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)

The clinical features of eight patients, four females, aged 4 to 15 years under chronic hemodialysis for terminal renal failure (creatinine clearance 10 ml.min.1,73 m2 or less) are reported. Initial diseases were Alport syndrome, systemic lupus erythematosus, chronic glomerulonephritis (n = 2), bilateral polycystic kidney, prune belly syndrome and reflux nephropathy (n = 2). Distal vascular approach by means of arteriovenous fistulas was preferred for these patients and the kinetic urea model was used to evaluate the performance of the procedure. Patients required nine to twelve hours of hemodialysis per week for optimal results. Mean weight decreases of 1 to 3 kg and reductions in blood urea nitrogen and serum potassium of 40 mg.dl and 2,5 mEq.1, respectively, were observed. The main complications of hemodialysis were the disequilibrium syndrome, infections at the site of insertion of the arteriovenous fistulae and congestive heart failure. Three patients were submitted to renal transplantation with live donors homografts: one died and the other two remain alive but under chronic hemodialysis. Five children are attending school regularly, and two of them are waiting a kidney donor for transplantation. Despite encouraging results chronic hemodialysis in children constitutes only primary supportive therapy prior to renal transplantation.
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PMID:[Chronic hemodialysis in children]. 208 91

The addition of urea to sodium dodecyl sulfate (SDS)-polyacrylamide gels has allowed the identification and characterization of the small nuclear ribonucleoprotein particle (snRNP) D' protein and has also improved resolution of the E, F, and G snRNP core proteins. In standard SDS-polyacrylamide gels, the D' and D snRNP core proteins comigrate at approximately 16 kilodaltons. The addition of urea to the separating gel caused the D' protein to shift to a slower electrophoretic mobility that is distinct from that of the D protein. The shift to a slower electrophoretic mobility in the presence of urea suggests that the D' protein has extensive secondary structure that is not totally disrupted by SDS alone. Both N-terminal sequencing and partial peptide maps indicate that the D and D' proteins are distinct gene products, and the sequence data have identified the faster moving of the two proteins as the previously cloned D protein (L. A. Rokeach, J. A. Haselby, and S. O. Hoch, Proc. Natl. Acad. Sci. USA 85:4832-4836, 1988). In the cytoplasm, the D protein is found primarily in the small-nuclear-RNA-free 6S protein complexes, while the D' protein is found primarily in the 20S protein complexes. Like the D protein, the D' protein is an autoantigen in patients with systemic lupus erythematosus and is recognized by some of the Sm class of autoimmune antisera.
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PMID:Identification and characterization of the small nuclear ribonucleoprotein particle D' core protein. 214 5

The present study was designed to evaluate the therapeutic activity of a novel immunosuppressive agent, deoxyspergualin (DSG, NKT-01) in male MRL/MpJ-lpr/lpr (MRL/lpr) mice suffering advanced systemic lupus erythematosus (SLE)-like lesions. Treatment with DSG in the early phase of the disease at doses of 1.5 and 3 mg/kg strongly suppressed the development of SLE-like lesions. When DSG was administered from week 21 through 29 to MRL/lpr mice in advanced phases of the disease, a daily iv dose of 3 mg/kg (5 days/week) markedly reduced the symptoms, whereas a dose of 1.5 mg/kg did not. Moreover, DSG treatment at a dose of 3mg/kg, started at the time when the blood urea nitrogen levels were over 50 mg/deciliter, significantly prevented deterioration of the hyperuremia. Taking these findings into consideration, DSG was found to be a promising agent for curing such established autoimmune disease.
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PMID:Deoxyspergualin therapy in autoimmune MRL/1pr mice suffering advanced lupus-like disease. 227 76

