Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “FACTOR XIII”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 667 records · Page 37Linked to original sources
PubMedDate not supplied

Plasmatic [corrected] factor XIII reduces severe pleural effusion in children after open-heart surgery.

Chylous effusions frequently occur after cardiac surgery due to severe damage to the lymphatic system, thus indicating that the insertion of a chest tube may be necessary. Factor XIII (FXIII) is discussed as being essential for wound healing. The aim of this retrospective study was to evaluate whether the application of a single dose of FXIII results in a reduced amount of pleural effusion, leading to an earlier release of patients from the hospital. The cases of 40 children with severe chylous effusions after open-heart surgery were examined. Twenty patients received FXIII and were compared to 20 age- and weight-matched patients who did not receive FXIII. Major parameters included the amount of effusion before and 1 and 3 days after the application of FXIII; the duration of chest tubes; the total amount of fluid loss via drainage; and the period of hospitalization. FXIII levels in plasma showed an inverse correlation with fluid loss. After application of a single dose of FXIII, a significant reduction of pleural effusion within the first 24 hours was detected. However, no difference was observed between the two groups when comparing the total amount of pleural effusions within the first 72 hours. Finally, the duration of hospitalization did not differ between the FXIII-treated and the control group. A single application of FXIII rapidly reduces the amount of chylous effusions in the early period after open-heart surgery. This effect is detectable only for 24 hours after the treatment and does not alter the further clinical outcome. Prospective clinical trials are warranted to determine if repeated application or a higher dose of FXIII may improve the clinical outcome of chylous leakages in children after open-heart surgery.

Case-Control Studies↗
PubMed2003

Plasminogen activator inhibitor 1 4G/5G polymorphism and coagulation factor XIII Val34Leu polymorphism: impaired fibrinolysis and early pregnancy loss.

BACKGROUND: A successful outcome of pregnancy depends on proper placental formation. In the very beginning of this process, trophoblast invasion and fibrin deposition into the wall of the decidual veins play an important part. Two polymorphisms, coagulation factor XIII (FXIII) Val34Leu and plasminogen activator inhibitor 1 (PAI-1) 4G/5G, interfere with fibrin cross-linking and regulation of fibrinolysis and may therefore contribute to early pregnancy loss. METHODS: We enrolled 49 unrelated Caucasian women with a history of two consecutive or three to six nonconsecutive early pregnancy losses and 48 unrelated parous healthy controls without a history of pregnancy loss and evaluated them for the following genetic variants: the factor V Leiden and prothrombin G20210A gene mutations, the methylenetetrahydrofolate reductase C677T and A1298C polymorphisms, and the PAI-1 4G/5G and FXIII Val34Leu polymorphisms. RESULTS: For the isolated occurrence of PAI-1 4G/5G or FXIII Val34Leu, we found no statistically significant difference between cases and controls. For homozygosity of either or compound carrier status of both mutations, the overall relative risk for early pregnancy loss was significantly increased (odds ratio = 2.4; 95% confidence interval, 1.1-5.5; P = 0.032). We observed no statistically relevant association of any of the other tested mutations with early pregnancy loss. CONCLUSION: Homozygosity for PAI-1 4G or FXIII 34Leu polymorphisms as well as compound carrier status is associated with early pregnancy loss.

Abortion, Habitual↗
PubMed2002

Factor XIII activity levels in patients with allogeneic haematopoietic stem cell transplantation and acute graft-versus-host disease of the gut.

Severe graft-versus-host disease (GvHD) of the gut clinically resembles Crohn's disease and ulcerative colitis. As low plasma levels of factor XIII (FXIII) have been described in chronic inflammatory bowel disease (CIBD) and as beneficial effects of FXIII concentrates in CIBD have been reported, we studied the FXIII plasma activity levels in patients undergoing allogeneic stem cell transplantation (SCT). In 20 of 22 patients with an uncomplicated course of SCT, FXIII stayed within the normal range (median 102 iu/dl, range 74-122), but was significantly reduced with the lowest FXIII levels on d 0 and 7 (d 0: median 83 iu/dl, range 55-165, d 7: median 83, range 70-101). In 20 of 22 patients with histologically proven GvHD of the gut, FXIII levels far below the normal range were observed (median 50, range 21-87) with a strong correlation between FXIII activity levels and degree of GvHD (r = -0.908; P < 0.001). We conclude that FXIII is consumed in patients with GvHD of the gut. As FXIII plays a a crucial role in haemostasis and wound healing, a study on the potential benefit of FXIII substitution in patients with severe GvHD of the gut might be rewarding.

