title: Infection with Pseudomonas cepacia in chronic granulomatous disease: role of nonoxidative killing by neutrophils in host defense.
abstract: Pseudomonas aeruginosa and Pseudomonas cepacia are catalase-producing bacteria, but only P. cepacia causes infections in patients with chronic granulomatous disease (CGD). The in vitro killing of P. aeruginosa and P. cepacia by polymorphonuclear leukocytes (PMNL) from patients with CGD and from healthy adults was assessed. Of 6 patients with CGD who developed severe infections with P. cepacia, 4 died. PMNL from the 2 survivors and 6 other patients with CGD killed P. aeruginosa strains efficiently and P. cepacia strains poorly. PMNL from 2 patients with autosomal recessive CGD and from 2 carriers for X-linked CGD killed P. cepacia intermediately between normal controls and patients with X-linked CGD. When superoxide anion and hydrogen peroxide were scavenged with superoxide dismutase and catalase, normal PMNL killed P. aeruginosa but not P. cepacia. Thus, P. cepacia, but not P. aeruginosa, is a pathogen in patients with CGD, because it resists neutrophil-mediated nonoxidative bactericidal effects.
authors.affiliation: Department of Pediatrics, University of British Columbia, Vancouver, Canada.
authors.forename: D P
authors.initials: DP
authors.lastname: Speert
authors.forename: M
authors.initials: M
authors.lastname: Bond
authors.forename: R C
authors.initials: RC
authors.lastname: Woodman
authors.forename: J T
authors.initials: JT
authors.lastname: Curnutte
mesh_terms: D016956:Burkholderia cepacia / Q000276:immunology* / Q000472:pathogenicity; D002374:Catalase; D002648:Child; D002675:Child, Preschool; D016166:Free Radical Scavengers; D006105:Granulomatous Disease, Chronic / Q000150:complications / Q000276:immunology*; D006579:Heterozygote; D006801:Humans; D008297:Male; D009504:Neutrophils / Q000276:immunology* / Q000378:metabolism; D011552:Pseudomonas Infections / Q000150:complications / Q000276:immunology*; D011550:Pseudomonas aeruginosa / Q000276:immunology; D013211:Staphylococcus aureus / Q000276:immunology; D013482:Superoxide Dismutase
chemical_list: D016166:Free Radical Scavengers; D002374:Catalase; D013482:Superoxide Dismutase