title: Use of recombinant factor VIIa in pediatric patients with liver failure and severe coagulopathy.
abstract: BACKGROUND
  Several reports have suggested a benefit for recombinant Factor VIIa (rFVIIa) in nonhematological conditions, including liver disease and transplantation. However, there are few reports of its use in children with liver failure. Recently, we used rFVIIa in four patients with liver failure and severe coagulopathy with bleeding who demonstrated significant laboratory and clinical improvement following its use with no side effects.
  
  
  METHODS
  All four patients were hospitalized with liver failure, coagulopathy, and bleeding that was controlled with fresh frozen plasma, platelets, and other therapies, as indicated. Their international normalization ratios (INR) ranged from 1.7 to 5.8 (normal 0.9-1.1). All four patients received rFVIIa for bleeding episodes that were not responding to their usual therapy, for procedures with a high risk of bleeding, or both. The dose of rFVIIa ranged from 0.067 to 0.3 mg/kg. The INR improved to normal or near normal in all four patients. In all cases, bleeding stopped within 10 minutes of receiving the rFVIIa, and there were no complications observed.
  
  
  CONCLUSIONS
  rFVIIa provided significant benefit in these children with liver failure and severe coagulopathy, in terms of clinical and laboratory improvement in their bleeding and coagulation profiles. There were no obvious side effects from the rFVIIa. This drug may be an important tool in the treatment of children with liver failure and more study is needed to define the optimal dosing for children.
authors.affiliation: Children's Hospital of Western Ontario, London Health Sciences Centre, University of Western Ontario, London, Ontario, Canada. paul.atkison@lhsc.on.ca
authors.forename: P R
authors.initials: PR
authors.lastname: Atkison
authors.forename: L
authors.initials: L
authors.lastname: Jardine
authors.forename: S
authors.initials: S
authors.lastname: Williams
authors.forename: R M
authors.initials: RM
authors.lastname: Barr
authors.forename: D
authors.initials: D
authors.lastname: Quan
authors.forename: W
authors.initials: W
authors.lastname: Wall
mesh_terms: D001778:Blood Coagulation Disorders / Q000209:etiology / Q000628:therapy*; D002648:Child; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007223:Infant; D017093:Liver Failure / Q000209:etiology / Q000628:therapy*; D008297:Male; D010289:Parenteral Nutrition, Total / Q000009:adverse effects; D011994:Recombinant Proteins / Q000627:therapeutic use; D012778:Short Bowel Syndrome / Q000150:complications
chemical_list: D011994:Recombinant Proteins; D015942:Factor VIIa