1/83. Recurring myocardial infarction in a 35 year old woman.A 35 year old woman presented with acute myocardial infarction without any of the usual risk factors: she had never smoked; she had normal blood pressure; she did not have diabetes; plasma concentrations of total cholesterol and high and low density lipoprotein cholesterol, fibrinogen, homocysteine, and Lp(a) lipoprotein were normal. She was not taking oral contraceptives or any other medication. coronary angiography showed occlusion of the left anterior descending coronary artery but no evidence of arteriosclerosis. Medical history disclosed a previous leg vein thrombosis with pulmonary embolism. Coagulation analysis revealed protein c deficiency. The recognition of protein c deficiency as a risk factor for myocardial infarction is important as anticoagulation prevents further thrombotic events, whereas inhibitors of platelet aggregation are ineffective.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
2/83. Long-term management of homozygous protein c deficiency: replacement therapy with subcutaneous purified protein C concentrate.We present the case of a full-term newborn in whom purpura fulminans developed shortly after birth. A diagnosis of homozygous protein c deficiency was established based upon undetectable plasma protein C activity and antigenemia in the newborn infant, and was later confirmed by protein C gene analysis. Specific replacement therapy with intravenous protein C concentrate was started 9 days after birth. This rapidly led to the complete regression of cutaneous lesions and consumption coagulopathy. After stabilization, oral anticoagulation was initiated in association with prophylactic treatment with intravenous protein C concentrate. However, oral anticoagulation was finally abandoned as the patient presented several thrombotic and hemorrhagic episodes clearly related to difficulties with anticoagulation. Due to the hazards related to prolonged venous access, we are currently using subcutaneous infusion of protein C concentrate for the long-term management of this condition, with satisfactory results.- - - - - - - - - - ranking = 3keywords = coagulation (Clic here for more details about this article) |
3/83. Ophthalmic manifestation of congenital protein c deficiency.Under normal conditions activated protein C is a natural anticoagulant that cleaves 2 activated coagulation factors, factor va and factor viiia, thereby inhibiting the conversion of factor X to factor xa and of prothrombin to thrombin. Additionally, activated protein C enhances tissue-plasminogen activator-mediated fibrinolysis by inhibition of plasminogen activator inhibitor-1. This results in an increase in circulatory plasminogen activator levels. protein c deficiency, a genetic or acquired thrombophilic abnormality, has been demonstrated to predispose to episodes of potentially blinding and lethal thromboembolic events. Heterozygous-deficient subjects usually remain asymptomatic until adolescence or adulthood. In homozygous-deficient patients, protein C activity is usually less than 1% (reference range, 70%-140%), resulting in thromboembolism as early as in the neonatal period. The major clinical symptoms in affected newborn infants have been purpura fulminans, vitreous hemorrhage, and central nervous system thrombosis. The age of onset of the first symptoms has ranged from a few hours to 2 weeks after birth, usually after an uncomplicated full-term pregnancy and delivery. In contrast to the genetic form, acquired neonatal protein c deficiency occurs particularly in ill preterm babies. Typical complications of prematurity such as respiratory distress syndrome, necrotizing enterocolitis, and neonatal sepsis may also be present. In the medical literature, there are only a few reports of homozygous protein c deficiency in neonates. We present 2 cases of homozygous protein c deficiency with ocular and extraocular manifestation.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
4/83. Acquired protein c deficiency following cisplatinum-navelbine administration for locally advanced breast cancer. Case report.Thromboembolic events have recently been reported following diverse regimens of chemotherapy for breast cancer. This is a report of a 39-year-old woman, a diagnosed case of locally advanced breast cancer, who received many regimens of chemotherapy. She presented with deep venous thrombosis 2 months after starting the cisplatinum-navelbine regimen. protein c deficiency was the only abnormal coagulation test that normalized after cessation of chemotherapy.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
5/83. Severe protein c deficiency and aseptic osteonecrosis of the hip joint: a case report.Homozygous congenital protein c deficiency is accompanied by severe thrombophilia. Thrombotic events can be reduced in number and severity by lifelong oral anticoagulation therapy. A 4-year-old boy was first diagnosed as having severe congenital homozygous protein c deficiency during the neonatal period when purpura fulminans occurred as the first manifestation of thrombosis. From this time he had been treated with phenprocoumon (INR 3.5-4.5). During an infection of the upper respiratory tract he developed signs of a new episode of purpura fulminans (INR 2.6). Additional anticoagulation therapy with LMW heparin (Clexane) and protein C concentrate was given while the oral anticoagulation therapy was continued. On the third day of this episode the boy suffered from pain of unknown origin. MRI of the abdomen and of the pelvis revealed nontraumatic osteonecrosis of the hip joint. After a few weeks of immobilisation no special treatment was necessary. One year later he was able to walk with no problem. CONCLUSION: Aseptic osteonecrosis of the hip joint is associated with a variety of disorders including vascular thrombosis and haemorrhage. Especially young children and handicapped patients with thrombophilia and pain of unknown origin are suspected to have thrombosis in atypical regions.- - - - - - - - - - ranking = 3keywords = coagulation (Clic here for more details about this article) |
6/83. Jejunal stricture complicating acute mesenteric venous thrombosis secondary to protein c deficiency and factor V Leiden gene mutation.We report a patient with high small intestinal obstruction due to ischemic stricture, secondary to acute mesenteric venous thrombosis. Tests done for hypercoagulability revealed dual etiology (protein c deficiency and factor V Leiden gene mutation). The patient underwent surgical resection and has been on anticoagulation treatment. At 2 years' follow up, she remains asymptomatic.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
7/83. A case of purpura fulminans is caused by homozygous delta8857 mutation (protein C-nagoya) and successfully treated with activated protein C concentrate.We report a Japanese patient who developed purpura fulminans and disseminated intravascular coagulation (DIC) shortly after birth. The patient was diagnosed to be homozygous for protein c deficiency and was treated with an activated protein C (APC) concentrate. Intravenous infusions of APC markedly improved the necrotic skin lesions and the anticoagulation by APC enabled successful DIC control. The identified mutation (Delta8857) results in impaired intracellular transport and protein maturation and would be the cause of the complete protein c deficiency. This is the seventh case of the mutation that has been exclusively reported in japan, but is the first report of a homozygous case. Our findings propose new therapeutic and diagnostic tools for the management of this fatal thrombotic disease.- - - - - - - - - - ranking = 2keywords = coagulation (Clic here for more details about this article) |
8/83. Successful anticoagulation with hirudin in a patient with mesenteric venous thrombosis and multiple coagulation abnormalities.A case of multiple thrombotic diatheses discovered in the setting of mesenteric venous infarction is discussed. The patient had deficiencies of protein C, protein s, antithrombin iii; was heterozygous for factor V Leiden; and had polycythemia vera. Adequate anticoagulation could not be established with heparin administration and hirudin was used. The diagnosis of mesenteric venous infarction, thrombotic tendency of multiple coagulation diatheses, and use of hirudin are discussed.- - - - - - - - - - ranking = 10keywords = coagulation (Clic here for more details about this article) |
9/83. superior sagittal sinus and cerebral cortical venous thrombosis caused by congenital protein c deficiency--case report.A 47-year-old male receiving anticoagulant therapy for thrombophlebitis in the left leg for several years presented with mild left hemiparesis and ipsilateral hypesthesia. The cause of the thrombophlebitis was still unknown. Magnetic resonance (MR) imaging showed subacute thrombosis of both the superior sagittal sinus (SSS) and a cortical vein in the right cerebral hemisphere. Fluid attenuation inversion recovery axial MR imaging demonstrated these lesions more obviously than conventional T2-weighted axial MR imaging. Right carotid angiography showed a partial SSS filling defect and occlusion of the cortical vein with collateral circulation. Coagulation studies revealed decreases in both protein C activity and antigen levels with normal levels of blood coagulation factors II, VII, IX, and X and protein s activity and antigen. The patient's mother had normal levels of both protein C activity and antigen, but his father had decreased levels. The diagnosis was SSS and cerebral cortical venous thrombosis caused by congenital protein c deficiency. The patient was treated conservatively, and his clinical course was uneventful. His neurological dysfunctions recovered within approximately 3 weeks after the onset. Ten months later, right carotid angiography showed recanalization of the SSS and partial filling of the cortical vein. Anticoagulant therapy has been continued, and no cerebral venous thrombosis has recurred during the 1.5 years after the onset.- - - - - - - - - - ranking = 44.690165452575keywords = blood coagulation, coagulation (Clic here for more details about this article) |
10/83. Multiple aortic thrombi associated with protein C and S deficiency.We describe a woman with an unusual case of thromboembolism of the mesenteric artery in whom multiple thrombi were subsequently found in the aorta and right heart chambers on transesophageal echocardiography. Further evaluation revealed a deficiency of protein C and S plasma proteins, inhibitors of the clotting system. The patient was treated successfully with systemic anticoagulation. Aortic thrombus is common in the setting of underlying atherosclerosis. However, the association of aortic thrombus with a deficiency of protein C and S is rare. To our knowledge, this is the first reported case of mural thrombus of the thoracic aorta associated with combined protein C and S deficiency. Our report underscores the important role of transesophageal echocardiography in the evaluation of patients with arterial thromboembolism.- - - - - - - - - - ranking = 1keywords = coagulation (Clic here for more details about this article) |
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