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)

Infection is one of the common causes of death in patients with systemic lupus erythematosus (SLE). It is associated with the use of immunosuppressive agents, renal failure, and increased disease activity. Fournier's gangrene is a necrotizing fasciitis occurring in the genital region. It is rare, but can be crucial if surgical drainage is delayed. We report a female case of Fournier's gangrene occurring in a patient with lupus nephritis and chronic renal failure. The patient was a 21-year-old female with chronic renal failure due to lupus nephritis. She had suffered from watery diarrhea one month before admission. It improved after increasing the dose of prednisolone, but, she was complicated with Bartholin abscess. The vaginal pain rapidly spread to the left lower quadrant abdomen despite treatment with oral cephalosporin. Focal incision was performed and black fluid emerged with a foul smell. Pelvic computed tomography (CT) revealed many bubbles in that region. She was found to have septic shock on transfer to our hospital. Thereafter, emergency debridement was performed, followed by antibiotic therapy and hyperbaric oxygen therapy. Organisms were found to be 5 anerobes, such as Bacteroides species, and 3 aerobics, such as Morganella morganii. Fournier's gangrene was improved via these treatments, but she needed maintenance hemodialysis. Fournier's gangrene complication should be considered in SLE with urogenital infection.
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PMID:[A female case of Fournier's gangrene in a patient with lupus nephritis]. 975 93

Brown tumors (osteoclastomas) are histologically benign lesions that are caused by primary or secondary hyperparathyroidism. Secondary hyperparathyroidism is a frequent complication of chronic renal failure. Skeletal brown tumors are relatively uncommon, and brown tumors that involve the spine are considered very rare. The authors present the case of a 37-year-old woman with systemic lupus erythematosus and hemodialysis-dependent anuric renal failure, in whom spinal cord compression developed due to a brown tumor and pathological fracture at T-9. The patient underwent transthoracic decompressive surgery and spinal reconstruction in which cadaveric femoral allograft and instrumentation were used. Brown tumors of the vertebral column require surgical treatment if medical therapy and parathyroidectomy fail to halt their progression or if acute neurological deterioration occurs. In patients with renal failure bone healing is delayed and there is an increased risk that healing will fail because the metabolic derangements can result in severe osteoporosis. Surgical reconstruction of the spine may require the use of augmentation with instrumentation and aggressive treatment of hyperparathyroidism to achieve successful outcomes.
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PMID:Chronic renal failure causing brown tumors and myelopathy. Case report and review of pathophysiology and treatment. 1019 56

We report a case of well-documented typhoid fever in a 30-year-old woman with inactive systemic lupus erythematosus with asymptomatic lupus anticoagulant and high-titer anticardiolipin antibody (aCL). Despite prompt eradication of the Salmonella typhi obtained with appropriate antibiotic therapy, multiple organ system dysfunction occurred. The central nervous system was involved, with ischemic infarcts in the occipital lobes. High-dose corticosteroid therapy failed to improve the neurologic manifestations, which responded to repeated plasmapheresis. A sharp fall in aCL and anti-beta2-glycoprotein I antibody titers was recorded before the start of plasmapheresis. At the same time, IgM and IgG antibodies to Salmonella group O:9 lipopolysaccharide became detectable; the IgM antibodies disappeared within 4 months, whereas the IgG antibodies remained detectable during the next 13 months. Despite treatment with high-dose corticosteroids and cyclophosphamide, rapidly progressive glomerulonephritis developed, leading to chronic renal failure. There is convincing evidence of a link between the S. typhi infection and the ensuing catastrophic syndrome in this patient, probably precipitated by bacterial antigens.
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PMID:Systemic lupus erythematosus-associated catastrophic antiphospholipid syndrome occurring after typhoid fever: a possible role of Salmonella lipopolysaccharide in the occurrence of diffuse vasculopathy-coagulopathy. 1032 64

We report the case of a 43-year-old woman with systemic lupus erythematosus who survived three episodes of catastrophic antiphospholipid syndrome. During the first episode symptoms involved predominantly the central nervous system, whilst during the second episode of multiorgan failure, the cardiovascular system, lungs and kidneys were particularly affected. Twenty months later, the patient experienced an acute exacerbation of chronic renal failure and later, died of massive pulmonary embolism. The characteristic findings of antiphospholipid syndrome included persistently high titers of IgG anticardiolipin antibodies, positive lupus anticoagulant, and microcytic anaemia with a distinct haemolytic component.
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PMID:[Three episodes of acute multiorgan failure in a woman with secondary antiphospholipid syndrome]. 1040 69

