title: Actinomyces in chronic granulomatous disease: an emerging and unanticipated pathogen.
abstract: BACKGROUND
  Chronic granulomatous disease (CGD) is a rare inherited disease of the phagocyte NADPH oxidase system that causes defective production of toxic oxygen metabolites, impaired bacterial and fungal killing, and recurrent life-threatening infections, mostly by catalase-producing organisms. We report for the first time, to our knowledge, chronic infections with Actinomyces species in 10 patients with CGD. Actinomycosis is a chronic granulomatous condition that commonly manifests as cervicofacial, pulmonary, or abdominal disease, caused by slowly progressive infection with oral and gastrointestinal commensal Actinomyces species. Treatment of actinomycosis is usually simple in immunocompetent individuals, requiring long-term, high-dose intravenous penicillin, but is more complicated in those with CGD because of delayed diagnosis and an increased risk of chronic invasive or debilitating disease.
  
  
  METHODS
  Actinomyces was identified by culture, staining, 16S ribosomal DNA polymerase chain reaction, and/or a complement fixation test in 10 patients with CGD.
  
  
  RESULTS
  All 10 patients presented with a history of fever and elevated inflammatory signs without evident focus. Diagnosis was delayed and clinical course severe and protracted despite high-dose intravenous antibiotic therapy and/or surgery. These results suggest an unrecognized and unanticipated susceptibility to weakly pathogenic Actinomyces species in patients with CGD because these are catalase-negative organisms previously thought to be nonpathogenic in CGD.
  
  
  CONCLUSIONS
  Actinomycosis should be vigorously sought and promptly treated in patients with CGD presenting with uncommon and prolonged clinical signs of infection. Actinomycosis is a catalase-negative infection important to consider in CGD.
authors.affiliation: Division of Immunology, University Children's Hospital, Zurich, Switzerland. janine.reichenbach@kispi.uzh.ch
authors.forename: Janine
authors.initials: J
authors.lastname: Reichenbach
authors.forename: Uri
authors.initials: U
authors.lastname: Lopatin
authors.forename: Nizar
authors.initials: N
authors.lastname: Mahlaoui
authors.forename: Bojana
authors.initials: B
authors.lastname: Beovic
authors.forename: Ulrich
authors.initials: U
authors.lastname: Siler
authors.forename: Reinhard
authors.initials: R
authors.lastname: Zbinden
authors.forename: Reinhard A
authors.initials: RA
authors.lastname: Seger
authors.forename: Louise
authors.initials: L
authors.lastname: Galmiche
authors.forename: Nicole
authors.initials: N
authors.lastname: Brousse
authors.forename: Samer
authors.initials: S
authors.lastname: Kayal
authors.forename: Tayfun
authors.initials: T
authors.lastname: Güngör
authors.forename: Stéphane
authors.initials: S
authors.lastname: Blanche
authors.forename: Steven M
authors.initials: SM
authors.lastname: Holland
mesh_terms: D000190:Actinomyces / Q000235:genetics / Q000472:pathogenicity*; D000196:Actinomycosis / Q000175:diagnosis* / Q000188:drug therapy* / Q000601:surgery / Q000628:therapy; D000293:Adolescent; D000328:Adult; D000658:Amoxicillin / Q000627:therapeutic use; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D017963:Azithromycin / Q000627:therapeutic use; D016026:Bone Marrow Transplantation; D002443:Ceftriaxone / Q000627:therapeutic use; D002648:Child; D002981:Clindamycin / Q000627:therapeutic use; D021821:Communicable Diseases, Emerging / Q000382:microbiology* / Q000473:pathology*; D004275:DNA, Ribosomal / Q000235:genetics; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000382:microbiology*; D006801:Humans; D008297:Male; D000077731:Meropenem; D010400:Penicillin G / Q000627:therapeutic use; D010404:Penicillin V / Q000627:therapeutic use; D016133:Polymerase Chain Reaction; D013420:Sulfamethoxazole / Q000627:therapeutic use; D013845:Thienamycins / Q000627:therapeutic use; D014295:Trimethoprim / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D000900:Anti-Bacterial Agents; D004275:DNA, Ribosomal; D013845:Thienamycins; D002981:Clindamycin; D002443:Ceftriaxone; D000658:Amoxicillin; D017963:Azithromycin; D014295:Trimethoprim; D000077731:Meropenem; D013420:Sulfamethoxazole; D010400:Penicillin G; D010404:Penicillin V