Perioperative management of a pediatric patient with Glanzmann's thrombasthenia during adenoidectomy.
OBJECTIVE
Glanzmann's thrombasthenia (GT) is an autosomal recessively inherited platelet disorder. There is not any specific treatment. Platelet transfusion is currently the standard treatment when bleeding does not respond to local measures and/or antifibrinolytic treatment, although it may result in alloimmunization. Recombinant activated factor VII (rFVIIa) might be used to avoid recurrent platelet transfusion.


METHODS
We present early treatment with low-dose rFVIIa additional to platelet transfusion in a 5-year-old pediatric case with diagnosis of GT who developed prolonged bleeding under an elective adenoidectomy surgery. A total dose of 1,200μg (60μg.kg(-1)) rFVIIa could successfully stop bleeding, what can be accepted as low dose usage.


CONCLUSIONS
Such case reports may encourage the use of early treatment with low doses of rFVIIa in severe bleeds that did not stop despite of platelet transfusion, as well as in preventing bleeding in surgical procedures in patients with GT. Actually, additional studies are needed to define the minimal effective dose and attempts to determine the lowest effective dose may be encouraged by the result of this case, considering financial restrictions in the health care system.
Faculty of Medicine, Department of Anesthesiology, Karadeniz Technical University, Trabzon, Turkey. dumanen@gmail.com
Erdem Nail
EN
Duman
Sedat
S
Saylan
Bahanur
B
Cekic
D000233:Adenoidectomy; D002675:Child, Preschool; D003131:Combined Modality Therapy; D015942:Factor VIIa / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D017713:Platelet Transfusion; D011182:Postoperative Care; D019106:Postoperative Hemorrhage / Q000209:etiology* / Q000628:therapy*; D011994:Recombinant Proteins / Q000627:therapeutic use; D013915:Thrombasthenia / Q000150:complications*
D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa