Glanzmann thrombasthenia is an inherited bleeding disorder characterized by qualitative or quantitative defects of the platelet-specific integrin, alphaIIbbeta(3). As a result, alphaIIbbeta(3) cannot be activated and cannot bind to fibrinogen, leading to a loss of platelet aggregation. Thrombasthenia is clinically characterized by mucocutaneous hemorrhage with episodes of intracranial and gastr...

A sensitive immunoblot technique for platelet glycoprotein IIIa (GPIIIa) was used to analyze the platelets of patients living in Israel who meet the diagnostic criteria for type I Glanzmann thrombasthenia. When reacted with solubilized normal platelets, a rabbit antiserum to GPIIIa identified a major band at molecular weight (mol wt) 90,000 and three additional minor bands at Mr 110,000, 81,000...

Introduction: La thrombasthénie de Glanzmann est une pathologie hémorragique héréditaire rare due à une déficience ou un dysfonctionnement du complexe glycoprotéique IIb/IIIa de la membrane plaquettaire. Le but de notre étude est de décrire les caractéristiques démographiques, cliniques et biologiques d'une série de patients atteints de thrombasthénie de Glanzmann. Méthodes: C'est une étude por...

Glanzmann thrombasthenia is an autosomal recessive bleeding disorder characterized by a decrease or absence of functional platelet glycoprotein (GP) IIb-IIIa (alpha IIb beta 3) integrin receptors. Although thrombasthenia is a rare disorder, its occurrence is increased in some regions of the world where intracommunity marriage and consanguinity are commonplace, resulting in increased expression ...

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Glanzmann thrombasthenia is an autosomal recessive disorder of the platelet glycoproteins (GP) lIb and lila. These glycoproteins normally serve as receptors for other adhesive glycoproteins. including fibrinogen, von Willebrand factor, and fibronectin. Most patients affected by Glanzmann thrombasthenia have low levels of GPIIb and GPIIIa; however. the separate mechanisms responsible for the def...

The general practitioner (GP) fundholding scheme was introduced as part of the Conservative governments 1991 National Health Service reforms and abolished by the Labour government in 1998. This paper contends that the scheme was introduced and abolished without policy-makers having any valid evidence of its effects. In particular, it focuses on the salient features of the decision to abolish. T...

Background—Platelets have been suggested to play a role in the early development of atherosclerosis. As one test of this hypothesis, we assessed whether patients with Glanzmann thrombasthenia who lack platelet glycoprotein IIb 3 (GPIIb/IIIa) complexes or both IIb 3 and the more ubiquitous v 3 cell membrane complexes are protected from development of atherosclerosis. Methods and Results—Seven pa...