نتایج جستجو برای: glanzmanns thrombasthenia

تعداد نتایج: 525  

Journal: :Blood 1990
J N George J P Caen A T Nurden

LANZMANN’S THROMBASTHENIA is a wellG defined inherited disorder of platelet function.’.” It is caused by a deficiency or abnormality of the membrane glycoprotein (GP) IIb-IIIa complex with bleeding due to defective platelet hemostatic plug formation. Thrombasthenia has achieved much recognition for such a rare disease because it has been important in defining G P IIb-IIIa as a platelet receptor...

Journal: :Orphanet Journal of Rare Diseases 2006

Faranoush M, Farsinezhad A, Hemmati T, Managhchi MR, Toogeh G,

Background: Activated normal platelets undergo many biochemical and morphological changes, some of which are apoptotic. Platelet derived microparticles and shrinked platelets as hallmark of platelet activation and apoptosis disperse surfaces containing procoagulant activity around injured vessels and tissues. This study was conducted to determine microparticles formation and platelet shrinkage ...

2015
J.W. Norris M. Pombo E. Shirley G. Blevins F. Tablin

BACKGROUND Two congenital bleeding diatheses have been identified in Thoroughbred horses: Glanzmann thrombasthenia (GT) and a second, novel diathesis associated with abnormal platelet function in response to collagen and thrombin stimulation. HYPOTHESIS/OBJECTIVES Platelet dysfunction in horses with this second thrombasthenia results from a secretory defect. ANIMALS Two affected and 6 clini...

Journal: :Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation 2016
Mani Ramzi Mehdi Dehghani Shirin Haghighat Hourvash Haghighi Nejad

Glanzmann thrombasthenia is an inherited auto-somal recessive disorder characterized by normal platelet count but lack of platelet aggregation due to absence of platelet glycoprotein IIb/IIIa. The disease usually is associated with mild bleeding, but severe fatal hemorrhage may occur. Allogeneic stem cell transplant is the only curative method of treatment. A literature search showed 18 previou...

Journal: :International journal of advanced research 2022

Glanzmann thrombasthenia is a rare genetic disorder. It of autosomal inheritance. usually presents as ecchymoses, petechiae, gum bleeding, musical and menorrhagia. An acute episode bleeding can be managed with intravenous antifibrinolytics, blood products transfusion later hormonal therapy. Newer modalities include Recombinant factor VIIa. A multidisciplinary approach needed for the diagnosis m...

2002
Barry S. Coller

http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#repub_requests Information about reproducing this article in parts or in its entirety may be found online at: http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#reprints Information about ordering reprints may be found online at: http://bloodjournal.hematologylibrary.org/site/subscriptions/index.xhtml Information abo...

Journal: :Journal of the College of Physicians and Surgeons--Pakistan : JCPSP 2008
Natasha Ali Bushra Moiz Usman Shaikh Salman Adil Bushra Rizvi Yasmeen Rahman

OBJECTIVE To platelet aggregometry and describe the clinical spectrum of Glanzmann's thrombasthenia diagnosed by platelet aggregometry. STUDY DESIGN A case-series. PLACE AND DURATION OF STUDY This study was carried out at the clinical laboratories at the Aga Khan University Hospital, Karachi from January 2003 to January 2006. PATIENTS AND METHODS All patients irrespective of age and gende...

Journal: :Blood 1988
M E Russell U Seligsohn B S Coller M H Ginsberg P Skoglund T Quertermous

Glanzmann thrombasthenia is an autosomal recessive disorder of the platelet glycoproteins (GP) IIb and IIIa. These glycoproteins normally serve as receptors for other adhesive glycoproteins, including fibrinogen, von Willebrand factor, and fibronectin. Most patients affected by Glanzmann thrombasthenia have low levels of GPIIb and GPIIIa; however, the separate mechanisms responsible for the def...

Journal: :Orphanet Journal of Rare Diseases 2006
Alan T Nurden

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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