26 results match your criteria: "Alpha2-Plasmin Inhibitor Deficiency"

Detection of factor XIII inhibitors in 33 patients with autoimmune factor XIII deficiency in Japan.

Int J Hematol

October 2024

Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Yamagata, 990-9585, Japan.

Article Synopsis
  • Autoimmune factor XIII (FXIII) deficiency (AiF13D) is a rare disease caused by anti-FXIII autoantibodies that disrupt FXIII function, classified into three types with different effects on FXIII activity.
  • A new assay, called the PI-BAPA assay, has been developed to effectively detect types Aa and Ab FXIII inhibitors which can be lethal if missed by standard tests.
  • This assay demonstrated high specificity and sensitivity in trials with 128 plasma samples, outperforming traditional methods, and is now being utilized for diagnosing AiF13D.
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Cross-linking of α2-plasmin inhibitor (α2-PI) to fibrin by activated factor XIII (FXIIIa) is essential for the inhibition of fibrinolysis. Little is known about the factors modifying α2-PI incorporation into the fibrin clot and whether the extent of incorporation has clinical consequences. Herein we calculated the extent of α2-PI incorporation by measuring α2-PI antigen levels from plasma and serum obtained after clotting the plasma by thrombin and Ca.

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Article Synopsis
  • A 64-year-old man developed ligneous conjunctivitis at 58 after lung surgery for suspected cancer, leading to chronic mucositis symptoms.
  • He was diagnosed with severe type I plasminogen deficiency, having low plasminogen activity and novel genetic mutations affecting his plasminogen protein.
  • Despite these conditions, he hasn't experienced thrombosis or recurrence of conjunctivitis for about 2.5 years, indicating a potential alternative fibrinolytic mechanism at play.
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Article Synopsis
  • Human α2-antiplasmin (α2AP) is the key protein that inhibits plasmin, an enzyme that breaks down blood clots; imbalances in α2AP levels can lead to bleeding issues or increased risk of blood clots.
  • α2AP is a complex protein that undergoes significant changes at both its N-terminal and C-terminal ends, affecting its ability to function and bind to other molecules necessary for blood clot regulation.
  • Recent studies highlight the importance of these modifications in α2AP's activity, suggesting potential clinical implications for managing conditions related to blood clotting disorders.
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Background: Endothelial protein C receptor (EPCR), expressed predominantly on endothelial cells, plays a critical role in the regulation of the coagulation system and also mediates various cytoprotective effects by binding and activating protein C. So far, the role of EPCR has not been studied in systemic sclerosis (SSc).

Objectives: To investigate the potential contribution of EPCR to the development of SSc.

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Article Synopsis
  • Factor XIII (FXIII) is essential for stabilizing blood clots, and defects in its A or B subunits can lead to severe bleeding disorders, requiring ongoing FXIII replacement therapy.
  • A 30-year-old male was diagnosed with severe FXIII-A deficiency after comprehensive testing showed undetectable FXIII activity and low protein levels, but normal FXIII-B antigen.
  • Genetic analysis identified two novel mutations in the F13A gene, confirming a hereditary condition and allowing for a targeted management plan to address his deficiency.
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Coagulation factor XIII (FXIII) exists as heterotetramer (FXIII-A₂B₂) in the plasma and as dimer (FXIII-A₂) in cells. Activated FXIII mechanically stabilizes fibrin and protects it from fibrinolysis by cross-linking fibrin chains and α₂-plasmin inhibitor to fibrin. FXIII is essential to maintaining haemostasis, and its deficiency causes severe bleeding diathesis.

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Introduction: It has been shown that thrombomodulin (TM) considerably delays factor XIII (FXIII) activation and this effect is abrogated by Factor V Leiden (FV(Leiden)) mutation. The aim of the study was to explore the effect of TM on the cross-linking of α(2)-plasmin inhibitor (α(2)-PI) to fibrin in plasma samples of different FV genotypes and how this effect is related to the impaired fibrinolysis of FV(Leiden) carriers.

Methods: In the plasma samples of fifteen individuals with different FV genotypes and in FV deficient plasma supplemented with wild type FV or FV(Leiden) coagulation was initiated by recombinant human tissue factor and phospholipids with or without recombinant human TM (rhTM).

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Changes of proteases and proteinase inhibitors in androgen-dependent advanced prostate cancer patients with alpha2-macroglobulin deficiency.

Clin Lab

June 2012

Department of Laboratory Medicine, School of Medicine, Kitasato University, 1-15-1 Kitasato, Sagamihara, Minami-ku, Kanagawa 252-0374, Japan.

Article Synopsis
  • There is a believed link between the imbalance of proteases and their inhibitors and the spread of cancer, particularly in advanced prostate cancer (PCa).
  • The study examined serum levels of PSA, MMP-2, alpha2M, and alpha2-plasmin inhibitor in 33 advanced PCa patients, revealing that those with low alpha2M had higher PSA and MMP-2 levels.
  • The results suggest that measuring these proteases and inhibitors, in addition to PSA, could help indicate the progression of PCa.
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Coagulation factor XIII/13 (FXIII/13) stabilizes fibrin molecules by creating crosslinks with other fibrin molecules as well as with α₂-plasmin inhibitor (α₂-PI). "Hemorrhagic acquired FXIII/13 deficiency" was formerly considered rare, but has been increasing recently in Japan. During the 10 months of our nationwide campaign, we diagnosed five new patients with "acquired hemorrhaphilia due to anti-FXIII/13 autoantibodies," after examining 20 newly suspected cases of "hemorrhagic acquired FXIII/13 deficiency.

