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)

Vibrio vulnificus infection with septicemia is a life threatening disease in the immunocompromised hosts. Renal involvement has not been documented. We reported herein 8 patients with V. vulnificus septicemia. All were immunocompromised hosts. Four patients had cirrhosis of the liver, 3 were heavy alcohol drinkers and one had systemic lupus erythematosis. Presenting symptomatology included fever, chills, leg pain and skin rash. Renal failure was observed in 6 patients. Four patients died shortly after admission. Two survived with clinical course of tubular necrosis. Renal failure is therefore common in V. vulnificus infection. This should be brought to attention, and vigorous antibiotic treatment is required. The disease may be confused with leptospirosis, scrub typhus, malaria and other forms of sepsis which also present with renal failure.
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PMID:Renal failure in vibrio vulnificus infection. 1084 44

A registry of United States residents with chronic granulomatous disease (CGD) was established in 1993 in order to estimate the minimum incidence of this uncommon primary immunodeficiency disease and characterize its epidemiologic and clinical features. To date, 368 patients have been registered; 259 have the X-linked recessive form of CGD, 81 have 1 of the autosomal recessive forms, and in 28 the mode of inheritance is unknown. The minimum estimate of birth rate is between 1/200,000 and 1/250,000 live births for the period 1980-1989. Pneumonia was the most prevalent infection (79% of patients; Aspergillus most prevalent cause), followed by suppurative adenitis (53% of patients; Staphylococcus most prevalent cause), subcutaneous abscess (42% of patients; Staphylococcus most prevalent cause), liver abscess (27% of patients; Staphylococcus most prevalent cause), osteomyelitis (25% of patients; Serratia most prevalent cause), and sepsis (18% of patients; Salmonella most prevalent cause). Fifteen percent of patients had gastric outlet obstruction, 10% urinary tract obstruction, and 17% colitis/enteritis. Ten percent of X-linked recessive kindreds and 3% of autosomal recessive kindreds had family members with lupus. Eighteen percent of patients either were deceased when registered or died after being registered. The most common causes of death were pneumonia and/or sepsis due to Aspergillus (23 patients) or Burkholderia cepacia (12 patients). Patients with the X-linked recessive form of the disease appear to have a more serious clinical phenotype than patients with the autosomal recessive forms of the disease, based on the fact that they are diagnosed significantly earlier (mean, 3.01 years of age versus 7.81 years of age, respectively), have a significantly higher prevalence of perirectal abscess (17% versus 7%), suppurative adenitis (59% versus 32%), bacteremia/fungemia (21% versus 10%), gastric obstruction (19% versus 5%), and urinary tract obstruction (11% versus 3%), and a higher mortality (21.2% versus 8.6%).
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PMID:Chronic granulomatous disease. Report on a national registry of 368 patients. 1084 35

We analyzed the clinical and laboratory features, treatment, and course of twenty-one children with systemic-onset juvenile rheumatoid arthritis (S-JRA) encountered at our institution over the past ten years. There were eleven boys and ten girls. The mean age at onset was 11.6 +/- 4.2 years. The mean duration of symptoms prior to diagnosis was 5.5 +/- 1.7 months, and the mean follow-up period was 45.7 +/- 9.5 months. The clinical and laboratory features at presentation were similar to previous reports, except that peripheral blood smear revealed toxic granulation of neutrophils in 60% of our patients. Although systemic manifestation could be readily controlled by non-steroidal anti-inflammatory drugs (NSAIDs) with or without additional steroids, nine patients suffered from chronic arthritis (duration > 6 months) requiring disease-modifying anti-rheumatic drugs (DMARDs). Of the nine children with chronic arthritis, six (67%) had a monocylic systemic course, and seven (78%) had polyarticular disease (five or more joints affected) at the disease onset. Five patients developed severe destructive polyarthritis, with persistent anemia, thrombocytosis, elevated serum C-reactive protein (CRP) levels, and marked functional limitation during follow-up. One of the five patients with severe arthritis developed systemic lupus erythromatosis after 8-year follow-up, and died of sepsis. Our study indicated significant morbidity in children with S-JRA in Taiwan.
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PMID:A follow-up study of systemic-onset juvenile rheumatoid arthritis in children. 1091 Jun 10

