title: Prophylactic recombinant factor VIIa administration to an infant with congenital systemic juvenile xanthogranuloma.
abstract: We report the case of an infant affected with congenital systemic juvenile xanthogranuloma scheduled for central venous access system implantation (Port-a-Cath) and a liver and bone marrow biopsy. The patient had impaired liver function, thrombocytopenia, and coagulopathy which was refractory to daily fresh-frozen plasma and platelet infusions: 80 microg x kg(-1) dose(-1) of recombinant factor VIIa (rFVIIa) was administered i.v. every 2 h starting 30 min before the procedure and ending 6 h afterwards. Very minor bleeding was observed during the procedure. In conclusion, rFVIIa therapy was effective as prophylaxis for both invasive procedures in this patient with a coagulopathy which was refractory to other different therapies.
authors.affiliation: Department of Anesthesiology, Children University Hospital Niño Jesus, Madrid, Spain. jdesantiago@telefonica.net
authors.forename: Jesus
authors.initials: J
authors.lastname: De Santiago
authors.forename: Ernesto
authors.initials: E
authors.lastname: Martinez-Garcia
authors.forename: Jorge
authors.initials: J
authors.lastname: Giron
authors.forename: Concepción
authors.initials: C
authors.lastname: Salcedo
authors.forename: Antonio
authors.initials: A
authors.lastname: Pérez-Gallardo
mesh_terms: D015942:Factor VIIa / Q000008:administration & dosage* / Q000627:therapeutic use*; D005260:Female; D006470:Hemorrhage / Q000150:complications / Q000517:prevention & control*; D006801:Humans; D007231:Infant, Newborn; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000627:therapeutic use; D014972:Xanthogranuloma, Juvenile / Q000150:complications / Q000151:congenital* / Q000601:surgery*
chemical_list: D011994:Recombinant Proteins; D015942:Factor VIIa