遺伝性出血性血小板無力症
WordNet
- of or relating to a hemorrhage (同)haemorrhagic
- a rare autosomal recessive disease in which the platelets do not produce clots in the normal way and hemorrhage results
PrepTutorEJDIC
- 世襲の,親譲りの / 遺伝的な,遺伝性の
UpToDate Contents
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English Journal
- Immunisation against αIIbβ3 and αvβ3 in a type 1 variant of Glanzmann's thrombasthenia caused by a missense mutation Gly540Asp on β3.
- Wihadmadyatami H, Röder L, Berghöfer H, Bein G, Heidinger K, Sachs UJ, Santoso S1.
- Thrombosis and haemostasis.Thromb Haemost.2016 Apr 21;116(2). [Epub ahead of print]
- Treatment of bleeding in patients with Glanzmann's thrombasthenia (GT) can be hampered by iso-antibodies against the αIIbβ3 integrin, which cause rapid clearance of transfused donor platelets. Type 1 GT patients with a total absence of αIIbβ3 from the platelet surface are known to be susceptible
- PMID 27098940
- The utility of International Society on Thrombosis and Haemostasis-Bleeding Assessment Tool and other bleeding questionnaires in assessing the bleeding phenotype in two platelet function defects.
- Kaur H1, Borhany M, Azzam H, Costa-Lima C, Ozelo M, Othman M.
- Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis.Blood Coagul Fibrinolysis.2016 Apr 20. [Epub ahead of print]
- The main objective of this study is to investigate the utility of International Society on Thrombosis and Haemostasis-Bleeding Assessment Tool (ISTH-BAT) in comparison with the condensed form of Molecular and Clinical Markers for the Diagnosis and Management of type 1 and WHO BATs, in assessing blee
- PMID 27100304
- Coated platelets function in platelet-dependent fibrin formation via integrin αIIbβ3 and transglutaminase factor XIII.
- Mattheij NJ1, Swieringa F1, Mastenbroek TG1, Berny-Lang MA2, May F3, Baaten CC1, van der Meijden PE1, Henskens YM4, Beckers EA5, Suylen DP1, Nolte MW3, Hackeng TM1, McCarty OJ2, Heemskerk JW1, Cosemans JM6.
- Haematologica.Haematologica.2016 Apr;101(4):427-36. doi: 10.3324/haematol.2015.131441. Epub 2015 Dec 31.
- Coated platelets, formed by collagen and thrombin activation, have been characterized in different ways: i) by the formation of a protein coat of α-granular proteins; ii) by exposure of procoagulant phosphatidylserine; or iii) by high fibrinogen binding. Yet, their functional role has remained uncl
- PMID 26721892
Japanese Journal
- 森田 久男,橋本 忠夫,加々美 光安,渡辺 朋郎,河本 泰彦,江幡 良子
- 臨床血液 8(2), 125-130, 1967
- … A 16 years old female with thrombasthenia (Glanzmann) was reported. … She had no proved hereditary consequence, but seemed to be congenital., because of the consanguinity of her parents.The laboratory findings on her blood were quite similar to that of 5 cases previously reported by us, She had slight splenomegaly and thrombocytopenia which might have some co-relation between them. …
- NAID 130004501889
Related Links
- thrombasthenia [throm″bas-the´ne-ah] 1. decreased platelet function; called also thromboasthenia. 2. Glanzmann thrombasthenia. Glanzmann thrombasthenia a hereditary platelet abnormality characterized by defective clot retraction ...
- Hereditary hemorrhagic thrombasthenia information including symptoms, causes, diseases, symptoms, treatments, and other medical and health issues. ... Introduction: Hereditary hemorrhagic thrombasthenia Description of ...
★リンクテーブル★
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- 英
- hereditary hemorrhagic thrombasthenia
- 関
- グランツマン血小板無力症
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- 関
- heredity、heritable、inherit、inheritable、inheritable character、inheritance、inherited
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- 関
- haemophilic、haemorrhagic、hemophilic