Erratum: Clinical utility gene card for: Glanzmann thrombasthenia

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Glanzmann thrombasthenia

Glanzmann thrombasthenia (GT) is a rare autosomal recessive bleeding syndrome affecting the megakaryocyte lineage and characterized by lack of platelet aggregation. The molecular basis is linked to quantitative and/or qualitative abnormalities of alphaIIb beta3 integrin. This receptor mediates the binding of adhesive proteins that attach aggregating platelets and ensure thrombus formation at si...

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Glanzmann thrombasthenia is a qualitative platelet function disorder manifested by skin bleeds, epistaxis, gingival bleeding, gastrointestinal hemorrhage, hematuria, hemarthrosis, intracranial hemorrhage and visceral hematomas. We report a six day old newborn presenting with hematuria following suprapubic aspiration, who was diagnosed as Glanzmann thrombasthenia. We believe it to be the younges...

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Glanzmann Thrombasthenia: A Clinicopathological Profile.

OBJECTIVE To describe the clinical presentation of patients with Glanzmann's thrombasthenia (GT) and evaluate their diagnostic, clinical, and laboratory parameters including platelet aggregometry. STUDY DESIGN Descriptive study. PLACE AND DURATION OF STUDY Department of Hematology and Blood Transfusion, The Children Hospital and Institute of Child Health, Lahore, from January 2006 to Decemb...

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Patients With Glanzmann Thrombasthenia Lacking Platelet Glycoprotein

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...

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Glanzmann thrombasthenia in a compound heterozygote for the α IIb gene . A novel missense

Glanzmann thrombasthenia is an autosomal recessive bleeding disorder characterized by a life-long hemorrhagic tendency and absent or severely reduced platelet aggregation in response to agonists. The thrombasthenic phenotype is associated with quantitative or qualitative abnormalities in the platelet fibrinogen receptor, the αIIbβ3 integrin or glycoprotein (GP) IIb-IIIa, which can also serve as...

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ژورنال

عنوان ژورنال: European Journal of Human Genetics

سال: 2012

ISSN: 1018-4813,1476-5438

DOI: 10.1038/ejhg.2012.178