Activated charcoal with various coating materials were screened and tested as adsorbents in hemoperfusion. Among them silicon rubber polyurethane, crosslinked agrose, polyvinyl acetate, polyhydroxy ethyl methacrylate showed good blood compatible properties. Various types of resins and carbonized resins with different functional groups, and structures were tested for the adsorption of small molecules i.e. creatinine, barbiturates, middle molecules i.e. VitB12, inulin, cytochrome C and large molecules i.e. unconjugated bilirubin. High adsorption capacities were obtained and uncoated carbonized resins showed satisfactory blood compatible properties. NK-107 a macroporous, non-coated resin is now being manufactured and successfully used clinically on patients for acute detoxification of hypnotic drugs. Polysaccharide dialdehyde and polystyrene derivatives etc. were used for the removal of urea, ammonia, potassium and phosphorus. DNA immune adsorbents were prepared for systemic lupus erythematosus therapy.
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PMID:Advances of adsorbents for hemoperfusion in China. 228 12

IEF, using 6 M urea, provides a unique opportunity to analyze the spectrotypes of antibodies in immune complexes (IC) in vivo. Using this technique, we have analyzed the clonotypes of anti-DNA antibodies expressing specific Id in the circulating IC of patients with active lupus nephritis. Serum anti-ssDNA and anti-dsDNA antibodies showed heterogeneous spectrotypes. The antibodies isolated from circulating IC had a restricted clonotype and a neutral charge and were directed mainly to ssDNA and, to a lesser extent, to dsDNA. These samples failed to form complexes with DNA when they were subjected to absorption to a DNA-coupled Sepharose column. Anti-DNA antibodies expressed specific Id, termed O-81 or NE-1, which were detected only in the IC of patients with active lupus nephritis. Anti-DNA clonotypes, including O-81 and NE-1 idiotypes, were also found in the eluates of renal glomeruli of lupus patients. These results indicate that subpopulations of anti-DNA antibodies in circulating IC are limited, and may play an important role in the pathogenesis of lupus nephritis.
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PMID:Clonotypes of anti-DNA antibodies expressing specific idiotypes in immune complexes of patients with active lupus nephritis. 233 34

We analyzed isoelectrofocusing (IEF) patterns of anti-DNA antibodies originated from sera and the renal eluates of patients with systemic lupus erythematosus (SLE). The spectrotypic patterns of serum anti-DNA-antibodies were heterogenous and bands with single-stranded (ss) and double-stranded (ds) DNA were detected in the PI 5.5-6.5 and PI 8-9.5 regions when SLE sera were tested, whereas healthy subjects failed to form bands even at different saline concentrations. The renal eluates from normal subjects never bound to DNA whereas those from SLE glomeruli showed relatively restricted IEF patterns which were detected mainly in PI 6.0 and PI 8.5, showing that some anti-DNA antibodies may be nephritogenic. However, the spectrotypic patterns of serum anti-DNA antibodies in patients with active lupus nephritis were similar with those in patients lacking renal lesions. The reasons why IEF analysis failed to indentify specific clonotypes of nephritogenic anti-DNA antibodies are discussed in association with pathogenesis of lupus nephritis. This study also suggests that the use of a high concentration of 6M urea in an IEF analysis may be able to expose antigen-binding sites of the circulating immune complex (IC)-derived antibodies.
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PMID:Are the clonotypes of serum IgG anti-DNA antibodies associated with lupus nephritis in humans? 235 53

We performed a series of studies to examine the sequential development of nephritis during murine malaria infections and to define the role of DNA-binding antibodies in the associated pathology. Serum levels of these antibodies were assessed throughout acute and chronic malaria infections. Increased levels of double-stranded DNA- and single-stranded DNA-binding antibodies were initially detected in mice infected with Plasmodium vinckei or Plasmodium yoelii nigeriensis during the middle stages of infection, and these levels were maintained until death. Infection with the more chronic organism Plasmodium berghei clone RC also resulted in increased single-stranded DNA-binding antibody titers, which fluctuated as the infection progressed. All three species caused kidney damage and dysfunction, as assessed by changes in morphology, blood urea nitrogen, and excreted albumin; this damage correlated with the extent of parasitemia and was observed before the levels of DNA-binding antibodies were detectably elevated in the serum. However, the results of immunohistochemical studies demonstrated that DNA-binding monoclonal antibodies bound ex vivo to glomeruli within kidneys prepared from mice at late stages of infection, after the initial damage had been incurred. Our findings suggest how DNA-binding antibodies could contribute to the kidney pathology associated with both malaria and certain autoimmune diseases, such as systemic lupus erythematosus.
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PMID:Role of DNA-binding antibodies in kidney pathology associated with murine malaria infections. 236 56

