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

A case of transient filling defects in the renal pelves and ureters, secondary to spontaneously occurring acquired anticoagulants, is presented. The relationship of this entity to hemophilia, immunologic disorders, such as systemic lupus erythematosis, and normal post partum patients is discussed. The differential diagnosis for filling defects in the renal pelvis is reviewed.
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PMID:Filling defects in the renal pelvis and ureter owing to bleeding secondary to acquired circulating anticoagulants. 97 26

A 27-year-old female with severe systemic lupus erythematosus with renal involvement developed extensive cutaneous hemorrhages 5 years after diagnosis. Routine coagulation tests confirmed a prolongation of activated partial thromboplastin time to 77 s. This was attributed to a marked reduction of factor VIII activity to less than 3%. An inhibitor with an activity of 1.4 Bethesda units against factor VIII was determined. Immunosuppressive therapy (steroids, azathioprin, cyclophosphamide, cyclosporine) had no influence on the hemorrhages. Later in the course of disease a life-threatening vaginal hemorrhage occurred in parallel with a flare-up of lupus activity. During that period a therapy of 7 S i.v. immunoglobulins (120 g within 5 days) was started. This led to an instant cessation of the bleeding. Factor-VIII activity rose from 3% ot 480% within 7 days and the ds-DNA-antibodies fell from 122 U/ml to 19.7 U/ml. Nine months later, under immunosuppressive therapy with cyclophosphamide and steroids, factor-VIII activity is still within the normal range and no bleeding episodes have occurred. This confirms the effectively of high-dose immunoglobulin therapy for hemophilia, due to acquired factor VIII antibodies, also in patients with severe SLE.
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PMID:[Long-term remission after i.v. immunoglobulin therapy in acquired antihemophilic factor hemophilia with systemic lupus erythematosus]. 212 57

The prevalence of lupus anticoagulant, using the dilute Russell's viper venom time (DRVT), was determined in 22 patients with mild to severe haemophilia A to see if there was any association with the presence of viral disease. Twelve haemophiliacs (58%) were lupus anticoagulant positive, with a mean patient:control ratio of 1.24 (range 1.15-1.52, normal range 0.84-1.06 which partially corrected with lysed, washed platelets). Nine of these patients were IgG or IgM, or both, anticardiolipin antibody positive and nine were human immunodeficiency virus (HIV) antibody positive, but associations between lupus anticoagulant, anticardiolipin antibodies, and HIV antibody positivity were not significant. Mixing studies of normal plasma and immune depleted factor VIII deficient plasma showed that the DRVT ratio increased when the factor VIII concentration fell below 0.15 IU/ml. There was no significant association between plasma factor VIII concentration and positive DRVT results in haemophiliacs. The addition of porcine factor VIII concentrate produced no correction in eight of the 12 with DRVTs indicative of lupus anticoagulant, suggesting that these were prolonged by antiphospholipid activity. It is concluded that the presence of lupus anticoagulant and anticardiolipin antibodies in haemophiliacs may represent an antiphospholipid response to viraemic challenge, not only to HIV but also to other viral antigens, and that a very low factor VIII concentration may produce a false positive DRVT result.
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PMID:Lupus anticoagulant, anticardiolipin antibodies, and human immunodeficiency virus in haemophilia. 250 Apr 59

We report studies of the validity and clinical application of a new amidolytic method for evaluating the activated partial thromboplastin time (APTT) compared with a conventional clotting method. The results with the two methods were well correlated for normal plasma and plasma from hemophilia patients. Congenital deficiencies of of the intrinsic coagulation pathway other than hypo- and dysfibrinogenemia detected by the amidolytic method agreed with those detected by the clotting APTT. The results with the two methods for plasma from patients under heparin treatment were statistically different, suggesting a lesser sensitivity of the amidolytic method to heparinization. The use of the amidolytic APTT reagent in combination with factor VIII and IX deficient plasma allowed the measurement of both factors. The results obtained with normal and hemophilic plasma were in agreement with those obtained with the one-stage clotting method in all except two occasions. Even though the amidolytic method demonstrated the presence of the lupus anticoagulant in the majority of tested samples of known lupus subjects, it was less sensitive to the abnormality than the clotting method.
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PMID:Automated amidolytic method for evaluating the activated partial thromboplastin time compared with a conventional coagulation method. 250 8

A 12.5 year old girl was admitted to hospital with the typical signs of hemolytic uremic syndrome, and systemic lupus erythematodes as well. On the basis of clinical, blood chemistry, and histological findings we assumed an hemolysis-induced form of hemolytic-uremic syndrome as the most likely pathogenic mechanism. The child also suffered from congenital IgA-deficiency and produced an inhibitor against coagulation factor VIII. Congenital IgA-deficiency, systemic lupus erythematodes, inhibitor-induced hemophilia and hemolytic uremic syndrome are suggested to form a pathogenic sequence.
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PMID:[Hemolytic uremic syndrome following juvenile lupus erythematodes disseminatus]. 309 24

