5,142 results match your criteria Factor XIII


A novel F13A1 gene mutation (Arg208Pro) in a Chinese patient with factor XIII deficiency.

Blood Coagul Fibrinolysis 2022 Sep;33(6):337-341

Department of Clinical Laboratory, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China.

The objective of the study was to analyse a novel F13A1 gene mutation in a Chinese patient with factor XIII (FXIII) deficiency and explore the molecular mechanism. Pedigree investigation, clinical diagnosis, phenotypic and genetic analysis were conducted. The F13A1 gene was amplified by PCR and directly sequenced. Read More

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September 2022

Transient Platelet Dysfunction in Congenital Factor XIII Deficiency with Enhanced Thrombin Generation Potential.

Clin Lab 2022 Aug;68(8)

Background: Congenital factor XIII (FXIII) deficiency is an extremely rare bleeding disorder with defects in the F13A1 or F13B genes. Here, we report a case of congenital FXIII deficiency patient who presented with trauma-induced intramuscular hemorrhage accompanied with transient platelet dysfunction with increased endogenous thrombin potential (ETP).

Methods: FXIII antigen and activity, F13A1 gene sequencing, and thrombin generation assay were measured. Read More

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Preparation and Storage of Cryoprecipitate Derived from Amotosalen and UVA-Treated Apheresis Plasma and Assessment of In Vitro Quality Parameters.

Pathogens 2022 Jul 18;11(7). Epub 2022 Jul 18.

Department of Blood Group Serology and Transfusion Medicine, Medical University Graz, Auenbruggerplatz 48, A-8036 Graz, Austria.

Cryoprecipitate is a plasma-derived blood product, enriched for fibrinogen, factor VIII, factor XIII, and von Willebrand factor. Due to infectious risk, the use of cryoprecipitate in Central Europe diminished over the last decades. However, after the introduction of various pathogen-reduction technologies for plasma, cryoprecipitate production in blood centers is a feasible alternative to pharmaceutical fibrinogen concentrate with a high safety profile. Read More

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Factor XIII Measurement and Substitution in Trauma Patients after Admission to an Intensive Care Unit.

J Clin Med 2022 Jul 19;11(14). Epub 2022 Jul 19.

Department of Anaesthesiology and Intensive Care Medicine, AUVA Trauma Center Linz, 4010 Linz, Austria.

Trauma patients admitted to an intensive care unit (ICU) may potentially experience a deficiency of coagulation factor thirteen (FXIII). In this retrospective cohort study conducted at a specialized trauma center, ICU patients were studied to determine the dependency of FXIII activity levels on clinical course and substitution with blood and coagulation products. A total of 189 patients with a median injury severity score (ISS) of 25 (16-36, IQR) were included. Read More

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Exploring the function of factor XIII free B subunit: Interactions with complement factors and a novel approach to identify potential binding partners.

Res Pract Thromb Haemost 2022 Jul 21;6(5):e12766. Epub 2022 Jul 21.

Experimental Haemostasis Group, Department for BioMedical Research DBMR University of Bern Bern Switzerland.

Background: The factor XIII (FXIII)-B subunit has a critical function as a carrier protein to stabilize FXIII-A in plasma and supply it to its main substrate, fibrinogen. However, the function of the excess free FXIII-B circulating in plasma is still elusive.

Objectives: In the present study, we explored potential interactions of free FXIII-B with complement factors and searched for novel binding partners. Read More

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Hyperfibrinolysis secondary to acquired factor XIII deficiency A case report.

Medicine (Baltimore) 2022 Jul 22;101(29):e29446. Epub 2022 Jul 22.

Department of Hematology, The Lu'an Hospital Affiliated to Anhui Medical University, Lu'an People's Hospital, Luan, Anhui 237000, China.

Introduction: Hyperfibrinolysis induced by factor XIII deficiency (FXIIID) is extremely rare, and patients with no manifestations of active bleeding can easily and frequently be neglected in clinical practice, leading to a missed diagnosis. Herein, we report a rare case of idiopathic FXIIID with secondary hyperfibrinolysis.

