Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034065 (pulmonary embolism)
 14,979 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The presence of lupus anticoagulant (LA) and anticardiolipin antibodies (ACA) are associated with recurring pregnancy loss. Of 387 consecutive patients investigated at a Recurring Miscarriage Clinic over a three year period, 63 (16%) were positive for LA and ACA or both. Fifty-nine patients by definition were classified as having antiphospholipid syndrome and four also had systemic lupus erythematosus (SLE). Fifty-three subsequent pregnancies occurred in 63 patients and of these 37 ended in a live birth giving an overall livebirth rate of 70%. Treatment included low dose aspirin alone in 37 pregnancies and low dose aspirin and low molecular weight heparin (LMWH) in 16 pregnancies. The decision for treatment was made empirically on past obstetric history and level of LA and ACA, and past history of venous thromboembolic disease. Obstetric outcome was worst in the group who were positive for both LA and ACA, with a success rate of 53%, compared to 72 or 81% in the single parameter groups. Complications in the 37 successful pregnancies included eight Caesarean sections, four cases of intra-uterine growth restriction, one case of pregnancy induced proteinuric hypertension, one deep vein thrombosis and one pulmonary embolism. Patients with antiphospholipid syndrome are at high risk of pregnancy loss as well as maternal morbidity, especially thrombo-embolic disease. A randomised prospective controlled trial is necessary to determine the optimum therapy for pregnancy conservation and thrombprophylaxis.
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PMID:Obstetric outcome in antiphospholipid syndrome. 925 8

We report a 40-year-old Japanese woman with antiphospholipid antibody syndrome (APS) associated with myasthenia gravis (MG). She had a history of miscarriage at the age of 27 followed by pulmonary embolism 3 weeks later. At the age of 40, she developed diplopia, bilateral ptosis and easy fatigability. Serum anti-acetylcholine receptor antibody and tensilon test were positive. She was diagnosed as having MG. The laboratory test revealed mild thrombocytopenia, prolonged activated partial thromboplastin time (aPTT) and positive findings for both beta 2-glycoprotein I-dependent anticardiolipin antibody and lupus anticoagulant. She fulfilled the diagnostic criteria of APS, but did not the criteria proposed by American Rheumatism Association for SLE. An extended total thymectomy was performed after administration of oral prednisolone and low-dose aspirin. This is a patient who had APS associated with MGs: both are known to result from autoimmune abnormality. The clinical and laboratory manifestations of APS were ameliorated after removal of the thymus, suggesting that thymectomy alleviates APS symptoms.
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PMID:[A case of antiphospholipid syndrome associated with myasthenia gravis]. 939 64

We report here, we believe for the first time, the primary antiphospholipid syndrome, presenting with fever, meningismus and skin rash. Serology was positive for antiphospholipid antibodies but negative for antinuclear factor. Such presentations, once meningitis has been excluded, should be screened for antiphospholipid antibodies. If serology proves to be positive, anticoagulation for life should be considered to avoid thrombotic episodes and death due to pulmonary embolism.
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PMID:Meningismus, fever and macular rash as presenting features of the primary antiphospholipid syndrome? 949 54

Antiphospholipid syndrome is characterized by thromboses, fetal losses and antiphospholipid antibodies. This group of antibodies includes lupus anticoagulant, false positive syphilitic reaction, anionic (such as anticardiolipin) and neutral phospholipids antibodies, and anti-phospholipid/cofactors antibodies (such as anti-beta 2-glycoprotein 1). Dominant manifestations are recurrent thrombophlebitis and pulmonary embolism, arterial occlusive disease of young people, obstetrical complications such as fetal losses, livedo, and thrombocytopenia. Warfarin is presently the main treatment.
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PMID:[Antiphospholipid antibodies syndrome]. 978 Nov 33

We report the case of a 43-year-old woman with systemic lupus erythematosus who survived three episodes of catastrophic antiphospholipid syndrome. During the first episode symptoms involved predominantly the central nervous system, whilst during the second episode of multiorgan failure, the cardiovascular system, lungs and kidneys were particularly affected. Twenty months later, the patient experienced an acute exacerbation of chronic renal failure and later, died of massive pulmonary embolism. The characteristic findings of antiphospholipid syndrome included persistently high titers of IgG anticardiolipin antibodies, positive lupus anticoagulant, and microcytic anaemia with a distinct haemolytic component.
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PMID:[Three episodes of acute multiorgan failure in a woman with secondary antiphospholipid syndrome]. 1040 69

Pregnancy has been widely recognized as a predisposing risk factor for deep vein thrombosis (DVT). However, it still remains unclear why pregnant women without a history of familial thrombophilia or antiphospholipid syndrome (APS) have a higher incidence of DVT and pulmonary embolism (PE) during pregnancy and puerperium. We examined the activated protein C (APC) system in healthy pregnant women and in patients with the onset of DVT during puerperium. Sixty unselected Japanese pregnant women without a past or family history of thrombosis or APS and 3 Japanese women with DVT during puerperium were evaluated. Endogenous thrombin potential-ratio (ETP-r) was measured by determination of thrombin-alpha2-macroglobulin complexes in thromboplastin-activated patient plasma. APC sensitivity ratio (APC-sr) was calculated by the determination of ETP-r in patient plasma in the presence and absence of APC (final concentration [conc.] 5.9 nM) to evaluate the functional APC anticoagulant activity. Mean APC-sr was significantly increased at 30 weeks' gestation (2.35 +/- 0.72) and remained high during puerperium compared with the mean APC-sr in nonpregnant women (1.15 +/- 0.63). Mean APC-sr in patients with DVT at the onset was significantly higher (3.57 +/- 0.54) than mean APC-sr during puerperium was, indicating that the sensitivity to APC was reduced in the ETP-based assay. These data suggest a significant reduction in the functional sensitivity to APC associated with an increased risk of venous thrombosis during pregnancy.
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PMID:Detection of decreased response to activated protein C during pregnancy by an endogenous thrombin potential-based assay. 1062 9