The objectives were to determine causes of consultation, hospitalization and outcome in a cohort of lupus patients in an emergency unit. Patients with systemic lupus erythematosus (SLE) who visited the emergency department for consultation from 1 September 1996 to 17 May 1997 were included in the study. They were evaluated during the visit by looking at 100 variables such as demographic, socioeconomic, clinical, therapeutical, behavioral, (compliance), emotional (Beck depression inventory), disease activity, (Mex-SLEDAI), disease severity (Lupus SDI), chronic damage (SLICC-ACR), and physician's and patient's global assessments of severity. All causes of consultation, hospitalization and outcome were registered. Descriptive statistics, univariate analysis and multiple logistic regression were used for analysis. Significance was set at the 0.05 level. 180 patients were included. 164 were female, mean age 31.7/11.39 y, mean Mex SLEDAI score 3.8, mean SLICC-ACR 1.3. Fever, poliarthralgia and abdominal pain were the main causes of consultation with 26, 25 and 18 cases each. 49 patients were hospitalized and these were statistically different than non-hospitalized patients in level of formal education (10.2 vs 11.8, P=0.03); compliance (7.6 vs 9, P=0.0001); malar rash (57% vs 82%, OR, 95% CI=0.28, 0.13-0.62, P=0.0008), chloroquine daily dose intake (45 vs 77 mg, P=0.04); disease severity in physician's global assessments (5.6 vs 2.1, P=0.0001) and Beck depression inventory (21 vs 16, P=0.01). Multiple logistic regression identified physician's global assessment, fewer ACR criteria and higher SLICC-ACR scores as the main variables associated with hospitalization. Five patients died; two with community acquired pneumonia, one with pancreatitis, multiple thromboses, and sepsis, one with pulmonary hemorrhage; and one with pulmonary thromboembolism. In conclusion, poor compliance, low level of formal education, severity, depression, lower ACR criteria and higher SLICC-ACR scores were important variables identified with hospitalization. Chloroquine use seemed to have a protective effect. Causes of death were related to infections and antiphospholipid syndrome.
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PMID:Lupus patients in an emergency unit. Causes of consultation, hospitalization and outcome. A cohort study. 1103 35

A 23-year-old female patient suffering from active systemic lupus erythematosus (SLE) was treated with azathioprine (2 mg/kg per day) and prednisone. Lupus nephritis class III with increasing proteinuria developed 28 months after disease onset. Treatment was switched to monthly pulse cyclophosphamide administered intravenously for 6 months (total dose 6.3 g), followed by oral azathioprine and low-dose prednisone to maintain partial remission. Eight months later, the patient developed an acute exacerbation of SLE with fever, proteinuria of 9.1 g/day, pancytopenia, and cerebral involvement with cephalgias and a grand mal seizure. She responded well to high-dose corticosteroids (500 mg prednisolone pulses over 3 days, i.v.) and was azathioprine switched from to methotrexate (12.5-15 mg per week). Under this treatment, lupus activity gradually decreased and the patient felt well again. Five years after the initial diagnosis of SLE, a rapidly increasing immunoglobulin G-kappa type (IgG-kappa) monoclonal gammopathy developed, reaching a maximal serum paraprotein concentration of 73.5 g/l. Bone marrow biopsy revealed 15% of moderately abnormal, highly differentiated plasma cells arranged in small clusters and expressing IgG-kappa. No bony lesions were detectable on skeletal radiographs. Pulses of dexamethasone (40 mg) were administered and led to a transient decrease of paraproteinemia to a minimum of 31.9 g/l, followed by an increase to 62 g/l. At that point, high-dose chemotherapy supported by autologous stem cell transplantation was considered. Due to an intermittent pneumococcal septicemia, methotrexate was discontinued and dexamethasone was replaced by 5-10 mg cloprednol. At this point, totally unexpectedly, the paraprotein decreased spontaneously without any further cytostatic treatment and was no longer detectable 1 year later. Concomitantly, plasma cell counts in bone marrow biopsies fell to below 5%. As SLE remained inactive, the patient became pregnant and gave birth to a healthy child. During late pregnancy, SLE activity flared up with rising proteinuria and blood pressure. Therefore, after delivery, cyclophosphamide (100 mg/day, orally) was readministered for 4 months, resulting in an improvement of kidney function with stable proteinuria of 1-2 g/l to date. Paraproteins are no longer detectable. In conclusion, this case report documents the rare event of transient paraproteinemia in a patient with SLE. A self-limiting regulatory defect in the control of a terminally differentiated B-cell clone may be the origin of this phenomenon.
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PMID:An unusual case of systemic lupus erythematosus, lupus nephritis, and transient monoclonal gammopathy. 1106 95

The objective of this study was to characterize the clinical profile of lupus patients with non-typhoidal salmonellosis. A retrospective review of the clinical charts of lupus patients diagnosed with bacteriologically proven non-typhoidal salmonellosis over the last 20 y was undertaken, paying special attention to risk factors, clinical presentation and treatment outcome. Most episodes were bacteraemic without a localizing focus; and some patients were afebrile. They usually occurred in patients prone to opportunistic infections, and at times of increased immunosuppression given for lupus flares (especially nephritis). However, salmonellosis also occurred in some patients presenting with lupus. The C-reactive protein level was found to be significantly higher during the infective episodes compared to episodes of non-infective febrile lupus flare. All isolates were sensitive to the usual first-line antibiotics and eminently treatable with 3 weeks of appropriate antibiotics without recurrence/persistence or significant morbidity/mortality, the exceptions being spinal osteomyelitis and septic arthritis involving deformed joints requiring surgical debridement and prolonged antibiotic therapy for eradication. Mortality occurred in the setting of septic shock from mixed-microbial sepsis and major organ failure from active lupus. There is a high association of non-epidemic, non-typhoidal salmonellosis with SLE, especially in patients with active disease on intensified immunosuppression. The C-reactive protein value may be helpful in distinguishing between fever from a pure lupus flare and one complicated by infection.
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PMID:Non-thyphoidal salmonellosis in patients with systemic lupus erythematosus. A study of fifty patients and a review of the literature. 1123 31

