5,026 results match your criteria Factor XIII

Quality of life of people with hereditary factor XIII deficiency treated at a reference centre.

Haemophilia 2021 Jul 30. Epub 2021 Jul 30.

Faculty of Medicine, Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil.

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A Case of Traumatic Cerebrospinal Fluid Rhinorrhea Successfully Treated Using Intravenous Factor XIII Administration.

Cureus 2021 Jun 14;13(6):e15633. Epub 2021 Jun 14.

Department of Neurosurgery, Kesennuma City Hospital, Kesennuma, JPN.

Traumatic cerebrospinal fluid (CSF) rhinorrhea occurs around 2% of severe head trauma. We should find the fistula and surgically seal it or perform conservative therapy with bed rest with/without lumbar spinal CSF drainage. However, the fistula may not be identified, and treatment may sometimes be challenging. Read More

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Mild Acquired Factor XIII Deficiency and Clinical Relevance at the ICU-A Retrospective Analysis.

Clin Appl Thromb Hemost 2021 Jan-Dec;27:10760296211024741

Institute of IMD Blood Coagulation Centre, Frankfurt/Bad Homburg, Germany.

Acquired FXIII deficiency is a relevant complication in the perioperative setting; however, we still have little evidence about the incidence and management of this rarely isolated coagulopathy. This study aims to help find the right value for the substitution of patients with an acquired mild FXIII deficiency. In this retrospective single-center cohort study, we enrolled critically ill patients with mild acquired FXIII deficiency (>5% and ≤70%) and compared clinical and laboratory parameters, as well as pro-coagulatory treatments. Read More

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Evaluation of trauma-induced coagulopathy in the fibrinogen in the initial resuscitation of severe trauma trial.

Transfusion 2021 Jul;61 Suppl 1:S49-S57

Department of Surgery, St. Michael's Hospital, Toronto, Ontario, Canada.

Background: Coagulopathic bleeding is frequently present after major trauma. However, trauma-induced coagulopathy (TIC) remains incompletely understood. This laboratory analysis of blood samples derived from our completed trial on fibrinogen in the initial resuscitation of severe trauma (FiiRST) was conducted to evaluate TIC and associated responses to fibrinogen replacement. Read More

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Fibrin Network Porosity and Endo-/Exogenous Thrombin Cross-talk.

Semin Thromb Hemost 2021 Jul 12. Epub 2021 Jul 12.

Department of Clinical Sciences, Danderyd Hospital, Karolinska Institutet, Stockholm, Sweden.

The earliest assessment of fibrin network porosity used a liquid permeation system and confocal 3D microscopy, which was later replaced by scanning electron microscopy. Although the methods have extensively been applied in studies of health or disease, there remains debate on the choice of a proper clotting trigger. In this review, we assess published data and convey our opinions with regard to several issues. Read More

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Differences in the biochemical composition of three plasma derived human fibrinogen concentrates.

Thromb Res 2021 Jul 6;205:44-46. Epub 2021 Jul 6.

TEM Innovations GmbH, Munich, Germany. Electronic address:

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Fibers Generated by Plasma Des-AA Fibrin Monomers and Protofibril/Fibrinogen Clusters Bind Platelets: Clinical and Nonclinical Implications.

TH Open 2021 Jul 6;5(3):e273-e285. Epub 2021 Jul 6.

Department of Cell and Developmental Biology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.

 Soluble fibrin (SF) is a substantial component of plasma fibrinogen (fg), but its composition, functions, and clinical relevance remain unclear. The study aimed to evaluate the molecular composition and procoagulant function(s) of SF.  Cryoprecipitable, SF-rich (FR) and cryosoluble, SF-depleted (FD) fg isolates were prepared and adsorbed on one hydrophilic and two hydrophobic surfaces and scanned by atomic force microscopy (AFM). Read More

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Decreased clot burden is associated with factor XIII Val34Leu polymorphism and better functional outcomes in acute ischemic stroke patients treated with intravenous thrombolysis.

PLoS One 2021 7;16(7):e0254253. Epub 2021 Jul 7.

Division of Clinical Laboratory Sciences, Department of Laboratory Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary.

Background: Intravenous thrombolysis using recombinant tissue plasminogen activator remains the mainstay treatment of acute ischemic stroke (AIS), although endovascular treatment is becoming standard of care in case of large vessel occlusions (LVO). To quantify the thrombus burden in LVO, a semiquantitative CT angiography (CTA) grading system, the clot burden score (CBS) can be used. Here we aimed to study the association between CBS and various hemostasis parameters, and to evaluate which parameters are major determinants of thrombolysis outcome. Read More

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Monocytes Expose Factor XIII-A and Stabilize Thrombi against Fibrinolytic Degradation.

Int J Mol Sci 2021 Jun 19;22(12). Epub 2021 Jun 19.

Aberdeen Cardiovascular & Diabetes Centre, Institute of Medical Sciences, University of Aberdeen, Aberdeen AB25 2ZD, UK.

Factor XIII (FXIII) is a transglutaminase that promotes thrombus stability by cross-linking fibrin. The cellular form, a homodimer of the A subunits, denoted FXIII-A, lacks a classical signal peptide for its release; however, we have shown that it is exposed on activated platelets. Here we addressed whether monocytes expose intracellular FXIII-A in response to stimuli. Read More

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Fibrin(ogen) as a Therapeutic Target: Opportunities and Challenges.

Int J Mol Sci 2021 Jun 28;22(13). Epub 2021 Jun 28.

Division of Cardiovascular & Diabetes Research, Leeds Institute of Cardiovascular and Metabolic Medicine (LICAMM), University of Leeds, Leeds LS2 9JT, UK.

Fibrinogen is one of the key molecular players in haemostasis. Thrombin-mediated release of fibrinopeptides from fibrinogen converts this soluble protein into a network of fibrin fibres that form a building block for blood clots. Thrombin-activated factor XIII further crosslinks the fibrin fibres and incorporates antifibrinolytic proteins into the network, thus stabilising the clot. Read More

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Predictive value of C-reactive protein for radiographic spinal progression in axial spondyloarthritis in dependence on genetic determinants of fibrin clot formation and fibrinolysis.

RMD Open 2021 06;7(2)

Department of Gastroenterology, Infectious Diseases and Rheumatology, Charité-Universitätsmedizin Berlin, Berlin, Germany.

Objective: Genetic determinants of fibrin clot formation and fibrinolysis have an impact on local and systemic inflammatory response. The aim of the present study was to assess whether coagulation-related genotypes affect the predictive value of C-reactive protein (CRP) in regards of radiographic spinal progression in axial spondyloarthritis (axSpA).

Methods: Two hundred and eight patients with axSpA from the German Spondyloarthritis Inception Cohort were characterised for genotypes of α-fibrinogen, β-fibrinogen (FGB) and γ-fibrinogen, factor XIII A-subunit (F13A) and α-antiplasmin (A2AP). Read More

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Elimination of fibrin γ-chain cross-linking by FXIIIa increases pulmonary embolism arising from murine inferior vena cava thrombi.

Proc Natl Acad Sci U S A 2021 Jul;118(27)

Leeds Thrombosis Collective, Discovery & Translational Science Department, Leeds Institute of Cardiovascular and Metabolic Medicine, University of Leeds, Leeds LS2 9NL, United Kingdom;

The onset of venous thromboembolism, including pulmonary embolism, represents a significant health burden affecting more than 1 million people annually worldwide. Current treatment options are based on anticoagulation, which is suboptimal for preventing further embolic events. In order to develop better treatments for thromboembolism, we sought to understand the structural and mechanical properties of blood clots and how this influences embolism in vivo. Read More

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Thrombin: A Pivotal Player in Hemostasis and Beyond.

Semin Thromb Hemost 2021 Jun 21. Epub 2021 Jun 21.

Department of Clinical Biochemistry, Thrombosis and Hemostasis Research Unit, Aarhus University Hospital, Aarhus, Denmark.

The serine protease thrombin, a naturally derived enzyme, plays a key role in hemostasis by converting fibrinogen to fibrin and activating coagulation factor XIII whereby the fibrin clot is stabilized. Furthermore, thrombin activates platelets through protease-activated receptors on the platelet surface. Conversely, thrombin also exerts anticoagulant effects, enhancing the protein C activity while complexed with thrombomodulin. Read More

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Genetic Background of Inherited Factor XIII-A Subunit Deficiency: Review of the Literature and Description of Two New Cases.

Semin Thromb Hemost 2021 Jun 10. Epub 2021 Jun 10.

Department of Hematology and Transfusion Medicine, National Centre of Hemostasis and Thrombosis, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, Martin, Slovakia.

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Expression and methylation status of vascular endothelial growth factor and thrombospondin-1 genes in congenital factor XIII-deficient patients with intracranial hemorrhage.

Blood Coagul Fibrinolysis 2021 Jul;32(5):317-322

Hematology Department, Allied Medical School, Tehran University of Medical Sciences, Tehran, Iran.

Congenital factor XIII (FXIII) deficiency is one of the rarest bleeding disorders, with an incidence of one per 2 million persons. Intracranial hemorrhage (ICH), a major cause of mortality in FXIII deficiency, is reported to be associated with vascular endothelial growth factor (VEGF) and thrombospondin-1 (TSP-1). Therefore, we investigated the association of VEGF and TSP-1 expression and methylation patterns with ICH in congenital FXIII deficiency patients. Read More

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A Rare Case of Factor XIII Deficiency in the Setting of Cancer Immunotherapy.

Khashayar Farzam

Cureus 2021 May 28;13(5):e15299. Epub 2021 May 28.

Family Medicine, University of Iowa Hospitals and Clinics, Iowa City, USA.

Factor XIII deficiency is a rare bleeding disorder, which may be congenital or acquired, and is most commonly diagnosed in early childhood. It has a prevalence that is as low as one in 5,000,000. Acquired factor XIII deficiency is considered to be a more rare form, with less than 100 cases reported in the literature. Read More

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Recombinant γY278H Fibrinogen Showed Normal Secretion from CHO Cells, but a Corresponding Heterozygous Patient Showed Hypofibrinogenemia.

Int J Mol Sci 2021 May 14;22(10). Epub 2021 May 14.

Department of Clinical Laboratory Investigation, Graduate School of Medicine, Shinshu University, Matsumoto 390-8621, Japan.

We identified a novel heterozygous hypofibrinogenemia, γY278H (Hiroshima). To demonstrate the cause of reduced plasma fibrinogen levels (functional level: 1.12 g/L and antigenic level: 1. Read More

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Alterations of the Platelet Proteome in Lung Cancer: Accelerated F13A1 and ER Processing as New Actors in Hypercoagulability.

Cancers (Basel) 2021 May 8;13(9). Epub 2021 May 8.

Centre for Physiology and Pharmacology, Institute of Vascular Biology and Thrombosis Research, Medical University of Vienna, 1090 Vienna, Austria.

In order to comprehensively expose cancer-related biochemical changes, we compared the platelet proteome of two types of cancer with a high risk of thrombosis (22 patients with brain cancer, 19 with lung cancer) to 41 matched healthy controls using unbiased two-dimensional differential in-gel electrophoresis. The examined platelet proteome was unchanged in patients with brain cancer, but considerably affected in lung cancer with 15 significantly altered proteins. Amongst these, the endoplasmic reticulum (ER) proteins calreticulin (CALR), endoplasmic reticulum chaperone BiP (HSPA5) and protein disulfide-isomerase (P4HB) were significantly elevated. Read More

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Fibrinolysis in Platelet Thrombi.

