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A pharmacogenetic effect of factor XIII valine 34 leucine polymorphism on fibrinolytic therapy for acute myocardial infarction.
OBJECTIVES: The aim of this study was to evaluate the pharmacogenetic role of the factor XIII (FXIII) valine 34 leucine (Val34Leu) polymorphism in the fibrinolytic therapy of acute myocardial infarction (MI). BACKGROUND: Fibrinolytic therapy is an established treatment for acute MI, but up to 40% of treated patients do not achieve optimal tissue reperfusion. The FXIII Val34Leu polymorphism is one of the most relevant functional polymorphisms described in the haemostatic system. The common Leu34 allele associates with an increased FXIII-transglutaminase activity, which results in an increased and faster rate of fibrin stabilization. METHODS: We genotyped this polymorphism in 293 consecutive MI patients (62 +/- 12 years; 231 males) from two different European populations. All patients were treated with standard doses of fibrinolytic drugs. Noninvasive assessment of the efficacy of coronary fibrinolysis was evaluated by serial electrocardiograms and creatine kinase time-activity curves. The clinical outcome was also re-evaluated at 24 h (death, reinfarction, or urgent revascularization). RESULTS: Multivariate analysis showed that Leu34 carriers displayed a significantly less efficient fibrinolysis than carriers of Val/Val genotype (p = 0.021; odds ratio [OR] 1.90, 95% confidence interval [CI] 1.10 to 3.28). At 24 h, Leu34 allele carriers had the worst outcome (p = 0.006; OR 2.14, 95% CI 1.25 to 3.68). Interestingly, the combination of the Leu34 allele and nonsmoking status increased the risk of non-reperfusion criteria (p = 0.003, OR 3.77), and worse outcomes at 24 h (p = 0.001, OR 4.55). CONCLUSIONS: In a large cohort of nonselected and consecutive acute MI patients from two different European populations, we show clinical evidence that the presence of the Leu34 allele reduces the efficacy of fibrinolytic therapy.
Combined occurrence of von Willebrand's disease and factor XIII deficiency: a case report.
We report the case of a three year old female child with combined occurrence of von Willebrand's disease and factor XIII deficiency, an extremely rare combination. The patient presented with prolonged bleeding following cuts and wounds. Clot solubility test using 5M urea was positive. Platelet aggregation using ristocetin was reduced, which corrected on adding normal plasma. Aggregation with other agonists was normal. We discuss the clinico- hematological profile of the case. Only one such case has been reported in literature in the past to the best of our knowledge.
Blood coagulation findings and the efficacy of factor XIII concentrate in premature infants with intracranial hemorrhages.
Coagulation findings were examined in 55 cases of neonatal intracranial hemorrhages (ICH). Marked decreases of the platelet count, fibrinogen, factor XIII (F XIII) activity, were observed in these cases. However, the relatively mild increases in the alpha 2-plasmin inhibitor activity, alpha 2-plasmin inhibitor.plasmin complex and fibrin/fibrinogen degradation products level were observed and only about one third of the cases showed abnormal values. In consideration of these coagulation findings, fifty-eight cases of premature infants were randomly divided into a treated group with a F XIII concentrate and a non-treated group. Thirty cases were administered within 6 hours after delivery to investigate the preventive effects against intraventricular hemorrhages (IVH). Compared in frequencies between both groups limited to the cases with a high risk of IVH, the frequency in the treated group was two out of 13 (15.4%), significantly low (p less than 0.05), while it was six out of eight cases (75.0%) in the non-treated group. From these results it was concluded that a F XIII concentrate is effective in the prophylaxis of IVH in premature infants.
Arg260-Cys mutation in severe factor XIII deficiency: conformational change of the A subunit is predicted by molecular modelling and mechanics.
To explore the implications of the structure/ function relationships in factor XIII. a patient with severe A subunit deficiency was examined at the DNA and RNA levels. Nucleotide sequence analysis of the patient's DNA amplified by PCR revealed that the patient had a replacement of C by T in the codon for Arg260. RT-PCR analysis demonstrated that only one kind of mRNA coding for the Arg260-Cys mutation was expressed in the patient at a normal level. Another possible defective allele of the A subunit gene with a G-A polymorphism was not expressed (null allele). The substitution of Arg260 by Cys located on the interface of two A subunits would preclude the reciprocal ionic interaction (salt bridge) between Arg260 and Asp404. Molecular modelling and, for the first time, molecular mechanics calculated that Cys260 changed the local conformation of the A subunit and reduced the electrostatic interaction between two monomers, suggesting destabilization of the molecule's dimer.
[Activation of plasma Factor XIII by acetylated thrombin].
It has been demonstrated that acetylated thrombin (thrombin-esterase), having practically no coagulating activity but possessing high esterase activity, is capable to activate factor XIII. This ability, however, is 2 times lower as compared to that of native thrombin.
