Fibrinogen Milano IV, Another Case of Congenital Dysfibrinogenemia with an Abnormal Fibrinopeptide A Release (Aα 16 Arg → His)
作者:
C. Bögli,
A. Hofer,
F. Baudo,
R. Redaelli,
M. Furlan,
期刊:
Pathophysiology of Haemostasis and Thrombosis
(Karger Available online 1992)
卷期:
Volume 22,
issue 1
页码: 7-11
ISSN:1424-8832
年代: 1992
DOI:10.1159/000216285
出版商: S. Karger AG
关键词: Dysfibrinogenemia;Fibrinogen variant;Fibrinopeptide A
数据来源: Karger
摘要:
An abnormal fibrinogen, denoted as ‘fibrinogen Milano IV, has been found in a 36-year-old woman without any bleeding manifestations or thrombotic tendency. Routine coagulation studies revealed prolonged thrombin and reptilase clotting times, very low plasma fibrinogen concentration determined by the functional assay but a normal fibrinogen concentration measured by the immunologic assay. Turbidity curves, measured following addition of thrombin to purified fibrinogen Milano IV, both in presence of calcium or EDTA, were markedly delayed. Release of fibrinopeptide B by thrombin was normal, whereas only half the normal amount of fibrinopeptide A was cleaved. The fibrinopeptide A peak of fibrinogen was preceded by an abnormal fibrinopeptide A*. Both peaks were collected for amino acid analysis which showed an exchange of arginine by histidine in position 16 of the Aα chain of the fibrinopeptide
点击下载:
PDF
(5991KB)
返 回