نتایج جستجو برای: bernard soulier syndrome
تعداد نتایج: 627301 فیلتر نتایج به سال:
cally confirmed Bernard-Soulier syndrome and MYH9 disorders2 were 5.3 ( 1.1) m (n 14) and 4.9 ( 1.1) m (n 81), respectively. These data are comparable with that of the patient in our study.1 Because routine automated blood cell counting systems differentiate blood cells by their size and therefore do not recognize giant platelets as platelets, mean platelet volume (MPV) does not reflect actual ...
OBJECTIVE The aim of this study was to assess the effectiveness of localized treatments to persistently stop epistaxis in patients with inherited bleeding disorders. METHODS In a self-controlled comparative clinical trial, to offer the best solution to stop epistaxis at home (within 10 minutes), patients with inherited bleeding disorders were treated using three different topical hemostatic a...
We describe glycoprotein (GP) Ib as a mediator of adhesion to fibronectin, specifically in flow. A monoclonal antibody (MoAb) directed to the von Willebrand factor (vWF)-binding site on this receptor or the absence of this receptor on the platelet membrane, in the case of a patient with the Bernard-Soulier syndrome, reduced platelet coverage to fibronectin to approximately 30% of the control va...
Different types of platelets in various types of plasma were subjected to levels of shear stress that produce irreversible platelet aggregation in normal platelet-rich plasma (PRP). At shear stresses of 90 or 180 dyne/cm2 applied for 30 seconds or five minutes, aggregation was either absent or only transient and reversible using severe von Willebrand's disease (vWD) PRP (less than 1% von Willeb...
Adhesion of platelets to extracellular matrix via von Willebrand factor (vWF) and activation of platelets by thrombin are critical steps in hemostasis. Glycoprotein (GP) V is a component of the GPIb-V-IX complex, the platelet receptor for vWF. GPV is also cleaved by thrombin. Deficiency of GPIb or GPIX results in Bernard-Soulier syndrome (BSS), a bleeding disorder in which platelets are giant a...
The search for the components of the platelet surface that mediate platelet adhesion and platelet aggregation began for earnest in the late 1960s when electron microscopy demonstrated the presence of a carbohydrate-rich, negatively charged outer coat that was called the "glycocalyx." Progressively, electrophoretic procedures were developed that identified the major membrane glycoproteins (GP) t...
The diagnosis of von Willebrand disease (VWD), the most common inherited bleeding disorder, is characterised by a variable bleeding tendency and heterogeneous laboratory phenotype. The sequencing of the entire VWF coding region has not yet become a routine practice in diagnostic laboratories owing to its high costs. Nevertheless, next-generation sequencing (NGS) has emerged as an alternative to...
Human Factor VIII desialylated by treatment with Vibrio cholerae neuraminidase (ASVIII) aggregated human platelets in the absence of ristocetin in platelet-rich plasma and, to a lesser extent, in washed platelet suspensions. Aggregation is accompanied by thromboxane formation and is completely inhibited by EDTA. Aspirin blocks the second phase of aggregation and abolishes thromboxane production...
Bostjan Kobe THE UNIVERSITY OF QUEENSLAND Understanding the mechanism of platelet activation requires the knowledge of the structural organization of the glycoprotein (GP) Ib-IX-V receptor. In this issue of Blood, an innovative structural approach was used to shed light on the interaction between GPIb and GPIX subunits.1 The GPIb-IX-V complex serves as the platelet receptor that mediates bindin...
cally confirmed Bernard-Soulier syndrome and MYH9 disorders2 were 5.3 ( 1.1) m (n 14) and 4.9 ( 1.1) m (n 81), respectively. These data are comparable with that of the patient in our study.1 Because routine automated blood cell counting systems differentiate blood cells by their size and therefore do not recognize giant platelets as platelets, mean platelet volume (MPV) does not reflect actual ...
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