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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Inflammation research 10 (1980), S. 528-535 
    ISSN: 1420-908X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report reviews some of our work on the relative importance of the glutathione redox system and catalase in protecting human neutrophils in vitro against hydrogen peroxide, generated either by these cells during phagocytosis or artificially in the medium by an enzyme system. Neutrophils deficient in glutathione reductase were rapidly inactivated during phagocytosis, unless protected by scavengers of oxidative products in the medium. In contrast, normal neutrophils remained functionally active. Thus, despite the presence of a normal catalase activity, a defect in the glutathione system totally impairs the protection of neutrophils against their own metabolic products. In catalase-inhibited or catalase-deficient neutrophils, no damage was observed during phagocytosis. We conclude that the glutathione redox system is the most important protection system against damage by oxidative products of neutrophils. During incubation of neutrophils with glucose + glucose oxidase, an extracellular system that generates hydrogen peroxide, we found that both catalase and the glutathione redox system were needed for adequate protection against oxidative injury. Apparently, this extracellular stress cannot be efficiently dealth with by the glutathione system alone: co-operation with catalase is needed in this situation. Under certain conditions, oxidative damage was observed even when the level of reduced glutathione was still relatively high, indicating that perhaps catalase and glutathione each protect different cell structures, and that both systems are needed together for the preservation of the total cell function.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1420-908X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 154 (1995), S. 295-298 
    ISSN: 1432-1076
    Keywords: Chronic granulomatous disease ; Interferon-gamma ; Infections
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This was an uncontrolled, open-label follow up study of a previous 12-month, randomized, double-blind, placebo-controlled trial performed to assess the long-term efficacy and safety of Recombinant Human Interferon Gamma (rIFN-γ) in patients with chronic granulomatous disease (CGD). In two centres, 28 patients (24 male, 4 female) with a mean age of 16 years (range 3–37) entered the open-label phase. The patients were treated for a mean of 880 days (range 97–1375 days). Visits were scheduled every 180 days and patients completed one to six visits. rIFN-γ was administered subcutaneously three times weekly at a dose of 0.05 mg per m2. During the open-label phase of the study 12 patients experienced a serious infection requiring hospitalization within 880 days. The median infection-free time was 993 days. No obvious increase of infections over time was seen. Phagocyte superoxide anion production and phagocyte staphylococcal killing were not influenced by therapy. Seven patients were withdrawn from the study, one because of an adverse reaction, three on their own wish and the other three because they changed to another trial. No patient died during the study. Conclusion Treatment of patients with CGD with intracellular active antibiotics and additional interferon gamma as infection prophylaxis is safe and justified.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 147 (1988), S. 444-445 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1076
    Keywords: Leucocyte ; Adhesion ; Complement receptor ; Interferon-γ
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe a patient with leucocyte adhesion deficiency (LAD). Clinically, the patient had delayed umbilical cord detachment, omphalitis, impaired wound healing and persistent leucocytosis. The patient had the severe form of LAD, with a total absence of leucocyte cell adhesion molecules (LeuCAMs) and undetectable mRNA for the β chain, the common subunit of the LeuCAMs. In vitro neutrophil chemotaxis, aggregation and oxygen consumption were severely impaired. In vitro incubation of neutrophils with recombinant human interferon-γ (rIFN-γ) showed an increase in oxygen consumption, but no effect on the expression of the LeuCAMs, or the β chain mRNA. In vivo treatment with IFN-γ was started. The FcγRI receptor appeared on the neutrophils, the LeuCAMs remained undetectable, while the neutrophil functions remained disturbed. The patient died of surgical complications after 10 weeks of rIFN-γ treatment. No new infections or side-effects due to rIFN-γ were observed.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1076
    Keywords: Scientific research ; Paediatrics ; The Netherlands ; Quality
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The board of the Dutch Paediatric Association requested a survey of the scientific research performed in all academic and non-academic paediatric hospitals, authorized to train medical doctors in paediatrics in the Netherlands. Contributions to the international and the Dutch scientific literature, in the form of regular publications, chapters in books, contributions to proceedings and Ph.D. theses were counted over two 5-year periods, i.e. 1981–1985 and 1986–1990. The quality of publications in the international journals was assessed using the average impact factor of the journals over the 10-year period. The number of publications in the international literature doubled during the observation period 1986–1990 compared to the period 1981–1985. Nevertheless, the quality of the publications remained the same. Metabolism, oncology/ haematology, immunology/infectious diseases and cardiology are the subspecialisations in which scientific research takes place in four or more academic paediatric hospitals. In total, 84 Ph.D. theses were produced in which a paediatrician was either the project leader (mostly a professor in paediatrics) or the investigator-in-charge. Insight into structure and major research efforts of paediatric hospitals in other countries of Europe may lead to exchange of views and, maybe, profitable co-operation.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 152 (1993), S. 896-899 
    ISSN: 1432-1076
    Keywords: Neutrophil ; Inflammation ; Immunodeficiency ; Granuloma ; Chronic granulomatous disease ; Infection ; Antibiotics ; Interferon ; Therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Chronic granulomatous disease (CGD) is a primary immunodeficiency disease which results from absence of the NADPH oxidase in the professional phagocytic cells [13] neutrophils, monocytes, macrophages and eosinophils. Deficiency of this oxidase renders the patient liable to infection by bacteria and fungi, and, as the name of the disease suggests, to chronic granulomatous inflammation. These patients present with a great variety of infections and other complications of their disease, which often tax the clinical and therapeutic skill of the doctors responsible for their care. Collectively we look after, or advise on the management of, over 100 of these subjects, and have developed experience in the diagnosis and management of the infections and other clinical problems they present. We thought that it might be timely to provide guidelines for their management based upon this experience. The numbers of patients are still relatively small, and the clinical presentations very varied, so it is immpossible to provide clear statistical proof of the veracity of this advice. It does, however, reflect the working practise of the physicians caring for many of these patients in Europe.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 154 (1995), S. 295-298 
    ISSN: 1432-1076
    Keywords: Key words Chronic granulomatous ; disease ; Interferon-gamma ; Infections
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This was an uncontrolled, open-label follow up study of a previous 12-month, randomized, double-blind, placebo-controlled trial performed to assess the long-term efficacy and safety of Recombinant Human Interferon Gamma (rIFN-γ) in patients with chronic granulomatous disease (CGD). In two centres, 28 patients (24 male, 4 female) with a mean age of 16 years (range 3–37) entered the open-label phase. The patients were treated for a mean of 880 days (range 97–1375 days). Visits were scheduled every 180 days and patients completed one to six visits. rIFN-γ was administered subcutaneously three times weekly at a dose of 0.05 mg per m2. During the open-label phase of the study 12 patients experienced a serious infection requiring hospitalization within 880 days. The median infection-free time was 993 days. No obvious increase of infections over time was seen. Phagocyte superoxide anion production and phagocyte staphylococcal killing were not influenced by therapy. Seven patients were withdrawn from the study, one because of an adverse reaction, three on their own wish and the other three because they changed to another trial. No patient died during the study. Conclusion Treatment of patients with CGD with intracellular active antibiotics and additional interferon gamma as infection prophylaxis is safe and justified.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
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