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)

Lupus anticoagulant, concentrations of anticardiolipin antibodies, antithrombin III, plasminogen, (free) protein S, protein C, prothrombin, platelet counts, and bleeding times were determined in 74 lupus patients (58 with systemic lupus erythematosus; 16 with lupus-like disease) to establish the presence of risk factors for thrombosis in these patients. Of the variables evaluated, lupus anticoagulant had the strongest association with a history of thrombosis. Both positive anticardiolipin antibody concentrations and the presence of (mild) thrombocytopenia were significantly associated with a history of thrombosis and the presence of lupus anticoagulant. Reduced concentrations of antithrombin III, plasminogen, (free) protein S, and protein C were found in some patients but were not associated with either thrombosis or lupus anticoagulant. Mean concentrations of total protein S were significantly lower in patients with thrombosis than in those without and in patients with lupus anticoagulant than in those without. The antigenic concentration of prothrombin was reduced in 3/74 (4%) lupus patients. These three patients had lupus anticoagulant but no history of thrombosis, which suggests that a low prothrombin concentration protects patients with lupus anticoagulant from the development of thrombosis. A prolonged bleeding time was associated with the presence of lupus anticoagulant but not with a history of thrombosis. Analysis by stepwise logistic regression did not disclose additional risk factors for thrombosis in lupus patients with lupus anticoagulant. Increased antithrombin III concentrations and decreased free protein S concentrations are often found in lupus patients, unrelated to lupus anticoagulant or thrombosis.
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PMID:Risk factors for thrombosis in lupus patients. 251 63

This study prospectively evaluates hypercoagulable states in patients under 51 years of age undergoing lower extremity revascularization for ischemia and assesses early outcome after operation. Twenty patients whose ages range from 23 to 50 years (mean 40.8 years) were identified prospectively who underwent lower extremity revascularization and evaluation of hypercoagulability. Fifteen patients were male (75%), 10 were black (50%), six had hypertension (30%), and four were diabetic (20%). All but two were cigarette smokers (90%). Seven aortoiliac procedures and 13 infrainguinal procedures were performed. Six patients had one or more abnormalities of regulatory proteins (protein S deficiency, four; protein C deficiency, three; presence of lupus-like anticoagulant, three; plasminogen deficiency, two). Eight of 17 patients in whom platelet aggregation profiles were obtained showed increased reactivity (47%). Only 4 of 17 patients (24%) were normal when tested for all parameters. Arterial or graft thrombosis developed in four of the 20 patients within 30 days after operation. Hypercoagulability was found in all four patients whose revascularizations failed. A high incidence of hypercoagulable states was found in patients under 51 years of age with lower limb ischemia requiring revascularization. Hypercoagulability may have contributed to early postoperative thrombosis of the vascular procedure.
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PMID:Hypercoagulable states and lower limb ischemia in young adults. 252 8

Since most patients with thrombophilia in Israel are referred for diagnosis to our center, it was possible to estimate the relative frequency of the hereditary disorders leading to thrombophilia. 107 unrelated patients were evaluated over 4 years. Diagnoses were established in 23 patients (21.5%) while in 84 (78.5%) no abnormality was detected. Antithrombin III deficiency was found in 8 patients (7.5%), dominant protein C deficiency in 6 (5.6%), recessive homozygous protein C deficiency in 1, protein S deficiency in 3 (2.8%) and dysfibrinogenemia in 1. Four additional patients (3.7%) had a lupus anticoagulant. The frequency of deep vein thrombosis and pulmonary embolism was similar in patients with and without a definite diagnosis. Thrombosis of visceral or cerebral vessels and a positive family history were more frequent among patients in whom a definite diagnosis was made. In both groups there was a substantial lag between the time of presentation of the first thrombotic episode and the time of evaluation. Since the number of referred patients with thrombophilia has gradually increased over the period of the study, it is at present impossible to establish the prevalence of the various hereditary disorders leading to thrombophilia in the population.
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PMID:The relative frequency of hereditary thrombotic disorders among 107 patients with thrombophilia in Israel. 252 86

