To explore the biological and clinical implications of the structure/function relationships in factor XIII, mutations in two patients with type II deficiency were identified and characterized in a mammalian expression system. Nucleotide sequence analysis of the A subunit gene showed that case no. 1 had a deletion of 4 bp (AATT) in exon XI and that, in case no. 2, Gly562 (GGG) had been replaced ...

BACKGROUND A correlation between decreased blood coagulation factor XIII activity and the severity of organ disorders in pediatric Henoch-Schönlein purpura (HSP) has been demonstrated, but possible correlations in adult HSP have not been thoroughly investigated. OBJECTIVES To investigate the association between factor XIII activity with varying clinical severities of HSP and the severity of o...

PREDICTING perioperative blood loss is traditionally fraught with great difficulties. Medical history is certainly crucial in the risk assessment of perioperative blood loss because it provides information on bleeding history, the use of antiplatelet drugs, and oral anticoagulation. However, so far there is no laboratory parameter that could reliably predict perioperative blood loss. According ...

Congenital factor XIII (FXIII) deficiency is potentially a severe bleeding disorder, but in some cases, the symptoms may be fairly mild. In this study, we have characterized the molecular mechanism of a mild phenotype of FXIII A-subunit deficiency in a Finnish family with two affected sisters, one of whom has even had two successful pregnancies without regular substitution therapy. In the scree...

Ca(II) ions are crucial during proteolytic conversion of Factor XIII zymogen into the active enzyme Factor XIIIa. Factor XIII proteolyzed by thrombin or trypsin in the presence of 5 mM-EDTA resulted in rapid inactivation of transglutaminase activity. Factor XIIIa formed by thrombin or trypsin in the presence of 40 microM-Tb(III) ions, however, was indistinguishable from Factor XIIIa formed in t...

Acquired factor XIII (FXIII) deficiency due to autoantibody against FXIII is a very rare severe hemorrhagic diathesis. Antibodies directed against the A subunit of FXIII, which interfere with different functions of FXIII, have been described. Here, for the first time, we report an autoantibody against the B subunit of FXIII (FXIII-B) that caused life-threatening bleeding in a patient with syste...

We have previously demonstrated that increasing factor XIII concentrations above that present in plasma (1 U/mL) results in the formation of very high molecular weight alpha fate polyacrylamide and agarose gel electrophoresis (SDS-PAGE). In this report, we have examined the effect of such crosslinking on plasmic susceptibility of fibrin prepared from purified fibrinogen and from plasma in the p...

background: factor xiii deficiency (fxiiid) is an inherited rare bleeding disorder with some life threatening clinical manifestation including intracranial haemorrhage (ich). among all polymorphisms found in fxiiid, thrombin activatable fibrinolysis inhibitor (tafi) thr325ile gene polymorphism increases probability ofichabout 20 fold in patients with fxiii .so, in this study we aimed to evaluat...

Background: Acquired factor XIII (FXIII) deficiency is associated with reduced clot firmness and increased bleeding in patients undergoing major surgery. In contrast, only limited information is available on the haemo static relevance of acquired FXIII deficiency in non-surgical patients. Case report: An 81-year-old patient, who had experienced acute type-A dissection of the aorta eight years e...