Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: 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)

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

We have studied the conditions of in vitro binding of platelet glycoprotein IIb/IIIa (GPIIb/IIIa) to fibrinogen and applied the results to identify and measure the serum inhibitors to the binding. For the enzyme-linked immunosorbent assay, platelet extract was delivered to a fibrinogen-coated microtiter plate that was incubated for 2 hours, followed by incubation with anti-GPIIb/IIIa monoclonal antibody for another 2 hours. The plate was then incubated with peroxidase-conjugated anti-mouse IgG for color development. The binding was shown to be calcium-dependent. The binding was partially blocked by treating the coated fibrinogen with anti-fibrinogen antibody. Reduction or dissociation of GPIIb/IIIa resulted in the total loss of its ability to bind to fibrinogen. Platelet extracts of patients with hemophilia showed decreased binding (25% and 14%, compared with control platelet extract), and an extract from a patient with Glanzmann's thrombasthenia showed no binding. With the enzyme-linked immunosorbent assay we have measured serum inhibitors to GPIIb/IIIa binding to fibrinogen in 35 hemophilia A, 17 immune thrombocytopenic purpura, 22 human immunodeficiency virus-related immune thrombocytopenic purpura, and 29 systemic lupus erythematosus serum samples. In those patients with inhibition by serum, polyethylene glycol precipitation of circulating immune complexes (CICs) decreased the inhibition by the supernatants, and all the resolubilized CIC precipitates demonstrated inhibition, which indicates that CICs play a major role in the inhibition of GPIIb/IIIa binding to fibrinogen. This, then, provides evidence of CIC-mediated impaired GPIIb/IIIa binding to fibrinogen in hemophilia A, HIV-ITP, and SLE.
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PMID:Inhibition of platelet GPIIb/IIIa binding to fibrinogen by serum factors: studies of circulating immune complexes and platelet antibodies in patients with hemophilia, immune thrombocytopenic purpura, human immunodeficiency virus-related immune thrombocytopenic purpura, and systemic lupus erythematosus. 200 77

We report three patients with acquired inhibitors against F VIII:C/F vW:Ag complex. Two patients had acquired hemophilia A. The three patients presented with bleeding diathesis. Case 1 was a 19 years old woman with Graves-Basedow disease; case 2 was a 40 years old woman with systemic lupus erythematosus of four years; and case 3 a 38 years old woman who had had rheumatoid arthritis for five years and was in her 3d month postpartum. The F VIII:C level was below 8 U/dL in all cases. The F vW:Ag, ristocetin cofactor and platelet aggregation with ristocetin were diminished in the two cases with von Willebrand. Inhibitor to F VIII:C was 50, 38 and 20 Bethesda units, respectively, for cases 1, 2 and 3. The three patients showed clinical response to DDAVP and cryoprecipitates with partial response in laboratory tests. All patients responded to corticosteroid treatment, but immunosuppressive treatment was necessary in case 3.
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PMID:[Report of 2 cases with acquired von Willebrand disease and one with acquired hemophilia A]. 756 65

Lupus anticoagulants (LAs) are immunoglobulins (IgG, IgM, or both) which interfere with in vitro phospholipid (PL) dependent tests of coagulation (e. g. APTT, dilute PT, dilute Russell Viper Venom Time). These antibodies may be identified in a wide variety of clinical settings. With the exception of heparinized patient samples, the presence of LAs is often the most common cause of an unexplained APTT in a routine clinical laboratory. The diagnosis of LAs is difficult due to variable screening reagent sensitivity and intrinsic heterogeneity of LAs. Recently, Rauch and colleagues have shown human monoclonal hybridoma LAs were inhibited by hexagonal (II) phase PLs. In contrast, lamellar phase PLs had no effect. We have evaluated a new assay system, Staclot LA, which utilizes a hexagonal (II) phase PL (egg phosphatidylethanolamine [EPE]) as a confirmatory test for LAs. Plasma samples from the following patient populations were studied: LA positive, heparinized, oral anticoagulated, hemophilia A and B, and specific factor inhibitors (factors V, VIII, IX). Unlike previous studies, the LA positive patients were a mixed population including: autoimmune diseases, drug-induced, and post-infection. Our findings confirm the specificity of hexagonal (II) phase PL neutralization of LAs.
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PMID:A hexagonal (II) phase phospholipid neutralization assay for lupus anticoagulant identification. 812 36