Genetic polymorphisms of the A and B subunits of coagulation factor XIII in the Chinese population.
Coagulation factor XIIIA and XIIIB polymorphisms in a random population sample from Chengdu in southwest China (n = 121) were studied using isoelectric focusing in polyacrylamide gels followed by immunoblotting with enzyme immunoassay. The allele frequencies were as follows: FXIIIA*1 = 0.8719, FXIIIA*2 = 0.1240, FXIIIA*3 = 0.0041; FXIIIB*1 = 0.2727, FXIIIB*2 = 0.0165, FXIIIB*3 = 0.7107. The distribution of phenotypes of FXIII agrees with the Hardy-Weinberg equilibrium. Comparing these allele frequencies with those reported in other populations, it was found that the allele frequencies of FXIIIA*1 and FXIIIB*3 in the Chinese population were higher.
Factor XII but not protein C, protein S, antithrombin III, or factor XIII is a predictor of recurrent miscarriage.
OBJECTIVE: To investigate whether a decrease in the values of protein C (PC), protein S (PS), antithrombin III (ATIII), factor XII (FXII), or factor XIII (FXIII) has predictive value for subsequent miscarriages. DESIGN: Prospective study. SETTING: Nagoya City University Medical School. PATIENT(S): A total of 536 patients with a history of two or more first-trimester miscarriages. INTERVENTION(S): One hundred and twelve patients treated with low-dose aspirin were excluded from the analysis. MAIN OUTCOME MEASURE(S): The subsequent pregnancy outcome of 424 patients was compared for abnormal and normal levels of each parameter. RESULT(S): There were no differences in the subsequent miscarriage rates between abnormal and normal values of PC, PS, ATIII, and FXIII. However, the rate with abnormal FXII is significantly higher than that with normal FXII. CONCLUSION(S): A decrease in FXII (but not in PC, PS, ATIII, or FXIII) predicts subsequent miscarriage in patients with a history of first-trimester recurrent miscarriages.
Factor XIII(A) subunit deficiency due to a homozygous 13-base pair deletion in exon 3 of the A subunit gene.
We investigated the molecular basis of factor XIII(A) subunit deficiency in a Greek family. Each of the 15 exons of the A subunit gene were individually amplified by polymerase chain reaction, using previously reported oligoprimers. The proband with severe deficiency was found to have a homozygous 13-base pair deletion in the 3' half of exon 3. The deleted sequence, extending from codons 82-86, results in a frameshift and generates a downstream termination codon in exon 4. Single strand conformation polymorphism (SSCP) analysis detected no additional mutations in the coding or consensus splice sequences of the A subunit gene. Both parents of the proband were heterozygous for the defect. Only one previous microdeletion (AG dinucleotide) has been reported in the A subunit gene, and was located at the intron B-exon 3 boundary. Further studies are necessary to determine whether this region of the gene is a "hot spot" for microdeletion mutations.
[Congenital deficiency in the fibrin stabilizing factor (factor XIII)].
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[Clinical significance of factor XIII analysis in blood coagulation tests].
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[Activity of the fibrin stabilising factor (factor XIII) in anaemias and polycythaemias (author's transl)].
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[Fibrin stabilization and factor XIII substitution in malignant tumors (author's transl)].
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Alterations of haemostasis parameters with special reference to fibrin stabilization, factor XIII and fibronectin in patients with obliterative atherosclerosis.
Intravascular and endoparietal fibrin deposition are thought to be involved in atherosclerotic process, especially when fibrin is stabilized by factor XIII of coagulation system. The study carried out on a group of 50 patients with obliterative atherosclerosis of the lower limbs revealed an increased plasma fibrin stabilizing activity apparently due to an increased F.XIII level. Furthermore, alterations in coagulation and fibrinolysis indicating hypercoagulable tendency were found. It is concluded, that the observed changes may contribute to the development of atherosclerotic process.
Differential behavior of coagulation factor XIII in patients with inflammatory bowel disease and in patients with giant cell arteritis.
The aim of this prospective study was to examine the role of coagulation factor XIII (FXIII) in relation to disease activity in inflammatory bowel disease (IBD) and in giant cell arteritis. Plasma FXIII activity was studied during active and inactive disease in newly diagnosed patients with Crohn's disease (CD; n = 20), ulcerative colitis (UC; n = 18) and giant cell arteritis (GCA; n = 19), in 3-month intervals (median follow-up 12 months). FXIII was also measured in two noninflammatory control groups, age and sex matched for IBD (n = 25) and GCA (n = 26). FXIII activity was significantly lower in active CD or UC than in active GCA or the noninflammatory controls. Both in CD and UC, FXIII activity correlated inversely with indices of clinical disease activity, the erythrocyte sedimentation rate, fibrinogen and C-reactive protein levels. Low FXIII activity is a characteristic feature of active IBD, and serial measurements may be useful to assess IBD activity.
