نتایج جستجو برای: glanzmanns thrombasthenia

تعداد نتایج: 525  

Journal: :Pathophysiology of haemostasis and thrombosis 2002
Uri Seligsohn

Glanzmann thrombasthenia (GT) is an autosomal recessive bleeding disorder characterized by deficient or dysfunctional glycoprotein (GP) IIb/IIIa compexes. The hallmark of the disease is impaired platelet aggregation stemming from defective fibrinogen binding to GPIIb/IIIa. Based on deciphering the abnormality in GT a monoclonal antibody, peptides and peptidominetic agents, all interfering with ...

Journal: :Kidney International Reports 2021

Crohn's disease (CD), a polygenic disorder, is the major subtype of inflammatory bowel disease. There heterogeneity and many patients present extraintestinal manifestations. However, association crohn's with another genetic has not been previously described. We report exceptional CD Glanzmann’s thrombasthenia which rare inherited bleeding disorder. Indeed, diagnosis in patient kidney amylosis, ...

Journal: :Japanese Journal of Thrombosis and Hemostasis 1997

Journal: :Blood 1990
P F Bray M A Shuman

The platelet fibrinogen receptor, which is composed of glycoproteins IIb (GPIIb) and IIIa (GPIIIa), belongs to a large family of receptors that participate in a multitude of biologically important adhesive interactions. Platelets from most patients with the autosomal recessive bleeding disorder, Glanzmann's thrombasthenia, are deficient in GPIIb and GPIIIa. We have used cDNA probes to analyze t...

Journal: :Proceedings of the Royal Society of Medicine 1970

Journal: :Journal of Clinical Pathology 1968

Journal: :Journal of Clinical Case Reports 2012

Journal: :Journal of obstetrics and gynaecology of India 2014
Chandrakala Magudapathi Suthanthira Kannan

Glanzmann’s thrombasthenia is a rare autosomal recessive hemorrhagic disorder caused by abnormal platelet glycoprotein complex (GP IIb-IIIa) presenting with hemorrhagic symptoms. Pregnancy is not uncommon because fertility is not affected but an association is rare. Also delivery often results in haemorrhage. Newborn thrombocytopenia is occasionally severe, but is always transitory. We report a...

Journal: :Haematologica 2015
Man-Chiu Poon Roseline d'Oiron Rainer B Zotz Niels Bindslev Matteo Nicola Dario Di Minno Giovanni Di Minno

Standard treatment for Glanzmann thrombasthenia, a severe inherited bleeding disorder, is platelet transfusion. Recombinant factor VIIa is reported to be effective in Glanzmann thrombasthenia with platelet antibodies and/or refractoriness to platelet transfusions. We aimed to evaluate recombinant factor VIIa effectiveness and safety for the treatment and prevention of surgical bleeding in patie...

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