title: Atypical presentation of chronic granulomatous disease with Burkholderia cepacia.
abstract: Chronic granulomatous disease (CGD) is a rare inherited disorder of neutrophil oxidative burst. In patients with CGD, phagocyte destruction of catalase-producing organisms is impaired, resulting in recurrent and potentially fatal infections. Burkholderia cepacia, a catalase-producing organism, is known to infect patients with dysfunctional immune systems. We report a case of a 3-year-old boy with this rare infection that unravelled the diagnosis of CGD.
authors.affiliation: Georgia Regents University, Augusta, Georgia, USA.
authors.forename: Mac
authors.initials: M
authors.lastname: Vining
authors.affiliation: Department of Pediatrics, Georgia Regents University, Augusta, Georgia, USA.
authors.forename: Nirupma
authors.initials: N
authors.lastname: Sharma
authors.affiliation: Department of Pediatric Pulmonology, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire, USA.
authors.forename: Margaret
authors.initials: M
authors.lastname: Guill
mesh_terms: D019121:Burkholderia Infections / Q000382:microbiology*; D016956:Burkholderia cepacia; D002374:Catalase / Q000378:metabolism; D002675:Child, Preschool; D006105:Granulomatous Disease, Chronic / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008297:Male; D009894:Opportunistic Infections / Q000382:microbiology*; D010586:Phagocytes; D016897:Respiratory Burst
chemical_list: D002374:Catalase
references.citation: Clin Exp Immunol. 2000 Oct;122(1):1-9
references.pmid: 11012609
references.citation: J Pediatr. 2000 Nov;137(5):687-93
references.pmid: 11060536
references.citation: Blood. 1996 Mar 1;87(5):1663-81
references.pmid: 8634410
references.citation: Medicine (Baltimore). 2000 May;79(3):155-69
references.pmid: 10844935
references.citation: J Antimicrob Chemother. 1994 Dec;34(6):965-74
references.pmid: 7730239
references.citation: Nat Rev Microbiol. 2005 Feb;3(2):144-56
references.pmid: 15643431
references.citation: Clin Infect Dis. 2009 Jun 1;48(11):1577-9
references.pmid: 19400745
references.citation: Am J Clin Pathol. 1979 May;71(5):582-5
references.pmid: 453076
references.citation: Psychosomatics. 2004 May-Jun;45(3):230-4
references.pmid: 15123849
references.citation: Allergol Immunopathol (Madr). 2007 May-Jun;35(3):83-9
references.pmid: 17594870
references.citation: Arch Dis Child. 1990 Sep;65(9):942-5
references.pmid: 2221966
references.citation: Pediatr Infect Dis J. 1999 Feb;18(2):155-6
references.pmid: 10048690
references.citation: J Pediatr. 1996 Jan;128(1):104-7
references.pmid: 8551399
references.citation: J Immunol. 2005 Mar 15;174(6):3562-9
references.pmid: 15749893
references.citation: Curr Opin Pulm Med. 2005 Nov;11(6):528-33
references.pmid: 16217180