Acta Haematologica
Original Paper
Hereditary Dysfibrinogenemia Characterized by Slow Fibrinopeptide Release and Competitive Inhibition of ThrombinLaugen R.H. · Bithell T.C.Blood Coagulation Research Laboratory, Department of Pathology, and Division of Hematology, Department of Internal Medicine, University of Virginia School of Medicine, Charlottesville, Va., USA
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Article / Publication Details
Received: February 02, 1983
Accepted: October 13, 1983
Published online: February 25, 2009
Issue release date: 1984
Number of Print Pages: 8
Number of Figures: 0
Number of Tables: 0
ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)
For additional information: https://www.karger.com/AHA
Abstract
A qualitative abnormality of fibrinogen was identified in a 40-year-old woman with recurrent thrombophlebitis. Fibrinogen levels were normal when determined by immunologic, gravimetric, or nephelometric methods (200–390 mg/dl), but were diminished when tested by techniques based on the thrombin time (13 mg/dl). Asymptomatic family members, including both parents of the proposita, were less severely affected (mean fibrinogen level 100 mg/dl). The rate of fibrinopeptide release from purified fibrinogen was abnormally slow, whereas purified fibrin monomers polymerized at a normal rate. The abnormal fibrinogen was found to act as a competitive inhibitor of thrombin with an inhibitor constant (Ki) of 0.2 μM. This value was the same as the Michaelis constant (Km) of the normal thrombin-fibrinogen reaction, an observation consistent with an abnormality that retards fibrinopeptide release without affecting enzyme-substrate affinity.
© 1984 S. Karger AG, Basel
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Article / Publication Details
Received: February 02, 1983
Accepted: October 13, 1983
Published online: February 25, 2009
Issue release date: 1984
Number of Print Pages: 8
Number of Figures: 0
Number of Tables: 0
ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)
For additional information: https://www.karger.com/AHA
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Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
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