Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: EC:3.4.21.6 (thromboplastin)
13,278 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a 69-year-old woman with bilateral adrenal hemorrhage complicated with antiphospholipid syndrome (APS). She was hospitalized with nausea and vomiting in September 2003. Laboratory data demonstrated hyponatremia, hypoglycemia and prolongation of activated partial thromboplastin time (aPTT). Abdominal computed tomography showed bilateral adrenal enlargement. In October 2003, she demonstrated altered mental status with progressive hyponatremia, a high level of ACTH, and a low level of serum cortisol. She also showed thrombocytopenia, anti-cardiolipin IgG antibody, anti-beta2GPI antibody, and lupus anticoagulants. After four months, anti-cardiolipin IgG antibody was still positive. Based on these findings, she was diagnosed as having APS complicated with adrenal insufficiency due to hemorrhagic infarction. After treatment with corticosteroid, a low dose of aspirin and normal saline infusion, her condition quickly improved. Platelet counts and aPTT were also normalized. To our knowledge, this is the second Japanese case of APS complicated with bilateral adrenal hemorrhage. APS should be considered an important underlying cause of adrenal insufficiency.
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PMID:Adrenal insufficiency complicated with antiphospholipid syndrome (APS). 1697 59

A 49-year-old Caucasian man with antiphospholipid syndrome who experienced an ischemic stroke required multidisciplinary decisions regarding acute and long-term care. The patient first received warfarin and unfractionated heparin, followed by low-molecular-weight heparin. However, he developed complications from these drugs (warfarin-induced necrosis and heparin-induced thrombocytopenia), resulting in thigh necrosis and multiple additional cerebral and peripheral infarcts. His condition improved after warfarin and the heparins were discontinued, and a direct thrombin inhibitor, argatroban, was given intravenously for acute treatment. Argatroban is the only anticoagulant known to be safe in patients who experience an acute ischemic stroke in the setting of heparin-induced thrombocytopenia. For long-term anticoagulation, fondaparinux, an indirect, selective factor Xa inhibitor, was given subcutaneously. The patient received intravenous dexamethasone, later changed to azathioprine, for immunomodulatory treatment. He had significant improvement in his neurologic deficits without recurrent events over the next 18 months. Management of anticoagulation therapy in patients with antiphospholipid syndrome is complex and challenging, and therapeutic strategies need to be evaluated further.
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PMID:Anticoagulation strategies for treatment of ischemic stroke and antiphospholipid syndrome: case report and review of the literature. 1699 62

Lupus anticoagulants are a heterogeneous group of autoantibodies detected by their effects on phospholipid-dependent coagulation assays. Persistent lupus anticoagulants are associated with thrombotic disease, but not all are clinically significant. Antibody heterogeneity and reagent and test variability dictate that at least 2 tests, of different types, should be used to screen lupus anticoagulants. The objective of this study was to investigate whether the activated seven lupus anticoagulant assay detects clinically significant antibodies. Eighty-two patients with antiphospholipid syndrome (APS) and 32 with systemic lupus erythematosus + positive for activated seven lupus anticoagulant and who were without thrombosis, who were positive by activated seven lupus anticoagulant assay, were investigated for lupus anticoagulants by dilute Russell's viper venom time, dilute activated partial thromboplastin time, and Taipan snake venom time, and for anticardiolipin antibodies. Fifty-seven of the APS patients were positive for lupus anticoagulants in multiple assays, 25 in activated seven lupus anticoagulant alone. Fourteen of the latter group were previously positive in other antiphospholipid antibodies assays, and 11 had only been positive for lupus anticoagulants by activated seven lupus anticoagulant. Twenty-eight had elevated anticardiolipin antibodies, 6 of whom were from the group that was positive in activated seven lupus anticoagulant only. Eight of the systemic lupus erythematosus + lupus anticoagulants (without thrombosis) patients were positive for lupus anticoagulant by activated seven lupus anticoagulant alone and had only been positive in activated seven lupus anticoagulant previously, and none had elevated anticardiolipin antibodies. The remaining 24 patients were lupus-anticoagulant positive in multiple assays, and 9 had elevated anticardiolipin antibodies. Dilute Russell's viper venom time and Dilute activated partial thromboplastin time are widely used to detect lupus anticoagulants and are considered to detect clinically significant antibodies. Activated seven lupus anticoagulant detected antibodies in APS patients who were positive by these assays and also lupus anticoagulants undetectable by the dilute Russell's viper venom time/dilute activated partial thromboplastin time reagents used, demonstrating its utility as a first-line or second-line assay.
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PMID:The activated seven lupus anticoagulant assay detects clinically significant antibodies. 1789 8

The antiphospholipid syndrome (APLS) is a complex autoimmune disease often connected to systemic lupus erythematodes. Main features are thromboses, fetal loss and specific antibodies. The involved autoantibodies are directed against plasma proteins such as beta2glycoprotein1 (beta2GPI) or prothrombin which depend on negatively charged phospholipids. Direct antibodies against phospholipids are of no importance for APLS. Clotting tests such as activated partial thromboplastin time or diluted Russell's viper venom test (dRVVT) can show a prolonged time for coagulation despite a prothrombotic state in vivo but the investigator needs awareness about disturbing phospholipid sources and other influential factors. Enzyme linked immuno sorbent assay tests for antibodies against cardiolipin, beta2GPI and prothrombin are valuable solid phase tests with different specificity. Antiphospholipid, anticardiolipin or lupus anticoagulant are misnomers in connection with APLS. They are preserved as a reminiscence of the pioneering work on the way to the still not exactly revealed basics of APLS. Valve operations in APLS patients seem to be rare; a meta-analysis of 57 cases proves that the perioperative management is, at the moment, an empirical approach with high morbidity and mortality in these young patients.
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PMID:The antiphospholipid syndrome and heart valve surgery. 1808 13

The aim of this study was to evaluate the efficacy of prophylaxis using low-dose non-fractioned heparin and aspirin in the prevention of intrauterine growth restriction and low birth weights in patients suffering from antiphospholipid antibody syndrome. Intrauterine growth retardation and birth weights of 34 gestations involving 28 women with histories of multiple miscarriages and elevated antiphospholipid antibody levels were evaluated in a prospective study in the period from April 1988 to July 2004. A control group was formed of 39 women without previous history of miscarriages over a total of 40 gestations. Intrauterine growth retardation was considered when the weight of the newborn baby was below the tenth percentile for gestational age according to the fetal weight chart. Diagnosis of antiphospholipid antibodies was achieved using the ELIZA test to measure the IgG and IgM immunoglobulin levels. Evaluation of lupus anticoagulant was performed using the activated partial thromboplastin time (aPTT). Women suffering from antiphospholipid antibodies underwent prophylactic treatment during gestation with low doses of acetylsalicylic acid (100 mg daily) associated to low doses of subcutaneous heparin (5000 IU twice daily). The non-paired Student t-test, Fisher Exact and Mann-Whitney tests were used for statistical analysis with an a error of up to 5% considered acceptable. A statistically higher number of newborns suffered intrauterine growth retardation and low birth weights in the study group than in the control group. In conclusion, children of mothers suffering from antiphospholipid antibody syndrome, even those undergoing prophylactic treatment with low-dose non-fractioned heparin and aspirin, are associated to intrauterine growth retardation and low birth weights.
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PMID:Antiphospholipid antibodies and growth retardation in intrauterine development. 1822 45

Comprehensive clinical laboratory studies were performed in 38 children with systemic lupus erythematosus. Screening tests (kaolin time, thromboplastin time with diluted thromboplastin, lebetox time) used which allowed secondary antiphospholipid syndrome to be diagnosed in 7 patients. IgG phosphatidylserine and cardiolipin antibodies were most frequently detected in the patients. Analysis of the findings has established the high sensitivity, specificity, and diagnostic value of the lebetox-echitox index, which permits this method to be recommended for the diagnosis of secondary antiphospholipid syndrome in children.
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PMID:[Diagnosis of secondary antiphospholipid syndrome in children by means of screening tests]. 1835 17

Protein Z (PZ) is a vitamin K-dependent factor identified in human plasma in 1984 characterized by an homology with other vitamin K-dependent factors (factor VII, IX, X, protein C). In contrast to these factors, PZ does not possess any enzymatic activity but is involved as a cofactor in the down-regulation of coagulation by forming a complex with the protein Z-dependent protease inhibitor (ZPI). ZPI inhibits the activated factor X (FXa) on phospholipid surface. In mice, the disruption of PZ gene is asymptomatic, but the association with the factor V Leiden mutation leads to a quasi complete mortality during the neonatal period with microvascular thrombosis. In humans, PZ is characterized by an unusual wide distribution in plasma, and a major decrease induced by warfarin. Isolated PZ deficiency does not seem to constitute a risk for venous thrombosis, but a severe PZ deficiency could increase the risk of well recognized venous thrombotic risk factors such as factor V Leiden, G20210A mutation or hyperhomocysteinemia. Unexpectedly, a relationship between PZ deficiency and ischemic arterial diseases such as stroke, acute coronary syndromes or peripheral arterial disease was described but not confirmed by all studies. PZ deficiency could be also a risk factor for early fetal losses, and increases the arterial risk in antiphospholipid syndrome. This review analyzes the different studies so far published and discusses the various results obtained in order to understand whether or not protein Z deficiency could be considered as an arterial ischemic risk factor.
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PMID:Protein Z, a protein seeking a pathology. 1884 Dec 75

The antiphospholipid syndrome is characterized by the occurrence of vascular thrombosis combined with the presence of antiphospholipid antibodies in plasma of patients. It has been published that antibeta2-glycoprotein I (beta2-GPI) antibodies, with lupus anticoagulant activity (LAC), highly correlate with thrombosis. Resistance related to antiphospholipid antibodies against activated protein C (APC) is one of the proposed mechanisms responsible for thrombosis. We investigated a possible correlation between a beta2-GPI-dependent LAC (titration of cardiolipin into an activated partial thromboplastin time-based assay) and increased APC resistance in a population of 22 plasma samples with LAC activity. Eleven plasma samples that displayed a beta2-GPI-dependent LAC also showed increased APC resistance. In contrast, only one of the 11 plasma samples with a beta2-GPI-independent LAC displayed increased APC resistance. In addition, a monoclonal antibeta2-GPI antibody and patient-purified immunoglobulin G (both with LAC activity) were diluted in plasma with/without protein C. Both antibodies only displayed a beta2-GPI-dependent LAC in plasma in the presence of protein C. This indicates that the principle of the beta2-GPI LAC-assay was based on increased resistance against protein C. Surface plasmon resonance analysis was used to investigate binding between APC and beta2-GPI. We observed that beta2-GPI was able to bind APC directly, especially in the presence of a monoclonal antibeta2-GPI antibody. In conclusion, our observations show a direct correlation between a major clinical symptom of antiphospholipid syndrome (thrombosis), a diagnostic assay (beta2-GPI-dependent LAC) and a potential mechanism responsible for thrombosis in the antiphospholipid syndrome (increased APC resistance).
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PMID:Correlation between the potency of a beta2-glycoprotein I-dependent lupus anticoagulant and the level of resistance to activated protein C. 1900 41

Prothrombin (PT) is a target for antibodies with lupus anticoagulant (LA) activity. Anti-prothrombin antibodies (aPT) were recently identified as antibodies directed toward a phospholipid-binding protein. aPT are a new serologic marker of antiphospholipid syndrome. The objective was to detect aPT in a group of 46 patients with acute ischemic stroke in order to correlate their presence with clinical diagnosis, laboratory and neuroradiological findings. We tested aPT, lupus anticoagulant (LA), anticardiolipin (aCL), and anti-beta2-glycoprotein I antibodies (anti-bbeta2-GPI) in 46 young women with acute ischemic stroke aged 34-45 years and 43 patients with nonischemic neurologic diseases and 141 normal controls. Anti-prothrombin antibodies were detected by calcium-containing aPT ELISA, aCL and anti-beta2-GPI by ELISA. All samples were screened using the activated partial thromboplastin time (aPTT); the dilute Russell viper venous time (dRVV) coagulation test was performed. The results were statistically analyzed. Anti-prothrombin antibodies were found in 26 (57%) of 46 stroke patients. Out of 43 patients with nonischemic neurological disorders, 2 (4.18%) were positive for aPT. aPT were detected in one (0.70%) of the normal controls. Ten stroke patients (21%) were positive for IgG aPT only, 9 stroke patients (18.2%) for IgM aPT only, and 8 stroke patients (16.9%) for both IgG and IgM isotypes of aPT. Two nonischemic neurological disorders patients (4.18%) presented IgM isotype of aPT. Patients with ischemic stroke presented aPT much more frequently than the healthy controls (OR 182.00 [95% CI 23.382-1416.6]. p < 0.0001). Patients with ischemic stroke presented aPT much frequently than the nonischemic neurological disorders patients (OR 26.650 [95% CI 5.743-123.66], p < 0.0001). When IgG or IgM aPT were considered separately, they were more frequently found in patients with ischemic stroke than in healthy control group (OR 38.889 [95% CI 4.817-313.95], p < 0.0001) and (OR 34.054 [95% CI 4.178-277.5], p < 0.0001), respectively. Simultaneous positive titers for both isotypes of aPT (IgG and IgM) were more frequently found in patients with ischemic stroke than in healthy control group (OR 29.474 [95% CI 3.573-243.12], p < 0.0001). Eleven stroke patients (43%) were negative for aCL, LA and anti-beta2-GPI, but positive for aPT (OR 0.03287 [95% CI 0.001794-0.6022], p < 0.001). aCL, LA and anti-beta2-GPI were not found both in nonischemic neurological disorders patients and in healthy controls.
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PMID:Detecting anti-prothrombin antibodies in young women with acute ischemic stroke. 1948 Mar

Anticoagulation with oral vitamin K antagonists (VKA) is the mainstay of the treatment of venous and/or arterial thromboembolism in patients with antiphospholipid syndrome (APS), although the optimal intensity of anticoagulation remains controversial. The limitations of existing anticoagulants have driven a search for novel agents. Dabigatran etexilate (Pradaxa), a direct thrombin inhibitor (DTI), and rivaroxaban (Xarelto), the first in a new class of drugs, the oral direct factor Xa (FXa) inhibitors, are both fixed-dose orally administered agents. They are now licensed in the UK and Europe for the prevention of venous thromboembolism (VTE) in adult patients undergoing elective total hip replacement (THR) or total knee replacement (TKR). Prospective randomized clinical trials suggest that these agents, and also apixaban, a further oral direct anti-Xa inhibitor, may have potential in other areas including the treatment of acute VTE, prevention of stroke or systemic embolism in patients with atrial fibrillation (AF) and acute coronary syndromes. Here, we summarize current indications for these agents and address the potential for their use in patients with thrombotic APS.
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PMID:Antithrombotic treatment failures in antiphospholipid syndrome: the new anticoagulants? 2035 92


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