In this study, we compared and reviewed the findings on lung perfusion scans performed in patients with systemic lupus erythematosus (SLE), systemic lupus erythematosus with associated antiphospholipid syndrome (SLE + APS), and primary antiphospholipid syndrome (PAPS), to evaluate the prevalence of pulmonary embolism in restricted samples of the patient groups. Lung perfusion scintigraphy with 99Tc(m)-macroaggregated albumin was performed in 31 patients (SLE = 7; SLE + APS = 14; PAPS = 10). The seven patients with SLE alone and the 10 patients with PAPS had normal perfusion lung scans. Six of the 14 SLE + APS patients showed a segmental uptake defect on multi-view perfusion scans. Thus, the SLE + APS patients were found to have a higher risk of pulmonary thromboembolism than the SLE alone and primary APS patients (P<0.05). The results of our study suggest that lung perfusion scintigraphy should be performed routinely in these patients, even in the absence of pulmonary clinical manifestations, to obtain baseline data for disease outcome and management.
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PMID:Comparison of lung perfusion scintigraphic findings in pulmonary thromboembolism in systemic lupus erythematosus, SLE plus antiphospholipid syndrome, and primary antiphospholipid syndrome. 1082 33

beta(2)-Glycoprotein I, an anionic phospholipid-binding 50-kDa plasma protein, circulates in the plasma at a concentration of 30-200 microg/ml. Its physiological role remains uncertain, but an important clue to this role is suggested by the finding that antibodies to this protein are frequently found in patients with antiphospholipid antibodies and thrombosis. beta(2)-Glycoprotein I belongs to the complement control protein (CCP) superfamily with five CCP domains. The fifth CCP domain of beta(2)-glycoprotein I has a unique structure and contains a stretch of positively charged amino acids that mediates the binding to phospholipids. This interaction may mediate the clearance of anionic phospholipid-containing surfaces from the circulation. Mutations in this domain affect its binding to phospholipids. We have identified a patient with primary antiphospholipid syndrome who is a compound heterozygous for two mutations in the fifth CCP. One mutation is located in exon 7 (codon 306), and the second mutation is in exon 8 (codon 316). The mutant beta(2)-glycoprotein I was present in normal quantities in his plasma but did not bind to cardiolipin. He had recurrent deep vein thrombosis and pulmonary embolism at age 28 and a thrombotic stroke at age 35, with no other identifiable risk factor for a hypercoagulable state. This report offers some insight into the mechanism of formation of antiphospholipid antibodies and suggests the possible role of the deficiency of beta(2)-glycoprotein I in the pathogenesis of thrombosis.
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PMID:Primary antiphospholipid antibody syndrome with mutations in the phospholipid binding domain of beta(2)-glycoprotein I. 1099 35

Anti-beta2-glycoprotein I antibodies are considered as a specific marker for the antiphospholipid syndrome. In contrast to lupus circulating anticoagulant and anticardiolipin (aCL) antibodies, they are usually not found at significant levels in infections. We report a case of pulmonary embolism in an adult with varicella. Transient significant levels of aCL antibodies and of IgM anti-beta2-GPI antibodies were observed. No other prothrombotic factor, including free protein S antigen deficiency, was found. The direct pathogenic role of these transient antibodies on the thrombotic event may then be suspected. They are probably associated with VZV acute infection and are absent two months after varicella.
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PMID:Pulmonary embolism and transitory anti-beta2-GPI antibodies in an adult with chicken pox. 1103 26

A 51-yr-old woman developed pulmonary embolism while undergoing surgery for breast cancer with Auchincloss method. General anesthesia was maintained with propofol and fentanyl. The course of anesthesia and operation were uneventful until the skin was sutured, when unexplained severe circulatory collapse developed. A widely dissociated EtCO2-PaCO2 suggested pulmonary embolism. The pulmonary circulation was restored 10 minutes after the start of heparin therapy, and the intravenous heparin administration was continued. The patient was extubated uneventfully 24 hours postoperatively. Lung perfusion scintigrams showed defect in right lower lungs (S 6, S 9). Venograms of the lower extremity disclosed thrombosis of the right popliteal vein. Furthermore, lupus anticoagulant was noted in the serum. Patients with antiphospholipid syndrome often develop pulmonary complications including pulmonary embolism and pulmonary hypertension. Intra-operative pulmonary embolism associated with lupus anticoagulant is a rare case.
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PMID:[A case of intraoperative pulmonary embolism associated with lupus anticoagulant]. 1107 59


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