Int J Mol Sci 2021 May 12;22(10). Epub 2021 May 12.

Faculty of Medicine, National Heart and Lung Institute, Imperial College, London SW3 6LY, UK.

The extent and duration of occlusive thrombus formation following an arterial atherothrombotic plaque disruption may be determined by the effectiveness of endogenous fibrinolysis. The determinants of endogenous fibrinolysis are the subject of much research, and it is now broadly accepted that clot composition as well as the environment in which the thrombus was formed play a significant role. Thrombi with a high platelet content demonstrate significant resistance to fibrinolysis, and this may be attributable to an augmented ability for thrombin generation and the release of fibrinolysis inhibitors, resulting in a fibrin-dense, stable thrombus. Read More

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Combined Administration of Fibrinogen and Factor XIII Concentrate Does Not Improve Dilutional Coagulopathy Superiorly Than Sole Fibrinogen Therapy: Results of an In-Vitro Thrombelastographic Study.

J Clin Med 2021 May 12;10(10). Epub 2021 May 12.

Department of Anesthesiology, Operative Intensive Care Medicine and Pain Therapy, University Hospital of Giessen, 35392 Giessen, Germany.

The early administration of fibrinogen has gained wide acceptance for the treatment of major hemorrhage, whereas the substitution of coagulation factor XIII (FXIII) is only supported by a low level of evidence. This study aimed to answer the question of whether a combined therapy of fibrinogen/FXIII substitution performs superiorly to sole fibrinogen administration in the treatment of dilutional coagulopathy. An in-vitro model of massive transfusion was used to compare the effect of combined fibrinogen/FXIII administration to that of sole fibrinogen therapy for the treatment of dilutional coagulopathy. Read More

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Factor XIII Cross-Linked Adhesive Chitosan Hydrogels.

ACS Biomater Sci Eng 2021 06 27;7(6):2198-2203. Epub 2021 May 27.

Tissue Engineering + Biofabrication Laboratory, Department of Health Sciences & Technology, ETH Zürich, Otto-Stern-Weg 7, 8093 Zürich, Switzerland.

Biomedical adhesives have been found to be an attractive alternative to suturing in several circumstances. However, to date most of the clinically approved formulations are based on synthetic and highly reactive toxic chemicals. In this work, we aimed to combine for the first time the bioactive properties of the cationic polysaccharide chitosan and its intrinsic electrostatic binding to negatively charged tissues with the biocompatible and clinically compliant enzymatic cross-linking scheme of fibrin glue. Read More

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Factor XIII activity at onset of labour and association with postpartum haemorrhage: an exploratory post-hoc study.

Int J Obstet Anesth 2021 Aug 1;47:103174. Epub 2021 May 1.

Department of Obstetrics, Sahlgrenska University Hospital, Gothenburg, Sweden; Institute of Clinical Science, Sahlgrenska Academy, University of Gothenburg, Sweden.

Background: Platelets, fibrinogen and factor XIII (FXIII) are required to form a stable clot in case of haemorrhage. The aims of this study were to evaluate a possible association between FXIII activity at the onset of labour and postpartum haemorrhage (PPH), and to ascertain whether FXIII activity at labour onset differs from after delivery.

Methods: FXIII activity in 239 women with PPH (blood loss >1 L) and in 76 women without PPH was compared, as was activity before and after delivery in a third group of 80 women. Read More

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Hemorrhagic Shock after Neonatal Circumcision: Severe Congenital Factor XIII Deficiency.

Case Rep Pediatr 2021 3;2021:5550199. Epub 2021 May 3.

Department of Pediatrics, Section of Pediatric Hematology, Oncology, Stem Cell Transplant, Comer Children's Hospital, Pritzker School of Medicine, University of Chicago, Chicago, IL, USA.

