title: Bacteremia and skin/bone infections in two patients with X-linked agammaglobulinemia caused by an unusual organism related to Flexispira/Helicobacter species.
abstract: Two patients with Bruton's X-linked agammaglobulinemia are described with bacteremia and skin/bone infection due to an organism which by 16S rRNA gene sequence analysis was most closely related to "Flexispira" rappini (and thus designated a Flexispira-like organism, FLO) and more distantly related to the Helicobacter species. The organism required microaerobic conditions and, supplemental H(2) gas for growth and was reliably stained with acridine orange. In common with Helicobacter cinaedi infections, the focus of the FLO infection was in one case in the blood vessels or lymphatics of an extremity and in the other case in the skin and adjacent bone of an extremity. In both cases, prolonged IV antibiotic therapy was necessary to clear the infection. The susceptibility of XLA patients to FLO infection appears to be related to the fact that XLA is associated with severe B cell (humoral) immunodeficiency and thus these patients have difficulty with intravascular or intralymphatic infection. These findings elucidate the nature of FLO infections in humans and point the way to their detection and treatment.
authors.affiliation: Mucosal Immunity Section, NIAID, Bethesda, MD 20892, USA.
authors.forename: B
authors.initials: B
authors.lastname: Cuccherini
authors.forename: K
authors.initials: K
authors.lastname: Chua
authors.forename: V
authors.initials: V
authors.lastname: Gill
authors.forename: S
authors.initials: S
authors.lastname: Weir
authors.forename: B
authors.initials: B
authors.lastname: Wray
authors.forename: D
authors.initials: D
authors.lastname: Stewart
authors.forename: D
authors.initials: D
authors.lastname: Nelson
authors.forename: I
authors.initials: I
authors.lastname: Fuss
authors.forename: W
authors.initials: W
authors.lastname: Strober
mesh_terms: D000328:Adult; D000361:Agammaglobulinemia / Q000235:genetics* / Q000382:microbiology; D016470:Bacteremia / Q000150:complications*; D001847:Bone Diseases / Q000150:complications* / Q000276:immunology; D020459:Genes, rRNA / Q000235:genetics; D008040:Genetic Linkage; D016998:Helicobacter / Q000235:genetics; D016481:Helicobacter Infections / Q000150:complications* / Q000276:immunology; D006801:Humans; D008297:Male; D017423:Sequence Analysis, RNA; D017192:Skin Diseases, Bacterial / Q000150:complications* / Q000276:immunology; D014960:X Chromosome