The association of systemic lupus erythematosus (SLE) and B-cell malignancy is widely reported in the literature. Here we report nine cases of concurrent of SLE or discoid lupus erythematosus (DLE) and lymphoma or plasma cell disorder. A MEDLINE search was done using the keywords, 'SLE' and 'lymphoma' and the characteristics of all identified cases were summarized and analyzed, along with data from our own cases. Numerous variants of B-cell malignancies were encountered in these patients. B-cell malignancy occurs after the diagnosis and treatment of SLE in most reported cases, although it may precede SLE, or occur synchronously with it. The age at onset of the B-cell neoplasm in SLE patients is similar to that in the general population. Mortality in patients with both diseases is associated with progressive B-cell neoplasm, sepsis secondary to either disease, or both. B-cell malignancy and SLE seem to run independent clinical courses rather than being affected by each other. The use of immunosuppressive drugs is common in patients with SLE diagnosed prior to B-cell lymphoma, arguing that the effect of immunosuppression on the pathogenesis of lymphoma can not be excluded. Three areas worthy of study regarding the probable mechanisms for the occurrence of SLE and B-cell malignancies are discussed. A tumor suppressor gene PTEN may link the two disorders via a defective apoptosis pathway to eliminate hyperactive B and T cells in SLE. The accumulation of clonally expanded hyperactive B-cells that recognize self-antigens in the lymph nodes of SLE may predispose these B-cells to DNA breaks, facilitating tumorigenesis. Lastly, EBV infection, found to have a high prevalence in SLE patients, may serve as a common etiological factor in both disorders.
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PMID:Systemic lupus erythematosus and B-cell hematologic neoplasm. 1178 71

We describe the case of a young woman with a known history of thrombocytopenia, who developed respiratory and circulatory failure thought to be caused by sepsis. She subsequently was shown to have high titres of anticardiolipin antibodies as well as lupus anticoagulant. Serological tests for various connective tissue diseases were negative. Multiple cerebral ischaemic lesions led to a poor outcome. This case highlights the potential difficulties in differentiating catastrophic anti-phospholipid syndrome from disseminated intravascular coagulation.
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PMID:Multi-organ failure secondary to catastrophic anti-phospholipid syndrome. 1193 48

Infections are common in systemic lupus erythematosus (SLE), and remain a source of mortality. The types of infections (such as pneumonia, urinary tract infection, cellulitis, and sepsis) in SLE patients are similar to the general population and include the same pathogens (Gram-positive and Gram-negative). SLE patients may also develop opportunistic infections, especially when treated with immunosuppressive agents. As a high-risk population, identification and treatment of chronic infections such as tuberculosis, hepatitis B, or human immunodeficiency virus (HIV), are important prior to the institution of immunosuppression to prevent reactivation or exacerbation of the infection. A common caveat is to distinguish between a lupus flare and an acute infection; judicious use of corticosteroids and cytotoxic drugs is critical in limiting infectious complications. The risk factors associated with susceptibility to disease include severe flares, active renal disease, treatment with moderate or high doses of corticosteroids and/or immunosuppressive agents, and others. Genetic factors (complement deficiencies, mannose-binding lectin, Fcgamma III, granulocyte macrophage colony-stimulating factor [GM-CSF], osteopontin) may predispose certain SLE patients to develop infections. Parameters including C-reactive protein (CRP) and adhesion molecules may help to differentiate an infectious disease from an exacerbation of the disease. Finally, the mechanism of molecular mimicry by specific microbial agents may play a role in the induction of SLE.
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PMID:SLE and infections. 1279 59

Roles for Toll-like receptors (TLRs) are emerging in conditions such as sepsis syndrome, systemic lupus erythromatosis, rheumatoid arthritis and asthma, suggesting that the selective targeting of TLRs might be useful therapeutically. TLRs are defined by the presence of extracellular leucine-rich repeats and an intracellular Toll/interleukin-1 receptor domain, and play a role in host defence and inflammation. Signalling pathways activated by TLRs show remarkable similarity to those activated by the pro-inflammatory cytokine interleukin-1 (the receptor for which also has a Toll/interleukin-1 receptor domain), although adaptor proteins specific for certain TLRs are starting to emerge (e.g. Mal and Trif). The common signalling pathways used by all members of the TLR superfamily are being targeted, with drugs that block nuclear factor-kappaB and p38 mitogen-activated protein kinase in clinical development for diseases such as rheumatoid arthritis and psoriasis. As we learn more about TLR signal transduction, more options are presenting themselves for pharmacological targeting.
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PMID:Therapeutic targeting of Toll-like receptors for inflammatory and infectious diseases. 1290 49


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