Patient Concerns: A 69-year-old man presented with ecchymosis of the right arm and chest wall. Read More

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Large cavernous carotid artery aneurysm with spontaneous thrombosis: is there more to a change in morphology than there seems to be? Illustrative case.

J Neurosurg Case Lessons 2021 Aug 16;2(7):CASE21288. Epub 2021 Aug 16.

Department of Neurosurgery, School of Medicine, Yokohama City University, Kanagawa, Japan.

Background: The pathogenesis and endovascular treatment strategy for spontaneously thrombosed unruptured cerebral aneurysms have not yet been comprehensively described.

Observations: The authors reported on a 78-year-old woman who had large bilateral unruptured cavernous carotid artery aneurysms that induced chronic disseminated intravascular coagulation and acquired factor XIII deficiency. The right aneurysm was symptomatic and partially thrombosed. Read More

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Congenital (hypo-)dysfibrinogenemia and bleeding: A systematic literature review.

Thromb Res 2022 Sep 14;217:36-47. Epub 2022 Jul 14.

Department of Laboratory Medicine, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China; Collaborative Innovation Center of Hematology, Shanghai Jiaotong University School of Medicine, Shanghai, China. Electronic address:

Ranging from bleeding to thrombosis, the clinical features of congenital fibrinogen qualitative disorders, including dysfibrinogenemia and hypodysfibrinogenemia, are highly heterogeneous. Although the associations between some specific fibrinogen mutations and the thrombotic phenotypes have been well elucidated, the underlying mechanism between fibrinogen variants and bleeding events remains underestimated. After systematically reviewing the literature of (hypo-)dysfibrinogenemia patients with bleeding phenotypes, we identified several well-characterized bleeding-related fibrinogen variants in those patients. Read More

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September 2022

Prediction of adult post-hemorrhagic hydrocephalus: a risk score based on clinical data.

Sci Rep 2022 Jul 16;12(1):12213. Epub 2022 Jul 16.

Department of Neurosurgery, Jiangxi Provincial People's Hospital, Fenghe North Rd No. 266, Nanchang, 330000, Jiangxi Province, China.

There is lacking research on risk factors and prediction models associated with Post-hemorrhagic hydrocephalus (PHH). Thus, this present study aimed to analyze the risk factors of PHH and establish a risk-scoring system through a large-scale study. A retrospective study of 382 patients with intracranial hemorrhage assessed age, history and diagnosis, Glasgow coma score (GCS), and fever time. Read More

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Autoimmune acquired factor XIII deficiency in Japan 2021 update: Focused on annual incidence and clinical features.

Haemophilia 2022 Jun 29. Epub 2022 Jun 29.

The Japanese Collaborative Research Group (JCRG) on Autoimmune Coagulation Factor Deficiency (AiCFD) supported by the Japanese Ministry of Health, Labor, and Welfare (MHLW).

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Fibrinolytic Proteins and Factor XIII as Predictors of Thrombotic and Hemorrhagic Complications in Hospitalized COVID-19 Patients.

Front Cardiovasc Med 2022 10;9:896362. Epub 2022 Jun 10.

Department of Immunohematology and Transfusion Medicine, Hospital Papa Giovanni XXIII, Bergamo, Italy.

Introduction: In a prospective cohort of hospitalized COVID-19 patients, an extensive characterization of hemostatic alterations by both global and specific assays was performed to clarify mechanisms underlying the coagulopathy and identify predictive factors for thrombotic and hemorrhagic events during hospitalization.

Materials And Methods: Intensive care unit (ICU; = 46) and non-ICU ( = 55) patients were enrolled, and the occurrence of thrombotic and hemorrhagic events was prospectively monitored. At study inclusion, thromboelastometry together with the measurement of specific coagulation proteins and hypercoagulation markers was performed. Read More

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Effects of Interleukin-17A on the Early Stages of Arterial Thrombosis in Mice.

