Familiaire dysfibrinogenemieën ; fibrinogeen Haarlem I en II
article
Types of familial dysfibrinogenaemia - A description is presented of a family with a form of congenital dysfibrinogenaemia, revealed by the abnormally low values obtained by the fibrinogen determination according to Clauss. The condition apparently does not lead to an enhanced bleeding tendency. The transmission of the abnormal fibrinogen (fibrinogen Haarlem I) is autosomal-dominant. The decelerated coagulation of purified fibrinogen revealed by the coagulation test could be attributed to abnormal polymerization of fibrin monomers.
A report is also presented of a patient from another family, who probably also had a congenital dysfibrinogenaemia (fibrinogen Haarlem II). Examination of several other members of his family revealed no dysfibrinogenaemia. In this patient, the dysfibrinogenaemia was not associated with a bleeding tendency, either. In both families, therefore, the abnormality appears to be harmless and detectable only by laboratory tests.
A report is also presented of a patient from another family, who probably also had a congenital dysfibrinogenaemia (fibrinogen Haarlem II). Examination of several other members of his family revealed no dysfibrinogenaemia. In this patient, the dysfibrinogenaemia was not associated with a bleeding tendency, either. In both families, therefore, the abnormality appears to be harmless and detectable only by laboratory tests.
TNO Identifier
288384
Source
Nederlands Tijdschrift voor Geneeskunde, 125(19), pp. 734-737.
Pages
734-737
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