CD5 is a 67 kDa type I glycoprotein which belongs to the Scavenger Receptor Cysteine-Rich (SRCR) family of receptors. This family includes either cell-surface (e.g. CD6) or secreted (e.g. Spalpha) proteins implicated in the development of the immune system and the regulation of immune responses. In this study, we purified and characterised a circulating natural soluble CD5 form (nsCD5) which is indistinguishable (in apparent molecular mass, glycosylation pattern, and antibody reactivity) from a recombinant soluble CD5 form (rsCD5) composed of the three extracellular SCRC domains. The nsCD5 is a N-glycosylated 52 kDa molecule present in normal human serum and in supernatants of in vitro phorbol ester- and CD3-stimulated peripheral blood mononuclear cells. The nsCD5 concentration in sera from healthy donors is relatively low (median 1.75 ng/ml, rn=166) and is similar to that found in sera from patients suffering of various autoimmune (systemic lupus erythematosus, primary Sjogren syndrome, rheumatoid arthritis) and non-autoimmune (chronic renal failure, B-cell chronic lymphocytic leukemia) disorders. In vitro experiments indicate that nsCD5 is released by proteolytic cleavage of the membrane form. These results represent the first evidence of proteolytic release of a transmembrane SRCR family member following cell activation.
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PMID:Identification of a natural soluble form of human CD5. 1048 39

Previous studies have shown that Kampo (traditional Chinese) prescriptions, mainly Wen-Pi-Tang (Onpi-to, [symbol see: text]), have a useful effect in patients with chronic renal failure (CRF). We aimed to examine the long-term effect of Kampo prescriptions on serum creatinine (Cr) among patients with CRF. Patients with serum Cr levels of 2 mg/dl more were enrolled if they had at least 4 recordings of serum Cr in the previous 6 months or more, and were followed-up until the start of dialysis. Eight patients aged 24-59 years with serum Cr 4.5 mg/dl were enrolled in the study for 40 to 402 weeks (mean; 228.1 +/- 118.8 weeks). The cause of CRF was chronic glomerulonephritis in 7 patients and systemic lupus erythematosus in 1 patient. The end points of the study were the slope of the reciprocal of the serum Cr concentration plot against time using Mitch's method, and the predicted period of pre-dialysis. The predicted pre-dialysis period was defined as an increase in serum Cr by 10 mg/dl. As a result, the individual slopes were improved in 6 of 8 cases, in particular, in 4 of 5 Wen-Pi Tang-treated cases. The average slope was improved significantly (p < 0.01) in Wen-Pi-Tang-treated cases, although it showed only a tendency to improve in all 8 cases. The predicted pre-dialysis period was prolonged from 79.2 +/- 74.8 weeks to 389.5 +/- 355.4 weeks and 55.6 +/- 37.0 weeks to 262.4 +/- 145.8 weeks in all 8 cases and Wen-Pi-Tang-treated cases, respectively. The observed pre-dialysis period was 228.1 +/- 118.8 weeks, which showed that Kampo prescriptions prolonged the predicted period for 186 additional weeks. In conclusion, this study demonstrated that the Kampo prescriptions, consisting mainly of Wen-Pi-Tang, retarded the progression of CRF.
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PMID:[Clinical evaluation of kampo medication, mainly with wen-pi-tang, on the progression of chronic renal failure]. 1065 25

Laminin is the main noncollagenous constituent of the basement membrane, and its serum levels could reflect the metabolic changes that occur in the basement membrane. Severe endothelial injury with thickening of basement membrane is a characteristic feature of thrombotic microangiopathy (TMA). With this background, the aim of the study was to investigate in a prospective way (1) the relationship among serum Lam-P1, the extent of renal histopathologic lesions, and the biochemical parameters commonly used as markers of TMA activity, and (2) the usefulness of serum Lam-P1 concentrations as a renal outcome prognostic index. To this end, 18 consecutive patients with active biopsy-proven TMA with renal involvement were studied. One hundred and twenty-one healthy control subjects, 20 patients with systemic scleroderma without renal involvement, and 35 patients with systemic lupus erythematosus (20 without nephropathy and 15 with diffuse proliferative type 4 lupus nephritis) were used as control groups. In addition, to analyze the influence of either renal failure or hemodialysis therapy on serum Lam-P1 levels, 91 patients on regular hemodialysis therapy and 81 patients with predialysis chronic renal failure of different etiologies were included in the study. Serum Lam-P1 was determined by RIA at admission, on days 10 and 30 of follow-up in all patients, and after 6 and 12 mo of follow-up in all surviving patients. Serum lactate dehydrogenase, haptoglobin, platelet count, hemoglobin, and serum creatinine were determined as markers of endothelial dysfunction and hemolysis. At admission, serum levels of Lam-P1 were significantly higher in patients with TMA than in healthy control subjects (3.39 +/- 0.56 U/ml versus 1.40 +/- 0.18 U/ml; P < 0.0001). In addition, patients with TMA had significantly higher serum Lam-P1 levels than the other groups included in the study. At the first control, Lam-P1 correlated with lactate dehydrogenase (P = 0.006) and hemoglobin (P = 0.002). During follow-up, platelet count and hemolysis indicators normalized in all patients, while serum Lam-P1 decreased only in patients with renal function recovery. In multivariate analysis, serum creatinine and Lam-P1 at day 10 were the only independent predictors of renal outcome (r2 = 0.94; P < 0.0001) and also correlated with indices of histopathologic damage (P < 0.001). Serum Lam-P1 normalized in all patients with chronic renal failure in the samples obtained at 6 and 12 mo of regular hemodialysis after solving active TMA, thus suggesting that histopathologic lesions, but not renal function itself, would be mainly responsible for the high Lam-P1 serum concentrations detected in TMA. In conclusion, serum Lam-P1 concentrations are increased in patients with active TMA. Furthermore, patients with poor renal outcome show a prolonged increase of serum Lam-P1 that is related to the extent of renal histologic lesions. Unlike the biochemical markers of hemolysis commonly used to assess TMA activity, the sequential determination of serum Lam-P1 provides valuable information about long-term renal prognosis in patients with TMA.
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PMID:Serum concentrations of laminin-P1 in thrombotic microangiopathy: usefulness as an index of activity and prognostic value. 1070 67

