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The synthetic pentasaccharide Arixtra or Fondaparinux is an indirect anti-Xa that effectively inhibits thrombin generation via its binding to anti-thrombin, the co-factor for Xa. A well-executed and designed clinical development of Fondaparinux in venous thrombosis prophyl ...

Preclinical and initial clinical experiences with the direct oral thrombin inhibitor ximelagatran demonstrated both a short and long-term potential utility for this agent in the prevention and treatment of various thromboembolic disorders associated with both arterial and v ...

The term pharmacogenetics, first introduced by Vogel in 1959 (1) is defined as the analysis of inherited factors that define an individual’s response to a drug, and generally refers to monogenetic variants that affect drug response. Pharmacogenetics refers to the monogenetic variants t ...

Thrombosis is still the leading cause of morbidity and mortality, and thus, effective antithrombotic strategies remain a critical therapeutic objective. The past decade has witnessed considerable progress in the development of newer anticoagulants, antiplatelets, and thro ...

Venous thromboembolism (VTE) is a term that includes both deep venous thrombosis (DVT) and pulmonary embolism (PE). Considerable progress has been made in the understanding of the risk factors for VTE. The clinical applications of molecular techniques have facilitated identificat ...

Despite recent major pharmacological and device advances, percutaneous coronary intervention (PCI) remains a costly procedure with significant periprocedural risk. Heparin has maintained the foundation of procedural anticoagulation, but heparin anticoagulation is ...

Intravascular thrombosis is one of the most frequent pathological events that affects mankind, and a major cause of morbidity and mortality in developed countries. There is abundant evidence suggesting that platelets play a pivotal role in the pathogenesis of arterial thrombotic dis ...

Low molecular weight heparins (LMWHs) are glycosaminoglycans (GAGs) of different chain length, molecular weight distribution, and different physiochemical characteristics that result from their diverse methods of preparation, which make them non-interchangeable. The ...

Despite the research and development efforts in newer anticoagulants, unfractionated heparin (UFH) and low molecular weight heparin (LMWH) will continue to play a pivotal role in the management of thrombotic disorders. Although bleeding and heparin-induced thrombocytopenia ...

Heparin remains the anticoagulant of choice in the therapy of thromboembolic events. Heparin is effective in the prevention and treatment of venous thromboembolism (VTE), as a surgical anticoagulant, and in interventional cardiology (1). Heparin is also used in the treatment of unstab ...

Heparin-induced thrombocytopenia (HIT) is associated with high morbidity and mortality. Because the pathophysiology of this complex disorder has remained unclear, so has the development of supportive diagnostic laboratory assays. The currently available laboratory met ...

Serine proteases play an important role in the process of thrombogenesis. In the coagulation network, various serine proteases are activated that facilitate the formation of factor Xa (1). The serine protease factor Xa has a central role in coagulation and platelet activation. Factor Xa is an ...

Tissue factor (TF) is a transmembrane glycoprotein that functions as a receptor and cofactor for activated factor VII (factor VIIa) to initiate blood coagulation. Although TF has been characterized best for its role in blood coagulation, recent studies have suggested a role for the molecule ...

The inherited bone marrow (BM) failure syndromes Fanconi anemia (1) and dyskeratosis congenita (2) are genetic disorders in which patients develop BM failure at a high frequency, usually in association with a number of somatic abnormalities. They are the best characterized and the most com ...

The hemoglobinopathies are a diverse group of inherited recessive disorders that include the thalassemias and sickle-cell disease. They were the first genetic diseases to be characterized at the molecular level and consequently have been used as a prototype for the development of new te ...

Diamond-Blackfan anemia (DBA) is a rare congenital pure red cell aplasia, with an incidence of 4–7 per million live births (1–5). Typically, affected children present in the second or third month of life with profound anemia, often in association with craniofacial (6) or thumb anomalies (2,7), and s ...

There are widely differing views about the acceptability of prenatal diagnosis and selective abortion for hemophilia both among affected families and healthcare professionals. This debate is beyond the scope of this chapter, but it is essential that the mechanistic approach outlin ...

Neonatal alloimmune thrombocytopenia (NAIT), or fetal maternal alloimmune thrombocytopenia (FMAIT), is a rare disease caused by maternal alloimmunization to inherited paternal human platelet antigens (HPA) expressed on fetal platelets (see Note 1). Maternal anti-HPA IgG ant ...

The congenital immunodeficiencies are a group of inherited conditions in which there are defects of immune function. The last decade has seen the identification of most of the genes defective in these disorders (1). Diagnostic genetic assays have been developed for many of these conditions ...

An extensive published literature now exists on the role of heritable thrombophilia in adult thromboembolic disease (1). Activated protein C resistance (APCR) secondary to the factor V (FV) Leiden mutation is a relatively common genetic defect, occurring in 2–15% of Caucasian populati ...