Glanzmann's Thrombasthenia
Divalent cation regulation of the surface orientation of platelet membrane glycoprotein IIb Correlation with fibrinogen binding function and definition of a novel variant of Glanzmann's thrombasthenia
... heterodimer, because it occurred at 22 degrees C and in response to mM magnesium as well as calcium. PMI-1 binding inversely correlated with fibrinogen binding. In addition, we identified a variant of Glanzmann's ...
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www.ijcep.com /IJCEP911005 Case Report Glanzmann’s thrombasthenia: report of a case and review of the literature
... Cutaneous purpura usually arise as a result of pressure or minor trauma, and truly spontane- ous purpura are rare [5]. As in normal patients epistaxis is common in childhood and usually subsides by adulthood, but it is ...
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Glanzmann’s Thrombasthenia: Role of Angioembolization and factor VII in recurrent bleeding from ulcerated duodenum
... Glanzmann’s thrombasthenia (GT), a rare functional disorder of platelets, inherited in autosomal recessive pattern with only few acquired cases reported (Varkey, ...
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A deletion in the gene for glycoprotein IIb associated with Glanzmann's thrombasthenia
... aggregation, resulting in Glanzmann's thrombasthenia (GT). In this paper, we describe a black thrombasthenic patient who is either homozygous or hemizygous for a deletion within the GPIIb gene. Initial Western ...
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Density of Platelet GPIIb-IIIa and Bleeding Severity in Iranian Patients with Glanzmann’s Thrombasthenia
... of bleeding in affected individuals cannot readily be predicted from currently available laboratory findings. Indeed, some patients have never had serious bleeding, and most have been healthy and free of bleeding ...
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Methylenetetrahydrofolate Reductase Polymorphisms in Iranian Patients with Glanzmann’s Thrombasthenia
... Material and Methods: In the present case-control study which performed at Pathology and Stem Cell Research Center at Kerman University of Medical Sciences, 65 patients with GT and 100 normal voluntary blood donors as ...
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Inheritance of the human platelet alloantigen, PlA1, in type I Glanzmann's thrombasthenia
... The hereditary of the human platelet alloantigen, PlA1, has been studied in Glanzmann's thrombasthenia. The PlA1 content of platelets from three patients, 20 kindred of these patients, including parents and ...
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Deletion of the Platelet Specific Alloantigen PlA1 from Platelets in Glanzmann's Thrombasthenia
... Expression of a Platelet-specific alloantigen (Pl A1 ) was studied in five unrelated patients with Glanzmann's thrombasthenia using immunologic techniques based on release of 51 Cr from tagged platelets by Pl A1 ...
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Diagnosis of Bernard Soulier syndrome and Glanzmann's thrombasthenia with a monoclonal assay on whole blood
... samples from six patients with Glanzmann's thrombasthenia and three patients with Bernard-Soulier syndrome. Patients with type I and type II Glanzmann's thrombasthenia were easily detectable with this ...
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Levonorgestrel-releasing intrauterine system for treatment of heavy menstrual bleeding in adolescents with Glanzmann’s Thrombasthenia: illustrated case series
... Background: Glanzmann ’ s Thrombasthenia (GT) is an inherited genetic disorder caused by defects in the platelet membrane glycoproteins IIb/IIIA, and is associated with heavy menstrual bleeding ...
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Hemorrhage Treatment Report of Patients Suffering from Glanzmann’s Thrombasthenia Resulting Hospitalization from 2006 to 2011 at Mofid Children’s Hospital
... Glanzmann’s thrombasthenia patients from 2006 to 2011 who were admitted to the Mofid Children’s Hospital, Tehran, Iran for controlling their bleeding or for surgery procedures were ...
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A new variant of Glanzmann's thrombasthenia (Strasbourg I) Platelets with functionally defective glycoprotein IIb IIIa complexes and a glycoprotein IIIa 214Arg 214Trp mutation
... We describe a new variant of Glanzmann's thrombasthenia (variant Strasbourg I). The patient (M.S.) showed an absence of platelet aggregation to ADP, thrombin, and collagen, and a decreased clot retraction. ...
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A variant of Glanzmann's thrombasthenia with abnormal glycoprotein IIb IIIa complexes in the platelet membrane
... example, after incubation with EDTA at room temperature, the patient's platelets bound little of the monoclonal antibodies AP-2 or T10 (anti-GP IIb-IIIa complex) although normally binding Tab (anti-GP IIb). Patient C.M. ...
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Immunochemical Evidence for Protein Abnormalities in Platelets from Patients with Glanzmann's Thrombasthenia and Bernard Soulier Syndrome
... The most prominent precipitate, 16, and platelet fibrinogen, 24 were not detected in platelets of two patients with type I thrombasthenia, whereas in one patient with type II thrombasthe[r] ...
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Platelet Membrane Defects in Glanzmann's Thrombasthenia: EVIDENCE FOR DECREASED AMOUNTS OF TWO MAJOR GLYCOPROTEINS
... Comparison of the labeled polypeptides of normal and thrombasthenic samples after reduction indicated decreased labeling of two major glycoproteins in thrombasthenic platelets: IIb and I[r] ...
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Autosomal recessive inherited bleeding disorders in Pakistan: a cross-sectional study from selected regions
... GT: Glanzmann ’ s thrombasthenia; NIBD: National Institute of Blood Diseases and Bone Marrow Transplantation; PFD: Platelet functional disorder; PT: Prothrombin time; VKCFD: Vitamin K-dependent ...
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A two amino acid insertion in the Cys146 Cys167 loop of the alphaIIb subunit is associated with a variant of Glanzmann thrombasthenia Critical role of Asp163 in ligand binding
... Glanzmann’s thrombasthenia (Stras- berg I): platelets with functionally defective glycoprotein IIb-IIIa complexes and a glycoprotein IIIa 214 Arg→ 214 Trp ...
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Isolation and Quantitation of the Platelet Membrane Glycoprotein Deficient in Thrombasthenia Using a Monoclonal Hybridoma Antibody
... abnormality in Glanzmann's thrombasthenia. Spleen cells from Balb/c mice immunized with human platelets were fused to mouse myeloma cell line Sp2/0-Ag14. Hybridoma lines producing a variety of antiplatelet ...
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Recognition and management of platelet-refractory bleeding in patients with Glanzmann’s thrombasthenia and other severe platelet function disorders
... Abstract: Patients with rare qualitative platelet disorders or platelet function disorders (PFDs) may present to the hospital physician with severe bleeding episodes or excessive surgical bleed- ing. Although standard ...
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Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options
... 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 ...
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