To confirm the significance of excretion of annexin V into the urine and the change of urinary annexin V concentration in kidney disease, a sandwich enzyme-linked immunosorbent assay (ELISA) was developed using two monoclonal antibodies. Urinary annexin V concentration was measured in healthy individuals and patients with kidney and other diseases. Urinary annexin V did not change over a range of pH between 5.0 and 8.0, and was stable during the course of the study for 24 h at room temperature and for 8 days at 4 degrees C. The mean urinary annexin V concentration in 105 normal healthy individuals was 1.5+/-1.5 ng/ml, while that in patients with nephrotic syndrome and systemic lupus erythematosis (SLE) nephritis was 9.3+/-9.1 and 6.6+/-6.7 ng/ml, respectively, and that in IgA nephropathy and chronic renal failure was 2.6+/-2.1 and 1.3+/-0.7 ng/ml, respectively. Annexin level correlated with urinary protein concentration (r=0. 717), but not the serum creatinine concentration, blood urea nitrogen (BUN) and 24-h creatinine clearance. Mean urinary annexin V concentration in patients with ischemic heart disease, hypertension, and diabetes mellitus was 1.4+/-1.0, 1.4+/-1.1, and 1.7+/-1.3 ng/ml, respectively. In one case of relapsing nephrotic syndrome, the urinary annexin V concentration was markedly increased in the early phase after admission and then decreased. This patient later required hemodialysis. These results suggest that a high urinary annexin V concentration may be an indicator of acute renal injury related to the urinary protein level.
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PMID:Measurement of urinary annexin V by ELISA and its significance as a new urinary-marker of kidney disease. 1087 2

We report a case of 52-year-old woman with primary antiphospholipid syndrome who developed mitral insufficiency and chronic renal failure. Continuous ambulatory peritoneal dialysis was started preoperatively due to thrombocytopenia that was aggravated by hemodialysis. Mitral annuloplasty was performed since the mitral valve was not severely damaged. Her postoperative hemodynamics were stable, and anticoagulant therapy was controlled easily. She recovered from severe thrombocytopenia while on continuous ambulatory peritoneal dialysis. Valvular heart disease is a well known feature of primary antiphospholipid syndrome, and there have been several reports about valve replacement in patients who had antiphospholipid syndrome with or without systemic lupus erythematosus. However, valve repair has been reported in only a few such patients. We believe that valve repair is better than valve replacement in patients with antiphospholipid syndrome because of its hypercoagulable tendency. In addition, it seems that continuous ambulatory peritoneal dialysis is a suitable method for the perioperative management of patients with antiphospholipid syndrome who suffer from chronic renal failure as well as thrombocytopenia, and require cardiac surgery under cardiopulmonary bypass.
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PMID:Mitral insufficiency associated with primary antiphospholipid syndrome and chronic renal failure. 1130 57

Fourteen cases of mesangial IgG glomerulonephritis characterized by exclusive or predominant mesangial IgG deposits are reported. The median age at onset was 19 yr (range, 13 to 47 yr). No patient exhibited evidence of systemic lupus erythematous or other systemic diseases. Proteinuria was present in all cases (median, 2.4 g/d; range, 1 to 13 g/d), microscopic hematuria in 12 cases, and macroscopic hematuria in two cases. Five patients were hypertensive at the time of referral. In all cases, renal biopsies revealed mesangial IgG deposits and varying degrees of mesangial matrix expansion, in the absence of significant mesangial cell proliferation. Complement component (mainly C3) deposits were present in virtually all cases. Subepithelial deposits were also noted in nine cases. IgG deposits were polyclonal and consisted mainly of IgG1 and IgG3 subclasses. In electron-microscopic analyses, deposits were electron dense and granular. Treatment was purely supportive. After a mean follow-up period of 11 yr, seven patients had experienced progression to chronic renal failure, including four who had reached end-stage renal failure. Three patients exhibited persistently normal renal function. For one patient, a symptomatic recurrence of mesangial IgG deposits in the renal graft was diagnosed 4 yr after renal transplantation. Such a recurrence highlights the specificity of this type of glomerulonephritis. Mesangial IgG glomerulonephritis is a distinct, albeit rare, type of glomerulonephritis that exhibits far from benign outcome and may recur in renal transplants.
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PMID:Mesangial IgG glomerulonephritis: a distinct type of primary glomerulonephritis. 1180 65


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