Acute Disease↗
PubMed1991

Factor XIII concentrate for prevention of recurrent subarachnoid hemorrhage: results of a multicenter pilot study. The FISAH Study Group.

Rebleeding after aneurysmal subarachnoid hemorrhage (SAH) has been attributed to fibrinolysis of the blood clot surrounding a rupture aneurysm. Since stability of the clot is influenced by the action of coagulation factor XIII (F XIII), high levels of F XIII might be beneficial in preventing recurrent SAH without causing the severe side effects of synthetic antifibrinolytics. We performed an open multicenter pilot study with administration of 10 x 1250 units of F XIII concentrate during the first 15 days after spontaneous SAH. Of 111 patients who were entered within 72 hours after SAH, 48 (43%) were in grades I and II, 33 (30%) were in grade III, and 30 (27%) were in grades IV and V according to Hunt & Hess. Aneurysm rupture was proved in 76 patients (68%) and aneurysm surgery was performed in 50 patients (45%). Overall mortality at four weeks was 27%. There were 11 recurrent hemorrhages in 9 patients (10%), 7 of which were fatal. Cerebral infarction occurred in 22 patients (20%) and hydrocephalus in 8 (7%). No adverse effects of therapy were noted. We conclude that F XIII therapy is a safe and promising new therapeutic approach in the management of SAH. It is currently being investigated in a large prospective, randomized, double-blind, placebo-controlled trial.

Adult↗
PubMed1995

Transformation of cellular factor XIII into an active zymogen transglutaminase in thrombin-stimulated platelets.

The cellular form of blood coagulation factor XIII (FXIII) is present in platelets, monocytes and macrophages. During long-term stimulation of platelets by thrombin cellular FXIII becomes activated and cross-links proteins, however, the mechanism of its activation has not been elucidated. It was shown that, contrary to plasma FXIII, the intracellular activation of platelet FXIII does not involve proteolysis. FXIII remained intact in thrombin-activated platelets, i.e., the activation peptide was not removed from the molecule. Part of the zymogen FXIII molecules, however, assumed an active configuration as was demonstrated both by the measurement of transglutaminase activity and by active-site-SH titration. These findings clearly indicate that during platelet activation, when intracellular Ca2+ concentration is raised, a slow non-proteolytic transformation of FXIII zymogen into an active transglutaminase occurs.

Blood Platelets↗
PubMed1997

Spectroscopic study of secondary structure and thermal denaturation of recombinant human factor XIII in aqueous solution.