Enzyme-linked immunosorbent assay for the determination of blood coagulation factor XIII A-subunit in plasma and in cell lysates.
A new one-step ELISA was developed for the determination of the concentration of blood coagulation factor XIII subunit A (FXIII-A) in plasma and in cell lysates. Monoclonal antibodies directed against different epitopes on FXIII-A were used for the assay. The capture antibody was biotinylated on its carbohydrate moiety and the detection antibody was labelled with horseradish peroxidase. The antigen-antibody reaction was carried out in the well of a streptavidin-coated microplate. Complex formation with FXIII subunit B (FXIII-B) and association to fibrinogen did not influence the accessibility of the antibodies to FXIII-A. The method could be performed within 2 h and demonstrated good reproducibility, recovery and sensitivity. Plasma samples could be assayed after storage at -20 degrees C for at least 6 months. However, in the case of platelet lysates freezing and rethawing resulted in a significant loss of FXIII-A. FXIII-A concentrations measured in the plasma samples of healthy individuals and patients correlated well with the concentrations of complexed plasma FXIII (A2B2) and with the results of FXIII activity measurements. A reference range of 46-82 fg/platelet was established for platelet FXIII-A.
Consecutive appearance of coagulation factor XIII subunit A in macrophages, megakaryocytes, and liver cells during early human development.
Thirty embryonic and fetal samples were investigated to study the appearance and characteristics of factor XIII subunit A (FXIIIA)-containing cells in the course of human development. Samples were either vacuum-embedded in paraffin for staining FXIIIA by a sensitive biotin-streptavidin system or snap-frozen for double-labeling studies to characterize FXIIIA-containing cells. FXIIIA appeared as early as the fifth gestational week in yolk sac samples in stellate-shaped cells. Nonparenchymal cells in liver samples showed intense labeling for FXIIIA from the sixth week of gestation. The relative amount of FXIIIA-containing cells rapidly diminished up to the 13th gestational week. When characterized, the majority of these cells proved to be KiM7-positive macrophages, while GPIb (CD42b)-labeled cells accounted for less than 10% of FXIIIA-positive cells. Liver cells did not show any staining for FXIIIA in first trimester samples. The earliest liver specimen showing FXIIIA was at the 20th week, when FXIIIA appeared in some liver cells, particularly in those surrounding the central veins. In bone marrow smears, FXIIIA-positive cells started to appear at week 10 in the clavicles and increased in number in subsequent stages of development. Intracellular FXIIIA was distributed between GPIb-, RFD7-, and KiM7-positive cells. The results indicate that, apart from liver cells, at least three different cell populations (KiM7+ RFD7+ GPIb-, KiM7- RFD7- GPIb-, and KiM7- RFD7- GPIb+) contain FXIIIA in the early phase of human development. We conclude that FXIIIA appears very early during human development and is detectable in both extra- and intraembryonic hematopoietic organs. Intracellular FXIIIA in early human development is distributed between different macrophages and megakaryocytes, and by week 20, it appears in liver cells as well.
Factor XIII A-subunit concentration predicts outcome in stroke subjects and vascular outcome in healthy, middle-aged men.
There is growing evidence for a role of factor XIII (FXIII) in vascular disease. FXIII measures were determined in (i) a nested case-control study from the Second Northwick Park Heart Study of 63 men with myocardial infarction (MI) and 124 age-matched controls and (ii) in a case-control study of 475 subjects with acute stroke and 461 controls followed up for 54 months for mortality. In both studies, measures of FXIII A- and B-subunit antigen, FXIII activity and prothrombin fragments (F1 + 2) were made. An in vitro model was used to investigate the effects of thrombin activity on FXIII A- and B-subunit antigen levels. In study 1, patients clinically free of coronary artery disease who later developed MI had lower adjusted FXIII A-subunit levels at recruitment (129.2%vs 113.3%, P = 0.007). In study 2, stroke patients with large vessel disease had lower A-subunit antigen levels (102.1%vs 127.2%, P < 0.001), but higher F1 + 2 levels (0.941%vs 0.753%, P < 0.05), than subjects with small vessel disease. Levels of FXIII A-subunit (100%vs 117%, P < 0.0001) were lower and F1 + 2 higher (1.020%vs 0.702%, P < 0.0001) in stroke patients who had died compared with those still alive at the end of the follow-up period. Low concentrations of FXIII A-subunit antigen predicted vascular outcome in otherwise healthy subjects and relate to both size of infarct and poor post-stroke survival in patients with acute ischaemic stroke. Low in vitro concentrations of FXIII A-subunit antigen wererelated to increased thrombin generation and, thus, increased risk of thrombotic events.