The ligand binding site(s) of the a subunit of integrin a IIb b 3 (GPIIb-IIIa), a prototypic non–I domain integrin, remains elusive. In this study, we have characterized a Japanese variant of Glanzmann thrombasthenia, KO, whose platelets express normal amounts of a IIb b 3 . KO platelets failed to bind the activation-independent ligand-mimetic mAb OPG2 and did not bind fibrinogen or the activat...

Glanzmann's thrombasthenia (GT) is a genetic platelet surface receptor disorder of GPIIb/IIIa (ITG αIIbβ3), either qualitative or quantitative, which results in faulty platelet aggregation and diminished clot retraction. Spontaneous mucocutaneous bleeding is common and can lead to fatal bleeding episodes. Control and prevention of bleeding among patients with GT is imperative, and remains chall...

BACKGROUND Glanzmann thrombasthenia (GT) is an exceedingly rare but well-defined inherited disorder of platelet function caused by a defect in the glycoprotein IIb/IIIa complex. The association of GT with consanguinity has been noted, especially in geographic regions in which intermarriage is common. In most patients, GT is diagnosed during early infancy or before the age of 5 years. Common man...

Glanzmann thrombastenia (GT) is a rare disease of an autosomal recessive inheritance characterized with fatal bleeding tendency. The anaesthesiologist should be cognizant of the risk involved and be prepared with necessary measures. In this paper, we present a GT case of a 9-year-old male with hypospadias, which was successfully repaired after platelet transfusions according to the thromboelast...

GT, a rare disorder of platelet function, can lead to life-threatening bleeding, particularly following the development of antiplatelet antibodies. Curative therapy includes HCT but previous reports are limited predominantly to matched siblings and have excluded CBT. Delayed or non-engraftment of platelets because of antiplatelet antibodies might be particularly concerning after CBT for GT. Her...

ttp://dx.doi.org/10.1016/j.bjorl.2014.08.017 808-8694/© 2014 Associação Brasileira de Otorrinolaringologia e Cirurg eserved. ther examination results were unremarkable. The patient’s istory was clear from trauma, barotrauma, chronic otitis edia, or anticoagulant therapy. An audiogram revealed ymmetrical, bilateral conductive hearing loss (Fig. 1C). ympanometry revealed flat bilateral tympanogra...