The secondary structure and thermal denaturation (in H2O vs D2O) of recombinant human factor XIII in aqueous solutions were investigated using infrared and circular dichroism (CD) spectroscopies. The infrared amide I spectrum of the protein in H2O solution at 25 degrees C exhibited an absorbance maximum near 1642 cm-1, indicating the presence of a predominantly beta-sheet structure. Quantitative analysis revealed that the native protein contains 13-16% alpha-helix, 41-49% beta-sheet, 29% beta-turn, and 10-14% extended strand structures. The presence of a strong low-wavenumber beta-sheet band at 1641 cm-1 and a weak high-wavenumber beta-sheet band at 1689 cm-1 indicated that the beta-sheet structure of the protein is predominantly antiparallel. Quantitative analysis of the CD spectrum using the SELCON method indicated a secondary structural content of 10% alpha-helix, 40-50% beta-sheet, 20-35% beta-turns, and 20-35% unordered elements, which matches that determined by X-ray crystallography. The apparent discrepancy with the contents of unordered element determined by infrared spectroscopy is reconciled by considering that CD spectroscopy and X-ray crystallography assign extended loops and strands to unordered elements, whereas infrared spectroscopy recognizes these as distinct structured elements. During heating above 60 degrees C, a pair of new infrared bands appeared at 1626 and 1693 cm-1 for the protein in H2O and 1619 and 1683 cm-1 in D2O, indicating a formation of intermolecular beta-sheet aggregates. The intensities of the new bands increased as a function of temperature, concomitant with an intensity decrease in bands for the native protein structural elements. As expected, there was an increase in thermal stability in D2O relative to that in H2O, which was manifested as an increase of about 5 degrees C in the temperature for initial loss of infrared bands assigned to native structural elements and for appearance of bands due to intermolecular beta-sheet. In addition, the midpoint of the thermally induced transitions in infrared spectra were about 2.5 degrees C higher in D2O than in H2O. Based on the infrared analysis, the thermally denatured state of the protein in both H2O and D2O contains predominantly intermolecular beta-sheet. The broad, poorly resolved absorbance that spans the region between the intermolecular beta-sheet bands was assigned to an ensemble of heterogeneous structural elements (including unordered), none of which is populated to a high enough degree to result in a distinct infrared band. Results from CD spectroscopy support these conclusions about the structure of the denatured, aggregated protein.

Circular Dichroism↗
PubMed1997

Lonomia achelous caterpillar venom (LACV) selectively inactivates blood clotting factor XIII.

Lonomia Achelous Caterpillar Venom (LACV) causes a severe acquired bleeding diathesis in exposed persons. The condition is characterized by prolonged bleeding from superficial wounds and mucous membranes. Intracranial or intra-abdominal hemorrhage is a common and often fatal complication. A constant finding in these cases is a profound decrease of clotting Factor XIII (FXIII) activity. In the present study, the effects of LACV on plasmatic and purified human FXIII has been evaluated. The results show that native LACV and one of its chromatographically purified fractions (termed FIID) produce a dose dependent decrease of FXIII activity accompanied with an impairment of fibrin(ogen) crosslinking. We conclude that the inactivation of FXIII by fraction FIID (which will be named Lonomin V), is one of the principal patho-physiological mechanisms behind the acquired bleeding diathesis seen in these cases.

Animals↗
PubMed1988

Autoimmune antibody (IgG Kansas) against the fibrin stabilizing factor (factor XIII) system.

Serum from a patient who died from massive hemorrhage within 4 months after onset of an acquired bleeding disorder at age 85 contained a potent inhibitor of fibrin stabilization. Other parameters of coagulation and fibrinolysis and his bleeding time were within normal limits. The inhibitor was shown to be an IgG with kappa light chains (IgG Kansas); its specific target was the factor XIII system itself. Although IgG Kansas combined with the virgin [ab] form of the zymogen, it did not block the thrombin-catalyzed conversion to [a'b]. However, IgG Kansas prevented the subsequent Ca2+-mediated activation of [a'b] to a + b, where a denotes the catalytically competent factor XIIIa species. IgG Kansas, in contrast to a previously studied autoimmune antibody from a similar bleeding disorder (IgG Warsaw), could also inhibit the transamidating activity of the preactivated a enzyme.

Aged↗
PubMed2001

Fibrin formation and proteolysis during ancrod treatment. Evidence for des-A-profibrin formation and thrombin independent factor XIII activity.

Ancrod is a purified fraction of venom from the Malayan pit viper Calloselasma rhodostoma, containing a serine protease that cleaves fibrinopeptides A from fibrinogen. We report on a study that involved intravenous and subcutaneous application of ancrod in healthy subjects in which it was shown that ancrod induces the formation of desAA-fibrin complexes that are partially crosslinked by factor XIII proenzyme, and act as cofactor in tPA induced plasminogen activation. The plasmin generated degrades fibrin, as well as fibrinogen, leading to the appearance of large amounts of fibrinogen and fibrin degradation products in the circulation, including fragment D-dimer. At low concentrations of ancrod, formation of desAA-fibrin is preceded by production of desA-profibrin, lacking only one fibrinopeptide A.

Ancrod↗