Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0149871 (deep vein thrombosis)
 12,364 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nine patients with, and 11 without, venous thromboses (DVT) from two families were studied. In family 1, four members with, and one without, DVT had t-PA activity below the lower limit of the controls (21.3 IU/ml, n = 19) after 20 min venous occlusion (VO). After VO t-PA antigen (t-PA:Ag) was below the lowest value of the controls (22.8 ng/ml) in all five cases with low t-PA activity. All the family members, both with and without thrombosis, had normal t-PA inhibitor activities (PAI). In family 2 t-PA activity after VO was low in three symptomatic and four asymptomatic family members. t-PA:Ag was also low in four of these. PAI level was normal in all but one family member. Mild type I von Willebrand's disease was discovered in four members of family 2. Deficient t-PA:Ag response was found in two of these. Antithrombin III, protein C and protein S were normal in both families. It is concluded that low fibrinolytic capacity, independent of PAI, is associated with familial DVT. Our data suggests autosomal dominant inheritance.
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PMID:Familial hypofibrinolysis and venous thrombosis. 249 18

Desmopressin acetate (1-deamino-8-D-arginine vasopressin [DDAVP]) improves hemostasis in hemophilia A and von Willebrand's disease and in some platelet disorders. In complex cardiac operations, excluding simple coronary artery bypass graft procedures, we found that desmopressin reduced blood loss by 40% and the need for transfusion by 34%. Conflicting reports followed. Future trials should emphasize patients with excessive bleeding. A possible post-desmopressin prothrombotic state was studied after hip replacement surgery. The incidence of deep vein thrombosis associated with warfarin sodium therapy was the same as that associated with desmopressin plus warfarin therapy. No desmopressin-induced thrombotic tendency was detected. A trend toward reduced blood loss with desmopressin was not significant. During cardiac catheterization, the plasma von Willebrand factor level was correlated with hemodynamic variables, including pulmonary vascular resistance, pulmonary arterial pressure, and (inversely) with cardiac index. von Willebrand factor concentration was highest in mitral stenosis. The relationship of these factors to the response to desmopressin remains to be defined.
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PMID:Adventures in hemostasis. Desmopressin in cardiac surgery. 843 Nov 22

The authors began to use 1-desamino-8-D-arginine vasopressin (DDAVP) Desmopressin Acetate routinely in their facelift patients who had a relatively high risk of bleeding, either from Von Willebrand factor deficiency, platelet dysfunction, aspirin intake, or ease of ecchymosis. Based on their observations that these patients had enhanced clotting and recovery times, they decided to undertake a prospective evaluation of the effects of Desmopressin Acetate when administered as a preoperative intravenous supplement during cosmetic facioplasty. A series of 200 consecutive patients undergoing surgery was divided into control and treatment groups. A total of 100 patients did not receive medication perioperatively, and 100 subsequent patients received preoperative DDAVP. All patients were observed for ecchymosis and swelling postoperatively and were graded by our postsurgical management team. Postsurgical ecchymosis was graded as mild (grade 1), moderate (grade 2), or severe (grade 3). Grade 4 indicated an expanding hematoma that required immediate surgical intervention. Grades 1 and 2 ecchymosis resolved within 3 weeks. Grade 3 ecchymosis or microhematomas required intervention such as needle aspiration or massage therapy. These small collections of blood generally required substantially longer to resolve and generated notable anxiety in the patients involved. Patients were excluded from the trial if there was a previous history of hypercoagulability or because treatment with DDAVP was medically contraindicated. A total of 23% of untreated patients required intervention for grade 3 ecchymosis compared with 3% of DDAVP-treated patients. No patients experienced any complications associated with DDAVP--namely, deep vein thrombosis, pulmonary embolus, electrolyte imbalance, or renal insufficiency. The authors undertook this study to determine whether DDAVP would help to decrease the incidence of microhematomas after facelift. Based on the results of their grading system and study, they think that the use of DDAVP is safe and efficacious in the prevention of troublesome microhematomas after facelift. Interestingly, although their male facelift patients challenged their efforts to obtain satisfactory hemostasis during and after surgery, the DDAVP-treated male patients responded with marked improvement in postoperative grading after treatment. Obtaining hemostasis intraoperatively was facilitated as well in these patients. This gender phenomenon was even more dramatic when compared with our female patient population.
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PMID:The use of DDAVP desmopressin reduces the incidence of microhematomas after facioplasty. 1135 16

All patients with von Willebrand's disease (vWD) who showed an arterial or venous thrombosis and were reported in the literature have been evaluated. 11 patients had arterial thrombosis while 19 had venous thrombosis for a total of 30 cases. 9 out the 11 cases with arterial thrombosis had myocardial infarction. Two had cerebral thrombosis. Associated risk factors for arterial thrombosis were available only for three patients who showed, respectively, smoking and dyslipidemia (2 cases) and smoking and intravenous desmopressin infusion (1 case). The majority of patients with venous thrombosis showed DVT with or without PE. Four patients presented with apparently isolated PE. In two instances thrombosis occurred in unusual sites (central retinal vein and portal vein, respectively). Several associated risk factors were present, mainly: infusion of FVIII or FVIII + vWF concentrates in 7 cases; surgery in 8 cases, pregnancy in 1, desmopressin infusion in 1, variable coagulation defects or polymorphisms in 5. More than one of these associated conditions were present in a few patients. The majority of vWD patients who showed thrombotic phenomena were type I patient, but in 6 cases were also type 3. The type of defect was not reported in 6 patients. As a conclusion of this review it seems safe to assume that both arterial and venous thrombosis appear rare in vWD. This is confirmed by the fact that arterial or venous thrombosis appears slightly more frequent in hemophilia A and B.
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PMID:Arterial and venous thrombosis in patients with von Willebrand's disease: a critical review of the literature. 1662 14

Several rodent models have been used to study deep venous thrombosis (DVT). However, a model that generates consistent venous thrombi in the presence of continuous blood flow, to evaluate therapeutic agents for DVT, is not available. Mice used in the present study were wild-type C57BL/6 (WT), plasminogen activator inhibitor-1 (PAI-1) knock out (KO) and Delta Cytoplasmic Tail (DCT). An electrolytic inferior vena cava (IVC) model (EIM) was used. A 25G stainless-steel needle, attached to a silver coated copper wire electrode (anode), was inserted into the exposed caudal IVC. Another electrode (cathode) was placed subcutaneously. A current of 250 muAmps over 15 minutes was applied. Ultrasound imaging was used to demonstrate the presence of IVC blood flow. Analyses included measurement of plasma soluble P-selectin (sP-Sel), thrombus weight (TW), vein wall morphometrics, P-selectin and Von Willebrand factor (vWF) staining, transmission electron microscopy (TEM), scanning electron microscopy (SEM); and the effect of enoxaparin on TW was evaluated. A current of 250 muAmps over 15 minutes consistently promoted thrombus formation in the IVC. Plasma sP-Sel was decreased in PAI-1 KO and increased in DCT vs. WT (WT/PAI-1: p=0.003, WT/DCT: p=0.0002). Endothelial activation was demonstrated by SEM, TEM, P-selectin and vWF immunohistochemistry and confirmed by inflammatory cell counts. Ultrasound imaging demonstrated thrombus formation in the presence of blood flow. Enoxaparin significantly reduced the thrombus size by 61% in this model. This EIM closely mimics clinical venous disease and can be used to study endothelial cell activation, leukocyte migration, thrombogenesis and therapeutic applications in the presence of blood flow.
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PMID:Thrombogenesis with continuous blood flow in the inferior vena cava. A novel mouse model. 2058 22

Children with inherited bleeding disorders often require central venous catheters (CVCs). Although CVCs are known to be complicated by deep venous thrombosis (DVT), little is known about the timeline of DVT development or risk of post-thrombotic syndrome (PTS). The aim of this study was to determine the timeline and confirm the incidence of thrombosis in patients with bleeding disorders who have CVCs. In 2002, we instituted a screening programme to monitor for CVC-related complications in children with haemophilia and von Willebrand disease. This is a retrospective review of this cohort. All children with CVC followed up between 1 January 2000 and 1 June 2009 were evaluated for DVT every 24 months with contrast venography and Doppler sonography. An institutional PTS severity scale was utilized at each visit. Thirty-six patients had 37 CVCs placed. Thirty patients had imaging studies, with DVT observed in 14 (47%). Most cases of DVT were diagnosed at the first venogram (median CVC duration 26 months). There were no abnormal ultrasound results. Sixteen patients (44%) had clinical findings consistent with PTS, including 10 (71%) with an abnormal venogram. Dilated chest wall veins appeared to be more strongly associated with underlying DVT (positive predictive value of 0.8) than arm circumference discrepancy. Successful transition to use of peripheral veins occurred at a median of 11 months after abnormal venograms. CVC-related DVT is common in children with inherited bleeding disorders and likely occurs earlier than previously thought. Clinical signs of PTS are also common, but long-term sequelae and severity of PTS are not known.
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PMID:Deep venous thrombosis screening in patients with inherited bleeding disorders and central venous catheters. 2143 17

Blood dose not normally coagulate in the blood vessels covered with endothelial cells, because these cells contain some substances responsible for antithrombotic action such as thrombomodulin, heparin-like substance, prostacyclin, nitric oxide and tissue plasminogen activator. Most important role of blood coagulation is hemostasis. Blood can coagulate in two ways: intrinsic coagulation pathway and extrinsic coagulation pathway that is activated by negatively charged substances and FVIIa-tissue-factor (TF) complex, respectively. Prothrombin time(PT) can represent extrinsic pathway, while activated partial thromboplastin time (APTT) can represent intrinsic pathway. PT is prolonged in such diseases as vitamin K deficiency, hepatic failure and warfarin intake, while APTT is prolonged such diseases as hemophilia A & B, von Willebrand disease and lupus anticoagulant. Cross mixing test is very useful to assess prolonged clotting time. FDP means fibrin/fibrinogen degradation products and D-dimer is the smallest products of fibrin degradation. These markers are often used to diagnose disseminated intravascular coagulation (DIC) and deep vein thrombosis (DVT). Thrombin-antithrombin complex (TAT) and plasmin-alpha2 plasmin inhibitor (PIC) can be used to evaluate the extent of coagulation and fibrinolysis activation, respectively. These two markers is essential for classify the pathophysiology of DIC: DIC with suppressed fibrinolysis, enhanced fibrinolysis or balanced fibrinolysis. In conclusion, exact interpretation of hemostatic and fibrinolytic markers is one of the most important abilities in clinical situation.
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PMID:[Interpretation of hemostatic and fibrinolytic markers]. 2218 80

Venous thromboembolism is common in the general population with increasing age as one of the most important risk factors. The care of hemophilia and von Willebrand disease has improved in recent decades, resulting in the expectation of a growing population of aging people with these disorders. Thrombosis seems rare in hemorrhagic disorders but studies documenting the true epidemiology are virtually lacking. Events have been reported, however, primarily catheter-related thrombophlebitis in hemophilia, but also deep vein thrombosis and pulmonary embolism have been described in von Willebrand disease, usually in conjunction with major surgery and prolonged replacement therapy with high factor levels. Thromboprophylaxis is likely not warranted in most cases. Instead, well-designed therapy and careful monitoring are important measures to prevent risk. In von Willebrand disease particularly, the variation of the phenotype and products used for replacement are challenges, as infusion of von Willebrand factor will increase the endogenous level of factor VIII. Long-term replacement therapy into old age is becoming more common in hemophilia but will not increase occurrence of thromboembolic disease, as factor levels still will be low and have a preventive effect.
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PMID:Thrombosis in patients with hemorrhagic disorders. 2351 93

The aim of this article was to investigate the prevalence of venous thrombosis in patients with von Willebrand disease. Personal records on 486 patients were reevaluated together with a time unlimited PubMed search. The venous thrombotic event had to be proven by objective means. Only cases of congenital von Willebrand disease were taken into consideration and all types of the diseases were included. No case of venous thrombosis was reported in our cohort of patients. On the contrary, 33 patients with proven venous thrombosis were gathered from the literature (17 cases of deep venous thrombosis with or without pulmonary embolism; isolated pulmonary embolism was seen in seven instances, superficial veins or portal system thrombosis was present in the remaining cases). Associated risk factors, mainly replacement therapy, were present in 26 cases. Therapeutic approach was usually based on heparin and Coumadin. Overall results were fair or good, as no fatalities occurred.
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PMID:Venous thrombosis in von Willebrand disease as observed in one centre and as reported in the literature. 2515 87

Pulmonary embolism is a complication of deep vein thrombosis. It occurs in the population with a normal clotting mechanism, but it may also occur in patients with congenital bleeding conditions. Here, we report on all cases of pulmonary embolism in congenital hemorrhagic disorders. All reported cases of pulmonary embolism in congenital coagulation disorders have been gathered by a time-unlimited PubMed search. Cross-checking of the references listed at the end of the single papers was carried out to avoid omissions. Seventy-two patients had an objectively demonstrated pulmonary embolism. The event occurred in patients with fibrinogen, factor V, factor VIII (FVII), FVIII, FIX, and FXI deficiency, and in those with von Willebrand's disease. No embolism was reported in FII, factor X, and FXIII deficiency. Thirty were women and 28 were men, whereas in the remaining 14 cases, sex was not reported. Age varied from 6 to 81 years (mean age 34.3 years). The management varied from only supportive to the administration of unfractionated heparin, low-molecular-weight heparin, and anti-vitamin K medications, accompanied by adequate replacement therapy. Evolution was fair or good in the majority of cases, but there were 10 fatalities. Risk factors were present in 61 patients. The most frequent of these were replacement therapy (35 cases), surgery (34), and old age (13). Some patients had more than one risk factor. Eleven patients had no risk factors. There are discrepancies in the prevalence of pulmonary embolism among different clotting disorders. The conditions most frequently affected are FVII deficiency and fibrinogen defects. The significance of the findings is discussed.
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PMID:Pulmonary embolism in congenital bleeding disorders: intriguing discrepancies among different clotting factors deficiencies. 2682 62


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