Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To evaluate the usefulness of preoperative screening for coagulation disorders in children, we prospectively studied laboratory and bleeding histories in 1603 children undergoing tonsillectomy. All patients had preoperative laboratory screening with a complete blood count, prothrombin time, activated partial thromboplastin time, and bleeding time. Persistent abnormalities on repeat testing 1 week later were investigated further by a standardized schema. A subset of 129 patients, including all those who bled perioperatively or had laboratory abnormalities, completed a standard historical questionnaire. Thirteen patients had persistent laboratory abnormalities diagnostic of lupus inhibitor (5), non-lupus inhibitor (6), mild hemophilia A (1), and vonWillebrand disease (1). Two patients had persistently prolonged activated partial thromboplastin times of undefined cause. Fourteen patients (10.8%) interviewed reported positive bleeding histories. Of these, five, including the patient with vonWillebrand disease, had persistent laboratory abnormalities. History alone failed to detect the patient with hemophilia A. For patients with inhibitors or prolonged activated partial thromboplastin times of unknown cause, surgery was delayed until the coagulation abnormalities resolved, and there was no perioperative bleeding. The patient with vonWillebrand disease had severe postoperative bleeding despite treatment with cryoprecipitate. In predicting perioperative bleeding, history and laboratory screening had a high specificity but a very low positive predictive value due to poor sensitivity and a low prevalence of bleeding. Some children with bleeding disorders may be identified first during routine preoperative coagulation testing, and replacement therapy or delay or cancellation of surgery may reduce or prevent perioperative hemorrhage. However, the large number of false positive laboratory tests and bleeding histories, coupled with the relative rarity of inherited and acquired coagulopathies, raises doubts about the overall value of routine screening.
 ...
PMID:Preoperative history and coagulation screening in children undergoing tonsillectomy. 851 74

Several coagulation assays identify lupus anticoagulants, but there is no uniform opinion regarding minimum criteria for the diagnosis or the most sensitive procedure to be used to screen for lupus anticoagulants. Based on test availability and the cumulative literature, a sensitive activated partial thromboplastin time appears to be the most appropriate screening test. The dilute Russell's viper venom time may be used as both a screening and a confirmatory procedure. The platelet neutralization procedure is the most sensitive and specific of the confirmatory procedures that use an increased amount of phospholipid to either "bypass" or "neutralize" the lupus anticoagulant.
 ...
PMID:Coagulation assays for the lupus anticoagulant: review and critique of current methodology. 156 67

An inherited deficiency of protein C, a recognized hypercoagulable state, may cause a clinically significant deep venous thrombosis. Only some persons with a deficiency of protein C experience thrombosis, and almost always the thrombotic event occurs in the venous circulation. Warfarin-induced skin necrosis, a rare event observed in some patients soon after treatment with warfarin is begun, is believed to be another manifestation of this deficiency. We describe a young woman whose basal functional and antigenic levels of protein C were about 45% and who experienced both deep venous thrombosis and warfarin-induced skin necrosis in a clinically severe course. Evidence for lupus anticoagulants was present, with prolonged activated partial thromboplastin time that was corrected when lysed platelets were added, prolonged Russell's viper venom time, anticardiolipin antibodies, and other laboratory evidence. Lupus anticoagulants are associated also with a significant incidence of thrombosis, including arterial thrombosis, and this patient developed concurrently arterial thrombosis. The combined effects of protein C deficiency and lupus anticoagulants, exacerbated by other potentially thrombogenic conditions, are believed responsible for the severe thrombotic events experienced by this patient.
 ...
PMID:Concurrent protein C deficiency and lupus anticoagulants. 156 44

