title: Fatal adenovirus type 7b infection in a child with Smith-Lemli-Opitz syndrome.
abstract: Adenovirus type 7 causes worldwide respiratory tract infections, mainly in children. Severe systemic infections can occur, especially in immunocompromised patients and in patients with underlying chronic diseases. This report describes the first case of a fatal disseminated adenovirus type 7 infection in a child with Smith-Lemli-Opitz syndrome, a rare autosomal recessive disorder due to a primary enzymatic defect in cholesterol metabolism. Nasopharyngeal secretions and autopsy specimens including liver, lung, pleural fluid, and rectum were collected for viral culture. Adenovirus serotype 7 strains were obtained from all anatomic sites, except the liver. All these clinical isolates were analyzed using restriction endonuclease digestion of the genome, identifying them as genome type 7b, a virulent type. In this case, the fatal evolution could have been accelerated by the presence of an immunodeficiency although immunodeficiency is not included in the definition of Smith-Lemli-Opitz syndrome. The frequent recurrent banal infections in Smith-Lemli-Opitz syndrome could be prevented by a cholesterol supplementation regimen. Finally, this report emphasizes the need for efficient therapy for disseminated adenovirus infections, especially for virulent genome types.
authors.affiliation: Laboratory of Virology, University Hospital Center La Milétrie, Poitiers, France.
authors.forename: A
authors.initials: A
authors.lastname: Beby-Defaux
authors.forename: L
authors.initials: L
authors.lastname: Maille
authors.forename: S
authors.initials: S
authors.lastname: Chabot
authors.forename: A
authors.initials: A
authors.lastname: Nassimi
authors.forename: D
authors.initials: D
authors.lastname: Oriot
authors.forename: G
authors.initials: G
authors.lastname: Agius
mesh_terms: D000258:Adenovirus Infections, Human / Q000150:complications* / Q000175:diagnosis / Q000821:virology; D000260:Adenoviruses, Human / Q000145:classification* / Q000235:genetics / Q000302:isolation & purification*; D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D009305:Nasopharynx / Q000821:virology; D019082:Smith-Lemli-Opitz Syndrome