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  • Adhesion  (1)
  • Key words Chronic granulomatous  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Recombinant human interferon-γ treatment in severe leucocyte adhesion deficiency (1992)
    Springer
    European journal of pediatrics 151 (1992), S. 103-107 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF01958952
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Recombinant human interferon-gamma in patients with chronic granulomatous disease – European follow up study (1995)
    Springer
    European journal of pediatrics 154 (1995), S. 295-298 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF01957365
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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