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)

Plasmas from 16 patients that were found to be positive both for anticardiolipin antibodies (ACA) and lupus anticoagulants (LA) were incubated with liposomes that contained anionic phospholipids. In 11 of these plasmas, ACA could be cosedimented with the liposomes in a dose-dependent manner, whereas LA activity of the remaining supernatant was unaffected. LA activity of purified total IgG from 6 patients was measured in three different coagulation tests, using normal plasmas from different species. Prolongation of the aPTT, KCT and dRVV clotting times was observed only with normal plasma from human origin, not with bovine, rat or sheep plasma. Highly purified coagulation factors Xa, Va and prothrombin, both of human and bovine origin, were used to establish for two patient IgG's the effect of LA on the rate of thrombin formation in the presence and absence of lipid vesicles composed of 20 mole% phosphatidylserine and 80 mole% phosphatidylcholine. A strong and dose dependent inhibition by LA was observed only when human prothrombin was used as substrate in the prothrombinase complex in the presence of lipids. No inhibition was found when bovine prothrombin was used as substrate. The inhibitory effect observed in the presence of human prothrombin was independent of the source of factors Xa and Va, and was not found in the absence of lipid. Preliminary binding studies suggest that LA only associate with a lipid surface, provided that human prothrombin and calcium ions are present. These data indicate that LA are not directed to phospholipids alone, but presumably recognize an epitope which becomes exposed upon Ca(2+)-mediated binding of human prothrombin to phospholipids.
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PMID:Lupus anticoagulant IgG's (LA) are not directed to phospholipids only, but to a complex of lipid-bound human prothrombin. 179 7

Phospholipids bearing a proportion of anionic species such as phosphatidylserine are necessary to promote the anticoagulant potential of the protein C pathway. Factor Xa (200 or 350 pM) was found to activate protein C in a thrombomodulin-independent reaction requiring only phospholipids in Al(OH)3,-adsorbed plasma resupplemented with physiological concentrations of protein C (70 nM) and protein S (130 nM). All experiments were performed in the presence of an excess of hirudin. The activity of activated protein C was assessed by the survival of factor Va. The optimal phospholipid concentration range was 5 to 25 microM with a proportion of phosphatidylserine of 50% (mol/mol) resulting in a half-life of factor Va of 7.5 min in the absence of protein S and 4.2 min in its presence. Dns-EGR-Xa, an inactive derivative of factor Xa, behaved as an apparent protector of factor Va. When replacing factor Xa, thrombin at 10 nM was not an efficient protein C activator in the absence of purified human placenta thrombomodulin. In the presence of 100 pM activated protein C, factor Va half-life was 2 min in the absence of protein S and 1.1 min in its presence in the above optimal phospholipid concentration range. The presence of protein S allowed reduction of phospholipid requirements. Annexin-V (placental anticoagulant protein-I), a potent phospholipid antagonist, fully protected factor Va from degradation by phospholipid-dependent mechanisms. Factor Va was partially protected in the plasma of a patient having experienced thrombosis associated with lupus-like anticoagulant and anti-phospholipid auto-antibodies.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The catalytic role of anionic phospholipids in the activation of protein C by factor Xa and expression of its anticoagulant function in human plasma. 179 56

The antiphospholipid antibody syndrome (APLAS) is a unique clinical syndrome with features of recurrent thrombosis, recurrent fetal loss, and thrombocytopenia. It is associated with a false positive test for syphilis, a prolonged partial thromboplastin time (PTT), a positive test for lupus anticoagulant (LA), and anticardiolipin antibodies (ACLA). A case report illustrating some of the clinical and laboratory abnormalities and therapeutic dilemmas is presented. The literature is then reviewed.
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PMID:Antiphospholipid antibody syndrome: a case report and review of the literature. 180 Nov 88

