Four patients with congenital defiother family were found to have varyciency of fibrin-stabilizing factor (facing degrees of factor XIII deficiency. tor XIII) from two families have been The observations support the hypothedescribed. The mother and the sibs in sis of autosomal recessive inheritance one family and both parents in the of factor XIII deficiency. T HE EXISTENCE OF A BLOOD FACTOR th...

Factor XIII (F XIII) is an essential parameter for final clot stability. The purpose of this study was to determine the impact of the addition of factor (F)XIII on clot stability as assessed by Rotation Thromboelastometry (ROTEM®). In 90 intensive care patients ROTEM® measurements were performed after in vitro addition of F XIII 0.32 IU, 0.63 IU, 1.25 IU and compared to diluent controls (DC; aq...

An assay for a direct photometric determination of F XIII in untreated and undiluted plasma was developed. In a one-step procedure F XIII is activated by thrombin and Ca2+ and cross-links glycine-ethylester to a specific glutamine containing peptide substrate. The released ammonia is incorporated into alpha-ketoglutarate by glutamate dehydrogenase, and the NADH consumption of this reaction is m...

Factor XIII deficiency is a rare inherited coagulopathy. Factor XIII is the last clotting factor in the coagulation cascade to insure strength and stability to fibrin clots. Without this enzyme, the fibrous clot is unstable and nonresistant to fibrinolysis. Gravid women with this congenital disease are especially at risk for complications including miscarriages and hemorrhage without appropriat...

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...

Background: Congenital factor XIII (FXIII) deficiency is one of the rarest bleeding disorders with a prevalence of one per 2 million in the general population. The disorder is accompanied by a high rate of life-threatening bleeding. Due to normal results of routine coagulation tests, diagnosis of the disorder is challenging, but molecular methods can be used for precise diagnosis. Direct mutati...

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Human fibrin-stabilizing factor (Factor XIII) has been studied immunologically by the preparation of specific anti-Factor XIII antiserum in rabbits. On immunodiffusion it was found that normal plasma produced two precipitin lines. One of the precipitin lines was identical with that present in soluble platelet extract (the alpha-component), the other with that present in normal serum (beta-compo...

Inhibitor associated clotting factor XIII deficiency is a potentially life-threatening bleeding disorder with normal baseline coagulation studies, and may be associated with various drugs and autoimmune diseases. Failure to recognize this rare condition may lead to catastrophic outcome. We report a 52-year-old male with systemic lupus erythematosus (SLE) presented with recurrent spontaneous hae...

We have previously demonstrated that increasing factor XIII concentrations above that present in plasma (1 U/mI) results in the formation of very high molecular weight a fate polyacrylamide and agarose gel electrophoresis (SDSPAGE). 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 presen...