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PubMed2003

Screening for multiple hereditary hypercoagulability factors using the amplification refractory mutation system.

Many hereditary factors have been implicated in the development of arterial and/or venous thromboembolic diseases. A number of these risk factors can be identified by the amplification refractory mutation system (ARMS). However, the underlying technical conditions for performing ARMS are highly variable, and depend on which risk factors are being analyzed. We have now developed a novel ARMS-based system to simultaneously screen for multiple hypercoagulability factors under identical PCR conditions. This can greatly simplify the process of screening for hereditary hypercoagulability.

Base Sequence↗
PubMed1997

Coagulation abnormalities in type 1 Gaucher disease are due to low-grade activation and can be partly restored by enzyme supplementation therapy.

In type 1 Gaucher disease a bleeding tendency occurs which is partly caused by thrombocytopenia due to massive splenomegaly. In addition, low levels of factors IX and XI have been described. The mechanism responsible for these clotting factor abnormalities is unknown. We performed a detailed study of parameters of coagulation and fibrinolysis in 30 type 1 Gaucher disease patients (14 splenectomized) before and after treatment with enzyme supplementation therapy. Pre-treatment aPTT and PT were prolonged in 42% and 38% of patients, respectively. In 30-60% serious deficiencies (< 50%) of coagulation factors XI, XII, VII, X, V and II were observed. The low levels of factor V correlated with platelet count and were inversely associated with splenic volume. Levels of inhibitors were mildly decreased. Markers for activation of coagulation (thrombin-antithrombin (TAT) complex) and fibrinolysis (PAP complex, fibrin cleavage product D-dimer) were significantly elevated, especially in the splenectomized patients, indicating ongoing activation of these processes. After 12 months of enzyme supplementation therapy partial correction occurred. Thus, severe disorders of the coagulation system occur in Gaucher disease, contributing to the bleeding tendency. The deficiencies may be the result of consumption of coagulation factors caused by ongoing low-level coagulation activation. possibly due to mononuclear cell activation.

Adult↗
PubMed2004

Use of recombinant factor VIIa, Novo Seven, in the management of acute haemorrhage.

PURPOSE OF REVIEW: The purpose of this paper is to propose the use of factor VIIa in treating severely bleeding patients. RECENT FINDINGS: Recombinant factor VIIa was developed in 1988 to treat patients with haemophilia A or B and antibodies against factors VIII and IX, or patients with a spontaneous inhibitor against factors VIII and IX. Since then factor VIIa has been shown to be very effective in treating many other bleeding disorders, such as diverse thrombocytopathias, antibodies against other coagulation proteins like factor V, and bleeding as a result of oral anticoagulation. SUMMARY: Recombinant factor VIIa may be the universal haemostatic agent, but further studies are needed to prove this.

Journal Article↗
PubMed1975

"Car. factor" deficiency revisited.

Three members of a Virginia family with a bleeding disorder were found to have a serum defect in thromboplastin generation similar to the previously reported "Car. factor" deficiency. Sera from three members of the original Car. family did not correct the defect of affected members of the Virginia family. Partial thromboplastin times of Car. deficient individuals and affected members of the Virginia family were normal. Although correction with normal serum is attained in vitro, the serum defect persisted after infusion of fresh frozen plasma. Platelet function studies of the Virginia family revealed less than 30% aggregation after the addition of exogenous ADP and disaggregation within 2 min. Evaluation of children with Noonan's syndrome, albinism, and "Portsmouth" syndrome showed coexistent platelet aggregation defects and nonspecific serum defects.

Adenosine Diphosphate↗
PubMed1995

Hypercoagulability and the hypercoagulability syndromes.

Hypercoagulability is a state in which an alteration of the blood shifts the hemostatic balance toward excessive platelet/fibrin deposition leading to arterial and/or venous thrombosis [1]. Although the concept of hypercoagulability has been recognized clinically for more than a century, in recent years a number of specific disorders have been define, diagnostic tests developed, and treatment regimens improved. The currently recognized disorders are generally classified as primary or secondary states, although some of the primary conditions may develop as a result of other disorders (Table 1). The primary disorders generally result from abnormalities of proteins in the coagulation or fibrinolytic systems. Although the number of secondary conditions is much greater, generally these syndromes are not as precisely defined on a molecular basis. Secondary hypercoagulability syndromes are subclassified into abnormalities of platelets, coagulation and fibrinolysis, and blood vessels and rheology (Table 1). While the prevalence and clinical significance of hypercoagulability are becoming increasingly apparent to physicians in general, this pathologic state is an especially practical concern to vascular interventionalists, as hypercoagulability is an important cause of early thrombosis after otherwise uncomplicated arterial interventional procedures. The purpose of this review is to describe the clinical presentation, diagnosis, and treatment of the hypercoagulability syndromes most likely to be encountered by the interventional radiologist.

Animals↗