We report a case of a 47-year-old man with anti-phospholipid antibody syndrome associated with subdural hematoma. The patient had several episodes of arthritis during his thirties. He developed venous thrombosis in his right lower leg at the age of 35, when laboratory studies demonstrated prolongation of activated partial thromboplastin time (APTT) and a biological false-positive result occurred on a syphilis test. When bulbar palsy and Horner's syndrome in the right eye suddenly appeared at the age of 42, he was diagnosed as having brain stem infarction. At the age of 47, he developed constructional apraxia, dyscalculia, skilled movement disturbances and generalized convulsions. Subdural hematoma and multiple lacunes in the cerebral white matter were demonstrated with brain MRI. Furthermore, the patient was positive for Rumpel-Leede phenomenon. Laboratory studies revealed mild thrombocytopenia, prolonged bleeding time and APTT, positive antinuclear antibody and positive test results for both lupus anticoagulant and an anti-cardiolipin antibody, namely anti-phospholipid antibodies. Based on these findings, we consider that the tendency of this patient to bleed may have been due to antiphospholipid antibodies, attacking the platelet membranes and that the bridging veins in the subdural space may be the site at which the bleeding tendency easily appears. Anti-phospholipid antibody syndrome accompanied by hemorrhagic complications had rarely been reported. We suggest that special attention should be given to hemorrhagic complications in patients with anti-phospholipid antibody syndrome associated with fragility of the vessels and/or platelet dysfunction.
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PMID:[A case of anti-phospholipid antibody syndrome associated with subdural hematoma]. 180 71

In 45 patients with diabetes mellitus (DM) without cerebro-cardiovascular diseases (CCVD) the modified method of the tissue thromboplastin inhibition test (TTIT) was studied. TTIT is the method of detection of the lupus anticoagulant (LA), LA, first recognized in patients with systemic lupus erythematosus, is presented by a prolonged activated partial thromboplastin time (APTT), a slightly to moderately prolonged prothrombin time (PT), and high incidence of biological false-positive seroreactions for syphilis (BFP). In patients with LA, thrombotic events have been reported. Six of the 45 diabetic patients were TTIT-positive (13.3%). All control subjects were TTIT-negative. In the TTIT-positive diabetics APTT and PT were normal. BFP also were not observed. The difference between LA and these results in TTIT-positive diabetics remains unclear. Clinical profiles except for duration of DM between the TTIT-negative and TTIT-positive diabetics did not differ. Follow-up studies may resolve an association between the results of TTIT and DM.
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PMID:The tissue thromboplastin inhibition test in diabetics without cerebro-cardiovascular diseases. 180 39

An 11-year-old boy developed florid choreic movements in his right extremities after having had an episode of febrile illness. He was evaluated at our hospital where MRI disclosed a honeycomb-like low signal intensity area rimmed by a thin Gd-enhanced layer in the left putamen. Arteriography revealed the lenticulostriate arteries being segmentally narrowed and a "ground glass" staining was observed in the left putamen in late venous phase. Sydenham's chorea, that had been the initial impression, was not substantiated because of negative pharyngeal culture for streptococci, negative ASLO/ASK titers and because of lack of clinical stigmata of rheumatic fever. However, prothrombin time was prolonged, and activated partial thromboplastin time (APTT), that had been also prolonged, was not normalized by adding healthy serum, indicating the presence of lupus anticoagulant. VDRL was false positive and anticardiolipin antibodies, both IgM and IgG classes, were also detected. However, systemic lupus erythematosus was unlikely in view of negative antinuclear antibody and LE phenomenon. He deteriorated rapidly due to development of severe bilateral chorea, thereby he was unable to walk or feed himself. He received a 3-day course of mega-dose intravenous methylprednisolone, that temporarily lessened the chorea, but soon it became worse. A second course of mega-dose methylprednisolone was given, followed by daily maintenance dose of prednisolone. His chorea gradually improved in severity and after 2 months only a trace of choreic movements was detected in his hands. He has been followed at our outpatient clinic where he no longer shows chorea and the APTT has improved to nearly normal time.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of chorea as a sole presentation of primary anti-phospholipid antibody syndrome]. 181 92

