11/31. Protein S and congenital protein s deficiency: the most frequent congenital thrombophilia in Japanese.Protein S is a natural anticoagulant. Congenital protein S (PS) deficiency is a confirmed risk factor of venous thromboembolism (DVT) which though occurs infrequently yet is a leading cause of maternal mortality and morbidity. Congenital PS deficiency may also be responsible for obstetric complications such as preeclampsia/eclampsia, recurrent fetal loss and intrauterine fetal restriction. Congenital PS deficiency has been identified in 1-7.5% of patients with DVT and in 0.03-0.13% general Caucasian population. However, Japanese people have higher prevalence both in VTE patients (12.7%) and general population (0.48-0.63%). Because PS deficiency is the most frequent congenital thrombophilia in Japanese people, Japanese obstetricians must understand this thrombophilia and also that women with PS deficiency have an increased risk of VTE and a necessity of prophylactic use of anticoagulant against recurrent VTE during pregnancy and puerperium. This article reviews the literature to understand PS and congenital PS deficiency, especially the association of this thrombophilia with pregnancy.- - - - - - - - - - ranking = 1keywords = thromboembolism, embolism (Clic here for more details about this article) |
12/31. protein c deficiency and pregnancy: a case report.Protein C is thought to play a key role in the regulation of hemostasis, and its deficiency has been associated with an increased risk of thromboembolism. Protein C-deficient women are at particular risk of developing thromboembolic complications during pregnancy and delivery. The incidence of thromboembolic events is estimated to be 500-1000 times higher than in normal women. We report the case of a 26-year-old woman with previous iliofemoral deep vein thrombosis who experienced a successful pregnancy and delivery despite severe congenital protein c deficiency (protein C antigen and activity 25%). She was anticoagulated with heparin during the second part of her pregnancy. Our observation suggests that ambulatory full-dose subcutaneous heparin therapy during pregnancy constitutes adequate prevention. However, definite guidelines will require more extensive studies.- - - - - - - - - - ranking = 1keywords = thromboembolism, embolism (Clic here for more details about this article) |
13/31. Antinuclear antibody-negative systemic lupus erythematosus (SLE) and severe renal involvement: close correlation between disease activity and appearance of circulating anticoagulant.A 16-year-old girl meeting the criteria for SLE is described. Salient features of the clinical course included active glomerulonephritis with dense subepithelial deposits on electron microscopy, pulmonary embolism, axillary vein thrombosis, arthritis, serositis and fever. disease activity correlated with the presence of lupus anticoagulant as measured by VDRL and partial thromboplastin time (PTT). Her serum was consistently negative to ANA, anti-DS-dna, anti-SS-dna, ENA, anti-Ro, anti-La, and LE cells for the entire 4-year course. She responded remarkably to prednisone and azathioprine. Reappearance of VDRL and elevated PTT preceded exacerbation of disease activity and served as a serological guide for modifying medical treatment.- - - - - - - - - - ranking = 0.039500568278338keywords = pulmonary embolism, embolism (Clic here for more details about this article) |
14/31. Lupus anticoagulant and lacunar infarctions.Lupus anticoagulant is more often associated with thromboembolism than hemorrhage. We have observed two cases of lupus anticoagulant associated with basal ganglion lacunar infarction, causing contralateral choreoathetosis. One patient had no evidence of lupus or other etiology, and responded to antiplatelet therapy, while the other was found to have systemic lupus erythematosus with nephritis and was successfully treated with steroids. The effects of lupus anticoagulant on platelet prostacyclin receptors or prostacyclin production, or its effect on cerebral vessels may permit small-vessel occlusion and lacunar infarction in susceptible patients.- - - - - - - - - - ranking = 1keywords = thromboembolism, embolism (Clic here for more details about this article) |
15/31. Hereditary protein s deficiency and venous thrombo-embolism. A study in three Dutch families.Protein S, a vitamin k-dependent coagulation factor, is involved in the regulation of the anticoagulant activity of activated protein C. Using an immunoradiometric assay for total protein S in plasma we identified 14 patients (7 male and 7 female) in three unrelated Dutch families as fulfilling the criteria for an isolated protein s deficiency. In 9 patients who were not receiving oral anticoagulant treatment the mean total protein S antigen concentration was 0.50 /- 0.08 U/ml ( /- S.D.) and the calculated free protein S concentration was 0.15 /- 0.01 U/ml ( /- S.D.). In the five patients who were on oral anticoagulant treatment the mean total protein S antigen was 0.23 /- 0.05 U/ml ( /- S.D.). Seven of the 14 patients had a history of venous thromboembolism occurring at a mean age of 25 years and often without an apparent cause. Protein S deficiency is inherited as an autosomal dominant trait.- - - - - - - - - - ranking = 1.0593939160509keywords = thromboembolism, embolism (Clic here for more details about this article) |
