China
Africa
Explore chapters and articles related to this topic
Liver Diseases
Published in George Feuer, Felix A. de la Iglesia, Molecular Biochemistry of Human Disease, 2020
George Feuer, Felix A. de la Iglesia
Effects on several organs are associated with chronic hepatic failure, such as the central nervous, cardiovascular, and hematopoietic systems. Neuropsychiatric abnormalities are the major manifestations of this condition. These central nervous system abnormalities include increased sensitivity to antidepressant drugs, hypoxia and acid-base disturbances, and changes in ammonia metabolism. Cardiovascular effects include increased cardiac output, tachycardia, and increased peripheral vasodilatation. Bleeding or ascites causes hypovolemic shock with associated renal failure. The hematologic abnormalities include excessive red blood cell destruction connected with increased spleen weight. Erythrocyte formation is impaired due to defects in vitamin B12 and folate metabolism. Impaired synthesis of clotting factors stimulates an increased bleeding tendency.
Acquired Bleeding Disorders Associated with Disease and Medications
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
William A. Rock, Sue D. Walker
Anistreplase (anisoylated plasminogen streptokinase activator complex, APSAC) is indicated in the management of acute myocardial infarction in adults, for the lysis of thrombi obstructing coronary arteries (134). The most common complication associated with Anistreplase therapy is bleeding, at any site. The overall incidence of bleeding in clinical trials was 14.6%. Critical location bleeding (intracranial, GI, etc.) should prompt immediate termination of any concomitant heparin, and consideration of protamine to reverse heparinization. If necessary, the bleeding tendency can be reversed with appropriate replacement therapy.
Hereditary Plasma Protein Disorders
Published in Genesio Murano, Rodger L. Bick, Basic Concepts of Hemostasis and Thrombosis, 2019
Factor XII deficiency was first described by Ratnoff and Colopy in 1955.41 This disorder is relatively rare; since the original description, only 160 to 180 cases have been described. Hageman deficiency is inherited as an autosomal recessive trait and usually is not associated with any bleeding diathesis. However, several patients with a mild bleeding tendency have been reported. Of particular significance, however, is the noting that an inordinately high number of patients with Hageman trait have died as a consequence of thrombosis (usually acute coronary artery thrombosis or acute pulmonary embolization).42 This may be related to the known role of Factor XII in activation of the fibrinolytic system (Chapter 3).
Testing strategies used in the diagnosis of rare inherited bleeding disorders
Published in Expert Review of Hematology, 2023
Patients with a high bleeding score but normal results of hemostatic testing may have collagen vascular bleeding disorder such as Ehlers-Danlos Syndrome (EDS). Major clinical manifestations of EDS variants include joint hypermobility, skin fragility, and hyperextensibility. The Beighton screening tool assesses for joint hypermobility (Table 3), seen in selected variants of EDS and assigns a score. This score provides a likelihood of joint hypermobility [12]; additional clinical findings in EDS variants with bruising as a prominent symptom are shown in Table 4. More detailed description of EDS variants is described in recent publications [13]. Although EDS variants were felt to be rare and are not included in the definition of RBD, a recent study estimated a prevalence of approximately 1 in 500 in the general population [14]. Follow-up testing for EDS, if indicated, consists of molecular genetic testing for genes implicated in each subtype of EDS listed in (Table 4). The modes of inheritance and genes implicated in the pathogenesis of each subtype vary. It should be noted that patients with EDS may also have co-existing hemostatic abnormalities [15]. Most of these variants have a relatively benign clinical course except for vascular type (formerly type IV), which has a propensity for life-threatening complications, such as arterial rupture. Finally, despite exhaustive testing, a defined group of patients with an evident bleeding tendency, but no diagnostic laboratory findings are categorized as bleeding disorders of unknown cause [16].
Pharmacotherapy for stroke prevention in nonvalvular atrial fibrillation: current strategies and future directions
Published in Expert Opinion on Pharmacotherapy, 2022
Antonio Gómez-Outes, M Luisa Suárez-Gea, Alejandro-Isidoro Pérez-Cabeza, Jose Manuel García-Pinilla
A number of oral (asundexian, milvexian) and parenteral (abelacimab, osocimab, xisomab, IONIS-FXIRX and fesomersen) factor XIa inhibitors are being developed for prevention of thromboembolism. Inhibition of factor XIa of the intrinsic (contact activation) pathway is believed to produce an antithrombotic effect without a significant increase in major bleedings. New agents with a low bleeding tendency may be potentially useful in patients at high risk of bleeding (i.e.: HAS-BLED ≥3), which comprises nearly half of the population with AF in real world [98]. Oral agents have the potential to be an alternative to DOAC in these patients at high bleeding risk, but more data from ongoing studies are needed to establish their place in therapeutics. In addition, weekly or monthly subcutaneous injections of ASOs (IONIS-FXIRX and fesomersen) or MABs (e.g. abelacimab, xisomab) might help to improve patient adherence to long-term anticoagulation.
First case of delayed traumatic intracerebral hemorrhage in a patient with undiagnosed factor XI deficiency: diagnosis and management review
Published in Brain Injury, 2020
Eduardo E. Espinosa-Rodríguez, María López-Gutiérrez, Gloria Tresserras-Giné, Nuria Pesa-Vendrell, Melcior Martínez-Pérez
Some possible causes for the variation in severity of bleeding tendency are described, such as inheritance of associated hemostatic defect (e.g., von Willebrand disease, platelet function defect), group O blood type, drug ingestion, reduced activation of thrombin activable fibrinolysis inhibitor resulting in clot susceptibility to fibrinolysis or the homozygote or heterozygote trait of the deficit. A hypothesis as to why most patients do not present spontaneous bleeding states that platelets contain a molecule with a high molecular weight on its membrane, which possibly consists of a tetramer of four identical subunits, functionally and immunologically similar to plasma FXI; thus, it has been described that under basal conditions, platelet FXI levels are normal and are able to compensate for the plasmatic FXI deficiency; therefore, bleeding occurs only when the platelet FXI levels are stressed, as in the context of post-traumatic or post-operative conditions (8,23).