Protein C (PC), a 62,000-molecular weight vitamin K-dependent serine protease zymogen, is a natural anticoagulant that occurs in plasma at 4 mg/L. Activated PC inactivates clotting factors V and VIII and is also profibrinolytic. Activated PC is enhanced in its anticoagulant activity by protein S (PS), another vitamin K-dependent protein. Protein S is found in platelets and endothelial cells as well as in plasma. Inherited PC deficiency and PS deficiency have been associated with venous thrombosis. Both heterozygous PC and PS deficiency appear to be inherited in an autosomal dominant manner in some families. Homozygous PC deficiency presents as neonatal purpura fulminans and results in massive venous thrombosis of the skin and other organs within the first few days of life. Symptomatic heterozygous PC deficiency and PS deficiency have been treated with oral anticoagulants, successfully minimizing recurrence of thrombosis. Coumarin-induced skin necrosis, a rare complication of oral anticoagulant therapy usually seen within three to five days of initiation of therapy, has also been associated with heterozygous PC deficiency. The short half-life of PC (six to eight hours) compared with most of the vitamin K-dependent clotting factors (greater than 30 hours) is the probable reason for this paradoxical response to oral anticoagulants in some PC-deficient patients, since a transient imbalance of procoagulant and anticoagulant factors may exist during initiation of oral anticoagulant therapy. Acquired deficiency of the PC pathway occurs in disseminated intravascular coagulation and possibly other diseases such as those associated with a lupus anticoagulant.
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PMID:Coumarin necrosis, neonatal purpura fulminans, and protein C deficiency. 296 8

Plasma samples from nineteen patients with well characterized lupus anticoagulants (LA) were evaluated using a series of test systems. An ELISA was used to determine if the plasmas contained antiphospholipid antibodies (APA); fifteen of nineteen LA plasmas contained APA, with five exhibiting IgG only, two exhibiting IgM, and eight plasmas containing both IgG and IgM. Anti-phosphatidyl serine (PS) was the predominant IgG specificity and all IgM APA-containing plasmas reacted with phosphatidyl inositol (PI). An ELISA was developed to determine if LA plasmas contained immunoglobulin which would associate with cultured human umbilical cord vein-derived endothelial cells (HUV); ten of nineteen plasmas contained endothelium associated immunoglobulin (EAI). There was significant concordance between the occurrence of EAI and IgM anti-PI. The occurrence of EAI or APA, either singly or in combination, did not correlate with a past history of thrombosis. Patient plasmas were incubated with HUV and examined for effects on HUV prostacyclin (PGI2) secretion; six plasmas significantly stimulated PGI2 secretion and one plasma was inhibitory. Finally, plasma levels of free and total antigenic protein S were determined by EID. Five plasmas contained significantly reduced levels of free antigenic protein S, and total antigenic protein S was reduced in ten plasmas. Patient histories were examined for evidence of thrombotic episodes; six patients had a history of either arterial or venous thrombosis, with five of these six patients having drug-induced LA. Thus, unlike previous studies, drug-induced LA were associated with thrombosis.
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PMID:Evaluation of lupus anticoagulants: antiphospholipid antibodies, endothelium associated immunoglobulin, endothelial prostacyclin secretion, and antigenic protein S levels. 297 88

Protein C (PC) is a vitamin K-dependent protein which functions as a physiologic anticoagulant. In the presence of another vitamin K-dependent protein, protein S, the activated form of protein C (APC) will degrade the active cofactors Va and VIIIa. Both hereditary and acquired deficiencies of PC have been associated with a predisposition to thromboembolic events. We have evaluated a commercial assay system (Stac lot Protein C) which utilizes an extract of snake venom (Protac) that directly activates protein C in vitro. Utilizing this assay, normal individuals, patients with hereditary protein C deficiency, patients who were stably anticoagulated with oral anticoagulants, and patients with lupus anticoagulants were evaluated. Significant discrepancies were noted between protein C antigen and protein C functional activity in patients receiving oral anticoagulants. In addition, patients with lupus anticoagulants may have falsely elevated values for functional protein C activity.
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PMID:Clinical application of a functional assay for protein C. 314 9

