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...

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This paper describes the topographic distribution of the multiple mRNAs coding for a novel human short-chain collagen, the alpha 1 chain of type XIII collagen. To identify the tissues and cells expressing these mRNAs, human fetal tissues of 15-19 gestational wk were studied by Northern and in situ hybridizations. The distribution pattern of the type XIII collagen mRNAs was compared with that of...

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...

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...

Fibrin polymers (des A,B fibrinogen) reduced the concentration of alpha-thrombin required for 50% activation of plasma factor XIII (a2b2 tetramer) by approximately 100-fold. In the presence of fibrin, the amount of gamma-thrombin required for activation was not affected. Catalytically inactive i-Pr2P- and D-Phe-Pro-Arg-CH2-alpha-thrombin were found to inhibit over 95% of the activation by alpha...

Homozygous patients with factor XIII deficiency are devoid of immunologically identifiable A protein, the active enzymatic component. Quantitative studies of transamidase activity of the factor are available in only a few cases, and the fibrin cross-linking pattern is not well known. The present paper deals with the quantitative estimation of factor XIII transamidase activity (dansylcadaverine ...