Antiphospholipid syndrome (APLS) is characterized by thrombocytopenia, thromboembolic phenomena, and recurrent fetal loss, associated with anticardiolipin antibodies (ACA) and/or lupus anticoagulant. The syndrome may be primary or may be associated with other conditions such as systemic lupus erythematosus. We have previously shown the ability to induce APLS in naive mice following passive transfer of serum and monoclonal ACAs. Similarly we generated the secondary APLS in BALB/c mice following immunization with a pathogenic anti-DNA antibody. In the current study we report on the induction of primary APLS following immunization of BALB/c mice with a human monoclonal ACA (H-3). The mice developed high persistent titers of ACA. The APLS was characterized by prolonged activated partial thromboplastin time, low fecundity rate (21% vs. 48% of control immunized mice), high resorption index of fetuses (25% vs. 3%), and low weights of embryos and placentae. Our study points to the ability of inducing primary APLS in naive mice. The induction of various presentations of APLS by different ACA may explain the diversity of clinical manifestations seen in patients with APLS.
 ...
PMID:Induction of primary antiphospholipid syndrome in mice by immunization with a human monoclonal anticardiolipin antibody (H-3). 156 94

Lupus anti-coagulant (LAC) has been recognised in association with venous and arterial thrombotic events. We retrospectively investigated the relationship between LAC and 21 patients with juxtarenal high aortic occlusion. Four of the 21 patients were LAC positive (19%), one of whom was a woman suffering from systemic lupus erythematosus (SLE). The three men had neither SLE nor any other connective tissue disease. The mean age of the four patients was 43.5 years and three of them (75%) were younger than 50 years of age. By contrast, the mean age of the 17 patients without LAC was 59.9 years and 14 of them (82.4%) were older than 50 years. The postoperative courses in all four patients with LAC undergoing arterial reconstructions were uneventful. Two patients were treated by either anti-coagulation or anti-platelet agents, postoperatively. The present paper demonstrates for the first time that, in patients with juxtarenal high aortic occlusion, the incidence of LAC positivity is higher in younger patients (below 50) than in older patients (above 50). In addition, our results show that a preoperative evaluation of the presence of LAC is required in cases with juxtarenal high aortic occlusion, particularly when the partial thromboplastin time (PTT) is prolonged and the patients are younger than 50 years old.
 ...
PMID:High aortic occlusion associated with lupus anti-coagulant. 159 33

We are reporting a young lady with protracted deep vein thrombosis of her left leg which turned out to be antiphospholipid (anticardiolipin) antibody syndrome of ANA positive systemic lupus erythematosus. Lupus anticoagulant was demonstrated by prolongation of activated partial thromboplastin time and Russell's viper venom time. She had no anti-thrombin III deficiency.
 ...
PMID:Antiphospholipid antibody syndrome of systemic lupus erythematosus presenting as deep vein thrombosis. 829 83

Commercial partial thromboplastin reagents markedly differ in their sensitivity to factor deficiency, heparin, or the lupus anticoagulant. These differences may be partly due to the variable phospholipid content of different commercially available reagents. For over 15 years, we have routinely used a partial thromboplastin prepared from human brain. In the past four years, we have been using a similarly prepared bovine partial thromboplastin reagent. This report describes the preparation of our partial thromboplastin reagent, as well as an analysis of the phospholipid composition of both the human and bovine thromboplastin reagents. Four separate brain preparations produced consistent percentages of the anionic phospholipids, phosphatidylserine, and phosphatidylinositol. The bovine reagent was also compared with commercial partial thromboplastin reagents in the detection of coagulation factor deficiency, heparin, and the lupus anticoagulant.
 ...
PMID:The preparation of a sensitive partial thromboplastin reagent from bovine brain. 164 6

Twenty-three patients with the 'primary' antiphospholipid syndrome were studied over 2-6 years. Twenty-two (96%) had antiphospholipid antibodies detected by ELISA (87% had antibodies to thromboplastin and 70% to cardiolipin), and 18 out of the 21 tested patients (86%) had lupus anticoagulant activity by coagulative assays. Mean age of the cohort was 29.9 years and the sex ratio (female:male) 4.75:1. Eleven patients presented 18 venous and/or arterial thrombosis and 13 had 25 foetal losses (84% occurred during the second and third trimester). Other clinical features were migraine, livedo reticularis, and epilepsy. Three patients had relatives with systemic lupus erythematosus. Thrombocytopaenia was seen in 33%, antinuclear antibodies in low or moderate titre in 30%, and haemolytic anaemia in 13%. During the follow-up, two patients presented recurrent thrombosis despite anticoagulant therapy, one of them dying because of recurrent pulmonary thromboembolism. Four patients achieved successful term pregnancies after treatment with aspirin and a further patient after treatment with aspirin and low dose prednisolone. No patient developed systemic lupus erythematosus or any other definable connective tissue disease. The 'primary' antiphospholipid syndrome may exist as a distinct clinical entity and all younger patients presenting with thrombotic events, foetal losses and/or thrombocytopaenia, without any evidence of a well defined disease, should be tested for antiphospholipid antibodies in order to rule out this syndrome.
 ...
PMID:The 'primary' antiphospholipid syndrome: antiphospholipid antibody pattern and clinical features of a series of 23 patients. 166 49

