title: Idiopathic disseminated bacillus Calmette-Guérin infection: a French national retrospective study.
abstract: OBJECTIVE
  Disseminated bacillus Calmette-Guérin (BCG) infection after inoculation of live vaccine is considered to result from impaired immunity of the child. However, in half of the cases, regarded as idiopathic, no well-defined immunodeficiency condition can account for the infection. The objective of the present study is to report the prevalence, clinical features, associated infections, and outcomes of children with idiopathic disseminated BCG infection.
  
  
  METHODS
  National retrospective survey during the period from 1974 through 1994 in France.
  
  
  METHODS
  All neonatology and pediatrics units in primary care and referral centers throughout France.
  
  
  METHODS
  Data were collected from 595 (82%) of 721 units, 377 (93%) of 407 centers, and 320 (93%) of 345 cities. Selection criteria included BCG infection, dissemination to at least two areas beyond the inoculation site, and no well-defined immunodeficiency condition. Sixteen children (8 girls and 8 boys), born to families unrelated to each other but often consanguinous (5 of 16) or abroad (5 of 16).
  
  
  RESULTS
  The minimal prevalence rate was estimated at 0.59 cases per 1 million vaccinated children born in France. Clinical features included fever and cachexia, disseminated BCG infection to lymph nodes (15 of 16), skin (13 of 16), soft tissues (11 of 16), lungs (11 of 16), spleen (11 of 16), liver (11 of 16), and bones (9 of 16). Eight children had associated or subsequent severe opportunistic infection (50%), with either nontyphi Salmonella enterica serotypes (7 of 16) or Mycobacterium abscessus (1 of 16). The outcome was poor: 8 children (50%) died; the cause of death was BCG infection for most children (7 of 8); 8 survived until the last follow-up (50%).
  
  
  CONCLUSIONS
  Idiopathic disseminated BCG infection is a rare but severe complication of BCG vaccination. The infection probably results from an as yet unknown genetically determined immunodeficiency condition that affects the killing of intracellular bacteria such as BCG and Salmonella.
authors.affiliation: Unité d'Immunologie et Hématologie Pédiatrique, CHU Necker Enfants Malades, Paris, France.
authors.forename: J L
authors.initials: JL
authors.lastname: Casanova
authors.forename: S
authors.initials: S
authors.lastname: Blanche
authors.forename: J F
authors.initials: JF
authors.lastname: Emile
authors.forename: E
authors.initials: E
authors.lastname: Jouanguy
authors.forename: S
authors.initials: S
authors.lastname: Lamhamedi
authors.forename: F
authors.initials: F
authors.lastname: Altare
authors.forename: J L
authors.initials: JL
authors.lastname: Stéphan
authors.forename: F
authors.initials: F
authors.lastname: Bernaudin
authors.forename: P
authors.initials: P
authors.lastname: Bordigoni
authors.forename: D
authors.initials: D
authors.lastname: Turck
authors.forename: A
authors.initials: A
authors.lastname: Lachaux
authors.forename: M
authors.initials: M
authors.lastname: Albertini
authors.forename: A
authors.initials: A
authors.lastname: Bourrillon
authors.forename: J P
authors.initials: JP
authors.lastname: Dommergues
authors.forename: M A
authors.initials: MA
authors.lastname: Pocidalo
authors.forename: F
authors.initials: F
authors.lastname: Le Deist
authors.forename: J L
authors.initials: JL
authors.lastname: Gaillard
authors.forename: C
authors.initials: C
authors.lastname: Griscelli
authors.forename: A
authors.initials: A
authors.lastname: Fischer
mesh_terms: D001500:BCG Vaccine / Q000009:adverse effects; D002423:Cause of Death; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D007223:Infant; D008297:Male; D009163:Mycobacterium bovis; D015995:Prevalence; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D014391:Tuberculosis, Miliary / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000209:etiology
chemical_list: D001500:BCG Vaccine