Meier Estimate; D007723:Korea; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012720:Severity of Illness Index; D016019:Survival Analysis; D015996:Survival Rate; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D000991:Antithrombins; D005338:Fibrin Fibrinogen Degradation Products; C036309:fibrin fragment D; D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human