Coagulaon factor XIII gene, protein structure and funcon Coagulaon factor XIII (FXIII) is a tetrameric (FXIII- A2B2) pro-transglutaminase enzyme with an essenal role in the final stage of coagulaon cascade by cross linking the fibrin monomers and stabilizing the fibrin clot. Congenital FXIII deficiency is a rare bleeding disorder, with an autosomal recessive trait inheritance, and a fre...

Factor V activity in suspensions of human platelets washed by albumin density gradient separation increased in response to stimulation by both collagen and adenosine diphosphate (ADP). The appearance of factor V activity extracellularly had the characteristics of platelet secretion and was partially inhibited by aspirin and by the antimetabolites 2-deoxyglucose and antimycin A. Some increase in...

Factor XIII deficiency is a congenital defect of the blood coagulation system. Activated factor XIII is the fibrin cross-linking enzyme that catalyzes the formation of N 6 y-glutamyllysyl bonds in fibrin. Congenital deficiency of factor XIII has been described in approximately 65 separate families. It is apparent that this is a hereditary trait, but the mode of inheritance is unclear. In this s...

Received: November 3, 2008. Accepted: February 13, 2009. Corresponding author: Tae Kwan Kim, M.D., Department of Anesthesiology and Pain Medicine, Holy Family Hospital, The Catholic University of Korea, Sosa 2-dong, Wonmi-gu, Bucheon 414-717, Korea. Tel: 82-32-340-2158, Fax: 82-32-340-2255, E-mail: [email protected] Copyright c Korean Society of Anesthesiologists, 2009 cc This is an open-ac...

from peripheral blood leukocytes as described previously (5). Amplification of genomic DNA was performed by PCR as described (1). The G3 A substitution at nucleotide position 20210 was detected by allele-specific restriction digestion by using HindIII (1). Among the 27 patients, 4 (14.8%) were heterozygous for the G 20210 3 A mutation in the prothrombin gene and 1 patient (3.7%) was homozy-gous...

The association between nephrotic syndrome (NS) and intravascular coagulation is well known, and thromboembolic disease is a relatively common complication of the condition. The association of NS with anticoagulant activity is less well recognized. Factor X deficiency, and sometimes Factor IX and Factor II deficiency, can rarely complicate AL amyloidosis, which may present with a NS. The mechan...

Severe factor V (FV) deficiency is associated with mild to severe bleeding diathesis, but many patients with FV levels lower than 1% bleed less than anticipated. We used calibrated automated thrombography to screen patients with severe FV deficiency for protective procoagulant defects. Thrombin generation in FV-deficient plasma was only measurable at high tissue factor concentrations. Upon reco...