title: Orthotopic liver transplantation in hemophilia B: a case report.
abstract: Liver transplantation is a treatment modality that is being used with increasing frequency in cases of liver-based metabolic defects. This is a case report of a patient with hemophilia B who was treated since childhood with factor IX replacement for recurrent hemarthroses. Subsequent hepatitis B (HBV) and C (HCV) infection had resulted in the development of chronic active hepatitis, ultimately leading to cirrhosis. Orthotopic liver transplantation performed for endstage liver disease resulted in a rise in factor IX levels from 2% to 83% of normal values within 24 h postoperatively, and levels remained above 90% of normal values after postoperative day 3 without factor IX replacement. To our knowledge, only two cases of hemophilia B treated by orthotopic liver transplantation have been reported. This procedure has, however, only been implemented in cases of terminal liver insufficiency in hemophiliacs.
authors.affiliation: Department of Surgery, University Hospital, Geneva, Switzerland.
authors.forename: Z
authors.initials: Z
authors.lastname: Kadry
authors.forename: P
authors.initials: P
authors.lastname: de Moerloose
authors.forename: E
authors.initials: E
authors.lastname: Giostra
authors.forename: P
authors.initials: P
authors.lastname: Morel
authors.forename: O
authors.initials: O
authors.lastname: Huber
authors.forename: E
authors.initials: E
authors.lastname: Meili
authors.forename: H E
authors.initials: HE
authors.lastname: Blum
authors.forename: G
authors.initials: G
authors.lastname: Mentha
mesh_terms: D016913:Blood Component Transfusion / Q000009:adverse effects; D002836:Hemophilia B / Q000150:complications / Q000601:surgery* / Q000628:therapy; D006509:Hepatitis B / Q000150:complications / Q000209:etiology; D006526:Hepatitis C / Q000150:complications / Q000209:etiology; D006801:Humans; D017093:Liver Failure / Q000209:etiology* / Q000601:surgery* / Q000821:virology; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged