1/28. 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 = thromboembolism, embolism (Clic here for more details about this article) |
2/28. warfarin-induced skin necrosis associated with factor v Leiden and protein s deficiency.Thrombotic events are rare complications during anticoagulation therapy. The thrombosis varies from localized cutaneous involvement to catastrophic thromboembolism and is usually associated with an underlying thrombophilia. We describe a patient who developed skin necrosis during warfarin treatment for a pulmonary thromboembolism. The management was complicated by the development of heparin-induced thrombocytopenia and further thrombotic events. thrombophilia screen demonstrated the presence of protein s deficiency and factor v Leiden as the prothrombotic factors, together with the demonstration of antiplatelet factor 4 antibodies, which confirms the diagnosis of heparin-induced thrombocytopenia (type II). Reinstitution of warfarin at a low loading dose was successful without the recurrence of skin lesions nor any further thrombosis.- - - - - - - - - - ranking = 1keywords = thromboembolism, embolism (Clic here for more details about this article) |
3/28. Recurrent pulmonary embolism in a 13-year-old male homozygous for the prothrombin G20210A mutation combined with protein s deficiency and increased lipoprotein (a).We report the case of a 13-year-old male presenting with recurrent symptoms of respiratory distress after a trauma of the lower limb. Pulmonary symptoms had been misinterpreted for several weeks and only marked symptoms of deep venous (DVT) and caval vein thrombosis later prompted the correct diagnosis of DVT and embolic events and subsequently a successful thrombolytic therapy. The case reported here shows a diagnostic pitfall of pulmonary embolism in an adolescent. It emphasizes the need to consider the possibility of thromboembolic events also in young children and adolescents presenting with atypical pulmonary symptoms and suffering from pulmonary diseases not responding to antibiotic therapy. In addition, although the homozygous PT A20210A gene mutation is a rare defect and its relevance as a risk factor on its own remains to be elucidated, this case suggests that a complete thrombophilia laboratory workup should be performed in young patients with a first symptomatic thromboembolic onset.- - - - - - - - - - ranking = 1.4090239099916keywords = pulmonary embolism, embolism (Clic here for more details about this article) |
4/28. Cutaneous necrosis revealing the coexistence of an antiphospholipid syndrome with acquired protein s deficiency, factor v Leiden and hyperhomocysteinemia.We report an exceptional case of cutaneous necrosis due to the coexistence of 4 thrombophilic factors, inherited and acquired. We would like to draw attention to these unrecognized associations. CASE REPORT: A 72-year-old woman was admitted with a 5 month history of necrotic nonhealing, painful ulcer of both legs and recently a purple toe. She had a history of 3 deep venous thromboses of the leg complicated by pulmonary embolism. A skin biopsy of the ulcer and purple toe showed only thrombosis in the dermal vessel. Laboratory findings showed a circulating lupus anticoagulant, positive anticardiolipin antibodies, antinuclear antibodies (1/320 dilution) and an anti Sm. Moreover, activated protein c resistance associated with factor v Leiden mutation and hyperhomocysteinemia was found; protein S was transiently low. With iloprost, oral anticoagulant, vitamin B12 and folic acid, the evolution was good, with healing of ulcer. COMMENTS: cutaneous necrosis can reveal hypercoagulable states, sometimes complex. We find 4 thrombophilic factors in our case, i.e. antiphospholipid antibodies, factor v Leiden, protein s deficiency and hyperhomocysteinemia. This is exceptional but highlights the role of several constitutional and acquired thrombophilic factors in the genesis of thrombosis. Extended protein C pathway disturbances could explain the mechanism that leads to cutaneous necrosis, in this patient, with an antiphospholipid syndrome. This case shows that it is necessary in some circumstances to make a complete hemostatic laboratory search to detect several thrombophilic factors. If they are present they can justify an oral anticoagulant treatment and a familial screening.- - - - - - - - - - ranking = 0.28180478199833keywords = pulmonary embolism, embolism (Clic here for more details about this article) |
5/28. Protected iliofemoral venous thrombectomy in a pregnant woman with pulmonary embolism and ischemic venous thrombosis.Although thromboembolism is uncommon during pregnancy and the postpartum period, physicians should be alert to the possibility because the complications, such as pulmonary embolism, are often life threatening. pregnant women who present with thromboembolic occlusion are particularly difficult to treat because thrombolysis is hazardous to the fetus and surgical intervention by any of several approaches is controversial. A 22-year-old woman, in her 11th week of gestation, experienced an episode of pulmonary embolism and severe ischemic venous thrombosis of the left lower extremity The cause was determined to be a severe protein s deficiency in combination with compression of the left iliac vein by the enlarged uterus. The patient underwent emergency insertion of a retrievable vena cava filter and surgical iliofemoral venous thrombectomy with concomitant creation of a temporary femoral arteriovenous fistula. The inferior vena cava filter was inserted before the venous thrombectomy to prevent pulmonary embolism from clots dislodged during thrombectomy When the filter was removed, medium-sized clots were found trapped in its coils, indicating the effectiveness of this approach. The operation resolved the severe ischemic venous thrombosis of the left leg, and the patency of the iliac vein was maintained throughout the pregnancy without embolic recurrence. At full term, the woman spontaneously delivered an 8-lb, 6-oz, healthy male infant.- - - - - - - - - - ranking = 2.4726334739883keywords = thromboembolism, pulmonary embolism, embolism (Clic here for more details about this article) |
6/28. Combined protein C and protein s deficiency in a family with repetitive thromboembolism and segregated gene mutations.A young man with repetitive deep venous thrombosis of the legs and the inferior vena cava, and his family were eventually diagnosed by means of molecular genetic analysis as having both hereditary protein C and protein s deficiency. There have been a few reports of families with combined protein C and protein s deficiency and only one report of such a family characterized at the dna level. This was the first reported family in japan with combined deficiency of protein C and protein S accompanied by segregation of gene lesions.- - - - - - - - - - ranking = 2keywords = thromboembolism, embolism (Clic here for more details about this article) |
7/28. Deficiency of protein S-mediated familial venous thrombophilia--a case report.Deficiency of protein S causes potential problems of thrombosis. Cases of familial venous thrombosis due to deficiency of protein S were presented. First, an 85-year-old woman had pulmonary thromboembolism due to left deep femoral venous thrombosis, which might be triggered by leg fracture and the long-term treatment with a plaster cast. Next, her 29-year-old granddaughter had episodes of recurrent venous thrombosis in her legs and arms, which might be triggered by the treatment with a plaster cast and abortion. In the latter part, the aspects of risks for thromboembolism, potential problems in gestational period, and an advisability of thromboprophylaxis in patients with deficiency of protein S are described.- - - - - - - - - - ranking = 1keywords = thromboembolism, embolism (Clic here for more details about this article) |
8/28. Intra-cardial thrombosis with systemic and pulmonary embolism as main symptoms in a patient with protein s deficiency.This report describes the unusual occurrence of both left and right atrial thrombosis with peripheral arterial and pulmonary embolism, respectively, as presenting symptoms of congenital protein s deficiency in a 31-year-old man. The coagulation study performed in the coumarin-treated propositus indicated a heterozygous protein S state. The finding of reduced free protein S antigen and protein S activity levels with normal total protein S and C4B-bp levels in five other family members (father, sister, and three relatives on the paternal side) confirmed the inherited nature of the defect. Since there is an increased frequency of arterial thrombosis in patients suffering from protein s deficiency, any case of idiopathic intra-cardial thrombosis requires careful haemostatic screening. In addition, the possibility of intra-cardial thrombosis should be considered in any thromboembolic event seen in inherited protein s deficiency.- - - - - - - - - - ranking = 1.4090239099916keywords = pulmonary embolism, embolism (Clic here for more details about this article) |
9/28. warfarin skin necrosis associated with protein s deficiency and a mutation in the methylenetetrahydrofolate reductase gene.The use of warfarin is rarely complicated by skin necrosis. We describe a 50-year-old woman who presented with a left leg deep venous thrombosis and subsequent pulmonary embolism. She was initially anticoagulated with low-molecular weight heparin and subsequently warfarin. Within 4 days abdominal skin necrosis developed. Investigations revealed the presence of protein s deficiency and in addition, a mutation in the methylenetetrahydrofolate reductase gene (MTHFR). We present, to our best knowledge, the first case of warfarin skin necrosis associated with a methylenetetrahydrofolate reductase mutation.- - - - - - - - - - ranking = 0.28180478199833keywords = pulmonary embolism, embolism (Clic here for more details about this article) |
10/28. Successful treatment of massive pulmonary embolism in the 38th week of pregnancy.In 1998, a 32-year-old woman in week 38 of pregnancy was referred to our hospital because of swelling of the lower extremities and dyspnea at rest. A massive pulmonary embolism and thrombi in the right and left atria were detected. After a successful cesarean section, emergent pulmonary embolectomy was performed with the use of cardiopulmonary bypass. Both the mother and the fetus recovered without severe complications. Postoperatively, activated protein s deficiency was detected.- - - - - - - - - - ranking = 1.4090239099916keywords = pulmonary embolism, embolism (Clic here for more details about this article) |
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