title: Preemptive administration of recombinant factor VII (rVIIa) in patients transplanted due to fulminant Wilson's disease.
abstract: BACKGROUND
  Fulminant liver failure (FLF) is a severe clinical condition usually accompanied by a coagulopathy, which is one of the key selection criteria for liver transplantation. Prolongation of prothrombin time can vary between etiologies of FHF, being one of the worst in fulminant presentation of Wilson's disease. Although INR value is not predictive for hemorrhage, it is commonly accepted that INR >1.9 carries a substantial risk of intraoperative bleeding. We tested the hypothesis that preemptive administration of recombinant factor VII (rFVIIa) is a safe and efficacious approach allowing performance of urgent liver transplantation without significant bleeding and thrombotic complications.
  
  
  METHODS
  In 14 analyzed cases of fulminant Wilson's disease subjected for orthotopic liver transplantation (OLT), a single bolus of rFVIIa 10 minutes before skin incision was given routinely in a median dose of 55.9 microg/kg.
  
  
  RESULTS
  Median value of INR dropped from 3.1 to 1.2 30 minutes after rFVIIa administration. Transfusion requirements in those cases did not differ from standard transfusion requirements of PRBCs observed in elective OLTx in our center (6 units v. 5, respectively). Recurrent bleeding was noted in 1 patient with the rupture of aorta. One-year survival rate in the studied group was 85.7%. Thrombotic complications were noted in none of the patients.
  
  
  CONCLUSIONS
  We conclude that preemptive use of rFVIIa allows performance of a broad surgical procedure without hemorrhagic complications and without increased risk of thromboembolic events in patients with severe coagulopathy.
authors.affiliation: Marie Curie Hospital, Szczecin, Poland.
authors.forename: Maria M
authors.initials: MM
authors.lastname: Czuprynska
authors.forename: Marta
authors.initials: M
authors.lastname: Wawrzynowicz-Syczewska
authors.forename: Lidia
authors.initials: L
authors.lastname: Jureczko
authors.forename: Janina
authors.initials: J
authors.lastname: Andrzejewska
authors.forename: Konrad
authors.initials: K
authors.lastname: Jarosz
authors.forename: Janusz
authors.initials: J
authors.lastname: Trzebicki
authors.forename: Marek
authors.initials: M
authors.lastname: Pacholczyk
authors.forename: Andrzej
authors.initials: A
authors.lastname: Chmura
authors.forename: Maciej
authors.initials: M
authors.lastname: Wojcicki
mesh_terms: D000293:Adolescent; D000328:Adult; D001778:Blood Coagulation Disorders / Q000188:drug therapy / Q000209:etiology; D016063:Blood Loss, Surgical / Q000517:prevention & control; D015942:Factor VIIa / Q000008:administration & dosage*; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000209:etiology / Q000601:surgery*; D006801:Humans; D017114:Liver Failure, Acute / Q000150:complications / Q000209:etiology / Q000601:surgery; D016031:Liver Transplantation / Q000009:adverse effects; D008297:Male; D011517:Prothrombin Time; D011994:Recombinant Proteins / Q000008:administration & dosage; D012189:Retrospective Studies; D015996:Survival Rate; D065227:Transfusion Reaction; D055815:Young Adult
chemical_list: D011994:Recombinant Proteins; D015942:Factor VIIa