von Willebrand disease "Vicenza" with larger-than-normal (supranormal) von Willebrand factor multimers
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چکیده
منابع مشابه
von Willebrand disease "Vicenza" with larger-than-normal (supranormal) von Willebrand factor multimers.
When normal volunteers or patients with type I von Willebrand disease (VWD) are given desmopressin (DDAVP), a set of larger-than-normal (supranormal) von Willebrand factor (VWF) multimers, similar to those present in VWF-containing cells such as platelets megakaryocytes and endothelial cells, appear transiently in postinfusion plasma. In two kindreds with mild lifelong bleeding symptoms transmi...
متن کاملvon Willebrand Disease “ Vicenza ” With Larger - Than - Normal ( Supranormal ) von
When normal volunteers or patients with type I von Willebrand disease (VWD) are given desmopressin (DDAVP). a set of larger-than-normal (supranormal) von Willebrand factor (VWF) multimers. similar to those present in VWFcontaining cells such as platelets megakaryocytes and endothelial cells, appear transiently in postinfusion plasma. In two kindreds with mild lifelong bleeding symptoms transmit...
متن کاملSupranormal von Willebrand Factor Multimers in Scieroderma
Platelet adhesion-aggregation reactions play an early and pivotal role in the pathogenesis of systemic sclerosis in scleroderma. but the mechanisms are incompletely understood. We determined whether or not plasma from 11 consecutive patients with scleroderma contained a subset of larger than normal (“supranormal”) multimers of von Willebrand factor (vWF) that are potent inducers of platelet agg...
متن کاملSupranormal von Willebrand factor multimers in scleroderma.
Platelet adhesion-aggregation reactions play an early and pivotal role in the pathogenesis of systemic sclerosis in scleroderma, but the mechanisms are incompletely understood. We determined whether or not plasma from 11 consecutive patients with scleroderma contained a subset of larger than normal ("supranormal") multimers of von Willebrand factor (vWF) that are potent inducers of platelet agg...
متن کاملReduced von Willebrand factor survival in type Vicenza von Willebrand disease.
Type Vicenza variant of von Willebrand disease (VWD) is characterized by a low plasma von Willebrand factor (VWF) level and supranormal VWF multimers. Two candidate mutations, G2470A and G3864A at exons 17 and 27, respectively, of the VWF gene were recently reported to be present in this disorder. Four additional families, originating from northeast Italy, with both mutations of type Vicenza VW...
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ژورنال
عنوان ژورنال: Blood
سال: 1988
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood.v71.1.65.bloodjournal71165