title: [A Jehovah's Witness child with hemophilia B and factor IX inhibitors undergoing scoliosis surgery].
abstract: OBJECTIVE
  To describe the successful perioperative hemostatic management of a Jehovah's Witness patient with hemophilia B and anaphylactic inhibitors to factor IX, undergoing scoliosis surgery.
  
  
  METHODS
  A 14 (1/2)-yr-old boy with severe hemophilia B who had a history of anaphylactic inhibitors to factor IX was scheduled to undergo corrective scoliosis surgery. He was initially started on epoetin alfa and iron supplementation to maximize preoperative red cell mass. Additionally, he was placed on a desensitization protocol of recombinant coagulation factor IX (rFIX) and was then treated with activated recombinant coagulation factor VII (rFVIIa) during the postoperative period. Tranexamic acid was given concomitantly. The intraoperative blood loss was approximately 350 mL. The nadir hemoglobin concentration was 111 g.L(-1) on postoperative days one and two. On postoperative day 11, the patient was stable and discharged home with a hemoglobin of 138 g.L(-1). He did not require blood transfusion and no adverse events were observed.
  
  
  CONCLUSIONS
  The use of rFIX, rFVIIa, erythropoetin, iron, and tranexamic acid before, during and after scoliosis surgery may be a viable and safe option for hemophilia patients with inhibitors, who refuse blood products.
authors.affiliation: Faculty of Medicine, University of British Columbia, British Columbia Children's Hospital, Vancouver, British Columbia, Canada.
authors.forename: Anthony
authors.initials: A
authors.lastname: Chau
authors.forename: John
authors.initials: J
authors.lastname: Wu
authors.forename: Mark
authors.initials: M
authors.lastname: Ansermino
authors.forename: Stephen
authors.initials: S
authors.lastname: Tredwell
authors.forename: Robert
authors.initials: R
authors.lastname: Purdy
mesh_terms: D000293:Adolescent; D000933:Antifibrinolytic Agents / Q000008:administration & dosage; D016063:Blood Loss, Surgical / Q000517:prevention & control / Q000706:statistics & numerical data; D019587:Dietary Supplements; D000068817:Epoetin Alfa; D004921:Erythropoietin / Q000008:administration & dosage; D005164:Factor IX / Q000008:administration & dosage / Q000037:antagonists & inhibitors*; D005167:Factor VII / Q000008:administration & dosage; D005500:Follow-Up Studies; D006397:Hematinics / Q000008:administration & dosage; D006454:Hemoglobins; D002836:Hemophilia B / Q000150:complications*; D006801:Humans; D007501:Iron / Q000008:administration & dosage; D033221:Jehovah's Witnesses; D008297:Male; D011994:Recombinant Proteins; D012600:Scoliosis / Q000150:complications / Q000601:surgery*; D014131:Trace Elements / Q000008:administration & dosage; D014148:Tranexamic Acid / Q000008:administration & dosage
chemical_list: D000933:Antifibrinolytic Agents; D006397:Hematinics; D006454:Hemoglobins; D011994:Recombinant Proteins; D014131:Trace Elements; D004921:Erythropoietin; D000068817:Epoetin Alfa; D014148:Tranexamic Acid; D005167:Factor VII; D005164:Factor IX; D007501:Iron