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  • 1
    Electronic Resource
    Electronic Resource
    New strategies in diagnosis and treatment of thrombotic thrombocytopenic purpura: case report and review (1999)
    Springer
    European journal of pediatrics 158 (1999), S. 883-887 
    Details
    ISSN: 1432-1076
    Keywords: Key words Chronic relapsing ; Thrombotic thrombocytopenic purpura ; Von Willebrand factor-cleaving protease ; Prophylactic treatment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The pentad of thrombocytopenia, haemolytic anaemia, mild renal dysfunction, neurological signs and fever, classically characterizes the syndrome of thrombotic thrombocytopenic purpura (TTP). TTP usually occurs in adults but also children have been described with this condition. The disorder may take a relapsing course, termed chronic relapsing TTP (CRTTP), which although very rare, may also begin in childhood. Deficiency of a recently identified enzyme, the von Willebrand factor (vWF)-cleaving protease, seems to play a major role in the development of TTP. We report on a 3-year-old boy with a dramatic but typical clinical course of CRTTP. At the time of diagnosis, neurological deficits and multiple cerebral infarctions had already occurred. In plasma, vWF-cleaving protease was completely absent, both during acute TTP and in remission. There was no protease inhibitor detected. Regular infusions of fresh frozen plasma were successfully given for replacement on a prophylactic basis. Conclusion Assay of von Willebrand factor-cleaving protease helps to diagnose a form of thrombotic thrombocytopenic purpura which may be managed by prophylactic treatment with fresh frozen plasma.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310051234
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Von Willebrand factor: molecular size and functional activity (1996)
    Springer
    Annals of hematology 72 (1996), S. 341-348 
    Details
    ISSN: 1432-0584
    Keywords: Key words von Willebrand factor ; von Willebrand disease ; Binding affinity ; Multimers ; Phenotype
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Von Willebrand factor (vWF) is the largest protein found in plasma. It circulates in blood as a series of multimers ranging in size from 500 to 20 000 kDa. The variable molecular weight of vWF is due to differences in the number of subunits comprising the protein. vWF mediates platelet adhesion to subendothelium of the damaged blood vessel. Only the largest multimers are hemostatically active. Each vWF subunit contains binding sites for collagen and for platelet glycoproteins GPIb and GPIIb/IIIa. Multiple interactions of repeating binding sites in vWF multimers with adhesive protein(s) of the subendothelium and with receptors on the platelet surface lead to "irreversible" binding of platelets to the exposed subendothelium. Functional properties of vWF are typical of multisubunit proteins encoded by autosomal loci. The phenotype of von Willebrand disease is determined by the properties of the dysfunctional subunits which become incorporated into heteropolymeric forms of vWF. Absence of large vWF multimers, seen in type 2A von Willebrand disease and in myeloproliferative disorders, is associated with bleeding tendency. On the other hand, in patients with vWF multimers of supranormal size, as they occur in thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), there is an increased risk of thrombosis. Proteolytic enzyme(s) are involved in physiologic regulation of the polymeric size of vWF. We have purified from human plasma a protease cleaving vWF at the same peptide bond that is also cleaved in vivo. vWF was quite resistant against the protease in a physiologic buffer but was degraded at low salt concentration or in the presence of 1 M urea. It appears that a conformational change in the vWF molecule exposes the specific protease-sensitive peptide bond and thus enhances degradation of vWF multimers. In some variants of type 2A vWF, the cleavage site in the vWF subunit is more susceptible to proteolytic degradation than in normal vWF, whereas in patients with TTP or HUS the protease activity may be suppressed. vWF-degrading protease plays an important role in pathogenesis of congenital or acquired disorders of hemostasis and thrombosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002770050184
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Subcutaneous low-molecular-weight heparin for treatment of Trousseau's syndrome (1997)
    Springer
    Annals of hematology 75 (1997), S. 165-167 
    Details
    ISSN: 1432-0584
    Keywords: Key words Low-molecular-weight ; Heparin ; Trousseau's syndrome ; Thromboembolism ; Neoplasms
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  We report the case of a 76-year-old man with recurrent thromboses despite oral anticoagulation with phenprocoumon and low-grade chronic disseminated intravascular coagulation. Workup revealed a bronchial carcinoma (NSCCL) with hilar and mediastinal lymph node metastases. The clinical condition was consistent with Trousseau's syndrome. Based on reports in the literature, the therapy was changed to intravenous unfractionated heparin (UFH), which was effective in controlling the thrombotic coagulopathy. For practical reasons, despite a lack of evidence of its effectiveness in Trousseau's syndrome, therapy with UFH was changed to subcutaneous low-molecular-weight heparin (LMWH, nadroparine) in therapeutic doses of 100 IU/kg body wt. 12 hourly. On an outpatient basis, five chemotherapy cycles were administered, and after metastases of the brain had been detected radiotherapy was initiated. Following 7.5 months of progressive neoplastic disease the patient died. He had remained free of thromboembolic complications under continued LMWH therapy during the last 6.5 months of his life. LMWH might be a convenient alternative to the established therapy with UFH in Trousseau's syndrome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002770050336
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    Paper (German National Licenses)
    Fulltext
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