A Caucasian male infant born full term via normal spontaneous vaginal delivery was given vitamin K after birth, circumcised on day of life (DOL) 1, and discharged from the nursery on DOL 2. At the time of circumcision, oozing from the surgical site was noted and initially resolved with silver nitrate. Over the next two days, he presented to local emergency rooms multiple times for recurrent bleeding, eventually developing hemorrhagic shock resulting in admission to the neonatal intensive care unit. Read More

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Biomarkers as a Prognostic Factor in COPD Exacerbation: A Cohort Study.

COPD 2021 Jun 10;18(3):325-332. Epub 2021 May 10.

Internal Medicine Department, Medical School, University of Antioquia, Medellín, Colombia.

The acute exacerbations of COPD (AECOPD) are one of the main causes of hospitalization and morbimortality in the adult population. There are not many tools available to predict the clinical course of these patients during exacerbations. Our goal was to estimate the clinical utility of C Reactive Protein (CRP), Mean Platelet Volume (MPV), eosinophil count and neutrophil/lymphocyte ratio (NLR) as in-hospital prognostic factors in patients with AECOPD. Read More

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Immunoglobulin A vasculitis without purpura in an elderly female patient: a case report.

Clin J Gastroenterol 2021 Aug 5;14(4):1090-1095. Epub 2021 May 5.

Department of Gastroenterology, National Hospital Organization Kyoto Medical Center, 1-1 Mukaihatacho, Fukakusa, Kyoto, 612-8555, Japan.

Immunoglobulin A (IgA) vasculitis mainly affects the joints, skin, kidneys, and gastrointestinal tract; however, purpura is an essential diagnostic criterion. Here, we report an unusual case of IgA vasculitis without purpura in an elderly woman. A 76-year-old woman was admitted to our hospital complaining of diarrhea and abdominal pain. Read More

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Factor XIII Deficiency Associated With Noonan Syndrome.

Cureus 2021 Mar 27;13(3):e14150. Epub 2021 Mar 27.

Medicine, Enloe Medical Center, Enloe Regional Cancer Center, California, USA.

Noonan syndrome (NS) is an autosomal dominant disorder with multisystem involvement. NS can be associated with bleeding disorders due to defects in platelet function or coagulation factors and diagnosis can be challenging. Factor XIII (FXIII) deficiency is uncommon in patients with NS. Read More

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[Hereditary coagulation factor XIII deficiency: three cases report and literaure review].

Zhonghua Xue Ye Xue Za Zhi 2021 03;42(3):256-258

Department of Hematology, The Affiliated Cancer Hospital of Zhengzhou University (Henan Cancer Hospital), Zhengzhou 450008, China.

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Potential predictors of the onset of focal intestinal perforation in extremely low birth weight infants based on an analysis of coagulation and fibrinolysis markers at birth: A case-control study based on ten years of experience at a single institution.

J Pediatr Surg 2021 Jul 28;56(7):1121-1126. Epub 2021 Mar 28.

Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University, Kagoshima, Japan. Electronic address:

Purpose: We aimed to investigate potential predictors of focal intestinal perforation (FIP) in extremely low birth weight infants (ELBWIs) among coagulation and fibrinolysis markers at birth.

Methods: We reviewed the medical records of FIP patients and their coagulation and fibrinolysis markers at birth between 2010 and 2019, and matched patients according to gestational age. FIP was diagnosed based on macroscopic intestinal perforation with a punched-out lesion without necrosis. Read More

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Acquired FXIII Deficiency is Associated with High Morbidity.

Thromb Haemost 2021 Apr 13. Epub 2021 Apr 13.

Department of Hematology, Gregorio Marañon Hospital, Madrid, Spain.

Background:  A factor XIII (FXIII) level >30% is considered necessary to prevent spontaneous bleeding. Bleeding is also a risk in patients with acquired FXIII deficiency, but the hemostatic level of FXIII in this context remains to be determined.

Methods:  We retrospectively analyzed all patients diagnosed with acquired FXIII deficiency at a large hospital over 3 years (study ID NCT04416594, http://www. Read More

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