title: Successful use of ReFacto continuous infusion in two paediatric patients with severe haemophilia A undergoing orthopaedic surgery.
abstract: In this report we describe the successful use of B-domain-deleted recombinant factor VIII (ReFacto) administered by continuous infusion during orthopaedic procedures in two children with severe haemophilia A. Both patients underwent ankle synovectomy and in patient 2, a medial patello-femoral ligament repair was performed in the same operative session. Patient 2 developed septic arthritis A in his knee joint and arthroscopic joint irrigation and debridement was performed 2 weeks after the initial procedure. Surgical cover was initiated with a bolus dose of ReFacto 50 IU kg(-1) followed by continuous infusion at 3.3-4.8 IU kg(-1) h(-1) which was maintained for up to 9 days postoperatively. Patient 2 received an additional bolus dose of 15 IU kg(-1) during the infusion period. All procedures were performed without haemostatic complications and long-term orthopaedic outcomes were good in both patients.
authors.affiliation: Department of Pediatrics, University of Virginia, Charlottesville, VA, USA.
authors.forename: M M
authors.initials: MM
authors.lastname: Sennett
authors.forename: P A
authors.initials: PA
authors.lastname: de Alarcón
mesh_terms: D000293:Adolescent; D000843:Ankle Joint; D016063:Blood Loss, Surgical / Q000517:prevention & control; D002648:Child; D002908:Chronic Disease; D005169:Factor VIII / Q000008:administration & dosage*; D006395:Hemarthrosis / Q000209:etiology / Q000601:surgery*; D006467:Hemophilia A / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D007262:Infusions, Intravenous; D007719:Knee Joint; D008297:Male; D010146:Pain / Q000209:etiology / Q000601:surgery; D010329:Patella / Q000601:surgery; D012008:Recurrence; D000074402:Synovectomy; D013585:Synovitis / Q000601:surgery*
chemical_list: C427184:recombinant factor VIII SQ; D005169:Factor VIII