title: ADAMTS13 activity may predict the cumulative survival of patients with liver cirrhosis in comparison with the Child-Turcotte-Pugh score and the Model for End-Stage Liver Disease score.
abstract: OBJECTIVE
  Decreased plasma ADAMTS13 activity (ADAMTS13:AC) results in accumulation of unusually large von Willebrand factor multimers and platelet thrombi formation. Our aim was to evaluate whether ADAMTS13:AC is a prognostic marker in patients with liver cirrhosis.
  
  
  METHODS
  Plasma ADAMTS13:AC and its related parameters were examined in 108 cirrhotic patients.
  
  
  RESULTS
  ADAMTS13:AC decreased as the severity of liver disease increased (means: controls 100%, Child A-cirrhotics 79%, Child B-cirrhotics 63%, and Child C-cirrhotics 31%). ADAMTS13:AC markedly decreased in the cirrhotics with hepatorenal syndrome, refractory ascites and hepatic encephalopathy. The cumulative survival time was the shortest (median: 4.5 months) in the cirrhotics with severe to moderate ADAMTS13:AC deficiency (<3-25%), followed by those with mild ADAMTS13:AC deficiency (25-50%), and was the longest in those with normal activity (>50%). In contrast, based on the Child-Turcotte-Pugh (CTP) score, Child C-cirrhotics had the worst survival, but the survival probabilities did not differ between Child A and B cirrhotics. Based on the Model for End-Stage Liver Disease (MELD) score, the survival was the worst for the cirrhotics in the fourth quartile, but it was not different among cirrhotics in the first three quartiles. Cox proportional-hazards regression analysis showed that ADAMTS13:AC and serum albumin were independent factors affecting the survival.
  
  
  CONCLUSIONS
  ADAMTS13:AC concomitantly decreases as the functional liver capacity decreases. This activity may be a useful prognostic marker that is equal or superior to the CTP score and the MELD score to predict not only the short-term prognosis but also the long-term survival of the cirrhotic patients.
authors.affiliation: Third Department of Internal Medicine             Departments of Blood Transfusion Medicine             Community Health and Epidemiology, Nara Medical University, Kashihara, Nara, Japan.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Takaya
authors.forename: Masahito
authors.initials: M
authors.lastname: Uemura
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Fujimura
authors.forename: Masanori
authors.initials: M
authors.lastname: Matsumoto
authors.forename: Tomomi
authors.initials: T
authors.lastname: Matsuyama
authors.forename: Seiji
authors.initials: S
authors.lastname: Kato
authors.forename: Chie
authors.initials: C
authors.lastname: Morioka
authors.forename: Hiromichi
authors.initials: H
authors.lastname: Ishizashi
authors.forename: Yuji
authors.initials: Y
authors.lastname: Hori
authors.forename: Masao
authors.initials: M
authors.lastname: Fujimoto
authors.forename: Tatsuhiro
authors.initials: T
authors.lastname: Tsujimoto
authors.forename: Hideto
authors.initials: H
authors.lastname: Kawaratani
authors.forename: Masahisa
authors.initials: M
authors.lastname: Toyohara
authors.forename: Norio
authors.initials: N
authors.lastname: Kurumatani
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Fukui