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近期对冯·维勒布兰德描述的奥兰群岛(芬兰)首个出血者家族的调查。

Recent investigations of the first bleeder family in Aland (Finland) described by von Willebrand.

作者信息

Nyman D, Eriksson A W, Blombäck M, Frants R R, Wahlberg P

出版信息

Thromb Haemost. 1981 Feb 23;45(1):73-6.

PMID:6972630
Abstract

The still living members of the original bleeder family on the Aland Islands described by von Willebrand in 1926 have been reinvestigated by using modern laboratory techniques for the measurement of the Factor VIII complex and with regard to platelet aggregation. The low level of F VIII : C activity demonstrated in 1957 could be confirmed in some of the family members, who however all had only mild bleeding symptoms. More consistently, in 6 out of 10, a low F VIIIR : Ag was found; all of those also had a low F VIII : RCoF. In none of the members were excessively low values for any of the parameters found. However, the spectrum of the whole F VIII complex indicates that the original family described by von Willebrand belongs to von Willebrand's disease, type I.

摘要

1926年冯·维勒布兰德描述的奥兰群岛上原出血家族中仍在世的成员,已通过使用现代实验室技术测量VIII因子复合物以及检测血小板聚集情况进行了重新研究。1957年所证实的低水平F VIII : C活性在一些家族成员中得到了确认,不过他们都仅有轻微的出血症状。更一致的是,在10名成员中有6名发现F VIIIR : Ag水平较低;所有这些成员的F VIII : RCoF也较低。在任何成员中均未发现任何参数的值极低。然而,整个VIII因子复合物的情况表明,冯·维勒布兰德所描述的原家族属于I型冯·维勒布兰德病。

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