Some molecular defects of components of the coagulation or fibrinolytic system are associated with thromboembolism. One possibility is that physiologic inhibitors of the coagulation system have an abnormal function e.g. protein C, protein S, antithrombin III and cofactor II of heparin. Also a hindered activation of the fibrinolytic system may predispose to thrombosis; the impaired activation may be due to deficient synthesis and/or release of tissue-plasminogen activator, an increased level of its inhibitor or a functional defect of the plasminogen molecule. A few cases of congenital dysfibrinogenemia have been described in which the functional defects of the molecule are held responsible for recurrent thrombosis. An acquired thrombotic disorder is due to the presence of immunoglobulins which prolongs phospholipid-dependent coagulation by binding to epitopes of some phospholipids. This so-called lupus anticoagulant was originally described in patients with systemic lupus erythematosus but is a misnomer as it is more frequently encountered in patients without lupus.
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PMID:[Molecular defects of coagulation factors and of the fibrinolytic system associated with thromboembolism]. 354 55

Free protein S:Ag and protein S activity determined by clotting assay are often regarded as synonymous; however, in the study group of patients, abnormal protein S activity (PS:Act) results correlated poorly with abnormal free (PS:Ag(r2 = 0.164). Significantly none of the patients in whom lupus anticoagulant and low free protein S:Ag were detected were found to have low PS:Act. These results could not be explained by activated protein C resistance, suggesting that lupus anticoagulant may cause prolongation of the clotting time within the PS:Act assay and thus give falsely high results. As reduced levels of free protein S:Ag and/or protein S activity are considered risk factors of thrombosis in systemic lupus erythematosus, these findings question the suitability of the protein S activity assay for patients who may have phospholipid antibodies.
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PMID:Assay of protein S in systemic lupus erythematosus. 754 80

The present study describes the epidemiological profile and clinical features of Takayasu's arteritis (TA) in Kuwait, as well as its association with other autoimmune diseases and primary hypercoagulable states. Thirteen patients were included from its start on 1 January 1989 till 30 June 1994. Diagnosis of TA was established by angiographic studies. Twelve patients were Arabs and 7 were Kuwaiti nationals. Five patients were males and renal disease secondary to isolated involvement of the abdominal aorta (TA, type II) was the main presentation in 4 patients. Coagulation tests were performed in 7 patients and included antiphospholipid antibody (aPL) assay as well as protein S, protein C and antithrombin III activity. Only one manifested recurrent thrombosis and laboratory tests confirmed the presence of a hypercoagulable state secondary to aPL and protein S deficiency. Serological tests of systemic lupus erythematosis (SLE) were positive in this patient. These data indicate that TA is not a rare disease in the Arabic population. In our study, female predominance was not a common feature of TA and renal disease secondary to TA type II disease was commonly encountered. The association of TA with SLE and primary hypercoagulable states was not a consistent finding in our patients with TA, and hence, the proposed role for thrombotic vasculopathy in the pathogenesis and progression of this disease was unfounded.
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PMID:Takayasu's arteritis in Kuwait. 756 55

A 16 year old girl with systemic lupus erythematosus (SLE) developed the rare complication of central retinal vein occlusion. Although classically a disease of older patients, it has been recognised in association with SLE but only in the presence of the lupus anticoagulant or antiphospholipid antibodies. The thrombosis occurred when free protein S concentrations were transiently reduced and there was no family history or other known causes of reduced protein S concentrations. No other prothrombotic risk factors were present.
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PMID:Acquired protein S deficiency in a patient with systemic lupus erythematosus causing central retinal vein thrombosis. 761 65


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