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  • 1
    ISSN: 1573-8280
    Keywords: Platonin ; JRA
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two children with juvenile rheumatoid arthritis (JRA) were given photosensitive dye Platonin in combination with prednisolone. Analysis of clinical and laboratory data showed that Platonin was efficacious in the improvement of the clinical symptoms and the severity of inflammation, or in the maintenance of the remission state. There were no adverse side effects during long-term administration of medication.
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  • 2
    ISSN: 1573-8280
    Keywords: polyarteritis nodosa ; Platonin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Polyarteritis nodosa (PN), characterized by vasculitis of medium and small sized arteries, is uncommon in children. This disease is thought to be concerned with some abnormal immune responses. Platonin (4,4′-dimethyl-3,3′-di-n-heptyl-8-[2-(4-methyl-3-n-heptylthiazole)]-2,2′-dicar bocyanine diiodine) is one of the photosensitive dyes of trithiazole pentamethine cyanine. It has been reported that platonin stimulated suppresser/cytotoxic T cells, but suppressed B cells. In clinical observations, platonin is shown to be efficacious for rheumatoid arthritis and juvenile rheumatoid arthritis. In this study, a Japanese boy (9-year-old) with PN was given platonin in combination with prednisolone. Prednisolone was gradually tapered under a cover of platonin. As a result, the relapse has not been exhibited for a long time (72 months). There were no adverse side effects during long term administration of medication.
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  • 3
    ISSN: 1573-8280
    Keywords: asparagine-linked sugar chains ; erythrocytes ; membrane glycoproteins ; Wiskott-Aldrich syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In both healthy controls and patients with Wiskott-Aldrich syndrome, the main oligosaccharide in asparagine-linked sugar chains of the membrane glycoproteins of erythrocytes was biantennary sugar chain with bisected G1cNAc (Gal2-GlcNAc2-Man3-GlcNAc-GlcNAc-Fuc-GlcNAcOT). Biantennary sugar chain with anα-fucosyl residue linked at the proximal GlcNAc was seen but biantennary sugar chain without anα-fucosyl residue at the proximal GlcNAc was not detected in each subject. There was no difference in quality and quantity of asparagine-linked sugar chains of erythrocyte membrane glycoproteins between healthy controls and the patients. These results suggest that asparagine-linked sugar chains in membrane glycoproteins of hematopoietic cells may not be impaired in Wiskott-Aldrich syndrome.
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  • 4
    ISSN: 1573-8280
    Keywords: asparagine-linked sugar chain ; B lymphoblastoid cell lines ; common variable immunodeficiency ; glycosylation ; membrane glycoproteins
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Asparagine-linked sugar chains of plasma membrane glycoproteins, which are formed by glycosylation during B cell maturation, were examined with B lymphoblastoid cell lines (LCLs) transformed by Epstein-Barr virus derived from healthy controls and patients with common variable immunodeficiency (CVI). Both two patients with CVI showed hypogammaglobulinemia and impaired B cell functions. LCLs from healthy controls and the patients showed CD19+ and HLA/DR+ in the cell surface and secreted IgM. In both healthy controls and the patients, the main oligosaccharide in asparagine-linked sugar chains of the membrane glycoproteins of LCLs was biantennary sugar chain with bisected GlcNAc (Gal2-GlcNAc2-Man3-GlcNAc-GlcNAc-Fuc-GlcNAcOT). Biantennary sugar chain with anα-fucosyl residue linked at the proximal GIcNAc was seen but biantennary sugar chain without anα-fucosyl residue at the proximal GlcNAc was little detected in each LCL. There was no difference in quality and quantity of asparagine-linked sugar chains between healthy controls and the patients. These results suggest that glycosylation during B cell maturation may not be impaired in patients with CVI.
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  • 5
    ISSN: 1573-8280
    Keywords: common variable immunodeficiency ; intravenous immunoglobulin ; replacement therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Five patients with common variable immunodeficiency treated in our hospital between December 1979 and December 1990 were given six kinds of intravenous immunoglobulin preparations (pepsin treated, S-sulfonated, polyethylene glycol treated, pH4 treated, alkylated, and pH4.25 formulation preparation) for replacement therapy. Duration of the therapy ranged from 7.6 to 11 years. Incidences of fever and acute infections were variable among patients, but no significant differences were seen in the incidences among periods given each preparation. Three cases revealed abnormal pulmonary functions in tests. Adverse reactions were rarely seen in our study periods, and no severe reactions were observed. No significant differences were seen in incidences of adverse reactions. Postinfusion levels of serum complement slightly decreased from preinfusion levels. However, the decrease in complement was not related to any adverse reaction. No long-term complications such as transmission of hepatitis have been observed. Our data suggest that no obvious differences exist between the efficacy and safety of each IVIG preparation. Differences of efficacy of IVIG replacement therapy may be due to the variable pathophysiology of each patient.
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  • 6
    ISSN: 1573-2592
    Keywords: Intravenous immunoglobulin ; B cells ; monocytes ; immunoglobulin production
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The proliferative responses and the immunoglobulin production of peripheral blood mononuclear cells to pokeweed mitogen were dose-dependently suppressed by sulfonated intravenous immunoglobulin (IVIG), polyethylene glycol-treated IVIG, pH 4-treated IVIG, or human γ-globulin, but they were not or only slightly suppressed by human serum albumin or pepsin-treated IVIG. Moreover, the suppression of immunoglobulin production by sulfonated IVIG, polyethylene glycol-treated IVIG, or pH 4-treated IVIG was seen in the cases in which B cells preincubated with IVIGs were cocultured with T cells and monocytes preincubated with or without IVIGs and in the cases in which monocytes preincubated with IVIGs were cocultured with T cells and B cells preincubated with or without IVIGs. However, in the cases in which only T cells were preincubated with IVIGs, immunoglobulin production was not suppressed. The suppression of the monocyte function by IVIGs tended to be less than the suppression of the B-cell function by IVIGs. Moreover, the suppression by IVIGs was blocked by antihuman IgG Fc. Our results suggest that IVIGs suppress the immunoglobulin production of lymphocytes through suppression of the B-cell function and the antigen presenting-cell function by attachment of IVIGs to Fc receptors of B-cell membranes and antigen presenting-cell membranes.
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  • 7
    ISSN: 1573-2592
    Keywords: Common variable immunodeficiency (CVI) ; μ messenger RNA ; interleukins ; Ig enhancer
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Five common variable immunodeficiency (CVI) patients were analyzed for expression of immunoglobulin (Ig) genes. In the pokeweed mitogen (PWM)-induced Ig-production assay, the combination of T and B cells showed that all patients' T cells had normal helper functions and all patients' B cells had profound defects. The defective B-cell maturation stages based on their Ig gene expression patterns were variable. One of five patients showed normal μ-chain gene expression and nearly normal IgM production, but neither IgG nor IgA production, which suggested that this patient's B-cell defects might lie on a μ- to γ or μ- to α class-switch stage. B cells in another patient showed low μ-chain gene expression and low IgM production, but an Ig enhancer region, which is an important region for expression of Ig genes, was intact. Thus, this patient might have a transacting factor defect which interacts with the Ig enhancer region. The other three patients showed no μ-chain gene expression and no IgM production. Thus, their B-cell defects lay on the B-cell maturation stage, similar to X-linked agammaglobulinemia. These results showed that primary B-cell defects in CVI occurred at several B-cell differentiation stages, which could be recognized by expression of Ig genes.
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