title: [Successful management of neurosurgical procedures with continuous infusion of recombinant factor IX in a child with hemophilia B].
abstract: This report describes the successful management of neurosurgical procedures with continuous infusion of recombinant factor IX (rFIX). A 1-year-old boy with severe hemophilia B was administered prophylactic therapy with rFIX after intracranial bleeding. We found the enlargement of an arachnoid cyst in a follow-up CT scan. He underwent marsupialization of the cyst under the continuous infusion of rFIX. FIX levels were examined in our hospital and the rFIX infusion rate was adjusted in an attempt to keep FIX levels above 90% intraoperatively, and 70% until his 7th post-operative day. We studied the pharmacokinetic profile of rFIX and found a half-time of 25 hours and mean in vivo recovery of 0.69 IU/dl/IU/kg. Reconstituted rFIX also retained at least 95% activity after 72 hours at room temperature. This is the first report of the perioperative management of a child undergoing a neurosurgical procedure under the continuous infusion of rFIX in Japan. Further studies are required before the routine use of this product for continuous infusion.
authors.affiliation: Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Japan.
authors.forename: Mariko
authors.initials: M
authors.lastname: Yamamoto
authors.forename: Hisaya
authors.initials: H
authors.lastname: Nakadate
authors.forename: Umefumi
authors.initials: U
authors.lastname: Iguchi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Masuda
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Sakai
authors.forename: Akira
authors.initials: A
authors.lastname: Ishiguro
mesh_terms: D005164:Factor IX / Q000008:administration & dosage / Q000493:pharmacokinetics* / Q000627:therapeutic use*; D002836:Hemophilia B / Q000188:drug therapy / Q000601:surgery*; D006801:Humans; D007223:Infant; D007564:Japan; D008297:Male; D019635:Neurosurgical Procedures / Q000379:methods; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D005164:Factor IX