Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 17-year-old female with a 5-year history of disseminated lupus erythematosus has remained without immunosuppressive therapy for the last 3 years. She was admitted to the hospital for acute abdominal pain, generalized edema, and rapidly developing dyspnea and somnolence. Although all symptoms were consistent with active SLE, septicemia was suspected because of leukocytosis (20,000/microliters), greatly elevated C-reactive protein (45 mg/dl), and normal complement values (C3 0.74 g/l, C4 0.21 g/l). Directly after bacterial blood cultures were prepared, a combined treatment was instituted consisting of plasmapheresis (3 x 2.1 l against fresh frozen plasma), antibiotics, prednisolone, and cyclophosphamide following the last plasmapheresis. Within three days cerebral function returned to normal, edema improved, and CRP fell to 0.5 mg/dl. The blood cultures and pericardial effusion displayed meningococcal colonies.
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PMID:Successful therapy of meningococcal sepsis in acute disseminated lupus erythematosus with plasmapheresis, immunosuppression, and antibiotics. 223 29

Fifteen patients suffering from severe systemic diseases were treated with monthly pulses administration of cyclophosphamide (0.7 g/m2 of body surface): 8 acute systemic lupus erythematosus, 2 Wegener's granulomatous, 1 polyarteritis nodosa, 1 rheumatoid vasculitis, 1 progressive systemic sclerosis, 1 relapsing uveitis and 1 dermatopolymyositis. The indications for cyclophosphamide were: glomerulonephritis (6 cases), resistance to previous treatments (7 cases) and undesirable side effects of corticosteroid therapy (2 cases). After 3 pulses, the disease was controlled in 12 patients (80%) and corticosteroids could be decreased in all 12 cases without an evolutive relapse of the disease. Five patients developed infections (2 septicemia, 1 zona, 1 herpes gingival stomatitis and 1 viral meningitis) which were treated without sequelae. One patient developed cystitis with hematuria after the 3rd pulse; association of mesna, a urinary tract protective agent, enabled the continuation of treatment without a cystitis relapse. At the end of our retrospective study, the efficacy of pulse cyclophosphamide administration seems to be satisfactory but the risk of undesirable side effects should limit its use to severe systemic diseases or those resistant to conventional therapies.
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PMID:[Treatment of systemic diseases with pulse cyclophosphamide: 15 cases]. 225 84

A patient with inactive systemic lupus erythematosus was successfully treated for pneumococcal sepsis complicated by disseminated intravascular coagulation, shock, renal failure, and functional asplenia. Functional asplenia was diagnosed from the total absence of uptake of intravenously administered 99mtechnetium-labeled sulfur colloid. Ten similar cases of functional asplenia occurring in patients with systemic lupus erythematosus were noted in a review of the literature. Six of these cases, including the current report, were complicated by pneumococcal (5) or salmonella (1) sepsis. The patient presented here had an excellent antibody response to pneumococcal vaccination. Spleen scan abnormalities fully reversed at 1 year. Although functional asplenia is a rare event in systemic lupus erythematosus, it appears to predispose to severe septic complications.
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PMID:Functional asplenia in systemic lupus erythematosus. 228 43

A 50 year old woman presented with pneumococcal septicaemia, septic arthritis, and a lobar pneumonia and was subsequently diagnosed as having systemic lupus erythematosus. The blood film and splenic 99mTc sulphur colloid uptake were normal, although selective functional hyposplenism was shown by the impaired clearance of immunoglobulin coated erythrocytes. Systemic lupus erythematosus presenting with fulminating pneumococcal sepsis in the presence of selective defects in spleen function is previously unreported.
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PMID:Systemic lupus erythematosus presenting as pneumococcal septicaemia and septic arthritis. 232 28

This is a case of a child with neonatal lupus and congenital atrioventricular (AV) block, born to a mother with asymptomatic, systemic lupus erythematosus (SLE). The child, despite pacemaker insertion, died of septicemia and myocarditis at the age of three months. Although the association of neonatal lupus with congenital AV block is well-recognized, there are only few pathologic studies of the conduction system reported in the literature. This is such a study in which we emphasize that, due to an altered immune system in the child, septicemia may be the cause of death in some cases.
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PMID:Neonatal lupus with congenital atrioventricular block and myocarditis. 244 30