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[Interpretation of hemostatic and fibrinolytic markers].

Rinsho Byori

October 2011

Department of Internal Medicine (III), Kanazawa University School of Medicine, Kanazawa 920-8641, Japan.

Article Synopsis
  • Blood normally doesn't clot in vessels due to endothelial cells releasing antithrombotic substances like thrombomodulin and nitric oxide, ensuring hemostasis.
  • Coagulation occurs through two pathways: intrinsic (activated by internal factors) and extrinsic (triggered by external factors like tissue factor), with tests like prothrombin time (PT) and activated partial thromboplastin time (APTT) to measure their function.
  • Markers such as D-dimer and thrombin-antithrombin complex help diagnose conditions like DIC and DVT, and understanding these markers is crucial for interpreting hemostasis and fibrinolysis in clinical settings.
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Epistaxis, superficial and deep hematomas, hemarthrosis, gastrointestinal bleeding, hematuria represent the most frequent hemorrhagic events in congenital coagulation disorders. Occasionally, bleeding manifestations occur in unusual sites or are peculiar. A clotting defect may alter the clinical aspect of skin conditions or infections (hemorrhagic scabies or varicella).

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Article Synopsis
  • The report discusses a patient with a deficiency in alpha2 plasmin inhibitor (alpha2PI), who experienced atherosclerosis and myocardial ischemia despite having several traditional risk factors.
  • The patient's treatment was challenging due to concerns about bleeding risks from antiplatelet medications and existing serious health issues.
  • While the specific role of alpha2PI in atherosclerosis and abnormal clotting is not yet well understood, this case highlights that low levels of alpha2PI can still contribute to cardiovascular problems.
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Circadian clock molecules CLOCK and CRYs modulate fibrinolytic activity by regulating the PAI-1 gene expression.

J Thromb Haemost

November 2006

Department of Clinical Molecular Biology, Faculty of Pharmaceutical Sciences, Teikyo University, Kanagawa, Japan.

Disruptions of circadian rhythms are associated with the development of many disorders. However, whether a disruption of the circadian clock can cause anomalies of the hemostatic balance remains unknown. The present study examines coagulation and fibrinolytic activities in circadian clock mutants, a homozygous Clock mutant and Cry1/Cry2 double knockout (Cry1/2-deficient) mice.

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We have recently demonstrated that interleukin (IL)-6 is protective against coagulatory and hemostatic disturbance and subsequent pulmonary hemorrhage induced by bacterial endotoxin, at least partly, via the inhibition of proinflammatory cytokines and chemokines using IL-6-null [IL-6(-/-)] mice and corresponding wild-type mice. Its role in fibrinolytic systems remains undefined, however. The present study elucidated the role of IL-6 in the activity of alpha(2)-plasmin inhibitor, an inhibitor of fibrinolysis, during inflammation induced by intraperitoneal administration of lipopolysaccharide in IL-6(-/-) and wild-type mice.

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Acute haemostatic changes in accidentally traumatised dogs.

Vet J

January 2005

Small Animal Clinic, Hannover School of Veterinary Medicine, Bischofsholer Damm 15, 30173 Hannover, Germany.

Article Synopsis
  • A study on 30 dogs after natural injuries showed significant decreases in platelet counts and coagulation factor activities, indicating a compromised haemostatic system.
  • Exceptions included stable alpha2-plasmin inhibitor levels and increased fibrinogen concentration, highlighting their roles in acute responses.
  • The findings suggest that acutely injured dogs often experience serious deficiencies in haemostatic components, as shown by abnormal screening test results and elevated fibrin degradation products.
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[Alpha2-plasmin inhibitor (alpha2PI)].

Nihon Rinsho

December 2004

Cell and Molecular Medicine, Center for Molecular Medicine, Jichi Medical School.

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Haemostatic management of intraoral bleeding was investigated in patients with congenital alpha2-plasmin inhibitor (alpha2-PI) deficiency or congenital plasminogen activator inhibitor- 1 (PAI-1) deficiency. When extracting teeth from patients with congenital alpha2-PI deficiency, we advocate that 7.5-10 mg kg(-1) of tranexamic acid be administered orally every 6 h, starting 3 h before surgery and continuing for about 7 days.

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A case of intramedullary haematoma associated with congenital alpha2-plasmin inhibitor deficiency.

Pediatr Radiol

December 1998

Service de Radiologie Pédiatrique, Hôpital d'Enfants Armand-Trousseau, 26 avenue du Dr Arnold-Netter, F-75 571 Paris Cedex 12, France.

The association of intramedullary haematoma of the left femoral diaphysis and congenital alpha2-plasmin inhibitor deficiency in a 6-year-old boy is reported. Congenital deficiency of this fibrinolytic system component is very rare and can result in severe bleeding. Clinically, an intramedullary haematoma causes pain but no swelling, and there is no history of trauma.

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The concentration of alpha 2-plasmin inhibitor in blood plasma is higher than that in serum obtained from the blood clotted in the presence of calcium ions, but is the same as that in serum obtained in the absence of calcium ions. Radiolabeled alpha2-plasmin inhibitor was covalently bound to fibrin only when calcium ions were present at the time of clotting of plasma or fibrinogen. Whereas, when batroxobin, a snake venom enzyme that lacks the ability to activate fibrin-stabilizing factor, was used for clotting fibrinogen, the binding was not observed.

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