Lupus anticoagulants (LA) are immunoglobulins (IgG, IgM, IgA or a mixture) which interfere with in vitro phospholipid (PL) dependent tests of coagulation (e.g. APTT, KCT, dilute Russell Viper Venom Time). LA are heterogeneous; consequently, the laboratory diagnosis is difficult and relies on multiple tests. We have developed a sensitive and relatively specific confirmatory test system based on fractions of two snake venoms. Textarin, a protein fraction of Pseudonaja textilis venom (Australian Eastern brown snake), activates prothrombin in the presence of PL, factor V and calcium ions. Ecarin, a protein fraction of Echis carinatus venom, will activate prothrombin in the absence of any cofactors. The activation of prothrombin by Textarin yields thrombin while Ecarin yields meizothrombin. In the presence of LA, the Textarin time is prolonged and the Ecarin time is unaffected. The test results are reported as a ratio of Textarin/Ecarin times (abnormal greater than 1.3). We have evaluated this test system in the following patient populations: LA positive, therapeutically heparinized, stable oral anticoagulated, liver disease, routine preoperative, anticardiolipin antibody positive LA negative, hemophilia A, various other hereditary factor deficiencies or dysfunctional proteins, and specific inhibitors of factor V and factor VIII. The LA positive patients represented a mixed population of autoimmune disease, drug-induced and post-infectious states. Our findings indicate the sensitivity of the Textarin/Ecarin system in the confirmation of LA. In order to use the test system most effectively, it is recommended to incorporate polybrene with Textarin when evaluating heparinized samples. Factor V deficiency and specific inhibitors of factor V yielded, in some instances, false positive results.
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PMID:The Textarin/Ecarin ratio: a confirmatory test for lupus anticoagulants. 816 13

Lupus anticoagulants (LA) are associated with an increased risk of thrombosis and laboratory detection is of major importance. Various tests are available for LA screening and confirmation, but they differ in sensitivity and specificity, frequently lacking the ability to discriminate between the presence of LA, heparin and oral anticoagulants. We noticed that a patient with LA who had a prolonged activated partial thromboplastin time (APTT) by our routine method, gave a normal result with a different APTT reagent. This latter reagent, which contained soy bean phosphatides (SBP), was compared with a reagent containing rabbit brain phospholipids complexed with kaolin (RBK), for APTT measurement in a variety of patients. There was no significant difference in APTT ratio between the two reagents in plasma samples from healthy normal subjects. In LA samples, SBP gave consistently lower APTT ratios than RBK (mean +/- SEM, 1.04 +/- 0.05 and 2.08 +/- 0.19 for SBP and RBK respectively; P < 0.001). In LA patients receiving oral anticoagulants for antithrombotic prophylaxis or treatment, the APTT ratio was again significantly shorter with SBP (1.60 +/- 0.17 and 3.40 +/- 0.67; P < 0.05). In LA negative patients receiving oral anticoagulants, the relationship was reversed, and a higher APTT ratio was obtained with SBP than RBK (1.61 +/- 0.13 and 1.31 +/- 0.12; P < 0.001). In addition, there were no significant differences in APTT ratios for the two reagents when samples from patients receiving heparin therapy, or patients with acquired factor VIII deficiency or inherited deficiency of factor VIII or IX were studied. The use of the SBP reagent alongside a LA sensitive APTT reagent allows a rapid screening for LA, as well as a confirmation of the phospholipid dependency of the inhibitor.
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PMID:A rapid screen for lupus anticoagulant with good discrimination from oral anticoagulants, congenital factor deficiency and heparin, is provided by comparing a sensitive and an insensitive APTT reagent. 916 15

