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)

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

Of 42 purified human myeloma proteins tested, two (IgG3 Her and IgM Mag) were found to possess strong lupus anticoagulant (LA) and anti-cephalin activity, as assessed by a dilute activated partial thromboplastin time (dAPTT) and ELISA test, respectively. For these proteins, we confirmed the observation reported by others that LA activity is present in the antigen-binding (Fab) portion of the immunoglobulin molecule. Rabbit anti-idiotype antibodies against IgG3 Her inhibited the anti-cephalin activity of this protein, suggesting that the anti-cephalin activity of IgG3 Her depends on the hypervariable part of the immunoglobulin and thus most probably is a true antigen-antibody reaction. The anti-Her idiotype antibodies were also able to bind to and inhibit the anti-cephalin activity of IgM Mag. ELISA binding and inhibition experiments showed that the anti-idiotype antiserum contained at least two sets of anti-idiotypes; one set that recognizes a cross-reactive idiotype shared by IgG3 Her and IgM Mag, and another set that seems to be unique to the immunizing protein IgG3 Her. Both sets of anti-idiotype antibodies also bound weakly to polyclonal (patient) IgG, indicating an idiotypic cross reaction.
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PMID:Shared idiotypic determinant in mono- and polyclonal anti-phospholipid antibodies with lupus anticoagulant activity. 190 96

Fifty patients with systemic lupus erythematosus were studied for the presence of lupus anticoagulant using three different assays--kaolin clotting time, platelet neutralization test, and tissue thromboplastin inhibition test. Lupus anticoagulant could be detected in seven cases (14%) with the use of one test in cases with a partial prothrombin time with kaolin more than five seconds greater than normal. The detection rate rose to 20% (10 cases) when using all three tests, so a panel of three assays could identify lupus patients apparently at risk for thrombotic complications.
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PMID:Assays for lupus anticoagulant: the sensitivity of different assays. 190 73

Anticardiolipin antibodies (ACA) and lupus-like anticoagulant (LLAC) have been studied in a group of 142 non-hospitalized and a group of 72 hospitalized HIV infected patients. We observed a variable frequency of ACA positivity ranging from 7.7% to 30.3% according to the groups of patients and the isotype of immunoglobulin fraction containing ACA activity. None of the patients investigated presented a prolongation of the activated partial thromboplastin time (APTT) compatible with the presence of a LLAC. Some patients presented a weak anticoagulant activity only detected by the tissue thromboplastin inhibition (TTI) test. No positive correlation was found between this latter test and ACA. We conclude that, like in syphilitic patients, ACA present in HIV infected patients are most often not associated with LLAC.
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PMID:Anticardiolipin antibodies (ACA) are most often not associated with lupus-like anticoagulant (LLAC) in human immunodeficiency virus (HIV) infection. 190 97

An iliac crest bone marrow aspiration in a 24-year-old man was followed by severe haemorrhage into the iliopsoas muscle. A lupus anticoagulant and severe hypoprothrombinaemia, as well as clinical and laboratory pointers to suggest the presence of a systemic lupus erythematosus-like syndrome, were demonstrated. Therapy with prednisone was commenced following recurrent severe epistaxis. His prothrombin time, activated partial thromboplastin time and prothrombin activity improved promptly and his bleeding ceased. The lupus anticoagulant is commonly encountered in the laboratory, but acquired hypoprothrombinaemia is extremely rare. The condition is reviewed and its treatment discussed.
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PMID:Acquired hypoprothrombinaemia and lupus anticoagulant: response to steroid therapy. 190 19

Eight cases with lupus anticoagulants (LA) were diagnosed over the last five years (1984-88). Of these, three were established cases of systemic lupus erythematosus (SLE), where bad obstetric history (2 cases) and recurrent deep venous thrombosis (DVT--1 case) prompted execution of laboratory tests for LA. In the remaining 5 cases, there was no clinical evidence of SLE. However, one case developed laboratory findings suggestive of SLE at a later date. One of these 5 patients was referred for unexplained abnormality in partial thromboplastin time (K). Three had recurrent abortions (one with additional history of DVT) while one had DVT with raised PTT (K). The clinical findings and laboratory tests by which lupus anticoagulants can be diagnosed have been discussed.
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PMID:Lupus anticoagulant. A report of 8 cases. 190 56


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