نتایج جستجو برای: glanzmanns thrombasthenia

تعداد نتایج: 525  

Journal: :Blood 1995
H Peretz N Rosenberg S Usher E Graff P J Newman B S Coller U Seligsohn

Glanzmann's thrombasthenia is a bleeding disorder characterized by a decrease or absence of the functional platelet membrane glycoprotein (GP) complex, GPIIb/IIIa (alpha IIb beta 3). We describe a new deletion-insertion mutation in the GPIIb gene causing type I Glanzmann's thrombasthenia in two siblings of a consanguineous Iranian-Jewish family. The proband's platelets bound more antibodies aga...

Journal: :The Journal of clinical investigation 1986
L A Miles M H Ginsberg J G White E F Plow

Glu-plasminogen, the native form of plasminogen, interacts in a specific and saturable manner with unstimulated human platelets, and the binding is enhanced fivefold by thrombin stimulation (Miles and Plow, 1985. J. Biol. Chem. 260:4303). This study characterizes the nature of the Glu-plasminogen binding sites by analyzing platelets deficient in selected proteins and functions. Platelets from p...

Journal: :Haematologica 2009
Paolo Gresele Emanuela Falcinelli Silvia Giannini Pio D'Adamo Angela D'Eustacchio Teresa Corazzi Anna Maria Mezzasoma Filomena Di Bari Giuseppe Guglielmini Luca Cecchetti Patrizia Noris Carlo L Balduini Anna Savoia

BACKGROUND Defects of integrin alpha(IIb)beta(3) are typical of Glanzmann's thrombasthenia, an inherited autosomal recessive bleeding disorder characterized by the failure of platelets to aggregate in response to all physiological agonists, but with no abnormalities in the number or size of platelets. Although large heterogeneity has been described for Glanzmann's thrombasthenia, no family has ...

Journal: :The Journal of clinical investigation 1983
M H Ginsberg J Forsyth A Lightsey J Chediak E F Plow

Thrombin stimulation results in increased surface expression of endogeneous fibronectin and binding of plasma fibronectin to human platelets. Platelets of patients with Glanzmann's thrombasthenia, a bleeding disorder, exhibit reduced thrombin-induced platelet aggregation, little or no clot retraction, and abnormal platelet spreading on glass surfaces. Thrombin stimulation of patient platelets f...

2002
Barry S. Coller

A 20-year-old woman from a consanguineous family in the Hunan Province of the People‘s Republic of China was diagnosed as having Glanzmann‘s thrombasthenia based on (1) nearly a lifelong history of epistaxis, gum bleeding, petechiae, and purpura; (2) severe menorrhagia resulting in anemia and need for whole-blood transfusion; (3) normal coagulation assays; (4) prolonged bleeding time; (5) absen...

Journal: :The Journal of Experimental Medicine 1994
Y Gruel E Brojer D J Nugent T J Kunicki

A patient (OG) with Glanzmann thrombasthenia became refractory to platelet transfusion after the production of an immunoglobulin G (IgG) isoantibody (Ab1) specific for the integrin subunit beta 3. To determine the frequency at which the OG idiotype is found in the general population and in immune-mediated disease states, we developed a rabbit polyclonal antibody (Ab2) specific for affinity-puri...

Journal: :Seminars in Thrombosis and Hemostasis 2013

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