We developed a simple ELISA for FIX inhibitors (FIXinh). The relationship between absorbance and various dilutions of plasma containing known FIXinh (28 Bethesda units/ml) was linear, ranging from 200 to 25,600-fold dilution on semi-log paper. Converted into the value of Bethesda Inhibitor Assay, ELISA can detect as low as 0.219 Bethesda units/ml. Absorbance for 30 normal individuals was 0.213 +/- 0.008, which corresponds to < 0.44 Bethesda units/ml. With ELISA, only the value for hemophilia B with FIXinh, which was revealed as positive by Bethesda Inhibitor Assay, was significantly higher than that of normal controls, hemophilia B without FIXinh, hemophilia A with FVIIIinh and systematic lupus erythematosus (SLE) with lupus anticoagulant (LA). The value in the patient with SLE, who had a high level of FVIIIinh (1000 Bethesda units/ml), was 3 Bethesda units/ml of FIXinh with Bethesda Inhibitor Assay, which was not significantly higher than that of normal subjects with ELISA. FIXinh in hemophilia B patients by ELISA for FIXinh was not affected by the presence of other anticoagulants.
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PMID:A simple method for detection of human factor IX inhibitor using ELISA. 945 90

By virtue of a severely prolonged aPTT with a normal thromboplastin time (prothrombin time) and a normal thrombin time, severe FXII deficiency has been diagnosed in a woman without a bleeding diathesis or a history of thromboembolic complications. A deficiency of a factor of the contact activation system (FXII, prekallikrein, high molecular weight kininogen) is usually diagnosed during routine coagulation tests demonstrating a prolonged aPTT. The severe and partial deficiency of FXII, of prekallikrein or high molecular weight kininogen is not associated with a bleeding tendency. In contrast, severely factor XI deficient subjects may suffer from a mild hemorrhagic diathesis, whereas FVIII deficiency (hemophilia A, autoimmune "hemophilia", von Willebrand disease) and FIX deficiency (hemophilia B) are associated with a bleeding tendency of varying severity, depending on the clotting activity of FVIII or FIX, respectively. An isolated prolongation of the aPTT due to a lupus anticoagulant, however, is frequently associated with arterial and/or venous thrombosis. Therefore, in case of a prolongation of the aPTT, its cause has to be determined.
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PMID:[A patient with isolated prolongation of aPTT without hemorrhagic diathesis anamnesis: severe, hereditary factor XII deficiency]. 1051 21

Activated partial thromboplastin time may be prolonged as the result of either of two different autoimmune complications of chronic lymphocytic leukemia: the development of antiphospholipid antibodies, such as lupus anticoagulant or anticardiolipin antibodies, or anti-factor VIII inhibitors, such as acquired hemophilia A. In the rare simultaneous occurrence of both inhibitors, differential diagnosis of a prolonged activated partial thromboplastin time poses a number of problems during laboratory work-up, due to mutual interference of the commonly performed tests. Only careful clinical follow-up can disclose the significance of the laboratory findings. We report the case of concurrent antiphospholipid antibodies (lupus anticoagulant positivity, anticardiolipin antibodies; IgM 3880 MPL/mL and IgG 265 GPL/mL) and anti-factor VIII antibodies (46.8 Bethesda Units) in a patient with chronic B-cell lymphocytic leukemia who had prolonged activated partial thromboplastin time (78.8 s). The relationship between lymphoproliferative and antiphospholipid syndrome, laboratory work-up in the case of the association of antiphospholipid and anti-factor VIII antibodies, and related problems that occur during clinical management of the patient are also discussed.
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PMID:[The antiphospholipid syndrome during chronic lymphatic leukemia. An association with anti-factor VIII antibodies]. 1052 24

Clinico-haematological features in 10 patients with acquired Haemophilia are presented. Three patients had FVIII inhibitors following pregnancy while in six the cause for development of inhibitors could not be determined. One patient had acquired von Willebrand's disease. Lupus anticoagulant coexisted with Factor VIII inhibitors in three patients. All patients presented with sudden onset of bleeding without any past or family history of a bleeding disorder. Factor VIII inhibitor levels ranged from 8 to 512 Bethesda units in the nine patients. Immunosuppressive therapy was given to 8 patients, consisting of CVP regimen or corticosteroids with endoxan or cyclosporin. Seven patients had clinical and laboratory responses and one patient did not respond. One patient had severe postpartum bleeding with acute shock which was controlled with FEIBA. Diagnosis and management of idiopathic acquired Haemophilia, thus, continues to be a major challenge, and among acquired Haemophilia, postpartum Haemophilia has good prognosis.
Haemophilia 2000 Mar
PMID:Acquired haemophilia - a study of ten cases. 1078 Nov 92


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