Thrombosis research
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Thrombosis research · Jan 2006
ReviewAdvances in the pathogenesis, diagnosis and treatment of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.
The thrombotic microangiopathies are microvascular occlusive disorders characterized by hemolytic anemia caused by fragmentation of erythrocytes and thrombocytopenia due to increased platelet aggregation and thrombus formation, eventually leading to disturbed microcirculation with reduced organ perfusion. Depending on whether brain or renal lesions prevail, two different entities have been described: thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). ⋯ Recent studies have contributed greatly to our current understanding of the molecular mechanisms leading to TTP and HUS. In this review, we briefly focus on the most important advances in the pathophysiology, diagnosis and treatment of these two thrombotic microangiopathies.
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The use of oral anticoagulant therapy is increasing in children. Managing anticoagulant therapy in children presents unique challenges, including poor venous access. The advent of point-of-care (POC) monitoring of anticoagulant therapy offers a potential solution to this challenge. This paper reviews the published literature relating to POC monitoring of oral anticoagulant therapy in children. ⋯ POC monitoring of oral anticoagulant therapy in children offers considerable advantages. The reviewed literature would suggest such monitoring can be performed accurately and reliably. The impact of quality control issues, such as calibration of thromboplastin ISI in POC devices, has not been explored in a paediatric population. Further studies are needed to clarify such issues and confirm the safety, reliability and efficacy of POC monitoring of oral anticoagulant therapy in children, including its home monitoring and self-management programs.
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Thrombosis research · Jan 2006
Accuracy of coding for possible warfarin complications in hospital discharge abstracts.
Hospital discharge abstracts could be used to identify complications of warfarin if coding for bleeding and thromboembolic events are accurate. ⋯ In our centre, the discharge abstract could be used to identify and exclude patients hospitalized with a major bleed or thromboembolism. If coding quality for bleeding is similar in other hospitals, these ICD-9-CM diagnostic codes could be used to study population-based warfarin-associated hemorrhagic complications using administrative databases.
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Thrombosis research · Jan 2006
Fibrinogen Guarenas, an abnormal fibrinogen with an Aalpha-chain truncation due to a nonsense mutation at Aalpha 467 Glu (GAA)-->stop (TAA).
Fibrinogen Guarenas is a dysfibrinogenemia with a nonsense mutation at G4731T that causes an Aalpha-chain truncation at Ser 466. This abnormal fibrinogen is associated with a bleeding diathesis, severe in the proposita and mild in one brother, even though the fibrinogen levels in plasma are normal. All other family members are asymptomatic. ⋯ When Guarenas clots were perfused with fibrinolytic enzymes, clot degradation was retarded. Clot structure studied by confocal 3D microscopy showed that the fibrin network was dense, made up of thin and highly branched fibers, which accounted for the decreased flow rates by buffer permeation and increased rigidity of the fibrin clots, measured using a torsion pendulum. It seems that the increased clot rigidity, decreased porosity, hypofibrinolysis and t-PA induced fibrinolysis, by itself are not necessarily associated with thrombotic disorders in dysfibrinogenemia.