The antiphospholipid syndrome was diagnosed in 19 of 1078 patients treated between 1987 and 1991. All patients with antiphospholipid syndrome had either anticardiolipin antibody (16/19) or lupus anticoagulant (10/19); three patients had thrombocytopenia, eight patients had a prolonged partial thromboplastin time, and 10 patients had an elevated erythrocyte sedimentation rate. The most common site of involvement was the cerebral circulation (nine patients), manifested by transient ischemic attacks or stroke. Eight patients had upper extremity disease, characterized by symptoms of Raynaud's phenomenon, with angiographic lesions involving the brachial, radial, ulnar, and/or digital arteries. Lower extremity disease occurred in seven patients, with clinical presentations similar to those of atherosclerosis and varying angiographic patterns. In comparison with the population having atherosclerosis, patients with arterial manifestations of antiphospholipid syndrome were more likely to be women (13 of 19 versus 411 of 1078, p less than 0.02), were significantly younger (46.2 years versus 63.6 years, p less than 0.0001), did not smoke (1 of 19 patients versus 700 of 1078, p less than 0.0001), had a higher percentage of upper extremity involvement (8 of 18 versus 13 of 1078, p less than 0.0001), and had a higher incidence of early graft failure (9 of 12 grafts versus 13 of 371 grafts, p less than 0.0001). The syndrome is associated with the repetitive failure of vascular reconstructions and occlusion of native vessels. Antiphospholipid syndrome should therefore be suspected in young, female, nonsmokers with vascular disease, especially those with involvement of the upper extremity, cerebrovascular disease with normal findings on extracranial carotid angiography, and premature graft failure.
 ...
PMID:Vascular disease in the antiphospholipid syndrome: a comparison with the patient population with atherosclerosis. 172 74

Lupus anticoagulant (LA) and anticardiolipin antibodies (ACA) have been associated with thrombotic events and recurrent fetal loss. In order to assess the role of LA with the thrombotic tendency in various disease states we evaluated 38 patients with confirmed LA [tissue thromboplastin index (TTI) greater than 1.3; circulating anticoagulant index (CAI) greater than 15], subgrouped as follows: a) LA associated with systemic lupus erythematosus (SLE) (n = 13); b) primary antiphospholipid syndrome (PAPS) (n = 16); and c) LA associated with other disorders (n = 9). Male/female ratio differed between the groups: 0/13, 6/10 and 4/5, respectively. Venous and arterial thrombotic events were more common in the PAPS group (87%) compared with the SLE group (61%) and the other disorders group (22%). Serum ACA antiphospholipid IgG levels by ELISA were increased in the SLE and PAPS patients, but did not differ between the groups (167 +/- 24 vs. 190 +/- 28 mu respectively). Antiphospholipid IgM levels were higher in the SLE group compared with the PAPS group (127 +/- 15 vs. 67 +/- 16 mu). Mean TTI and CAI levels did not differ between the SLE, PAPS and other disorders groups (1.8 +/- 0.19, 2.8 +/- 0.9, 2.0 +/- 0.3 for TTI; 25 +/- 4, 33 +/- 4, 32 +/- 5 for CAI). Likewise TTI, CAI and ACA levels did not differ in patients with or without thrombosis. We conclude that the prevalence of thrombotic manifestations varies among patients with similar serum intensities of LA and levels of ACA, suggesting that other factors may be involved in the pathogenesis of thrombosis in these patients.
 ...
PMID:Association of lupus anticoagulant and anticardiolipin antibodies with thrombosis in patients with systemic lupus erythematosus, primary antiphospholipid syndrome and other disorders. 173 97


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>