Can low level of von Willebrand factor decrease the risk of thrombosis in families with antithrombin or protein C deficiency?
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Biomedical subjects
Publications and source records attributed to M Palester-Chlebowczyk.
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We present the case of 37-year-old man with Sneddon's syndrome and antiphospholipid antibodies. His chief neurological manifestation was rapidly progressive dementia, which developed 6 years after the appearance of livedo reticularis.
Lupus anticoagulant and anticardiolipin antibodies belong to a heterogeneous family of antiphospholipid antibodies, directed probably against complexes composed of protein and negatively charged phospholipids. The antiphospholipid syndrome consists in the association of antiphospholipid antibodies and both venous and arterial occlusive events. The mechanism by which the antiphospholipid antibodies could induce thrombosis remains unclear.
Own material of 7 patients is presented pathology: venous thrombosis involving both hepatic veins and portal system, resulting in severe impairment of liver function and portal circulation, leading up to a fatal end (if not treated). Neither surgical decompression nor liver transplantation is feasible from the technical point of view. Patients were treated with activators of fibrinolysis. In four cases the treatment was effective and resulted in recanalisation of the previously occluded veins. Early re-thrombosis was the reason of death in one case. One patient died 4 years later because of cerebral vascular thrombosis. Two others live over 5 years, symptom-free. The authors consider thrombolytic treatment as a life saving procedure in cases of hepatic and portal venous thrombosis.
Basic data on pathomorphology and symptomatology of the alcohol-induced liver cirrhosis accompanied by portal hypertension are discussed. Respective data were compared with the group of cirrhotic patients not abusing alcohol. A high percentage of encephalopathic disorders and nearly 50% of the patients suffering from the hemorrhage from esophageal varices were the first sign of the cirrhosis in both groups. Despite hemorrhage from esophageal varices a few patients obtained surgical help preventing recurrence of the hemorrhage. Liver functional reserve, incidence of encephalopathies and the degree of liver involvement are in favour for non-alcohol cirrhosis. Inflammatory process in the liver, splenomegaly and hypersplenism were more frequent in the liver cirrhosis of non-alcohol origin.
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