Lupus anticoagulant, anticardiolipin, antinuclear, anti-deoxyribonucleic acid, antithyroglobulin, and antithyroid microsomal antibodies were assayed during third-trimester pregnancy (100 normal, 100 with complications). In spite of a normal activated partial thromboplastin time in all instances, lupus anticoagulant was further investigated by three additional procedures: tissue thromboplastin inhibition time, platelet neutralization procedure, and cephalin neutralization test. The prevalence of autoantibodies in pregnancies with hypertension reaches 16% (four with lupus anticoagulant, two with anticardiolipin, and two with antithyroid microsomal antibodies), which is significantly greater than that for idiopathic fetal growth retardation (2%) (one with lupus anticoagulant antibodies) and normal pregnancies (3%) (two with antithyroglobulin and one with autithyroid microsomal antibodies) (p less than 0.01). Autoantibodies were equally distributed between patients with gestational hypertension and those with preeclampsia. When compared with the 42 patients with hypertension and no autoantibodies, the eight patients with autoantibody had a more frequent history of fetal growth retardation (p less than 0.05), but there was no difference in the severity of hypertension, the frequency of obstetric complications, or the outcome of pregnancy. They did not require any specific treatment.
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PMID:The prevalence of autoantibodies during third-trimester pregnancy complicated by hypertension or idiopathic fetal growth retardation. 185 15

A 19-year-old primigravida with known factor XII deficiency (prepregnant factor XII level of 21%) presented with placental abruption and preterm labor at 26 weeks' gestation. A healthy 925-g female infant was born by spontaneous vaginal delivery. The mother had no postpartum hemorrhage or further complications, and the infant demonstrated no intracranial or other forms of hemorrhage up to 70 days of age. The infant's factor XII level was 34% (normal for her age). There are only two previous reports of factor XII deficiency in pregnancy cited in the English literature, and both were uncomplicated. In view of the risk of thromboembolic complications in nonpregnant individuals with factor XII deficiency, pregnant women with a prolonged activated partial thromboplastin time and no lupus anticoagulant or anticardiolipin antibody syndrome should also be investigated for deficiencies of factors VIII, IX, and XII. These patients should be given the appropriate counseling and should be monitored for features of thromboembolism if factor XII deficiency is confirmed.
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PMID:Factor XII deficiency and pregnancy. 187 Aug 3

Chorea has been related to the presence of antiphospholipid antibodies (a-PL) in the context of systemic lupus erythematosus (SLE). Here we report the case of a 13-year-old girl with a-PL antibodies, who had developed thrombophlebitis at the age of 11 years and chorea two years later, in the absence of clinically evident SLE. Serological tests revealed a false positive test for syphilis, a prolonged activated partial thromboplastin time, hypocomplementaemia and positive anti-DNA antibodies.
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PMID:Chorea as a manifestation of the antiphospholipid syndrome in childhood. 187 92

Lupus anticoagulants are antibodies that interfere with in vitro phospholipid-dependent coagulation reactions. In vivo, they have been associated with a variety of thromboembolic problems. Samples from patients with lupus anticoagulants were included in the 1986 and 1987 College of American Pathologists proficiency survey program. Participant performance on these samples demonstrated significant variation in the responsiveness of different activated partial thromboplastin reagents to lupus anticoagulants. The level of factor VIII in these samples reported by the participants also varied with the reagent used. Follow-up studies demonstrated striking reagent-dependent differences in the dilutional effect on apparent factor VIII, IX, XI, and XII activity. These results point out the importance of selecting sensitive and responsive reagents for appropriate identification of lupus inhibitors. In addition, the results indicate that the choice of reagent used for factor assays can affect the apparent factor activity as well as whether a dilutional effect is noted when a lupus anticoagulant is present in the test sample, an important consideration when trying to distinguish a lupus anticoagulant from a specific factor inhibitor.
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PMID:Effect of lupus anticoagulants on the activated partial thromboplastin time. Results of the College of American Pathologists survey program. 189 55


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