1/13. 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 (Clic here for more details about this article) |
2/13. 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 (Clic here for more details about this article) |
3/13. 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 = 0.5keywords = thromboembolism (Clic here for more details about this article) |
4/13. 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 (Clic here for more details about this article) |
5/13. 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 (Clic here for more details about this article) |
6/13. 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 = 0.5keywords = thromboembolism (Clic here for more details about this article) |
7/13. Failure of thromboprophylaxis in pregnancy caused by dual deficiencies of protein S and protein c.Protein S and protein c deficiencies are commonly identified biochemical abnormalities associated with a hypercoagulable state; they result in an increased incidence of venous thromboembolism. We report the case of a pregnant woman with dual deficiencies of protein S and protein c, who encountered a venous thromboembolism 7 days postpartum despite of the use of danaparoid.- - - - - - - - - - ranking = 1keywords = thromboembolism (Clic here for more details about this article) |
8/13. Identification of a novel PROS1 c.1113T-->GG frameshift mutation in a family with mixed type I/type III protein s deficiency.We report a family with type I and type III protein S (PS) deficiency, which showed to be phenotypic variants of the same genetic disease. Direct sequencing analysis of the PROS1 gene was performed to establish the genotype. The ratio of protein c antigen and total PS antigen levels (protein c/S ratio) was used to classify subjects at risk of venous thromboembolism. All PS deficient subjects had increased protein c/S ratios as well as a novel PROS1 c.1113T-->GG frameshift mutation.- - - - - - - - - - ranking = 0.5keywords = thromboembolism (Clic here for more details about this article) |
9/13. Mesenteric vein thrombosis as presenting manifestation of hereditary protein s deficiency.protein s deficiency is inherited as an autosomal dominant trait. Heterozygotes with a reduction of 50% in the plasma protein S concentration are at risk for the development of venous thromboembolism, often occurring at an early age without an apparent cause. In the majority of the patients thrombosis is restricted to the superficial or deep venous system of the legs. In this case report we describe the presence of mesenteric vein thrombosis in a 30-yr-old man with hereditary protein s deficiency. In his family protein s deficiency was also recognized in his mother, brother, and niece. Both his mother and brother had a history of thrombotic disease.- - - - - - - - - - ranking = 0.5keywords = thromboembolism (Clic here for more details about this article) |
10/13. Caesarean section in a patient with protein s deficiency.Protein S is a nonenzymatic and vitamin k-dependent cofactor of activated protein c. Without protein S, the anticoagulant function of protein c is almost depleted and thrombotic events occur. We report a parturient with hereditary protein s deficiency in whom the risk of thromboembolism was further complicated by pregnancy and who required emergency Caesarean section for fetal distress.- - - - - - - - - - ranking = 0.5keywords = thromboembolism (Clic here for more details about this article) |
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