Yonsei Med J 2022 Jul;63(7):632-639

Department of Neurology, Yonsei University College of Medicine, Seoul, Korea.

Purpose: Interleukin (IL)-17A has been suggested to play a role in the growth and organization of thrombi. We examined whether IL-17A plays a role in the early stages of thrombosis and whether there are sex differences in the effects of IL-17A.

Materials And Methods: We performed a blinded, randomized, placebo-controlled study to compare time to thrombotic occlusion and sex differences therein between mice treated with IL-17A and those treated with saline using a ferric chloride-induced model. Read More

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Factor XIII deficiency in a neonate presenting as subpial haemorrhage.

SA J Radiol 2022 20;26(1):2344. Epub 2022 May 20.

Department of Radiodiagnosis, Mahatma Gandhi Medical College and Research Institute, Pondicherry, India.

Subpial haemorrhage is a rare cause of seizures in term neonates. A 3-day-old male infant, born at term with no history of perinatal hypoxia, presented with seizures and unremarkable physical examination in the interictal state. Imaging demonstrated left temporal subpial haemorrhage with the classic 'yin-yang sign' on MRI. Read More

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Coagulation Factor XIII Val34Leu Polymorphism in the Prediction of Premature Cardiovascular Events-The Results of Two Meta-Analyses.

J Clin Med 2022 Jun 15;11(12). Epub 2022 Jun 15.

Department of Electrocardiology and Heart Failure, Faculty of Health Sciences in Katowice, Medical University of Silesia, 40-635 Katowice, Poland.

Background: Polymorphisms within the gene that encodes for coagulation factor XIII (FXIII) have been suggested to be involved in the pathogeneses of ischemic stroke (IS) and myocardial infarction (MI). The Val34Leu polymorphism is one of the most commonly analysed polymorphisms. However, studies on the role of the Val34Leu polymorphism in the aetiology of vascular diseases often show contradictory results. Read More

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Molecular Detection of Venous Thrombosis in Mouse Models Using SPECT/CT.

Biomolecules 2022 06 13;12(6). Epub 2022 Jun 13.

Department of Biochemistry, CARIM-School for Cardiovascular Diseases, Maastricht University, 6229 ER Maastricht, The Netherlands.

The efficacy of thrombolysis is inversely correlated with thrombus age. During early thrombogenesis, activated factor XIII (FXIIIa) cross-links α2-AP to fibrin to protect it from early lysis. This was exploited to develop an α2-AP-based imaging agent to detect early clot formation likely susceptible to thrombolysis treatment. Read More

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New Mechanisms of Bromelain in Alleviating Non-Alcoholic Fatty Liver Disease-Induced Deregulation of Blood Coagulation.

Nutrients 2022 Jun 1;14(11). Epub 2022 Jun 1.

Department and Graduate Institute of Physiology, College of Medicine, National Taiwan University, Taipei 10051, Taiwan.

Bromelain, an enzyme extracted from the stems of pineapples, exerts anticoagulant effects; however, the regulatory mechanisms are not fully understood. Here, we aimed to investigate the effects of bromelain on non-alcoholic fatty liver disease (NAFLD)-induced deregulation of blood coagulation and the underlying molecular mechanisms. C57BL/6 mice were fed a high-fat diet (HFD), with or without bromelain (20 mg/kg/day) administration, for 12 weeks. Read More

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Factor XIII-A Val34Leu and Tyr204Phe variants influence clot kinetics in a cohort of South African type 2 diabetes mellitus patients.

Gene 2022 Aug 4;834:146637. Epub 2022 Jun 4.