Successful pregnancies in women on regular hemodialysis treatment are infrequent but increasing. We present two such cases; both pregnancies were diagnosed early, and hemodialysis was intensified, leading to significant reductions in predialysis serum urea levels (70-100 mg/dl). One case was particularly unusual in that systemic lupus erythematosus was the underlying disease, and the patient had no residual renal function at the time of conception. Both patients delivered by cesarean section at 32 and 35 weeks, and their infants are well at ages 2 years and 18 months, respectively. The management and the complications associated with such pregnancies are discussed.
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PMID:Successful pregnancies in women on regular hemodialysis treatment. 238 24

The characteristics of eukaryotic ribosomal proteins P0, P1, and P2 (P proteins) and their antigenic determinants were studied using the sera of patients with systemic lupus erythematosus (SLE). P0, P1, and P2 were isolated as a macromolecular complex by preparative isoelectric focusing and anion-exchange chromatography in the presence of 6 M urea. The apparent molecular size of the complex was 140 kDa as determined by gel filtration on a Sephadex G-200 column. P0 may, therefore, be the eukaryotic equivalent of Escherichia coli ribosomal protein L10. In addition, all three P proteins were detected in the postribosomal supernatant of HeLa cells, and P0 and P1 were found to be more acidic than their ribosome-bound counterparts. Partial proteolysis experiments revealed that SLE anti-P sera recognized one or both ends of the P2 equivalent protein from Artemia salina (eL12). Sixteen SLE sera containing antibodies to P0, P1, and P2 reacted with a carboxyl-terminal peptide 22 amino acids in length of eL12 and not with an amino-terminal peptide of 20 amino acids. Even though the carboxyl-terminal peptide completely inhibited the ability of the antiserum to react with all three proteins on an immunological blot, the same peptide produced only small decreases in binding of the SLE antibody to the native, nondenatured P proteins. These findings indicate that SLE anti-P antibodies react with a single sequential (linear) antigenic determinant on all three P proteins, but that additional antibodies recognize a conformational determinant(s).
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PMID:Identification and chemical synthesis of a ribosomal protein antigenic determinant in systemic lupus erythematosus. 242 5

MRL/Mp-lpr/lpr (MRL/l) mice spontaneously develop autoimmune kidney disease which resembles human systemic lupus erythematosus (SLE). Employing this strain of mouse, we examined the effect of several immunosuppressants and a newly synthetized anti-nephritic agent, 4-chloro-3',6'-dimethyl-2,2'-iminodibenzoic acid (TO-115) on both the development of immunological abnormalities and the clinical symptoms of autoimmune kidney disease. This study aimed to determine how much the magnitude of autoantibody suppression related to the degree of prevention of autoimmune nephritis. Immunological abnormalities were assessed by measuring the serum levels of anti-deoxyribonucleic acid and anti-trinitrophenyl antibodies, rheumatoid factor (RF), and immune complex (IC). The status of autoimmune kidney disease was monitored by means of the appearance of proteinuria and survival time and the measurement of serum levels of blood urea nitrogen (BUN) and cholesterol. Immunosuppressants such as cyclophosphamide (CY), 6-mercaptopurine (6 MP) and sodium aurothiomalate (SAT) remarkably suppressed the development of immunological abnormalities in a dose dependent manner. Interestingly, however, only CY showed the suppressive effect on the development of autoimmune kidney disease with prolongation of survival time and the excretion of proteinuria. In contrast, 6-MP and SAT did not inhibit the development of autoimmune kidney disease. On the other hand, TO-115 did not suppress the development of immunological abnormalities, but it restrained the symptoms of autoimmune kidney disease. Taken together, the suppression of autoantibody production does not always lead to prevention of the development of autoimmune kidney disease.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Effect of several kinds of drugs on the development of autoimmunity in MRL/Mp-lpr/lpr mice; lack of correlation between the suppression of autoantibody production and prevention of autoimmune disease. 252 11


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