Over a period of nine years we observed 52 children with acute neurological symptoms which were caused by a cerebrovascular disease. Fourteen patients had congenital vascular malformations, most frequently AV-angiomas (9 patients). A Sturge-Weber-Syndrome and a venous angioma were found in two cases and one patient had an aneurysm of the middle cerebral artery. Thirty-eight patients had acquired cerebrovascular diseases such as ischaemic infarctions (22), intracranial haemorrhages without vascular malformations (14) and thromboses of the dural sinus (2). The cerebral infraction was a complication of a congenital heart disease in 8 children, two others suffered from chronic renal insufficiency and were on haemodialysis. Two children had a trauma of the internal carotid artery and in one patient a large haemorrhagic infarct was caused by hypernatremic dehydration. In 9 patients (6 females, 3 males) no obvious aetiology of the infarct could be found. However, in most of these cases a nonspecific febrile illness preceded the neurological manifestations. The thrombosis of the dural sinus occurred in a 6-week old previously healthy infant and in a 3-year old boy as a complication of a nephrotic syndrome. Intracranial haemorrhages (without cerebrovascular malformations) occurred in 14 patients, mainly as a complication of haematological diseases (acute lymphatic leukaemia, severe aplastic anaemia, haemophilia A, lupus erythematodes). Four children had spontaneous intracerebral haemorrhages without obvious causes. The prognosis for survival was good in children with infarcts, but persisting neurological deficits were more severe than in children with haemorrhages. At the acute stage the lethality was higher in children with intracranial haemorrhages.
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PMID:[Cerebrovascular diseases in childhood--etiology, clinical aspects and prognosis]. 395 16

A variety of immune phenomena have been associated with chlorpromazine. One such factor, the lupus-like anticoagulant, while nonspecific, has been reported in 26 to 75% of chronic chlorpromazine recipients, although the exact incidence is unknown and requires further investigation. Such patients may have an enhanced risk of bleeding profiles (hemophilia-like) or, conversely, thrombotic events. A case of such a lupus-like anticoagulant with a circulating inactivator of coagulation is discussed in light of the recent literature.
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PMID:A circulating lupus-like coagulation inhibitor induced by chlorpromazine. 642 Apr 48

This study has identified IgG and IgM anticoagulant antibodies in the synovial membranes of patients suffering from haemophilia and rheumatoid arthritis (RA) but not in synovial tissues from normal subjects or in patients with other arthritides. In the majority of cases the antibody appeared to have the specificity of the lupus-like anticoagulant (LLA) seen in patients with systemic lupus erythematosus (SLE). The importance of these findings with regard to the treatment of certain cases of haemophilia and RA and the possible relation between the presence of these antibodies and viral infections is discussed.
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PMID:Anticoagulant antibodies in the synovial membranes of patients suffering from haemophilia, rheumatoid arthritis and other rheumatic disorders. 680 34

A New method for the detection of lupus anticoagulant was developed. Plasma factor V activities using tissue thromboplastin (simplastin auto-SA) and partial thromboplastin (platelin excel LS-LS) were measured simultaneously. Furthermore, the ratio of the two activities was calculated (SA/LS ratio) as a marker of lupus anticoagulant. The normal range of SA/LS ratio was 0.79-1.39 (mean +/- 3SD). The high SA/LS ratios were detected in all of 15 patients with positive lupus anticoagulant, in 3 (5.8%) of 52 SLE patients with normal activated partial thromboplastin time, in 2 (28.7%) of 7 patients with hemophilia, in 1 (33.3%) of 3 patients with factor VIII inhibitor and in 4 (3.3%) of 122 patients with various diseases, respectively. All of 15 patients with liver dysfunction, 54 patients under warfarin treatment and 8 patients under heparin treatment had normal SA/LS ratio.
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PMID:[Newly developed method by the use of factor Y assay for the detection of the lupus anticoagulant]. 836 Oct 39

The inosithin neutralization test was performed in 14 patients in whom lupus anticoagulant was detected. To test its specificity, it was also performed in 10 patients with severe hemophilia and in three patients with Factor VIII inhibitors. Prolonged kaolin clotting time was corrected by adding varying amounts of inosithin (Asolectin, 0.19 to 100 micrograms), a soybean-derived phospholipid, in all patients with lupus anticoagulant but not in patients with hemophilia or in two patients with Factor VIII inhibitors. In one patient, both Factor VIII inhibitors and lupus anticoagulant were present. The concentration of lupus anticoagulant in a patient was expressed as the amount of inosithin (measured in micrograms) required to normalize the prolonged kaolin clotting time. This amount correlated significantly with the occurrence of thrombosis and/or recurrent abortion. The inosithin neutralization test is useful to measure lupus anticoagulant.
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PMID:Inosithin neutralization test to measure lupus anticoagulants. 842 19


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