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Fibrinogen Amsterdam, Another Hereditary Abnormality of Fibrinogen
Author(s) -
Janssen Catherine L.,
Vreeken J.
Publication year - 1971
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1971.tb07039.x
Subject(s) - fibrinogen , fibrin , coagulation , thrombin , hemorrhagic diathesis , diathesis , abnormality , factor xiii , medicine , chemistry , immunology , platelet , psychiatry
S ummary . A coagulation defect, characterized by a prolonged thrombin and pro‐thrombin time, was discovered in a family without haemorrhagic diathesis. The disorder is caused by a delayed aggregation of fibrin monomers. Patient's and normal purified fibrin monomers showed the same aggregation properties, but the patient's fibrin monomers aggregated abnormally in the presence of the α 2 ‐globulin fraction of plasma. The presence of an abnormal fibrinogen is postulated. The inheritance of this fibrinogen is compatible with an autosomal dominant trait.