Department of Physiology, School of Medicine, Faculty of Health Sciences, University of Pretoria, Gauteng, South Africa. Electronic address:

Factor XIII, a transglutaminase that plays a crucial role in clot formation, consists of subunits A and B. Single nucleotide polymorphisms in Factor XIII-A have been linked to thrombotic risk. In Type 2 Diabetes mellitus (T2DM), a hypercoagulable state is thought to contribute to the high mortality rate associated with thrombotic diseases. Read More

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Fibrinogen and Factor XIII in Venous Thrombosis and Thrombus Stability.

Arterioscler Thromb Vasc Biol 2022 08 2;42(8):931-941. Epub 2022 Jun 2.

Department of Pathology and Laboratory Medicine and UNC Blood Research Center, University of North Carolina, Chapel Hill.

As the third most common vascular disease, venous thromboembolism is associated with significant mortality and morbidity. Pathogenesis underlying venous thrombosis is still not fully understood. Accumulating data suggest fibrin network structure and factor XIII-mediated crosslinking are major determinants of venous thrombus mass, composition, and stability. Read More

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Global hemostatic profiling in patients with decompensated cirrhosis and bacterial infections.

JHEP Rep 2022 Jul 20;4(7):100493. Epub 2022 Apr 20.

General Internal Medicine and Thrombotic and Hemorrhagic Diseases Unit, Department of Medicine, Padova University Hospital, Padova, Italy.

Background & Aims: Bacterial infections in cirrhosis are associated with increased bleeding risk. To assess the factors responsible for bleeding tendency in patients with bacterial infections, we conducted a prospective study comparing all 3 aspects of hemostasis (platelets, coagulation, and fibrinolysis) in hospitalized patients with decompensated cirrhosis with . without bacterial infections. Read More

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Dietary Fiber Supplementation in Gestating Sow Diet Improved Fetal Growth and Placental Development and Function Through Serotonin Signaling Pathway.

Front Vet Sci 2022 12;9:831703. Epub 2022 May 12.

Key Laboratory for Animal Disease-Resistance Nutrition of the Ministry of Agriculture, Animal Nutrition Institute, Sichuan Agricultural University, Chengdu, China.

The experiment was conducted to investigate the effects of dietary fiber (DF) supplementation in gestation diet on fetal growth and placental development and function and explore the possible mechanism of DF improving sow reproductive performance. A total of 16 Large White × Landrace crossbred gilts were randomly allotted to two groups and fed a semi-purified basal diet [non-fiber (NF) group, 0.1% total DF] or a basal diet supplemented with 8. Read More

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Ethacrynic acid is an inhibitor of human factor XIIIa.

BMC Pharmacol Toxicol 2022 06 1;23(1):35. Epub 2022 Jun 1.

Division of Basic Pharmaceutical Sciences, College of Pharmacy, Xavier University of Louisiana, 1 Drexel Drive, New Orleans, LA, 70125-1089, USA.

Background: Ethacrynic acid (EA) is a loop diuretic that is approved orally and parenterally to manage edema-associated diseases. Nevertheless, it was earlier reported that it is also associated with bleeding upon its parenteral administration. In this report, we investigated the effects of EA on human factor XIIIa (FXIIIa) of the coagulation process using a variety of techniques. Read More

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The Effect of Activated FXIII, a Transglutaminase, on Vascular Smooth Muscle Cells.

Int J Mol Sci 2022 May 23;23(10). Epub 2022 May 23.

Division of Clinical Laboratory Science, Department of Laboratory Medicine, Faculty of Medicine, University of Debrecen, 98 Nagyerdei Krt, H-4032 Debrecen, Hungary.

Plasma factor XIII (pFXIII) is a heterotetramer of FXIII-A and FXIII-B subunits. The cellular form (cFXIII), a dimer of FXIII-A, is present in a number of cell types. Activated FXIII (FXIIIa), a transglutaminase, plays an important role in clot stabilization, wound healing, angiogenesis and maintenance of pregnancy. Read More

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Factor XIII deficiency: Lessons from two patients with unusual bleeding.

Authors:
Suvir Singh

Natl Med J India 2021 Sep-Oct;34(5):276-278

Department of Clinical Haematology and Bone Marrow Transplantation, Dayanand Medical College, Ludhiana, Punjab, India.

