Abstract:
:The variant von Willebrand disease (vWd) variant type 2M (Vicenza) was identified in 13 patients of 7 unrelated families. 11 patients were from different parts of germany and 2 patients from Turkey. Hitherto this variant of vWd has been described only in two families originating from the province of Vicenza in Northern Italy. Characteristically this type of vWd shows the supranormal multimers of von Willebrand factor (vWf) in plasma similar to those seen in normal plasma after desmopressin infusion. Clinically, the patients present with bleeding symptoms. Other laboratory abnormalities include reduced values for vWf antigen (vWf: Ag), factor VIII coagulant activity (VII:C), ristocetin cofactor activity (RiCof) and collagen binding activity (vWf: CBA). The hereditary transmission of this variant is consistent with an autosomal dominant mode of inheritance. The patients with vWd type 2M (Vicenza) could be allocated into two subgroups: type "platelet normal" with normal vWf properties in platelets and type "platelet low" with reduced vWf: Ag levels in platelets.
journal_name
Thromb Resjournal_title
Thrombosis researchauthors
Zieger B,Budde U,Jessat U,Zimmermann R,Simon M,Kätzel R,Sutor AHdoi
10.1016/s0049-3848(97)00104-7subject
Has Abstractpub_date
1997-07-01 00:00:00pages
57-64issue
1eissn
0049-3848issn
1879-2472pii
S0049384897001047journal_volume
87pub_type
杂志文章abstract:BACKGROUND:Cardiovascular mortality is especially low in southwest France (the French Paradox). In previous experimental studies, we found that alcohol-free extracts of armagnac could inhibit human platelet function in vitro and experimental thrombosis in vivo. To test the possible relevance of these findings, we teste...
journal_title:Thrombosis research
pub_type: 临床试验,杂志文章,随机对照试验
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journal_title:Thrombosis research
pub_type: 临床试验,杂志文章
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更新日期:1996-12-15 00:00:00
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journal_title:Thrombosis research
pub_type: 杂志文章
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更新日期:2010-05-01 00:00:00
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journal_title:Thrombosis research
pub_type: 杂志文章,多中心研究
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journal_title:Thrombosis research
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更新日期:1996-06-15 00:00:00
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更新日期:2016-04-01 00:00:00
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更新日期:1985-12-01 00:00:00
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pub_type: 临床试验,杂志文章,随机对照试验
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journal_title:Thrombosis research
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pub_type: 杂志文章,随机对照试验
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更新日期:2019-09-01 00:00:00
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更新日期:2011-07-01 00:00:00
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pub_type: 杂志文章,随机对照试验
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pub_type: 杂志文章
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更新日期:1994-12-15 00:00:00
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journal_title:Thrombosis research
pub_type: 杂志文章
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更新日期:1983-11-01 00:00:00
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更新日期:2012-10-01 00:00:00