title: Granulocyte-macrophage colony stimulating factor does not improve neutrophil oxidative metabolism in a patient with variant X-linked chronic granulomatous disease.
abstract: Variant X-linked chronic granulomatous disease (CGD) is characterised by a decreased but still measurable respiratory burst and cytochrome b content of phagocytes resulting in a clinically milder form of the disease. We examined the in vivo effect of recombinant human granulocyte-macrophage colony stimulating factor (rh-GM-CSF) on the neutrophil functions of a patient treated for liver abscess. The number of white blood cells was markedly increased at the highest dose of GM-CSF injected (30 micrograms/kg per day). This was mainly due to a large increase in eosinophils and to a lesser extent in neutrophils. No change in the deficient neutrophil respiratory burst nitroblue tetrazolium (NBT)-reduction, superoxide (O2-)-production and cytochrome b content was observed during 6 weeks of therapy with increasing doses of GM-CSF. No significant clinical improvement of the liver abscess was observed during treatment with GM-CSF.
authors.affiliation: Division of Immunology-Haematology, Childrens Hospital, University of Zürich, Switzerland.
authors.forename: T J
authors.initials: TJ
authors.lastname: Mühlebach
authors.forename: H J
authors.initials: HJ
authors.lastname: Feickert
authors.forename: K
authors.initials: K
authors.lastname: Welte
authors.forename: R A
authors.initials: RA
authors.lastname: Seger
mesh_terms: D000293:Adolescent; D002648:Child; D003573:Cytochrome b Group / Q000032:analysis; D008040:Genetic Linkage; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000494:pharmacology* / Q000627:therapeutic use; D006105:Granulomatous Disease, Chronic / Q000150:complications / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D007958:Leukocyte Count; D008100:Liver Abscess / Q000188:drug therapy / Q000209:etiology; D008297:Male; D019255:NADPH Oxidases; D009504:Neutrophils / Q000737:chemistry / Q000187:drug effects* / Q000378:metabolism*; D010084:Oxidation-Reduction; D013481:Superoxides / Q000378:metabolism; D014960:X Chromosome
chemical_list: D003573:Cytochrome b Group; D013481:Superoxides; D016178:Granulocyte-Macrophage Colony-Stimulating Factor; C036888:cytochrome b558; D019255:NADPH Oxidases