title: Treatment of patients with chronic granulomatous disease with recombinant human interferon-gamma does not improve neutrophil oxidative metabolism, cytochrome b558 content or levels of four anti-microbial proteins.
abstract: Recombinant interferon-gamma (rIFN-gamma) has been described to enhance phagocyte functions in vitro and in vivo in several patients with chronic granulomatous disease (CGD). To demonstrate the clinical usefulness of this treatment, 128 patients were treated in a randomized, double-blind multi-centre study with a placebo preparation or with rIFN-gamma. We analysed parameters of neutrophil oxidative and non-oxidative metabolism in 16 patients enrolled in this study. No enhanced superoxide-release was observed in patients treated with rIFN-gamma compared to placebo-treated patients. Phagocyte cytochrome b558 content also remained unchanged. Levels of four non-oxidative antimicrobial proteins (cathepsin G, azurocidine, p29b, lactoferrin) rose, fell, or remained unchanged, irrespective of treatment with rIFN-gamma or placebo.
authors.affiliation: Division of Immunology-Haematology, University Children's Hospital, Zürich, Switzerland.
authors.forename: T J
authors.initials: TJ
authors.lastname: Mühlebach
authors.forename: J
authors.initials: J
authors.lastname: Gabay
authors.forename: C F
authors.initials: CF
authors.lastname: Nathan
authors.forename: C
authors.initials: C
authors.lastname: Erny
authors.forename: G
authors.initials: G
authors.lastname: Dopfer
authors.forename: H
authors.initials: H
authors.lastname: Schroten
authors.forename: V
authors.initials: V
authors.lastname: Wahn
authors.forename: R A
authors.initials: RA
authors.lastname: Seger
mesh_terms: D001798:Blood Proteins / Q000187:drug effects / Q000276:immunology; D003573:Cytochrome b Group / Q000172:deficiency / Q000187:drug effects; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000188:drug therapy* / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000187:drug effects; D007371:Interferon-gamma / Q000627:therapeutic use*; D008297:Male; D009247:NADH, NADPH Oxidoreductases / Q000187:drug effects; D019255:NADPH Oxidases; D009504:Neutrophils / Q000187:drug effects; D011994:Recombinant Proteins
chemical_list: D001798:Blood Proteins; D003573:Cytochrome b Group; D007074:Immunoglobulin G; D011994:Recombinant Proteins; D007371:Interferon-gamma; C036888:cytochrome b558; D009247:NADH, NADPH Oxidoreductases; D019255:NADPH Oxidases