Chabchoub Ben Abdallah R, Trabelsi L, Ben Ameur S, Kammoun F, Boukédi A, Ben Salah M, Ben Hlima N, Mahfoudh A
Service pédiatrie, urgences et réanimation pédiatriques, CHU Hédi Chaker, Route El Ain Km 0.5, Sfax, Tunisia.
Arch Pediatr. 2010 Jul;17(7):1062-4. doi: 10.1016/j.arcped.2010.04.006. Epub 2010 Jun 8.
Glanzmann thrombasthenia (TG) is a congenital platelet function disorder characterized by frequent and occasionally severe bleeding events. Treatment is based on platelet transfusion at the time of bleeding. We report a case of GT revealed in the neonatal period, a severe hemorrhagic syndrome refractory to transfusions, treated at the age of 6 years. Activated recombinant factor VII (Novoseven) injections were necessary. The advantages of recombinant activated factor VII in GT patients with platelet antibodies and/or platelet transfusions refractoriness are discussed.