Search results for "Factor X"
showing 10 items of 80 documents
Severe plasma prekallikrein deficiency : clinical characteristics, novel KLKB1 mutations, and estimated prevalence
2020
BACKGROUND Severe plasma prekallikrein (PK) deficiency is an autosomal-recessive defect characterized by isolated activated partial thromboplastin time prolongation. To date, no comprehensive methodologically firm analysis has investigated the diagnostic, clinical, and genetic characteristics of PK deficiency, and its prevalence remains unknown. PATIENTS/METHODS We described new families with PK deficiency, retrieved clinical and laboratory information of cases systematically searched in the (gray) literature, and collected blood of these cases for complementary analyses. The Genome Aggregation Database (gnomAD) and the population-based Gutenberg Health Study served to study the prevalence …
Low-dose rivaroxaban and aspirin among patients with peripheral artery disease: a meta-analysis of the COMPASS and VOYAGER trials.
2021
Abstract Aims Peripheral artery disease (PAD) patients suffer a high risk of major cardiovascular (CV) events, with athero-thrombo-embolism as the underlying pathophysiologic mechanism. Recently, two large randomized clinical trials evaluated the efficacy and safety of low-dose rivaroxaban twice daily plus aspirin in stable PAD outpatients and those immediately after peripheral revascularization. We sought to determine if the effects of low-dose rivaroxaban and aspirin compared to aspirin alone are consistent across this broad spectrum of PAD patients. Methods and results We conducted a random-effects meta-analysis of the COMPASS and VOYAGER randomized trials among 11 560 PAD patients (4996…
Home treatment of patients with low-risk pulmonary embolism with the oral factor Xa inhibitor rivaroxaban: Rationale and design of the HoT-PE Trial
2016
SummaryPulmonary embolism (PE) is a potentially life-threatening acute cardiovascular syndrome. However, more than 95 % of patients are haemodynamically stable at presentation, and among them are patients at truly low risk who may qualify for immediate or early discharge. The Home Treatment of Pulmonary Embolism (HoT-PE) study is a prospective international multicentre single-arm phase 4 management (cohort) trial aiming to determine whether home treatment of acute lowrisk PE with the oral factor Xa inhibitor rivaroxaban is feasible, effective, and safe. Patients with confirmed PE, who have no right ventricular dysfunction or free floating thrombi in the right atrium or ventricle, are eligib…
Anti-Factor Xa levels correlate with recurrent venous thromboembolism and clinically relevant bleeding in patients receiving low-molecular-weight hep…
2019
Background: The aim of our study was to investigate whether anti-Xa levels assessed in selected groups of LMWH-treated patients with VTE correlate with the risk of developing thromboembolic or bleeding complications. Methods: Retrospective study, in adult patients treated with anti-Xa-adjusted LMWH analysing the association between anti-Xa activity and recurrent VTE or bleeding. Primary outcomes were symptomatic fatal or nonfatal objectively diagnosed VTE and clinically relevant bleeding (ISTH criteria). Results: From 2007-2017, 192 patients were recruited. Mean age was 62 (SD 18) years and 48% were men. Indications for anti-Xa monitoring were: VTE recurrence under anticoagulation (20.8%), …
Hereditary angioedema: an update on causes, manifestations and treatment.
2019
Hereditary angioedema is a rare genetic disorder caused by deficiency of C1 esterase inhibitor (C1-INH) and characterized by recurrent episodes of severe swelling that affect the limbs, face, intestinal tract and airway. Since laryngeal oedema can be life-threatening as a result of asphyxiation, correct diagnosis and management of hereditary angioedema is vital. Hereditary angioedema attacks are mediated by bradykinin, the production of which is regulated by C1-INH. Hereditary angioedema therapy relies on treatment of acute attacks, and short- and long-term prophylaxis. Acute treatment options include C1-INH concentrate, icatibant and ecallantide. Self-administration of treatment is recomm…
An Evaluation of Fibrin(ogen) Determinants of Thromboelastography
2011
Abstract Abstract 2251 Thromboelastography (TEG) was used in ∼200 studies published in 2009, is mainly used for global blood coagulation assessment, and is thrombin and fibrinogen concentration-dependent. Reflecting the time course of fibrin polymerization, TEG measures lag time, rate, and maximum amplitude (MA) of clot stiffness, and is substantially enhanced by platelets. To identify more specific fibrin(ogen) determinants potentially relevant to interpretation we investigated MA under different TEG conditions. The procedure utilizes a polymethylmethacrylate (hydrophobic surface) cup and pin set, the cup oscillating through 4° 45' (6 cycles/minute). The pin (sensor) is suspended via a tor…
Mutations in the Factor XII Gene in Solitary Cases of Recurrent Angioedema with Normal C1 Inhibitor Induced or Worsened by Oral Contraceptives or Hor…
2012
Missense Mutations in the Proline-Rich Region of Coagulation Factor XII in Hereditary and Idiopathic Angioedema.
2006
Abstract Hereditary angioedema (HAE) is characterized by recurrent skin swelling, abdominal pain attacks, and potentially life-threatening upper airway obstruction. The two classic HAE types (I and II) are caused by mutations in the complement C1 inhibitor gene, resulting in a functional deficiency of C1 inhibitor. Recently, a novel type of hereditary angioedema has been described, these patients showing normal C1 inhibitor concentration and activity in plasma (HAE with normal C1 inhibitor, HAE type III). With few exceptions, all reported patients have been women, and exposition to estrogens appears to be an important precipitating factor. Screening of twenty unrelated female patients with …
Hereditary angioneurotic oedema and blood-coagulation: interaction between C1-esterase-inhibitor and the activation factors of the proteolytic enzyme…
1983
C-1-inactivator (C-1-INA) does not only exert its important inhibitory functions in the complement system but also in the first step in the activation of the coagulation, fibrinolytic and kallikrein system. We therefore determined in nine patients with hereditary angioneurotic oedema (HANE) with obvious quantitative or functional defects of C-1-INA, and one further patient with Quincke-type oedema of different origin, the coagulation factors of the initial phase such as Hageman factor, plasma thromboplastin antecedent (PTA) and high molecular weight kininogen (HMWK). These factors were further correlated with the concentration as well as functional activity of C-1-INA. Nine of ten patients …