The Effect of Combined Factor XI Deficiency with von Willebrand Factor Abnormalities on Haemorrhagic Diathesis
The Effect of Combined Factor XI Deficiency with von Willebrand Factor Abnormalities on Haemorrhagic Diathesis
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因子 XI 缺乏与冯维勒布兰德因子异常联合对出血素质的影响
DOI：
10.1055/s-0038-1645682
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发表时间：
1990
影响因子：
6.7
通讯作者：
Llana Tatarsky
中科院分区：
文献类型：
--
作者：
S. Tavori;B. Brenner;Llana Tatarsky
为了解释缺乏因子XI（FXI）的患者和出血表现之间缺乏相关性，我们在212例FXI缺乏患者中关联了FXI和血管性血友病因子（vWF）联合缺乏的患病率。54例患者合并FXI和vWF缺乏症：16例患者为重度FXT缺乏症，38例患者为轻度FXT缺乏症。在一组28例轻度FXI缺乏症患者中，14例出血者的平均vWF、Ag、利托那肽辅因子和利托那肽诱导的血小板聚集显著低于根据相似FXI水平选择的14例非出血者。这些结果表明，FXI和vWF缺乏症的组合是常见的，并可能影响轻度FXI缺乏症的出血倾向。
Summary To account for the lack of correlation between the level of factor XI (FXI) in deficient patients and haemorrhagic manifestations, we correlated the prevalence of combined FXI and von Willebrand’s factor (vWF) deficiency in 212 FXI-deficient patients. Fifty-four patients had a combined FXI and vWF deficiency: 16 patients had severe and 38 patients had mild FXT deficiency. In a group of 28 patients with comparably mild FXI deficiency, 14 bleeders had significantly lower mean vWF, Ag, ristocetin cofactor and ristocetin induced platelet aggregation than 14 non-bleeders selected on the basis of comparable FXI levels. These findings suggest that the combination of FXI and vWF deficiency is common and may affect the bleeding tendency in mild FXI deficiency.
25个XI因子缺陷家系的出血倾向、XI因子凝血活性和XI因子抗原比较。
影响因子：
20.3
作者：
Ragni,MV;Sinha,D;Seaman,F;Lewis,JH;Spero,JA;Walsh,PN
通讯作者：
Walsh,PN