title: Behavior of ADAMTS13 and Von Willebrand factor levels in patients after living donor liver transplantation.
abstract: BACKGROUND
  Thrombotic microangiopathy (TMA) is one of the important complications occurring after liver transplantation (LT), and it is suggested that a Von Willebrand factor (VWF) and ADAMTS13 may play an important role in the onset of TMA and poor outcome after LT.
  
  
  METHODS
  In 81 patients after living donor LT (LDLT), 17 patients who had both severe thrombocytopenia and hemolytic anemia with fragmented red cell were diagnosed as TMA- like syndrome (TMALS) and 10 patients died.
  
  
  RESULTS
  ADAMTS13 activities were slightly low, and plasma levels of VWF and VWF propeptide (VWFpp) antigens and the ratio of VWFpp/VWF were significantly high before LDLT. ADAMTS13 activities were significantly reduced from day 1 to day 28 after surgery, and plasma levels of VWF antigen slightly decreased on day 1 and plasma levels of VWFpp continued to be high. The ratio of VWFpp/VWF was significantly high on day 1 after surgery. The mortality was high in the patients with TMALS and the frequency of TMALS was high in non-survivors. VWF levels were significantly low and the ratio of VWFpp/VWF was significantly high in those with TMALS on day 1 after surgery. The ADAMTS13 activity was significantly low, and the VWFpp and the VWFpp/ADAMTS13 ratio were significantly high in non-survivor on day 28 after surgery.
  
  
  CONCLUSIONS
  These findings suggest that VWF and ADAMTS13 might therefore play an important role in the onset of TMA and poor outcome after LT. The VWFpp may therefore be a more useful marker for the diagnosis of TMALS than VWF.
authors.affiliation: Hepatobiliary Pancreatic and Transplant Surgery, Mie University Graduate School of Medicine, Mie, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Takahashi
authors.forename: Hideo
authors.initials: H
authors.lastname: Wada
authors.forename: Masanobu
authors.initials: M
authors.lastname: Usui
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Kobayashi
authors.forename: Naomi
authors.initials: N
authors.lastname: Habe-Ito
authors.forename: Takeshi
authors.initials: T
authors.lastname: Matsumoto
authors.forename: Shinji
authors.initials: S
authors.lastname: Uemoto
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Nobori
authors.forename: Shuji
authors.initials: S
authors.lastname: Isaji
mesh_terms: D051722:ADAM Proteins / Q000097:blood*; D000071120:ADAMTS13 Protein; D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D004912:Erythrocytes / Q000166:cytology; D005260:Female; D005786:Gene Expression Regulation; D006801:Humans; D007223:Infant; D017114:Liver Failure, Acute / Q000150:complications / Q000628:therapy*; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D008875:Middle Aged; D013921:Thrombocytopenia / Q000201:enzymology*; D057049:Thrombotic Microangiopathies / Q000150:complications* / Q000209:etiology; D016896:Treatment Outcome; D055815:Young Adult; D014841:von Willebrand Factor / Q000378:metabolism*
chemical_list: D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human