Factor B is a centrally important component of the alternative complement pathway. Alternative pathway activation results in factor B cleavage and production of the amino-terminal Ba and the carboxyl-terminal Bb fragments which have molecular weights of approximately 30,000 and 63,000 daltons, respectively. Both Ba and Bb fragments have been reported to express a variety of biological activities in vitro. Thus, binding of Ba and Bb fragments to specific B lymphocyte surface receptors modulates proliferation of prestimulated B cells. In addition, the enzymatically active Bb fragment induces activation and spreading of human and murine macrophages and monocytes as well as regulates C5a des Arg chemotactic activity. The fractional catabolic rate and metabolism of factor B in vivo is similar to that of C3, C4 and C5 complement proteins, which are among the most metabolically active plasma proteins in the circulatory system. Factor B hyperconsumption and increased catabolism, concomitant with factor B fragment production, occurs in a wide variety of diseases, including gram-negative sepsis, autoimmune diseases and burns. Measurement of alternative pathway activation in vivo has been attempted utilized a number of different techniques to quantitate factor B fragments in biological fluids. However, the recent development of enzyme immunoassays (EIA) employing monoclonal antibodies (MoAbs) reactive with factor B fragment neoepitopes provides the best approach currently available for the quantitation of factor B activation fragments. Results obtained using these new MoAb-based EIAs have indicated that factor B fragment concentrations were elevated, as compared with normal donor levels, in EDTA plasma samples obtained from patients with rheumatoid arthritis and systemic lupus erythematosus (SLE). Plasma concentrations of factor B fragments, especially Ba fragment levels, in these patients showed a positive correlation with disease activity scores. One of the highest disease activity correlations was obtained with Ba fragment measurements in SLE plasma samples. In fact, the results strongly suggested that quantitation of Ba fragment levels in SLE plasma samples more accurately reflected disease activity and was a more sensitive predictor of impending flare in these patients than any other test(s) currently available.
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PMID:Ba and Bb fragments of factor B activation: fragment production, biological activities, neoepitope expression and quantitation in clinical samples. 247 21

We have reported on the clinical courses of 4 cases of adult Listeria monocytogenes (Lm) infection, and the autopsy findings of 2 cases, those we have observed over the past 5 years. They were 2 cases of meningitis, 1 case of meningitis and sepsis and 1 case of sepsis. These 4 cases had CML, neoplastic angioendotheliosis, SLE and post-renal transplant condition, as their underlying diseases, and all were receiving immunosuppressive therapy. One meningitis patient who recovered showed mild liver dysfunction during her clinical course. The other 3 patients who died had jaundice at the time of onset and severe liver dysfunction. The 2 cases those were autopsied were the sepsis cases. The one with an acute course and hepatic failure showed multiple miliary necrotic foci in the liver, where the presence of Lm in the cells could be verified. The other autopsy case, which had received adequate antibiotic therapy and the Lm infection had been cured, showed no necrotic foci in the liver. The case that had necrotic foci in the liver was the first such adult case in Japan. We have discussed the hepatic Lm infection in adult compromised hosts, which conventionally has not been considered a serious problem.
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PMID:[Four cases of adult Listeria monocytogenes infection in the last 5 years--hepatic necrotic foci in the adult septic case]. 250 7

Systemic lupus erythematosus (SLE) is one of the most common autoimmune diseases and patients suffering from this disease often died of massive hemorrhage. We report the case of a patient who died of acute massive hemorrhage three weeks after mitral valve replacement. The patient, a 42 year-old woman, had been diagnosed as having valvular heart disease at the age of 10. She underwent mitral commissurotomy at the ages of 18 and 32. SLE was diagnosed 8 years previously and corticosteroid therapy was initiated. The patient was experiencing exertional dyspnea again 1 year ago and mitral valve replacement was performed for recurrent stenosis. The postoperative course seemed to be uneventful, but on the 21st postoperative day, acute massive mediastinal hemorrhage occurred, and the patient eventually died of septicemia. Massive hemorrhage in SLE patients usually occur in the central nervous system or alimentary tract. However, bleeding can occur anywhere, so great care must be taken in regulating anticoagulant therapy.
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PMID:[Acute massive mediastinal hemorrhage three weeks after mitral valve replacement in a patient with systemic lupus erythematosus]. 261 20

The possible infections of 43 hospitalized patients with systemic lupus erythematosus (SLE) has been assessed. Out of the 43 patients 17 had some type of infection (41%). The diagnosis was proven by positive cultures in 84% of the cases. Urinary infections due to E. coli were the infections most commonly found. Four infections due to staphylococcus, 2 to S. typhi and 2 to invasive Aspergillosis were also found. Tuberculosis was observed only in 2 patients. In twelve out of the 17 patients with infections, SLE was active and 10 of the latter had renal failure. In addition to the 18 urinary infections diagnosed, sepsis was encountered in 5 patients and pneumonias in 4. Only two infected patients died (those with invasive aspergillosis). Thus, infections are frequent in patients with SLE and precise diagnosis often requires positive culture test.
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PMID:[Infection in systemic lupus erythematosus]. 277 94

A poor prognosis was observed in patients who had end-stage renal disease (ESRD) as a result of systemic lupus erythematosus (SLE). This was true even in patients in whom SLE disease activity was transiently quiescent during the period of hemodialysis. Six of 9 patients with ESRD and SLE died with active SLE and/or sepsis 1-28 months following the onset of dialysis. In 5 of the 6 patients, acute inflammatory activity of SLE flared within 1 month of the patient's death. Four patients died with superimposed sepsis, but only 2 of the 4 were receiving high-dose concomitant immunosuppressives for more than 1 week prior to death. Infected hemodialysis vascular access sites were implicated as the source of septicemia in 3 of 4 infectious deaths. The 3 surviving patients had minimal lupus activity prior to the development of ESRD, a possible marker for stability in SLE patients who require hemodialysis. Our results suggest that hemodialyzed lupus patients with nonautologous vascular access sites may be at continued increased risk for life-threatening inflammatory and septic complications.
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PMID:Poor prognosis in end-stage lupus nephritis due to nonautologous vascular access site associated septicemia and lupus flares. 281 17


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