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Definition (MSHCZE)
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Autozomálně recesivně dědičná trombocytopatie se závažnými projevy krvácení. Podstatou je defekt destičkových glykoproteinů IIb/IIIa, které jsou nutné pro agregaci destiček během zástavy krvácení, kvalita trombu je snížená. Je prodloužená doba krvácení, počet trombocytů je normální.(cit. Velký lékařský slovník online, 2012 http://lekarske.slovniky.cz/ )
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Definition (NCI)
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A rare, autosomal recessive inherited and less frequently acquired platelet disorder. It is characterized by deficient or dysfunctional glycoprotein IIb/IIIa complex. It leads to defective platelet aggregation, resulting in bleeding.
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Definition (MSH)
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A congenital bleeding disorder with prolonged bleeding time, absence of aggregation of platelets in response to most agents, especially ADP, and impaired or absent clot retraction. Platelet membranes are deficient in or have a defect in the glycoprotein IIb-IIIa complex (PLATELET GLYCOPROTEIN GPIIB-IIIA COMPLEX).
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Definition (CSP)
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platelet abnormality characterized by defective clot formation, impaired aggregation, and prolonged bleeding time; other manifestations include nosebleeds, inappropriate bruising, and excessive bleeding.
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| Concepts |
Disease or Syndrome
(T047)
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| MSH |
D013915
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| ICD10 |
D69.1
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| SnomedCT |
32942005, 191310008, 30577005 |
| English |
Glanzmann Thrombasthenia, Thrombasthenia, Glanzmann, Thrombasthenias, THROMBOASTHENIA, Thromboasthenia, Glanzsmann's disease, Glycoprotein IIb/IIIa defect, Heredit.haemorr.thrombasthenia, Glanzmann's disease, GT, Glanzmann thrombasthenia, PLATELET GLYCOPROTEIN IIb-IIIa DEFICIENCY, PLATELET FIBRINOGEN RECEPTOR, DEFICIENCY OF, GLYCOPROTEIN COMPLEX IIb-IIIa, DEFICIENCY OF, GP IIb-IIIa COMPLEX, DEFICIENCY OF, Thrombasthenia, thromboasthenia (diagnosis), thromboasthenia, thrombocytasthenia, thrombocytasthenia (diagnosis), hereditary thromboasthenia, hereditary thromboasthenia (diagnosis), Hereditary thrombasthenia, Thrombasthenia of Glanzmann and Naegeli, Thrombasthenia [Disease/Finding], glanzmann thrombasthenia, glanzmann's thrombasthenia, glanzmann's disease, thrombasthenia, glanzmanns thrombasthenia, BDPLT2, BLEEDING DISORDER, PLATELET-TYPE, 2, GLANZMANN THROMBASTHENIA, THROMBASTHENIA OF GLANZMANN AND NAEGELI, Glycoprotein IIb/IIIa defect (disorder), Glanzmann thromboasthenia, Glanzmann-Naegeli disorder, Glanzmann's syndrome, Hereditary haemorrhagic thrombasthenia, Hereditary hemorrhagic thrombasthenia, Glanzmann's thrombasthenia, Glanzmann's thrombasthenia (disorder), Hereditary thromboasthenia, Hereditary thromboasthenia, NOS, Thromboasthenia, NOS, Thrombasthenia, NOS, Thrombasthenia, hemorrhagic, Thrombasthenia, hereditary, Thrombocytasthenia |
| Spanish |
TROMBASTENIA, Tromboastenia hereditaria, Enfermedad de Glanzmann, Tromboastenia, trombastenia de Glanzmann (trastorno), trombastenia de Glanzmann, tromboastenia hereditaria (trastorno), tromboastenia hereditaria, tromboastenia, trastorno de Glanzmann - Naegeli, tromboastenia de Glanzmann, Trombastenia, Trombastenia de Glanzmann |
| German |
THROMBASTHENIE, Glanzmann-Krankheit +, hereditaere Thrombasthenie, Glanzmann-Naegeli-Syndrom, Glanzmann-Thrombasthenie, Thrombasthenie |
| Italian |
Trombastenia, Tromboastenia ereditaria, Malattia di Glanzmann, Tromboastenia di Glanzmann, Tromboastenia |
| Dutch |
erfelijke trombastenie, tromboastenie, ziekte van Glanzmann, trombasthenie, Asthenie, tromb-, Glanzmann-syndroom, Trombasthenie |
| French |
Maladie de Glanzmann, Thromboasthénie, THROMBASTHENIE, Thrombasthénie de Glanzmann-Naegeli, Thrombasthénie héréditaire, Thrombasthénie, Purpura hémorragique héréditaire, Thrombasthénie de Glanzmann, Thrombasthénie hémorragique héréditaire |
| Portuguese |
Doença de Glanzmann, Trombastenia hereditária, TROMBOPLASTINEMIA, Tromboastenia de Glanzmann, Trombastenia, Trombastenia de Glanzmann |
| Swedish |
Trombasteni
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| Japanese |
ｲﾃﾞﾝｾｲｹｯｼｮｳﾊﾞﾝﾑﾘｮｸｼｮｳ, ｹｯｼｮｳﾊﾞﾝﾑﾘｮｸｼｮｳ, ｸﾞﾗﾝﾂﾏﾝﾋﾞｮｳ, Glanzmann血小板無力症, 血小板無力症, グランツマン病, グランツマン血小板無力症, 栓球無力症, 遺伝性血小板無力症, 栓球無力 |
| Czech |
trombastenie, Dědičná trombastenie, Trombastenie, Glanzmannova nemoc, Glanzmannův-Naegeliho syndrom, Glanzmannova trombastenie, Naegeliho syndrom trombocyt |
| Finnish |
Trombastenia
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| Russian |
TROMBASTENIIA, TROMBASTENIIA GLANTSMANNA, ТРОМБАСТЕНИЯ, ТРОМБАСТЕНИЯ ГЛАНЦМАННА |
| Polish |
Tromboastenia, Trombastenia, Niewydolność płytek krwi, Zaburzenia funkcji komórek płytkowych, Zaburzenia funkcji płytek krwi |
| Hungarian |
thrombasthaenia, Öröklött thrombasthaenia, Thromboasthenia, Glanzmann-betegség |
| Norwegian |
Glanzmanns sykdom, Tromboasteni, Trombasteni, Glanzmanns trombasteni |
