title: Use of rFVIIa in 4 children with Glanzmann thrombasthenia.
abstract: Inherited deficiencies of platelet surface glycoproteins, such as Glanzmann thrombasthenia (GT), occasionally result in severe bleeding episodes. Platelet transfusion is considered standard therapy for securing hemostasis in subjects with GT when local measures and antifibrinolytic agents are inadequate. We describe 4 case studies which suggest that recombinant activated factor VII may be an effective alternative to platelet transfusion in preventing or controlling bleeding, including surgical bleeding, in patients with GT.
authors.affiliation: Hemophilia and Thrombophilia Center, Rush University Medical Center and Rush Children's Hospital, 1653 West Congress Parkway, Chicago, IL 60612-3833, USA. lvalentino@rush.edu
authors.forename: Leonard A
authors.initials: LA
authors.lastname: Valentino
mesh_terms: D000293:Adolescent; D002648:Child; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005167:Factor VII / Q000008:administration & dosage / Q000627:therapeutic use*; D015942:Factor VIIa; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000627:therapeutic use; D012680:Sensitivity and Specificity; D013915:Thrombasthenia / Q000175:diagnosis / Q000188:drug therapy* / Q000601:surgery; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D011994:Recombinant Proteins; D005167:Factor VII; C103587:recombinant FVIIa; D015942:Factor VIIa