16/31. Congenital deficiency of plasminogen and its relationship to venous thrombosis.In a patient with deep venous thrombosis, plasma concentrations of coagulant and inhibitor proteins were normal except for moderate deficiency of plasminogen. family studies revealed a similar deficiency in the mother and sister of the propositus. Evaluation of purified plasminogen demonstrated that it functioned normally. The patient represents our only example of plasminogen deficiency in 435 German individuals evaluated with a history of thromboembolism.- - - - - - - - - - ranking = 1keywords = thromboembolism, embolism (Clic here for more details about this article) |
17/31. Cerebral haemorrhagic infarction in young patients with hereditary protein c deficiency: evidence for "spontaneous" cerebral venous thrombosis.Among 53 patients with hereditary protein c deficiency belonging to 20 families three women were encountered who, aged 27, 34, and 38 respectively, had had cerebral haemorrhagic infarction, probably due to intracranial venous thrombosis. All three had also had venous thrombosis of the leg and pulmonary embolism either before or after their cerebral infarction. One patient sustained cerebral infarction while receiving an oral contraceptive, but infarction in the two others occurred "spontaneously." One patient also had an intraventricular and subarachnoid haemorrhage during the induction phase of coumarin treatment, which was assumed to have resulted from haemorrhagic infarction of the chorioid plexus, analogous to coumarin provoked haemorrhagic skin necrosis in protein c deficiency. Hereditary protein c deficiency should be considered in young patients with acute or subacute cerebral symptoms, especially if they have a family or personal history of venous thromboembolism.- - - - - - - - - - ranking = 1.0395005682783keywords = thromboembolism, pulmonary embolism, embolism (Clic here for more details about this article) |
18/31. Arterial thrombosis and embolism in malignancy.In most reviews of arterial embolism or thrombosis the source of emboli or the cause of thrombosis can reasonably be established in over 90% of patients. Still about 10% remain without demonstrable cardiac or intraarterial sources. Although hypercoagulability induced by malignancy has been alluded to as a cause of unexplained intravascular thrombosis reports of arterial thromboembolism with such association are rare. Seven patients with unequivocal thromboembolism are presented. Two distinct clinical patterns are observed, one with in situ thrombosis of small arteries and the other with occlusion of large arteries causing limb ischemia or fatal organ infarction. The various pathogenetic mechanisms of arterial thrombosis or embolism in malignancy include sustained spasm of arteries, precipitation of cryoglobulins or other abnormal proteins in small arteries, direct tumor invasion of arteries, fragmentation and embolization of intracardiac or intraarterial metastases and spontaneous arterial thrombosis due to hypercoagulability. The hypercoagulable state can be recognized by the observation of shortened bleeding and clotting times, partial thromboplastin and prothrombin times, elevation of coagulation factors, platelets and yield stress index and resistance to anticoagulation. patients presenting with arterial thromboembolic events with out demonstrable source should be investigated for malignancy. Conversely patients with malignancy should be searched for evidence of hypercoagulability in an attempt to prevent arterial thromboembolic complications.- - - - - - - - - - ranking = 2.0890908740764keywords = thromboembolism, embolism (Clic here for more details about this article) |
19/31. The lupus anticoagulant, pulmonary thromboembolism, and fatal pulmonary hypertension.A patient with a circulating lupus anticoagulant in the absence of systemic lupus erythematosus developed recurrent deep venous thromboses and pulmonary emboli. Pulmonary emboli recurred despite prolonged oral anticoagulant therapy and resulted in fatal pulmonary arterial hypertension. Extended anticoagulant therapy alone may not prevent recurrent thromboembolism in patients with a lupus anticoagulant. Pulmonary thromboembolism may be an important factor in the pathogenesis of pulmonary hypertension in patients with a lupus anticoagulant.- - - - - - - - - - ranking = 6keywords = thromboembolism, embolism (Clic here for more details about this article) |
20/31. Treatment with stanozolol of type I protein c deficiency in an Italian family.Functional and immunological assays specific for protein C were employed in the study of a family with congenital protein c deficiency associated with venous thromboembolism. By both assays, four members of the family belonging to two generations had half-normal PC levels. These findings, as well as the normal mobility of the protein in crossed immunoelectrophoresis, suggest that PC deficiency in this family is due to the decreased synthesis of a functionally normal protein. In one member of the family oral administration of the anabolic steroid stanozolol increased PC levels until normal values, suggesting that the defect can be overcome by pharmacological stimulation of protein synthesis.- - - - - - - - - - ranking = 1keywords = thromboembolism, embolism (Clic here for more details about this article) |
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