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Exploring Diverse Coagulation Factor XIII Subunit Expression Datasets: A Bioinformatic Analysis.

Int J Mol Sci 2022 Apr 25;23(9). Epub 2022 Apr 25.

Institute of Experimental Hematology and Transfusion Medicine, University Hospital of Bonn, Building 043, Venusberg Campus 1, 53127 Bonn, Germany.

Coagulation factor XIII (FXIII) circulates in plasma as a pro-transglutaminase heterotetrameric complex (FXIIIAB), which upon activation by thrombin and calcium covalently crosslinks preformed fibrin polymers. The heterotetrameric complex is composed of a catalytic FXIIIA subunit and a protective/regulatory FXIII-B subunit coded by and genes, respectively. The catalytic FXIIIA subunit is encoded by the gene, expressed primarily in cells of mesenchymal origin, whereas the FXIIIB subunit encoded by the gene is expressed and secreted from hepatocytes. Read More

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Characterization of laboratory coagulation parameters and risk factors for intraventricular hemorrhage in extremely premature neonates.

J Thromb Haemost 2022 Aug 22;20(8):1797-1807. Epub 2022 May 22.

Bleeding & Clotting Disorders Institute, Peoria, Illinois, USA.

Background: Extremely premature neonates have increased risk for bleeding, perhaps the most devastating version of which being intraventricular hemorrhage (IVH). Limited data are available for coagulation parameters in this vulnerable population.

Objectives: We conducted a prospective cohort study characterizing coagulation laboratory parameters in extremely premature neonates 23-30 weeks gestational age (GA) and determined coagulation parameters and clinical risk factors associated with IVH. Read More

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Effect of Minocycline Pleurodesis in Infants With Refractory Chylothorax After Palliative Surgery for Complex Congenital Heart Disease.

Cureus 2022 Mar 26;14(3):e23506. Epub 2022 Mar 26.

Pediatric Neurology, School of Medicine, Iwate Medical University, Shiwa, JPN.

Chylothorax is a critical complication after surgery for congenital heart disease, which markedly compromises the postoperative course with increased mortality. As the cardiovascular load additively causes stagnation of the thoracic duct, chylothorax after palliative cardiac surgery can be highly refractory to the therapies. Here we report a case of two patients with refractory chylothorax attributed to hemodynamic load which was successfully treated with minocycline pleurodesis. Read More

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Factor XIII is a newly identified binding partner for platelet collagen receptor GPVI-dimer-An interaction that may modulate fibrin crosslinking.

Res Pract Thromb Haemost 2022 Mar 24;6(3):e12697. Epub 2022 Apr 24.

Department of Biochemistry University of Cambridge Cambridge UK.

Background: In the fibrin-forming process, thrombin cleaves fibrinogen to fibrin, which form fibrils and then fibers, producing a gel-like clot. Thrombin also activates coagulation factor XIII (FXIII), which crosslinks fibrin γ-chains and α-chains, stabilizing the clot. Many proteins bind to fibrin, including FXIII, an established regulation of clot structure, and platelet glycoprotein VI (GPVI), whose contribution to clot function is largely unknown. Read More

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Acquired Factor XIII Deficiency in a 19-year-Old Male Following Ballistic Injury.

Am Surg 2022 Apr 27:31348221091970. Epub 2022 Apr 27.

Division of Trauma and Critical Care, Department of Surgery, 21693University of Mississippi Medical Center, Jackson, MS, USA.

Factor XIII deficiency is a rare cause of post-operative bleeding. It poses a diagnostic challenge as standard coagulation tests including prothrombin time, international normalized ratio, and activated partial thromboplastin time are usually normal in factor XIII deficiency. We present the case of our patient, a 19-year-old male with acquired factor XIII deficiency diagnosed after ballistic injury with a post-operative course complicated by hemorrhage. Read More

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