Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Protein C (PC) and protein S (PS) are components of a potent, natural anticoagulant system. A deficiency of one of these two inhibitors is associated with thrombotic events in young people. A significant reduction in functional PS activity has been observed during normal pregnancy, and recurrent fetal loss may occur in women with lupus anticoagulant (LA) inhibitor. We measured functional PS activity and free PS antigen in 16 non pregnant patients with LA inhibitor and in 17 normal women as controls. A significant difference was observed between patients and controls in functional PS activity (65 +/- 23% vs 87 +/- 15%, p = 0.02) but not in free PS antigen (88 +/- 17% vs 93 +/- 17%). Functional PS activity decreased only in six patients (37%). Removal of IgG from plasma reduced the difference in functional PS activity between patients and controls. Immunologic IgG levels did not correlate with anti-phospholipid antibodies (APA) activities, activated partial thromboplastin time/kaolin clotting time (aPTT/KCT) data or functional PS activity.
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PMID:Functional protein S in women with lupus anticoagulant inhibitor. 153 34

Patients with systemic lupus erythematosus may develop premature atherosclerosis, notably coronary artery disease. A group of 10 patients with peripheral vascular disease presenting with intermittent claudication or gangrene were studied from a group of 563 patients followed prospectively at the Wellesley Hospital Lupus Clinic. These 10 patients were compared with the next lupus clinic patient matched for age and sex, with respect to demographic characteristics and risk factors. The patients and controls did not differ significantly in lupus activity criteria count, partial thromboplastin time, the number with antibody to cardiolipin, number receiving steroids or mean steroid dose, family history of atherosclerosis, hyperlipidaemia, smoking, hypertension or use of oral contraceptives. The risk factors for developing peripheral vascular disease were a longer duration of systemic lupus erythematosus and a longer duration of use of steroids. Eight of the 10 patients had coexistent coronary artery disease or transient ischaemic attack.
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PMID:Peripheral vascular disease in patients with systemic lupus erythematosus. 154 39

In a retrospective case control study of 96 obstetrical patients 48 cases had partial thromboplastin time (TTPA) with kaolin over 4 seconds compared with the test group. The control group of 48 women with normal TTPA were also studied. Age, socioeconomic status, weight, family and personal illness history were included. Habitual abortion,neonatal death, and hypertension were recorded. The average TTPA value was 53.6 +or- 7.87 seconds for the case group vs 38.8 =or- 4.9 for the controls which was not statistically significant. No statistical significance was found regarding age, start of menarche, nutritional and socioeconomic status, and blood group. The body weight of the case group was higher with 58.5 kg =or- 14.4 kg (a range of 43.4-81.4 kg). There were 7 cases of thrombophlebitis (14.5%) in the lower extremities in the case group and none in the controls. There were 7 cases of habitual abortion in the case group defined as 3 or more miscarriages before 20 weeks of gestation vs 2 cases in controls. There were 4 cases of neonatal deaths associated with premature delivery in the case groups and none in controls. Acute hypertensive disease associated with pregnancy totaled to 8 cases in the 1st group (16.6%) and 4 cases in controls (8.3%). In both groups there were 2 cases of fetal death. In the case group there was 1 case of chromosomopathy and in the control group 1 case of premature expulsion of placenta. The TTPAs test is used mostly for the initial phase of studying patients suspected of having lupus anticoagulant (LA). LA belongs to abnormalities characterized by the presence of antiphospholipid antibodies. It is often used for diagnosing initial stages of autoimmunity which can frequently occur in thrombotic process, fetal loss, intrauterine growth retardation, and increased hypertensive illness in pregnancy.
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PMID:[Presence of inhibitors of activated partial thromboplastin time (TTPA). Clinical repercussion in obstetric patients]. 155 88

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.
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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.
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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.
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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.
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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.
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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.
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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.
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PMID:The preparation of a sensitive partial thromboplastin reagent from bovine brain. 164 6


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